Management of Intersexuality

Guidelines for dealing with individuals with ambiguous genitalia

by Milton Diamond, Ph.D. and H. Keith Sigmundson, M.D.

from the Archives of Pediatrics and Adolescent Medicine

Following publication of our paper on a classic case of sex reassignment [1] the media attention was rapid and widespread e.g., [2-4] and so too the reaction of many clinicians.

Some wanted to comment or ask questions but many contacted us directly or indirectly [e.g., [5] asking for specific guidelines on how to manage cases of traumatized or ambiguous genitalia.

Below we offer our suggestions. We first, however, add this caveat: These recommendations are based on our experiences, the input of some trusted colleagues, the comments of intersexed persons of various etiologies and the best interpretation of our reading of the literature. Some of these suggestions are contrary to today's common management procedures. We believe, however, that many of those procedures should be modified. These guidelines are not offered lightly. We anticipate that time and experience will dictate that some aspects be changed and such revisions will improve the next set of guidelines to be offered. Underlying our presentation is the key belief that the patients themselves must be involved in any decision as to something so crucial to their lives. We accept that not every one will welcome this opportunity or these suggestions.

GUIDELINES

Foremost, we advocate use of the terms "typical," "usual," or "most frequent" where it is more common to use the term "normal." When possible avoid expressions like maldeveloped or undeveloped, errors of development, defective genitals, abnormal, or mistakes of nature. Emphasize that all of these conditions are biologically understandable while they are statistically uncommon. It helps in discussion with parents and child that they come to accept the genital condition as normal although atypical. Individuals with these genitalia are not freaks but biological varieties commonly referred to as intersexes. Indeed, it is our understanding of natural diversity that a wide offering of sex types and associated etiologies should be anticipated (see e.g., [6, 7] ). Our overall theme is to destigmatize the conditions.

1) In all cases of ambiguous genitalia, to establish most probable cause, do a complete history and physical. The physical must include careful evaluation of the gonads and the internal as well as external genital structures. Genetic and endocrine evaluations are usually needed and interpretation can require the assistance of a pediatric endocrinologist, radiologist and urologist. Pelvic ultrasonography and genitography may be required. Do not hesitate to seek expert help; a team effort is best. The history must include assessment of the immediate and extended family.

Be rapid in this decision making but take as much time as needed. Hospitals should have established House Staff Operating Procedures to be followed in such cases. Many consider this a medical emergency (and in cases of electrolyte imbalance this may be immediately so) nevertheless, we believe that most doubt should be resolved before a final determination is made. We simultaneously advise that all births be accompanied by a full genital inspection. Many cases of intersex go undetected.

2) Immediately, and simultaneously with the above, advise parents of the reasons for the delay. Full and honest disclosure is best and counseling must start directly. Insure that the parents understand this condition is a natural variety of intersex that is uncommon or rare but not unheard of. Convey strongly to the parents that they are not at fault for the development and the child can have a full, productive and happy life. Repeat this counseling at the next opportunity and as often as needed.

3) The child's condition is nothing to be ashamed of but it is also nothing to be broadcast as a hospital curiosity. The child and family confidentiality must be respected.

4) In the most common cases, those of hypospadias and congenital adrenal hyperplasia (C.A.H.) diagnosis should be rapid and clear. In other situations, with a known diagnosis, declare sex based on the most likely outcome for the child involved. Encourage the parents to accept this as best; their desire as to sex of assignment is secondary. The child remains the patient. When assignment is based on the most likely outcome, most children will adapt and accept their gender assignment and it will coincide with their sexual identity.

5) The sex of assignment, when based on the nature of the diagnosis rather than only considering the size or functionality of the phallus, respects the idea that the nervous system involved in adult sexuality has been influenced by genetic and endocrine events that will most likely become manifest with or after puberty. In the majority of cases this sex of assignment will indeed be in concert with the appearance of the genitalia (e.g., in A.I.S. [8] . In certain childhood situations, however, such assignment will be counter to the genital appearance (e.g., for reductase deficiency [9] . Our concern is primarily how the individual will develop and prefer to live post puberty when he or she becomes most sexually active.




Rear as male:

XY individuals with Androgen Insensitivity Syndrome (A.I.S.) (Grades 1-3)

XX individuals with Congenital Adrenal Hyperplasia (C.A.H.) with extensively fused labia and a penile clitoris

XY individuals with Hypospadias

Persons with Klinefelter syndrome

XY individuals with Micropenis

XY individuals with 5-alpha or 17-beta reductase deficiency

Rear as female:

XY individuals with Androgen Insensitivity Syndrome (A.I.S.) (Grades 4-7)

XX individuals with Congenital Adrenal Hyperplasia (C.A.H.) with hypertrophied clitoris

XX individuals with Gonadal dysgenesis

XY individuals with Gonadal dysgenesis

Persons with Turner's syndrome

For those individuals with mixed gonadal dysgenesis (MGD) assign male or female dependent upon the size of the phallus and extent of the labia/scrotum fusion. The genital appearance of individuals with MGD can range from that of a typical Turner's syndrome to that of a typical male. Evaluation of high male-like testosterone levels in these cases is also rationale for male assignment.

True hermaphrodites should be assigned male or female dependent upon the size of the phallus and extent of the labia/scrotum fusion. If there is a micropenis, assign as male.

Admittedly, in some cases a clear diagnosis is not possible, the genital appearance will seem equally male as female and prediction as to future development and gender preference is difficult. There is little evidence a poorly functioning clitoris and vagina is any better than a poorly functioning penis and there is no higher reason to save the reproductive capacity of ovaries over testes. In such difficult cases, whichever decision is made, the likelihood of the individual independently switching gender remains. The medical team in such cases will be taxed to make the best management decision.

6) While sex determination is ongoing, the hospital administration can wait for a final diagnosis before entering a sex of record and Staff can refer to the child as "Infant Jones" or "Baby Brown." After a sex designation has been made, naming and registration can occur. In those cases mentioned above, where prediction of future outcome is in doubt, parents might consider that a name be used that is appropriate for either males or females (e.g., Lee, Terry, Kim, Francis, Lynn, etc.).

7) Perform no major surgery for cosmetic reasons alone; only for conditions related to physical/medical health. This will entail a great deal of explanation needed for the parents who will want their children to "look normal." Explain to them that appearances during childhood, while not typical of other children, may be of less importance than functionality and post pubertal erotic sensitivity of the genitalia. Surgery can potentially impair sexual/erotic function. Therefore such surgery, which includes all clitoral surgery and any sex reassignment, should typically wait until puberty or after when the patient is able to give truly informed consent.

Major prolonged steroid hormone administration (other than for management of C.A.H.) too should require informed consent. Many intersex or sex reassigned individual's have felt they were not consulted about their use and effects and regretted the results.

8) In individuals with A.I.S, do not remove gonads for fear of potential tumor growth; such tumors have not been reported to occur in prepubertal children. Retention of the gonads will forestall the need for hormone replacement therapy and possibly help reduce osteoporosis.

Furthermore, delaying gonadectomy until after puberty will allow the young woman to come to terms with her diagnosis, understand the reason for her surgery and participate in the decision.

Advice regarding gonad removal from true hermaphrodites, individuals with streak gonads and others where malignancies can potentially occur is not so clear. Prophylactically it is common to remove these early; particularly in cases of gonadal dysgenesis [10, 11] .

Watchful waiting with frequent checks is always prudent [12] . Our suggestion, whenever the gonads are removed, is to explain as best as possible why the procedure is needed and attempt to get consent. If the child is too young to understand the reason for the surgery, its necessity should be explained as early as possible.

9) In rearing, parents must be consistent in seeing their child as either a boy or girl; not neuter. In our society intersex is a designation of medical fact but not yet a commonly accepted social designation. With age and experience, however, an increasing number of hermaphroditic and pseudohermaphroditic individuals are adopting this identification. In any case, advise parents to allow their child free expression as to choices in toy selection, game preference, friend association, future aspirations and so forth.




10) Offer advice and tips on how to meet anticipated situations, e.g., how to deal with grandparents, siblings, baby sitters and others that might question the child's genital appearance (e.g., "He/she is different but normal. When the child is older he/she and the doctors will do what seems best.") Parents should minimize the opportunities for such questioning by strangers.

11) Be clear that the child is special and, in some cases might, before or after puberty, accept life as a tomboy or a sissy or even switch gender altogether. The individual may demonstrate androphilic, gynecophilic or ambiphilic orientation. These behaviors are not due to poor parental supervision but will be related to an interaction of the biological, psychological, social and cultural forces to which a child with intersexuality is subject. Some individuals will be quite sexually active and others will be altogether reserved and have little or no interest in sexual relationships.

12) The patient's special situation will require guidance as to how to meet potential challenges from parents, peers and strangers. He or she will need love and friendly support.

Not all parents will be helpful, understanding, or benign and childhood, adolescent, and adult peers can be cruel. Positive peer interaction should be facilitated and encouraged.

13) Maintain contact with family so that counsel is available particularly at crucial times.

Counseling should be multi-staged (at birth, and at least again at age two, at school entry, prior to and during pubertal changes, and yearly during adolescence) and it should be detailed and honest. Counseling should be straight-forward, neither patronizing or paternalistic, to parents and to the child as he or she develops with as much full disclosure as the parents and child can absorb. The counseling should ideally be by those trained in sexual/gender/intersex matters.

14) As the child matures there must be opportunity for private counseling sessions and it is essential the door remains open for additional consultation as needed. On the one hand, the full impact of the situation will not always be immediately apparent to the parents or child. On the other hand, they might magnify the developmental potential of the genital ambiguity. As above, the counseling should ideally be by those trained in sexual/gender/intersex matters.

15) Counseling must include developmental sequelae to be anticipated. This should be along medical/biological lines and along social/psychological lines. Do not avoid honest and frank talk of sexual and erotic matters. Discuss the probabilities of puberty such as the presence or absence of menses and the potential for fertility or infertility. Contraception advice may be needed and safe-sex advice is always warranted. Certainly the full gamut of heterosexual, homosexual, bisexual and even celibate options --however these are interpreted by the patient-- must be offered and candidly discussed. Adoption possibilities can be broached for those that will be infertile. It is better to discuss these issues early rather than late. Do not obfuscate; knowledge is power enabling the individuals to structure their lives accordingly.

16) The family should be encouraged to openly discuss the situation among themselves, with and without a counselor present, so the child and parents can honestly come to terms with whatever the future holds. Parents have to understand their child's needs and feelings and the child has to understand the concerns of the parents.

17) As early as possible put the family in touch with a support group. There are such groups for individuals with Androgen Insensitivity Syndrome, Congenital Adrenal Hyperplasia, Klinefelter Syndrome, and Turner's Syndrome. Intersexed individuals as a whole (hermaphrodites and pseudohermaphrodites of all etiologies) have a support group, the Intersex Society of North America [addresses for these groups are listed below]. It is emphasized that one on one contact with another person having similar experiences can be the most uplifting factor in an intersexed person's healthy development!

Individual groups or chapters might be more inclined toward parental concerns while others might be tilted toward the intersexed person's concerns. Both perspectives are needed and separate meetings for each faction are useful. Parents need to talk about their feelings in an environment free of intersexed children and adults and the intersexed children and adults similarly need to be able to discuss their feelings and concerns free of their parents. There are times when it is appropriate for physicians to be present and times when it is not.

18) Keep genital inspection to a minimum and request permission for inspection even from a child. Hold in mind that a child may not feel able to deny a physician's request even though that might be his/her wish. The individuals must come to realize that their genitals are their own and they, not the doctors, parents or anyone else, have control over them. Allow others to view the patient only with his or her permission. Often the genital inspections themselves become traumatic events.

19) Let the child grow and develop as normally as possible with a minimum of interference other than needed for medical care and counseling. Let him/her know that help is available if needed. Listen to the patient; even when as a child. The physician should be seen as a friend.

With increasing maturity the designation of intersex may be acceptable to some and not to others. It should be offered as an optional identity along with male and female.

20) As puberty approaches be open and honest with the endocrine and surgical options and life choices available. Be candid at the sexual/erotic and other trade-offs involved with surgery or gender change and insure that any decision finally be that of the fully informed individual regardless of age. To have him/her discuss the treatment with someone who has undergone the procedure is ideal.




21) Most individuals are convinced by the age of 10-15 as to the direction that would be most suitable for them; male or female. Some decisions, however, should be stalled as long as possible to increase the likelihood that the individual has some experience with which to judge. For instance, a female with a phallic clitoris, sexually inexperienced with partner or masturbation, may not realize the loss in genital sensitivity and responsivity that can accompany cosmetic clitoral reduction. Insure that sufficient information is provided to aid in any decision.

22) Most intersex conditions can remain without any surgery at all. A woman with a phallus can enjoy her hypertrophied clitoris and so can her partner. Women with the androgen insensitivity syndrome or virilizing congenital adrenal hyperplasia who have smaller than usual vaginas can be advised to use pressure dilation to fashion one to facilitate coitus; a woman with partial A.I.S. likewise can enjoy a large clitoris. A male with hypospadias might have to sit to urinate without mishap but can function sexually without surgery. An individual with a micropenis can satisfy a partner and father children.

There is disagreement as to whether gonads that might prove masculinizing or feminizing at puberty should be removed early on to prevent such changes in a child that does not desire such changes. The disagreement involves the concept that the individual faced with such changes might actually come to prefer them to the habitus of rearing but will only become aware of them post hoc. Our bias is to leave them in so any genetic-endocrine predisposition imposed prenatally can come to be activated with puberty. We admit, however, there is no good body of clinical data from which the best prognosis can be made in such cases. There are some indications, however, that even without the onads the adrenals might prod pubertal changes.

23) If a gender change is being considered, have the individual experience a real-life living test (see e.g., [13, 14] ). In this way the individual will have first hand experience in how it actually is to live in the other role. Experience has shown that most indeed make the switch permanent but some return to their original sex of rearing. Some, usually as adults, will accept an identity as an intersex and plot their own course.

24) Maintain accurate medical, surgical, and psychotherapy records of all aspects of each case. This will facilitate whatever treatment is needed and assist in future research to enhance management of subsequent intersex cases. These records should be available to the patient.

Whenever possible, long term follow-up evaluations, e.g., at 5, 10, 15, and even 20 years of age, should become part of the record.

25) Last, we believe we have to be "authorities" in providing information and advice to the best of our ability yet not be "authoritarian" in our actions. We must allow the postpubertal individual time to consider, reflect, discuss and evaluate and then, have the last word in his or her genital modification and gender role and final sex assignment.

FINAL COMMENT

We are often asked about those intersexed individuals that have had early surgery of one sort or another, or even sex reassignment, and gone on to be happy and lead successful lives. Doesn't that demonstrate the wisdom of past practices? Our response: Humans can be immensely strong and adaptable. Certainly some intersexed individuals can, in dignity, maintain themselves in a manner that they neither would have chosen nor in which they feel comfortable -- as have others with a life condition from birth that cannot be changed (from cleft palate to meningomyelocele).

Many can adjust to surgery and reassignment for which they were not consulted and many have learned to accept secrecy, misrepresentations, white and black lies and loneliness.

People make life accommodations every day and try to better their lot for tomorrow.

We are aware of individuals that have come to terms with their life regardless of how stressed or painful. To them we offer our praise and admiration for their fortitude, strength and courage. Similarly we do the same for those that have rebelled against their circumstances and changed their lives with elective sex reassignment, surgery or whatever [15].

However, unlike individuals who have been given neonatal surgery for cleft palate or meningomyelocele, many of those who have had genital surgery or been sex reassigned neonatally have complained bitterly of the treatment. Some have sex reassigned themselves. Others treated similarly have reasons not to make an issue of the matter but are living in silent despair but coping.

The suggestions and guidelines we present are an attempt to consider ways to better life and adjustment for those intersexed and genitally traumatized persons still battling with these issues and for those yet to come.

Milton Diamond, Ph.D., is Director of the Pacific Center for Sex and Society, University of Hawaii at Manoa, John A. Burns School of Medicine, Department of Anatomy and Reproductive Biology, 1951 East-West Road Honolulu, HI 96822




 

next: Sexual Scientists Question Medical Treatment of Hermaphroditism

 

REFERENCES

We have purposely kept our references limited to facilitate use of these guidelines and reduce

complexity.

1. Diamond M, Sigmundson HK: Sex Reassignment at Birth: A Long Term Review and Clinical Implications. Archives of Pediatrics and Adolescent Medicine 1997; 151(March): 298-304.

2. Leo J: Boy, girl, boy again. U.S. News World Report, 1997; 17.

3. Gorman C: A boy without a penis. Time, vol. 1997, 1997; 83.

4. Angier N: Sexual identity not pliable after all, report says. New York Times 1997 14 March 1997; A1, A18.

5. Benjamin JT: Letter -to-editor. Archive of Pediatric and Adolescent Medicine 1997; 151.

6. Fausto-Sterling A: The Five Sexes: Why Male and Female Are Not Enough. The Sciences 1993; 1993(March/April): 20-25.

7. Diamond M, Binstock T, Kohl JV: From fertilization to adult sexual behavior. Hormones and Behavior 1996; 30(December): 333-353.

8. Quigley C, De Bellis A, Merschke KB, El-Awady MK, Wilson EM, French FS: Androgen Receptor Defects: Historical, Clinical and Molecular Perspectives. Endocrine Reviews 1995; 16(3): 271-321.

9. Imperato-McGinley J: 5-alpha-reductase deficiency. In: Bardin CW, ed. Current Therapy in Endocrinology and Metabolism, 5th ed. St. Louis, Mo.: C. V. Mosby, 1994; 351-354.

10. Donahoe PK, Crawford JD, Hendren WH: Mixed gonadal dysgenesis, pathogenesis, and management. Journal of Pediatric Surgery 1979; 14: 287-300.

11. McGillivray BC: Genetic aspects of ambiguous genitalia. Pediatric Clinics of North America 1992; 39(2): 307-317.

12. Wright NB, Smith C, Rickwood AM, Carty HM: Imaging children with ambiguous genitalia and intersex states. Clinical Radiology 1995; 50(12): 823-829.

13. Clemmensen LH: The "Real-life Test" for Surgical Candidates. In: Blanchard R, Steiner

BW, eds. Clinical Management of Gender Identity Disorders in Children and Adults, vol. 14.

Washington, D.C.: American Psychiatric Press, 1990; 121-135.

14. Meyer JK, Hoopes JE: The Gender Dysphoria Syndromes: A Position Statement on So-Called Transsexualism. Plastic and Reconstructive Surgery 1974; 54: 444-451.

15. Diamond M: Sexual Identity and Sexual Orientation in Children With Traumatized or Ambiguous Genitalia. Journal of Sex Research 1997; 34(2): 199-222.

 

INTERSEX SUPPORT GROUPS

For addresses or contact with groups outside the United States contact one of the groups below.

 

AIS (Androgen Insensitivity Syndrome) Support Group of the U.S.

A support group for those with AIS, also their family and partners.

4203 Genessee Ave. #103-436

San Diego, CA 92117-4950

Phone: (619) 569-5254

email: aissg@aol.com

Ambiguous Genitalia Support Network

A support group for parents and others.

428 East Elm St. #4D

Lodi, CA 95240

 




 

CAH Support Groups

 

For individuals or families with congenital adrenal hyperplasia

National Adrenal Diseases Foundation

505 Northern Boulevard

Great Neck, NY 11021

Phone: (516) 487-4992

web site: http://medhlp.netusa.net/www/nadf.htm

Congenital Adrenal Hyperplasia Support Association

 

1302 County Road 4

Wrenshall, MN 55797

Phone: (218) 384-3863

H.E.L.P. (Hermaphrodite Education and Listening Post)

A support group for parents and others affected by any sex differentiation disorder.

PO Box 26292

Jacksonville, FL 32226

web site: http://www.isna.org/faq.html#anchor643405

email: help@southeast.net

Intersex Society of North America

A peer support and advocacy group of and for intersexuals.

PO Box 31791

San Francisco CA 94131

email: info@isna.org

web site: http://www.isna.org

K. S. Associates (Klinefelter syndromes of all variety)

A support and education group for families and professionals dealing with Klinefelter syndrome.

P.O. Box 119

Roseville, CA 95661-0119

web site: http://www.genetic.org/

email: ks47xxy@ix.netcom.com

Turner's Syndrome Society of the U.S.

A support group for those with Turner's syndrome, their family and friends.

1313 Southeast 5th Street (Suite 327)

Minneapolis MN 55414

Phone: 1-(800) 365-9944

Fax: (612) 379-3619

web site: http://www.turner-syndrome-us.org

 

 




GENERAL SUPPORT GROUPS

National Organization for Rare Disorders (NORD)

 

Support and educational group for those concerned with any rare disorder:

P.O. Box 8923

New Fairfield, CT 06812-8923

Phone: (800) 999-NORD

Fax: (203) 746-6518

http://www.pcnet.com/~orphan/

Our-kids

Support group for parents of children with any sort of special need:

web site: http://wonder.mit.edu/our-kids.html

PFLAG (Parents and Friends of Lesbians and Gays)

A support group for parents and friends of lesbians and gays.

1012-14th Street NW, Suite 700

Washington, DC 20005

Phone: (202) 638-4200

email: PFLAGNTL @ aol.com

 

SEXUALITY/GENDER/INTERSEX COUNSELORS

Appropriate counselors might be contacted at one of these national organizations.

 

American Academy of Clinical Sexologists (AACS)

P.O. Box 1166

Winter Park, Florida 32790-1166

Phone: (800) 533-3521

Fax: (407) 628-5293

American Association of Sex Educators, Counselors and Therapists (AASECT)

P.O. Box 238, Mount Vernon, Iowa 52314

Phone (319) 895-8407

Fax (319) 895-6203

Society for the Scientific Study of Sexuality (SSSS)

P.O. Box 208, Mount Vernon, Iowa 52314

Phone (319) 895-8407

Fax (319) 895-6203

Society for Sex Therapy and Research (SSTAR)

Secretary: Blanche Freund, Ph.D., R.N.

419 Poinciana Island Drive

N. Miami Beach FL 33160-4531

Phone: 305 243-3113

Fax 305 919-8383



next: Sexual Scientists Question Medical Treatment of Hermaphroditism
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APA Reference
Staff, H. (2007, August 9). Management of Intersexuality, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/management-of-intersexuality

Last Updated: March 15, 2016

Genital Surgery On Intersexed Children

This letter was sent from Cheryl Chase, Exec. Dir., Intersex Society of North America to a judge in Columbia, South America.

7 February 1998

Mr. Rodrigo Uprimny
Corte Constitucional
Calle 72 No 7-96
Bogotá
COLOMBIA SOUTH AMERICA

Dear Mr. Uprimny,

Thank you for providing the opportunity to comment on this case. As I understand the case, physicians have asked for the Court to either approve performing genital surgery on a six year old intersexed child, or to wait and allow the child to make any decisions about surgery herself, when she is old enough to evaluate risks and benefits. Apparently the surgery contemplated is clitoral reduction, vaginoplasty (to create or deepen a vagina), or both. In a previous case regarding an emasculated boy, the Court determined that all choices involving sexual identity must be made directly by the person, and not by the parents.

We argue, in keeping with the Court's previous determination, that only the child has the right to make decisions regarding her sexual identity and cosmetic genital surgery. To impose surgery on her would subject her to an unnecessary risk of irreversible harm and violate her human rights.

During the past several years, there has been an explosion of new scholarly work which considers medical management of intersex children, and the surrounding psychosocial issues. Based upon that work, a growing consensus of surgeons, psychologists, psychiatrists, and ethicists argue against early genital surgery on intersex children (Diamond 1996; Diamond and Sigmundson 1997b; Dreger 1997a; Dreger 1998 forthcoming-a; Drescher 1997; Kessler 1998 forthcoming; Schober 1998). It would be a pity for the Court to create a precedent insulating doctors from any liability for harm caused by performing non-consensual genital surgery on children precisely at the moment when scholarly opinion is changing. It would be even more ironic for the Court at this moment to reverse its previous opinion, and negate the right of a child to make for herself all decisions regarding her sexual identity.

Given the fact that genital surgery is not medically necessary, that it is irreversible and potentially harmful, that there is growing controversy among medical intersex specialists, and that the child can always choose surgery if she wishes when she is old enough to give informed consent, to impose surgery now would violate the first principle of medicine: "Primum, non nocerum" (First, do no harm).

Please refer also to enclosed Declarations from Cheryl Chase (Director of the Intersex Society), Justine Schober M.D. (pediatric urological surgeon), Alice Dreger Ph.D. (narrative ethicist), and Lisset Barcellos Cardenas (a Peruvian woman subjected to nonconsensual genital surgery at age 12). All of these argue that cosmetic genital surgery should never be performed without the express informed consent of the patient. Also enclosed is a letter in the original Spanish from Ms. Barcellos to her doctor in Lima, insisting that this practice is harmful, unethical, and must be stopped.

1. There is no medical reason to reduce the size of a large clitoris. Large clitorises do not cause illness or pain. The sole motivation for the surgery is the unproven belief that it may enhance psychological well-being. There is no medical reason to create or deepen a vagina in a pre-pubescent child. The sole motivation for such surgery is the unproven belief that it may ease parental discomfort now or that the decision would be traumatic for the patient to make later, so the surgery should be performed before she is able to participate in the decision.

2. The surgery is irreversible. Tissue removed from the clitoris can never be restored; scarring produced by surgery can never be undone. Setting potential and speculative "psychological" benefits aside, there no medical advantage or benefit to performing surgery now as opposed to later, when the child can make her own choice and when her gender identity is clearly established. "Surgery makes parents and doctors comfortable, but counseling makes people comfortable too, and it is not irreversible" (Schober 1998, p20).

There are, in fact, clear medical benefits to delaying the surgery. When she is grown, her genitals will be larger and thus easier for a surgeon to work on. One reason for poor surgical outcomes may be that scar tissue is negatively affected by the changes in size and shape that accompany normal growth and pubertal development; surgery performed after puberty would avoid that risk. It is likely that surgical techniques will have improved by the time she has grown; waiting will allow her to benefit from advances in technology.

There are many documented cases of people with her history who lived as adult women and were happy to keep their large clitoris intact, in some cases actually refusing surgery when it was offered (Fausto-Sterling 1993; Young 1937).

There is clear documentation that a significant fraction of children with her specific medical condition and history develop a male gender identity, and live as men during adulthood. If she lives as a man, she will be grateful that surgery was not performed without her consent.

Physicians in this case have asserted that the child can never live as a man, because her penis is never going to be sexually functional. But sexual function may mean different things to different people. The boy in the previous case, who was accidentally emasculated, chose to live as a man even though he had lost his penis. The men investigated in (Reilly and Woodhouse 1989) were able to have satisfying lives as men, with no impairment of sexual function, with small penises that would be judged "inadequate" according to the medical protocols used on intersex children. A small penis is capable of providing sexual arousal, genital pleasure, and orgasm. The video tape "I Am What I Feel To Be" (Fama Film A.G. 1997) presents interviews in Spanish with a number of people who were born as male pseudo hermaphrodites, raised female, and later changed to live as men. Both they and their partners describe their lives as sexually fulfilling, in spite of penises so small that they lived as girls until puberty (Fama Film A.G. 1997).




3. There is considerable evidence that genital surgery can cause harm, including such physical harm as scarring, chronic pain, chronic irritation, reduction of sexual sensation, and psychological harm. Indeed, apart from the harm specific to genital surgery, surgery is never without risk.

4. No significant data has been collected on long term outcomes. The belief that these surgeries provide any benefit at all is speculative and unexamined. Given the clear risk of harm, the Court is obligated to protect the child's human rights by declining to approve the surgery.

5. The very fact that the physicians in this case hesitate to perform surgery before operating indicates that they are aware that the surgery is risky and may cause immediate or future harm.

6. Surgeons argue that genital surgeries must be performed on intersex children in order to save them from feeling different from other children, or being marginalized by society. But many children grow up with physical differences which may cause them to be marginalized by society, yet we do not advocate using plastic surgery to eliminate all physical differences. For instance, children of racial minorities are often marginalized, teased, and even subject to violence. Yet few would condone using non-consensual plastic surgery during infancy to eliminate racial characteristics.

Prejudice against people with unusual genitals is culturally determined. Some cultures have high regard for people with intersex genitals (Herdt 1994; Roscoe 1987). As even Dr. Maria New, a pediatric endocrinologist who advocates early genital surgery, concedes, our own culture was much less prejudiced before medical intervention began. [During the European Middle Ages and Renaissance,] "Hermaphrodites were integrated quite forthrightly into the social fabric" (New and Kitzinger 1993, p10).

But some surgeons who advocate early genital surgery for intersex infants might consider surgical elimination of racial characteristics potentially acceptable. Dr. Kenneth Glassberg, a surgeon who heads the Urology Section of the American Academy of Pediatrics, was interviewed on the national television news show NBC Dateline. He said that it was unrealistic to ask people to be accepting of genital difference, because many people are unaccepting of racial difference (Dateline 1997). Yet the law addresses the problem of racism by trying to mitigate the power of racists to harm members of racial minorities, rather than by trying to eliminate the physical characteristics which mark members of racial minorities.

Likewise, in this case, if there is intolerance of physical difference, then the intolerance should not be addressed by using medically unnecessary, irreversible, potentially harmful plastic surgery to try to hide the physical difference without the patient's consent. This is particularly true for a physical difference that is not visible to others in the course of normal social interaction.

7. There is good evidence that adults would not choose clitoral surgery for themselves. Psychologist Dr. Suzanne Kessler has documented this by surveying college students (Kessler 1997). There are many adult intersex women who express regret and anger that genital surgery was imposed on them as children.

8. Worldwide medical thinking about surgical management of intersexuality has been strongly influenced by a case in which a boy whose penis was accidentally destroyed during circumcision, and who after being surgically reassigned and raised female, was reported to have had a successful adjustment. However, it is now known that, like the previous case of accidental emasculation which the Court is considering, the female reassignment was a disaster (Diamond and Sigmundson 1997a). The patient now lives once again as a man, and reconsideration of this case is causing experts to assert that early genital surgery requires the informed consent of the patient (1997b; Diamond and Sigmundson 1997b; Dreger 1998 forthcoming-a). "I recommend that genital reconstruction be delayed until the individual is competent to decide for himself or herself how this should best be fashioned" (Diamond 1996). "This damage [due to surgery] may be something a patient is willing to risk, but that is a choice he/she should be able to make for him/herself" (Fausto-Sterling and Laurent 1994, p10).

9. A safer alternative is clearly available, and is endorsed by credible experts.

Sex researcher Milton Diamond of the University of Hawaii Medical School and psychiatrist Keith Sigmundson of the University of British Columbia, based upon their research of intersex management, provide clear recommendations for how doctors can best serve intersex children. They recommend that the parents' emotional difficulties about their child's intersexuality be treated by providing counseling for the parents, that ongoing counseling and honest information be provided to the intersex child in age-appropriate fashion as she grows, and that early genital surgery be avoided because it is irreversible and potentially harmful. "[The parents] desire as to sex of assignment is secondary. The child remains the patient." "Most intersex conditions can remain without any surgery at all. A woman with a phallus can enjoy her hypertrophied clitoris and so can her partner. Women with [intersex conditions] who have smaller-than-usual vaginas can be advised to use pressure dilation to fashion one to facilitate coitus; a woman with [an intersex condition] likewise can enjoy a large clitoris." "As the child matures there must be opportunity for private counseling sessions ... the counseling should ideally be done by those trained in sexual/gender/intersex matters" (Diamond and Sigmundson 1997b) .

Pediatric urology surgeon Dr. Justine Schober, in her review of clitoral reduction and vaginoplasty, concludes that "Surgery must be based on truthful disclosure and support decision-making by parents and patient. . . . Our ethical duty as surgeons is to do no harm and to serve the best interests of the patient" (Schober 1998).

Narrative ethicist Dr. Alice Dreger recommends that intersex patients be allowed to choose surgery only with full informed consent of the patient, and that counseling and peer support be made available to parents, family, and patient (Dreger 1997b).

10. Given the fact that genital surgery is not medically necessary, that it is irreversible and potentially harmful, that there is growing controversy among medical intersex specialists, and that the child can always choose surgery later if she wishes, to impose surgery now would violate the first principle of medicine: "Primum, non nocerum" (First, do no harm).

11. Many of the factors which determined the Court's decision in the case of the emasculated boy apply in exactly the same way in the present case. Just as in that case, there is no urgency to perform the surgery as evidenced by the fact that three years have now passed since the diagnosis and without surgery. Just as in that case, the child is unable to give the informed consent which is necessary before such an important and life-altering decision can be made for her. Just as in the previous case, there is no proof that this surgery would provide any benefit at all.




12. BOTH THE NUREMBERG CODE AND BASIC PRINCIPLES OF HUMAN RIGHTS LAW PROHIBIT SUBJECTING A CHILD TO INVOLUNTARY, IRREVERSIBLE, AND MEDICALLY UNNECESSARY GENITAL SURGERIES.

The sole purpose of these surgeries is to enhance the long-term psychological well-being of the patient. Yet there is no evidence that they do enhance the long-term psychological well-being of the patient, there is no data which assures that they preserve sexual sensitivity and orgasmic function, and considerable data implies that they may actually harm the long-term psychological well-being of the patient. Therefore, although these surgeries have been performed for many years, with numerous refinements of technique, and are considered by many surgeons to be standard practice, in pragmatic terms they should be considered experimental techniques which must not be imposed without the patient's full informed consent.

The Charter and the Judgment of the International Military Tribunal (IMT), collectively titled the Nuremberg Code, carry the weight of binding international law. See History of the United Nations War Crimes Commission and the Development of the Laws of War (1948) and Affirmation of the Principles of International Law Recognized by the Charter of the Nuremberg Tribunal, 1946-1947 U.N.Y.B. 54, U.N. Sales No. 1947.I.18. The very first trials held by the IMT at Nuremberg concerned the use of medical practices on unwilling subjects. The medical trials at Nuremberg in 1947 deeply impressed upon the world that medical intervention on unconsenting human subjects is morally and legally repugnant.

The Tribunal classified the commission of experimental medical practices without the consent of the patient both as war crimes and as crimes against humanity. See History of the United Nations War Crimes Commission and the Development of the Laws of War 333-334 (1948). The first principle of the Nuremberg Code provides the patient/subject with the right of informed consent: "The voluntary consent of the human subject is absolutely essential. This means that the peson involved should have legal capacity to give consent; should be so situtated as to be able to exercise free power of choice, without the intervention of any element of force, fraud, deceit, duress, over-reaching, or other ulterior form of constraint of coercion; and should have sufficient knowledge and comprehension of the elements of the subject matter involved as to enable him to make an understanding and enlightened decision." 2 Trials of War Criminals Before the Nuremberg Military Tribunals under Control Council Law No. 10, at 181-82 (1949). See also the Helsinki Declaration, adopted by the World Medical Association in 1964 (recognizing the principle of informed consent and the right to be free from involuntary medical intervention.)

The prohibition on involuntary medical intervention and the requirement of informed consent are absolute; the Nuremberg Code governs therapeutic research that is intended to directly benefit or provide effective medical therapy for the research subjects, as well as nontherapeutic research concerned with the discovery of data. (See previous citation.)

The Nuremberg Code prohibits involuntary surgical procedures designed to alter the genitals of a six year old child for purely esthetic as opposed to medically necessary reasons. As discussed in more detail in the preceding sections, these surgeries are plainly experimental: (1) They are not medically necessary to alleviate pain or any physiological dysfunction. (2) There is no medical consensus that these procedures are advisable or beneficial. On the contrary, there is growing concern over the efficacy and ethics of these procedures among medical experts in many fields. (3) There are no outcome studies to support the hypothesis that these painful, invasive, and irreversible surgical procedures result in any psychosocial benefit to the child or enhance the child's well-being in any way. Conversely, an increasing number of adults who were forced to undergo these procedures as children are coming forward to report profound physical and psychological harm, including pain, scarring, urological problems, loss of sexual sensation and functioning, and severe emotional trauma. (See Declaration of Lisset Barcellos Cardenas.)

The fundamental human right to be free of involuntary medical experimentation is especially clear and compelling under the circumstances of this case, which involves a six year old child who is incapable of providing informed consent. Although parents have the right to consent to medical treatments on behalf of a minor child under ordinary circumstances, this right does not apply (1) when the medical treatment is not necessary to alleviate illness or pain; (2) when the only rationale for the treatment is speculative and purely psychosocial, i.e., to alleviate the possibility of social stigma by physically altering a child's genitals to more closely conform to a cultural stereotype or ideal; (3) when the procedures involved are irreversible, painful, and may result in profound physical and/or emotional harm; and (4) where the irreversible outcome of the procedures will deprive the child of her right to determine her own sexual identity when she is old enough to choose.

It is repugnant and contrary to a child's basic human rights to allow a parent to consent to medically unnecessary genital surgery for the purpose of dictating the child's future gender identity or of altering the child's body to conform to an idealized cultural notion of "normal" genital appearance. This principle has been established in the analogous context of female genital mutilation, where a wide variety of human rights authorities and organizations have determined that involuntary genital surgery performed on female children violates basic human rights to bodily integrity and personal dignity and autonomy. See Amnesty International, Women's Rights are Human Rights (1995).

Many human rights bodies have condemned female genital mutilation, defined as the removal of all or part of the clitoris, inner labia, or outer labia. "Feminizing genital surgery" reduces the size of the clitoris by removing parts of the clitoris. (An earlier surgical technique which buried the clitoris has been abandoned because it results in pain upon genital arousal.) Clitoral reduction surgery is thus clearly covered by the definition of female genital mutilation. Female genital mutilation has been condemned by the United Nations Commission on Human Rights, UNICEF, the World Medical Association, the World Health Organization, the 1993 United Nations World Conference on Human Rights, and numerous non-governmental organizations. See especially the Minority Rights Group International, Female Genital Mutilation: Proposals for Change (1992): "While an adult woman is quite free to submit herself to a ritual or tradition, a child has no formed judgment and does not consent, but simply undergoes the operation while she is totally vulnerable."




There is no guarantee that the child will have a female gender identity as an adult. As discussed above, a significant fraction of children with her specific medical condition and history have a male gender identity as adults. If the child grows up to have a male gender identity, then the surgeries that the doctors seek to perform will have been a terrible mistake. Moreover, even if her adult gender identity is female, there is no guarantee that she will not regret any genital surgeries that were performed without her consent as a child especially given the uncertain outcome of current surgical techniques. Given the deeply personal and irreversible nature of genital surgeries, the child herself is the only person who has the right to weigh the risks and to decide what kind of genital alterations, if any, she would like to undergo.

Parents have considerable legal control over their children, but they do not have the right to disregard the child's intrinsic human rights to privacy, dignity, autonomy, and physical integrity by altering a child's genitals through irreversible surgeries based on an unproven and controversial psychosocial rationale. See, for example, the American Convention on Human Rights, Article 1 (stating that "every human being" is entitled to the rights and freedoms recognized in the Convention); Article 5 (recognizing the right to "physical, mental, and moral integrity"); Article 11 (recognizing the right to privacy); and Article 19 (stating that "every minor child has the right to the measures of protection required by his condition as a minor on the part of his family, society, and the state"). See, for example, United Nations Convention on the Rights of the Child (signed by Colombia 26 January 1990, ratified 28 January 1991), Article 19 (requiring all states "to protect the child from all forms of physical or mental violence, injury or abuse, neglect or negligent treatment, maltreatment or exploitation . . . while in the care of parent(s), legal guardian(s) or any other person who has the care of the child"); and Article 37 (requiring all states to ensure that "no child shall be subjected to torture or other cruel, inhuman or degrading treatment").

Summary

Therefore, we urge the Court not to approve the surgery, as a violation of the child's human rights as previously ruled by this Court and as guaranteed by international law, and specifically not to indemnify physicians against liability for what they obviously regard as a questionable procedure with a significant likelihood of resulting in regret, anger, and motivation to seek legal redress as the patient grows up and is able to take legal action on her own behalf.

Yours Truly,

Cheryl Chase
Executive Director, ISNA

PS: You specifically asked for a copy of the article "The Five Sexes," by Dr. Anne Fausto-Sterling. I have enclosed that article, but I would like to emphasize that, though the article is titled "Five Sexes," neither Dr. Fausto-Sterling nor I nor ISNA is suggesting that there are actually five sexes. Dr. Fausto-Sterling and ISNA support the recommendations of (Diamond and Sigmundson 1997b). In the current case, those recommendations indicate that the child should continue to be raised as a girl, but that no genital surgery be done unless at her own initiative and with her informed consent.

Appendix A

 

Feminizing Genital Surgery is Medically Unnecessary

 

"Our needs and the needs of the parents to have a presentable child can be satisfied. We argue that surgery in an infant maximizes a child's social adjustment and acceptance by the family. But do we truly realize and promote the best interest of the adult patient in terms of psychosocial outcomes? This knowledge is still obscure and much remains to be discovered" (Schober 1998, p19).

"The only indication for performing this surgery [clitoral reduction] has been to improve the body image of these children so that they feel 'more normal'" (Edgerton 1993).

"Scientific dogma has held fast to the assumption that without medical care hermaphrodites are doomed to a life of misery. Yet there are few empirical studies to back up that assumption, and some of the same research gathered to build a case for medical treatment contradicts it" (Fausto-Sterling 1993).

"The major justification for early surgery is the belief that children will suffer terrible psychological damage if they and those around them are not crystal clear about which sex they belong to. Surgically altering ambiguous genitalia is seen as an important component of clarifying the situation initially for family and friends, and as the child becomes conscious of his or her surroundings, for the child as well" (Fausto-Sterling and Laurent 1994, p8).

Hopkins surgeons justify early genital surgery because it "relieves parental anxiety about the child with relatives and friends" (Oesterling, Gearhart, and Jeffs 1987, p1081).

"For a small infant, the initial objective is to feminize the baby to make it acceptable to the parents and family" (Hendren and Atala 1995, p94).

"Although gender assignment by genital surgery reassures adults, it does not necessarily require surgery, based on anecdotal reports of untreated patients" (Drescher 1997).

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Appendix B

Long Term Outcomes of Feminizing Genital Surgery are Unknown

These surgeries have been widely practiced since the late 1950s. During that time there has been a disturbing lack of follow-up. Because it is not known whether these surgeries enhance psychological well-being, which is their sole legitimate purpose, these surgeries must be considered experimental.

In her forthcoming review of feminizing genital surgeries, pediatric urological surgeon Dr. Justine Schober notes that, "The psychosocial long-term outcomes represent the most necessary information to determine if we are successful in treating intersexual patients. However, in conditions other than congenital adrenal hyperplasia, outcomes are generally unavailable" (Schober 1998, p20).

In a forthcoming book, Dr. Suzanne Kessler, professor of Psychology at the State University of New York at Purchase, presents results from her ten year investigation of medical management of intersexuality. She notes that "Surprisingly, in spite of the thousands of genital operations performed every year, there are no meta-analyses from within the medical community on levels of success." "Even recent reports are susceptible to a criticism about vagueness: The clitoroplasty is a `relatively simple procedure that gave very good cosmetic results . . . and quite satisfactory results.' The reader searches in vain for any assessment by which that was determined." "In none of the follow-up studies is there any indication that a criterion for success includes the intersexed adult's reflection on his or her surgery" (Kessler 1998 forthcoming, p106-7).

Dr. William Reiner, who switched in mid-career from urological surgeon to pediatric psychiatrist, notes that "Past decisions about gender identity and sex reassignment when genitalia are greatly abnormal have by necessity occurred in a relative vacuum because of inadequate scientific data" (Reiner 1997a, p224).

Brown University Professor of Medicine Dr. Anne Fausto-Sterling, in her review of every case study located (in English, French, and German) on feminizing genital surgeries from the 1950s through 1994, concludes that "these standard treatment procedures are not based in careful clinical analysis"(Fausto-Sterling and Laurent 1994, p1).

"Long term results of operations that eliminate erectile tissue [that is, clitoral reduction surgery] are yet to be systematically evaluated" (Newman, Randolph, and Parson 1992).

Pediatric urologist Dr. David Thomas of the University of Leeds, addressing the American Academy of Pediatrics in late 1996, noted that very few studies have been done to gauge the long-term results of early feminizing surgery, and the psychological issues "are poorly researched and understood" (1997a).

Hopkins Pediatric urological surgeon Robert Jeffs, reacting to picketers demonstrating against early genital surgeries at a 1996 Boston meeting of the American Academy of Pediatrics, conceded to a journalist that he has no way of knowing what happens to patients after he performs surgery on them. "Whether they are silent and happy or silent and unhappy, I don't know" (Barry 1996).

"Although these procedures have been performed for decades, no controlled studies have compared the adaptations of children who had surgery to those who did not. Anecdotal reports [that is, reports of former patients including intersex activists] carry much weight in an area in which data on long-term outcomes are sparse" (Drescher 1997).

The very fact that, in the current case, physicians hesitate to proceed without the approval of the Court, is evidence that they consider the procedure risky and likely to motivate the patient to later litigation.

Appendix C

Feminizing Genital Surgery Can Cause Harm

There is a wealth of evidence that these surgeries can cause profound physical and emotional harm.

See the attached Declaration of Lisset Barcellos Cardenas, which describes reduced sexual sensation, chronic irritation and bleeding, and abnormal appearance after cosmetic genital surgery imposed without her consent in Lima Peru in approximately 1981. Ms. Barcellos would be happy to address the Court, in her native Spanish, on the ways in which surgery has decreased her quality of life and her belief that these surgeries should never be imposed on unconsenting children.

Dr. Anne Fausto-Sterling documents scarring, pain, multiple surgeries, and patient or parental refusal of additional surgeries as evidence that surgery does actual harm (Fausto-Sterling and Laurent 1994,p5).

In a recent review of a dozen girls aged 11 to 15 who had undergone clitoroplasty and vaginoplasty, Dr. David Thomas concluded "The results are indifferent and, frankly, disappointing" with reconstructions showing visibly different appearance from the original cosmetic result, clitorises withered and obviously nonfunctional, and "every girl required some additional vaginal surgery"(1997a).

Angela Moreno, who was subjected to modern clitoroplasty by experienced surgeons in 1985, recounts that the surgery destroyed her orgasmic function (Chase 1997, p12).

"Surgical reduction of an enlarged clitoris can at times damage sensation and thus reduce orgasmic potential and genital pleasure and, like ablation of the testes, is irreversible" (Reiner 1997b, p1045).

"Aside from reducing potential adult genital sensitivity, [clitoral reductions] neglect the significance of any behavioral or psychological predisposition toward the individual's own preferred sexual identity or gender roles" (Diamond 1996, p143).

Sex therapist Dr. H. Martin Malin discusses patients who had been subjected to early genital surgeries. "[their conditions, such as micropenis or clitoral hypertrophy] were not life-threatening or seriously debilitating. . . . [T]hey were told that they had vaginoplasties or clitorectomies because of the serious psychological consequences they would have suffered if surgery had not been done. But the surgeries had been performed and they were reporting long-standing psychological distress" quoted in (Schober 1998).

"[S]urgery not only risked problems in psychological adjustment, but also can permanently damage the individual's ability to achieve orgasmic sexual function. This damage may be something a patient is willing to risk, but that is a choice he/she should be able to make for him/herself" (Fausto-Sterling and Laurent 1994, p10).

Hopkins surgeons Oesterling, Gearhart, et al have recently acknowledged in the Journal of Urology that the most modern clitoral surgery "does not guarantee normal adult sexual function" (Chase 1996).

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Appendix D

Women can be well adjusted with large clitorises

There is no evidence that these surgeries are required for healthy psychosocial development. Indeed, there are many counterexamples, of people who lived or are living happily without surgery.

Historian Alice Dreger has documented many male pseudo hermaphrodites who lived happily as women during the 19th century with atypical genitals intact (Dreger 1998 forthcoming-b).

Anne Fausto-Sterling documents 70 cases of children who grew up with ambiguous genitalia, most of whom seem to have developed ways of coping with their anatomical difference (Fausto-Sterling and Laurent 1994).

Hopkins surgeon Hugh Hampton Young documents a number of women with large clitorises who were sexually active and who rejected his offers of surgical correction (Fausto-Sterling 1993; Young 1937).

The video, Hermaphrodites Speak!, contains an interview (at 24:35 on the tape) with Hida Viloria, a young woman who discusses in a video interview how happy she is to have been able to keep her large clitoris intact (ISNA 1997).

Eli Nevada also discusses her relief at having escaped genital surgery (Nevada 1995).

"Despite a large clitoris [this patient] does not wish any [surgical] modifications to be made" (Patil and Hixson 1992).

Appendix E

Some male intersexuals raised female switch to male sex role

There is evidence that some male pseudo-hermaphrodites, even if raised female, even if subjected to genital surgery, and in spite of having an "inadequate" penis, will change sex role during adolescence or early adulthood, living as men rather than as women.

Money found that three (10%) of 23 patients who were male pseudo- hermaphrodites raised female switched to living as men as adults (Money, Devore, and Norman 1986). Dr. Howard Devore, a co-author of this study, is a clinical psychotherapist with extensive experience in assisting intersexual patients and parents of intersexual children. Dr. Devore is an outspoken opponent of early genital surgery and a member of ISNA's advisory board.

"In fact, present data is increasing that despite great care in rearing these [male pseudo hermaphrodites] as females, some, or perhaps many of them, have strong male tendencies or may even change their assigned sex when they reach 12 to 14 years of age" (Reiner 1997a, p224). Dr. Reiner is engaged in a prospective investigation of fifteen male pseudo hermaphrodites assigned and raised female, with early genital surgery. To date, two out of the seven who have reached adolescence have declared themselves male. The other eight are too young yet for any assessment (1997b). Reiner reports a similar case, without prospective investigation, in (Reiner 1996).

Even female pseudo-hermaphrodites assigned and reared female, with early genital surgery, are considerably likely to switch to living as men as adults (Meyer-Bahlburg et al. 1996).

The video tape "I Am What I Feel To Be" (Fama Film A.G. 1997) presents interviews in Spanish with a number of people who were born as male pseudo hermaphrodites, raised female, and later switched to live as men (Fama Film A.G. 1997).

Appendix F

Men can be well adjusted with small penises

Surgeons Justine Schober M.D. (neé Reilly) and C R J Woodhouse M.D. interviewed 20 patients who were diagnosed in infancy with micropenis. Twelve of these patients were adults (17 years of age or older) at the time of the interview. All had stretched penis length smaller than the 10th percentile one was only 4 cm (erect penis length cannot exceed stretched flaccid penis length). "The group appears to form close and long-lasting relationships. They often attribute partner sexual satisfaction and the stability of their relationships to their need to make extra effort including nonpenetrating techniques. . . . The small penis has not deferred them from a male sexual role. [Nine of twelve of the adult patients] are already sexually active. . . . Vaginal penetration usually is possible but adjustment of position or technique may be necessary. . . . Two main conclusions may be drawn from our series: a small penis does not preclude normal male role and a micropenis or microphallus alone should not dictate a female gender assignment in infancy" (Reilly and Woodhouse 1989).

"My own experience is that men with the smallest and most deformed penis can have a satisfying relationship with their partner" (Woodhouse 1994).

The video tape "I Am What I Feel To Be" (Fama Film A.G. 1997) presents interviews in Spanish with a number of people who were born as male pseudo hermaphrodites, raised female, and later changed to live as men. Both they and their partners describe their lives as sexually fulfilling, in spite of penises so small that until puberty they were considered to be girls (Fama Film A.G. 1997).




Appendix G

Most adult women would not choose clitoral surgery for themselves

Dr. Suzanne Kessler, professor of psychology at the State University of New York, surveyed college women on their feelings about clitoral surgery.

The women were asked: "Suppose you had been born with a larger than normal clitoris and it would remain larger than normal as you grew to adulthood. Assuming that the physicians recommended surgically reducing your clitoris, under what circumstances would you have wanted your parents to give them permission to do it?" ... All the subjects were shown a scale with the normal ranges for clitorises and penises demonstrated in actual size, and labeled in centimeters..."

"About a fourth of the women indicated they would not have wanted a clitoral reduction under any circumstance. About half would have wanted their clitoris reduced only if the larger than normal clitoris caused health problems. Size, for them, was not a factor. The remaining fourth of the sample could imagine wanting their clitoris reduced if it were larger than normal, but only if having the surgery would not have resulted in a reduction in pleasurable sensitivity. Only one woman mentioned that other people's comments about the size of her clitoris might be a factor in her decision" (Kessler 1997, p35).

There is a wealth of literature available in which adults who were subjected to non-consensual cosmetic genital surgery as children express grief over the physical and emotional suffering caused by the surgery, and anger toward doctors who performed the surgery and parents who gave permission (Chase 1997; ISNA 1997). To date, no adult has come forth to say that she was grateful for having had this surgery performed without her consent.

Appendix H

Response to Doctors' Questions

1. Our recommendations are informed by academic research.

For example, our recommendations are in concordance with those of the following respected academic researchers:

Justine Schober M.D.
Pediatric Urologist
Hamot Medical Center

Anne Fausto-Sterling Ph.D.
Professor of Medical Science
Brown University

Milton Diamond Ph.D.
Professor of Psychology
University of Hawaii School of Medicine

Kieth Sigmundson M.D.
Department of Psychiatry
University of British Columbia

Suzanne Kessler Ph.D.
Professor of Psychology
State University of New York at Purchase

Alice Dreger Ph.D.
Adjunct Professor
Center for Ethics
Michigan State University

Howard Devore Ph.D.
Life Clinical Fellow
American Academy of Clinical Sexologists

2. ISNA performs research.

We are currently engaged, with the assistance of Aron Sousa, M.D. and Justine Schober, M.D., in a project which will use the new "Evidence Based Medicine" methodology to analyze all available published outcome data on intersex medical interventions. We are also engaged in a project, with the assistance of Justine Schober, M.D., to gauge the psychological adjustment of intersex adults, using a structured survey instrument.

 




3. Our recommendations are not based only upon technical limitations of older surgeries.

There is no evidence that "newer" surgeries preserve sensation or function. Indeed, because the surgery involves deep dissection and removal of highly innervated and vascular tissue, it is literally impossible for sensation to be unaffected. Outcome data from surgeries using similar microsurgical techniques for reconstruction after trauma in adults (for example, facial reconstruction, or transfer of a toe to replace an amputated finger) indicate that sensation is typically greatly reduced, but may be altered in character, or even painful.

Several people have come forward, whose surgery was performed during adolescence, and who are now young adults. Thus, they provide good information about surgical outcomes of only a decade ago. They report that surgery either greatly reduced or eliminated clitoral sensation, or left them with chronic pain. In some cases the pain did not develop until many years later.

Surgery inflicts emotional harm, by legitimating the idea that the child is not lovable unless "fixed" with plastic surgery that is medically unnecessary and carries great risks. Some individuals subjected to old style clitorectomy surgery were lucky enough to retain sensation. They find themselves no less emotionally harmed by the surgery. For examples, see (Coventry 1997; Coventry 1998; Holmes 1997) and the letter from Lisset Barcellos Cardenas to her physician in Lima.

Surgeons claiming to be developing the newest techniques concede that they have no proof that surgery does not damage sexual function. The published response of authors Oesterling, Gearhart, and Jeffs to (Chase 1996) concedes that their technique "does not guarantee normal adult sexual function."

There is even some evidence that the newer surgeries may be more harmful than the older ones. All of the cases of chronic genital pain that we are aware of are in patients who were subjected to "modern clitoroplasty" rather than older style clitorectomy.

4. Our recommendations represent the views of a large number of intersex people and the growing consensus of professionals in many disciplines.

ISNA maintains a mailing list currently numbering 1000 people. Of those, approximately 250 have told us that they, or a child, or a spouse is intersexed.

In the past several years there has been a world-wide explosion of intersex activism, with groups representing both intersex people and parents of intersex patients in many countries. See the Fall 1997 issue of the newsletter Hermaphrodites with Attitude for news from intersex patient-advocacy movements in New Zealand and Japan. The following are among the intersex patient-advocacy groups which criticize current medical protocols:

Intersex Society of North America

Ambiguous Genitalia Support Network (USA)

Hermaphrodite Education and Listening Post (USA)

Middlesex Group (USA)

Androgen Insensitivity Support Group (USA, UK, Canada, Germany, Holland, Australia)

Congenital Adrenal Hyperplasia Support Network (USA)

Intersex Society of Canada

Intersex Society of New Zealand

Peer Support for Intersexuals PESFIS (Japan)

Genital Mutilation Survivor's Support Network (Germany)

Workgroup on Violence in Pediatrics and Gynecology (Germany)

5. To date, no intersex person who was subjected to early surgery has come forth to say that the views expressed by these intersex patient advocacy groups are not representative, or to say that they believe genital surgery should be performed on intersex children.

6. Surgery cannot prevent psychological problems.

Indeed, in many cases it is clear that surgery itself is the cause of psychological problems. However, even if there were some former patients who felt that they were helped by early genital surgery, we would still argue that non-consensual genital surgery on infants is unethical, because so many people are harmed.

 




7. Surgery does not provide "normal" looking genitals.

In a recent review of a dozen girls aged 11 to 15 who had undergone clitoroplasty and vaginoplasty, Dr. David Thomas concluded "The results are indifferent and, frankly, disappointing" with reconstructions showing visibly different appearance from the original cosmetic result, clitorises withered and obviously nonfunctional, and "every girl required some additional vaginal surgery." (1997a; Scheck 1997). Even surgeries performed by leading experts had poor outcomes: "Dr. Thomas pointed out that 70% of the original surgeries had been performed by full-time pediatric urologists in three specialist centers" (1997a).

8. Surgery does not prevent emotional suffering.

In fact, there is evidence that it causes emotional suffering. "Many intersexuals report that the very treatments designed to prevent them from feeling like shameful freaks are in fact causing them to feel that way" (Dreger 1997a). "Children born as intersexes face psychological difficulties no matter what treatment choice is made, and sophisticated on-going counseling for both parent and child must certainly become, where it is not already, the central component of the treatment process" (Fausto-Sterling and Laurent 1994, p 8).

next:Trauma

References

1997a. Is early vaginal reconstruction wrong for some intersex girls? Urology Times (International Medical News), February, 10-12. (Enclosed: See Tab D)

1997b. The Pediatric Forum (letters). Archives of Pediatric and Adolescent Medicine 151:1062-64. (Enclosed: See Tab E)

Barry, Ellen. 1996. United States of Ambiguity. The Boston Phoenix (Styles section), 22 November, 6-8. (Enclosed: See Tab F)

Chase, Cheryl. 1996. Re: Measurement of Evoked Potentials during Feminizing Genitoplasty: Techniques and Applications (letter). Journal of Urology 156 (3):1139-1140. (Enclosed: See Tab G)

Chase, Cheryl. 1997. Special issue on intersexuality. Chrysalis: The Journal of Transgressive Gender Identities, fall. (Enclosed: See Tab H)

Coventry, Martha. 1997. Finding the words. Chrysalis: The Journal of Transgressive Gender Identities. (Enclosed: See Tab H)

Coventry, Martha. 1998. On Early Surgery. (Enclosed: See Tab I)

Dateline. 1997. NBC Dateline: Gender Limbo. New York: NBC. national broadcast television show. 17 June.

Diamond, Milton. 1996. Prenatal disposition and the clinical management of some pediatric conditions. Journal of sex and marital therapy 22 (3):139-147. (Enclosed: See Tab J)

Diamond, Milton, and HK Sigmundson. 1997a. Sex Reassignment at Birth: A Long Term Review and Clinical Implications. Archives of Pediatric and Adolescent Medicine 150:298-304.

Diamond, Milton, and H. Keith Sigmundson. 1997b. Commentary: Management of Intersexuality: Guidelines for dealing with persons with ambiguous genitalia. Archives of Pediatrics and Adolescent Medicine 151:1046-1050. (Enclosed: See Tab K)

Dreger, Alice Domurat. 1997a. Ethical problems in intersex treatment. Medical Humanities Report (Center for Ethics and Humanities in the Life Sciences, Michigan State University) 1:1+4-6. (Enclosed: See Tab L)

Dreger, Alice Domurat. 1997b. Listening to Hermaphrodites: Ethical Challenges to the Medical Treatment of Intersexuality. East Lansing Michigan: Center for Ethics and Humanities in the Life Sciences. (Enclosed: See Tab M)

Dreger, Alice Domurat. 1998 forthcoming-a. Ethical Issues in the Medical Treatment of Intersexuality and "Ambiguous Sex". Hastings Center Report. (Enclosed: See Tab N)

Dreger, Alice Domurat. 1998 forthcoming-b. Hermaphrodites and the Medical Invention of Sex. Cambridge: Harvard University Press. (Enclosed: See Tab O)

Drescher, Jack. 1997. Spare the knife, study the child. Ob.Gyn.News, 1 October, 14. (Enclosed: See Tab P)

Edgerton, Milton T. 1993. Discussion: Clitoroplasty for Clitoromegaly due to Adrenogenital Syndrome without Loss of Sensitivity (by Nobuyuki Sagehashi). Plastic and Reconstructive Surgery 91 (5):956.

Fama Film A.G. 1997. I am what I feel to be (Guevote). Berne Switzerland: Fama Film A.G. video. (Enclosed)

Fausto-Sterling, Anne. 1993. The Five Sexes: Why Male and Female are not Enough. The Sciences 33 (2):20-25. (Enclosed: See Tab Q)

Fausto-Sterling, Anne, and Bo Laurent. 1994. Early genital surgery on intersexual children: A re-evaluation. (Enclosed: See Tab R)




Hendren, W. Hardy, and Anthony Atala. 1995. Repair of high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome. Journal of Pediatric Surgery 30 (1):91-94.

Herdt, Gilbert, ed. 1994. Third Sex, Third Gender: Beyond Sexual Dimorphism in Culture and History. New York: Zone Books.

Holmes, Morgan. 1997. Is Growing Up in Silence Better than Growing Up Different? Chrysalis, Fall, 7-9. (Enclosed: See Tab H)

ISNA. 1997. Hermaphrodites Speak! San Francisco: ISNA. video. (Enclosed)

Kessler, Suzanne. 1997. Meanings of Genital Variability. (forthcoming in) Chrysalis: The Journal of Transgressive Gender Identities 2 (5):33-38. (Enclosed: See Tab H)

Kessler, Suzanne. 1998 forthcoming. Lessons from the Intersexed: Rutgers University Press. (Chapter Four Enclosed: See Tab T)

Meyer-Bahlburg, Heino, Rhoda S. Gruen, Maria I. New, Jennifer J. Bell, Akira Morishima, Mona Shimshi, Yvette Bueno, Ileana Vargas, and Susan W. Baker. 1996. Gender change from female to male in classical congenital adrenal hyperplasia. Hormones and Behavior 30:319-322.

Money, John, Howard Devore, and B. F. Norman. 1986. Gender identity and gender transposition: Longitudinal outcome study of 32 male hermaphrodites assigned as girls. Journal of Sex and Marital Therapy 12 (3).

Nevada, Eli. 1995. Lucky to have escaped genital surgery. Hermaphrodites with Attitude, 6. (Enclosed: See Tab S)

New, Maria I., and Elizabeth Kitzinger. 1993. Pope Joan: A Recognizable Syndrome. Journal of Clinical Endocrinology and Metabolism 76 (1):3-13.

Newman, Kurt, Judson Randolph, and Shaun Parson. 1992. Functional Results in Young Women having Clitoral Reconstruction as Infants. Journal of Pediatric Surgery 27 (2):180-184.

Oesterling, Joseph E., John P. Gearhart, and Robert D. Jeffs. 1987. A Unified Approach to Early Reconstructive Surgery of the Child with Ambiguous Genitalia. Journal of Urology 138:1079-1084.
Patil, U., and F. P. Hixson. 1992. The role of tissue expanders in vaginoplasty for congenital malformations of the vagina. British Journal of Urology 70:556.

Reilly, Justine M., and C. R. J. Woodhouse. 1989. Small Penis and the Male Sexual Role. Journal of Urology 142:569-571. (Enclosed: See Tab U)

Reiner, William. 1997a. To be male or female that is the question. Archives of Pediatric and Adolescent Medicine 151:224-5. (Enclosed: See Tab V)

Reiner, William George. 1996. Case Study: Sex Reassignment in a Teenage Girl. Journal of the Academy of Child and Adolescent Psychiatry 35 (6):799-803.

Reiner, William G. 1997b. Sex Assignment in the Neonate with Intersex or Inadequate Genitalia. Archives of Pediatric and Adolescent Medicine 151:1044-5. (Enclosed: See Tab W)

Roscoe, Will. 1987. Bibliography of Berdache and Alternative Gender Roles among North American Indians. Journal of Homosexuality 14 (3-4):81-171.

Scheck, Anne. 1997. Attitudes changing toward intersex surgery, but for the better? Urology Times, August, 44-45. (Enclosed: See Tab X)

Schober, Justine M. 1998. Long Term Outcomes of Feminizing Genitoplasty for Intersex. In Pediatric Surgery and Urology: Long Term Outomes, edited by P. Mouriquant. London: (forthcoming from) W. B. Saunders. (Enclosed: See Tab Y)

Woodhouse, C. R. J. 1994. The sexual and reproductive consequences of congenital genitourinary anomalies. Journal of Urology 152 (August 1994):645-651.

Young, Hugh Hampton. 1937. Genital Abnormalities, Hermaphroditism, and Related Adrenal Diseases. Baltimore: Williams and Wilkins.



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APA Reference
Staff, H. (2007, August 9). Genital Surgery On Intersexed Children, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/genital-surgery-on-intersexed-children

Last Updated: March 15, 2016

The True Story of John / Joan

J O H N / JOAN

By John Colapinto
The Rolling Stone, December 11, 1997. Pages 54-97

Self-Portrait, 1980

In 1967, an anonymous baby boy was turned into a girl by doctors at Johns Hopkins Hospital. For 25 years, the case of John/Joan was called a medical triumph — proof that a child's gender identity could be changed — and thousands of "sex reassignments" were performed based on this example. But the case was a failure, the truth never reported. Now the man who grew up as a girl tells the story of his life, and a medical controversy erupts.

In late June 1997, I arrive at an address in a working-class suburb in the North American Midwest. On the front lawn, a child's bicycle lies on its side; an eight-year-old secondhand Toyota is parked at the curb. Inside the house, a handmade wooden cabinet in the corner of the living room holds the standard emblems of family life: wedding photos and school portraits, china figurines and souvenirs from family trips. There is a knockoff-antique coffee table, a well-worn easy chair and a sofa - which is where my host, a wiry young man dressed in a jean jacket and scuffed work boots, seats himself. He is 31 years old but could pass for a decade younger. Partly it's the sparseness of his beard - just a few blond wisps that sprout from his jaw line; partly it's a certain delicacy to his prominent cheekbones and tapering chin. Otherwise he looks, and sounds, exactly like what he is: a blue-collar factory worker, a man of high school education whose fondest pleasures are to do a little weekend fishing with his dad in the local river and to have a backyard barbecue with his wife and kids.Ordinarily a rough-edged and affable young man, he stops smiling when conversation turns to his childhood. Then his voice - a burred baritone - takes on a tone of aggrievement and anger, or the pleading edge of someone desperate to communicate emotions that he knows his listener can only dimly understand. How well even he understands these emotions is not clear: When describing events that occurred prior to his 15th birthday, he tends to drop the pronoun I from his speech, replacing it with the distancing you - almost as if he were speaking about someone else altogether. Which, in a sense, he is.

"It was like brainwashing," he is saying now as he lights a cigarette. "I'd give just about anything to go to a hypnotist to black out my whole past. Because it's torture. What they did to you in the body is sometimes not near as bad as what they did to you in the mind - with the psychological warfare in your head."

Joan, age 2, at a relative's farm, within a year of sex reassignmentHe is referring to the extraordinary medical treatment he received after suffering the complete loss of his penis to a botched circumcision when he was 8 months old. On the advice of experts at the renowned Johns Hopkins medical center, in Baltimore, a sex-change operation was performed on him, a process that involved clinical castration and other genital surgery when he was a baby, followed by a 12-year program of social, mental and hormonal conditioning to make the transformation take hold in his psyche. The case was reported as an unqualified success, and he became one of the most famous (though unnamed) patients in the annals of modern medicine.

 




 

It's a fame that derives not only from the fact that his medical metamorphosis was the first sex reassignment ever reported on a developmentally normal child but also from a stunning statistical long shot that lent a special significance to the case. He was born an identical twin, and his brother provided the experiment with a built-in matched control - a genetic clone who, with penis intact, was raised as a male. That the twins were reported to have grown into happy, well-adjusted children of opposite sex seemed unassailable proof of the primacy of rearing over biology in the differentiation of the sexes and was the basis for the rewriting of textbooks in a wide range of medical disciplines. Most seriously, the case set a precedent for sex reassignment as the standard treatment for thousands of newborns with similarly injured, or irregular, genitals. It also became a touchstone for the feminist movement in the 1970s, when it was cited as living proof that the gender gap is purely a result of cultural conditioning, not biology. For Dr. John Money, the medical psychologist who was the architect of the experiment, this case was to be the most publicly celebrated triumph of a 40-year career that recently earned him the accolade "one of the greatest sex researchers of the century."

But as the mere existence of this young man in front of me would suggest, the experiment was a failure, a fact revealed in a March 1997 article in the Archives of Adolescent and Pediatric Medicine. Authors Milton Diamond, a biologist at the University of Hawaii, and Keith Sigmundson, a psychiatrist from Victoria, British Columbia, documented how the twin had struggled against his imposed girlhood from the start. The paper set off shock waves in medical circles around the world, generating furious debate about the ongoing practice of sex reassignment (a procedure more common than anyone might think). It also raised troubling questions about the way the case was reported in the first place, why it took almost 20 years for a follow-up to reveal the actual outcome and why that follow-up was conducted not by Dr. Money but by outside researchers. The answers to these questions, fascinating for what they suggest about the mysteries of sexual identity, also bring to light a 30-year rivalry between eminent sex researchers, a rivalry whose very bitterness not only dictated how this most unsettling of medical tragedies was exposed but also may, in fact, have been the impetus behind the experiment in the first place.

But what for medicine has been a highly public scandal involving some of the biggest names in the world of sex research has been for the young man sitting in front of me a purely private catastrophe. Apart from two short television appearances (his face obscured, his voice disguised), he has never spoken on the record to a journalist and has never before told his story in full. For this article, he granted more than 20 hours of candid interviews and signed confidentiality waivers giving me exclusive access to a voluminous array of legal documents, therapists' notes, Child Guidance Clinic reports, IQ tests, medical records and psychological work-ups. He assisted me in obtaining interviews with his former therapists as well as with all of his family members, including his father, who, because of the painfulness of these events, had not spoken of them to anyone in more than 20 years.

The young man's sole condition for talking to me was that I withhold some details of his identity. Accordingly, I will not reveal the city where he was born and raised and continues to live, and I have agreed to invent pseudonyms for his parents, whom I will call Frank and Linda Thiessen, and his sole sibling, the identical twin brother, whom I will call Kevin. The physicians in his hometown I will identify by initials. The young man himself I will call, variously, John and Joan, the pseudonyms given for him by Diamond and Sigmundson in the journal article describing the macabre double life he has been obliged to live. No other details have been changed.

"My parents feel very guilty, as if the whole thing was their fault," John says. "But it wasn't like that. They did what they did out of kindness, and love and desperation. When you're desperate, you don't necessarily do all the right things."

The irony was that Frank and Linda Thiessen's life together had begun with such special promise. A young couple of rural, religious backgrounds, they grew up on farms near each other and met when Linda was just 15, Frank 17. Linda, an exceptionally pretty brunette, had spent much of her teens fighting off guys who were too fresh. Frank, a tall, shy fair-haired man, was different. "I thought, 'Well, he's not all hands,' "Linda recalls." 'I can relax with him.' " Three years later, at ages 18 and 20, they married and moved to a nearby city. Linda remembers Frank's joy soon after, upon learning that he was going to be the father of twins - and his euphoria when the brothers were born, on Aug. 22, 1965. "The nurse asked him, 'Is it boys or girls?' " Linda recalls. "And he said, 'I don't know! I just know there's two of 'em!' "

Shortly before the births, Frank had landed his highest-paying job ever, at a local unionized plant, and the couple now moved with their newborns into a sunny one-bedroom apartment on a quiet side street downtown. But when the twins were 7 months old, Linda noticed that their foreskins were closing, making it hard for them to urinate. Their pediatrician explained that the condition, called phimosis, was not rare and was easily remedied by circumcision. He referred them to a surgeon. The operations were scheduled for April 27, 1966, in the morning. Because Frank needed the family car to get to his job on the late shift, they brought the kids in the night before. "We weren't worried," Linda says. "We didn't know we had anything to worry about."

But early the next morning, they were jarred from sleep by a ringing phone. It was the hospital. "There's been a slight accident," a nurse told Linda. "The doctor needs to see you right away."




In the children's ward, they were met by the surgeon. Grim-faced, businesslike, he told them that John had suffered a burn to his penis. Linda remembers being shocked into numbness by the news. "I sort of froze," she says. "I didn't cry. It was just like I turned to stone." Eventually she was able to gather herself enough to ask how their baby had been burned. The doctor seemed reluctant to give a full explanation - and it would, in fact, be months before the Thiessens would learn that the injury had been caused by an electro-cautery needle, a device sometimes used in circumcisions to seal blood vessels as it cuts. Through mechanical malfunction or doctor error, or both, a surge of intense heat had engulfed John's penis. "It was blackened," Linda says, recalling her first glimpse of his injury. "It was like a little string. And it went right up to the base, up to his body." Over the next few days, the burnt tissue dried and broke away in pieces.

John, with a catheter where his penis used to be remained in the hospital for the next several weeks, while Frank and Linda, frantic, watched as a parade of the city's top local specialists examined him. They gave little hope. Phallic reconstruction, a crude and makeshift expedient even today, was in its infancy in the 1960's - a fact made plain by the plastic surgeon when he described the limitations of a phallus that would be constructed from flesh farmed from John's thigh or abdomen: "Such a penis would not, of course, resemble a normal organ in color, texture or erectile capability," he wrote in a report to the Thiessens' lawyer. "It would serve as a conduit for urine, but that is all."

Even that was optimistic, according to a urologist: "Insofar as the future outlook is concerned," he wrote, "restoration of the penis as a functional organ is out of the question." A psychiatrist summarized John's emotional future this way: "He will be unable to consummate marriage or have normal heterosexual relations; he will have to recognize that he is incomplete, physically defective, and that he must live apart...."

Now desperate, Frank and Linda took baby John on a daylong train trip to the Mayo Clinic, in Rochester, Minn., where he was examined by a team of doctors who merely repeated the dire prognoses delivered by the Thiessens' local physicians. Back home, with nowhere to turn, the couple sank into a state of mute depression. Months passed during which they could not speak of John's injury even to each other. Then one evening in December 1966, some seven months after the accident, they saw a TV program that jolted them from their despondency.

On their small black-and-white television screen appeared a man identified as Dr. John Money. A suavely charismatic and handsome individual in his late 40s, bespectacled and with sleekly brushed-back hair, Dr. Money was speaking about the wonders of gender transformation taking place at the Johns Hopkins medical center, where he was a medical psychologist. Also on the program was a woman - one of the satisfied post-operative transsexuals who had recently been converted at Johns Hopkins.

Today, with the subject of transsexualism a staple of daytime talk shows, it's difficult to imagine just how alien the concept seemed on that December evening in 1966. Fourteen years earlier, a spate of publicity had attended the announcement by American ex-GI George Jorgensen that he had undergone surgical transformation to become Christine. But that operation, performed in Denmark, had been roundly criticized by American doctors, who refused to perform such surgeries. The subject had faded from view - until now, when Johns Hopkins announced that it had not only performed two male-to-female sex changes (a first in America) but also established the world's first Gender Identity Clinic, devoted solely to the practice of converting people from one sex to the other. Along with gynecologist Howard W. Jones Jr., the driving force behind Hopkins' pioneering work in the study and treatment of transsexuals was the man on the Thiessens' television screen: Dr. John Money.

"He was very self-confident, very confident about his opinions," Linda recalls of her first glimpse of the man who would have such a lasting effect on the Thiessen's lives. "He was saying that it could be that babies are born neutral and you can change their gender. Something told me that I should get in touch with this Dr. Money."

She wrote to him soon after and described what had happened to her child. Dr. Money responded promptly, she says. In a letter, he expressed great optimism about what could be done for her baby at Johns Hopkins and urged her to bring John to Baltimore without delay. He also happened to inquire, Linda says, about the twin brother whom she had mentioned in passing. "He asked if they were identical twins," Linda says. She informed him that they were. Dr. Money replied that he would like to run a test on the babies at Johns Hopkins, just to make sure.

After so many months of grim predictions, bleak prognoses and hopelessness, Dr. Money's words, Linda says, felt like a balm. "Someone," she says, "was finally listening."

Dr. Money was, indeed, listening. But then, Linda's cry for help was one that he might have been waiting for his entire professional life.




At the time that the Thiessen family's plight became known to Dr. Money, he was already one of the most respected, if controversial, sex researchers in the world. Born in 1921 in New Zealand, Money had come to America at about age 26, received his Ph.D. in psychology from Harvard and then joined Johns Hopkins, where his rise as a researcher and clinician specializing in sexuality was meteoric. Within a decade of joining Hopkins, he was already widely credited as the man who had coined the term "gender identity" to describe a person's inner sense of himself or herself as male or female, and was the world's undisputed authority on the psychological ramifications of ambiguous genitalia. "I think he's a thoroughly ethical and professional person," says John Hampson, a child psychiatrist who co-authored a number of Money's groundbreaking papers on sexual development in the mid-1950s. "He was a very conscientious scientist when it comes to collecting data and making sure of what he's saying. I don't know very many social scientists who could match him in that regard." According to Hampson, Money's ability to persuade others to adopt his point of view is one of the psychologist's chief strengths: "He's a terribly good speaker, very organized and very persuasive in his recital of the facts regarding a case." Indeed, Hampson admits that Money is almost too good at the art of persuasion. "I think a lot of people were envious," says Hampson. "He's kind of a charismatic person, and some people dislike him. As a person, he was a little bit . . . oh . . . flamboyant; he might have been a little glib."

Dr. John Money in 1986: Money's often-overweening confidence actually came to him at some cost. His childhood and youth in rural New Zealand had been beset by anxieties, personal tragedies and early failure. The son of an Australian father and an English mother, he was a thin, delicate child raised in an atmosphere of strict religious observance - or what he has called "tightly sealed, evangelical religious dogma." At age 5 he was bullied by his classmates and took shelter with a female cousin in the girls' play shed, where no boy would be caught dead. "My fate was sealed," he wrote in an anthology titled How I Got Into Sex. "Having not measured up as a fighter, I was set on the pathway of outwitting other kids by being an intellectual achiever. That was easier for me than for most of them."

He was 8 years old when his father, after a long illness, died. "His death was not handled very well in our family," Money wrote. Three days after watching his father get mysteriously carried off to the hospital, the boy was told that his father had died. His shock was compounded by the trauma of being informed by an uncle that now he would have to be the man of the household. "That's rather heavy duty for an 8-year-old." Money wrote. "It had a great impact on me." Indeed. As an adult, Money would forever avoid the role of "man of the household." After one brief marriage ended, he never remarried, and he has never had children.

Following his father's death, Money was raised by his mother and spinster aunts. A solitary adolescent with passions for astronomy and archaeology, he also harbored ambitions to be a musician. His widowed mother could not afford piano lessons, so Money worked as a gardener on weekends to pay for music classes and used every spare moment to practice. It was an ambition doomed to disappointment, partly because Money had set the bar so high for himself: "It was difficult for me to have to admit that, irrespective of effort, I could never achieve in music the goal that I wanted to set for myself. I would not even be a good amateur."

Upon entering Victoria University, in Wellington, Money discovered a new passion into which he would channel his thwarted creativity: the science of psychology. Like so many drawn to the study of the mind and emotions, Money initially saw the discipline as a means of solving certain gnawing questions about himself. His first serious work in psychology, the thesis for his master's, concerned "creativity in musicians"; in it, Money writes, "I began to investigate my relative lack of success in comparison with that of other music students."

His later decision to narrow his studies to the psychology of sex had a similarly personal basis. Having lost his religious faith in his early 20s, Money increasingly reacted against what he saw as the repressive religious strictures of his upbringing and, in particular, the anti-masturbatory, anti-sexual fervor that went with them. The academic study of sexuality, which removed even the most outlandish practices from moral considerations and placed them in the "pure" realm of scientific inquiry, was for Money an emancipation. From now on, he would be a fierce proselytizer for sexual exploration. According to journalist John Heidenry, a personal confidant of Money's and author of the recent book What Wild Ecstacy, which traces Money's role as a major behind-the-scenes leader of the sexual revolution of the 1960s and '70s, the psychologist's sexual explorations were not confined to the lab, lecture hall or library. An acknowledged but discreet bisexual, Money engaged in affairs with a number of men and women - "some briefly," Heidenry writes, "others over a longer duration." Indeed, by the mid-1970s, with the sexual revolution in full rampage, Money would step out publicly as a champion of open marriage, nudism and the dissemination of explicit pornography. His promotion of the culture's sexual unbuttoning seemed boundless. "There is plenty of evidence that bisexual group sex can be as personally satisfying as a paired partnership, provided each partner is 'tuned in' on the same wavelength," he wrote in his 1975 pop-psych book, Sexual Signatures. A former patient who was treated by Money in the 1970's for a rare endocrine disorder recalls the psychologist once casually asking him if he'd ever had a "golden shower." The patient, a sexually inexperienced youth at the time, did not know what Money was talking about. "Getting pissed on," Money airily announced with the twinkling, slightly insinuating little smile with which he delivered such deliberately provocative comments.

According to colleagues and other former patients, such sexual frankness in conversation is a hallmark of Money's personal style. Dr. Fred Berlin, a professor of psychiatry at the Johns Hopkins School of Medicine and a colleague who considers Money one of his most important mentors, agrees that Money is aggressively outspoken. "Because he thinks it's important to desensitize people in discussing sexual issues, he will sometimes use four-letter words that others might find offensive," says Berlin. "Perhaps he could be a little more willing to compromise On that. But John is an opinionated person who isn't looking necessarily to do things differently from the way he's concluded is best."




But while Money's conclusions about the best approach to sexual matters merely raised eyebrows in the mid-1970's, they provoked outrage at the dawn of the more conservative 1980's. Undaunted, Money continued to push on into uncharted realms. In an April 14, 1980, article in Time, Money was sharply criticized for what looked dangerously like an endorsement of incest and pedophilia. "A childhood sexual experience, such as being the partner of a relative or of an older person, need not necessarily affect the child adversely," Money told Time. And according to a right-wing group critical of his teachings, Money reportedly told Paidika, a Dutch journal of pedophilia, "If I were to see the case of a boy aged 10 or 12 who's intensely attracted toward a man in his 20s or 30s, if the relationship is totally mutual, and the bonding is genuinely totally mutual, then I would not call it pathological in any way."

Money's response to criticism has been to launch counterattacks of his own, lambasting his adoptive country for a puritanical adherence to sexual taboos. In an autobiographical essay included in his book Venuses Penuses, Money describes himself as a "missionary" of sex - and points out, with a lofty and defiant pride, "It has not been as easy for society to change as it had been for me to find my own emancipation from the 20th-century legacy of fundamentalism and Victorianism in rural New Zealand."

Money's experimental, taboo-breaking approach to sex was paralleled in his professional career. Eschewing the well-traveled byways of sex research, Money sought out exotic corners of the field where he could be a pioneer. He found just such a relatively undiscovered realm of human sexuality while in the first year of his Ph.D. studies in psychology at Harvard. In 1948, in a social-relations course, he learned of a 15 year-old male who was born not with a penis but with a tiny, nublike phallus resembling a clitoris and who, at puberty, developed breasts. It was Money's first exposure to hermaphroditism - also known as intersexuality - a condition that, in its extreme or its milder forms, is estimated to occur once in every 2,000 births. Characterized by ambiguities of the external sex organs and the internal reproductive system, intersexuality is caused by any of a wide variety of genetic and hormonal irregularities, and can vary from a female born with a penis-sized clitoris and fused labia resembling a scrotum to a male born with a penis no bigger than a clitoris, undescended testes and a split scrotum indistinguishable from a vagina.

Money became fascinated with intersexuality and wrote his doctoral dissertation on the subject, which led to his invitation, in 1951, to join Johns Hopkins, where the world's largest clinic for the study of intersexual conditions had been established. Up until then, the syndrome had been studied solely from a biological perspective. Money came at it from a psychological angle and would make a name for himself as a pioneer in examining the mental and emotional repercussions of being born as neither boy nor girl. At Hopkins, he enlisted Hampson and Hampson's wife, Joan, to help him study some 105 intersex children and adults. Money claimed to have observed a striking fact about people who had been diagnosed with identical genital ambiguities and chromosomal makeups but who had been raised as members of the opposite sex: More than 95 percent of these intersexes fared equally well, psychologically, whether they had been raised as boys or as girls. To Money, this was proof that the primary factor that determined an intersexual child's gender identity was not biological traits but the way that the child was raised. He concluded that these children were born psychosexually undifferentiated.

This theory was the foundation on which Money based his recommendation to pediatric surgeons and endocrinologists that they surgically and hormonally stream intersexual newborns into whichever sex the doctors wished. Such surgeries would duly range from cutting down enlarged clitorises on mildly intersexual girls to performing full sex reversals on intersexual boys born with testicles but a penis deemed too small. Money's only provisos were that such "sex assignments" be done as early as possible - preferably within weeks of birth - and that once the sex was decided on, doctors and parents never waiver in their decision, for fear of introducing dangerous ambiguities into the child's mind. In terms of the possible nerve destruction caused by the amputation of genital appendages, Money assured doctors that according to studies he had conducted with the Hampsons, there was no evidence of loss of sensation. "We have sought information about erotic sensation from the dozen non-juvenile . . . women we have studied," he wrote in a 1955 paper. "None of the women . . . reported a loss of orgasm after clitoridectomy."

Money's protocols for the treatment of intersexual children hold to this day. Placing the greatest possible emphasis on the child's projected "erotic functioning" as an adult and taking into account that medical science had never perfected the reconstruction of injured, or tiny, penises, Money's recommendations meant that the vast majority of intersexual children, regardless of their chromosome status, would be turned into girls. Current guidelines dictate that to be assigned as a boy, the child must have a penis longer than 2.5 centimeters; a girl's clitoris is surgically reduced if it exceeds 1 centimeter.

By providing a seemingly solid psychological foundation for such surgeries, Money had, in a single stroke, offered physicians a relatively simple solution to one of the most vexing and emotionally fraught conundrums in medicine: how to deal with the birth of an intersexual child. As Money's colleague Dr. Berlin points out, "One can hardly begin to imagine what it's like for a parent when the first question - 'Is it a boy or a girl?' - results in a response from the physician that they're just not sure. John Money was one of those folks who, years ago, before this was even talked about, was out there doing his best trying to help families, trying to sort through what's obviously a difficult circumstance."




The twins at home, around age 3.But Money was not interested solely in intersexes. As he has stated often in his writings, he saw intersexual syndromes, which he called "experiments of nature," chiefly as a way to learn about the sexual development of so-called normal humans. Thus, he immediately generalized his theories about intersexes to include all children, even those born without genital irregularities. "In the light of hermaphroditic evidence " he wrote in a 1955 paper that would become a classic in the field of sexual development, "it is no longer possible to attribute psychological maleness or femaleness to chromosomal, gonadal or hormonal origins. . . . The evidence of hermaphroditism lends support to a conception that, psychologically, sexuality is undifferentiated at birth and that it becomes differentiated as masculine or feminine in the course of the various experiences of growing up." In simple terms, Money was advancing the view that all children form a sense of themselves as male or female according to whether they are dressed in blue or pink, given a masculine or feminine name, clothed in pants or dresses, given guns or Barbies to play with.

In a retrospective essay written in 1985 about his career as a sex researcher, Money offered crucial insight into the way he arrived at some of his more unusual theories about human sexual behavior. "I frequently find myself toying with concepts and working out potential hypotheses," he mused. "It is like playing a game of science fiction. . . . It is as much an art as the creative process in painting, music, drama or literature."

Money's theory that newborns are psychosexually neutral was both unorthodox and against the current climate of science, which for decades had centered on the critical role of chromosomes and hormones in determining sexual behavior. But if his colleagues considered Money's ideas to be science fiction, they weren't prepared to say so publicly. His papers outlining his theory became famous in his field, helping not only to propel him to international renown as a sex researcher but also to speed his rise up the ladder at Johns Hopkins, where he ascended from assistant to associate professor of medical psychology, teaching his theory of infant sexual development to generations of medical students. By 1965, the year of John and Kevin Thiessen's birth, Money's reputation was virtually unassailable. He had for more than a decade been head of Hopkins' Psychohormonal Research Unit (his clinic for treating and studying intersex kids), and he was shortly to help co-found Hopkins' groundbreaking Gender Identity Clinic - a coup that helped earn him a reputation, says John Hampson, as "the national authority on gender disorder."

There was, however, at least one researcher who was willing to question Money. He was a young graduate student at the University of Kansas. The son of struggling Ukrainian-Jewish immigrant parents, Milton Diamond, whom friends call Mickey, was raised in the Bronx, where he had sidestepped membership in the local street gangs for the life of a scholar. As an undergraduate majoring in biophysics at City College of New York, Diamond became fascinated by the role of hormones in the womb and their possible role in defining a person's gender identity and sexual orientation. In his late 20s, as a grad student in endocrinology at Kansas, he conducted animal research on the subject, injecting pregnant guinea pigs and rats with different hormone cocktails to see how pre-birth events would affect later sexual behavior. The evidence in Diamond's lab suggested a link between the hormones that bathe a developing fetus's brain and nervous system and its later sexual functioning. It was in an effort to raise funds for his continued research that Diamond applied for a grant from the National Science Foundation Committee for Research in Problems of Sex an application that required the submission of a research paper. For his topic, Diamond decided to write a response to Money's now-classic papers on sexual development.

Diamond's critique appeared in The Quarterly Review of Biology in 1965. Marshaling evidence from biology, psychology, psychiatry, anthropology and endocrinology to argue that gender identity is hardwired into the brain virtually from conception, the paper was an audacious challenge to Money's authority (especially coming from an unknown grad student at the University of Kansas). First addressing the theory about the psychosexual flexibility of intersexes, Diamond pointed out that such individuals suffer "a genetic or hormonal imbalance" in the womb. Diamond argued that even if intersexuals could be steered into one sex or the other as newborns, this was not necessarily evidence that rearing is more influential than biology. It might simply mean that the cells in their brains had undergone, in utero, an ambiguity of sexual differentiation similar to that of the cells in their genitals. In short, intersexes have an inborn, neurological capability to go both ways - a capability, Diamond hastened to point out, that genetically normal children certainly would not share.

Even a scientist less thin-skinned than John Money might have been stung by the calm, relentless logic of Diamond's attack - which, near the end, raised the most rudimentary, Science 101 objection to the widespread acceptance of Money's theory of psychosexual malleability in normal children. "To support [such a] theory," Diamond wrote, "we have been presented with no instance of a normal individual appearing as an unequivocal male and being reared successfully as a female."

It was a year and a half after Diamond had thrown down the gauntlet that Dr. Money received Linda Thiessen's letter describing the terrible circumcision accident that had befallen her baby boy.




The Thiessens made their first trip to Johns Hopkins early in 1967, within weeks of first seeing Dr. Money on TV. The young couple were awestruck by the vast medical center dominating the top of a rise on Wolfe Street. Dr. Money's Psychohormonal Research Unit was located in the Phipps Clinic, a gloomy Victorian building tucked away in a courtyard; the unit's offices, located on an upper floor, were reached by way of a rickety turn-of-the-century elevator. Money's own inner sanctum (where most of his meetings with the Thiessens would take place during the ensuing 12 years) was furnished with a couch, Oriental rugs and potted plants - reminding Frank more of a living room than of an office. There was also a collection of carved aboriginal sculptures of erect phalluses, vaginas and breasts that adorned a mantel. But if these artifacts were unsettling, Money himself, with his smoothly confident, professional manner - not to mention the diplomas on his wall - made the Thiessens feel that they were in the best possible hands. "I looked up to him like a god," says Linda, who at the time was not yet out of her teens. "I accepted whatever he said." And what Dr. Money had to say was exactly what the Thiessens ached to hear.

In his many published versions of this first interview, Money has recounted how he spelled out to the young couple the advantages of sex reassignment for baby John - "using nontechnical words, diagrams and photographs of children who had been reassigned." What is not clear from Money's accounts is whether Linda and Frank, whose educations at the time did not go beyond the sixth grade, understood that such a procedure was, in fact, purely experimental - that while such surgeries had been performed on intersexual children, no such sex changes had ever been attempted on a child born with normal genitals and a normal nervous system. Today, Frank and Linda say that this was a distinction they did not fully grasp until later. The crucial point that they gleaned from Dr. Money was his conviction that the procedure had every chance for success. "I see no reason," Linda recalls him saying, "that it shouldn't work."

Indeed, Money's eagerness to begin is evident in a description of the interview written almost 10 years later. In Sexual Signatures, he wrote: "If the parents stood by their decision to reassign the child as a girl, surgeons could remove the testicles and construct feminine external genitals immediately. When she was 11 or 12 years old, she could be given the female hormones."

If Dr. Money seemed to be in a hurry, he was. He explained to Frank and Linda that they would have to make up their minds quickly. For according to one of the finer points of his theory, the "gender identity gate" - Money's term for that moment after which a child has locked into an identity as a male or a female - comes a little after 2 years of age. John was now 17 months. "The child was still young enough so that whichever assignment was made, erotic interest would almost certainly direct itself toward the opposite sex later on," Money wrote, "but the time for reaching a final decision was already short."

Frank and Linda, however, needed time to decide on something as momentous as having their child undergo a surgical sex change. They went home to think about it. Linda says that Dr. Money made no secret of his impatience with the delay. "He wrote in a letter that we were 'procrastinating,' " Linda recalls. "But we wanted to move slow, because we had never heard of anything like this."

Back home, they canvassed opinions. Their pediatrician recommended against such drastic treatment, and so did their parents. But finally, Frank and Linda realized that they alone had to decide. They alone were the ones living with the reminder, at each diaper change, of John's terrible injury. After months of indecision, they made up their minds.

That summer, five months after their first meeting with Money, they returned to Baltimore with their baby. Now 22 months old, the child was still within the window of 30 months that Money had established as safe for an infant sex change. And so, on July 3, 1967, the baby underwent surgical castration. According to the operating-room record, Dr. Howard W. Jones Jr. slit open the baby's scrotum along the midline and removed the testes, then reclosed the scrotal tissue so that it resembled labia. The urethra was lowered to approximate the position of the female genitalia, and a cosmetic vaginal cleft was made by forming the skin around a rolled tube of gauze during the healing. It was also during this visit to Johns Hopkins, says Linda, that the promised chromosome test was conducted on the twins to determine if they were, indeed, identical. They were.

Linda and Frank say that by the time they decided to have their baby undergo clinical castration, they had eradicated any doubts they might have had about the efficacy of the treatment - a crucial turnabout, since, according to Dr. Money, it was a "vital consideration" that the parents of a sex-reassigned child harbor no second thoughts. "For any lingering doubts whatsoever in their minds," Money wrote, "would weaken the child's identification as a girl and woman."

Whether Money himself was able to eradicate his own doubts about the child's future development is debatable. In a letter he wrote a few weeks after the castration, his tone admitted of considerable caution regarding the prognosis. But then this was perhaps to be expected, since the letter was addressed to the lawyer whom Frank and Linda had hired to sue the hospital that botched the circumcision.

"The reassignment of a baby's sex is usually undertaken only in cases of a birth defect of the genitalia," Money wrote. "Then one usually expects that the child's psychosexual differentiation will be congruous with the sex of rearing. In any given case, however, it is not possible to make an absolute prediction."




Central to Money's program for sex reassignment of hermaphrodites was his edict that the children, when very young, know nothing of their ambiguous sexual status at birth. Money put the same stricture into effect in the case of the Thiessens' baby, whom they now called Joan. "He told us not to talk about it," Frank says. "Not to tell Joan the whole truth and that she shouldn't know she wasn't a girl."

Linda had sewn dresses and bonnets for her new daughter. It was shortly before Joan's second birthday when Linda first put her in a dress. "It was a pretty, lacy little dress," Linda recalls. "She was ripping at it, trying to tear it off. I remember thinking, 'Oh, my God, she knows she's a boy and she doesn't want girls' clothing. She doesn't want to be a girl.' But then I thought, 'Well, maybe I can teach her to want to be a girl. Maybe I can train her so that she wants to be a girl.' "

Linda and Frank did their best to do just that. When Joan's brother, Kevin, at age 4, was watching Frank shave and asked to shave, too, Frank gave him an empty razor and some shaving cream to play with. But when Joan also clamored for a razor, Frank refused. "I told her that girls don't shave," Frank recalls. "I told her girls don't have to." Linda offered to put makeup on her. But Joan didn't want to wear makeup.

"I remember saying, 'Oh, can I shave, too?' " John says of this incident, which forms his earliest childhood memory. "My dad said, 'No, no. You go with your mother.' I started crying, 'Why can't I shave, too?' " Kevin says that the incident was typical of the way their parents tried to steer them into opposite sexes - and how such efforts were, inevitably, doomed to failure.

"I recognized Joan as my sister," Kevin says, "but she never, ever acted the part. She'd get a skipping rope for a gift, and the only thing we'd use that for was to tie people up, whip people with it. Never used it for what it was bought for. She played with my toys: Tinkertoys, dump trucks. Toys like this sewing machine she got just sat."

Today, with the twins having rejoined each other on the same side of the gender divide, the stark physical differences between them eerily testify to all that John has been through. At 32, Kevin is a dark-bearded, bearlike man with the thickly muscled arms and shoulders of a manual laborer. To see him standing alongside his scarecrow-thin, scantily bearded brother, you would never guess that every cell in their bodies bears identical DNA - until you compare their eyes, noses and mouths, which are indistinguishable from one twin to the other.

As children, their physical differences were, if less pronounced, equally deceptive. Photographs of them as preschoolers show a puppy-eyed little boy with a crew cut and a slim, brown-eyed girl with wavy chestnut hair framing a face of delicate prettiness. But by all accounts, this illusion of two children occupying opposite sexes disappeared the second that Joan moved, spoke, walked, gestured. "When I say there was nothing feminine about Joan," Kevin laughs, "I mean there was nothing feminine. She walked like a guy. She talked about guy things, didn't give a crap about cleaning house, getting married, wearing makeup.... We both wanted to play with guys, build forts and have snowball fights and play army." Enrolled in Girl Scouts, Joan was miserable. "I remember making daisy chains and thinking, 'If this is the most exciting thing in Girl Scouts, forget it,' " John says. "I kept thinking of the fun stuff my brother was doing in Cubs."

Linda and Frank were troubled by Joan's masculine behavior. But they had been told by Dr. Money that they must not entertain any doubts about their daughter, and they felt that to do so would only increase the problem. Instead, Frank and Linda seized on those moments when Joan's behavior could be construed as stereotypically feminine. "And she could be sort of feminine, sometimes," Linda says, "when she wanted to please me. She'd be less rough, keep herself clean and tidy, and help a little bit in the kitchen."

Joan at age 13, with the effects of estrogen treatment now visible in her physique.In her letters to Dr. Money describing Joan's progress, Linda made sure to emphasize those moments so that the psychologist would know that she and Frank were doing everything they could to implement his plans. Meanwhile, Linda comforted herself by thinking of her daughter as a tomboy. "I have seen all kinds of women in my life," she says, "and some of them, you'd swear they were men. So I thought, 'Well, maybe it won't be a problem, because there are lots of women who aren't very effeminate. Maybe it could work.' I wanted it to work."

Kevin didn't question his sister's boyish ways until they went off to school. "I was in grade one or two," he says, "and I saw all the other girls doing their thing - combing their hair, holding their dolls. Joan was not at all like that. Not at all." At that time, Joan had voiced the ambition to be a garbage man. "She'd say, 'Easy job, good pay,' " Kevin recalls. "She was 6 or 7 years old. I thought it was kinda bizarre - my sister a garbage man?" Indeed, Kevin would finally grow so perplexed with his sister's unconventional behavior that he went to his mother about it. "Well, that's Joan being a tomboy," Linda told him. "I accepted that," Kevin says and shrugs.

That was not an explanation Joan's schoolmates were prepared to accept. Upon entering kindergarten, she became the object of instant ridicule from classmates, both male and female. "As you'd walk by, they'd start giggling," John remembers. "Not one, but almost the whole class. It'd be like that every day. The whole school would make fun of you about one thing or another."




"They were cruel," says Kevin, who witnessed his sister's humiliation at school. "Teased every day. It wasn't a weekly thing. Or a monthly thing. This was a daily thing. They'd call her names, ignore her, not involve her in the groups."

"It started the first day of kindergarten," Linda says. "Even the teacher didn't accept her. The teachers knew there was something different."

By then, Joan also knew that there was "something different" about her. But she didn't know what. "You know generally what a girl is like," John says, "and you know generally what a guy is like. And everyone is telling you that you're a girl. But you say to yourself, 'I don't feel like a girl.' You think girls are supposed to be delicate and like girl things - tea parties, things like that. But I like to do guy stuff. It doesn't match. So you figure, 'Well, there's something wrong here. If I'm supposed to be like this girl over here but I'm acting like this guy, I guess I gotta be an it.' "

Joan's personal difficulties were obvious in her functioning in the classroom. Though tests had revealed her to be in the normal intelligence range, she seemed unable, or unwilling, to master the skills required in kindergarten. When the school threatened to hold Joan back, Linda complained to Dr. Money. He wrote a letter to the school, urging that Joan, despite her emotional difficulties, be promoted to first grade. But her problems only got worse. On Oct. 29, 1971, a few weeks after she started first grade, her behavior prompted a teacher to file a report with the district's Child Guidance Clinic. The teacher noted that Joan "has been doing just the opposite of anything the other children do" and described the girl as "very negativistic."

It was at a December 1972 meeting of the American Association for the Advancement of Science in Washington, D.C. that John Money unveiled, for the first time, his "twins case." Time magazine ran a full-page story on the debut, which happened to coincide, that same week, with the release of Money's book Man Woman, Boy Girl. Co-authored with his colleague Dr. Anke Ehrhardt, the book contained his first written account of the extraordinary twins case.

Man Woman, Boy Girl made mention of Joan's "tomboyish traits" in passing but focused on the ways in which she conformed to the stereotypes of female behavior - examples of which were culled from Linda's hopeful cataloging, over the years, of Joan's fitful attempts to act more like a girl. "One thing that really amazes me is that she is so feminine," Linda is quoted as saying. "I've never seen a little girl so neat and tidy as she can be when she wants to be." No mention was made of the problems Joan had been having in school.

Indeed, the account portrayed the experiment as an unqualified success - a conclusion bolstered by what Money pointed out was an "extreme unusualness" to the case. He was referring, of course, to the existence of the identical male twin, whose interest in "cars and gas pumps and tools" was contrasted to his sister's interest in "dolls, a doll house and a doll carriage" - a sharp division of tastes along gender lines that seemed to provide compelling evidence that boys and girls are made, not born. The significance of the case to the then-burgeoning women's movement was obvious, since feminists had been arguing against a biological basis for sex differences for years. Indeed, Money's own papers from the 1950's on the total psychosexual flexibility of newborns were cited by Kate Millett in her best-selling, seminal 1970 feminist text, Sexual Politics. Money's new twins case buttressed the feminist claim that the observable differences in the tastes, attitudes and behaviors of men and women are attributable solely to cultural expectations.

"This dramatic case," Time duly reported in its Jan. 8, 1973, edition, "provides strong support for a major contention of women's liberationists: that conventional patterns of masculine and feminine behavior can be altered. It also casts doubt on the theory that major sexual differences, psychological as well as anatomical, are immutably set by the genes at conception." The New York Times Book Review hailed Man Woman Boy Girl as "the most important volume in the social sciences to appear since the Kinsey reports" and praised Money for producing "real answers to that ancient question: Is it heredity or environment?" But it was on the pediatric wards of hospitals around the world that the twins case would have its most lasting impact.

"It was the hallmark case," says Dr. William Reiner a child psychologist at Johns Hopkins. "It was the hallmark because it was followed and written up a number of times by Money and then essentially was the source of his statements - and subsequent statements in any of the pediatric textbooks in endocrinology, urology, surgery and psychology - that you can reassign the sex of a child because it's the social situation that is the most important." The undisputed success of the twins case legitimized the practice of infant sex reassignment globally, says Reiner. Once confined principally to Johns Hopkins, the procedure soon spread and today is performed in virtually every major country, with the possible exception of China and India. While no annual tally of infant sex reassignments has ever been made, Reiner makes a rough, "conservative" estimate that three to five cases crop up in every major American city each year - giving the U.S. alone a total of 100 to 200 sex reassignments a year. Globally, he puts the figure at perhaps 1,000 per year. In the 25 years since Money's twins case was first published, as many as 15,000 similar sex reassignments may have been performed.




Dr. Mel Grumbach, a pediatric endocrinologist at the University of California, San Francisco, and a world authority on the subject, confirms that the findings detailed in Money's twins case were the decisive factor in the widespread acceptance of the practice. ""Doctors] were very influenced by the twin experience." he says. "John Money stood up at a conference and said, 'I've got these two twins, and one of them is now a girl, and the other is a boy.' They were saying they took this normal boy and changed him over to a girl. That's powerful. That's really powerful. I mean, what is your response to that? This case was used to reinforce the fact that you can really do anything. You can take a normal XY male and convert it into a female in the neonatal period and it won't make any difference." Grumbach adds, "John Money is a major figure, and what he says gets handed down and accepted as gospel by some."

Mickey Diamond, 1996: He disputed Money's findings from the start.But not all. In the seven years since he had first published his challenge to Money, Mickey Diamond, who had been hired as a biology professor at the University of Hawaii, continued his laboratory research into how the sexual nervous system is organized before birth. His studies had further convinced him that neither intersexes nor normal children are born psychosexually undifferentiated - a conviction that made him view with alarm the expanding practice of infant sex reassignment. And he was more convinced than ever that converting a non-intersexual infant from one sex to the other would be impossible. "But I didn't have any proof at the time," Diamond says. "I didn't have anything except a theoretical argument to challenge the case."

Diamond vowed to follow the case of the sex-changed twin closely - a decision, he says, that was affected by purely scientific motives. But if, by now, Diamond also felt a degree of personal involvement in his dispute with Money, that was perhaps understandable: In the chapter directly following his account of the twins case in Man Woman, Boy Girl, Money lashed out at Diamond and his colleagues, characterizing their work as "instrumental in wrecking the lives of unknown numbers of hermaphroditic youngsters."

In 1967, at the time of John's castration, Money stipulated that he see the child once a year for counseling. The trips, which were sometimes separated by as many as 18 months, were, as Money put it in his letter to the Thiessens' lawyer, meant to "guard against the psychological hazards" associated with growing up as a sex-reassigned child. But according to the Thiessens and to contemporaneous clinical notes, the trips to the Psychohormonal Research Unit at Johns Hopkins only exacerbated the confusion, fear and dread that Joan was already suffering.

"You get the idea something happened to you," John says of those mysterious annual visits to the unit, "but you don't know what - and you don't want to know." Kevin, who was also required on each visit to submit to sessions with Dr. Money, found the trips equally bewildering and unsettling: "For the life of me, I couldn't understand why, out of all the kids in my class, why am I the only one going with my [sister] to Baltimore to talk to this doctor? It made us feel like we were aliens." The twins developed a conviction that everyone, from their parents to Dr. Money and his colleagues, was keeping something from them. "There was something not adding up," Kevin says. "We knew that at a very early age. But we didn't make the connection. We didn't know."

All they did know was that from the time they were 6 years old, Dr. Money wanted to talk to them, both singly and together, about subjects that, as Joan would later complain to an outside therapist, "I can't even talk to my mom about."

"Dr. Money would ask me, 'Do you ever dream of having sex with women?' " Kevin recalls. "He'd say, 'Do you ever get an erection?' And the same with Joan. 'Do you think about this? About that?' "

While attempting to probe the twins' sexual psyches, Money also tried his hand at programming Kevin's and Joan's respective sense of themselves as boy and girl. One of his theories of how children form their different "gender schemes" - Money's term - was that they must understand, at an early age, the differences between male and female sex organs. Pornography, he believed, was ideal for this purpose. "Explicit sexual pictures," he wrote in his book Sexual Signatures, "can and should be used as part of a child's sex education"; such pictures, he said, "reinforce his or her own gender identity and gender role."

"He would show us pictures of kids, boys and girls, with no clothes on," Kevin says. John recalls that Dr. Money also showed them pictures of adults engaged in sexual intercourse: "He'd say to us, 'I want to show you pictures of things that moms and dads do.' "

During these visits, the twins discovered that Money had two sides to his personality. "One when mom and dad weren't around," Kevin says, "and another when they were." When their parents were present, they say, Money was avuncular, mild-spoken. But alone with the children, he could be irritable or worse. Especially when they defied him. The children were particularly resistant to Money's request that they remove their clothes and inspect each other's genitals. Though they could not know this, such inspections were central to Money's theory of how children develop a sense of themselves as boy or girl - and thus, in Money's mind, were crucial to the successful outcome of Joan's sex reassignment. As Money stressed in his writings of the period: "The firmest possible foundations for gender schemes are the differences between male and female genitals and reproductive behavior, a foundation our culture strives mightily to withhold from children. All young primates explore their own and each others' genitals . . . and that includes human children everywhere.... The only thing wrong about these activities is not to enjoy them."




But the children did not enjoy these enforced activities, which they were instructed to perform sometimes in front of Dr. Money, sometimes with as many as five or six of his colleagues in attendance. But to resist Money's requests was to provoke his ire. "I remember getting yelled at by Money because I was defiant," John says. "He told me to take my clothes off, and I just did not do it. I just stood there. And he screamed, 'Now!' Louder than that. I thought he was going to give me a whupping. So I took my clothes off and stood there, shaking." In a separate conversation with me, Kevin recalls that same incident. " 'Take your clothes off - now!' " Kevin shouts.

As early as age 8, Joan began to resist going to Baltimore. Dr. Money suggested to Linda and Frank that they sweeten the pill of the annual visits by blending the trip to Hopkins with a family vacation. "Soon," Linda says, "we were promising Disneyland and side trips to New York just to get her to go."

It was also around Joan's eighth birthday that Dr. Money began increasingly to focus on the issue of vaginal surgery. At the time of her castration at 22 months, Joan was left with only a cosmetic exterior vagina; the surgeon had elected to wait until Joan's body was closer to full grown before excavating a full vaginal canal. For Dr. Money, there was now an urgent need for Joan to prepare for this operation. Because genital appearance was critical to Money's theory of how one "learns" a sexual identity, Money believed that Joan's psychological sex change could not be complete until her physical sex change was finished.

There was only one problem: Joan was determined not to have the surgery - ever. The child's increasingly stubborn refusal was not only a result of her deep-seated fear of hospitals, doctors and needles. It also had to do with the realization that she'd made around the time of grade two - that she was not a girl and never would be, no matter what her parents, her doctor, her teachers or anyone else said. For when Joan daydreamed of an ideal future, she saw herself as a 21-year-old male with a mustache and a sports car, surrounded by admiring friends. "He was somebody I wanted to be," John says today, reflecting on this childhood fantasy. By now Joan was ever more certain that submitting to vaginal surgery would lock her into a gender in which she felt increasingly trapped.

She quietly told Dr. Money that she did not want to have the surgery. But the psychologist did not seem to want to hear this. Instead, Dr. Money would once again break out his cache of photographs of naked women. He would focus Joan's gaze on the labia, vulva, clitoris. "Can't you see that you're different?" he would say. "That's why you need the surgery."

Joan, frightened but adamant, would simply refuse to lift her eyes. "Don't you want to be a normal girl?" Dr. Money would ask repeatedly. "Don't you want to be a normal girl?"

Dr. Money also continued to probe for the content of Joan's sexual fantasies. She tried to keep this information secret from the psychologist, and she believed herself successful. But, according to Frank and Linda, she was wrong. By the time Joan turned 9, Dr. Money had informed them that something had come up in his private sessions with Joan. "Money told us that he had asked Joan what partner she would rather have, a boy or a girl," Frank recalls. "Joan had said, 'A girl.' " Frank recalls that Dr. Money wanted to know how they felt about raising a lesbian. At a loss as to how to respond to this news but relieved that Money did not seem to think it significant, Frank said what he honestly believed about homosexuality: "It's not the most important thing in life."

Money evidently agreed, for this clinical finding was not included in his next report on the twins, which appeared in 1975, when they were 10 years old. Published in the Archives of Sexual Behavior, the update was, if anything, a more glowing report than the one from three years before. After recapping the earlier findings and adding a new example of the girl's happy femininity, Money concluded: "No one [outside the family] knows [that she was born a boy]. Nor would they ever conjecture. Her behavior is so normally that of an active little girl, and so clearly different by contrast from the boyish ways of her twin brother, that it offers nothing to stimulate one's conjectures."

That same year, Money published yet another account of Joan's successful metamorphosis. But this time the intended audience was not only Money's scientific and medical colleagues but also the general public. Sexual Signatures, co-authored with journalist Patricia Tucker, was Money's bid for a wider audience. Stripped of the often-impenetrable psychological jargon that characterizes his earlier reports of the sex reassignment, the book offered Money's most unrelievedly upbeat, almost triumphant, account of the case yet. Describing Joan's sex reassignment as "dramatic proof that the gender-identity option is open at birth for normal infants," Money went on to say of baby John's castration as an infant, "The girl's subsequent history proves how well all three of them [parents and child] succeeded in adjusting to that decision."

Up to the age of 11, Joan's only psychological therapy was her annual visits to Dr. Money at John Hopkins. But this changed in the fall of 1976, when she entered a new school, where her anxiety, social isolation and fear immediately drew the attention of teachers, who, once again, notified the Child Guidance Clinic. "Joan's interests are strongly masculine," a teacher wrote in her report. "She has marvelous plans for building treehouses, go-carts with CB radios, model gas airplanes . . . and appears to be more competitive and aggressive than her brother and is much more untidy both at home and in school." A session with the clinic's psychologist revealed that Joan had "strong fears that something [had] been done to her genital organs" and that she had had "some suicidal thoughts."




Her case was referred to the unit's head of psychiatry, Dr. Keith Sigmundson, an amiable, self-deprecating 34-year-old whose career ascent had been rapid. "Because I was just ahead of the baby boomers, I got a position that I was too young for and probably didn't deserve in the first place," he says. From his very first meeting with Joan, Sigmundson was struck by the child's appearance. "She was sitting there in a skirt with her legs apart, one hand planted firmly on one knee," Sigmundson says. "There was nothing feminine about her." But despite strong misgivings, he decided that in overseeing Joan's psychiatric treatment, he would support the process that Money had begun. It had gone too far to turn back, Sigmundson decided, so he attempted to persuade the child to accept herself as a girl and to submit to vaginal surgery. To increase Joan's female identification, he referred her case to a woman psychiatrist, Dr. M.

As Dr. M.'s clinical notes reveal, early in her sessions Joan voiced her conviction that she was "just a boy with long hair in girl's clothes" and that people looked at her and said she "looks like a boy, talks like a boy." She also opened up about how she dreaded the trips to Baltimore, where people looked at her and "a man show[ed] her pictures of nude bodies." But the psychiatrist reassured Joan that she was, indeed, a girl and impressed upon her the necessity that she undergo surgery on her genitals.

Troubled nonetheless by the case, the psychiatrist wrote to Dr. Money and told him of Joan's emotional difficulties and school problems. Money wrote back in January 1977 that he was very pleased that Dr. M. was willing to become involved in treating Joan. He explained that the second stage of Joan's vaginal surgery had not yet been performed due to the child's "fanatical fear of hospitals" - a fear, Money wrote, "that I have encountered on only one other occasion in 25 years of work at Johns Hopkins." He added that mention of hormone treatments or surgery induced in Joan a "panic so intense that it's impossible to broach any conversation on such matters without the child fleeing the room, screaming." Nevertheless, Money continued, there was now an "urgency" that Joan's fears be overcome, because the need for hormone therapy and surgery was rapidly increasing with her approaching adolescence. "It will be one of the best things you can do for her," Money wrote to the psychiatrist, "if you can help her break down this extraordinary veto."

Despite all efforts, Joan continued to hold out against surgery. Nine months passed, and she remained unmovable - refusing even to permit her pediatric endocrinologist to conduct a physical exam of her genitals. Then, in the late summer of 1977, when Joan turned 12, she suddenly had to fend off an attack on another front. On her last several trips to Baltimore, Dr. Money had spoken about the medication she would soon need in order to become a "normal girl." He was talking about estrogen, the female hormone needed to simulate the effects of female puberty on Joan's broad-shouldered, narrow-hipped boy's physique. Like vaginal surgery, the prospect of developing a female figure struck Joan as nightmarish. So she was suspicious when, one day, her father produced a bottle of pills and told her to start taking them.

"What's this medicine for?" Joan asked.

Frank, struggling for the best way to put it, finally came up with: "It's to make you wear a bra."

"I said, 'I don't wanna wear a bra!' " John recalls. "I threw a fit."

But after repeated entreaties from her parents and the endocrinologist (not to mention the threat, which Dr. Money had introduced, that she would grow disproportionate limbs if she failed to take the drugs), Joan finally, and with great reluctance, began to take the pills.

It was around this time that Dr. Money authored another update on the twins. The report would appear in a 1978 journal. Once again, the outlook was sunny. "Now prepubertal in age, the girl has . . . a feminine gender identity and role, distinctly different from that of her brother," he reported. Perhaps forgetting what he had told Joan's parents four years earlier about her sexual orientation, he wrote: "The final and conclusive evidence awaits the appearance of romantic interest and erotic imagery."

Though Joan often only pretended to take her estrogen pills, by May 1978, three months prior to her 13th birthday, the effects were visible. A pair of small but distinct breasts had appeared on her chest, along with a padding of fat around her waist and hips. But she remained stubbornly opposed to further surgery - a fact that became dramatically clear during her visit that spring to Johns Hopkins. It would prove to be the last time Joan would ever consent to go to Baltimore.

That something remarkable had occurred during Joan's visit is obvious from a letter that Dr. Money wrote in August 1978, some weeks after the encounter. He said that Joan was still determined to avoid talk of sex or surgery and, when she was pressed on those points, she left the room to join her brother. "I followed," Money wrote, "and, in bringing the session to a close, put my hand on her shoulder in what most youngsters would accept as a reassurance. She fled in panic." Money then described how one of his students followed Joan to help her recover her composure. "They walked, saying little, for about a mile." In concluding his oddly elliptical-sounding account of these events, Dr. Money referred to the student as a woman.

What he did not mention was that the woman had begun life as a man. She was a male-to-female transsexual one of many readily available from the Johns Hopkins Gender Identity Clinic. She had apparently been enlisted by Money to speak to Joan about the positive aspects of surgical construction of a vagina.

"Dr. Money said, 'I've got someone for you to talk to who's been through what you're going to be going through,' " John recalls.




Joan was then ushered into the presence of a person whom she immediately identified as a man wearing makeup, dressed in women's clothing, with a woman's hairstyle. When the person spoke, it was in a breathy, artificially high-pitched voice.

"He's telling me about the surgery," John says, "how fantastic it was for her and how her life turned out beautifully."

Joan sat immobile, silent, apparently listening. But the words reached her through a clamoring, rising panic in her mind: "I was thinking, 'I'm gonna end up like that?' "

Today, John cannot remember bolting from the room. "I remember running," John says. "That's all."

Joan ran, blindly, until she reached a set of stairs, which she dashed up. She emerged onto a rooftop, where she tried to hide. But the transsexual had followed - only increasing Joan's panic. Coaxed down from the roof, Joan told her mother that if forced to return to see Dr. Money, she would kill herself.

But Dr. Money was, it seemed, not inclined to lose contact with this unique patient so easily. In early 1979, roughly eight months after Joan's last trip to Hopkins, Money wrote to Linda, saying that he would soon be passing through her city to give a talk at the local university and medical center. He said he would like to drop by the house and see the Thiessens.

On a gray day in mid-March 1979, Money arrived at their doorstep carrying only a single knapsack. The twins, aware of Money's arrival, disappeared into the basement and refused to come upstairs. The adults engaged in small talk. Money had said that he was catching a flight later in the day. But both Frank and Linda noticed that he was showing no symptoms of being in a hurry. On a tour of the small house, Money complimented Linda's ink drawings, which decorated the walls, and looked at a wooden wall cabinet that Frank had made. He reminisced about his childhood in New Zealand. Finally, Dr. Money announced that he had missed his flight. Frank and Linda looked at each other and felt that it was the right thing to do to invite Dr. Money to stay over, although they had only a foam air mattress in the front room for him to sleep on. To their surprise, the eminent psychologist from Johns Hopkins accepted the offer. In order to accommodate their unexpected house guest, the Thiessens phoned out for a bucket of chicken. The children continued to hide in the basement.

"We didn't want to come up," Kevin recalls. "We were forced into it. They said, 'Come up,' so we came up."

"I wound up being Mr. Polite," John says, recalling the stiff encounter. Kevin remembers that Dr. Money asked "general questions" about how the twins were doing in school. Kevin asked how Dr. Money liked their city and how long he was staying. "Then," Kevin says, "we wanted to go." But before the two retreated back into the basement, Dr. Money pulled out his wallet and, saying something about how he would have spent the money on a hotel room anyway, bestowed on the children $15 each. The kids fled to the basement and did not emerge until the next morning, when the world-famous sexologist had left for the airport. It was the last that the family and Dr. Money would ever see of each other.

By the time she turned 14, in August 1979, Joan had been on female hormones for almost two years. But the drugs were now in competition with her male endocrine system, which, despite the absence of testicles, was now in the full flood of puberty - a fact readily apparent not only in her loping walk and the angular manliness of her gestures, but also in the dramatic deepening of her voice, which, after a period of breaking and cracking, had dropped into its current rumbling register. Physically, her condition was such that strangers turned to stare at her (as was noted by her therapist in contemporaneous clinical notes). But to the close observer, it was Joan's mental state that would have drawn particular scrutiny and pity. For as photographs from this period reveal, Joan, for all her attempts to drag a smile onto her face, had the wounded eyes of a shamed and hunted animal.

It was at this point that Joan took the matter of her sexual destiny into her own hands and simply stopped living as a girl. Therapy notes from November 1979 reveal that she refused to wear dresses and now favored a tattered jean jacket, ragged cords and work boots. Her hair was unwashed, uncombed and matted. "I was at that age where you rebel," John says. "I got so sick to death of doing what everyone wanted me to do. I got to that point in my life, I knew I was an oddball, I was willing to live my life as an oddball.... If I wanted to wear my hair in a mess, I wore it in a mess. I wore my own clothes the way I wanted to."

And Joan had more private ways of rebelling. Since childhood she had been instructed, both by her parents and by her doctors, to urinate in the sitting position - despite a strong, overriding urge to address the toilet standing up. For years she had tried to adhere to this stricture on her bodily function. But no longer. "If no one was around, I'd stand up," John recalls. "It was no big deal; it was easier for me to do that. Just stand up and go. I figured, what difference did it make?"

But it made a difference to her peers. That fall, Joan had transferred to a technical high school, where she enrolled in an appliance-repair course. There she was quickly dubbed Cave-woman and Sasquatch and was openly told, "You're a boy." But it was her inclination to urinate in the male posture that caused the greatest friction between her and her schoolmates. The girls barred her from using their bathroom. She tried sneaking into the boys' room but was kicked out and threatened with a knifing if she returned. With nowhere else to go, Joan was reduced to urinating in a back alley. By December, she simply refused to go to school.




By now, it was impossible for the local treatment team to ignore the obvious. After almost four years of fruitlessly trying to implement Dr. Money's plan, several physicians experienced a change of heart. Among those who believed that Joan would never submit to vaginal surgery was Dr. McK., a particularly empathetic female psychiatrist, then in semi-retirement, who had taken over Joan's case in the winter of 1979. Joan's endocrinologist, Dr. W., was among the last holdouts for the surgery, since he remained certain that it was the appearance of Joan's uncompleted vagina that formed the stumbling block to her psychological acceptance of herself as a girl. But now, even he began to waver. "Early on I had . . . pushed for early surgery," he wrote in a letter to Dr. McK. "I am not as convinced now that this is a good idea and therefore at the present time have no specific plans or opinions as to the proper time for the operation."

Ultimately, Joan forced the endocrinologist to come down off the fence. During an appointment in his office, Joan refused to remove her hospital gown for a breast exam. The doctor asked again. She refused. The standoff lasted 20 minutes. "It comes to a point in your life where you say, 'I've had enough,' " John says. "There's a limit for everybody. This was my limit."

But Dr. W. had reached his limit, too. "Do you want to be a girl or not?" he demanded. It was a question Joan had heard before - a question that Money had been asking her since the dawn of her consciousness, a question the local doctors had badgered her with for four years, a question she'd heard once too often.

She raised her head and bellowed into his face: "No!"

The doctor left his office for a moment, then returned. "OK," he said. "You can get dressed and go home."

Only later would John learn that Dr. W. had, in stepping out into the hallway, spoken with Dr. McK. He told her that in his opinion, it was time that the teenager was told the truth of who she was and what had happened to her.

It was Frank's custom to pick up Joan in the car after her weekly sessions with the psychiatrist. The afternoon of March 14, 1980, was no exception. But when Joan climbed into the car that day, Frank said that instead of driving straight home, they should get an ice-cream cone.

Immediately, Joan was suspicious. "Usually, when there was some kind of disaster in the family, good old dad takes you out in the family car for a cone or something," John says. "I was thinking: 'Is mother dying? Are you guys getting a divorce? Is everything OK with Kevin?' "

"No, no," Frank said to Joan's nervous questioning. "Everything's fine."

And, indeed, he couldn't find the words to explain until Joan had bought her ice cream and Frank had pulled the car into the family's driveway.

"He just started explaining, step by step, everything that had happened to me," John says.

"It was the first time," Linda says, "that John ever saw his father cry."

Joan herself remained dry-eyed, staring straight ahead through the windshield, the ice-cream cone melting in her hand.

"She didn't cry or anything," Frank says almost two decades after this extraordinary encounter between father and child. "She just sat there, listening, real quiet. I guess she was so fascinated with this unbelievable tale that I was telling her."

Today, John says that the revelations awoke many emotions within him anger, disbelief, amazement. But he says that one emotion overrode all the others. "I was relieved," he says, blinking rapidly, his voice charged. "Suddenly it all made sense why I felt the way I did. I wasn't some sort of weirdo."

Joan did have a question for her father. It concerned that brief, charmed span of eight months directly after her birth, the only period of her life that she ever had been, or ever would be, fully intact.

"What." she asked. "was my name?"

Joan's decision to undergo a sex change was immediate. She changed her name to John and demanded male-hormone treatments and surgery to complete her metamorphosis back from girl to boy. That fall, he had his breasts surgically excised; the following summer, a rudimentary penis was constructed. The operation was completed one month prior to his 16th birthday.

Socially, John says, it proved relatively easy to effect the change to his true status. Joan's lifelong social rejection had guaranteed that no one had ever gotten close enough to her to remark on her sudden vanishing. Still, John did take the precaution of lying low for several months in his parents' basement. "Watching TV, that's all I did," says John. "I wasn't really happy; I wasn't really sad." But gradually he began to emerge, hanging out at the local fast-food joints, the roller rink and bars with Kevin and his friends, who immediately accepted him as one of the guys.

It was in John's relations to girls that complications developed - and they were only exacerbated by the fact that by age 18 he was not merely a passably attractive young man but an arrestingly handsome one. His sudden popularity with what was now the opposite sex introduced a terrible dilemma, because he knew that his penis neither resembled nor performed like the real thing (it was incapable of becoming erect). "How do you even start dating?" John says, recalling this period of his life. "You can't. You're in such an embarrassing situation. At the same time, if you're not honest with them . . . they're gonna want to start getting frisky with you."




Eventually, he did date a girl two years his junior, a pretty but flighty 16-year-old. Several months into the relationship, John entrusted her with his secret, telling her that he had suffered an "accident." Within days, John says, "everyone knew." Just as in his childhood, he was suddenly the object of muttered comments, giggling, ridicule. Days later, he swallowed a bottle of anti-depressants and lay down on his parents' sofa to die. His parents discovered him unconscious. "Me and Linda looked at each other," Frank recalls, "and we were wondering if we should wake him up."

Linda recalls her doubts: "I said to Frank, 'I wonder if we should just leave him, because that kid has done nothing but suffer all his life. He really wants to die.' Then I said, 'No, no, I can't let him die. I have to try to save him.' " They lifted him and rushed him to the hospital, where his stomach was pumped. On his release a week later, he tried it again. This time, Kevin saved him.

John withdrew from the world. He spent sojourns of up to six months at a time alone in a cabin in the woods, winter or summer. Unable to face people, he fantasized about committing a crime that would land him in solitary confinement for the rest of his days. "I despised myself; I hated myself," he says. "I hated how my life turned out. I was frustrated and angry, and I didn't know who I was angry at."

At age 21, he underwent a second operation on his penis that yielded a significant improvement over his first phalloplasty (his penis resembled a real one, and nerve grafts from his arm supplied the organ with sensation), but it would be two years before John used it for sex. The delay had less to do with his feelings of confidence about his penis, he says, than with the legacy of what had been done to him by Dr. Howard W. Jones in the operating room at Johns Hopkins when he was 22 months old. "I kept thinking, 'What am I going to say to the woman I meet who I want to marry?' " John remembers. " 'What am I going to say to her when she says she wants children and I can't give her children?' "

His brother, Kevin, had by that time married and become a father - everything that John had wanted for himself since high school. "I got so terribly lonely," John says. "I decided to do something I'd never done before. I wound up praying to God. I said, 'You know, I've had such a terrible life. I'm not going to complain to you, because you must have some idea of why you're putting me through this. But I could be a good husband if I was given the chance; I think I could be a good father if I was given a chance.' "

Two months later, Kevin and his wife introduced John to a young woman they had met. At age 26, she was three years John's senior - a pretty, loving single mother of three children by three separate fathers. "By the time I met John," she says with a rueful laugh, "I'd come to the end of my rope with men. I kept trusting them - then it was, 'You're pregnant? I'm out of here.' " She says that John's condition did not make a difference to her. "It probably would have if I didn't already have kids. But after what I'd been through with men, I figured, 'What does it matter what he's got between his legs? If he's good to me and the kids that's all that matters.' "

The two immediately hit it off. She liked John's old-fashioned gallantry. "He still sends me flowers and writes me notes," she says. "How many people have that after nine years together?" John fell in love with what he calls her "true heart."

Less than a year after they started going out, John asked her to marry him. She accepted, and when John was 25, they wed. John landed a well-paying factory job, bought a house in a trim and tidy middle-class neighborhood near his parents, and settled down with his wife and three adopted children into a life of domestic anonymity.

For years, Keith Sigmundson had been seeing the advertisements. They appeared like clockwork every year in the American Psychiatric Society Journal, and they always said the same thing: "Will whoever is treating the twins please report." Below this entreaty was always the same address: Dr. Milton Diamond, University of Hawaii. "I would see it," Sigmundson says, "but I couldn't bring myself to answer."

In the past, Sigmundson himself had toyed with the idea of publishing the true outcome of John's case. But he hadn't done it - and for a very simple reason. "I was shit-scared of John Money," he admits. "He was the big guy. The guru. I didn't know what it would do to my career." So he would put the idea out of his head. Diamond's annual ad was an awkward reminder. A couple of times, he'd almost answered it. But he'd always resisted the urge.

Diamond, however, was not one to give up so easily. At 63, he's a sad-eyed man with the white beard of a scholar, his intensity hidden behind soft-spokenness. Diamond is the author of more than a hundred journal articles and eight books on sexuality. The majority of Diamond's time in Honolulu during the past 30 years has been spent hunched over his computer in the cluttered, windowless office he calls his "cave," his work habits obvious to anyone who has seen his pale skin. It was from his cave that Diamond, in early 1991, decided to redouble his efforts to locate, and learn the fate of, the famous twins. That spring, he managed to track down Dr. M., the psychiatrist who had treated Joan Thiessen almost 21, years earlier. She had moved from the Thiessens' hometown soon after referring Joan to a new psychiatrist and thus knew nothing of the girl's sex change. She did, however, offer to give Diamond a phone number for the man who had overseen Joan's psychiatric treatment: Keith Sigmundson.

"It's funny," Diamond says with a chuckle, "I remember the first words Sigmundson said to me [when I called]. It was to the effect of, 'I was wondering how long it would take for you to get here.' "

Sigmundson shakes his head at the memory of the call he'd been half hoping for, half dreading

"Mickey said, 'Keith, we gotta do this,' " Sigmundson recalls. "I said, 'Well, I haven't really got the time and the energy....' So Mickey kept on badgering me a little bit."




As someone who had himself seen firsthand the disastrous results of a so-called "successful" sex reassignment, Sigmundson was inclined to agree with Diamond's argument that the procedure is wrongheaded. But Sigmundson admits that some of his reservations about joining Diamond in a long-term follow-up on John's case derived from colleagues who had warned him that Diamond was a "fanatic" with an ax to grind regarding Dr. Money. Further conversations with Diamond, and a reading of his journal articles on sexual development, convinced Sigmundson otherwise: "I came to see that Mickey is a serious researcher and a caring guy who really believed that Money's theory had caused - and was continuing to cause - great harm to children." Sigmundson agreed to contact John Thiessen and to ask if he would be willing to cooperate with a follow-up article on his case.

By then, John had been married for two years and wanted nothing more than to put his tortured past behind him. He, at first, refused to participate. But in a later meeting with Dr. Diamond - who flew in from Hawaii, John learned, for the first time, about his fame in the medical literature and how his reportedly successful switch from boy to girl stood as the precedent upon which thousands of sex reassignments had since been performed - and continued to be performed at an estimated rate of five a day globally. "There are people who are going through what you're going through every day," John recalls Diamond telling him, "and we're trying to stop that."

That was good enough for John. In the spring of 1994, and over the course of the following year, John, his mother and his wife sat for a series of interviews with Diamond and Sigmundson in which they recounted John's harrowing journey from boy to girl and back again. Using these interviews, plus the detailed clinical records that Sigmundson had kept on Joan's case, Diamond wrote up the results in a paper in which John's life was cast as living proof of precisely the opposite of what Money had said it proved 25 years earlier. Diamond wrote that John's case is evidence that gender identity and sexual orientation are largely inborn, and that while rearing may play a role in helping to shape a person's sexual identity, nature is by far the stronger of the two forces so much so that even the concerted 12-year efforts of parents, psychologists, psychiatrists, surgeons and hormone specialists could not override it.

The paper, powerful as it was as anecdotal evidence of the neurobiological basis of sexuality, was also a clear warning to physicians about the dangers of sexual reassignment - and not just for children like John, who are born with normal genitals. Diamond argued that the procedure is equally misguided for intersexual newborns, since physicians have no way of knowing in which direction, male or female the infant's gender identity has differentiated. To stream such children, surgically, into one sex or the other, Diamond argued, is guesswork that consigns a large percent of them to lives as tortured as John Thiessen's.

It took nearly two years for Diamond and Sigmundson to find a publisher for their paper. "We were turned down by all these journals that said it was too controversial," says Sigmundson. "The New England Journal, American Psychiatric, American Pediatric." The article was finally accepted for publication by the American Medical Association's Archives of Adolescent and Pediatric Medicine in September 1996, with publication set for March 1997. In the intervening seven months, Diamond and Sigmundson felt considerable apprehension as they waited for their bombshell to go off. "We were basically telling all these physicians that they'd been doing the wrong thing for the past 30 years," Sigmundson says. "We knew we were going to be pissing a lot of people off."

They were not wrong. One pediatric endocrinologist who has attended medical meetings on the subject since the article's publication has reported that the discussions cannot even be termed debates: "It's like screaming fights in these medical conventions at the moment." Some critics of the article have attempted to dismiss it on the grounds that Diamond is simply using John's history to embarrass a scientific rival. But Dr. Melvin Grumbach, the eminence grise of pediatric endocrinology, offers a more measured response. "I think Diamond does have a case," he says. "I think testosterone in utero and an XY-chromosome constitution does do things to you. But the question is: Is it invariable?"

Grumbach points out that sex reassignment is always done as a last resort and only when every other treatment option has been ruled out. And while he admits that sex reassignments are not foolproof, Grumbach insists that they can, and do, work "with good support." But asked to offer up a "satisfied customer," Grumbach voices the Catch-22 of every pediatric specialist contacted for this article. "I really lose track of all my patients after young adulthood," he says.

Astonishingly, in the four decades since the first sex reassignments were performed, no comprehensive, long-term follow-up study of the patients has ever been conducted. Such a study was, finally, launched at the Johns Hopkins medical center in June 1995. Child psychiatrist (and former pediatric urologist) Bill Reiner has been following the lives of 16 reassigned people, focusing on six genetic males who were born without penises, castrated in infancy and raised as girls. Two years into his study, Reiner says that all six are closer to males than to females in attitudes and behavior. Two have spontaneously (without being told of their XY male chromosome status) switched back to being boys. "These are children who did not have penises," Reiner points out, "who had been reared as girls and yet knew they were boys. They don't say, 'I wish I was a boy,' or 'I'd really rather be a boy,' or 'I think I'm a boy.' They say, 'I am a boy.' " Reiner (who wrote a supportive editorial to accompany Diamond and Sigmundson's John/Joan paper) points to the parallel between the children he is studying and Joan Thiessen, who also "knew," against all evidence to the contrary, that she was a he.




Reiner says that both the John/Joan case and the trend in his study support the findings that have emerged since Diamond's early-1960s research into the neurobiological origins of gender identity and sexual orientation. A 1971 study done at Oxford University showed anatomical differences between the male and female brain in rats - and six years later, at UCLA, researchers narrowed these differences to a cluster of cells in a gland in the brain called the hypothalamus. A study done in the mid-l980s in Amsterdam located the corresponding area in the human hypothalamus, noting that it is twice as large in homosexual men as it is in heterosexual men. Further studies done by others in the early 1990s support this finding. Then, in 1993 and again in 1995, researcher Dean Hamer announced a breakthrough on the genetic front: He was able, in two separate studies of gay male brothers, to find a certain distinctive pattern on their X chromosomes. The finding suggests that male homosexuality may have a genetic origin.

While many of these studies still need to be replicated, few sex researchers today dispute the mounting evidence of a strong inborn bias for sex and sexuality. "Which is why," Reiner says, "I have been advising physicians to be very prudent when prescribing sex reassignment for infants. Because it's quite clear that the vast majority of boys born with functioning testicles have masculine brains." Reiner endorses Diamond and Sigmundson's recommendation (published in a recent journal article) that in cases of injury or intersexuality, the assignment of sex be made socially, in terms of hair length, clothing and name, but any irreversible surgical intervention be delayed until the children are old enough to know, and are able to say, which gender they feel closest to. "We have to learn to listen to the children themselves," Reiner says. "They're the ones who are going to tell us what is the right thing to do."

Well before Diamond and Sigmundson's journal article appeared in the Archives of Adolescent and Pediatric Medicine last March, the American Medical Association's PR department alerted the media that something explosive was coming. "The AMA knew it was a big deal," Diamond says, "so they notified the big newspapers in advance." On the day of the article's publication, the New York Times ran a front-page story headlined SEXUAL IDENTITY NOT PLIABLE AFTER ALL, REPORT SAYS, which described John Thiessen's life as having "the force of allegory." Time (24 years after publishing news of the case's success) now ran a story declaring, "The experts had it all wrong." Similar news accounts appeared around the world - and soon Diamond was deluged with calls from reporters in several countries seeking interviews with the young man now known simply as John/Joan.

I met John for the first time in New York City in June 1997. Dr. Diamond, with whom I had spent months corresponding and whom I had visited in Hawaii, made the introduction. At that first meeting, John spoke bluntly about his difficulty in trusting strangers, but he quickly decided to talk to me for publication. His decision was based on his desire to warn people about the perils of infant sex reassignment. Over a beer at the Hard Rock Cafe on 57th Street, he began our conversation by telling me that he owes his survival to his family, his sole comfort in a childhood that he called "a pit of darkness." But a formidable sense of humor also clearly played a role in John's ability to rise above his sufferings. Describing the physical differences between him and his heavier, slightly balding twin, he shouted over the pounding music: "I'm the young, cool Elvis. He's the fat, old Elvis." But the strongest impression I was left with after that first meeting was of John's intense, unequivocal masculinity. His gestures, walk, attitudes, tastes, vocabulary - none of them betrayed the least hint that he had been raised as a girl. And, indeed, when asked whether he thought that his extraordinary childhood had given him a special insight into women, he dismissed the question. Like the sex-reassigned boys in Reiner's study, John had apparently never been a girl - not in his mind, where it counts.

John's story, as told by Diamond and Sigmundson, loosed a flood of coverage on television and in magazines and newspapers on the heretofore unexamined phenomenon of infant sex reassignment. With this coverage, another set of voices in the debate began to be heard. These are the voices of those intersexes born after the publication of Money's 1955 protocols. Once cloaked in shame and silence, they had already begun to emerge, largely because of the efforts of one person: a San Francisco activist named Cheryl Chase.

Cheryl Chase: Christened Charlie, reassigned at age 1, now an intersex activistAt her birth, in 1956 in New Jersey, Chase presented a classic case of ambiguous genitalia - with a somewhat vaginalike opening behind the urethra and a phallic structure of a size and shape that could be described as either an enlarged clitoris (if she was assigned as a girl) or a micropenis (if assigned as a boy). After three days of deliberation, the doctors assigned Chase as a boy. She was christened Charlie. But a year and a half later, her parents, still troubled by Charlie's unusual appearance, consulted another team of experts, who (partly on the basis of her fairly normal vagina) reassigned her as a girl. Her name was changed from Charlie to Cheryl, and her phallus was amputated.

Like John Thiessen, Chase was then raised without knowledge of her true birth status (though her entire family knew). Thus, like John, she suffered a childhood punctuated with mysterious, unexplained surgeries and genital and rectal exams. Also like John, she grew up confused about her gender. "I was more interested in guns and radios," Chase says, "and if I tried to socialize with any kids, it was generally boys, and I would try to physically best my brother." As a pre-adolescent, she recognized that her erotic orientation was toward females.




At 19, Chase understood that she'd been subjected to a clitoridectomy. She began an investigation into her medical history but was thwarted by her doctors, who refused to reveal her past. It took three years for her to find a doctor who would show Chase her medical records. Only then did she learn that she had been born a "true hermaphrodite" - a person with both ovarian and testicular tissue - and that the operation she had undergone at age 8 (to relieve "stomachaches") had actually been to cut away the testicular part of her gonads.

Horrified and angered at the deception perpetrated upon her, and aggrieved at the loss of her clitoris, which has rendered her incapable of orgasm, Chase began to seek out others like her for emotional support. Through Internet postings and mailings, she established a network of intersexes in cities across the country and, in 1993, dubbed the group the Intersex Society of North America, a peer-support, activist and advocacy group.

To meet with Chase and members of ISNA - as I did last spring, when they held a peaceful demonstration outside Columbia Presbyterian Hospital, in New York, where Chase's clitoral amputation was conducted - is to enter a world where it is impossible to think of sex with the binary, boy-girl, man-woman distinction we're accustomed to. There's Heidi Walcutt (genetically female but born with uterine, ovarian and testicular tissue and a micropenis, she describes herself as a "true American patchwork quilt of gender") and Martha Coventry, who was born with a penis-sized clitoris but a fully functioning female reproductive system and is the mother of two girls. Kira Triea was assigned as a boy at age 2 and did not learn of her intersexuality until puberty, when she began to menstruate through her phallus. She was a patient of Dr. Money's at the Johns Hopkins Psychohormonal Research Unit from age 14 to 17; this was in the mid-1970s, concurrent with John Theissen.

They have never met, but Triea's story bears striking parallels to his. She describes how Dr. Money, evidently attempting to ascertain whether she had assumed a male or female gender identity, questioned her about her sex life - in the frank language for which he was well known. "Have you ever fucked somebody?" she remembers Dr. Money asking. "Wouldn't you like to fuck somebody?" She also describes how Dr. Money showed her a pornographic movie. "He wanted to know who I identified with in this movie," she says. Contrary to Money's theory that an intersex reared as a boy will likely develop a male gender identity, Triea's sexuality and sense of self were far more complicated than that. At 17, she agreed to undergo feminizing surgery to create female genitals, but when she became sexually active for the first time, at age 32, her erotic orientation was toward women.

Impossible to classify as simply male or female, Chase and her colleagues want to, she says, "end the idea that it's monstrous to be different."

Chase emphasizes that ISNA's aim is to abolish all cosmetic genital surgery on infants - whether it be the full castration and sex reversal of microphallus boys or the supposedly less intrusive process of reducing a girl's enlarged clitoris. Chase says that such procedures are equally invasive. She denounces as "barbaric" the medically unnecessary treatments on newborns, who are not in a position to authorize surgery that may have an irreversible effect on their erotic or reproductive functioning. And Chase strongly endorses Diamond and Sigmundson's new recommendation against operating on newborns with ambiguous genitalia.

The medical establishment, she says, has shunned ISNA. According to Chase, she has tried for six years to gain an audience with the leading pediatric endocrinologists and surgeons at Johns Hopkins and elsewhere. They have refused to speak to her. Indeed, in a 1996 New York Times article on Chase and ISNA, Dr. John Gearhart, head of pediatric urology at Hopkins, dismissed the group as "zealots." In a conversation with me, he addressed ISNA's complaints. He maintained that sex reassignment is a viable option for boys who are born with micropenises or who lose their penises to injury although he adds that advances in penile reconstruction make him more hesitant to recommend the procedure today. "If John/Joan happened today," he says, "I would sit down with those parents and say, 'The child has testicles; it's a normal male child; and we can now make penises, and they're pretty functional and pretty cosmetic' - and I would probably not give them the option. I would suggest that youcould change the child's gender, but I would not recommend that, because reconstructive genital surgery has come light years since John/Joan's accident."

Gearhart insists that advances in medicine render ISNA's concerns obsolete. "When these people in ISNA were operated on, 25 and 30 years ago, there weren't really children's reconstructive surgeons around," he says. "So most of [these babies] had their clitoris or their penis amputated. That was wrong. OK? That was wrong. But the surgeons didn't know any better. Nowadays, people in modern reconstructive surgery are not cutting off little babies' clitorises or penises, or anything along those lines." Gearhart says that modern microsurgery retains sensation. "And if sensation is important to orgasm," he says, "then we retain orgasm."

Chase disputes this and says that Gearhart's electric-diagnostic test of sensation, which is administered immediately following genital surgery, doesn't prove anything. "How this [test] relates to sexual function 15, or 20 years later is anybody's guess," she says.




Chase says she understands why the medical establishment has resisted listening to ISNA. As she once wrote: "Our position implies that they have unwittingly at best and through willful denial at worst - spent their careers inflicting a profound harm from which their patients will never fully recover." So she does not expect doctors like Gearhart to change their views unless forced. "I think a context will open up for surgeons who keep doing this to be vulnerable to lawsuits," Chase says. "But it's going to take a while to create that context. Right now, we can't sue, because it's standard practice and parents give permission. The first thing that we want to have happen is that when they recommend this to parents, they tell them it's experimental and there's no evidence that it works and that there's plenty of people who've had it done to them who are mad as hell."

Other large changes will have to take place. Anne Fausto-Sterling, an embryologist at Brown University, endorses Diamond and Sigmundson's recommendation for delaying surgery but says that the medical establishment will have to provide education and emotional support to help parents with the difficult task of raising an infant whose genitals are atypical.

"A different kind of support system has to start getting built," Fausto-Sterling says. "At the moment there is no ongoing counseling done by people skilled in psychosexual development." Currently, she points out, counseling is done neither by experts trained in gender issues or psychology nor by intersexual peer-support counselors - it's handled by surgeons or endocrinologists, who conduct only cursory follow-up exams once a year. "If there was really a wholesale change in this," she continues, "the medical profession would have to do something like what they've done with genetic counseling - which is to develop a specialty of people who would work with these families long-term and help them resolve both emotional and practical questions. The practical questions are very real: 'What do I do when it comes to undressing in gym? How do I intervene with the school system?' There are a lot of things that have to happen to make what I'm arguing or Cheryl's arguing or Mickey's arguing work. There's a different infrastructure that has to get built and put into place. I think it's the responsibility of the medical profession to do it."

Now 76 years old and in semi-retirement, John Money has nevertheless remained a prolific and opinionated writer on the subject of sex and sexuality. His latest book, called Principles of Developmental Sexology, came out this year. Through the 1980s, his books and articles continued to appear with regularity - although his later work showed a shift from his earlier extreme position on the primacy of rearing over biology in the making of boys and girls. Indeed, in a May 1988 Psychology Today profile publicizing the publication of his book Gay, Straight and In-Between, Money characterized himself as a longtime champion of the role of biology in psychological sex differentiation. Money is quoted saying that in the 1950s, when he was publishing papers on the behavioral influence of prenatal sex hormones, "many people in various branches of the social sciences were just enraged at the idea that hormones in the bloodstream before you were born could have a sex-differentiating influence on you." In the same article, Money reiterated his claim that male babies with undeveloped penises and fully formed testicles can, with surgery and hormone treatment, be turned into heterosexual women.

To the many news organizations that requested comment from Money about the now-infamous John/Joan case, the psychologist refused to speak, citing confidentiality laws.

But he did speak with me briefly on the phone in early November, after six months of appeals. Though he refused to discuss John Thiessen directly, Money claimed that the media's reporting of the case has reflected a conservative bias. "It's part of the antifeminist movement," he said. "They say masculinity and femininity are built into the genes, so women should get back to the mattress and the kitchen." As to his failure to report the case's outcome, Money was unapologetic, saying that he had lost contact with the Thiessens when they did not return to Johns Hopkins and that the opportunity to conduct a follow-up had been denied to him. He stood by his original reporting of the case and dismissed my suggestion that he "misperceived" what was going on with the child. Furthermore, he implied that John's sex change to male at age 15 may not have been entirely his own decision. "I have no idea," Money said, "how much he was coached in what he wanted, since I haven't seen the person." He also hinted that the Diamond-Sigmundson paper had a hidden agenda. "There is no reason I should have been excluded from the follow-up, was there?" he asked. "Someone had a knife in my back. But it's not uncommon in science. The minute you stick your head up above the grass, there's a gunman ready to shoot you." (Diamond insists that there was "nothing personal" in his decision to publish the outcome of John's case.)

When I asked Money about Diamond's appeal to delay surgery on intersexual babies until they are old enough to speak for themselves, Money emphatically rejected the idea. "You cannot be an it," he declared, adding that Diamond's recommendations would lead intersexes back to the days when they locked themselves away in shame or worked as circus freaks.

I reminded Money that his book Man Woman, Boy Girl is still in print and that it reports the John/Joan case as a success. Asked if it would not be worthwhile for him to make changes in the text for a future edition, Money said flatly, "I'll be dead by then."




John Thiessen's final contact with Dr. Money was almost 20 years ago, when the famous sexologist slipped him $15 in his parents' living room. In the intervening years, John has often imagined what he might say, or do, to the psychologist if they were ever to meet face to face. As a younger man, his fantasies, he admits, ran to violence. But no more. "What's done," John says, "is done." He refuses to dwell on a past that he cannot change. In their paper, Diamond and Sigmundson describe John as a "forward-looking person." In conversation, Diamond calls him a "true hero." John's life today defies the dire prognosis of the local psychiatrist who, 31 years ago, declared that John would never marry and "must live apart." John's second phalloplasty allows him to have intercourse with his wife, and he is a strict but loving father to their three children, ages 15, 12 and 9. He has even mustered the emotional maturity to tell his eldest child about his painful history. And he prefers to focus on the positive changes that have resulted from his speaking out in public. For despite the brave four-year efforts of Cheryl Chase, despite the 30 years that Mickey Diamond spent trying to warn the medical establishment about the dangers of the current protocols for treatment of ambiguous or injured genitals, and despite the long-term follow-up of sex-reassigned youngsters in Bill Reiner's study, the medical establishment remained unwilling to address the issue until John went public.

His story has shaken to its foundations the edifice constructed on John Money's theories from the 1950s. And it has exposed a central flaw in a theory that has held sway for most of the 20th century. It was Sigmund Freud who first stated that a child's healthy psychological development as a boy or a girl rests largely on the presence, or absence, of the penis - the notion central to Money's theory of sexual development and the ultimate reason that John Thiessen was converted to girlhood in the first place. It is a notion that, today, has also been called into question by neurobiological research that, in the sexual realm, is leading scientists toward the conclusion that, as Dr. Reiner puts it, "the most important sex organ is not the genitals; it's the brain."

John Thiessen puts it another way when he speaks of his pride in his role as husband, father and sole breadwinner in the family that he never believed he would be lucky enough to have. "From what I've been taught by my father," he says, "what makes you a man is: You treat your wife well. You put a roof over your family's head. You're a good father. Things like that add up much more to being a man than just bang bang bang - sex. I guess John Money would consider my children's biological fathers to be real men. But they didn't stick around to raise the children. I did. That, to me, is a man."



next: Intersex Survivors of Domestic Violence
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APA Reference
Staff, H. (2007, August 9). The True Story of John / Joan, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/the-true-story-of-john-joan

Last Updated: April 21, 2017

Syndromes of Abnormal Sex Differentiation

I. Introduction

From the Johns Hopkins Children's Center, this booklet is designed to help parents and patients understand intersexuality and the challenges that accompany syndromes of "abnormal" sex differentiation.

Sexual differentiation is a complex process which results in a newborn baby who is either male or female. If errors in development occur, sexual development is abnormal and the sex organs of the baby are malformed. In such cases, individuals may develop both male and female characteristics. This is referred to as intersexuality.

Children born with deviations from normal development of the sex organs can be expected to grow up successfully and to lead enriched lives. However, their problems must be considered carefully. In cases of abnormal sex differentiation, efforts should be made to determine the reason for the abnormality as treatment may vary according to the cause of the disorder. There may also be a need for specific surgical repair and/or hormonal therapy. Finally, it is extremely important for parents and patients to have a good understanding of both the condition of sex differentiation that affects them, as well as possible ways for dealing with the condition. With this approach, patients will be better able to lead a fulfilled life, and to look forward to an education, career, marriage, and parenthood.

This booklet has been prepared to help parents and patients better understand intersexuality and the unique challenges that accompany syndromes of abnormal sex differentiation. We believe that informed individuals are better prepared to face these challenges and are more likely to meet successfully the demands of childhood, adolescence, and adulthood.

First, normal sex differentiation will be described. The understanding of this pattern of development will help patients and their families to understand the problems of ambiguous sex differentiation, which are subsequently outlined. Finally, a glossary of terms and a list of helpful support groups are provided.

II. Normal Sex Differentiation

Human sexual differentiation is a complicated process. In a simple manner, one can describe four major steps which constitute normal sexual differentiation. These four steps are:

  1. Fertilization and determination of genetic sex
  2. Formation of organs common to both sexes
  3. Gonadal differentiation
  4. Differentiation of the internal ducts and external genitalia

Step 1: Fertilization and Determination of Genetic Sex

The first step of sex differentiation takes place at fertilization. An egg from the mother, which contains 23 chromosomes (including an X chromosome), is combined with a sperm from the father, which also contains 23 chromosomes (including either an X or Y chromosome). Therefore, the fertilized egg has either a 46,XX (genetic female) or 46,XY (genetic male) karyotype.

Step 1 in sex differentiation: Determination of genetic sex

Egg (23,X) + Sperm (23,X)=46,XX genetic girl

OR

Egg (23,X) + Sperm (23, Y)=46, XY genetic boy

Step 2: Formation of Organs Common to Both Sexes

The fertilized egg multiplies to form a large number of cells, all of which are similar to each other. However, at specific times during the growth of an embryo, the cells differentiate to form the various organs of the body. Included in this development is the differentiation of the sex organs. At that stage, both 46,XX and 46,XY fetuses have similar sex organs, specifically:

  1. the gonadal ridges
  2. the internal ducts
  3. the external genitalia

a. The gonadal ridges can be easily recognized by 4-5 weeks of gestation. At that time, they already include the undifferentiated germ cells which will later develop into either eggs or sperm. The formation of gonadal ridges similar in both sexes is a prerequisite step to the development of differentiated gonads. This organization of cells into a ridge requires the effects of several genes, such as SF-1, DAX-1, SOX-9, etc. If any one of these genes is non-functional, then there is no formation of a gonadal ridge and therefore no formation of either testes or ovaries.

b. By 6-7 weeks of fetal life, fetuses of both sexes have two sets of internal ducts, the Mullerian (female) ducts and the Wolffian (male) ducts.

c. The external genitalia at 6-7 weeks gestation appear female and include a genital tubercle, the genital folds, urethral folds and a urogenital opening. (see Figure 2)




Step 3: Gonadal Differentiation

The important event in gonadal differentiation is the commitment of the gonadal ridge to become either an ovary or a testis.

a. In males, the gonadal ridge develops into testes as a result of a product from a gene located on the Y chromosome. This product has been termed the "testis determining factor" or "sex determining region of the Y chromosome" (SRY).

b. In females, the absence of SRY, due to the absence of a Y chromosome, permits the expression of other genes which will trigger the gonadal ridge to develop into ovaries.

Step 3 in Sex Differentiation: Determination of Gonadal Sex

XX fetus=ovary
(with no SRY)

OR

XY fetus=testes
(with SRY located on the Y chromosome)

Step 4: Differentiation of the Internal Ducts and External Genitalia

The next step in sex differentiation depends upon the formation of two important hormones: the secretion of Mullerian (female) Inhibiting Substance (MIS) and the secretion of androgens.

If testes are developing normally, then Sertoli cells of the developing testes produce MIS which inhibits the growth of the female Mullerian ducts (the uterus and fallopian tubes) which are present in all fetuses early in development. Additionally, the Leydig cells of the testes start secreting androgens. Androgens are hormones that produce growth effects on the male Wolffian ducts (the epididymis, vas deferens, seminal vesicles) which are also present in all fetuses early in development.

Unlike the testes, the ovaries do not produce androgens. As a result, the Wolffian ducts fail to grow and consequently disappear in fetuses with ovarian development. In addition, the ovaries do not produce MIS at the appropriate time, and as a consequence, the Mullerian (female) ducts can develop.

In other words, two products of the developing testes are needed for normal male development. First, MIS must be secreted to inhibit female duct growth and androgens must be secreted to enhance male duct growth. In contrast, a female fetus with no developing testes will produce neither MIS nor androgens, and hence female ducts will develop and male ducts will disappear.

Step 4 in Sex Differentiation: Determination of Internal Ducts

Males
Testes produce MIS=inhibit female development
Testes produce androgens=enhance male development

OR

Females
Ovaries do not produce MIS=enhance female development
Ovaries do not produce androgens=inhibit male development

External Genitalia

In the female, absence of androgens permits the external genitalia to remain feminine: the genital tubercle becomes the clitoris, the genital swellings become the labia majora and the genital folds become the labia minora.

In the male, fetal androgens from the testes masculinize the external genitalia. The genital tubercle grows to become the penis and the genital swellings fuse to form the scrotum. The following diagrams illustrate each of these processes.




Figure 1

Genitalia

Figure 2

Genitalia

 

Figure 3

Genitalia

 




Summary of Normal Sex Differentiation

  • genetic sex is determined
  • testes develop in XY fetus, ovaries develop in XX fetus
  • XY fetus produces MIS and androgens and XX fetus does not
  • XY fetus develops Wolffian ducts and XX fetus develops Mullerian ducts
  • XY fetus masculinizes the female genitalia to make it male and the XX fetus retains female genitalia

III. Disorders of Sex Differentiation - A General Outline

Sex differentiation is a complex physiological process comprised of many steps. Problems associated with sex differentiation, or syndromes of intersexuality, occur when errors in development take place at any
of these steps.

Genetic Sex

Problems can arise at fertilization when chromosomal sex is established. For example, girls with Turner Syndrome have a 45,XO karyotype and boys with Klinefelter Syndrome have a 47,XXY karyotype. It is also known that some women have a 46,XY or 47,XXX karyotype and some men a 46,XX or 47,XYY karyotype. Clearly then, when it is stated that 46,XY refers to male sex and 46,XX refers to female sex, this is a generalization which applies to most, but not all, individuals.

Gonadal Sex

Disorders of sex differentiation can occur when a bipotential gonad is incapable of developing into a testis or an ovary. The inability to develop testes may occur if a gene such as SRY is absent or deficient. When this is the case, a 46,XY fetus will not receive the SRY signal to develop testes despite the presence of a Y chromosome. Additionally, 46,XY fetuses may begin to develop testes, but this development can be thwarted, and subsequently MIS and androgen production may be absent or diminished.

Finally, the normal disappearance of germ cells associated with ovarian development in fetuses is so accelerated in Turner Syndrome that by birth these babies possess gonadal streaks as opposed to normal ovaries.

Mullerian and Wolffian Duct Development

Intersexuality can also result as a consequence of problems related to Mullerian or Wolffian duct development. For example, MIS secretion accompanied by the absence of androgens or the inability to respond to androgens can result in a fetus lacking both male and female internal duct structures. In contrast, the absence of MIS accompanied by androgen secretion can result in a fetus possessing both male and female internal duct structures to varying degrees.

External Genitalia

Babies born with sex differentiation syndromes possess external genitalia that can usually be classified as either:

  1. normal female
  2. ambiguous
  3. normal male but with a very small penis (micropenis)

Normal female external genitalia develop among 46,XY intersex patients when the genital tubercle, genital swellings, and genital folds either completely lack exposure to, or are totally incapable of responding to, male hormones. As a result, masculinization of the external genital structures is not possible. In such cases, the genital tubercle develops into a clitoris, the genital swellings develop into the labia majora and the genital folds develop into the labia minora.

Ambiguous external genitalia develop in female patients when the external genital structures are exposed to greater-than-normal amounts of male hormones (masculinized females) or in male patients when less-than-normal amounts of male hormones (under-masculinized males) occurs. Thus, in these patients, external genitalia develop in a manner that is neither female nor male, but rather is somewhere in between the two.

For instance, patients with ambiguous external genitalia may possess a phallus which ranges in size from resembling a large clitoris to a small penis. Additionally, these patients may possess a structure that resembles partially fused labia or a split scrotum. Finally, patients with ambiguous external genitalia often possess a urethral (urinary) opening that is not at the tip of the phallus (normal male position), but is instead located elsewhere on the phallus or perineum. The atypical positioning of the urethra in such instances is referred to as hypospadius.

Babies born with a penis that is much smaller than normal (micropenis) have a completely normal appearing external genitalia (i.e)., the urethra is properly located at the tip of the phallus and the scrotum is completely fused). However, the size of the phallus is closer to that of a normal clitoris than a normal penis.




IV. Specific Syndromes of Sex Differentiation

1. Androgen Insensitivity Syndrome (AIS)

Androgen Insensitivity Syndrome occurs when an individual, due to a mutation of the androgen receptor gene, is incapable of responding to androgens. Two forms of AIS exist, Complete AIS (CAIS) and Partial AIS (PAIS).

CAIS


CAIS affects 46,XY individuals. CAIS patients have normal appearing female external genitalia due to the their complete inability to respond to androgens. This is because the genital tubercle, genital swellings, and genital folds can not masculinize in these patients despite the presence of functional testes located in the abdomen. Similarly, Wolffian duct development does not occur because the Wolffian duct structures can not respond to androgens produced by CAIS patients. Mullerian duct development is inhibited in CAIS individuals because MIS is secreted by the testes.

In addition to possessing normal female external genitalia, CAIS individuals also experience normal female breast development along with sparse pubic and axillary hair growth at puberty. The following chart illustrates the steps of sex differentiation associated with CAIS compared to those of unaffected males and females.

Normal Female Development CAIS Development Normal Male Development
XX XY XY
ovaries develop testes develop testes develop
no androgen produced androgen produced, but body can not respond androgen produced
Wolffian Ducts regress Wolffian Ducts regress Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts do not develop Mullerian Ducts do not develop
external genitalia are female external genitalia are female external genitalia are male
feminizing puberty feminizing puberty without menses masculinizing puberty

PAIS

PAIS also affects 46,XY individuals. PAIS patients are born with ambiguous external genitalia due to their partial inability to respond to androgens. The genital tubercle is larger than a clitoris but smaller than a penis, a partially fused labia/scrotum may be present, the testes may be undescended, and perineal hypospadius is often present. Wolffian duct development is minimal or nonexistent and the Mullerian duct system does not develop properly.

PAIS patients will experience normal female breast development at puberty, along with a small amount of pubic and axillary hair. The chart on the following page illustrates the steps of sex differentiation associated with PAIS compared to those of unaffected males and females.

Normal Female Development PAIS Development Normal Male Development
XX XY XY
ovaries develop testes develop testes develop
no androgen produced androgen produced, but body partially unresponsive androgen produced
Wolffian Ducts regress Wolffian Ducts develop minimally Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts do not develop Mullerian Ducts do not develop
external genitals are female external genitalia are ambiguous external genitalia are male
feminizing puberty partial masculinizing puberty with testosterone therapy
OR
feminizing puberty with estrogen therapy
masculinizing puberty



2. Gonadal Dysgenesis

Unlike AIS in which affected individuals possess functioning testes but can not respond to the androgens their testes produce, patients with Gonadal Dysgenesis can respond to androgens but develop abnormal testes which are incapable of producing androgens. Like AIS, two forms of Gonadal Dysgenesis exist (Complete and Partial).

Complete Gonadal Dysgenesis

Complete Gonadal Dysgenesis affects 46,XY individuals and is characterized by abnormally formed gonads which were originally on the path to testis differentiation (these abnormally formed gonads are referred to as gonadal streaks), female external genitalia, Mullerian duct development, and Wolffian duct regression. Female external genitalia develop due to the failure of the gonadal streaks to produce androgens necessary to masculinize the genital turbercle, genital swellings, and genital folds. Additionally, because the gonadal streaks are incapable of producing either androgens or MIS, the Wolffian duct system regresses while the Mullerian duct system develops. The following chart illustrates the steps of sex differentiation associated with Complete Gonadal Dysgenesis compared to those of unaffected males and females.

Normal Female Development Complete Gonadal Dysgenesis Normal Male Development
XX XY XY
ovaries develop streak gonads testes develop
no androgen produced no androgen produced androgen produced
Wolffian Ducts regress Wolffian Ducts regress Wolffian Ducts develop
no MIS produced no MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts develop Mullerian Ducts regress
external genitalia are female external genitalia are female external genitalia are male
feminizing puberty feminizing puberty with estrogen therapy masculinizing puberty

Partial Gonadal Dysgenesis

Partial Gonadal Dysgenesis also affects 46,XY individuals, and this condition is characterized by partial testes determination usually accompanied by ambiguous external genitalia at birth. Affected patients may have a combination of Wolffian and Mullerian duct development. The combination of both Wolffian and Mullerian duct development, along with ambiguity of the external structures, indicates that the testes produced more androgens and MIS than those of Complete Gonadal Dysgenesis patients, but not as much as would be seen in normal male development. The chart on the following page illustrates the steps of sex differentiation associated with Partial Gonadal Dysgenesis compared to those of unaffected males and females.

Normal Female Development Partial Gonadal Dysgenesis Normal Male Development
XX XY XY
ovaries develop partial testes determination testes develop
no androgen produced variable amount of androgen produced androgen produced
Wolffian Ducts regress some Wolffian Duct development Wolffian Ducts develop
no MIS production variable amount of MIS production MIS production
Mullerian Ducts develop some Mullerian Duct development Mullerian Ducts do not develop
external genitalia are female ambiguous external genitalia external genitalia are male
feminizing puberty feminizing puberty with estrogen therapy
OR
masculinizing puberty with testosterone therapy
masculinizing puberty



3. 5alpha-Reductase Deficiency

During fetal development, the genital tubercle, genital swellings, and genital folds masculinize when exposed to androgens. Androgens, or male hormones, are a general term for two specific hormones Ñ testosterone and dihydrotestosterone (DHT). DHT is a stronger androgen than testosterone, and DHT is formed when the enzyme 5alpha-Reductase converts testosterone to DHT.

5-alpha reductase enzyme

Testosterone -----------a Dihydrotestosterone

5alpha-Reductase deficiency affects 46,XY individuals. During fetal development, the gonads differentiate into nomal testes, secrete appropriate amounts of testosterone, and patients are able to respond to this testosterone. However, affected individuals are unable to convert testosterone to DHT , and DHT is necessary for the external genitalia to masculinize normally. The result is a newborn baby with functioning testes, normally developed Wolffian ducts, no Mullerian ducts, a penis resembling a clitoris, and a
scrotum resembling labia majora.

At puberty, testosterone (not DHT), is the essential androgen for masculinization of the external genitalia. Therefore, stereotypical signs of masculine pubertal development will be observed in patients. These signs include an increase in muscle mass, lowering of the voice, growth of the penis (although it is unlikely that it will reach a normal male length), and sperm production if the testes remain intact. These patients have a fair amount of pubic or axillary hair growth, but they have little or no facial hair. They do not experience female breast development. The following chart illustrates the steps of sex differentiation associated with 5alpha-Reductase Deficiency compared to those of unaffected males and females.

Normal Female Development 5alpha-Reductase Deficiency Normal Male Development
XX XY XY
ovaries develop testes develop testes develop
no androgen produced testosterone but no DHT produced androgen produced
Wolffian Ducts regress Wolffian Ducts develop Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts regress Mullerian Ducts regress
external genitalia are female ambiguous external genitalia external genitalia are male
feminizing puberty testes left intact, partial masculinizing puberty
OR
feminizing puberty with removal of testes and estrogen therapy
masculinizing puberty



4. Testosterone Biosynthetic Defects

Testosterone is produced from cholesterol through a number of biochemical conversions. In some individuals, one of the enzymes needed for these conversions is deficient. In such cases, patients are unable to make normal amounts of testosterone despite the presence of testes. Testosterone Biosynthetic Defects affect 46,XY individuals and can be complete or partial, which leads to newborns who appear either completely female or ambiguous, respectively. Four Testosterone Biosynthetic Defects are
listed below:

  1. Cytochrome P450,CYP11A Deficiency
  2. 3B-Hydroxysteroid Dehydrogenase Deficiency
  3. Cytochrome P450,CYP17 Deficiency
  4. 17-Ketosteroid Reductase Deficiency

The first three enzyme deficiencies listed above result in Congenital Adrenal Hyperplasia (CAH) (described later) as well as decreased testosterone production by the testes. The fourth enzyme, 17-Ketosteroid Reductase Deficiency, is not associated with CAH. The following chart illustrates the steps of sex differentiation associated with Testosterone Biosynthetic Defects compared to those of unaffected males and females.

Complete Biosynthetic Defect

Normal Female Development Complete Testosterone Biosynthetic Defect Normal Male Development
XX XY XY
ovaries develop testes develop testes develop
no androgen produced no androgens due to enzyme deficiency androgen produced
Wolffian Ducts regress Wolffian Ducts regress Wolffian Ducts develop
no MIS is produced MIS is produced MIS is produced
Mullerian Ducts develop Mullerian Ducts regress Mullerian Ducts regress
external genitalia are female external genitalia are female external genitalia are male
feminizing puberty feminizing puberty if given estrogen therapy masculinizing puberty

Partial Biosynthetic Defect

Normal Female Development Partial Testosterone Biosynthetic Defect Normal Male Development
XX XY XY
ovaries develop testes develop testes develop
no androgen produced partial production of androgens androgen produced
Wolffian Ducts regress some Wolffian Duct development Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts regress Mullerian Ducts regress
external genitalia are female ambiguous external genitalia external genitalia are male
feminizing puberty partial masculinizing puberty with testosterone therapy
OR
feminizing puberty with estrogen therapy
masculinizing puberty



5. Micropenis

Androgens are necessary at two different points in fetal development for a normal penis to form: (1) early in fetal life to masculinize the genital tubercle, genital swellings, and genital folds into a penis and scrotum, and (2) later in fetal life to enlarge the penis. Individuals with a micropenis possess a normally developed penis, except that the penis is extremely small. The condition of micropenis is thought to occur in 46,XY individuals if androgen production is insufficient for penile growth after the first part of masculinization of the external genitalia has already occurred. The chart on the following page illustrates the steps of sex differentiation associated with micropenis compared to those of unaffected males and females.

Normal Female Development Micropenis Normal Male Development
XX XY  
ovaries develop testes develop testes develop
no androgen produced androgens early in fetal life, deficient later in fetal life androgen produced
Wolffian Ducts regress Wolffian Ducts develop Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts regress Mullerian Ducts regress
external genitalia are female micropenis external genitalia are male
feminizing puberty partially masculinizing puberty if exposed to testosterone
OR
feminizing puberty if given estrogen therapy
masculinizing puberty

6. Timing Defect

The many steps of sex differentiation are further complicated by the fact that proper timing of these steps is necessary for normal development. If all of the steps required for male sex differentiation are working, yet these steps are delayed by even a few weeks, the result can be ambiguous differentiation of the external genitalia in a 46,XY individual. The following chart illustrates the steps of sex differentiation associated with a Timing Defect compared to those of normal males

Normal Female Development Timing Defect Normal Male Development
XX XY XY
ovaries develop testes develop testes develop
no androgen produced androgen produced at incorrect time androgen produced
Wolffian Ducts regress Wolffian Ducts develop Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts regress Mullerian Ducts regress
external genitalia are female external genitalia range from female to ambiguous external genitalia are male
feminizing puberty partially masculinizing puberty with testosterone therapy
OR
feminizing puberty with estrogen therapy
masculinizing puberty

 




7. Congenital Adrenal Hyperplasia (CAH) in 46,XX Individals

In CAH excess adrenal androgens are produced as an indirect result of a cortisol biosynthetic defect (by far the most frequent defect is a cytochrome P450,CYP21 deficiency). In 46,XX individuals, excess adrenal androgens can lead to ambiguous development of the external genitalia, so that these babies have an enlarged clitoris and a fused labia which resembles a scrotum. The chart on the following page illustrates the steps of sexual differentiation associated with 46,XX CAH (21-hydroxylase deficiency) individuals compared to those of unaffected males and females.

Normal Female Development 46,XX CAH Normal Male Development
XX XX XY
ovaries develop ovaries develop testes develop
no androgen produced no testicular androgens but excessive adrenal androgens produced androgen produced
Wolffian Ducts regress Wolffian Ducts regress Wolffian Ducts develop
no MIS produced no MIS produced  
Mullerian Ducts develop Mullerian Ducts develop Mullerian Ducts regress
external genitalia are female ambiguous external genitalia external genitalia are male
feminizing puberty feminizing puberty if treated with cortisol masculinizing puberty

 

8. Klinefelter Syndrome

Klinefelter Syndrome is the term given to individuals with a 47,XXY karyotype. At puberty Klinefelter men can experience female breast growth, low androgen production, small testes, and decreased sperm production. Additionally, although Klinefelter men undergo normal male differentiation of the external genitalia, they often possess a penis that is smaller than that of normal men. The following chart illustrates the steps of sexual differentiation associated with individuals who have Klinefelter Syndrome, compared to those of unaffected males and females.

Normal Female Development Klinefelter Syndrome Normal Male Development
XX XXY XY
ovaries develop small testes at puberty testes develop
no androgen produced often decreased androgen production androgen produced
Wolffian Ducts regress Wolffian Ducts develop Wolffian Ducts develop
no MIS produced MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts regress Mullerian Ducts regress
external genitalia are female male external genitalia with small penis external genitalia are male
feminizing puberty masculinizing puberty with possible decreased androgen production masculinizing puberty



9. Turner Syndrome

Turner Syndrome is the term given to individuals with a 45,XO karyotype. Turner patients can exhibit webbing of the neck, a broad chest, horseshoe kidneys, cardiovascular abnormalities, and short stature. Turner patients do not possess ovaries, but instead possess gonadal streaks. Turner patients have normal female external genitalia, but because they lack functioning ovaries (and thus the estrogens produced by ovaries) neither breast development, nor menstruation occurs spontaneously at puberty. The following chart illustrates the steps of sexual differentiation associated with Turner Syndrome compared to those of unaffected males and females.

Normal Female Development Turner Syndrome Normal Male Development
XX XO XY
ovaries develop gonadal streaks develop testes develop
no androgen produced no androgen produced androgen produced
Wolffian Ducts regress Wolffian Ducts regress Wolffian Ducts develop
no MIS produced no MIS produced MIS produced
Mullerian Ducts develop Mullerian Ducts develop Mullerian Ducts regress
external genitalia are female external genitalia are female external genitalia are male
feminizing puberty feminizing puberty with estrogen therapy masculinizing puberty

10. 45,XO/46,XY Mosaicism

Individuals born with 45,XO/46,XY Mosaicism can appear male, female, or ambiguous at birth. Males experience normal male sex differentiation and females are essentially identical to girls born with Turner Syndrome. For the purpose of this booklet, only patients with 45,XO/46,XY Mosaicism, who experience ambiguous sex differentiation, will be described on the following chart.

Mosaicism means that two or more sets of chromosomes influence the development of an individual. 45,XO/46,XY Mosaicism represents the most common mosaic condition involving the Y chromosome. Because the Y chromosome is affected, abnormal sex differentiation can result from this condition. The following chart illustrates the steps of sex differentiation associated with 45,XO/46,XY Mosaicism compared to those of unaffected males and females.

Normal Female Development 45,XO/46,XY Mosaicism Normal Male Development
XX XY XY
ovaries develop partial testes determination testes develop
no androgen produced variable amount of androgen produced androgen produced
Wolffian Ducts regress some Wolffian Duct development Wolffian Ducts develop
no MIS production   MIS production
Mullerian Ducts develop some Mullerian Duct development Mullerian Ducts do not develop
external genitalia are female ambiguous external genitalia external genitalia are male
feminizing puberty feminizing puberty with estrogen therapy
OR
masculinizing puberty with testosterone therapy
masculinizing puberty



V. Summary

Sexual differentiation refers to the physiological development of a fetus along male or female lines. Disorders of sexual differentiation, or syndromes of intersexuality, result when errors occur at any of these steps. This booklet is organized to serve as a basic explanation of the process of normal sexual differentiation, and it is also meant to explain the deviations from normal development underlying several syndromes of sexual differentiation.

Endocrine Treatment

1. What is the procedure for identifying and treating intersex syndromes in newborns?

When a child with an intersex syndrome also has ambiguous (undifferentiated) external genitalia, the syndrome is usually identified at birth. We recommend that a team consisting of a pediatric endocrinologist, gynecologist, urologist, geneticist and psychologist experienced in dealing with intersex conditions work together to treat these children.

Although difficult for parents, it is important not to assign a sex to an affected newborn until after proper diagnosis is agreed upon by the parents and team of doctors. We think this because it is more difficult for families to reassign a baby's sex than to postpone an initial assignment until after a diagnosis is agreed upon.

The examinations and laboratory tests necessary for attempting to establish a diagnosis may take several days. During that time, we advise parents to report to well-wishers that the infant was born with incompletely developed genitalia, and that it may take several days before the sex of the baby can be determined.

Until a diagnosis is made, it is important to use neutral terms such as baby, gonad and phallus instead of sex-specific terms like boy or girl, testes or ovaries and penis or clitoris. By using neutral terms, it is easier for families to adopt the appropriate sex of assignment for the child after a diagnosis has been made.

The following chart shows the recommended schedule for diagnostic tests and examinations for establishing a diagnosis as quickly and as accurately as possible.

Each day, weigh infant and check levels of serum electrolytes and blood glucose

  • Day 1: karyotype
  • Day 2: plasma testosterone, dihydrotestosterone,androstenedione
  • Day 3: plasma 17-hydroxyprogesterone, 17-hydroxypregnenolone, androstenedione
  • Day 4: sonogram for gonads and uterus, genitogram with or without IVP
  • Day 5:repeat plasma 17-hydroxyprogesterone, 17 hydroxypregnenolone, androstenedione

The karyotype determines if a child is 46,XX, 46,XY, or a variant of the two. Androgens should be measured on Day 2 because the concentrations of these hormones decrease after that time. 17-hydroxyprogesterone, progesterone and androstenedione may be elevated after birth, but by Day 3 it is possible to detect abnormal concentrations of these hormones. Both a sonogram and genitogram allow physicians to determine which parts of the Mullerian and Wolffian duct system are present, and where they are located. In some cases, a stimulation test with human chorionic gonadotropin (HCG) is used to determine the nature of steroid secretion of the gonads, particularly if the exam is after 3 months of age. Studies on Day 5 will confirm values obtained on previous days. Finally, it is extremely important to closely monitor weight, serum electrolytes and blood glucose levels to assure that the newborn will not experience an adrenal crisis, a common occurrence in some syndromes of sex differentiation.

2. What is the procedure for identifying and treating intersex syndromes in older children?

While we recommend that sex assignment be postponed until after a diagnosis is made for a newborn with an intersex syndrome, older infants or children will have already lived as either a boy or girl regardless of diagnosis. In such instances, it is usually best to continue with the original sex assignment because such a change is often unsuccessful if it occurs after the first 18 months of life. We feel that sex reassignment within the first month of life is most likely to be successful if such a change is determined necessary by parents and doctors. For most older children, a reassignment should only be considered if desired by the child.

After 3 months of age and before puberty, one often uses an HCG test to determine if the gonad can secrete androgens. This is accomplished by administering a series of injections of human chorionic gonadotropin (HCG).




3. What are the goals of endocrine treatment for intersex patients ?

For patients raised as males, the goals of endocrine treatment are to encourage masculine development, and correspondingly suppress feminine development, of sexual characteristics. For example, increased penile size, hair distribution and body mass can be accomplished for some individuals through the use of testosterone treatment.

For patients raised as females, the goals of treatment are to simultaneously encourage feminine development and discourage masculine development of sexual characteristics. For example, breast development and menstruation can occur for some individuals following estrogen treatment.

In addition to sex hormones, patients with Congenital Adrenal Hyperplasia may also take glucocorticoids and salt-retaining hormones. Glucocorticoids can help these patients maintain appropriate reactions to physical stress as well as suppress unwanted masculine sexual development in female patients.

4. How long do patients need to take their hormone treatments?

Sex hormone therapy is usually initiated at puberty and glucocorticoids are administered when appropriate much earlier, usually at the time of diagnosis. Whether patients take male hormones, female hormones or glucocorticoids, it is important to continue with these medications throughout life. For example, male hormones are needed in adulthood to maintain masculine sexual characteristics, female hormones to protect against osteoporosis and cardiovascular disease, and glucocorticoids to protect against hypoglycemia and stress-related illnesses.

Surgical Treatment

1. What is the goal of reconstructive female genital surgery?

The goal of reconstructive female genital surgery is to have external feminine genitalia which look as normal as possible and will be correct for sexual function. The first step is to reduce the size of the markedly enlarged clitoris while preserving the nerve supply to the clitoris, and to place it in the normal female hidden position. The second step is to exteriorize the vagina so that it comes to the outside of the body in the area just below the clitoris.

The first step is usually more appropriate early in life. The second step is probably more successful when the patient is ready to start her sex life.

2. What are the goals of reconstructive male genital surgery?

The major goals are to straighten the penis, and to move the urethra from wherever it lies to the tip of the penis. This can be done in one step. However, in many cases, it will take more than one step particularly if the amount of available skin is limited, the curvature of the penis is marked, and the overall condition is severe.

3. What are the pros and cons of early surgery vs. late surgery in the male sex of rearing?

As far as male sex of rearing is concerned, early surgery can be performed easily between the ages of 6 months and 11/2 years. Generally speaking, it is better to try to obtain full correction of the genitalia before the child is two years of age, when he will be less aware of the problems related to surgery.

Late surgery in males would be defined after two years of age. Most male surgery should be performed early in life and should not be postponed until adolescence.

4. What are the pros and cons of early surgery vs. late surgery in the female sex of rearing?

As far as female sex of rearing is concerned, when the vaginal opening is easily reached and the clitoris is not markedly enlarged, exteriorization of the vagina without clitoral correction can be done early in life. If there is a great deal of masculinization with a markedly enlarged clitoris and an almost closed vagina (or a vagina located high and very posterior), then it is often advised to postpone exteriorization of the vagina until adolescence.

There are two distinct schools of thought in reconstructive surgery today concerning bringing the vagina down to the normal female position. Some people recommend that this all be done in infancy so that the entire reconstruction is complete by two years of age, accepting that mild complications may occur later in life. Others think that the surgery should be postponed until puberty, until the girl is under the influence of estrogen and the vagina can be brought down more easily when the young woman is ready to begin her sex life.

5. What are the complications associated with each type of procedure?

In male reconstructive surgery the complications include failure to get the penis straight, resulting in continued bending of the penis. Another complication would be a fistula or leak in the reconstructed male urethra. Neither one of these are severe complications presently and can be repaired without a great deal of difficulty. However, successful reconstruction does not result in a fully normal penis, as a rebuilt urethra is not surrounded by normal spongious tissue (corpus), nor does surgery correct the size of the penis.

In female reconstructive surgery, complications depend on the location of the vagina. One complication that can occur is that scar tissue forms where the vagina exits the inside of the body and causes stenosis or narrowing of the entrance to the vagina. With a high vagina, which is up near the bladder neck in the urinary control area (sphincter), the urinary control mechanism could be damaged and the child could become incontinent of urine as a result. This is why surgery should be performed by a surgeon who is experienced in dealing with birth defects of this magnitude. On occasion, it is necessary to reconstruct a neo-vagina. In such cases, the neo-vagina is normally functional but it may not look like normal female genitalia.




6. On average, how many surgeries are needed to obtain a desirable cosmetic and functional result?

In males, this depends on the location of the urethra, the amount of available skin and the degree of bending of the penis. In favorable cases, the maximum number of operations can be two or three.

In females with a low vagina and a slightly enlarged clitoris, usually one operation is performed in infancy, followed often by a "touch up" operation in adolescence. In females with a high vagina, surgery in infancy feminizes the external genitalia, with subsequent surgery to bring down the vagina in late childhood or
early adolescence, depending on the preference of the patient.

7. What is required for post-surgical maintenance in females?

We usually do not advise vaginal dilation in our young patients because we think this is stressful, both on parents and children. However, dilation may be needed in post-pubertal women. We do accept the fact that some patients may need touch up surgery when they are older.

Psychological Treatment For Intersex Patients

1. Who should receive counseling?

In our opinion, all intersex patients and family members should seriously consider counseling. Counseling can be provided by a pediatric endocrinologist, psychologist, psychiatrist, clergyman, genetic counselor or other individual the family is comfortable talking with. It is important, however, that the individual offering counseling services be very familiar with diagnostic and treatment issues related to intersex conditions. Additionally, it is helpful if the counselor has a background in sex therapy or sex counseling.

The following topics are often addressed during counseling sessions: knowledge about condition and treatment, infertility, sexual orientation, sexual function and genetic counseling. At different times throughout their lives, we think that all patients and parents are troubled by a number of these topics and could therefore benefit from counseling.

2. How long do patients and family members need to see a counselor?

Each person is different in their need for counseling. We believe individuals benefit from talking to a counselor throughout life, but that the need to do so may increase or decrease at different points in development. For example, parents may seek the service of a counselor more frequently as their child ages and subsequently asks more questions about their condition. Additionally, patients may find it particularly helpful to seek the services of a counselor once they have decided to become sexually active.

Glossary of Terms

Adrenal glands:
a pair of glands in males and females, located above the kidneys, which produce a number of hormones, including androgens
Androgens:
the main hormones testosterone and dihydrotestosterone secreted from the testes
Estrogen:
the primary hormones produced by the ovaries
Genital folds:
common to both males and females early in development. In males the genital folds develop into the scrotum and in females develop into the labia majora
Genital ridges:
fetal tissue that can develop into either an ovary or a testis
Genital tubercle:
common to both males and females early in development. In males the genital tubercle develops into a penis and in females develops into the clitoris.
Intersexuality:
An alternative term for hermaphroditism
Karyotype:
A photograph of a person's chromosomes, arranged according to size
Mullerian ducts:
A system present in both sexes early in fetal development. Upon development this system differentiates into a uterus, fallopian tubes and posterior portion of the vagina.
Mullerian Inhibiting Substance (MIS):
Produced by the Sertoli cells, and inhibits Mullerian duct formation
Ovary:
female gonad which manufactures estrogens and eggs
SRY:
a gene on the Y chromosome whose product instructs the fetal germinal ridge to develop into a testis
Testes:
male gonad which manufactures testosterone and sperm
Urethral folds:
common to both males and females early in development, in males the urethral folds develop into the urethra and corpora and in females into the labia minora.
Wolffian ducts:
a system present in both sexes early in fetal development; upon development, this system differentiates into the epididymis, vas deferens, and seminal vesicles

Intersex Support Group Contact Information

Some of the Available Support Groups for Individuals Affected by Syndromes of Abnormal Sexual Differentiation



next: A Single Word: Stop!
~ all inside intersexuality articles
~ all articles on gender

APA Reference
Staff, H. (2007, August 9). Syndromes of Abnormal Sex Differentiation, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/syndromes-of-abnormal-sex-differentiation

Last Updated: March 15, 2016

A Single Word: Stop!

If there were only one word I could give to parents of a child born with ambiguous genitalia (intersexual, hermaphrodite, Androgen Insensitivity Syndrome, et al), that word would be: Stop! Repeat after me, ''STOP!''

When the doctors say, "Your child could never function as a male/female, so we suggest that we cut..." Say ''STOP!''

When an expert surgeon is brought in to tell you that your child has malformed genitals, that she/he needs adjustment, and now is the best time to do it. Say ''STOP!''

You have allowed genetic tests, which have come in with extra chromosomes or with a pattern that does not concur with external appearances. Say ''STOP!''

As much as every parent wants to bring home a girl, or a boy, your child may not be distinctly either. Often immediate corrective procedures are necessary for the life of the child. When well meaning clinicians attempt to make a package deal, say ''STOP!''

Early invasive treatments literally change the destiny, the identity, the 'me' of your child. It is OK to wait. Gender assignment (mutilation) is NOT COSMETIC SURGERY! It is OK to wait and see who the 'me' your child turns out to be, and then bring your child into the decision loop.

If your child is born absolutely mud-ugly, you would love your child, and you would see the wondrous beauty God has created in them. Even more important, you don't have to call your baby 'son' or 'girl'. Love your 'dear one'. Hug your 'precious child'. Enjoy their unique life. As their 'me' begins to be revealed, that is the starting point for mainstreaming toward a gender identity, if any. Depending on the diagnosis, that procedure could begin under 4 years old, or later.

It is our belief at I.S.G.I. that a certain degree of social conformity will eventually need to occur. We believe that God's creation of sexuality is a special gift, and the distinctive of 'male' and 'female' are part of His design. Those of us, your children, who don't exactly fit the mold will be held accountable before God for our lives. Do not rush to decisions for which your child will forever bear the consequences.

You may ask, "What if we make a mistake? What if we have chosen wrongly?" I was raised as a male, though my 'me' was female. The decision to 'adjust' my gender was not made well. My parents labeled my 'me' as odd, perverse, and as sin. They have missed out on a wonderful woman, Who's Who of American Women, a mom to three quality kids. Because they 'sat their minds in concrete' they refused to believe such a person as I could exist.

If you made early decisions to treat/not-treat which your child has voiced as wrong, don't let your pride add to the wall of mistakes. Accept your child, love your child, try to identify how your child is expressing their 'me'. Enjoy the marvelous gift of your 'unique' child, a child designed and built by God.

by Deborah E. Brown, Director Intersex Support Group International



next: Report on Treatment of Newborns With Genital Abnormalities
~ all inside intersexuality articles
~ all articles on gender

APA Reference
Staff, H. (2007, August 9). A Single Word: Stop!, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/ambiguous-genitalia-stop-gender-assignment

Last Updated: March 15, 2016

Multi-Dimensionality of Gender

When we speak of gender, in a context other than language, it is a recent concept in our culture, both lay and professional. In 1955, John Money, Ph.D. first used the term "gender" to discuss sexual roles, adding in 1966 the term "gender identity" while conducting his gender research at Johns Hopkins. In 1974, Dr. N.W. Fisk provided our now familiar diagnosis of Gender Dysphoria. Previously, one's sexual role was considered one of two discrete, non-overlapping congenital attributes--male or female. These two mutually exclusive categories allowed for no variation. Of course, we acknowledged the cultural differences in sexual roles, but there still could be only two modes of expression.

Now we know that one's gender is on a continuum, a blending, analogous to a "gray scale." But, our distribution of gender is bimodal, that is, most people are lumped at the two ends (see graphic) with only a minority in the middle. The great majority will see themselves as either male or female with all that implies.

Probably more upsetting to our conventional view of gender than this fuzziness of gender roles is that we can be a MIX of male and female identities within the same individual. Several researchers have developed theories of how the brain develops prenatally along sexual lines arising from androgen mediation. Dr. Milton Diamond concludes from his research that the brain has four stages of gender imprinting. The first is Basic Sexual Patterning such as aggressiveness vs. passivity. Second comes Sexual Identity (gender identity), third, the Mating Centers develop (sexual orientation), and fourth, the Control Centers for sexual equipment such as orgasm.

Gunter Dörner in Germany, using his research with rats, sees only three stages. He believes that first the Sex Centers develop giving typical male and female physical characteristics, then the Mating Centers (sexual orientation) and then the Gender Role Centers which are similar to Diamond's "Basic Sexual Patterning."

As a psychotherapist, I don't presume to enter into the discussion of what develops in what order and how. I take a more pragmatic stance and seek to observe what behaviors are linked, or independent from one another. From this research and observation, I have developed the list of five semi-independent attributes of gender. Not as a fixed dogma, but as a working theory, a map if you will, to help us understand this complex often hotly emotional issue of gender. Consider sexual identity / behavior springing from five semi-independent attributes. These five attributes are:

Genetic Our chromosomal inheritance.
Physical Appearance Our primary and secondary sexual characteristics.
"Brain Sex" Functional structure of the brain, along gender lines.
Sexual Orientation Love/sex object, "Love Maps."
Gender Identity How we see ourselves: As male, female or a combination.

It is my contention that it is possible for an individual to view oneself and function as male or female to varying degrees in each of the five sub-categories independent of the others. For example, an individual may be XX female (chromosomal female), physically female, have a "female brain," be heterosexual but see her(him)self as male--or any other combination. One can be either male or female in each of the five sub-categories independent of each other. If we use "F" for female identity/function, and "M" for male identity/function and one through five for the semi-independent attributes listed above we could describe each individual according to their particular breakdown:

1M ----- 2M ----- 3M ----- 4M ----- 5F
A Gender Dysphoric, Morphological Male

1M ----- 2M ----- 3M ----- 4F ----- 5M
A Homosexual Male

1F ----- 2F ----- 3M ----- 4F ----- 5F
A Dominant, But Heterosexual, Even Feminine, Female

Since each of these independent attributes are graded, it is easy to see the possible combinations and degrees number in the thousands. With regard to gender, we each can be in a category of one--ourselves.

Whether it's gender identity, sexual orientation, or brain sex, the expression usually remains constant from childhood throughout one's life.

Now, for a more detailed description and illustration of the five sub-categories of gender:

The first sub-category, Genetics, is only beginning to be understood. How and how much do genetic influences effect one's expression of gender? We do know that besides the traditional XX chromosome of a typical female and the XY of a typical male, that there are other combinations such as XXY, XYY, and XO.

A XXY combination results in 47 rather the 46 chromosomes. This condition is called Klinefelder's syndrome and occurs in one in every 500 births. Individuals with Klinefelder's are sterile, have enlarged breasts, small testicles and penis, and a eunuch body shape much like the "Pat" character on "Saturday Night Live." They show little interest in sex.

Another 47 chromosome occurrence is XYY Syndrome. In this syndrome, the hormonal and physical appearance of the individual are evidenced asDistribution of Gender a normal male, but behavior is effected. Typically, XYY Syndrome people are bisexual or paraphilic (pedophillia, exhibitionism, voyeurism, etc.), and show very poor impulse control.

Where Klinefelder's and XYY Syndrome are examples of an extra chromosome, Turner's syndrome is a case of a missing sex chromosome. These individuals possess 45 chromosomes (written as XO), are unable to develop gonads, and are free of all sexual hormones, except those crossing over from the mother during fetal life.




Turner's Syndrome people have external sex organs approximating a female, and their behavior is characterized as hyper-feminine, baby care oriented, and showing very poor spatial and math skills. The Turner's personality, free of all influence from testosterone, tends to be in direct opposition to the typical set of "Tom Boy" traits.

Turner's Syndrome relates well to our second category of Physical Gender--that being our primary and secondary sexual characteristics. To discuss this aspect of gender we need to examine hormonal involvement, in particular testosterone. All sexual differentiation, physical, mental, and emotional are produced by hormones which may be amplified and/or specified by one's social environment. During fetal life, the amount present, or the absence of testosterone determines our sexuality -- physically, mentally and emotionally. There are key times or periods during development when the fetus will go towards the male or the female depending on the level of testosterone. These windows of opportunity may be only open for a few days and if the needed level of testosterone is not present, a basic female orientation develops regardless of the testosterone levels before or after this critical period, and the resulting sexual imprint.

The first critical period is at conception when the presence of the SRY gene (Sex-Determining Region of the Y chromosome) will determine our physical gender. The SRY gene is normally found on the short arm of the Y chromosome, but can detach making for a XY female (the Y missing its SRY gene) or a XX male (the SRY attaching to the X).

The SRY gene causes the fetus to release TDF (Testes Determining Factor) which turns the undifferentiated gonad into testes. Once testes have formed, they release androgens such as testosterone, dihydrotestosterone, and anti-mullerian hormone.

Before the release of TDF, the developing fetus has two tiny structures, the mullerian and wolffian ducts, and two small undifferentiated gonads, neither testes nor ovaries. Without the influence of TDF and testosterone, the gonads form into ovaries and the mullerian duct forms into the female internal sex organs, the wolffian duct disappears and the external sexual tissue becomes the labia major, clitoris, labia minor and clitoral hood. With the influence of TDF, the gonads become testicles and the wolffian duct forms the male internal sex organs, the mullerian ducts dissolve and the external tissue develop into the penis, scrotum, penile sheaths and foreskin. In other words, without testosterone all fetuses develop into females. Adam springs from Eve, not Eve from Adam.

As the primary sexual differentiation proceeds towards our physical gender, sometimes deviations occur. These anomalies are sometimes called "experiments of nature." One such "experiment" is a condition termed congenital adrenal hyperplasia (CAH) when the female fetus releases a steroid hormone form her adrenal glands which resembles testosterone. The resulting child often has confusing genitals ranging from deformed female genitals to an appearance of male genitals. If the child is raised as male, following any "adjusting" surgery and given male hormones at puberty, the individual develops as a "normal" but sterile male with XX chromosomes. On the other hand, if the infant is surgically corrected to female and given female hormones, there is a 50/50 chance of lesbian expression.

Another revealing "experiment of nature" is Androgen Insensitivity Syndrome. In this case, there is normal amounts of testosterone circulating in a XY chromosome fetus, but each cell of its body is unable to react to it. This is similar to Turner's Syndrome in that neither the mullerian or wolffian ducts mature and the external genitalia develops into an approximation of normal female genitals, but differs in that TDF stimulates the gonads into becoming functioning testicles in a XY chromosome body. The child is raised as a girl and is seen as a normal female until she fails to menstruate because she has no uterus. If enough estrogen is produced by her testes, she develops into a completely normal appearing, sterile female with XY chromosomes and internal testicles.

Now we must leave the comfortable arena of biology and development and enter the more rocky, emotional and even political arena of psychology, anthropology, and sociology. An arena where deduction, speculation and circumstantial evidence is more evident than "hard fact."

The third, forth and fifth attributes all reside in the brain and there is controversy on both a congenital vs. environmental level and on a developmental one. It is still argued by some that sexual orientation is a choice and there is no difference in the mental abilities of men and women. Others argue that the evidence, both direct and circumstantial, is becoming overwhelming that these stands are incorrect.

Because of the controversy over whether significant differences in brain structure do exist between the genders, I will confine my discussion of the "Brain Sex" attribute to some behavioral differences that have been noted between morphological male and female infants and children. At all times keep in mind that Physical Gender does NOT always indicate "Brain Sex" Gender. And, while these differences are the norm, they are not absolute. Individual children may differ.

Even a few hours after birth, significant behavioral differences are noted between morphologically normal boys and girls. Newborn girls are much more sensitive to touch and sound than their male counterparts. Several day old girls spend about twice as long looking back at an adult face than boys, and even longer if the adult is speaking. A girl can distinguish between the cries of another infant from other extraneous noises long before a boy. Even before they can understand language, girls do better at identifying the emotional context of speech.

Conversely, during the first few weeks of infant life, boys are inattentive to the presence of an adult, whether speaking to the infant or not. However, baby boys tend to show more activity and wakefulness. At the age of several months, girls can usually distinguish between the faces of strangers and people they know--boys usually do not demonstrate this ability.

As infants grow into children, the differences seem to intensify and polarize. Girls learn to speak earlier than boys and do a better job of it. Boys want to explore areas, spaces and things, girls like to talk and listen. Boys like vigorous play in a large space where girls like more sedentary games in smaller spaces. Boys like to build, take things apart, explore mechanical aspects of things and are interested in other children only for their "use" (playmates, teammates, allies, etc.). Girls see others more as individuals--and will likely exclude a person because their "not nice," and will more readily include younger children and remember each other's names. Girls play games involving home, friendship, and emotions. Boys like rough, competitive games full of "'zap, pow' and villainy." Boys will measure success by active interference with other players, preferring games where winning and losing is clearly defined. In contrast, girl play involves taking turns, cooperation and indirect competition. Tag is a typical boy's game, hopscotch is a girl's game.




If "Brain Sex" is controversial, the fourth attribute of Sexual Orientation is ever more so. Although there is public and political controversy, the overwhelming majority of medical and psychological practitioners agree that sexual orientation may prove to be mainly congenital, or at least firmly established in early childhood. The term "Sexual Orientation" is a bit misleading. It is more an erotic or love orientation in that Sexual Orientation determines the physical gender we find attractive, with whom we fall in love, and have romantic as well as sexual fantasies.

From experiments with animals, "experiments of nature" in humans, and genetic and neurological studies come a consistent, though still circumstantial, stream of evidence that indicates one's sexual orientation is largely hormonally determined by the presence of testosterone at key periods in fetal development, and possibly even beyond. As we have seen with congenital adrenal hyperplasia (CAH), female fetuses exposed to testosterone-like agents develop a 50/50 chance of a lesbian versus heterosexual orientation if raised as girls. Studies of identical twins also indicate that when one twin shows homosexual or lesbian expression, there is a 50/50 chance of homosexual or lesbian expression in the other twin—whether raised together or apart.

The remaining 50% of determination may be continued hormonal development, environmental considerations or a combination. One interesting consideration with determination may be during our early postnatal development since the fetal stage for human babies is not completed during gestation, but continues for a year or more outside the womb. And during this critical time after birth, we have the highest level of testosterone present, excluding the onset of puberty--with many brain receptors to receive this powerful hormone. At any rate, between the ages of three and six years, one's erotic orientation is established but may not be acted upon for decades, if at all.

The last of our five attributes, Gender Identity, is the last to be identified, and the least understood and researched. When one's Gender Identity does not match their Physical Gender, the individual is termed Gender Dysphoric. Like Sexual Orientation, gender dysphoria is not pathological in itself, but a natural aberration occurring within the population. As with sexual orientation, the percentage of the population having gender dysphoria is in dispute, with estimates ranging between one in 39,000 individuals to three percent of the general population.

Although it is useful for psychotherapists and other behavioral scientists to use diagnostic nomenclature in order to describe an individual, we must remember that these categories are often fluid. An individual may see and express themselves for years as a crossdresser, then change their self-identity to a more transgendered or transsexual one. This change may be because the individual actually changes their self-view with age, or more information and experience lead to a clearer understanding of self.

Gender dysphoric individuals commonly, even frequently, have a sexual orientation markedly different from their gender identity, which suggests that the key periods of these formations occur at differing times. While gender dysphoric individuals display a wide gamut of incongruity and discomfort with their physical gender, three main groups have been delineated:

Crossdresser

Those individuals with a desire to wear the clothing of the other sex are termed crossdressers. Most crossdressers are heterosexual men--one's sexual preference has nothing to do with crossdressing. Many men like to wear women's clothing in private or in public, and may even occasionally fantasize about becoming a woman. Once referred to as a transvestite, crossdresser has become the term of choice.

Transgenderist

Transgenderists are men and women who prefer to steer away from gender role extremes and perfect an androgynous presentation of gender. They incorporate elements of both masculinity and femininity into their appearance. They may be seen by some persons as male, and by others as female. They may live part of their life as a man, and part as a woman, or they may live entirely in their new gender role but without plans for genital surgery.

Transsexual

Men and women whose gender identity more closely matches the other sex are termed transsexual. These individuals desire to rid themselves of their primary and secondary sexual characteristics and live as members of the other sex. Hormonal and surgical techniques make this possible, but it is a difficult, disruptive, and costly process, and must not be undertaken without psychological counseling, careful planning, and a realistic understanding of the likely outcome. Most transsexual people are born and first live as male.

Transsexuals are diagnostically divided into the sub-categories of Primary or Secondary. Primary transsexuals display an unrelenting and high degree of gender dysphoria, usually from an early age (four to six years of age). Secondary transsexuals usually come to a full realization of their condition in their twenties and thirties, and may not act on their feelings until they are much older. Typically, secondary transsexuals first go through phases that would be self-assessed as being a "crossdresser or transgenderist."

The outcomes of transsexuals vary greatly. There seems to be no significance in the outcome differences between primary and secondary transsexuals. Those who complete this gender reassignment process (the process of "transition") and have exercised due diligence throughout generally do very well for themselves and lead happy and fulfilling lives. Unfortunately, others who go through the process on a perfunctory basis may be unprepared to fully and comfortably assimilate into their new gender role. In conclusion, when we think of gender, we need to realize that many combinations in gender exist, and that they are all natural. Although most people are morphologically male or female, those who homogeneously fill all five gender categories as the same gender may be in the minority. The largest minority, but still a minority.




Carl W. Bushong, Ph.D., LMFT, LMHC

about the author

Carl W. Bushong holds a doctorate in clinical psychology, and has been in private practice since 1977. Dr. Bushong is the Director of the Tampa Gender Identity Program (TGIP), where a full range of transgender services are provided using his Informed Choice method--the individual uses their own decision-making capabilities after being provided with the necessary information and feedback to do so. In other words, the patient is ultimately in charge of the decision-making process--and has access to the facilities and expertise of a well-trained gender team.

Copyright 1995 Tampa Stress Center, Inc.
Source: Tampa Stress Center, Inc., PO Box 273107, Tampa, Florida 33688. Telephone (813)884-7835.

next: First-Person Stories By Intersexuals About Their Lives

References

Benjamin, H. The Transsexual Phenomenon: A Scientific Report on Transsexualism and Sex Conversion in the Human Male and Female. New York, Julian Press, 1966.

Buhrich, N., Bailey, J.M. and Martin, N.G. Sexual orientation, sexual identity, and sex-dimorphic behaviors in male twins. Behavior Genetics, 21:75-96, 1991.

Diamond, M. Human sexual development: biological foundations for social development. Human Sexuality in Four Perspectives. Beach, F.A. (ed.), Baltimore, Johns Hopkins Press, 38-61, 1977.

Dittman, , R.W., Kappes, M.E. and Kappes, M.H. Sexual behavior in adolescent and adult females with congenital adrenal hyperplasia.Psychoneuroendocrinology, 1991.

Docter, R.F. Transvestites and Transsexuals: Toward a Theory of Cross-Gender Behavior. New York, Plenum Press, 1988.

Dörner, G. Hormones and sexual differtiation of the brain. Sex, Hormones and Behaviour, CIBA Foundation Symposium 62, Amsterdam, Excerpta Medica, 1979.

Dörner, G. Sexual differentiation of the brain. Vitamins and Hormones. 38:325-73, 1980.

Dörner, G. Sex hormones and neurotransmitters as mediators for sexual differentiation of the brain. Endokrinologie, 78. 129-38, 1981.

Dörner, G. Sex-specific gonadotrophin secretion, sexual orientation and gender role behaviour. Endokrinologie, 86. 1-6, 1985.

Fisk, N.M. Gender dysphoria syndrome: (The how, what, and why of a disease). In Proceedings of the 2nd Interdisciplinary Symposium on Gender Dysphoria Syndrome. (D.R. Laub and P Gandy, eds.). Division of Reconstructive and Rehabilitation Surgery, Stanford University Medical Center, 1974.

Kaplan, A.G. Human sex hormone abnormalities viewed from an androgenous perspective: a reconsideration of the work of John Money. The Psychobiology of Sex Differences and Sex Roles. Parson, J. (ed.). Hemisphere, 81-91,1980.

Kimura, D., and Harshamn, R. Sex differences in brain organization for verbal and non-verbal functions. Progress in Brain Research. De Vreis, GJ. it al. (eds.), Amsterdam, Elsevier, 423-40, 1984.

Kimura, D. Are men's and women's brains really different? Canadian Psychol., 28(2). 133-47, 1987.

Moir, A., and Jessel, D. Brain Sex: The Real Difference Between Men and Women. New York, Dell Publishing, 1989.

Money, J. Gay Straight, and In-Between: The Sexology of Erotic Orientation. New York, Oxford University Press, 1988.

Money J., and Ehrhard, A.A. Man and Woman, Boy and Girl: The Differentiation and Dimorphism of Gender Identity from Development to Maturity. Baltimore, Johns Hopkins Press, 1972.

Money, J., Schwartz, M., and Lewis, V.G. Adult herotosexual status and fetal hormonal masculinization and demasculinization: 46,XX congenital virilizing adrenal hyperplasia and 46, XY androgen insensitivity syndrome compared. Psychoneuroendocrinology, 9:405-414, 1984.

Stein, S. Girls and Boys: The Limits of Non-Sexist Rearing. London, Chatto and Windus. 1984.



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APA Reference
Staff, H. (2007, August 9). Multi-Dimensionality of Gender, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/multi-dimensionality-of-gender

Last Updated: March 15, 2016

Intersexuality: A Plea for Honesty and Emotional Support

The physicians and nursing staff in the delivery room grew suddenly silent, almost grim. "Is there something wrong with my baby?" queried the exhausted new mother. A nurse whisked the infant off to the warming unit, while another explained that the baby needed to be checked out and would be returned as soon as possible. Meanwhile, a pediatrician, an endocrinologist, and a plastic surgeon were summoned with all possible haste to the hospital. The baby was not sick; it had been born with "ambiguous genitals." They might be described as a split scrotum, in the shape of a Parker House roll, with a tiny tiny penis peeking out from between the sections, and the urethra behind the penis, rather than at the tip. Or were they partially fused outer labia, with a clitoris enlarged to 10 times the usual size? The experts would work around the clock to make a decision as quickly as possible, and would then use surgery and hormones to make the baby look as "normal" as possible, removing "discordant" structures, including all but a tiny bit of the clitoris.

"It seems that your parents weren't sure for a time whether you were a girl or a boy," the gynecologist explained, as she handed over three fuzzy photocopied pages. The young woman had asked the doctor's help to obtain records of a mysterious hospitalization which occurred while she was yet a tiny infant, too young to recollect. She was desperate to obtain the complete records, to determine who had surgically removed her clitoris, and why. "Diagnosis: true hermaphrodite. Operation: clitoridectomy."

"We advise you to fake an injury and leave quietly," Olympic officials told Spanish hurdler Maria Patino. They had just received the result of a laboratory test that indicated that her cells had only a single X chromosome. Patino was disqualified. Statistics are hard to come by, but it seems that as many as one in 500 women competitors are disqualified by the sex test. None are men masquerading as women; they are people whose chromosomes defy the notion that male and female is as simple as black and white. Patino is a woman with "male" chromosomes; the medical label for her condition is androgen insensitivity syndrome.

Each of these scenarios illustrates the traumatic repercussions when intersexuality is brought to light in a culture which insists on believing that sex anatomy is a dichotomy, with male and female conceived of as so different as to be nearly different species. However, developmental embryology, as well as the existence of intersexuals, proves this to be a cultural construction. Genitals may be intermediate in form between the male and the female pattern. Some have female genitals with internal testes, or fairly male genitals with internal ovaries and uterus. About one in 400 men have two x chromosomes. At least one in a few thousand people are born with a body which violates the dualism of "male" and "female" strongly enough to place them at serious risk of parental rejection, stigmatization, often harmful medical interventions, and the emotional pain of secrecy, shame, and isolation.

In modern Western culture, the events of an intersexual's birth are hidden in shame and half-truths. Parents most often will not reveal their ordeal to anyone, including the child as s/he comes of age. The child is left physically damaged, and in an emotional limbo without access to information about what has happened to them. The burden of pain and shame is so great that virtually all intersexuals stay deep in the closet throughout their adult lives.

Current medical thinking treats the birth of an intersexual infant as a "social emergency" that must be resolved by assigning a sex and erasing any ambiguity as soon as possible. Medical texts advise the clinician to systematically pursue even a slight doubt about the sex of a newborn, but not to reveal such doubts to the anxious parents. Intersexual children's bodies combine male and female characteristics, and the decision to register the child's birth as a girl or as a boy is made by the physician, largely on the basis of the prognosis for genital plastic surgery. One surgeon, asked why intersex children are usually assigned female, explained, "It's easier to dig a hole than to build a pole." That is, surgeons find it easier to assign the child as a girl, construct an opening, and remove enlarged clitoral tissue, than to assign the child as a boy and try to enlarge and reshape the small penis. Surgeons and endocrinologists have not considered leaving the child's body intact and providing emotional support for being different to be an option.

Although physicians understand that they will actually impose, rather than determine, a sex, they tell parents that tests will reveal the child's true sex, in a day or two at most, and assure them that surgery will let their child grow up normal, and heterosexual. They are careful to avoid words like "hermaphroditism" or "intersexuality," and speak only of "improperly formed gonads," never of ovaries or testes. When, years later, the intersexual adult tries to determine what was done to him or her, and why, s/he will encounter these tabooed words many times in medical literature and sprinkled liberally throughout their medical records.

This medical treatment amounts to a policy of denial. Secrecy and taboo disrupt emotional development and stress the whole family. Many adult intersexuals have had to discover their history and status independently without emotional support of any kind. As a result, more than a few are estranged from their families. Surgery destroys genital anatomy and many intersexual children are subjected to repeated surgeries, over a dozen in some cases. Genital surgery disrupts the infant's erotic development and interferes with adult sexual function. Surgery performed on infants precludes choice; the actual goal of early surgeries may be the parents' emotional comfort rather than the child's ultimate well-being. Even at clinics which have specialized in treating intersexual children for decades, there is generally no program of professional counseling. Some physicians privately concede that they perform any needed counseling themselves during annual evaluations. From the point of view of the intersexual adolescent such a physician may be seen as allied with the parents against any trace of sexual difference or criticism of medical treatment, rather than as a trusted counselor and advisor.

As a growing number of adult intersexuals have come forth to speak about their experiences, it is apparent that surgery has generally been more harmful than helpful. The "conspiracy of silence", the policy of pretending that intersexuality has been medically eliminated, in fact simply exacerbates the predicament of the intersexual adolescent or young adult who knows that s/he is different, whose genitals have often been mutilated by "normalizing" plastic surgery, whose sexual functioning has been severely impaired, and whose treatment history has made clear that acknowledgment or discussion of intersexuality violates a cultural and a family taboo.

A few are now beginning to organize to oppose this silence. The San Francisco-based peer support group Intersex Society of North America strongly recommends counseling for the entire family of a newborn intersexual and for the intersexual child as soon as s/he is old enough. They oppose "normalizing" cosmetic surgery performed on infants and children who cannot provide informed consent. ISNA believes that, with appropriate emotional support, intersexual infants and children would fare better without genital plastic surgery. Like ISNA, Britain's Androgen Insensitivity Support Group advocates the provision of competent psychological support for inter-sexuals and their families and decries physicians who believe that counseling can be accomplished in a few minutes of explanation by a pediatric endocrinologist or urologist. The California mother who founded the Ambiguous Genitalia Support Network, a parents' group, says she made a conscious decision to avoid euphemism in naming her group. "If parents can't deal with the words "ambiguous genitalia," how are they going to be able to accept their children?"

Children born intersexual face psychological difficulties no matter what treatment choice is made, and sexually sophisticated, ongoing counseling for both family and child must become the central component of the treatment process. Parents and medical staff must be reeducated about sexuality. Intersexual children need early access to a peer support group where they can find role models and discuss medical and lifestyle options.

Bo Laurent, a doctoral student at the Institute for Advanced Study of Human Sexuality in San Francisco, is a consultant to the Intersex Society of North America.



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APA Reference
Staff, H. (2007, August 9). Intersexuality: A Plea for Honesty and Emotional Support, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/intersexuality-a-plea-for-honesty-and-emotional-support

Last Updated: March 15, 2016

Intersex Survivors of Domestic Violence

All of these terms are more likely to be heard in a therapist's office than a shelter, but knowing them can help one understand the complexities facing those who transcend stereotypes of gender expression or physical sex: including those who are usually known as intersexual.

An intersex or intersexual person has a body with external sexual characteristics typical of both male and female bodies. Nonetheless, in our society, children who are born intersexual are nearly always assigned a male or female gender role, although because of external sexual ambiguities, that assignment may not occur at birth. Intersexual children in the United States typically have their genitals surgically altered before age three to conform to gender assignment.

Intersex Survivors

In preliminary data, the Gender, Violence, and Resource Access Survey of trans and intersex individuals found 50% of respondents had been raped or assaulted by a romantic partner, though only 62% of those raped or assaulted (31% of the total sample) identified themselves as survivors of domestic violence when explicitly asked.

Clearly, intersex survivors exist. There are many reasons why so few intersex survivors are served by the community that typically aids and advocates for survivors of domestic violence. This early punishment for simply expressing gender identity leaves many scars, but the experiences that lead intersexual domestic violence survivors to believe that it's normal for "people like me" to live with abuse only increase in magnitude as the intersex survivor matures.

Perhaps the most damaging force is the one that teaches intersexual persons that "helping" institutions are often anything but, and may actually harm them. Although these stories' power is anecdotal and not statistical, they and others like them are widely known and retold among intersex individuals. Because of the extreme cruelty and casual indifference of authorities and institutions exemplified in these common stories, an intersex survivor may fear an unknown service institution more than a familiar abuser.

A second level of fear intersex survivors face when seeking help is the possibility that their intersex status, if previously hidden, might become known and expose them to more violence, as in the Brandon Teena case. Exposure might also lead to the loss of a job, as very few jurisdictions provide employment discrimination protection to intersexed persons, and stories of job loss or workplace harassment upon exposure are legion.

Should a intersex survivor decide to brave these risks and seek help despite them, she or he faces other barriers. Some information suggests that intersex survivors have frequently been multiply abused for years or decades. Often a intersex survivor has a unique body and/or a unique vulnerability to the emotional aftermath of sexual violence; either can make difficult or impossible discussing this abuse with an unfamiliar victims' advocate.

Related to this problem is the shame and self-doubt that is endemic in this community, due to the pressures intersex persons have felt from their earliest years to deny their feelings and conform to others' expectations. Adding to this shame and self-doubt is the widespread perception that intersex individuals are mentally ill. Abusers use this shame and self-doubt against their intersex victims to undermine their victims' perceptions and to convince them that no one else will want them.



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APA Reference
Staff, H. (2007, August 9). Intersex Survivors of Domestic Violence, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/intersex-survivors-of-domestic-violence

Last Updated: March 15, 2016

About Me

A Brief Life Story

This "Other"

Berdache Jordan, Intersexual

A Thumbnail History of Myself

Brief History of a Hermaphrodite

(A Freak Of Nature, Born At A Time Prior To DNA Testing)

The only term that was known, as far as I was ever told, was that I was an Open Birth, meaning I had ambiguous genitalia, that I could not be identified by the people who examined me as either a male or a female. These people were a Catholic Sister and a Veterinarian, both in a small western town, dealing with a premature and abandoned miscarriage.

This was a condition ascribed to the youth and/or illness of my biological Mother at the time of my birth. In certain conversations discussing me, I had overheard this on various occasions. I recall that I was stripped naked many times and exhibited to other adults. As a small child, from age 2-4, I remember that I enjoyed being the center of attention and awe and speculation by adults.

The man I knew as Dad was, I believe, related to me. Whether he was, in fact, my biological parent is unknown. His sibling and a younger sister were suspected to be in an incestuous relationship. My genealogical research indicates that my genetic condition existed in the family. Two of three "brothers" immigrated from Germany and came to America. The youngest was listed as the "wife" of the older sibling on passports and ship manifests. German/Gypsy/Native American lineage, I have reason to believe, carried my rare DNA karyotype. Research is continuing at this time.

True Hermaphrodite

I am one of several types of true hermaphrodites. The XXXY (mosaic) karyotype is extremely rare. One theory of the cause of my own condition has a lot of support. It maintains that two ovum were produced simultaneously in my mother and the eggs fertilized independently as maternal twins. During gestation the eggs merge into a single fetus, one ova was destined as male the other as female.

My understanding is that sometimes both ova might well have been male XY or female XX, in which case the child would have both distinctive DNA chromosome karyotypes, a XY/XY or a XX/XX (mosaic).

These people would appear perfectly male or female unless some medical situation required a chromosome karyotyping. That is an unusual procedure. How many are like this is unknown, as few are tested. They, like me, would have two separate chromosome karyotypes, similar to Siamese Twins sharing one body to varying extents. It is also my understanding that present fertility drugs today may make this condition much more common. In my case, my karotype is XX/XY, hence I have characteristics of both male and female.

Confusion and Abuse

I was given a boy's name and a separate girl's name by my "Father" and his wife, my "Mother," with two birth certificates. Neither was recorded at the time, but awaited a future decision. Eventually, I was "recorded" later as a male, but called by an ambiguous nickname of a comic strip character, a child that no one knew what sex the child was, which was fitting (from Barney Google).

I was left "as is" awaiting further physical development. From the age of four to sixteen years old, I was sexually, physically and mentally abused by several members of my own family. Then I was able to stop the most invasive abuse by choosing to be a male, at age fifteen, accomplished by taking massive doses of testosterone to effect secondary male sexual characteristics. ( Deeper voice, body and facial hair, that allowed me to pass as a male.)

If you refer to the sexual abuse links, you may get some idea of the traumas caused by this abuse. The intersexual condition caused me to be abused by both "normal" sexes. Something seemed to drive others to experience their sexual fantasies, with me as the unwelcome recipient. Personally, all I derived was pain, frustration, and fear of failing to please those I was dependent on as a child. I suffered extreme feelings of guilt when I learned what these acts were.




Not Everything Bad

Not all of my life was bad or sad. Many parts were humorous, as I kept my own sense of humor, much of which was due to my masquerade as a macho male, as I perceived and played that role in many all-male environments, ie: military, jails, and prison. I never succumbed to a male/male relationship, due to inhibitions and prior abuse by homophobic males. Actually, the only way I could even have a homosexual relationship would be to have sex with another like myself (not likely), so homophobia is not an issue with me.

In public, I have been, in all respects, a male heterosexual. And like many real males, I've felt woefully inadequate for much of that time. I had several advantages as a partner: insight, companionship, and
communication, and my own desire for lengthy foreplay.

Although inadequate, I was married for eighteen years to two "normal" women. However, when I revealed my condition (to the extent, I was aware of it), they reacted first with disbelief for a few years, then rejection, as they had homophobic reactions, social concerns with "their being bisexual," worries about themselves being gay. Both sought "normal" affairs while married, unable to accept me as is, and mostly unable to deal or cope with their own sexuality .

A Good Parent

Having raised three children alone through pre-teen and teenage years (11 years), I can appreciate the problems of single parenting. With two girls, I discovered the bias and prejudices of a male dominated school system. With 90% of discretionary funds expended on male sports activities, I resented and fought the system on behalf of my daughters, and my son, feeling he too was being brainwashed into participating in aggressive sports.

No one that knows me could fault me on my parenting skills. The idea that a gay, lesbian or gender dysphoric person is unfit as a parent for "normal children" is ludicrous! My own children never knew my intimate identity, until a local laboratory gossip leaked my DNA tests results. The two oldest have not changed and support me, however, my youngest daughter (15) was teased at school and chose to go live in another state with my ex-wife. They are all heterosexual as far as I know, but perhaps they are more tolerant of others, having known, loved and respected me.

More Information on Hermaphrodites

For those who want to know more about the strange disassociated life I led, and my own traumas, I can recommend a book, Herculine Barbin: Memoirs of a French Hermaphrodite. Herculine was raised as a female in convent surroundings. I felt the trauma and the emotions expressed in this book as my own. It is sad that there has been so little difference in public tolerance. Much has remained unchanged since 1838.

My own book "Masquerade," is being edited as I write this. Perhaps it will make a little difference in public attitudes, and assist some lost soul who feels like a lonely outcast, as I did, to perhaps accept themselves better and find out they're not alone. Hopefully, others can find acceptance and be themselves, live a productive life, beyond a sick social preoccupation with pseudo-gender differences, associated sex and hate crimes and stop the reactionary response habits to childhood traumas.

As you look through my site, I hope you'll think about these questions:

  • Can we heal the mindless wounds we and others have inflicted on us, for the crime of being different outside than we are inside, by divisive and false social conditioning?
  • Are we not all the same ambi-gendered children of the universe, residing in some arbitrary physical form?
  • Who's agenda is furthered by dividing us?

more: The Berdache Tradition



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APA Reference
Staff, H. (2007, August 9). About Me, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/gender/inside-intersexuality/about-me

Last Updated: April 12, 2017

How to Treat Coexistent Mental Illness and Substance Abuse

ABSTRACT: Integration of mental illness and substance abuse treatments is important for patients with dual diagnosis, the coexistence of an emotional ailment and a chemical dependency. Such comorbidity is usually associated with a poor prognosis. Both disorders require appropriate therapy so that remission of psychiatric symptoms and maintenance of sobriety become reachable goals for these individuals. A joint therapeutic approach improves outcome, functional expectation, and community adjustment.

ABSTRACT: Integration of mental illness and substance abuse treatments is important for patients with ìdual diagnosis: the coexistence of an emotional ailment and a chemical dependency.COEXISTENCE of mental illness and a substance abuse disorder known as dual diagnosis vastly compromises management of both conditions. The lifetime prevalence rate for psychiatric conditions is 22.5% in the general population, and 19.6% of people have a chemical dependence; having both at the same time occurs in approximately one third of people with either of these conditions. Comorbidity results in an outcome much worse than when only one of these conditions is present.

Managing both aspects of the dual diagnosis conjointly can be advantageous. The core treatment provides an opportunity to approach both illnesses at the same time. Staff members are trained to assess and treat these problems in a unified way. The therapeutic team can integrate within the program the knowledge and skill needed to treat both disorders and lessen the patient's denial of either entity.

In addition, prognosis for improvement in each of these disorders is enhanced by treatment of the other condition. Physicians, other clinicians, and health care planners document benefit from an integrated, therapeutic method versus an isolated approach to each ailment separately. For example, a patient with severe mental illness and alcoholism could derive significant improvement in psychologic symptoms by avoiding intoxication; in the same manner, control over a component of psychiatric illness would help an emotionally compromised drug addict attain sobriety. Compliance with prescribed treatment and adherence to follow-up is greatly improved when both aspects of the problem are attended. One study assessing the 4-year outcome of patients with mental illness treated in a dual diagnosis program showed a 61% remission from alcohol abuse.

Dichotomous, suboptimal care is sometimes the result of poor coordination of therapeutic services by mental health agencies and substance abuse centers. Some individuals with either one of these ailments are barred from treatment facilities that focus on the other condition, leaving them in a gap between these two disciplines.2,4 Because they are difficult to treat, people with chemical addictions and mental illnesses are excluded from care by some clinicians. Differences in treatment philosophy or mistrust between professionals can lead to barriers between the psychiatric, general medical, and addiction treatment teams, exaggerating the already negative effects on recovery rates.3 On the other hand, a comprehensive, dual diagnosis approach to both types of illness from the beginning potentially improves the prognosis.

Careful diagnostic evaluation in dual diagnosis cases offers important prognostic and treatment implications. More severe psychiatric disorders indicate worse outcomes.5 The prognosis for patients with psychiatric disorders is typically poorer with accompanying substance abuse than without such problems. For individuals with chemical dependence, the best predictor of improvement is a lessening in severity of concomitant psychiatric symptoms.5 Improved mental status has a positive impact on the addiction remission potential.

TREATMENT METHODS

Central State Hospital in Louisville, Ky, has a dual diagnosis treatment unit. Admission criteria for this section of the hospital include patient motivation with a comorbid, major psychiatric disorder and substance abuse. Exclusions include persons who are medically unstable or require one to one personal psychiatric supervision, those who are unable to comprehend educational material or participate in group and milieu offerings, and those whose behavior is uncontrolled to the point of exhibiting a high violence potential. The dual diagnosis staff screens applicants for admission on an elective basis, with primary focus on acceptance of chemically dependent individuals with motivation, who have the desire for sobriety and need for psychiatric treatment. Admission is denied to those not highly committed to the process of recovery.

A comprehensive history and physical examination are done on the day of admission.6 Appropriate laboratory studies are done. Feedback about the patient from family, a previous physician, or through other means greatly improves the evaluation. Assessment and observation of symptoms that could arise from either or both conditions help identify problems and direct therapy in the proper direction.1,4

Dual diagnosis treatment begins with the detoxification procedure, lasting a week or more, depending on the type and quantity of substances used. The detoxification period is also an opportune time to develop doctor-patient rapport and carefully assess the source of psychiatric manifestations, determining whether they are primary or induced by drug abuses.1 In this population, establishing a productive therapeutic alliance is critical to gaining trust and keeps the patient in the program. Psychiatric management follows a thorough evaluation of all signs, symptoms, and history. Routine psychiatric pharmacotherapies and/or electroconvulsive therapy are used as warranted by clinical indications. Psychotherapy, individual counseling, and group therapy are provided, as are educational and recreational activities.

Attendance at Alcoholics Anonymous (AA) meetings is expected. With its strong peer group involvement, AA is a potent factor in confronting denial. Patients are given the opportunity to initiate support from the community by choosing AA sponsors from people in recovery from chemical dependencies.7 Such contacts should be maintained through discharge from the inpatient program. These sponsors are an essential part of recovery, facilitating the individual's growth in the process of long-term rehabilitation by their counsel and regular interpersonal contact. Each individual receives support in this manner. They are instructed to choose as sponsors recovering individuals who have maintained steady recovery for at least 1 year. Finding enough local sponsors has never been a problem; many such people are willing to assist patients with dual diagnosis in their integration to the AA community.

Double Trouble is a new type of 12-step program8 for persons with both a psychiatric disorder and an addiction. Smaller than the traditional group, it provides stronger support and openness to its members. Double Trouble groups are available to our discharged patients.

Educational programs, film and discussion groups about drug abuse, family conferences, and sessions with chemical dependence counselors are other treatment modalities for people with these difficulties.1 Such activities are effective in rallying members of the immediate family to give full support not only to the program, but most of all to the patient, who at one time was alienated and lost credibility with family and friends.


SOCIAL THERAPIES

Self-help groups for people with chemical dependency are important therapeutic modalities. Patient education, psychotherapy, and similar rehabilitative offerings are also typical treatment methods.

Self-Help Groups

Alcoholics Anonymous meeting attendance is mandatory, 7 days per week. This actively confronts denial of the substance abuse problem, thus diminishing the major barrier to treatment in this population. The routine 12 steps of the AA format are the focus of therapy for chemical dependence.2-4,7 Group participation, with oral and written assignments, is part of this approach. Substance abuse counselors facilitate this procedure with concentration primarily directed toward AAís first three rehabilitation steps, (1) recognizing helplessness over addiction, (2) recognizing the possibilities for recovery, and (3) deciding to commit to the process of recovery.7,9

On discharge, regular AA meeting attendance and completion of all 12 AA steps are anticipated.7 Discussions about all the steps of therapy in AA are readily available in the literature; peer counseling regarding these steps may be the most effective remediation of a substance abuse disorder.7,10

Education and Counseling

Discussions, lectures, and films are incorporated into the program for the purpose of informing and teaching patients about the deleterious effects of substance abuse on themselves, as well as on their families, employment, and future. Counseling and group or individual psychotherapy play a pivotal role in the attitudinal change being encouraged.1,4 A personalized approach opens the door to involvement in the program. One-on-one teaching augments individual advancement in the process. Gaining insight on self-care and improving judgment are other goals.

Rehabilitation Suggestions

The program presents several options that are available to persons reforming their lives. Vocational rehabilitation services are vital. People devastated by long periods of psychiatric disability and/or addiction can greatly benefit socially from sobriety. They are extended the privilege of working for a few weeks through the state vocational rehabilitation services agency after completion of the inpatient program. The job, though short, increases self-esteem. The vocational rehabilitation services agency then directs the patient to a permanent job, further education, or other related activities.

Placement Plans

The purpose of placement is to assist patients in finding not only a safe place for continued drug-free life, but also one that encourages prolonged sobriety, stability, and well-being while maintaining appropriate psychiatric therapies. A good social support network is important; thus, halfway houses or day programs are made available, as well.

Discharge planning begins at admission. Available options are discussed, with the patient playing a significant part in the decision-making process. The failure or success of placement after discharge often depends on the choice made. Placement is as vital as the formal part of the program, since the choice made often predicts the prognosis. Outpatient follow-up care and a stable place in which to live are assured for all cases.

In our experience, people who opt for halfway house placement outside their community have a greater chance of staying sober for a longer time period. Given the occasion to start over, they begin a life-style conducive to recovery, as opposed to those who choose to stay in their community. Of course, relapse occurs in all groups. Patients who remain close to the AA community generally have a better sobriety success rate.

VARIABLES

Variables such as young children, parents, and spouses or significant others also determine choices and outcome. Problems or concerns in these areas affect the patient. Small children without day care, for example, present an obstacle that is at times insurmountable; some parents are therefore unable to join in the treatment program. Few halfway houses offer assistance to women with children. Unfortunately, many placement programs are not prepared to handle child care. Those that do focus on helping their residents to be responsible and accountable.

Some people benefit from court-ordered treatment; a program that works with the legal system can enforce rehabilitation via sentencing as required by law. A program such as the one we have outlined should provide gratifying results in a population that is difficult to treat.

Variations in management are individualized to the patient, medical team, and institution. Facilities have widely different philosophies about treatment, for example, concerning the value of total abstinence versus controlled drinking as a therapeutic goal or the use of pharmaceuticals such as disulfiram (Antabuse) or naltrexone (ReVia) as an aid to sobriety.1 Dual diagnosis programs such as ours are generally recognized as being successful, despite dealing with a notoriously relapse-prone patient population.

Joel Velasco, MD, Arthur Meyer, MD, and Steven Lippman, MD Louisville, Ky

References

1. Zimberg S: Introduction and general concepts of dual diagnosis. Dual Diagnosis: Evaluation, Treatment, Training and Program Development. Solomon J, Zimberg S, Shollar E (eds). New York, Plenum Press, 1993, pp 3-21

2. Miller NS: Addiction Psychiatry: Current Diagnosis and Treatment. New York, Wiley-Liss, 1995, pp 206-225

3. Minkoff K: Models for addiction treatment in psychiatric populations. Psychiatric Annals 1994; 24:412-417

4. Miller NS: Prevalence and treatment models for addiction in psychiatric populations. Psychiatric Annals 1994; 24:399-406

5. First M, Gladis M: Diagnosis and differential diagnosis of psychiatric and substance abuse disorder. Dual Diagnosis: Evaluation, Treatment, Training and Program Development. Solomon J, Zimberg S, Shollar E (eds). New York, Plenum Press, 1993, pp 23-37

6. Anthenelli RM: The initial evaluation of the dual diagnosis patient. Psychiatric Annals 1994; 24:407-411

7. Twelve Steps and Twelve Traditions. New York, Alcoholics Anonymous World Services Inc, 1993

8. Zaslav P: The role of self-help groups in the treatment of the dual diagnosis patient. Dual Diagnosis:Evaluation, Treatment, Training and Program Development. Solomon J, Zimberg S, Shollar E (eds). New York, Plenum Press, 1993, pp 105-126

9. Alcoholics Anonymous: The Story of How Many Thousands of Men and Women Have Recovered From Alcoholism. New York, Alcoholics Anonymous World Services Inc, 3rd Ed, 1976

10. Chappel J: Long-term recovery from alcoholism. Psychiatr Clin North Am 1993; 16:177-187

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APA Reference
Staff, H. (2007, August 6). How to Treat Coexistent Mental Illness and Substance Abuse, HealthyPlace. Retrieved on 2024, December 20 from https://www.healthyplace.com/addictions/articles/how-to-treat-coexistent-mental-illness-and-substance-abuse

Last Updated: June 28, 2016