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A Patient's View of Ambiguous Genitalia

A presentation given by a UK AISSG member based in Continental Europe at a symposium titled "The Newborn with Ambiguous Genitalia: Diagnosis, Sex Assignment and Treatment" organised by Dr. Silvano Bertelloni and Dr. Paolo Ghirri, held in Pisa, Italy, on 8 Nov 2002.

I am very grateful for this opportunity to be allowed to present a patient's view of the issues surrounding ambiguous genitalia. It is encouraging that so many doctors all over the world are willing to re-consider and improve medical practice in this controversial area. You may not agree with everything I have to say, but I thank you for listening with an open mind. I shall not review the medical literature, but concentrate on the patient perspective.

Personal background

I have no medical training; I have degrees in English, French and Education from the Universities of Cambridge, Oxford and Tübingen and a psychological diploma as a school counsellor.

Nothing abnormal was noticed at my birth; I presented at 14 with clitoromegaly, was hospitalized and an orchidectomy, but no clitoral surgery was performed. Williams and McIndoe vaginoplasty were suggested, but I refused both. Hormone replacement therapy began. As a result, breast growth improved and the clitoris reduced in size. I know now that I was diagnosed with Partial Androgen Insensiivity Syndrome, but did not discover the full truth until I was 35. Vaginal length of about 2 cms was extended through normal marital relations and some help from dilation to about 11cms.

I started attending meetings of the AIS support group in England in 1995 and have been active in the German support group for women with XY chromosomes since 1998.

Both groups provide very intensive personal support and information for patients and parents and are involved in dialogue with the medical profession so as to find out the best possible treatment for people with intersex conditions. Through the Internet, more and more intersex people from all over the world are making contact with support groups. The new bestseller book by the African model, Waris Dirie, has also made female circumcision a much talked about issue.

Ambiguous genitalia at birth

A baby born with ambiguous genitalia or a teenager presenting at puberty is in the first instance a psychological not a surgical emergency. How can doctors be most effective in this situation?

Interpersonal communication is approximately 80% non-verbal and if doctors personally regard an intersex birth as monstrous or repulsive, they will communicate this to the parents whether they wish to or not and this will not help the parents to accept their child. Sexuality and sexual identity are areas of human existence where we all are at our most fragile and vulnerable. Doctors who are at peace with their own sexuality and can talk openly about the subject without fear or embarrassment have been found to be particularly helpful when dealing with intersex patients.

Doctors should take the necessary time to provide full and honest facts about an intersex baby's medical condition and make sure that parents really understand it, even if this takes several meetings. Garry Warne (Melbourne, Australia) and Gerard Conway (London) have produced publications for parents and teenagers on AIS and CAH which are honest, comprehensive and easy to understand.

Parents will also need experienced and well-trained psychologists over a period of time to help them to come to terms with their understandable grief and anger about their child's diagnosis. The aim should be that they learn to accept their child as it is and that no human beings are perfect. The illusion that perfection is possible is one of the great sources of unhappiness in our time. In my experience true happiness is only possible when one becomes reconciled to reality, says goodbye to illusions and stops brushing things under the carpet. The unresolved problems of childhood will find expression in later life in poor relationships, and in psychological or psychosomatic problems. Of course, coming to terms with the fact that you have an intersex condition is not easy, but there is no reason why intersex men or women with ambiguous genitalia should not lead as happy and fulfilled lives as people with big noses, short legs or hare-lips.

Faced with parents' strong emotions, it may be tempting to operate away the problem soon after birth, but parents should be made aware that they may face bitter resentment if their grown-up children are not content with their surgery or their assigned role. Are the doctors who operate the genitals of children able to face these people as adults and willing to accept reponsibility for the monumental distress and anger that some of these people have about their childhood surgery? I have met some of them personally and have been shocked to experience the depth of their pain. There are sadly too many of these cases, people who feel that they have been physically mutilated by unsuccessful genital or vaginal surgery, or that their lives have been destroyed by being assigned to the wrong gender, pumped full of what they see as being the wrong hormones.

I know of one very distressing case in 2002 of a teenager assigned to the female sex as a baby, whose parents put her in a psychiatric hospital this year because she became so aggressive and difficult. She does not know about her diagnosis, but is convinced that she is male, not female. The psychiatrists could find nothing wrong with her and came to the conclusion that her anger with parents was justified and healthier than sinking into depression and becoming suicidal. In another case, an intersex baby with normal female organs, but enlarged clitoris was assigned to the male sex at the mother's request. But already at age 4 the mother had to beat the child with a stick to make it wear boy's clothes. The child began a double life and wore female clothes in secret; when this was discovered, her mother punished her and told her she was perverse. After 37 unhappy years of living as a man, she was convinced that she was transsexual and asked for a sex change. Then her internal organs were discovered. She described this discovery like winning millions in a lottery. She now lives as a woman and no longer feels that she is a split personality. She has appeared on television several times to tell her story. But her relationship to her mother has been destroyed.

Of course these are extreme cases, but parents faced with the difficult decisions of gender assignement and surgery have a right to know what can go wrong as they may have to cope for the rest of their lives with children who become traumatized adults as a result of irreversible genital surgery and wrong gender assignment. Parents I know in Britain, Germany and Switzerland are increasingly making the decision to make only a temporary gender assignment for their children with ambiguous genitalia, and wait with surgery until their children are old enough to express their wishes.

There is naturally a danger that children with ambiguous genitalia may suffer torment from their peers and that this may harm their self-confidence. I have memories of other children doubting that I was a girl and of girls pointing at me in a swimsuit when I was 13 and saying that my genitals looked strange. But as I was not in any doubt about my gender identity and was otherwise very self-confident, it did not bother me greatly. My doctor always told me my clitoris was „within the range of normal" which I found helpful. I now know from friends who are urologists and gynaecologists that there is an enormous variation in the size and appearance in male and female genitals. If we start prescribing a norm to which they must all conform, surgeons will be operating day and night till the end of the world. Of course, everything should be done to protect children and bring them up to feel confident about themselves and if one has a child with ambiguous genitalia one will have to be doubly careful. One cannot always protect any child from suffering at school or elsewhere, but one can make it strong enough to deal with the situation. A disturbing proportion of parents of children born with ambiguous genitalia also report massive pressure from some doctors to consent to genital surgery on the ground that it is the only moral and medical way forward. I think this is a mistake.

Doctors should be honest about the fact that things are not so clear-cut. We do not have enough research data on the long term effects of gender assignment, or about the limitations of genital surgery. Some parents are given the impression that one quick operation will put things right for ever. According to a recent British study, almost 90% of women who undergo genital surgery as children need more surgery later in life. I therefore strongly feel that little girls born with vaginal agenisis do not need neovaginas and should not be operated on so early in life. Delaying vaginal surgery till puberty at the earliest reduces the total amount of surgical intervention necessary and increases the chances of even better operative techniques being available to the patient at a later date. Knowing several women who have had unsuccessful Williams and McIndoe vaginoplasties, I am relieved that I did not opt for these procedures. The Vecchietti procedure (I presume this is an Italian success story?) which is being inceasingly used in Germany, is non-invasive, leaves virtually no scarring, lubrication is good and sexual partners are highly pleased with the result.

I do not know to what extent detailed follow-up studies of adults who had genital surgery as children or were gender-assigned in Italian hospitals have been carried out, but I very much hope that this can be done. We need all the data we can possibly get, to help doctors, patients and parents make the best-informed decisions. And until we know more, we should tread very carefully and make decisions which leave the most options open for the future.

Truth disclosure

I was lied to by doctors and parents till I was 35 when I consulted a specialist in Cambridge. The story I was told as a teenager was that I had suffered from underdevelopment of uterus and ovaries and that these were removed to prevent cancerous tissue forming. It was obvious to me that both doctors and parents were extremely worried and were not telling me the truth, so for years I was convinced that I had cancer and was going to die. This was a terrible and unnecessary burden on a teenager, far worse than knowledge of an intersex condition.

In hospital, aged 14, I was displayed naked for medical students; I cannot express how traumatic this experience was and how long it has taken me to get over it. Being photographed naked made me feel like a circus exhibit. In Britain today it is against the law to take medical photographs without the consent of the patient.When I left home at 18, I was given a medical note with the untrue medical facts. This is what I told the man I married when I met him. You can perhaps imagine how betrayed he felt, when after 17 years, we discovered the truth. We have now been together for 27 years, but it nearly destroyed our marriage. A partner must have the right to make a free decision whether to marry someone with an intersex condition or not. Not everyone is so secure in their personal and sexual identity that they can cope with having an intersex partner and one has to respect this.

The Bible says: „The truth shall make you free." In my experience and that of the overwhelming majority of the support groups in Britain and Germany, knowing the truth about one's intersex condition is far, far preferable to a world of ignorance, fear and futile speculation. It is hard to describe the relief and peace of mind I felt when, at 35, I at last had the facts and everything made sense. Children are not stupid - they know when adults are lying to them, they know that something is seriously wrong with them and, as we know from children with terminal illnesses, they are capable of dealing with the truth if it is presented to them in a way that is suitable for their age. Just as we translate languages, we need to put time and effort into translating an intersex diagnosis into language which is both truthful and appropriate for children. Lying destroys trust. And without trust, we cannot live.

University College and Middlesex Hospital, London under Gerard Conway have an interdisciplinary team with psychologists, gynaecologists, urologists, endocrinologists, paediatricians and surgeons for intersex families. Patients and parents are extremely satisfied and grateful for the excellent service that is provided. I can only recommend it to you. It is also important that doctors form networks and cooperate with each other, as the University Clinic in Lübeck, Germany, under Olaf Hiort has done in an exemplary way. Patients will benefit more from a pooling of experience than if individual doctors all do their own thing and regard each other as rivals.

Support groups

Support groups provide an invaluable service. People who have battled alone for a lifetime with shame and secrecy encounter a situation of openness, warmth and honesty in meetings; a subject which has been unsettling and frightening becomes the most normal thing in the world. The psychological healing that takes place by sharing with people who are struggling with or who have come to terms with the same problem is greater than any psychologist can provide. Parents can talk to others who have been through the same difficult decision-making processes and can benefit from their knowledge. The British AIS Support group website (http://www.aissg.org)is a mine of well-researched information on AIS and related intersex issues .

One of my most moving experiences at a British AIS meeting was seeing 3 eight-year old girls who had never met before playing together all day. They all knew the basic facts of their condition, and were forming friendships which will be so important for their teenage and adult years. They will not have to suffer the isolation as my generation did. They experience a community of adults who are demonstrating their ability to deal constructively with a difficult diagnosis. Going to a support group meeting for the first time is perhaps a bit like coming home at last after having been forced to live in a foreign country all one's life. The women and parents I have had the privilege to get to know at support group meetings are some of the most impressive human beings I have met in my life. It is hard to believe that there are people who think that it would have been better if these women had never been born. Through the internet I have had moving email contact with intersex women as far away as Singapore and China who have lived a life of secrecy, shame and fear and are deeply grateful to have at last got to know someone who knows exactly what they have been through. I do hope that those of you who have kept in contact with past patients and parents will encourage them to set up support groups in Italy. Doctors may feel that support groups make the „consumer" more demanding, but support groups can also take a lot of pressure off doctors, and open dialogue and sharing of experiences can only improve the situation for the patients, whose welfare, after all, is our main concern here today.

The general public needs to be much better educated about the high frequency of intersex births, that nature has more variations than are commonly realized and that there is no shame in being intersex or having an intersex child. In Britain, Germany and the USA there is a lot of media interest in the subject, and recently there have been a number of films, radio interviews, magazine and newspaper articles, books etc. This can only help to create a climate of openness which is the best way to overcome fear and taboos.

Gender roles

Just as we all have a unique mixture of male and female hormones in our bodies, so there is infinite variation between the conventional poles of male and female, not just among people with intersex conditions. The immense variety of fishes that one can see on a coral reef are a source of wonder, but for some reason, when we encounter variation in gender issues, it worries us. But we all know people of both sexes who do not have intersex conditions, are not homosexual or transsexual, but who do not conform to the cliché of what makes a typical man or a woman. C.G. Jung encourages people to discover the attributes of the other sex inside them. Seeing male and female in black and white terms does not correspond to biological and psychological reality and is not helpful, unless seen as a very broad category. As long as society views gender in a bi-polar way, temporary gender assignment will, however, probably still be necessary. I know people who advocate a third sex or even five or more categories and while I respect this view and acknowledge the fact that some people feel neither male nor female, I personally am not happy about introducing another rigid box. Gender identity is influenced by so many factors and there is such enormous variety amonst human beings. I prefer to see gender as a sliding scale with every nuance of difference between the extremes. Every individual is unique and at the end of the day we must leave it up to the individual to decide where on the sliding scale between male and female they align themselves and feel at home.

Decisions

Everyone agrees that decision-making is extremely difficult for both doctors and parents when a child is born with ambiguous genitalia. Everyone involved wants to make the best decision for the child. But the best decision will be the one which proves to have been right in the long term and which corresponds most closely to the individual's deepest sense of self as an adult.

Wherever possible, the patient should decide themselves about such essential things as gender assignment and genital surgery, particularly when the surgery which is done can never be reversed. I found out as an adult that my parents had been asked which sex to assign me to when I was 14. Luckily, they made the right decision. But I see no justification whatsoever for not consulting an intelligent teenager on her opinion on an issue with such far-reaching consequences for her future happiness. What if my parents had decided that they would have preferred to have a boy?

I am grateful to be one of the few women with an enlarged clitoris who did not have clitoral surgery with the possible risk of lack of sensation. I have no problems using a mixed sauna today and feel at home in my body. Maybe my clitoris is larger than average. So what?

Closing remarks

I would like to close with a brief tribute to an Italian, whose life is an inspiration when dealing with taboo subjects and groups which are marginalised by society. I am sure you all know the story of how St. Francis of Assisi embraced a leper. He didn't just overcome his revulsion for a few minutes, give the man a quick hug and then return for ever to his safe world. From this time on, he spent an enormous amount of time with lepers, behaved as a brother to them, not as some elevated person who condescends to give a little time or money, but remains distant. His followers spent their novitiates in leper colonies. His fellow citizens, however, expelled these people from society; one could say that in psychological terms the society split off and projected that part of itself which it could not accept on to these lepers. In the eyes of many today, intersex people with or without ambiguous genitalia are seen like lepers. But I have met excellent doctors, who not only have great knowledge about intersex issues, but who have the courage to talk to many intersex patients and find out how they feel about their treatment. They have had the humility, even if they were not responsible for it themselves, to listen to criticism and anger about how these patients have been failed. Their primary concern as doctors is to serve. It is my hope that there will be more and more doctors like this, who will encounter the lepers of our time in a Franciscan spirit. When I visited Assisi two months ago and heard that even „Brother Death" has a place in the Cantico del Sole in which St. Francis praises the diversity of Creation, I realized that people with intersex conditions like me also have a place and can sincerely say „Laudato si, o mio signore - anche per questo corpo!„ Thank you.

Barbara, XY women's support group, Germany, Oct 2002.