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Glossary

Born Without

This is an article by Margaret Horsfield which was published in ‘She’ magazine (approx 1983). It gives an account of a girl with no vagina (but not with AIS) who achieved success with the Frank Pressure Dilatation method under the guidance of the then Chelsea Hospital for Women. London. The Gynaecology Department at the hospital (now renamed Queen Charlotte’s and Chelsea Hospital) still uses this as their method of choice and has a support mechanism in place to provide the necessary encouragement and motivation (see note at end).

Congenital absence of the vagina is a rare condition, but not as rare as most of us would guess; about one in 4,000 females are affected. Half, like Susan, opt to try and overcome their condition without surgery. So far, hers is a remarkable success story.

In her early teens it wasn't so hard for Susan when the other girls were talking about their periods. She listened with interest. When her time came, all these comments about tampons and menstrual cramps and how to avoid gym class would come in handy. As the years passed, though, she became increasingly anxious – 14, 15, 16, and still no periods. She kept tampons and sanitary towels on hand, just in case, even hopefully packed them in her bag when she went away on holiday, but to no avail. Her friends and her sisters ceased to mention the subject to her, and she turned away, or tried to talk of something else, when periods came up in conversation. Visits to the doctor got her nowhere: “You're just a late starter,” she was told. So she waited some more; through 0-levels, through A-levels, but still no periods. By now she had reached 18.

All this time, Susan's mother Janice had also been concerned. But her worry was partly eased by the fact that Susan's development was otherwise perfectly normal. She was a very pretty, very bright teenager. She excelled at school in music and art; she had lots of outside interests and lots of friends, and a cheerful and comfortable family setting as one of four children close in age. To look at Susan, you would assume that nothing but good fortune surrounded her. But by the time she was due to leave home for university, Susan and her mother had decided that they needed to know more. Why had she not yet had a period? They both thought that there could be a hormonal imbalance. Susan went to see an endocrinologist.

Their hopes rose, because at first it looked as if they might be right. The reason must be hormonal. To try to jolt Susan's hormones into action, the endocrinologist put her on the pill for a month. But nothing happened. Susan returned to the specialist and was told that a rectal examination would be necessary, a technique which, combined with pressing on the abdomen, can reveal if anything is wrong with the shape of the uterus. The result was that Susan was referred to the Chelsea Hospital for Women to see Professor Sir John Dewhurst. Her problem was not hormonal, it was physiological.

Examination succeeded examination, and then one day Susan's mother was asked to go to the hospital on her own. Her daughter's condition was explained to her before Susan was told. The reason why she had not yet menstruated was that she had no vagina, and only a vestigial uterus. Congenital absence of the vagina, as the condition is known, is very rare, affecting approximately one woman in 4,000. When the vagina is absent, the uterus is almost always absent as well.

Professor Dewhurst, now retired from Chelsea Hospital, is one of about 20 doctors in Britain who specialise in this condition. Over the years he has seen scores of cases, but it is perfectly possible that a consultant gynaecologist could spend a lifetime in the business without seeing a single case like Susan's. At Chelsea Hospital two or three new cases might be referred every month, but these come from all over the country, and indeed, from all over the world. And almost always, the condition is completely unknown to, and unsuspected by, the patient and her family.

“We realised there must be something wrong physically,” says Janice, “but I never imagined anything so absolute. I thought perhaps the uterus might be poorly formed, or somehow not linked up properly to the vagina, but it never crossed my mind that there might be a total absence of the vagina and an almost total absence of uterus.”

Susan was still unaware of all this. But just before the Christmas of her first term at university, Susan and her mother and her younger sister went up to London to spend most of a day at Chelsea. It was then that Susan was told.

“I just felt very cold” she recalls, “They gave me ultrasound and I could see my uterus. It's a sort of tiny stubby end – want me to draw it for you?” And she sketched out something about the size of a large knobbly end of a thumb. This ‘uterus’ is just useless muscle fragments; evidence of something that never formed in Susan’s body, and never will. Leading from it is no vagina at all; nothing but a mass of soft internal tissue.

Susan's case is typical. Externally, the genital area is perfectly normal, with clitoris, labia, and urethra all in place. But where the vagina should be there is only a small dimple or indentation. The length of this can vary from case to case, but is rarely more than a couple of centimetres at most, and can be barely perceptible. A visual medical check would not necessarily reveal the nature of the problem because the ‘vagina’ would resemble that of any virgin whose hymen is intact. Because doctors are generally reluctant to attempt an internal examination on virgins, the full nature of this condition is rarely revealed until late in the teens, and then by specialists. The earliest diagnosis Prof Dewhurst recalls making was for a 13-year-old, but her condition was somewhat different. She was suffering from the even more rare, and certainly more dangerous, condition of having no vagina but having a functional uterus, with a cavity capable of containing menstrual blood. She was at the age when menstruation began, but the blood could not escape from the uterus, and infection set in, with great pain. And diagnosis can come very late indeed. There is a recorded case of a woman who had been married for two years before finally seeing her doctor who referred her to Chelsea. She had been a virgin when she married, and she had never had periods, and when her husband was unable to penetrate she took it to be entirely her fault, something she was doing wrong. In fact, she had no vagina.

Many permutations of this condition are possible. Usually when the vagina is absent, so is the uterus, but it is impossible [we think this should be 'possible'] to have a uterus and no vagina. It is even possible to be born with vagina and uterus, but no cervix. To spell out to a teenage girl that she has neither vagina nor uterus instantly gives rise to two reactions. Not only “I can never have children”, but “I can never have sexual intercourse”. And to help with these reactions, a long interview session is always set up at Chelsea Hospital for Women once the nature of the condition has been revealed. Psychologically, it is a great shock, and the emotional maturity of the girl is always taken into account before telling her of her condition. Family support is also considered vital.

In Susan’s case, help and support were on offer from the outset. It was made clear right away that sexual activity was not impossible at all. A vagina had to be created and she had two options. She could create a vagina for herself through what is known as ‘dilatation’ technique, or she could undergo surgical procedures to create a vagina. She would be able to enjoy normal sexual activity like anyone else. On the question of children, having no uterus, she can never carry her own child, but because, as in most cases of this kind, her ovaries are present and functioning normally, there is the possibility of in vitro fertilisation. One of her eggs could be extracted and fertilized with her husband's sperm, and the child could be borne by a surrogate mother. Susan will never forget the reactions of both her sister and her mother when they heard that. Her sister instantly volunteered to act as surrogate if she was needed, and her mother declared that if only she were ten years younger, she'd gladly bear Susan's child herself.

Often mothers like Janice feel guilty, and wonder if somehow it is their fault that their daughters should have this rare condition. The doctors are frequently asked if it could be due to a drug taken in pregnancy. But there is simply no known cause. As Susan puts it, “It's just something you're born without.”

It's now four years since Susan was first told of her condition. During those four years she has completed her university degree, started post-graduate work, travelled abroad a bit, and had several boyfriends, though none of them too serious. She has also succeeded in creating her own vagina through the dilatation, or pressure technique. She shied away from the prospect of surgery, and was actively encouraged to try dilatation, which the doctors always prefer patients to try as a first course of action. But because this procedure can be slow and discouraging, and requires high motivation, about 50% of patients end up resorting to surgery. Not Susan, however. She began with a ‘number one’ dilator, a tiny glass tube, like the smallest size of test tube, and set herself to the task of applying pressure for half an hour, morning and night, on where her vagina should be.

“It was terribly hard at first, because I didn't really know where to press – I just didn't know where my vagina should be! If there was an existing dimple I wasn't sure where it was, or at what angle I should aim the dilator.”

But with guidance from the hospital, Susan persisted. The soft tissue slowly gave way to pressure, and a definite indentation appeared. It wasn't painful, just uncomfortable, though sometimes a local anaesthetic gel was put on the tip of the dilator as it went deeper. Susan began to use larger dilators. In less than two years she was up to a number four, which she still uses. It's over an inch in diameter and about five inches long. She's not now as persistent about the half-hour ritual twice daily as she used to be, but she knows she has to continue the dilatation, because the vagina will contract and begin to close up if she doesn't. The whole idea of the technique is that dilators are used until regular sexual intercourse occurs, and this should do away with the need for constant dilatation. But because Susan has not yet established any pattern of sexual activity, the pressure technique must continue.

“It's actually quite hard work,” she says. “My wrist gets terribly stiff, and the dilator digs deep ridges into my fingers and they all go numb.”

About a year ago, Susan was offered the option of surgery when it appeared that she was tiring of the pressure technique, but she again rejected the idea. Intercourse is possible about two or three weeks after surgery, but it takes the better part of a year of applying dilators before intercourse can be attempted.

The surgical procedure involves two stages. The tip of the existing vaginal ‘dimple’ is incised, and the tissue is spread, usually simply with the surgeon's fingers. Little cutting is required because the tissue is all so soft. When the required vaginal depth is reached, a mould is inserted, and the labia are stitched together to hold the mould in place. In earlier years the mould would have been covered with a skin graft, taken from the buttock or thigh, but recently a new technique has been introduced. The mould is covered not with skin, but with amnion, the membrane containing the waters surrounding the baby in the womb. Amnion is also at times used in plastic surgery and in the treatment of burns, and it is usually taken from Caesarian section deliveries. The amnion encourages the growth of soft and elastic vaginal skin, and when the mould has been in place for a week, it is removed, covered with a fresh amnion, re-inserted and sewn back in place, to be removed finally after another week.

Susan is relieved to be in the successful 50% who have not needed surgery. She thinks her vagina is about the right size now, though perhaps a bit short – it's difficult to tell. She hasn't been back to the hospital for a check-up for several months now, though it's about time, because a lot of questions have come to her mind, especially as she sees more and more of her friends get married and start families.

“I'd do anything to have my own child, and I keep thinking of crazy-sounding things, like would I ever be able to breast feed, or has the possibility of a uterus transplant ever been considered?”

Her mother Janice is more concerned with the existing possibilities if Susan wishes to have children. Given the increasingly tight controls on in vitro fertilisation and surrogacy, she hopes that Susan's unusual case might be given special consideration, and she even wonders if Susan could be given priority if she ever wanted to adopt a baby. But until Susan shows signs of wishing to marry, all this is very much in the future.

Susan hopes to take next year off to travel, to put aside her academic work and have a year of freedom before grappling with her career. But whereas ten years ago she travelled hopefully with tampons in her bag, now she'll have to pack her dilator. Is it ever possible to forget about her condition?

“Oh yes, sometimes I do just shut it out, but then I'm always reminded – friends complain about their period pains, or someone asks me if I want to have children, and says I'd make a wonderful mother.” Very few people know about Susan's condition; her family, and two close friends, that’s all. No boyfriends so far.

“That'll be a very great hurdle for her to get over, telling a boyfriend,” says Janice. “And naturally, she will fear rejection because of it – and who can blame her?”

All Susan can do is hope for the best and trust her own judgment in how and when she tells any serious boyfriend about her condition. Sexually, all should be well, but of course Susan is nervous. Her vagina is a hard-won achievement, and she just hopes it's as it should be.

Despite all the help and support from her family and from the Chelsea Hospital for Women, Susan is effectively on her own in dealing with her rare problem and in determining how it will affect her life. So far, she's come through triumphantly.


Notes:

A member of the AIS Support Group met Susan at one of the support meetings at Queen Charlotte’s in the mid 1990s. She was happily married and had recently adopted a baby.

We believe amnion has been out of favour in some quarters, since the outbreak of AIDS, although a member of the AIS Support Group has been told by Gill Little (who runs the dilation support meetings at Queen Charlotte’s for patients with vaginal agenesis) that Mr Keith Edmunds who now heads the Gynaecology Department there will use amnion in some cases, after it has been sterilized in hydrogen peroxide.

The main problem with the Queen Charlotte's approach (has it changed since the mid 1990s?) is that they have a paternalistic policy of non-disclosure of diagnostic information in XY-female conditions, so any mention of XY chromosomes, testes etc. (e.g. by those who might have been referred there already knowing the truth about their condition) is expressly forbidden at their dilation support meetings. So it is not a support group for XY-female conditions, only a support and motivation mechanism for vaginal ditation.