Issues for women with CAH
For those women who find coping with the medical, surgical, psychological or sexual problems of CAH difficult, support and counselling should be made available. Sensitive discussion with specialists, general practitioners, fertility specialists, gynaecologists, counsellors, psychologists and CAH support groups can all be helpful. The difficulties experienced by some women with CAH arise because of decisions made many years ago when less was known about medical and surgical care. Young adults should no longer have to face many of the problems addressed in this section.
Unwanted hair growth and acne
The high androgen levels that occur when cortisol treatment is too low, commonly cause problems for women with CAH. For many women acne and excess hair growth, 'hairiness' or hirsutism are the first features of high androgen levels. The aim of changing the dose of cortisol treatment is to reverse this process and it can be a very fine line between keeping free of unwanted hair and suffering the side effects of cortisol treatment - in particular weight gain. Because of these difficulties, some women with CAH require additional treatment for excess hair growth to oppose the action of androgens. The 'anti-androgen' treatments available in the United Kingdom are cyproterone acetate, spironolactone, flutamide and finasteride. Spironolactone should not be used for women with CAH because it interferes with the action of fludrocortisone. Anti-androgens are often given together with the combined oral contraceptive pill which reduces the amount of testosterone made by the ovary. Hirsutism takes several months to respond to treatment but cosmetic methods of treating excess hair such as waxing, shaving, creams or electrolysis are perfectly acceptable options which in no way cause hair to become thicker.
Contraception
There is no restriction for the use of different contraceptives for women with CAH. The choices of contraception include the sheath, the diaphragm, the coil and oral contraceptive pills. The combined oral contraceptive pill may have the added benefit of making irregular periods regular. Some women, however, find that the pill makes it more difficult to lose weight although a low dose pill might get around this problem. Dianette is commonly advised for women with CAH because it is particularly effective in suppressing unwanted hair growth. Progesterone only pills - 'mini-pills' can be used by women with CAH but they can cause difficulties with irregular periods.
Irregular periods
Irregular periods occur in about one third of women with non-salt losing CAH and one half of women with salt-losing CAH compared to one in ten of women without CAH.
Fertility
Most women with CAH have polycystic ovaries when pictured by an ultrasound machine. In fact, one in four of women without CAH also have polycystic ovaries, so this in itself is not a worrying feature though, of course, a concern to the individual. Polycystic ovaries are slightly larger than average and contain more small follicles, where the eggs develop, than average. Polycystic ovaries are associated with irregular periods and infertility which are both common in women with CAH. It may be that high levels of testosterone in childhood cause the development of polycystic ovaries in some women. High levels of testosterone from the adrenal gland cause irregular periods and failure to produce an egg - failure to ovulate. Irregular periods and failure to ovulate can improve with higher doses of cortisol treatment but the balance of treatment in this instance can be very difficult.
Fertility prospects for women with CAH should be considered as normal (see achieving pregnancy). With careful adjustment of steroid treatment with particular attention to progesterone levels throughout the cycle, normal fertility treatment is rarely required. This is in complete contrast to older information suggesting that only 1 in 10 women with severe CAH could conceive.
Pregnancy
Once pregnant, both the mother with CAH and her child should expect to be healthy in every way. There is discussion as to whether there should be a slight increase in dosage of cortisol replacement treatment in late pregnancy but high dose treatment should be avoided. Dexamethasone should not be used in pregnancy because it crosses the placenta. The placenta protects the baby from any hormone imbalance in the mother and destroys any excess hydrocortisone or testosterone in the circulation. In a recent review of 46 children born to mothers with CAH, all babies were healthy and normal. Two thirds of babies were delivered by caesarean section and only one third by vaginal delivery. There are many reasons why caesarean sections might be common in women with CAH but one worry is that earlier genital surgery might make normal labour difficult. Pain relief and the use of epidurals is the same in CAH as normal.
Gender Issues
Issues about gender often cause great concern but are not always addressed. It is important to understand that male and female gender identities are established before birth and not affected by hormone changes later in life. However, it is often why in late teenage years or later that gender preferences become obvious.
Many women with CAH have successful heterosexual relationships, though for some finding a partner is difficult. There are some women with CAH who are more comfortable with bisexual and homosexual relationships but this may in some cases, have more to do with their concerns about the appearance of their genitalia, with feelings of vulnerability and apprehension of a sexual relationship with men. The main point to remember is that if a woman has established heterosexual feelings, there is nothing about CAH that will change this, even if the testosterone levels rise from time to time.
In all women, testosterone levels can affect sex drive or libido. The effect of testosterone on libido however, is very unpredictable. Some women with CAH find that when testosterone levels are high, sex drive is increased. Reducing testosterone with higher dose of cortisol treatment can reduce excessive sex drive. A low libido is a more difficult problem as it is rarely improved by changing the dose of treatment – counselling might be an answer here.
The attitude of parents and doctors to gender and sex, and their own perceptions of this area, can influence how girls and women cope with CAH. Women manage much better if, as children and young adults, their parents and doctors were able to discuss openly and sensitively, not just the medical side but also the emotional and sexual issues. This affects how women perceive their genitalia/bodies as acceptable to others.
Those who have been brought up with no information about the condition and are unable to talk to their parents or doctors, find it difficult to over come common barriers when problems are brushed aside and sexual matters are not considered as a matter for discussion. A young adult, who will probably have had vaginal surgery will find it hard to ask for advice. She may find it very difficult to be comfortable with her body, be anxious about the prospect of sexual activity and unable to see it as something pleasurable.
Genital surgery
All girls with CAH are born with a vagina, but sometimes it is difficult to establish the position or size as these can vary considerably. The vagina may be covered with a small membrane and the clitoris is nearly always enlarged. Some girls with mild forms of CAH, do not need any surgery.
The timing of any surgery is dependent on the degree of virilisation and the feelings of the parents after discussion with their specialist as to the options available. In order to feel confident of decisions about surgery a second opinion may well be helpful. A specialist gynaecologist with experience in surgery for women with CAH is advised. Surgery to reduce the size of the clitoris, a clitoroplasty, and to make a larger vagina, a vaginoplasty, can be done at any age and opinions vary as to the best timing.
The aim of a clitoral reduction is to reduce the erectile tissue and preserve the nerves and blood vessels to the glans, the tip of the clitoris, so that it remains sensitive. Surgery required for the vagina and urethra in more complicated and varies considerably. If the vagina is positioned high near the bladder there can be the possibility of damage to the neck of the bladder, which controls the release of urine. Great care must be taken as any damage to the bladder could cause incontinence. Pelvic floor exercises can be of great help with any minor weakness with the bladder control, and will also help to strengthen the vaginal walls.
An examination just before the start of menstruation is often carried out to establish that the vagina is able to allow the use of tampons and also to make sure that the vagina will be big enough for comfortable sexual intercourse. Some specialists prefer to do this examination under a general anaesthetic, which though not strictly necessary, can make it less embarrassing for the young adult.
Some women may require surgery later in life for an enlarged clitoris or because of a small vaginal opening. It is rare for the clitoris to enlarge in adult life unless the control of CAH is very poor for a long period of time so that high testosterone levels cause the growth of the clitoris. Despite earlier surgery, the clitoris may remain relatively large in some women. In adulthood, vaginal surgery may be required to ensure comfortable sexual intercourse and to reinforce confidence in feeling feminine. Even if surgery on the vagina has taken place in childhood, it is not unusual for the opening of the vagina to be small. There are many surgical procedures using different methods to enlarge and line the vagina. ‘McIndoe’ techniques use skin grafts from the buttock or thigh to form a ‘tube’ like new vagina. Alternatively, a part of the colon can be transplanted to the vaginal space. Women needing vaginal enlargement should have information on the various techniques available and be allowed to discuss all the possibilities. For example, the two surgical techniques described above leave scars and increasingly experts feel that the less invasive use of dilators is best for women even for those who have a very short vagina. There are some surgeons who feel that vaginal surgery for an adult should only be considered if there is already an established relationship. The alternative view, often held by women themselves, is that early surgery is necessary for sufficient confidence and self esteem to establish a sexual relationship.
Surgery now has much improved over the past 30 years and the involvement of patients in decisions affecting their care has increased. Adults, who had surgery many years ago, may experience more difficulties from the effects of surgery, than those who have had more up to date operations. For instance, the practice of complete clitoral removal, clitorectomy, was once quite common. Alternatively, some erectile tissue of the clitoris might have been left secured under the skin in front of the vagina, this can be extremely painful during intercourse. Also, the glands that secrete the vaginal lubrication prior to and during intercourse might have been damaged making is necessary for a lubricant, such as KY jelly to be used for comfortable intercourse. Some women notice a build up of scar tissue after more than one operation on the vagina. This can cause lack of sensitivity within the vagina or pain during intercourse as the scar tissue is not pliable and does not stretch easily. Oestrogen cream applied to the vagina or the use of dilators can overcome some of these problems
In most women, a small vaginal opening or shortened vagina can be improved with dilators of various sizes that will allow successful sexual intercourse. By applying gentle but firm pressure to the vaginal opening by inserting a plastic dilator and, over a period of time, gradually introducing a larger size of the dilator, the size of the vagina will increase. Within months a significant increase in size can be achieved. For those women who are not sexually active, the vagina will tend to contract if dilators are not used regularly. It may be necessary to keep up a regular alternate day use of dilators for ten minutes or so. Some women feel less happy with the dilators and prefer to use their fingers. However, this method takes time, perseverance, motivation, and the ability to feel comfortable in touching the genital areas. Also, some women with CAH may have relatively small fingers; if there is a partner, their help may be of benefit until intercourse is comfortable. The use of a dildo may also be acceptable. Intercourse itself can gradually increase the size of the vagina but it takes an unusual degree of self-confidence to feel comfortable with this technique.
Some women find that everything to do with the mechanics of sex is very embarrassing. Teenagers, in particular, may find it difficult to find support and understanding if their parents find this uncomfortable territory. Extreme sensitivity is needed so that they can discuss how they can improve what they may see as an inadequacy of their body. An experienced sex therapist might be useful here. It must be remembered that women without CAH have genitalia in all shapes and sizes. The penis is a very adaptable organ which also varies greatly in size.
It may be that the surgical and psychological issues of sex make it difficult for women with CAH to achieve an orgasm. The complete removal of the clitoris, pain on intercourse, poor vaginal lubrication or lack of self-confidence may all contribute to a failure to achieve orgasm. A gynaecologist with specialist experience or sex therapist should be able to help overcome these problems.
For those women who find coping with the medical, surgical, psychological or sexual problems of CAH difficult, support and counselling should be made available. Sensitive discussion with specialists, general practitioners, fertility specialists, gynaecologists, counsellors, psychologists and CAH support groups can all be helpful. The difficulties experienced by some women with CAH arise because of decisions made many years ago when less was known about medical and surgical care. Young adults should no longer have to face many of the problems addressed in this section.
Unwanted hair growth and acne
The high androgen levels that occur when cortisol treatment is too low, commonly cause problems for women with CAH. For many women acne and excess hair growth, 'hairiness' or hirsutism are the first features of high androgen levels. The aim of changing the dose of cortisol treatment is to reverse this process and it can be a very fine line between keeping free of unwanted hair and suffering the side effects of cortisol treatment - in particular weight gain. Because of these difficulties, some women with CAH require additional treatment for excess hair growth to oppose the action of androgens. The 'anti-androgen' treatments available in the United Kingdom are cyproterone acetate, spironolactone, flutamide and finasteride. Spironolactone should not be used for women with CAH because it interferes with the action of fludrocortisone. Anti-androgens are often given together with the combined oral contraceptive pill which reduces the amount of testosterone made by the ovary. Hirsutism takes several months to respond to treatment but cosmetic methods of treating excess hair such as waxing, shaving, creams or electrolysis are perfectly acceptable options which in no way cause hair to become thicker.
Contraception
There is no restriction for the use of different contraceptives for women with CAH. The choices of contraception include the sheath, the diaphragm, the coil and oral contraceptive pills. The combined oral contraceptive pill may have the added benefit of making irregular periods regular. Some women, however, find that the pill makes it more difficult to lose weight although a low dose pill might get around this problem. Dianette is commonly advised for women with CAH because it is particularly effective in suppressing unwanted hair growth. Progesterone only pills - 'mini-pills' can be used by women with CAH but they can cause difficulties with irregular periods.
Irregular periods
Irregular periods occur in about one third of women with non-salt losing CAH and one half of women with salt-losing CAH compared to one in ten of women without CAH.
Fertility
Most women with CAH have polycystic ovaries when pictured by an ultrasound machine. In fact, one in four of women without CAH also have polycystic ovaries, so this in itself is not a worrying feature though, of course, a concern to the individual. Polycystic ovaries are slightly larger than average and contain more small follicles, where the eggs develop, than average. Polycystic ovaries are associated with irregular periods and infertility which are both common in women with CAH. It may be that high levels of testosterone in childhood cause the development of polycystic ovaries in some women. High levels of testosterone from the adrenal gland cause irregular periods and failure to produce an egg - failure to ovulate. Irregular periods and failure to ovulate can improve with higher doses of cortisol treatment but the balance of treatment in this instance can be very difficult.
Fertility prospects for women with CAH should be considered as normal (see achieving pregnancy). With careful adjustment of steroid treatment with particular attention to progesterone levels throughout the cycle, normal fertility treatment is rarely required. This is in complete contrast to older information suggesting that only 1 in 10 women with severe CAH could conceive.
Pregnancy
Once pregnant, both the mother with CAH and her child should expect to be healthy in every way. There is discussion as to whether there should be a slight increase in dosage of cortisol replacement treatment in late pregnancy but high dose treatment should be avoided. Dexamethasone should not be used in pregnancy because it crosses the placenta. The placenta protects the baby from any hormone imbalance in the mother and destroys any excess hydrocortisone or testosterone in the circulation. In a recent review of 46 children born to mothers with CAH, all babies were healthy and normal. Two thirds of babies were delivered by caesarean section and only one third by vaginal delivery. There are many reasons why caesarean sections might be common in women with CAH but one worry is that earlier genital surgery might make normal labour difficult. Pain relief and the use of epidurals is the same in CAH as normal.
Gender Issues
Issues about gender often cause great concern but are not always addressed. It is important to understand that male and female gender identities are established before birth and not affected by hormone changes later in life. However, it is often why in late teenage years or later that gender preferences become obvious.
Many women with CAH have successful heterosexual relationships, though for some finding a partner is difficult. There are some women with CAH who are more comfortable with bisexual and homosexual relationships but this may in some cases, have more to do with their concerns about the appearance of their genitalia, with feelings of vulnerability and apprehension of a sexual relationship with men. The main point to remember is that if a woman has established heterosexual feelings, there is nothing about CAH that will change this, even if the testosterone levels rise from time to time.
In all women, testosterone levels can affect sex drive or libido. The effect of testosterone on libido however, is very unpredictable. Some women with CAH find that when testosterone levels are high, sex drive is increased. Reducing testosterone with higher dose of cortisol treatment can reduce excessive sex drive. A low libido is a more difficult problem as it is rarely improved by changing the dose of treatment – counselling might be an answer here.
The attitude of parents and doctors to gender and sex, and their own perceptions of this area, can influence how girls and women cope with CAH. Women manage much better if, as children and young adults, their parents and doctors were able to discuss openly and sensitively, not just the medical side but also the emotional and sexual issues. This affects how women perceive their genitalia/bodies as acceptable to others.
Those who have been brought up with no information about the condition and are unable to talk to their parents or doctors, find it difficult to over come common barriers when problems are brushed aside and sexual matters are not considered as a matter for discussion. A young adult, who will probably have had vaginal surgery will find it hard to ask for advice. She may find it very difficult to be comfortable with her body, be anxious about the prospect of sexual activity and unable to see it as something pleasurable.
Genital surgery
All girls with CAH are born with a vagina, but sometimes it is difficult to establish the position or size as these can vary considerably. The vagina may be covered with a small membrane and the clitoris is nearly always enlarged. Some girls with mild forms of CAH, do not need any surgery.
The timing of any surgery is dependent on the degree of virilisation and the feelings of the parents after discussion with their specialist as to the options available. In order to feel confident of decisions about surgery a second opinion may well be helpful. A specialist gynaecologist with experience in surgery for women with CAH is advised. Surgery to reduce the size of the clitoris, a clitoroplasty, and to make a larger vagina, a vaginoplasty, can be done at any age and opinions vary as to the best timing.
The aim of a clitoral reduction is to reduce the erectile tissue and preserve the nerves and blood vessels to the glans, the tip of the clitoris, so that it remains sensitive. Surgery required for the vagina and urethra in more complicated and varies considerably. If the vagina is positioned high near the bladder there can be the possibility of damage to the neck of the bladder, which controls the release of urine. Great care must be taken as any damage to the bladder could cause incontinence. Pelvic floor exercises can be of great help with any minor weakness with the bladder control, and will also help to strengthen the vaginal walls.
An examination just before the start of menstruation is often carried out to establish that the vagina is able to allow the use of tampons and also to make sure that the vagina will be big enough for comfortable sexual intercourse. Some specialists prefer to do this examination under a general anaesthetic, which though not strictly necessary, can make it less embarrassing for the young adult.
Some women may require surgery later in life for an enlarged clitoris or because of a small vaginal opening. It is rare for the clitoris to enlarge in adult life unless the control of CAH is very poor for a long period of time so that high testosterone levels cause the growth of the clitoris. Despite earlier surgery, the clitoris may remain relatively large in some women. In adulthood, vaginal surgery may be required to ensure comfortable sexual intercourse and to reinforce confidence in feeling feminine. Even if surgery on the vagina has taken place in childhood, it is not unusual for the opening of the vagina to be small. There are many surgical procedures using different methods to enlarge and line the vagina. ‘McIndoe’ techniques use skin grafts from the buttock or thigh to form a ‘tube’ like new vagina. Alternatively, a part of the colon can be transplanted to the vaginal space. Women needing vaginal enlargement should have information on the various techniques available and be allowed to discuss all the possibilities. For example, the two surgical techniques described above leave scars and increasingly experts feel that the less invasive use of dilators is best for women even for those who have a very short vagina. There are some surgeons who feel that vaginal surgery for an adult should only be considered if there is already an established relationship. The alternative view, often held by women themselves, is that early surgery is necessary for sufficient confidence and self esteem to establish a sexual relationship.
Surgery now has much improved over the past 30 years and the involvement of patients in decisions affecting their care has increased. Adults, who had surgery many years ago, may experience more difficulties from the effects of surgery, than those who have had more up to date operations. For instance, the practice of complete clitoral removal, clitorectomy, was once quite common. Alternatively, some erectile tissue of the clitoris might have been left secured under the skin in front of the vagina, this can be extremely painful during intercourse. Also, the glands that secrete the vaginal lubrication prior to and during intercourse might have been damaged making is necessary for a lubricant, such as KY jelly to be used for comfortable intercourse. Some women notice a build up of scar tissue after more than one operation on the vagina. This can cause lack of sensitivity within the vagina or pain during intercourse as the scar tissue is not pliable and does not stretch easily. Oestrogen cream applied to the vagina or the use of dilators can overcome some of these problems
In most women, a small vaginal opening or shortened vagina can be improved with dilators of various sizes that will allow successful sexual intercourse. By applying gentle but firm pressure to the vaginal opening by inserting a plastic dilator and, over a period of time, gradually introducing a larger size of the dilator, the size of the vagina will increase. Within months a significant increase in size can be achieved. For those women who are not sexually active, the vagina will tend to contract if dilators are not used regularly. It may be necessary to keep up a regular alternate day use of dilators for ten minutes or so. Some women feel less happy with the dilators and prefer to use their fingers. However, this method takes time, perseverance, motivation, and the ability to feel comfortable in touching the genital areas. Also, some women with CAH may have relatively small fingers; if there is a partner, their help may be of benefit until intercourse is comfortable. The use of a dildo may also be acceptable. Intercourse itself can gradually increase the size of the vagina but it takes an unusual degree of self-confidence to feel comfortable with this technique.
Some women find that everything to do with the mechanics of sex is very embarrassing. Teenagers, in particular, may find it difficult to find support and understanding if their parents find this uncomfortable territory. Extreme sensitivity is needed so that they can discuss how they can improve what they may see as an inadequacy of their body. An experienced sex therapist might be useful here. It must be remembered that women without CAH have genitalia in all shapes and sizes. The penis is a very adaptable organ which also varies greatly in size.
It may be that the surgical and psychological issues of sex make it difficult for women with CAH to achieve an orgasm. The complete removal of the clitoris, pain on intercourse, poor vaginal lubrication or lack of self-confidence may all contribute to a failure to achieve orgasm. A gynaecologist with specialist experience or sex therapist should be able to help overcome these problems.