AHN - CAH Issues for Females
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 now should not 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, many 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. Cyproterone acetate is the only one of these to be in common usage and it works by competing with testosterone at the hair follicle to block the stimulation of hair growth. 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.
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.
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 but this is far from certain. High levels of testosterone from the adrenal gland cause irregular periods and failure to produce an egg, ovulation. 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.
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. There is surprisingly little information about fertility in women with CAH and most of our knowledge on this subject comes from a time before modern fertility treatments. This 'historical' information says that two thirds of women with non-salt losing CAH are fertile without the need for treatment whereas only one in ten of women with salt-losing CAH are fertile. Most specialists feel that the prospects for fertility are better than these figures would indicate nowadays. If a woman with CAH has very irregular periods, then it is likely that specialist fertility treatment will be required. On the whole, fertility treatments will be the same as for women without CAH where a fertility tablet, clomiphene citrate, can be given. If tablet treatment is not successful then hormone injections (gonadotrophins, LH and FSH) can be used to bring about ovulation. If all of these treatments fail then in vitro fertilisation (IVF) might be needed but a specialist fertility clinic with experience in CAH should be consulted at this stage.
For women with late onset CAH fertility problems are usually less marked. In the mildest forms, steroid treatment might be necessary in order to conceive but it may be able to be stopped in 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. The placenta protects the baby from any hormone imbalance in the mother and destroys any excess hydrocortisone 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.
? Dr G. S. Conway 1999
This Page was updated 21 May 2018
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