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Treatment of CAH
People
with CAH have a normal life expectancy and for most people there is very little
interference in every day life if the condition is well managed.
Adults with CAH require life long follow up in specialist hospital
clinics in order to maintain the correct level of medical care and to provide
early advice on fertility when needed. It
is advisable to have regular medical checks even if everything is well
controlled. For women, checks
should be made 2 - 3 times per year. For
men, once per year is usually sufficient. The main aim of treatment is to
maintain normal cortisol levels and control of salt loss, in those who are salt
losers. In women, good management
of CAH brings about control of testosterone levels, regular periods and improved
fertility. In men, good management
can reduce an aggressive behaviour and control excessive libido.
Also, the sperm count can fall if CAH is not well treated and an increase
in the dose of steroid treatment for some months can restore fertility in men. Replacement of cortisol
The
object of cortisol treatment in CAH is to replace inadequate production of
cortisol by the adrenal glands allowing the body to recognise normal levels of
cortisol in the body, which reduces the need for the adrenal gland to produce
excessive amounts of testosterone. How
do doctors work out how much cortisol to take? If
the cortisol dosage is too low the adrenal gland will try and make more, but
only succeed in making more testosterone. The
effect of these excess androgens is growth of body hair in women and infertility
in both sexes. In
severe forms of CAH, too little cortisol treatment may make the body unable to
cope with stress and infection, so that
major surgery or an illness like ‘flu’ can be life threatening.
If the dose of cortisol treatment is too low, a blood test will establish
this by showing high levels of several adrenal hormones of which the most
commonly measured are:
Other side effects of steroid treatment are:
There
are four types of cortisol replacement treatment: Hydrocortisone, Cortisone
acetete (now rarely available in the UK), Prednisolone and Dexamethasone.
They vary in their dose and duration of action.
Hydrocortisone is another name for cortisol.
Prednisolone is 5 times more potent, and dexamethasone is 40 times more
potent than cortisol. Both
prednisolone and dexamethasone are comparatively long acting, where as cortisone
acetate and hydrocortisone are shorter acting, and need to be taken 2 to 3 times
a day. The dose in each
dexamethasone tablet is not convenient for fine-tuning of treatment leading to a
danger of taking too high a dose. Dexamethasone
is established for use in pregnancy for women who are at risk of having a child
with CAH. Hydrocortisone is also
used as an injection at times of adrenal crisis or when vomiting prevents the
tablets from being taken. Variety
of steroid tablets and examples of the range normal daily doses.
The
exact dose needed each day for each individual will differ, due to the variation
in body size, different rates of absorption in the bowel. It is important to assess the individual response, taking
into account timing of the day that medication is taken. It is important to be clear how many tablets are missed so
that the correct balance of dosage can be achieved.
Many people find it difficult to take tablets regularly and this can lead
to conflict with doctors. At
times of stress such as during illness or physical or emotional shock, the dose
of cortisol replacement treatment should be increased in a controlled way.
The exact regime should be agreed in prior discussion with you doctor.
For example, a double or treble dose is usually advised at times of
stress. The body increases cortisol
in situations of high mental stress and it may therefore be necessary to give an
increased dose even if only for one day for exams, a driving test, or a sudden
bereavement for example. If an
illness causes prolonged vomiting which prevents tablets from being taken, then
an injection of hydrocortisone 100 mg should be given into the muscle and a
doctor should be called. The
technique of injection can be learnt by members of the family with instruction
from the practice nurse. Unless
there has been substantial over dosing of fludrocortisone, blood pressure should
not present a problem taking into account the wide variation in general blood
pressure levels. Replacement of aldosterone
All
individuals with salt losing CAH need to replace the lack of aldosterone.
Only one form of treatment is used here.
Fludrocortisone is given in a dose of 50 – 300 micrograms.
Too little fludrocortisone can cause low blood pressure and dizziness.
Too much fludrocortisone can cause high blood pressure and headaches.
The correct level of fludrocortisone is determined by measuring blood
pressure, potassium and the salt sensitive hormone renin
in the blood every few years. © Dr G. S. Conway 1999 |
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