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:

  • 17 Hydroxyprogesterone

  • Androstenedione

  • Testosterone  


Figure 3

 

Other side effects of steroid treatment are:
  • Headaches (sometimes associated with taking prednisolone and dexamethasone)

  • Feeling sick / nausea / tiredness

  • Finding it difficult to cope with stress

  • Water retention (sometimes associated with taking Hydrocortisone and Cortisone acetate)

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.

Type of steroid

Tablet sizes

Dose range for adults

Hydrocortisone

10 mg, 20 mg

10 – 40 mg

Prednisolone

No coating: 1 mg, 5 mg

Enteric coating: 2.5 mg, 5 mg

2.5 – 10 mg

Dexamethasone

0.5 mg, 2 mg

0.1 – 0.75 mg

 

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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