So you have discovered that your child has CAH and you know a little about it........ what now ?
Below are a list of questions which offer you a basic understanding of how this will effect your lives. For more information, go to the Links Page where you will find other sites and books which will help.
Will my child grow normally and reach a reasonable adult height ?
With careful treatment it should be possible for children with CAH to grow at a rate sufficient to achieve an adult height within the normal range. However, even with the very best treatment, it is not always possible to achieve perfect growth in CAH, and some individuals may end up shorter adults than they would otherwise have been. Also, the dose of hydrocortisone required to control the CAH may make it difficult for some children to keep their weight down, and this can be a particular problem for girls at adolescence. It may be helpful to have the input of a dietician - you should discuss this with your specialist.
Can my child receive live vaccinations despite being on steroids ?
Yes, the dose of steroids used in CAH are equivalent to the amount produced by the adrenal glands under normal conditions, and there is NO reason for your child to be managed differently where vaccinations are concerned. The situation is quite different for people who are receiving larger doses of continuous steroids for different medical reasons. It is suggested that you consult your specialist about whether it is necessary to increase your childs hydrocortisone prior to vaccination.
Should I be giving hydrocortisone and fludrocortisone to my baby as a suspension?
Both hydrocortisone and fludrocotisone are relatively unstable in suspension and this medication would have to be constituted by your local pharmacy every couple of days. It is much more effective to use tablets which can be crushed up if necessary. The practice in the UK is different to that in the US where the use of suspensions is more commonplace.
Will my daughter need to have corrective surgery? When? And to what extent?
The nature of surgery required depends upon the degree of masculinisation. Usually the surgeon will need to reduce the size of the clitoris very carefully, preserving the delicate supply of nerves and blood vessels to the tip so that normal sexual relationships can be experienced in the future. Also the surgeon may try to open the entrance to the vagina. The length of stay in hospital varies, but is between 5 and 10 days.
Surgical treatment of CAH is usually carried out when the child is about one year of age, by which time she is big enough to be operated on safely, but not old enough to have become embarrassed about the appearance of her genitalia.
Sometimes it is not possible to open the vagina completely at the first operation and in some girls a future operation may be needed, optimally at the time of puberty and certainly well before they want to start a sexual relationship. Once puberty has started, therefore, it is recommended that such girls are referred either back to the original surgeon, or to a gynaecologist, in order to reassess whether any further surgery is going to be needed. When surgery is needed in young adulthood, patient and parent support is advisable. This should be initiated long before surgery and should be through a psychologist with experience in this area.
My daughter is 16, she stopped growing at 14 and has a weight problem. She has always been on twice a day medication. Would it be appropriate to change now to three times a day to try to get more growth ?
If she has stopped growing and the bones in her body have fused, there is nothing you can do to get more growth. If she is on longer-acting steroids (prednisolone) then twice a day is sufficient. If she is very overweight, she would probably benefit from proper hormone re-evaluation and, although you can do nothing about growth, you should now concentrate on adequate control to permit fertility for the future.
My 6 year old still wets the bed. Could it be related to CAH ?
A lot of children wet the bed and this simply reflects normal delay in bladder maturation. However, in CAH bed wetting should always raise the question of under-replacement with fludrocortisone. In this instance we make quite sure that the fludrocortisone is sufficient (checking that the plasma renin is properly suppressed). If a child with renin suppression on a good dose of fludrocortisone, with no symptoms of salt craving, but is still wetting the bed, then it is safe to conclude that he/she is showing 'normal' bed wetting tendency.
My 7 year old never old never has blood tests. He is weighed and measured, but his consultant will not give blood tests, do you feel this is acceptable ?
At the end of the day the most important measurements of the condition are that the child is healthy and well, they are growing along a percentile which is appropriate for that child and that the bone age is satisfactory. Monitoring varies from centre to centre and child to child depending on the severity of the CAH, but do be sure to check that if blood or urine tests are not being performed, that growth and bone age are appropriate for your child's age.
I've heard that the blood sugar levels go down in acute illness, do sugar drinks help ?
One of the responses to acute illness is that the blood sugars drop and cortical levels go up to combat this. If you measure glucose levels in children with CAH who are drowsy, you often find they are low and that is when an injection of hydrocortisone should be given without delay. Injecting promptly in this situation lessens the severity and shortens the period of illness.
How ill does a child have to be before increasing their dosage or giving an injection ?
In the event of a mild to moderate illness (e.g. cold, cough, sore throat, tummy upset), the total daily dose of hydrocortisone should be doubled and given in 3 equal portions (morning, afternoon, evening) for the duration of the illness. If the child does not get better after increasing the tablets orally (due to continued vomiting), the hydrocortisone must be given by injection and the child taken to hospital.
What is renin ?
Renin is a hormone made by the kidneys and is important in control of salt balance. If salt is replacement is inadequate renin is high. If salt replacement is adequate renin is low. Therefore, by measuring renin, the correct dose of fludrocortisone can be established.
How often should a renin test be done
About once a year, if the child is growing well and they have normal blood pressure and electrolytes. There are no hard and fast rules. If the child's metabolic control did not appear satisfactory, then it may need to be done more often.
How often should a child have a bone age X-ray ?
Ideally on an annual basis after the age of three years. Prior to this there are so few epiphiseal centres to rate and it is difficult to ensure that you are getting the right answer.
What is the right age to tell a child about their condition and who should tell them ?
This was a question asked during a recent survey, the overwhelming response was the parents and not the doctor and more often than not the mother. Predictably perhaps they find it more difficult to talk to their daughters than their sons. Children develop at different rates and there is no magic age. However their questions should always be answered. Sometimes they pick up half the stories and get the whole thing wrong. They will only retain what they have the ability to understand but it is important to be open an honest with then . You will have to tell them more than once.
These were sourced from the CAH Booklet Series No. 6 & CAH Family Conference 1997
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