CAH
Accident, Sickness & Increasing Steroids in Congenital Adrenal Hyperplasia
Dr Paul Hofman, Pediatric Endocrinologist, Starship Hospital. I have given some general guidelines but as with all medical issues, different physicians have their own way of approaching things. The key is to get medical advice when in doubt, as inadequate steroid cover can result in adrenal crisis and prolonged excess can also have serious consequences.
Increased steroids are needed after significant trauma. Injuries such as a significant laceration, concussion or a broken bone require double or treble steroid doses. The next dose should be given straight away! This is needed for a day or so, depending on the nature of the injury. Where injuries are severe intra-muscular (IM) or intravenous (IV) hydrocortisone is required.
When giving increased steroids, I like to give four or more doses a day. In hospital I treat severe infections with four hourly hydrocortisone. At home (especially if prednisone is used at night) I would make sure stress-dose steroids are spread over at least 3-4 doses in a 24-hour period. The length of time needed on increased steroids varies according to the severity of the illness. Your physicians advice is needed.
Vomiting cannot be ignored. The patient needs more hydrocortisone! If it cannot be taken by mouth it will need to be given by injection (IM or IV). Without extra medication an adrenal crisis can easily occur! (Note: this is not the case for people with milder forms of CAH.)
Generally, after the first vomit, I suggest making two attempts to give the medication. If the patient vomits after both attempts it is vital to have steroids by injection. It takes about 1 hour for medicine to reach the small intestine where it is absorbed so if there is less than 1 hour between giving medication and the second vomit, then IM or IV hydrocortisone should be given.
For example, if a child's usual daily dose was 10mg hydrocortisone (morning), 2.5mg (noon) and 10mg (evening) this would give a total daily dose of 22.5mg. Tripling this for sickness would give a total dose of 66.5mg and dividing this into four equal doses would be 17.8mg. Therefore, we would give the child 15-20mg of hydrocortisone every six hours.
For brief periods of sickness - such as a "24-hour bug" - this six hour spreading of doses is not so critical. It is however, very important to do this for significant prolonged sickness. Again, if in doubt, contact your physician.
Diarrhoea is easier to deal with. If the patient is able to take tablets (i.e: not vomiting) I suggest increasing the dose according to how sick the child is. Trebling the medication for mild diarrhoea is entirely okay. If there is profuse ongoing diarrhoea, bloody diarrhoea, incapacitating abdominal pain or diarrhoea with fever it would be appropriate to increase the dose by 5 times. Diarrhoea is usually caused by viral infection but sometimes by more dangerous problems like appendicitis. It is important to make sure that adequate fluid is given to prevent dehydration. If a child is close to adrenal crisis they may well need IV fluids. If there is ANY doubt whatsoever seek medical advice!
Dr Margaret Zacharin made the comment that during periods of illness it was important for children to have a sweet drink prior to going to sleep.
She also suggested that the child be woken for another sweet drink through the night. These measures are important during periods of sickness because of the tendency for blood sugars to fall overnight (i.e. hypoglycemia).
Advice when visiting a new or different Doctor in an Emergency:
When taking children to a new Doctor for emergency care parents are advised to refer to the condition by it's full name rather than "CAH." This is because it could well be confused with another condition which shares the same initials such as Chronic Active Hepatitis. Use key descriptions such as adrenal crisis, vomiting and diarrhoea etc. Make sure to mention if your child is salt wasting CAH. Always have a letter or information handy so that this can be handed to a new Doctor dealing with your child which is pertinent to the care needed when sick such as the above.