Hi Chris,

Was it the King of Siam who used the phrase “Is a puzzlement!”?  I often think of that when I’m reflecting on our experiences with CAH. 

My older daughter Julia has had a fairly easy time of it, with small fluctuations in meds mostly attributable to the switch from suspension to pill form and normal growth adjustments.  She has had periods of over-suppression, but has never had significant swelling or weight gain.  Her only symptom of over-suppression has been a deceleration in linear growth.  When she has escaped control, it’s been gradually and predictably.  A small adjustment in meds has been all it has taken to get her levels back where we want them.

Claire has been another story altogether.  She is all over the place.  We knew from labs in February that she was headed for escape, but her numbers were still safe (and she is not a salt waster) so we left her dosage alone.  In April her 17-OHP was 4000 ng/dl, which required immediate action, of course.  We went to 3x a day dosing and the smallest possible dose increase (1.25 mg).  A month later her 17-OHP was 28 ng/dl.  See what I mean?  To be fair, we did have a period of lovely control after we finally broke out of the over-suppression 18 months ago. 

Here is my wisdom (and it applies only to my child, but you can peek).  When she escapes control, we double her dose for 3 days before beginning the new slightly higher dose.  Our endo calls this “stomping” on the adrenals to calm them back down.  She says they don’t always respond well to sneaking up on them, and our experience has proven this to be true.  I think of it as a reset button.  Similarly, it worked for us to do a fairly drastic cut when she couldn’t break out of over-suppression (a kind of reverse stomping?).  It is so natural to want to respond to high labs with a big dose increase, but we have learned that this will surely lead to the up, down cycle you mentioned.  A very small increase (after stomping) has been just the right thing to do for Claire when she escapes control, with follow-up testing in 4-6 weeks.

Jack is so young that he will need frequent dose adjustments, I suspect.  How often do you test?  What dosing schedule do you use?  I have come to think of optimal treatment as a moving target.  You take your best shot with the information presently at hand while keeping an eye to your child’s history.  Our first priority has to be our children’s safety, but I am more and more concerned with protecting optimal growth.  Long term over-suppression will not result in optimal adult height, and that will be a factor in the quality of their lives for years to come.  I’ve become a nit-picker!  Our endo is great, and welcomes my incessant questions. 

I wish I had the magic answer.  We just keep trying.

Laura K

P.S.  Claire’s bone age is not advanced – in fact it is two years younger than her chronological age due to the periods of over-suppression.  This leaves us a little wiggle room to let her escape control without worrying about worsening an already advanced bone age.