Hi Chris,

Jack does sound a lot like Claire.  You are very fortunate to be keeping him on the 50% percentile for height given the period of over-suppression.  The 3.9mg/m2/day sounds very low, I know, but it isn’t all that strange.  Claire did well on 4.5mg/m2/day for several months after the big decrease, and she is currently doing well on 5.5 mg/m2/day.  Jack must be making a lot of the cortisol his body needs on his own.  This is a good thing!

You mention that Jack’s 17-OHP level jumped to 551 ng/dl, and this was considered an escape from control.  Given Claire’s history and her lack of bone age advancement, we would be fairly comfortable with this level.  There is a lot of disagreement on this point, so you need to go with what works for Jack.  For Claire, we consider the 200-600 range ideal.  My older daughter Julia had advanced bone age at diagnosis, so we’ve kept her numbers under tighter control to prevent further bone age advancement.

From our experience, a 17-OHP<5 ng/dl is horribly over-suppressed.  Claire would be wider than she is tall at that level, and she wouldn’t grow at all.  Reducing his dose until he escaped control was exactly the right thing to do.  What was his 17-OHP after the first post-551 increase?

Your current dosing schedule sounds great.  If you need to add another ¼ pill, I would add it to the nighttime dose.  Isn’t it frustrating that there isn’t a lower dose pill for our little ones?

Laura K