Hi Jim,

Well this is one of my favorite things to talk about. I was really, really stuck on labels in the beginning. I wanted to distinguish SW from SV because I wanted what my son had to be less severe.  He ended up being rediagnosed as SV. Unfortunately it is hard to tell where in the CAH spectrum your child lies until some time has gone by and you get a feel for how he reacts under different circumstances.  Just because he is diagnosed as a SV doesn’t mean he will act like one. The spectrum can go from a SV acting like a SW or a SW acting like SV. Each CAH person is different.

 As far as a crisis, there are essentially two types of crisis. The first being the salt wasting crisis and the second being the adrenal crisis. The SW will go into a SWcrisis rather quickly and proceed to a full adrenal crisis.  Most SV will not have a SW crisis but they can have an adrenal crisis if their body is stressed and a stress does is not available.   You  just have to wait and see though how your child reacts under stress. Not that we want to do trial and error on our child to see how they respond but there will be times such as when they are sick or in accidents that you will see how quickly things go from bad to worse.  All Classical CAH  patients have a tendency to waste salt but the degree of that loss is different from person to person.  Again, that is why they say that even if genetically you are a SV, your phenotype (the type you show) can be that of a SW.  In time you will be able to judge how your child does. For example, my little guy so far has done great. He started his medication at 30 days old and has never (knock on wood) had a problem to this day. In fact my son’s 17ohp levels looked  very similar to your sons.  My son never had problems with weight or with sleeping. He is now 19 months old and just as busy as his two older brothers were at this age. He thinks he is just as old as his older brothers because he jumps right in for a wrestling match. He has been through a few high fevers (double dosed), one ear infection, bronchitis and a burn on his arm (no stress dose per endo) and did great! He has never had a crisis or even come close to one.  I tend to think that my son will not be as prone to crisis as maybe other SV’s who are more like SW.  In time, the picture of how your son reacts in a crisis will become clearer. Until then, be prepared with the solucortef, extra doses of cortef and most of all become very very familiar with all the in’s and out’s of CAH so you can ask questions and stay ontop of your son’s care. 

Good luck,

Sandra

P.S. I don’t konw if this makes you feel better but there are SV CAH people out there who have never had a crisis or needed a solucortef shot. Sometimes you will read about people that have been given a shot but sometimes it was not really a crisis or not necessarily a necessity to give a shot. The best thing to do is to see how your child does. You can’t compare too much...