Re: To Sandra....Simple Virilizing, Salt-Wasting, and Sliding Scales
12/14/01 9:24 AM

Normally, I would say, go have fun, don't let this stop you from travelling, but in this case, I completely agree with Val.  I am also a bit concerned.....low total dose, 2X/day, no florinef (for a child who appears to be borderline salt-wasting), now with an ear infection....the combination makes me a bit nervous, and I am not normally the nervous type, when it comes to routine illnesses. 

Sandra, I know that having Jackson be labelled a Simple-Virilizer, rather than a Salt-Waster, was very important to you, but I'd like to reiterate that the label makes very little difference when it comes to treatment.  It is not uncommon for Simple Virilizers to need florinef, and to receive the exact same treatment (medication wise) as Salt-wasters.  In fact, I would go so far as to say most Simple-Virilizers probably DO take florinef.  Whatever you want to call him, if a child's renin level is elevated, the accepted thing to do is to prescribe florinef. 

There was  a thread a while ago about being "borderline," and a few of the comments had to do with the fact that ALL CAH patients are somewhat salt-wasting.  Well, this is true and the reason why doctors say this is because ALL Cah patients cannot produce a certain enzyme.  If you have a 21-Hydroxylase deficiency, it means you can't produce adequate amounts of the enzyme 21-Hydroxylase (21-OH).  21-OH is necessary in order to make BOTH aldosterone and cortisol, therefore ALL 21-OH Cah patients cannot make aldosterone and cortisol TO SOME DEGREE. 

The treatment that you receive, and the classification that you fall under, has to do with the DEGREE to which you are unable to produce aldosterone and cortisol.  If you basically don't produce ANY of the 21-OH enzyme, then you will make little to no aldosterone and cortisol, which will make you a salt-waster.  The more aldosterone and cortisol that you make, the farther away from salt-wasting you will be, all the way to non-classic patients, who can normally make enough aldosterone and cortisol to get by, in order to avert crises. 

The point is that salt-wasting is A SLIDING SCALE, which is somewhat artificially divided into 3 different sections, with everyone over on the left called a Salt-Waster; everyone on the right,  Non-Classic; and everyone in the middle, a Simple-Virilizer.  It is probably not too difficult to determine what to do with those who are way over on the left, or way over on the right, but it is a bit more difficult to figure out what to do with those who are in the middle.  Some, who are in the middle, will probably make enough aldosterone and cortisol where their treatment will follow the protocol of a non-classic patient.  Others, who are in the middle, will make so little that they are lumped in with the salt-wasters.  As I said, the determining factor, in deciding who follows what protocol, is usually an elevated renin number. 

As a SV, Jackson is one of those people who are in the middle, but what concerns me is that he is obviously to the LEFT side of middle---enough to have his first endo out-and-out call him a Salt-Waster.  And he is to the LEFT of middle enough where even his current endo (though she acknowledges that he "officially" falls into the Simple-Virilizing band of the spectrum) agrees that he needs to be treated with florinef (as are many other Simple-Virilizers.) 

The label matters little---the biochemical indicators (what his electrolytes, aldosterone, renin numbers say) are much more important, when you are determining treatment.  IF, after looking at the biochemical indicators, Jackson's current endo is concerned enough that she is suggesting RESTARTING him on florinef (even after he was just purposefully taken off by Dr. New), then I think it is important to find out WHY.  She is seeing something (as did the first endo) that is raising a red-flag (and for good reason, BORDER-LINE salt-wasting numbers).  I think it would be wise to get to the bottom of this, especially with a sick child, prior to starting a long-distance trip.

One other thing: I seem to remember you saying that you had taken Jackson off of sodium supplementation after he had been taken off florinef.  IF it is determined that he really needs to restart the florinef, make sure you ask his endo about restarting sodium, as well.  Apparently, sodium is needed for florinef to be effective, so if a child is taking florinef, but with inadequate sodium, the florinef will not do any good, and crises can still occur. 

Please let us know how you make out. 

Carol
Rare Disease Search Engine, Homeschool Sites, Online Homeschool, Online Income, Ethical Adsense, Creative writing, Family Web Hosting, Christian Radio, Tulsa Parks