Sorry for the delay in this…it wasn’t my intention!  However, I do have to share with you that we had a wonderful trip up north this weekend to visit the Polar Express!  This was our 4th year doing it and we had a wonderful time!  The kids got to play in the snow, they saw Santa, played with their friends, and overall, a fun time was had by all!!  And you know how it is, after a trip comes all the fun that unpacking can bring…laundry, groceries, tidying up again,  and a wishful early bedtime for me!

In my last post, I described to you what the meaning of ONH meant.  It’s more than Emma not being able to see well.  Of course we didn’t know that during her diagnosis at 4 months old!  But boy did we learn!  If you need a refresher about how Emma got diagnosed with SOD, read this.  Oh, and just a refresher, SOD is the acronym for Septo Optic Dysplasia.  In this post, I’ll tell you what exactly SOD means for Emma.  The interesting this about SOD, is that there’s no such thing as a textbook case.  I haven’t found two children that present the exact same symptoms that SOD can bring.  It’s a HUGE spectrum disorder, just like autism.

In my opinion, Emma’s case of SOD is pretty mild.  I had a medical professional disagree with me on that, once.  She said that there are no varying degrees of SOD.  I beg to differ, and I’ll tell you why.  First of all, there are 3 components of SOD, and you need to have 2 of them to be diagnosed with it.  The three components are hormone deficiency, Optic Nerve Hypoplasia, absence or malformation of the septum pellucidum and/or corpus callosum.  What?  Foreign language?  Click on the terms to get the wikipedia definition.  Or I’ll give you my very short answer.  Both of those (SP and CC) divide the brain into their hemispheres.  If I’m correct, there’s a bit of a debate as to how much they really contribute to a person.  One helps with communication between hemispheres (SP, I believe).  The other one helps with emotions.  I don’t know much about the corpus callosum or the septum pellucidum because Emma’s are both in tact.  However, she obviously has the ONH and she also has  hormone deficiency.

The hormone deficiency is a big deal.  She doesn’t produce hormones!  Now, it’s a little embarrassing to admit to this, but I don’t know exactly how many hormones a child her age produces.  I just know the ones she doesn’t produce.  Shouldn’t I find out the answer to that?!?  Anyway, she doesn’t produce thyroid, growth hormone, and cortisol.  Of those 2, the growth hormone and cortisol are the most important.  The growth hormone is a shot that we give her every night.  Our body naturally produces GH and releases it at night time.  The interesting thing is, GH doesn’t just help with lateral growth.  It helps all of our organs function!  So when people ask if she’ll stop taking it as an adult, the answer is NO.  She’ll need it for life (or, like Brett says, “until medical science catches up”).  Does she mind the shot?  Not now.  In fact when we tell her we’re getting it ready, she’ll pull her sleeve up and have her twiggy little arm waiting for us! Of course it wasn’t always like this.  It took quite a few months for her to not scream every time we came near her with it.  I can’t say I blame her!  Personally, I couldn’t get myself to give it to her for about 2 months after her diagnosis.  Just couldn’t do it.  But now that she’s on it, she’s about 50th percentile for height and about 35th for weight.  Perfect.  (It should be mentioned that before her diagnosis, she was falling off the growth charts)  Now for the cortisol.  For anyone who doesn’t know, cortisol is a hormone that our body automatically produces more of when we’re stressed, injured, or sick.  It’s our “stress hormone”.  Our body is always producing it, which is why she gets it 3 times a day…breakfast, lunch, and dinner.  The critical part of her not producing it is when she gets stressed, injured, or sick.  Our bodies produce more of it naturally…hers does not.  So, if she’s sick with a stomach bug, and can’t keep anything down, it isn’t possible for us to give her a “stress dose” (which is 3X her normal amount of medicine).  It’s imperative that we make the right decision: do we give her solu-cortef (which is her emergency cortisol that is injected into the muscle with this HUGE needle) or do we take her to the ER.  Fortunately for us, we’ve never had to give her the solu-cortef shot.  We’ve always gone to the ER where they pump her full of fluids and extra cortef (the brand name) if need be.  We are supposed to give her the solu-cortef when she goes into adrenal crisis, basically when her organs start shutting down.  There are signs to look for…fortunately we have never gotten to that point.  That’s the scariest part: the fear of adrenal crisis.  It can happen quickly and without warning.  We carry around the solu-cortef everywhere we go.  We also carry a blood sugar monitor to test her blood sugar, as low blood sugar can be a sign of adrenal crisis.  Remember when I took her to the ER that first time?  Her blood sugar was 11.  It’s supposed to be anywhere from 80′s-110′s.  You could say she was in adrenal crisis then.  We just didn’t know it.

Did I mention that 80% of kids with ONH also have hormone deficiency?  80%. Are you wondering why Emma’s opthalmologists didn’t share that with us when she got diagnosed?  Us too.  That would’ve saved us a lot of stress, heartache, and turmoil, and all that for Emma as well.  That is why I feel it is so important to get the word out about ONH and SOD.  Here’s another little tidbit of information.  ONH is the leading cause of blindness in children.  Ponder that for a bit!

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