Nutrition Low Down

So deciding to get a "tubie" for Benson was a rough decision.  Surgery is a big deal and terrifying to a three year old.  Plus, there are always risks associated with going under the knife, so we had to be sure it was something we felt was absolutely necessary.  When Benson's weight was stagnating and his younger brother's was starting to crawl higher than his, we knew it was no longer "genetics" being the only reason for his low digits.  And when your son drops off the growth chart, <3rd percentile is considered Failure To Thrive.  Ouch, that one hurts.  So enter gtube.  I think I imaged getting the tube would be the end of our feeding drama.  Before the tube, I was constantly trying new recipes, trying to find something ANYTHING!  that would load in the nutrients and Benson would love as much as candy.  He's kind of got a sweet tooth :)  But, little did I know, it was only going to get messier

 Yep.  Literally.  We left the hospital with one formula, tried a new one, then another, then switched back to the second, then decided we had to have pureed for fruits for...ahem....regularity.  And that is where we're at now.  Trying to find the right balance of what to puree and which formula.  Insurance only complicates matter since they aren't so generous with their formula offerings.  But I still haven't explained this pix.  Right now, our routine goes like this.  Benson goes to bed, I stumble into the kitchen and mix up whatever fruity goodness we have in our new Blendtec (best and most useful Christmas present to date!)  Then we use this awesome contraption to do a gravity feeding with "the thick stuff."

This was invented by my dad and brother in law.  This is just the prototype.  Don't worry, you'll hear about it when it's the real deal.  It's going to be awesome.  Anyway, after that I hook Benson up to the Kangaroo Joey pump and do a slow drip all night.  The mess is from trying to keep it dark enough that he doesn't wake up while light enough that I can see.  And, awake enough that I don't pour the mixes into his syringe like a drunk from sleep depravity.  I'm getting better at getting his mixes right so that is cutting down on time significantly.  Sometimes it has taken me over two hours to do 8 ounces!  But this last week, I think I only had two minor spills.  Progress baby!
So, why is nutrition such a big deal?  My other kids can survive with minimal nutrients.  Why is it such a big deal for Benson?  There are a myriad of reasons, all very convoluted and interwoven into the one main problem with SMA.  His muscle is literally wasting away.  How do you swallow?  Muscle.  How do you digest your food?  Muscle.  Weight bearing makes your bones stronger and how do you stand?  Muscle.  See my point?  Muscles are a big deal.  Yes, people think of SMA and think of a wheelchair.  But that is just the thing that you can see, not the reason these kids end up in the hospital fighting for their life. 
So, back to nutrition.  When you are in a fasting state, your body breaks down skeletal muscle for energy.  SMA kids don't have enough for that to happen so fasting is a no-no.  There are also some lesser-understood problems with fat metabolism.  It is most likely related to a weaker digestive tract so the intestines simply can't break the food down and get it out.  Constipation is a prevalent problem.  A lot of kids do what is called an amino acid diet which is basically a vegan diet.  But as all diets go even with healthy people, it's tricky to nail down all the bits and pieces for each individual person. 
Since getting Benson's gtube and getting him better nutrition (though it definitely still has room to improve) we have seen huge improvements in his strength.  In fact, he did a first EVER just the other day.  He was sitting on the steps of the therapy pool about chest high in the water.  He held onto the side and pulled himself to standing.  It was a perfect, beautiful moment.