Coming Up Short

My children have always been small.  Their weights at birth (13 weeks early) were 2-6, 2-3, 1-13, and 1-11, and they didn't exactly hit the genetics jackpot for height.   As they've grown, the identical twins have really lagged behind the other two.  I always thought it was a result of their poor eating habits and put a lot of pressure on myself to try and get them to eat.  However, their appetites were almost non existent and you just can't force feed a kid :o(  Their last trip to the pediatrician triggered a referral for all three boys to an endocrinologist because of their lack of growth.  Amanda has always been a champion eater and although she is also small, she is at least scraping the bottom of the charts.  Unfortunately, the boys growth curve hasn't been much of a curve at all, rather a flatline. Jaxon height wasn't as much of a concern but he is still not growing at an average rate.  All three boys were sent for initial blood tests.  The twins tests showed problems that needed further examination so they were admitted to the Rapid Treatment Unit at a local hospital for a six hour test.  Jaxon's growth factor levels were low, but not low enough to be alarming so he squeaked out of the big test...

Mason

Both boys were given blood pressure drugs that made them very sleepy.  This simulates what happens at night when children sleep which is the time their bodies make growth hormone.  Blood was drawn every half hour for four hours.  The boys slept through most of the test.

Carson

Jaxon and Amanda came along as "support."

Even Daddy took the day off work for the boys!

We waited a week for the test results.  I wasn't quite sure how I wanted them to come back because if they were hormone deficient, it meant expensive shots twice a day until puberty.  But, if it came back normal, we would be back to the same grind of trying to force feed kids that were't hungry and trying to get calories in them any way we could.  When the call finally came in, the twins were diagnosed with Severe Primary Insulin Like Growth Factor 1 deficiency, or IGF-1 for short, (no pun intended.)   It's a really long way of saying that they make enough  actual growth hormone, but it does not metabolize into a growth factor that is primarily responsible for bone length.   Bingo!  We finally know that it isn't our fault that the boys are so small.  A relief and yet stressful all at the same time.  It's a little overwhelming to find out that your kids will need shots for the next 7-8 years, I have no idea how those who have diabetic children handle it.  My heart goes out to them.

A couple of weeks later, after the treatment was approved by our insurance, our packages arrived...

One huge box for each boy containing a total of six vials of Increlex, 200 needles, two sharps containers, and two boxes of alcohol wipes.  We were ready to go!

It was kind of like Christmas when you get a gift that you really don"t want.  I almost hyperventilated because the specialty pharmacy sent the medication in two separate boxes instead of consolidating into one.  I really HATE waste and that just seemed silly.  Seriously, did you see the size of those boxes?

In an attempt to not make this story any longer, I'll just say that the twins have adjusted better than I had expected to their shots.  The medication is working well.  Both boys have grown considerably in the last six weeks and they finally have appetites now that their bodies are growing and calling for calories.  We are still watching Jaxon as his growth is poor but his blood levels of IGF-1 are not low enough to qualify for treatment.  It's quite expensive ($800-$1000 per vial) so insurance has very strict requirements that patients have to meet before qualifying for treatment.  The dosage is weight based so as they get bigger, the dose goes up.  Mason has already gone from needing 3 vials per month to needing 4.  I'm grateful for the weight gain but the cost is always in the back of my mind.  I would be sick if our kids medical costs caused our insurance to not renew with our entire company.

Mason taught himself to do his own shots last week and has since taken over the responsibility of giving Carson his shots as well.  He is very proud of himself.  They understand that this is a long term treatment that they will likely be on until 17 years old or later.  Once their growth plates close, the drug will no longer work.  The growth plates will be monitored with yearly x-rays.

I just have to keep reminding myself...One day at a time.