So, what's the going rate these days for the tooth fairy? Does she take check cards? Can I get a receipt? Is it tax deductible?Surely, SURELY I don't have a kid who is old enough to have a loose tooth! (Yes you do, and stop calling me Shirley.) Miss Abidabs has her first loose tooth, and I think I have enough time to convince her that the tooth fairy gives a big honkin' nickle for each tooth.
My niece, Hailey, is being admitted to UK Children's next Monday (the 13th) for IV fluids and some pre-op procedures. She will have a g-tube placed on Wednesday and then within a few days of this admission, she will be readmitted for heart surgery. Please pray for Hailey...and Ashley and John! I'll keep the blog updated when I know more information.We received more results from Nathan's muscle biopsy yesterday, and it made me realize how amazing the human body is! Mitochondria are in every cell in your body (except red blood cells). And inside these mitochondria is an electron transport chain (ETC...also called the respiratory chain) that creates 90% of the energy your body needs to operate correctly. The ETC consists of 5 complexes that work with one another to create energy. This much, I've kind of had somewhat of a tiny grasp of understanding...but then you have supercomplexes within the complexes, and that is about the point where my head explodes and refuses to let in anymore confusing information.
Anyway, after I was reading a little about the supercomplexes within the complexes within the mitochondria within the cells within the body last night, I was just in awe of this one minute detail of God's creation. Wow. So complex! So amazing! And then I realized that not only did God create such incredible details in each of us, but he also created people who actually understand a little about how these things are supposed to work and how to test and research them. How thankful I am that He opened up the door for us to meet someone who actually understands all of this.So, the additional testing revealed that Nathan has decreased supercomplex formation and decreased levels in the monomeric form of Complex III, whatever in the world any of that means. All we can understand is that this does indeed confirm the fact that his defect lies in Complex I and Complex III. They are currently running genetic testing to see if they can find the genetic mutation, but only 10-15% of patients with this disease find their mutation...obviously, we aren't holding our breath on these results.
And for more medical news (I hope that we are almost finished with medical updates for a while...I think I can see the light at the end of the "endless appts" tunnel), Nate had an appt with our hepatologist today...essentially, he is a GI doc who specializes in liver diseases. Oh, he was so sweet and informative. I have to admit that we have some amazing doctors at Shands!! This doc said that based on Nathan's prolonged jaundice as a newborn (the little dude had jaundice for 2 months) and the fact that he has a hard time making haptoglobin periodically seems to be from defective mitochondria in his liver cells...just like we (and 2 other docs) suspected. We will keep an eye on his liver function labs each time blood work is taken.He also wants another ultrasound of Nathan's liver done this summer to make sure there are no fatty infiltrates and to take a good look at the vessels to and from the liver. He said that he doesn't usually see problems with the vessels in mitochondrial patients, but he wants to make sure everything looks fine anyway. I was extremely impressed in his knowledge of mitochondrial disease! Most of our docs have only heard of it a few times or seen a few patients with it in their lifetime, but this doc obviously does tons of research and was very well aware of how mito can affect the liver before he stepped foot into the exam room. I can not tell you how incredible it feels to have doctors periodically who actually seem to understand this rare disease.The one thing that did stump him (and has stumped every other doctor we have met) is Nathan's elevated Alpha Fetoprotein (which is given off by the liver). He said that this is definitely not normal and that he was going to do more research on this in relation to Complex I and III defects. But he did say that he is almost positive that it is elevated due to mito. This doc wants to keep an eye on Nate and his liver function tests, so we are going to see him again in 6 months.Okay, in order to keep this blog post from putting you completely to sleep, I leave you with Emma's prayer from dinner last night...
"Dear Heavenly Father, I hope that Daddy and Mama and Abby and Nate all be sweet to me. Amen. LET'S EAT!!!!!"