Noah 101

(This is copied directly from Facebook for those who prefer to read it here.)

A lot of people who've been praying for Noah are very new to him and his story.  We've gotten a lot of sweet suggestions as well as a lot of questions, so here's the "Reader's Digest" version of Noah's underlying condition and what it means for him.  This is by NO means exhaustive and is just very abbreviated run down.

Noah's primary diagnosis is mitochondrial encephalomyopathy.  We all have mitochondria in our cells and they are responsible for creating energy for cells (and therefore our body systems) to function.  Noah has a genetic defect in his mitochondria.  It was present at conception and cannot be fixed or reversed.  Because his mitochondria don't function correctly, his body is like a city without enough power for the grid.  On a good day, just living and growing takes a toll on his body because of that power deficit, and if he is sick (or even too tired or too hot or too cold), the additional power diverted to cover the crisis means there will be a power outage somewhere else.

Noah hasn't had enough power to run his GI system since he was a baby, so he has a central line (large, semi-permanent IV access surgically implanted in his chest) called a Broviac.  He receives all of his nutrition through that line in the form of TPN.  The TPN provides all of his calories, protein, carbs, vitamins, etc. directly into his blood stream.  While he could not live without TPN, it also puts him at serious risk for sepsis and for liver failure.

A year ago his GI system shut down completely as the result of a simple upper respiratory virus. While the respiratory system and GI system could be considered unrelated, the power drain of the virus diverted power from his gut and shut it down.  This is probably THE VERY hardest thing for people to understand - Noah cannot eat.  Not at all.  He cannot get ANY nutrition through his gut (stomach, intestines, etc.)  He cannot get ANY medicine through his gut.  We would love to give him nutritional supplements, but we cannot.  He has no use of his gut at all.  His intestines are literally falling apart inside him.  They ooze blood continually and blood, bile, and gastric juices back up into his stomach and drain out of a surgically implanted drain in his stomach that drains into a bag.  If he takes a sip of a drink, or a bite of something "liquid" like ice cream, it immediately. instantly drains out into that bag.  If he is thirsty, he could drink a gallon and never quench his thirst because it all drains out of his stomach into that bag instantly.

Noah gets a lot of IV medicine for pain (migraines, nerve pain, and global, all-over pain).  He also gets IV medicine to control nausea/retching, gastric acid, force his kidneys to make urine, and much more.  He is affected in other body systems.  His bladder no longer works, so he has a supra-pubic catheter to keep his bladder drained.  This is not the typical kind of catheter that most people think of - it is surgically implanted right under his belly button and is changed out once a month.

His bone marrow is affected and he can no longer make enough red blood cells, so he gets a transfusion every two weeks on average.

His overall energy is very low and he is in bed most of the time.  He can only walk short distances and uses a wheelchair when he's out of the house.  He has at least 3 and usually 4 IV pumps running at all times and is also hooked up to his stomach drain bag and bladder drain bag, so it is difficult for him to move around much.

A couple of days before Christmas Noah caught a simple virus from one of his siblings and nearly died.  He began third-spacing (fluid was leaking out of his blood vessels into his tissues) and his lungs were filling up with fluid.  He was having a lot of bleeding from all of his tubes and lines as well as petechial hemorrhaging all over his body.  He is also dealing with MRSA in his bladder and is receiving IV antibiotics for this infection.  Every illness leaves Noah with a worse baseline than he had before the illness, and the total fall-out from this latest round of illness has yet to be fully seen.

Noah has been a hospice patient since this fall.  In our state (SC), pediatric hospice patients can still receive life-saving and life-extending care, and we are still treating infections and taking other steps to help Noah fight until he decides that this fight is over.  He has fought a long, hard fight - 2011 alone brought 16 hospitalizations, 7 ambulance rides, 4 Angel Flights, 5 surgeries, and much more.  In spite of everything, Noah is an incredibly bright, cheerful, happy boy who almost never acts as sick as he is and who never complains.  He loves dragons, octopuses, Angry Birds, dinosuars, volcanoes, and anything to do with Egypt.  He is our seventh (out of eight) child and is adored by his entire family.

No matter how much we love him, we recognize that he is ultimately God's child, and we trust him to the One who loves him even more than we do.  Noah has a beautiful, child-like faith and has great peace about heaven.  We see our job as being responsible to take the best possible care of him here on earth, then trust the Lord with him when it is time for Noah to go home with Jesus.

In addition to writing about Noah on Facebook (www.facebook.com/kate.estes), I keep a blog about Noah at www.prayingfornoah.com.