Understanding

I had a hard time naming this blog.  I like alliteration so I liked the idea of "Lincoln's Liver".  But this journey includes more than just his liver so it didn't fit.  I think I landed with an ok blog name.  I'm not sure if you can change it later or not, but whatever.

I've been considering starting a blog to keep everyone who is interested up to date on Lincoln's health status.  That way I can update everyone without big long posts on facebook.  Then my cousin Darla suggested it as sort of a form of therapy, which couldn't hurt, so that gave me the extra push. 

In case anyone reading this doesn't know, Lincoln is mine and my husband's (John) son.  He was born September 16, 2013 and is awesome!  He also has some unique challenges which his liver doctor believes are the result of Alagille's syndrome.  Alagille's is a genetic disorder that causes problems mainly with the liver, heart and kidneys.  Luckily Lincoln's heart and kidney situations aren't too bad so far, it's just the liver giving us issues.  These are some great resources for additional information about Alagille's:
http://www.chop.edu/service/alagille-syndrome-clinical-care-program/about-alagille-syndrome/
http://www.cincinnatichildrens.org/health/a/alagille-syndrome/

As a result, Lincoln has neonatal cholestatis, which means bile is backing up in his liver into his blood stream.  This is because he doesn't have enough bile ducts in his liver, which is common in alagille's kids. Bile is supposed to go from your liver to your intestines, where it helps the body absorb fat so you can grow. Without enough bile ducts there isn't enough bile in the intestines to absorb fat. This means Lincoln doesn't absorb a good amount of what he eats. It also means his body has a hard time absorbing certain vitamins that are fat soluble. The way the body absorbs these vitamins (specifically vitamins A, D, E and K) is through fat. No fat absorbtion, no vitamin absorbtion. So he needs to eat as much as possible to make up for it. Unfortunately, he's not a great eater.

What I mean when I say that Lincoln isn't a great eater is that he doesn't have much of an appetite to begin with and frequently throws up. He's been on Zantac for reflux, which has helped a lot, but it's still slow going to get the size of his bottles up. Added to this is that the less Lincoln eats, the less he wants to eat. For example, a typical bottle size these days (when the NG feeds aren't going) is 3.5-4.5 ounces. Say Lincoln has a bad feeding and won't eat more than 2 ounces, or throws up and doesn't want to eat again. You'd think that at his next bottle he'd be famished. Nope, not Lincoln. When it's time for his next bottle he'll be tired (probably from not eating) and eat less than normal. From our experience so far, if we don't get him relaxed and reset ASAP this will continue and he'll eat less and less at each feeding until he's eating next to nothing and ends up in the emergency room with IV fluids.

This is the reason for the NG tube (the tube you see in photos going into his nose), to give him extra calories. During the day the tube isn't attached to anything, but for 12 hours each day it's attached to a pump that gives him just under an ounce of food each hour.  The slow rate helps make sure he tolerates the feeds and doesn't throw up.  The tube delivers the same thing he normally eats.  We put a special formula called Pregestimil in his breastmilk. This formula contains MCT oil, a type of fat that's more easily aborbed, and also increases the calorie content of the milk. Usually breastmilk contains 20 calories per ounce and our recipe increases that to 24 calories per ounce (seems small, but it's a 20% increase). And we make sure to feed him on a strict schedule so that he gets enough calories during the day.

I've realized that the strict feeding schedule we keep and the worry we put into counting the ounces Lincoln eats probably seems ridiculous to anyone who doesn't see or talk to us regularly. I realize that it's difficult to understand if you haven't seen first hand the road we've been on.  While leaving a wedding and driving home late at night might seem extreme to some, to John and I this weekend it was the most reasonable option and neither of us thought twice about it. 
See, this past weekend we drove up to Cleveland for my brother Paul's wedding to his new wife Briana.  Yay wedding!  Unfortunately Lincoln was completely thrown off his schedule while we were out of town and decided to go on a hunger strike the day of the wedding.  We're not sure if he's too used to being fed by mom and didn't like her being gone for wedding festivities all day or if he just wanted to be home, but we couldn't risk another day falling short on the ounces he needs to eat so we packed up and headed home.  Luckily when the hospital gave us his NG feed supplies they gave us a backpack to put the pump into in case we needed to be out of the house.  At the time we thought this was ridiculous, it's not like we planned on taking him anywhere while on the feeds, but it sure did come in handy in this situation!  So after getting the NG feed pump set up, feeding him as much as we could and getting him strapped in the car, then having him decide he was suddenly starving, pooping, us realizing it was time for his vitamins, then realizing we needed to have his liver medicine ready for while we were driving.....2 hours later we were on the road and made it back to Cincinnati by 1:30am.

Like I said, we realize our situtation is difficult to understand.  It may seem like we're being overprotective.  Maybe we are.  But we've made great progress so far and aren't willing to risk any sort of setback that could be avoided.  They make special growth charts for Alagille's kids because they have poor growth.  So far Lincoln hasn't had to be on it, he's in a low percentile but is still on the "normal" kid charts, and we hope to keep it that way.