Well, we had our meeting with a geneticist, per a recommendation from the cardiologist. Our meeting was at 12:30pm on Friday. The geneticist entered the room around 4:45pm. Yep, over 4 hours late. In hindsight, I don't know why we kept waiting. I guess once you've packed up an infant and waited an hour, then two, you're kind of committed to the appointment.
I've mentioned before that the liver doctor ordered genetic tests that are still pending. They've done 2 tests on Lincoln's Jag1 gene, the most common gene to be the issue for Alagille's, both of which have come back negative. There is a Notch2 gene test pending. If that's negative then there's one more Jag1 test still left to be done.
So, back to the geneticist...she confirmed that she thinks it's Alagille's but we are going to wait to see how the genetic tests turn out. Yea, NOT worth the 4 hour wait. I'm not sure why the cardiologist recommended we see her now, knowing the liver doctor had genetic tests pending. I'm not sure why we didn't point that out before making the appointment. But it's done now, and there were some good points. She did take a look at Lincoln's facial features since Alagille's kids tend to have certain facial characteristics in common. Prominent foreheads, pointy chins (so they have kind of a triangular face), long noses. And it was nice to have someone combine Lincoln's separate issues and take them all into consideration at once. While the doctor's at Children's are great, they don't necessarily talk to each other and can have varying opinions. The liver doctor thinks Lincoln's forehead looks like Alagille's, the cardiologist disagrees. The urologist doesn't think the kidney issues are related at all. The geneticist brought it all together. She agrees with the liver doctor that Lincoln's forehead has some consistencies with Alagille's, mostly the indentations on the sides of his head. She doesn't think he has any of the other features at this point, but he's still only 3 1/2 months old so that can obviously change. She thinks the kidney issues are related to the heart and kidney.
John and I have mixed feelings about the fact that this is likely Alagille's. We certainly don't want Lincoln to have a genetic disorder. But the liver doctor told us recently that there is no other known cause of a liver duct shortage like Lincoln has. His hope is Alagille's over something new we don't even know about. My hope is that the liver resolves itself and it turns out it's not Alagille's so there are no genetic ramifications, but that's looking unrealistic. So short of that I guess I should hope for an Alagille's confirmation as well.
Monday, December 30, 2013
Monday, December 16, 2013
We're tube free!
We had an exciting liver appointment last Thursday! We met with the nutritionist first, she was
very nervous about us cutting a full 6 hours from Lincoln’s NG feeds. She said that was a big cut, usually they go
in 2 hour increments, but to continue with it because he was doing well. Then the liver doctor came in, saw how
Lincoln has been growing, and we told him how Lincoln wakes up hungry but eats
less while on the feeds. We've been tracking and charting everything Lincoln eats so we were also able to show them how Lincoln had been trending. Then the doctor told us to
try removing the NG completely! We were
beyond excited!
This is sooner than we or the doctor thought the tube would
be coming out, and I credit pure stubbornness on the part of me and John in
getting things moving. We’ve been
fighting for, tracking and charting every half ounce, and will continue to do
so. Removing the tube doesn’t mean he’s
better, but it does mean he gets to be a little more “normal”. And that’s what we want for him.
So far Lincoln’s no-NG calorie
intake has been going ok…not great, but not terrible. He missed his goal by half an ounce on
Friday, surpassed it by an ounce Saturday, and missed it by 1.5 ounces on
Sunday. If he can’t consistently keep up
his volume the tube may make another appearance. We need a good day for the next few days!
In terms of stats, his direct bilirubin was back to what
John and I consider his “usual”. He had
been sick a few weeks ago and landing in the emergency room and at that point
his direct bilirubin was above 5.
Thursday it was down to 4.6. Not
his lowest of 4.2, but at least improved.
His vitamin D had been too low and is now at the low end of normal, but
within the normal range. His cholesterol had been well above normal and is now
at the high end of normal, but also within the normal range. So we were excited about that! Alagille’s kids (and probably liver kids in
general, I'm not sure) can get cholesterol buildup in their blood and it can lead to
xanthomas – hard bumps in their skin caused by cholesterol deposits. That’s no fun for anyone so we were happy
we’re in the normal range for now.
We had a weigh in with the pediatrician today; this was his
first weigh in since we started the weaning process 2 weeks ago. 11 pounds 10 ounces, which is up 6 ounces
over 2 weeks. With the NG he had been
growing about a pound every two weeks so we’re a little nervous that his growth
has slowed down since the weaning process began. The pediatrician said it wasn’t ideal growth,
but not terrible since normal infant growth rates start to level off in general
by 3 months. And Lincoln is 3 months
today.
Hopefully he’ll even himself out and get the hang of eating
all on his own quickly. For now we are
loving not having to schedule our evenings around getting the feeds set up,
loving having a much less congested little baby, and loving how much more he’s
started “talking” since we took the tube out. He is all around a much happier little man!
Lincoln also had his echocardiogram and EKG last week. The cardiologist saw some new things he
didn’t love, but we’re not sure if things really changed or if they just didn’t
show up a month ago since Lincoln was smaller and it can be hard to get a
good picture. No action at this point
and still might not be a big deal, just another echo in two months to monitor
things. So for now we’re not going to
worry about it.
We really can’t thank our friends and family enough for all
their love and support through all this.
Sometimes things get hectic and we forget to respond to messages, but we
hope you know that we see them and read them to Lincoln. I’m sure I’ve said it a hundred times, but
everyone’s kind words, thoughts and prayers really help us stay positive and as
energetic as possible.
We aren’t going to get around to sending Christmas cards
this year (heck, we haven’t even sent Lincoln’s birth announcements yet!), but
hope everyone is having a happy and healthy holiday season!
Tuesday, December 10, 2013
Even more weaning
Well, weaning is going all right so far! We ended up changing the plan a bit. The original plan was to wean 3 hours total from Lincoln's 12 NG hours. We did this for 2 days and then had an update call with the pediatrician. He said he'd really like to see how Lincoln did with a full 6 hour cutoff, so we gave it a try. It's been 4 days since we cut off 6 hours and so far he's met his required ounce intake on 3 of the 4. Every 8-10 days Lincoln has a day where he doesn't eat much and one of those happened over this past weekend after the weaning started and he missed his goal, so we're hoping another one of those doesn't happen for at least another week.
The next step for weaning is to meet with the liver doctor on Thursday and show him, the nurse and the nutritionist all of Lincoln's intake tracking before and after the 6 hour weaning. We have to be able to show that Lincoln is still getting all of his required ounces, and hopefully that he's getting just as many calories as he was before the wean. We'll also get bloodwork done on Thursday to check bilirubin, cholesterol and vitamin D levels. We're hoping for low bilirubin and cholesterol, high vitamin D.
We also met with the urologist this week to talk about the ultrasound from last week. His hydronephrosis (fluid in his left kidney) is unchanged and the ultrasound showed some increased echogenicity (this is a bad thing for a kidney). The urologist feels the echogenicity is negligible and could have just been caused by different settings on the ultrasound machine, so we're not going to worry too much about it. But since his left kidney hasn't improved he has to have a kidney function test. They use dye to see how long it takes fluid to move through the kidney. If it takes around 10 minutes then we can continue to wait and see if they hydronephrosis improves. If it takes closer to 20 minutes then Lincoln would likely require surgery to correct the blockage or whatever is messing up the kidney function. We haven't scheduled that test yet, but hopefully it'll be in the next couple of weeks.
John and I are doing all right. It's been a little stressful as we're getting into a new routine with me going to back to work, but we're getting there. Emotionally exhausted, but getting there. This weaning process, and really the health issues in general, comes with extreme highs and extreme lows and can really wear you down. For example, yesterday morning both John and I were so disappointed and discouraged because Lincoln wouldn't eat more than half an ounce at his normal 4 ounce bottle time. Then 3 hours later he ate 5.5 ounces, more than he's ever eaten at once before, and followed it up in the evening by finishing a 6 ounce bottle. He ended up meeting his ounce goal after we were sure he'd miss it, we were so excited! Now Lincoln won't wake up after eating only 3 ounces, while still short of his goal for the day...and so the up and down process starts over again. He has 20 minutes or so to eat a couple more ounces or we have to start the NG feed early instead of continuing the weaning, so I'm going to go and give it another try. All we can do is keep on trying!
The next step for weaning is to meet with the liver doctor on Thursday and show him, the nurse and the nutritionist all of Lincoln's intake tracking before and after the 6 hour weaning. We have to be able to show that Lincoln is still getting all of his required ounces, and hopefully that he's getting just as many calories as he was before the wean. We'll also get bloodwork done on Thursday to check bilirubin, cholesterol and vitamin D levels. We're hoping for low bilirubin and cholesterol, high vitamin D.
We also met with the urologist this week to talk about the ultrasound from last week. His hydronephrosis (fluid in his left kidney) is unchanged and the ultrasound showed some increased echogenicity (this is a bad thing for a kidney). The urologist feels the echogenicity is negligible and could have just been caused by different settings on the ultrasound machine, so we're not going to worry too much about it. But since his left kidney hasn't improved he has to have a kidney function test. They use dye to see how long it takes fluid to move through the kidney. If it takes around 10 minutes then we can continue to wait and see if they hydronephrosis improves. If it takes closer to 20 minutes then Lincoln would likely require surgery to correct the blockage or whatever is messing up the kidney function. We haven't scheduled that test yet, but hopefully it'll be in the next couple of weeks.
John and I are doing all right. It's been a little stressful as we're getting into a new routine with me going to back to work, but we're getting there. Emotionally exhausted, but getting there. This weaning process, and really the health issues in general, comes with extreme highs and extreme lows and can really wear you down. For example, yesterday morning both John and I were so disappointed and discouraged because Lincoln wouldn't eat more than half an ounce at his normal 4 ounce bottle time. Then 3 hours later he ate 5.5 ounces, more than he's ever eaten at once before, and followed it up in the evening by finishing a 6 ounce bottle. He ended up meeting his ounce goal after we were sure he'd miss it, we were so excited! Now Lincoln won't wake up after eating only 3 ounces, while still short of his goal for the day...and so the up and down process starts over again. He has 20 minutes or so to eat a couple more ounces or we have to start the NG feed early instead of continuing the weaning, so I'm going to go and give it another try. All we can do is keep on trying!
Wednesday, December 4, 2013
Weaning & Kidney Ultrasound
We got the official ok from the liver doctor to cut back on Lincoln's NG feeds! We're going to start by cutting 3 hours, so we'll be down to 9 hours on the feeding pump. We'll start it 1.5 hours later and turn it off 1.5 hours sooner. If he does well then next week when we see the liver doctor maybe we'll talk about cutting another 3 hours. The liver doctor has told us in the past that he'd like it to be a slow process. It's a fine line of weaning him when he's starting to show signs of being ready, but not going so fast that we throw off his growth.
Today was the first day we shut off the pump at 6:30am instead of 8am, and we had great results! What we usually see is that Lincoln will wake up around 5am, eat 1-1.5 ounces, then wake up again at 8am and eat another 1-1.5 ounces. Today he had his normal bottle at 5am, but ate 3 ounces at 8am. Great job Lincoln! To make up for the NG hours we cut he has to eat an extra 2.5 ounces over the day and he's already eaten an extra 1.5. It's only the first day, but I'm thrilled!
We also had a kidney ultrasound today. We don't meet with the urologist to talk about it until Monday, but I think his left kidney is still an issue. I'm no expert, but even I could see the difference between his left and right on the screen. So we'll see what the urologist has to say and take it from there.
Today was the first day we shut off the pump at 6:30am instead of 8am, and we had great results! What we usually see is that Lincoln will wake up around 5am, eat 1-1.5 ounces, then wake up again at 8am and eat another 1-1.5 ounces. Today he had his normal bottle at 5am, but ate 3 ounces at 8am. Great job Lincoln! To make up for the NG hours we cut he has to eat an extra 2.5 ounces over the day and he's already eaten an extra 1.5. It's only the first day, but I'm thrilled!
We also had a kidney ultrasound today. We don't meet with the urologist to talk about it until Monday, but I think his left kidney is still an issue. I'm no expert, but even I could see the difference between his left and right on the screen. So we'll see what the urologist has to say and take it from there.
Monday, December 2, 2013
Back to work
Today was my first day back to work and I survived! I kind of feel like it wasn't a "real" day - lots of sorting through emails and getting updates. An awesome coworker even brought dinner for me to take home for John and I. We are seriously lucky with all of the amazing people in our lives!
I do think it's going to be a challenge to keep up pumping at work, but I'm determined to make it work - especially when I think of how much Lincoln's special formula would cost otherwise! Frugalness is a great motivator.
Today also involved a check-in with the pediatrician. We were hoping for 11.5 pounds and were awfully close at 11 pounds 4 ounces! The doctor was thrilled with Lincoln's growth. He's still small, falling into about the 10th percentile for both length and weight, but he's growing at the same rate as a "normal" baby. And Lincoln's head circumference falls into the 75th percentile. We'll have to wait to find out for sure, but we're pretty sure that means he's a genious.
We also talked to the pediatrician about Lincoln's eating schedule. Like I've said, Lincoln is on NG feeds from 8pm to 8am. When the feeds aren't going he eats 3.5-4 ounces per bottle. When the feeds are going he eats less often, but is hungry around 10pm, 5am and 8am. Each of these bottles are only 1-1.5 ounces. The pediatrician believes (and John and I have been thinking the same thing) that those 10pm, 5am and 8am bottles would be bigger if the feeds weren't going. So he told us to start the weaning process! The goal is for Lincoln to prove that he can eat (and not throw up) just as many ounces without the NG feeds as with, and this will be the first step. We're going to check in with the liver doctor to make sure there isn't something we're not thinking of, but assuming we get the ok we'll start turning the feeds off at 5am instead of 8am and see if the 8am bottle makes up for the 3 lost hours of NG feed. We really hope it does, wish us luck!
I do think it's going to be a challenge to keep up pumping at work, but I'm determined to make it work - especially when I think of how much Lincoln's special formula would cost otherwise! Frugalness is a great motivator.
Today also involved a check-in with the pediatrician. We were hoping for 11.5 pounds and were awfully close at 11 pounds 4 ounces! The doctor was thrilled with Lincoln's growth. He's still small, falling into about the 10th percentile for both length and weight, but he's growing at the same rate as a "normal" baby. And Lincoln's head circumference falls into the 75th percentile. We'll have to wait to find out for sure, but we're pretty sure that means he's a genious.
We also talked to the pediatrician about Lincoln's eating schedule. Like I've said, Lincoln is on NG feeds from 8pm to 8am. When the feeds aren't going he eats 3.5-4 ounces per bottle. When the feeds are going he eats less often, but is hungry around 10pm, 5am and 8am. Each of these bottles are only 1-1.5 ounces. The pediatrician believes (and John and I have been thinking the same thing) that those 10pm, 5am and 8am bottles would be bigger if the feeds weren't going. So he told us to start the weaning process! The goal is for Lincoln to prove that he can eat (and not throw up) just as many ounces without the NG feeds as with, and this will be the first step. We're going to check in with the liver doctor to make sure there isn't something we're not thinking of, but assuming we get the ok we'll start turning the feeds off at 5am instead of 8am and see if the 8am bottle makes up for the 3 lost hours of NG feed. We really hope it does, wish us luck!
Sunday, December 1, 2013
Stats
A quick summary of Lincoln's current stats as a starting point...
On November 15th he weighed in at 10.2 oz and was 21.5 inches long. He'll get weighed and measured again tomorrow (12/2/13).
November 15th was also his most recent bloodwork. One of the main things they check is his bilirubin level, this measures his liver function. The less bilirubin in his blood, the better his liver is working. There are two types of bilirubin, direct and indirect. Indirect is what causes most infant jaundice and can be treated with UV lights. Lincoln's jaundice is caused by direct bilirubin, the worse of the two.
On the 15th Lincoln's direct bilirubin was at 5.4, up from his lowest of 4.2. His next labs will be December 12th. The doctor would like to see the bilirubin much lower. He could probably maintain his health at levels around 2. Zero is ideal. If levels stay high then scarring (liver cirrhosis) could start, which means we'd have to start discussing a transplant at some point.
The first genetic tests actually came back negative for Alagille's. This test was for the most easily found mutations of the Jagged1 gene. The next round of testing, currently under way, will test the less easily found Jagged1 mutations and will also check the Notch2 gene. Most people with Alagille's have the Jagged1 mutation, but 2-5% have the Notch mutation. These results will be available anytime in the next few weeks/months, depending on how long the lab takes.
Lincoln's heart condition is called aortic stenosis. This is the condition that I'm a little ashamed to say I understand the least. But John understands it so we're covered. His last echocardiogram and EKG showed that the condition is very mild and should only require occassional monitoring. Our next echo and EKG are on December 13th to make sure that's still the case.
We also meet with the cardio geneticist in late December to discuss whether they agree that Lincoln may have Alagille's syndrome or whether it might be something else.
Lincoln's left kidney had an ultrasound in November, it still looks like it's holding fluid (the condition is called hydronephrosis). His next kidney ultrasound is later this month and will determine whether we need to do additional testing to verify that fluid is actually backing up or if the kidney is just stretched out due to fluid retention while his kidney was still being formed. If the kidney is holding fluid the doctor believes it is likely due to a blockage and can be fixed with a relatively common and successful surgery to remove the blockage.
That's where we are to date! Next pediatrician appointment is tomorrow to check his growth and discuss any concerns. One of our concerns is that Lincoln sleeps a lot and is sometimes lethargic so we'll get the pediatrician's thoughts on that.
On November 15th he weighed in at 10.2 oz and was 21.5 inches long. He'll get weighed and measured again tomorrow (12/2/13).
November 15th was also his most recent bloodwork. One of the main things they check is his bilirubin level, this measures his liver function. The less bilirubin in his blood, the better his liver is working. There are two types of bilirubin, direct and indirect. Indirect is what causes most infant jaundice and can be treated with UV lights. Lincoln's jaundice is caused by direct bilirubin, the worse of the two.
On the 15th Lincoln's direct bilirubin was at 5.4, up from his lowest of 4.2. His next labs will be December 12th. The doctor would like to see the bilirubin much lower. He could probably maintain his health at levels around 2. Zero is ideal. If levels stay high then scarring (liver cirrhosis) could start, which means we'd have to start discussing a transplant at some point.
The first genetic tests actually came back negative for Alagille's. This test was for the most easily found mutations of the Jagged1 gene. The next round of testing, currently under way, will test the less easily found Jagged1 mutations and will also check the Notch2 gene. Most people with Alagille's have the Jagged1 mutation, but 2-5% have the Notch mutation. These results will be available anytime in the next few weeks/months, depending on how long the lab takes.
Lincoln's heart condition is called aortic stenosis. This is the condition that I'm a little ashamed to say I understand the least. But John understands it so we're covered. His last echocardiogram and EKG showed that the condition is very mild and should only require occassional monitoring. Our next echo and EKG are on December 13th to make sure that's still the case.
We also meet with the cardio geneticist in late December to discuss whether they agree that Lincoln may have Alagille's syndrome or whether it might be something else.
Lincoln's left kidney had an ultrasound in November, it still looks like it's holding fluid (the condition is called hydronephrosis). His next kidney ultrasound is later this month and will determine whether we need to do additional testing to verify that fluid is actually backing up or if the kidney is just stretched out due to fluid retention while his kidney was still being formed. If the kidney is holding fluid the doctor believes it is likely due to a blockage and can be fixed with a relatively common and successful surgery to remove the blockage.
That's where we are to date! Next pediatrician appointment is tomorrow to check his growth and discuss any concerns. One of our concerns is that Lincoln sleeps a lot and is sometimes lethargic so we'll get the pediatrician's thoughts on that.
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.
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.
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