Lincoln's repeat cardiac cath was last week, and the results were better than the one prior. This was really good news! His heart isn't perfect. It's still pumping more blood than it should have to. They said his heart is squeezing well but isn't relaxing well. But it's better than before, and the cardiologist believes this particular problem is directly related to his sick liver. Unfortunately, the subaortic membrance is also coming back. It's not as bad as it was before but we'll have to keep a close eye on it.
A kidney test called a GFR was the last test we had to do before listing. The doctors wanted to know how his kidneys were working so they could plan out the post-transplant anti-rejection medications. His kidney also aren't perfect, but are doing better than they were before surgery.
Finally, on April 22nd, Lincoln was officially put on the liver transplant waiting list! It was the most exciting and terrifying news we've ever received. And now we just wait. In the meantime, myself and a couple other people are being evaluated to be living donors.
We're still in the hospital but Lincoln has been feeling better for the last few days. He's smiling, kicking his legs and playing with his toys. He has some physical delays, he can't sit up on his own yet and he can't push himself up when he's on his belly. This is pretty normal for liver kids. His belly gets very bloated and really makes it hard for him to lie on his tummy. And of course a couple of surgeries and having to stay in bed for weeks will delay most things. Poor kid just doesn't get the play time other kids his age get. The nurses tell us that once kids get new livers they catch right up and I hope that's true.
Another piece of good news is that the doctors think he's right on track mentally. He's interested in things, recognizes people and reacts appropriately to noises and new people. Of course there is just no way to know the long term effects of all this, but it's reassuring that he seems to be developing so far!
Thursday, April 24, 2014
Wednesday, April 16, 2014
Lots of updates
It's been an unacceptably long amount of time since I've updated the blog, and I apologize for that. As more time passed it started to seem like a more and more daunting task so it never got done. I'll do a quick overview of the past couple of months, and then an update of where we are now. Sorry, it may get long. Also, I am far from a medical expert, so I will just try to explain things as I understand them.
February
Heart surgery happened and was a success. Lincoln had a little bit of a hard time going on heart lung bypass but came off bypass fine. He never picked up eating like he was before surgery, but we went home in the hopes that he'd pick up once in a few days. Unfortunately, he didn't, and his electrolytes dropped low enough that we were readmitted after only a few days at home.
March
The first few weeks of being back in the hospital were a terrible roller coaster. Lincoln wasn't feeling great and wasn't eating great so we started him back on ng feeds. However, he was throwing up occasionally and since the doctors weren't sure why they kept ordering him NPO (traslation: not allowed to eat). They'd try to put him on fluids but he generally doesn't react well to that - he kept holding too much fluid then would have trouble breathing because the fluid in his belly pushed against his lungs. He landed in the PICU (pediatric intensive care unit) a couple of times. This back and forth continued for a couple of weeks, and during that time Lincoln got very little nutrition and lost a good amount of weight. Finally, after many sometimes intense conversations with Lincoln's doctors they finally started him on TPN, which is IV nutrition. This wasn't ideal since obviously we'd rather he get his milk, and TPN is hard on the liver, but we were just happy he was getting any sort of nutrition.
Once he was stable the doctors wanted to get the kidney surgery knocked out. The surgery to get rid of his UPJ obstruction (blockage in his ureter) happened 25 days after the heart surgery, and that was a success as well. The urologist had been concerned that the kidney would be in bad shape and he'd have to remove it, but it looked better than he expected and he was able to remove the obstruction. Yay! As an interesting side note, the surgeon also told us that Lincoln's bilirubin isn't only in his skin. His internal organs, fat, tissues, etc all have a yellow tint to them. The sugeon was a little surprised I think I had never thought about it before, but it makes sense.
Recovery from kidney surgery was not easy. They removed the breathing tube a couple of days after surgery, but then Lincoln had trouble breathing so the next day they had to reintubate him (sedate him and put the breathing tube back in). Then a day later (I think it was the next day anyway...it all blends together) he went into septic shock. His blood pressure dropped very low and he had a lot of fluid in his lungs, and was generally having a hard time. Turns out he had RSV, which probably contributed at least a little to his difficult recovery. RSV is a virus that in an adult might not show up as anything more than a runny nose. But in kids, especially kids like Lincoln who are fresh out of surgery, it can lead to some pretty serious complications. He finally stabilized but it was still a couple of weeks until he was able to kick the RSV symptoms and his breathing improved. I'll take this moment to remind people that if you're not feeling well, don't risk it! Don't visit little ones and don't go into hospitals. What shows up in you as just the sniffles could be a very big deal to someone else. It's always better to be safe than sorry.
After he was recovered we finally got Lincoln on a steady nutrition plan. He was, and is still, on ng feeds and then eats whatever else he wants by mouth. A week or so after he was stable Lincoln had another cardiac cath to assess whether his heart was now strong enough for a liver transplant. He was approved for listing, but liver transplants involve clamping some major blood vessels so the old liver can be removed and that puts a lot of stress on the heart. The doctors wanted to be sure his heart could handle it. Turns out his heart function wasn't ideal. His heart was pumping more blood than it should have to, likely because of his sick liver. It was making his vessels floppy. The extra blood flow was making them expand, and then they were having trouble relaxing. So picture a balloon you've blown up then let the air out of. The balloon never quite goes back to its original shape. This is common with liver disease but unfortunately makes a transplant riskier. To try to improve his heart the plan was to take a week to give him dieretics to get some fluid off him, give him time on his steady nutrition plan, and give him extra oxygen.
However, before we could get a repeat cath, Lincoln got a bacterial infection. Another complication of liver disease. Crazy how much your liver does, huh? Since Lincoln's liver can't filter out things like bacteria he's at increase risk of a blood infection. The bacteria he got is one that's found in the stomach, it made its way to his blood stream. Luckily we found it super early and he didn't really have any of the complications it can cause. Sidebar...
From the start of our latest hospital admission John and I felt like we were struggling to be heard by the doctors and nurses. We know Lincoln and wanted to be involved in his care decisions. For example, we knew he doesn't react well to being on IV fluids. However, he kept being put on IV fluids even when John and I emphatically asked that he not be and instead be allowed to start his ng feeds. It's especially hard because the doctors rotate every week. So it felt like once we finally got through to one team and they were listening to our thoughts, they'd leave and a whole new team would come in. Finally, after weeks of pushing, we got a core team of doctors who knew Lincoln and knew us, and they are now the "go to" team for decisions for Lincoln. And they listen to us, which is awesome. Back to the bacteria...
One morning John felt that something was up with Lincoln. Lincoln just wasn't acting right, and John expressed his concerns to the doctor. Because we now have this great team who really cares about our input, the doctor listened to John and took him seriously. She ran a bunch of tests, one of which was a blood culture, to make sure nothing was wrong. That's how we caught the bacterial infection so early. The care team worked exactly like it should and Lincoln's health benefitted.
Turns out the bacteria had gotten onto Lincoln's PICC (basically a long term IV, makes it so they can draw labs and give him medicines without having to stick him every time) so they had to pull it out. Of course the cardiac cath was postponed.
Got rid of the bacteria, and that brings us to today. Lincoln is getting his repeat cath, and hopefully things look better. They originally wanted to get another liver biopsy on the same day but that didn't work out. The transplant team will go over the cath results in a meeting tomorrow and will decide if a biopsy is really necessary. He had one when he was very small, but they may need to repeat it to see if anything has changed. They want to be as sure as they can that a new liver will fix the problem.
We need this cath to show that his heart is in better shape. Othewise they won't be able to list him and accept a new liver for him. Updates to come...hopefully it won't take me 2 months to write an update this time!
February
Heart surgery happened and was a success. Lincoln had a little bit of a hard time going on heart lung bypass but came off bypass fine. He never picked up eating like he was before surgery, but we went home in the hopes that he'd pick up once in a few days. Unfortunately, he didn't, and his electrolytes dropped low enough that we were readmitted after only a few days at home.
March
The first few weeks of being back in the hospital were a terrible roller coaster. Lincoln wasn't feeling great and wasn't eating great so we started him back on ng feeds. However, he was throwing up occasionally and since the doctors weren't sure why they kept ordering him NPO (traslation: not allowed to eat). They'd try to put him on fluids but he generally doesn't react well to that - he kept holding too much fluid then would have trouble breathing because the fluid in his belly pushed against his lungs. He landed in the PICU (pediatric intensive care unit) a couple of times. This back and forth continued for a couple of weeks, and during that time Lincoln got very little nutrition and lost a good amount of weight. Finally, after many sometimes intense conversations with Lincoln's doctors they finally started him on TPN, which is IV nutrition. This wasn't ideal since obviously we'd rather he get his milk, and TPN is hard on the liver, but we were just happy he was getting any sort of nutrition.
Once he was stable the doctors wanted to get the kidney surgery knocked out. The surgery to get rid of his UPJ obstruction (blockage in his ureter) happened 25 days after the heart surgery, and that was a success as well. The urologist had been concerned that the kidney would be in bad shape and he'd have to remove it, but it looked better than he expected and he was able to remove the obstruction. Yay! As an interesting side note, the surgeon also told us that Lincoln's bilirubin isn't only in his skin. His internal organs, fat, tissues, etc all have a yellow tint to them. The sugeon was a little surprised I think I had never thought about it before, but it makes sense.
Recovery from kidney surgery was not easy. They removed the breathing tube a couple of days after surgery, but then Lincoln had trouble breathing so the next day they had to reintubate him (sedate him and put the breathing tube back in). Then a day later (I think it was the next day anyway...it all blends together) he went into septic shock. His blood pressure dropped very low and he had a lot of fluid in his lungs, and was generally having a hard time. Turns out he had RSV, which probably contributed at least a little to his difficult recovery. RSV is a virus that in an adult might not show up as anything more than a runny nose. But in kids, especially kids like Lincoln who are fresh out of surgery, it can lead to some pretty serious complications. He finally stabilized but it was still a couple of weeks until he was able to kick the RSV symptoms and his breathing improved. I'll take this moment to remind people that if you're not feeling well, don't risk it! Don't visit little ones and don't go into hospitals. What shows up in you as just the sniffles could be a very big deal to someone else. It's always better to be safe than sorry.
After he was recovered we finally got Lincoln on a steady nutrition plan. He was, and is still, on ng feeds and then eats whatever else he wants by mouth. A week or so after he was stable Lincoln had another cardiac cath to assess whether his heart was now strong enough for a liver transplant. He was approved for listing, but liver transplants involve clamping some major blood vessels so the old liver can be removed and that puts a lot of stress on the heart. The doctors wanted to be sure his heart could handle it. Turns out his heart function wasn't ideal. His heart was pumping more blood than it should have to, likely because of his sick liver. It was making his vessels floppy. The extra blood flow was making them expand, and then they were having trouble relaxing. So picture a balloon you've blown up then let the air out of. The balloon never quite goes back to its original shape. This is common with liver disease but unfortunately makes a transplant riskier. To try to improve his heart the plan was to take a week to give him dieretics to get some fluid off him, give him time on his steady nutrition plan, and give him extra oxygen.
However, before we could get a repeat cath, Lincoln got a bacterial infection. Another complication of liver disease. Crazy how much your liver does, huh? Since Lincoln's liver can't filter out things like bacteria he's at increase risk of a blood infection. The bacteria he got is one that's found in the stomach, it made its way to his blood stream. Luckily we found it super early and he didn't really have any of the complications it can cause. Sidebar...
From the start of our latest hospital admission John and I felt like we were struggling to be heard by the doctors and nurses. We know Lincoln and wanted to be involved in his care decisions. For example, we knew he doesn't react well to being on IV fluids. However, he kept being put on IV fluids even when John and I emphatically asked that he not be and instead be allowed to start his ng feeds. It's especially hard because the doctors rotate every week. So it felt like once we finally got through to one team and they were listening to our thoughts, they'd leave and a whole new team would come in. Finally, after weeks of pushing, we got a core team of doctors who knew Lincoln and knew us, and they are now the "go to" team for decisions for Lincoln. And they listen to us, which is awesome. Back to the bacteria...
One morning John felt that something was up with Lincoln. Lincoln just wasn't acting right, and John expressed his concerns to the doctor. Because we now have this great team who really cares about our input, the doctor listened to John and took him seriously. She ran a bunch of tests, one of which was a blood culture, to make sure nothing was wrong. That's how we caught the bacterial infection so early. The care team worked exactly like it should and Lincoln's health benefitted.
Turns out the bacteria had gotten onto Lincoln's PICC (basically a long term IV, makes it so they can draw labs and give him medicines without having to stick him every time) so they had to pull it out. Of course the cardiac cath was postponed.
Got rid of the bacteria, and that brings us to today. Lincoln is getting his repeat cath, and hopefully things look better. They originally wanted to get another liver biopsy on the same day but that didn't work out. The transplant team will go over the cath results in a meeting tomorrow and will decide if a biopsy is really necessary. He had one when he was very small, but they may need to repeat it to see if anything has changed. They want to be as sure as they can that a new liver will fix the problem.
We need this cath to show that his heart is in better shape. Othewise they won't be able to list him and accept a new liver for him. Updates to come...hopefully it won't take me 2 months to write an update this time!
Saturday, February 22, 2014
Surgery Date
There has been a lot of back and forth this week regarding Lincoln's heart surgery date. The surgeon is booked, but Lincoln's liver is getting worse so we can't wait. If liver function gets too bad then the surgery can't happen. But the surgery has to happen before Lincoln can be listed for a liver transplant.
After a lot of conversations and meetings between Lincoln's doctors and the surgeon we got a surgery date of this Monday. We get admitted early Sunday afternoon. Lincoln will get an exam and blood work Sunday and surgery will be first thing Monday morning. The only minor hiccup is that Lincoln just got his 6 month vaccines on Thursday because he's on an accelerated vaccine schedule to prepare him for transplant. The heart surgical team doesn't like kids to have been vaccinated within the 2 weeks before surgery because the heart/lung bypass machine can wreck havoc on the immune system. The one vaccine in particular they're concerned with is for rotovirus because that vaccine is a live virus. They think it'll be ok, they're more concerned with things like the MMR and flu vaccine before surgery, but they're triple checking with the infectious diseases team to be sure it's ok.
The surgery itself takes 4-5 hours and is open heart. They go in through the aorta to get to the membrane below his aortic valve. They'll also perform a myectomy (I think that's what it's called, I didn't write it down), which involves removing some heart tissue to change the shape a little. This will change the way the blood flows and will lessen the pressure at the spot the membrane grew, with the hope that with less pressure the membrane will be less likely to grow back. I apologize if anyone reading this knows more about the heart than I do, I'm sure I oversimplified how the myectomy works, but I think that's the basic idea. The heart is really complicated to understand!
We and the surgeon are confident and hopeful for this surgery, but in the spirit of full disclosure I will say that there are risks. The surgeon has performed plenty of surgeries on subaortic membranes but they are usually on bigger kids (5-7 years old is the more common time for a subaortic membrane to become an issue). Since Lincoln is younger his heart is obviously smaller, and his aorta runs small. Don't get too nervous, the surgeon has performed the procedure successfully on kids as small as Lincoln. But he has not performed the surgery on someone as yellow as Lincoln. The main risk we're running is how Lincoln's liver is going to react to all this. But it's a risk John and I feel we have to take.
Fingers crossed that surgery can happen on Monday. We'll update as we can.
After a lot of conversations and meetings between Lincoln's doctors and the surgeon we got a surgery date of this Monday. We get admitted early Sunday afternoon. Lincoln will get an exam and blood work Sunday and surgery will be first thing Monday morning. The only minor hiccup is that Lincoln just got his 6 month vaccines on Thursday because he's on an accelerated vaccine schedule to prepare him for transplant. The heart surgical team doesn't like kids to have been vaccinated within the 2 weeks before surgery because the heart/lung bypass machine can wreck havoc on the immune system. The one vaccine in particular they're concerned with is for rotovirus because that vaccine is a live virus. They think it'll be ok, they're more concerned with things like the MMR and flu vaccine before surgery, but they're triple checking with the infectious diseases team to be sure it's ok.
The surgery itself takes 4-5 hours and is open heart. They go in through the aorta to get to the membrane below his aortic valve. They'll also perform a myectomy (I think that's what it's called, I didn't write it down), which involves removing some heart tissue to change the shape a little. This will change the way the blood flows and will lessen the pressure at the spot the membrane grew, with the hope that with less pressure the membrane will be less likely to grow back. I apologize if anyone reading this knows more about the heart than I do, I'm sure I oversimplified how the myectomy works, but I think that's the basic idea. The heart is really complicated to understand!
We and the surgeon are confident and hopeful for this surgery, but in the spirit of full disclosure I will say that there are risks. The surgeon has performed plenty of surgeries on subaortic membranes but they are usually on bigger kids (5-7 years old is the more common time for a subaortic membrane to become an issue). Since Lincoln is younger his heart is obviously smaller, and his aorta runs small. Don't get too nervous, the surgeon has performed the procedure successfully on kids as small as Lincoln. But he has not performed the surgery on someone as yellow as Lincoln. The main risk we're running is how Lincoln's liver is going to react to all this. But it's a risk John and I feel we have to take.
Fingers crossed that surgery can happen on Monday. We'll update as we can.
Sunday, February 16, 2014
We're tired
Not sure how to describe this past week. It was just...a lot.
The cardiologist called us Tuesday to give an update on what the surgical team thought of Lincoln. They all agree that the surgery needs to happen soon. Unfortunately, the surgeon's schedule is booked for the next 1.5 months so we're still waiting for a surgery date. Everyone agrees that 1.5 months is too long to wait so the cardiologist is talking to the surgeon daily to try and figure out how to fit Lincoln in to the schedule. They want him in within the next week or so.
We did ask if they'd ever consider fixing the kidney at the same time as the heart, that answer was no. Lincoln will have to be on heart/lung bypass for the surgery, which can create havoc on other organs, so they don't want to cause any other shocks to the system.
The surgical team also wanted more specific details about Lincoln's heart before going in to surgery. They wanted to perform a cardiac cath procedure. This involves putting a catheter in through the artery in Lincoln's groin and winding it up to the heart, and it happened on Wednesday. They measured the different pressures in different parts of the heart. I'm going to try to describe what it was they were checking, but I'm no expert so I'll just try to explain as best I can and the way I understand it.
The aorta is a vessel that takes blood into and out of the left ventricle of the heart (the difference between the left and right ventricle isn't important for this explanation). The aortic valve is part of the aorta and is at the spot there the aorta ends and the left ventricle begins - kind of like the door between 2 rooms. It opens and closes so blood leaves and enters the left ventricle when its supposed to. The issue with Lincoln's heart is that there is a membrane beneath the aortic valve. This membrane partially blocks the blood's path. Just like when you put your thumb over a hose and the pressure increases, the blood starts pushing through with more pressure. This is what they found with the cardiac cath. The pressure in the aorta is normal, the pressure at the aortic valve is higher, and the pressure within the left ventricle is higher still. This is bad for the heart so they need to remove that subaortic membrane.
There are also some abnormalities with the other side of Lincoln's heart, the pulmonary side. This didn't used to be the case so things are changing over there, but no intervention or real problems at this point.
They also checked the pressure going to his lungs and that was just fine.
Lincoln had to stay over night for observation after the procedure but did great and seems to be back to himself. Unfortunately he's running a slight fever and has a little congestion, my guess is it's something he picked up in the hospital, but is otherwise doing fine. The heart surgery can't happen if he has a fever so hopefully he gets better soon!
They did more blood work and his direct bilirubin was down to 18. But his INR, a measure of blood clotting, was higher (we wanted lower) even after his shot of vitamin K last week. The gastroenterologist said it was weird for one to improve and the other to get worse, and he would have rather seen the bilirubin stay where it was and the INR to improve. His liver isn't happy and it's just not acting the way they'd expect.
To try to get more clues leading to a diagnosis Lincoln had a couple extra tests this week.
He saw the ophthalmologist. They were looking for something called posterior embryotoxin. This is a thin line seen at the edge of the iris. It doesn't affect vision in any way, but is seen in 90% of people with Alagille syndrome and only 8-15% of the general population. Lincoln doesn't have it.
He had arm and leg x-rays. There are other disorders that cause bone/joint abnormalities so we were ruling those out. X-rays were normal.
He'll have a head ultrasound this week. I didn't ask what they were looking for here. I'll update once we know more.
The cardiologist called us Tuesday to give an update on what the surgical team thought of Lincoln. They all agree that the surgery needs to happen soon. Unfortunately, the surgeon's schedule is booked for the next 1.5 months so we're still waiting for a surgery date. Everyone agrees that 1.5 months is too long to wait so the cardiologist is talking to the surgeon daily to try and figure out how to fit Lincoln in to the schedule. They want him in within the next week or so.
We did ask if they'd ever consider fixing the kidney at the same time as the heart, that answer was no. Lincoln will have to be on heart/lung bypass for the surgery, which can create havoc on other organs, so they don't want to cause any other shocks to the system.
The surgical team also wanted more specific details about Lincoln's heart before going in to surgery. They wanted to perform a cardiac cath procedure. This involves putting a catheter in through the artery in Lincoln's groin and winding it up to the heart, and it happened on Wednesday. They measured the different pressures in different parts of the heart. I'm going to try to describe what it was they were checking, but I'm no expert so I'll just try to explain as best I can and the way I understand it.
The aorta is a vessel that takes blood into and out of the left ventricle of the heart (the difference between the left and right ventricle isn't important for this explanation). The aortic valve is part of the aorta and is at the spot there the aorta ends and the left ventricle begins - kind of like the door between 2 rooms. It opens and closes so blood leaves and enters the left ventricle when its supposed to. The issue with Lincoln's heart is that there is a membrane beneath the aortic valve. This membrane partially blocks the blood's path. Just like when you put your thumb over a hose and the pressure increases, the blood starts pushing through with more pressure. This is what they found with the cardiac cath. The pressure in the aorta is normal, the pressure at the aortic valve is higher, and the pressure within the left ventricle is higher still. This is bad for the heart so they need to remove that subaortic membrane.
There are also some abnormalities with the other side of Lincoln's heart, the pulmonary side. This didn't used to be the case so things are changing over there, but no intervention or real problems at this point.
They also checked the pressure going to his lungs and that was just fine.
Lincoln had to stay over night for observation after the procedure but did great and seems to be back to himself. Unfortunately he's running a slight fever and has a little congestion, my guess is it's something he picked up in the hospital, but is otherwise doing fine. The heart surgery can't happen if he has a fever so hopefully he gets better soon!
They did more blood work and his direct bilirubin was down to 18. But his INR, a measure of blood clotting, was higher (we wanted lower) even after his shot of vitamin K last week. The gastroenterologist said it was weird for one to improve and the other to get worse, and he would have rather seen the bilirubin stay where it was and the INR to improve. His liver isn't happy and it's just not acting the way they'd expect.
Lincoln's diagnosis is still a mystery. Some of his characteristics, particularly his liver, fit with Alagille syndrome. But his other characteristics only kind of fit, and the doctors want something more concrete. This is because his liver function has deteriorated enough that after the heart surgery they want him evaluated for a liver transplant. A panel of 9 physicians will evaluate him to see if they feel he's a good candidate. To be a good candidate he has to be as healthy as possible apart from the liver (the reason for completing the heart surgery first) and the majority of the panel has to be convinced that the new liver would fix the problem. They can't justify giving someone a new liver if it's possible the problem isn't the liver itself and the new liver would also fail. A confirmed Alagille diagnosis would go a long way in convincing them a new liver would fix the problem, so that's what we're hoping for.
He saw the ophthalmologist. They were looking for something called posterior embryotoxin. This is a thin line seen at the edge of the iris. It doesn't affect vision in any way, but is seen in 90% of people with Alagille syndrome and only 8-15% of the general population. Lincoln doesn't have it.
He had arm and leg x-rays. There are other disorders that cause bone/joint abnormalities so we were ruling those out. X-rays were normal.
He'll have a head ultrasound this week. I didn't ask what they were looking for here. I'll update once we know more.
To summarize, here's what we know so far:
The liver - Bile duct shortage, definitely consistent with Alagille.
The heart - Subaortic membrane is not common with Alagille syndrome. Issues with the pulmonary side of the heart are.
The kidney - Doesn't quite fit. Alagille related kidney issues are usually functional, where Lincoln's blockage is structural.
Facial features - Lincoln has a little bit of the prominent forehead common with Alagille's, but not the triangle shaped face or pointed chin
The eyes - Not consistent with Alagille
So that was our week. We're tired. A friend sent us dinner on Thursday night and I almost cried in relief. After spending the night at Children's on Wednesday I don't think we even had the energy to order pizza on Thursday. I really don't know what we'd do without all the love from our family and friends.
And thank you for all of the positive thoughts and prayers! Staying positive is the best thing we can do right now. Don't get me wrong, we're not saints over here. We have our moments when we scream and cry and are bitter and say this isn't fair and we can't do it any more. But we can't stay in that place - it doesn't get us anywhere. It's good for letting out frustration, anger and fear, but after that's done positive thoughts keep us moving in the direction of a solution.
Sunday, February 9, 2014
Progression
It's been a few weeks since I've given an update, so here's how the past few weeks have been going:
-More bloodwork, bilirubin up to 15 and clotting factor looking worse
-Increase Ursodiol dosage (liver medication), increase ADEKs dosage (vitamins) and start on an additional vitamin K supplement
-Lincoln goes on a hunger strike, not sure if it's related to the increase in medication dosage, but we suspect it is
-Kidney surgery moved up to Feb. 17
-Another liver ultrasound, blood flow to the liver is good
-Somehow Lincoln's growth is still on track, he is so amazing!
That gets us up to this past week. On Friday we had an echocardiogram, EKG and consultation with the cardiologist. They had originally wanted to sedate Lincoln for the tests, which we were against, so they agreed to let us try it without sedation. And it worked, phew! He wasn't exactly cooperative the entire time, but he wasn't terrible.
The original plan had been for me to head back to work after the echo and have John and the cardiologist call me so I could hear the update. I was getting ready to head out when the cardiology fellow came in, told us they were looking at Lincoln's echo, and asked me to stay. Never a good sign.
Lincoln has a piece of tissue growing beneath his aortic valve. They call it a subaortic membrane. We knew it was there and were hoping for no progression. Unfortunately, it progessed from mild/moderate to severe over the past two months. The doctor is concerned at the quick progression and wants to schedule surgery to remove the membrane, plus remove a little extra in hopes of getting rid of any faulty cells that like to grow there. While they're doing that, they'd also look at his valves and correct or replace any that have been damaged. The heart team has a weekly meeting on Tuesdays to discuss all the cases and we'll know for sure after that meeting, but the cardiologist told us to expect the kidney surgery to be postponed and heart surgery scheduled in the next month.
While we were at the hospital we also stopped into the GI clinic to have Lincoln's doctor take a look at his belly. The night before it had looked a little bigger than it normally does. The doctor told us to head down to get bloodwork and come back in a few hours for another liver ultrasound (Lincoln can't eat for four hours before the ultrasound so we had to wait). The bloodwork showed Lincoln's direct bilirubin up to 26 and that his clotting had worsened a little. So he got a shot of vitamin K to help with the clotting, still nothing we can do about the worsening liver function.
The liver ultrasound also showed that there is decreased blood flow to the liver. This isn't good, but there is a bright side. The liver folks think it's possible that the heart is putting stress on the liver and that could be the reason for the sudden worsening of liver function. It makes sense - both the heart and liver declined quickly over the past couple of months. It stands to reason that the two are related. The hope is that once the heart is corrected that stress will be taken off the liver and we'll be back to something reasonable. It wouldn't fix the liver, Lincoln still doesn't have enough bile ducts, but it could help.
John, Lincoln and myself are staying strong and positive. It's not easy, and we have our moments, but being sad and feeling sorry for ourselves doesn't get us anywhere. We ask that you continue to send Lincoln your prayers and/or good thoughts. We also ask that when you think of Lincoln please keep a positive outlook on your end as well. Feeling sad for Lincoln doesn't help him, but positive thoughts and confidence in him and his team of doctors does.
-More bloodwork, bilirubin up to 15 and clotting factor looking worse
-Increase Ursodiol dosage (liver medication), increase ADEKs dosage (vitamins) and start on an additional vitamin K supplement
-Lincoln goes on a hunger strike, not sure if it's related to the increase in medication dosage, but we suspect it is
-Kidney surgery moved up to Feb. 17
-Another liver ultrasound, blood flow to the liver is good
-Somehow Lincoln's growth is still on track, he is so amazing!
That gets us up to this past week. On Friday we had an echocardiogram, EKG and consultation with the cardiologist. They had originally wanted to sedate Lincoln for the tests, which we were against, so they agreed to let us try it without sedation. And it worked, phew! He wasn't exactly cooperative the entire time, but he wasn't terrible.
The original plan had been for me to head back to work after the echo and have John and the cardiologist call me so I could hear the update. I was getting ready to head out when the cardiology fellow came in, told us they were looking at Lincoln's echo, and asked me to stay. Never a good sign.
Lincoln has a piece of tissue growing beneath his aortic valve. They call it a subaortic membrane. We knew it was there and were hoping for no progression. Unfortunately, it progessed from mild/moderate to severe over the past two months. The doctor is concerned at the quick progression and wants to schedule surgery to remove the membrane, plus remove a little extra in hopes of getting rid of any faulty cells that like to grow there. While they're doing that, they'd also look at his valves and correct or replace any that have been damaged. The heart team has a weekly meeting on Tuesdays to discuss all the cases and we'll know for sure after that meeting, but the cardiologist told us to expect the kidney surgery to be postponed and heart surgery scheduled in the next month.
While we were at the hospital we also stopped into the GI clinic to have Lincoln's doctor take a look at his belly. The night before it had looked a little bigger than it normally does. The doctor told us to head down to get bloodwork and come back in a few hours for another liver ultrasound (Lincoln can't eat for four hours before the ultrasound so we had to wait). The bloodwork showed Lincoln's direct bilirubin up to 26 and that his clotting had worsened a little. So he got a shot of vitamin K to help with the clotting, still nothing we can do about the worsening liver function.
The liver ultrasound also showed that there is decreased blood flow to the liver. This isn't good, but there is a bright side. The liver folks think it's possible that the heart is putting stress on the liver and that could be the reason for the sudden worsening of liver function. It makes sense - both the heart and liver declined quickly over the past couple of months. It stands to reason that the two are related. The hope is that once the heart is corrected that stress will be taken off the liver and we'll be back to something reasonable. It wouldn't fix the liver, Lincoln still doesn't have enough bile ducts, but it could help.
John, Lincoln and myself are staying strong and positive. It's not easy, and we have our moments, but being sad and feeling sorry for ourselves doesn't get us anywhere. We ask that you continue to send Lincoln your prayers and/or good thoughts. We also ask that when you think of Lincoln please keep a positive outlook on your end as well. Feeling sad for Lincoln doesn't help him, but positive thoughts and confidence in him and his team of doctors does.
Saturday, January 18, 2014
Those stupid three steps forward
We had two big appointments this week – one with urology and
one with gastroenterology (the liver doctor).
First was urology…
We already knew that the renal scan showed 83% of the work
being done by Lincoln’s right kidney and that Lincoln was likely to need
surgery. The urologist confirmed. The left kidney is doing 17% of the work and 10%
is the urologist’s cutoff for function that is too low to salvage the kidney. But at 17% he thinks he can save it.
The urologist believes Lincoln has a UPJ (ureter-pelvis
junction) obstruction. Here’s a little
science lesson for you…The UPJ is the spot where the ureter meets up with the
kidney (the pelvis is part of the kidney, not the pelvis we’d usually think
of). When the ureter is formed it starts
at the bladder and grows up to the kidney.
While it’s growing it’s not a hollow tube yet – it’s solid. It becomes hollow later. A UPJ obstruction happens when the hollowing
out process for some reason isn’t complete.
The urologist fixes this by removing the part of the ureter that isn’t
completely hollow and reconnecting it to the kidney. The surgery has a very high success rate and
according to the urologist is relatively common in his world. The hope is for Lincoln’s left kidney to
return to full function. Lincoln goes in
for surgery on February 27th then will be in the hospital for
monitoring afterwards for 2-3 days. We’re
not exactly looking forward to the surgery, but are ready for it to be done and
over with so we can move on.
Then came the gastro appointment…you know what they say
about 3 steps forward and what they lead to…
Every time we have a liver appointment Lincoln gets blood
work beforehand. This past week the
blood work showed his liver function levels at the worst they’ve been to
date. You might remember that we were
hoping for direct bilirubin levels around 4 or so. His last test showed direct bilirubin a
little above 5. The highest he’s ever
had was around 8. This time we were
above 11. His ALT and AST (measures of
liver inflammation) had doubled since last month. We also had our first abnormal INR reading,
which is a measure of blood clotting and another test of liver function. Previously Lincoln’s liver hadn’t shown any
signs of enlargement or hardening (cirrhosis) when the doctors felt it, but now
it does.
So what now? We’re
back to blood work every two weeks instead of once a month. We’ve upped Lincoln’s vitamins and liver
medicine. The doctors will keep an extra
eye on the blood clotting factor, especially given his upcoming surgery. If it gets worse they can give him a shot of
vitamin K, since vitamin K is one of the vitamins Lincoln has a hard time
absorbing and also is a big factor in blood clotting. The INR reading wasn’t bad enough for the
liver doctor to put a stop to the kidney surgery. He'd like that kidney to be healthy sooner rather than later.
Now that we have signs of liver hardening and elevated
bilirubin we also had to have a discussion with the doctor about new things to
keep an eye out for. He told us when to
call him and when to get to the ER. He explained
that he wants the kidney fixed before the liver gets too bad in case Lincoln
ends up needing a liver transplant. It’s
better to be as healthy as possible in all other aspects when going into a
transplant. Yep, fun conversation.
Going through that appointment was an odd experience. On one hand, we’re a little bit used to
medical issues and appointments so we knew how to react. On the other hand, Lincoln was doing so well
that this felt like it came out of the blue.
I knew he was looking more yellow and had seemed like he wasn’t feeling
great for a few days before the blood work, so I wasn’t expecting his bilirubin
to have dropped. But I wasn’t expecting
11. That caught me off guard. I felt like our three steps forward was followed by four steps back.
But Lincoln seems like he’s feeling better today, he's still eating great
and has been smiling and laughing, so all we can do for now is keep hoping for
the best and keep having fun with him.
Through everything he just keeps being so much fun, we couldn’t have
asked for a better baby! Hopefully the increased liver medicine dosage helps things and we can get back to our baseline levels soon. Any good thoughts or prayers you want to send our way are appreciated!
Saturday, January 4, 2014
We survived the holidays...and a renal scan!
Lincoln had his first Christmas, and it was a blast! We didn't do any traveling on Christmas day, this was actually the first Christmas day spent in this house since I bought it. Lincoln of course didn't really know what was going on, but he did like the lights on the Christmas tree and he liked the jumper/bouncy thing we got him. He hasn't gotten the hang of actually jumping or bouncing yet but he likes just hanging out in it.
Lincoln got a ton of toys and cute clothes, he was spoiled! He's still getting the hang of a few of them, but there were some instant hits with a little elephant that makes a crinkly noise and a glow worm. It's so fun to watch him get excited to see his toys and start to figure out how to play with/try to eat them.
We did travel down to John's parents' house the weekend before Christmas, and I'm pleased to say that Lincoln handled both the 4 hour drive each way and staying in an unfamiliar house really well. We needed to pay a little extra attention to eating because of course he was distracted with all the fun people to play with. Nothing major though, and he met his intake goal both days we were gone.
Since Christmas we've had another weight check with the pediatrician, and Lincoln is still gaining weight since being off the tube! He's small for his age, around the 7th percentile for weight, but he's consistently in the 7th percentile. That means he's gaining weight at the same rate as a normal baby, and that's what we want. His height was a tiny bit of a concern, his percentile for height dropped a little. He can be short, that's not a huge deal, he just needs to be consistently the same level of short to show that he's growing properly. Alagille's kids do tend to be small, but it's not because of the faulty gene, it's because they have a hard time absorbing fat and vitamins so they have a hard time getting good nutrition. Nothing to get worked up about at this point, just something to remind us to stay diligent.
We also had a renal scan to check on the hydronephrosis of Lincoln's left kidney. The test took about an hour and a half from start to finish and Lincoln was a champ. He napped for part of it and just hung out while John showed him different toys on his phone for the rest.
To do the test they hooked him up to an IV so they could administer what they call nuclear medicine. Basically just medicine that can be followed through his body by the scanning machine. They monitor how long it takes for the medicine to go into his kidneys and then how long it takes to leave the kidneys. We meet with the Urologist on the 14th to officially go over the results and discuss next steps, but we know the test showed that 83% of the work is being done by the right kidney. This means there is a blockage in the left kidney and the last time we met with the Urologist he said if there is a blockage it would have to be surgically corrected. He also told us the procedure is relatively common in the urology world and has a great success rate, so that's reassuring. We're not happy about the results, but if surgery is what has to be done to get this little guy a small step towards better then that's what we'll do.
In other news, MBA classes for both John and I start on the 13th. We'll both have class two nights a week and are pretty nervous about how this is going to go. Luckily Lincoln's Aunt Maria and Uncle Brian are going to babysit one night a week so that will help a ton. Having a babysitter means we'll both be able to take a class on the same night instead of having to alternate class nights. This means we'll be able to see each other 4 evenings a week out of 7 instead of only 3. Honestly, I seriously considered taking another semester off. However, that would mean my loans would come due before I had my degree. I can't stomach that thought, especially since I only have 4 classes to go until I graduate. So the Spurriers are just going to stay energetic and positive and keep on keeping on! Wish us luck!
Lincoln got a ton of toys and cute clothes, he was spoiled! He's still getting the hang of a few of them, but there were some instant hits with a little elephant that makes a crinkly noise and a glow worm. It's so fun to watch him get excited to see his toys and start to figure out how to play with/try to eat them.
We did travel down to John's parents' house the weekend before Christmas, and I'm pleased to say that Lincoln handled both the 4 hour drive each way and staying in an unfamiliar house really well. We needed to pay a little extra attention to eating because of course he was distracted with all the fun people to play with. Nothing major though, and he met his intake goal both days we were gone.
Since Christmas we've had another weight check with the pediatrician, and Lincoln is still gaining weight since being off the tube! He's small for his age, around the 7th percentile for weight, but he's consistently in the 7th percentile. That means he's gaining weight at the same rate as a normal baby, and that's what we want. His height was a tiny bit of a concern, his percentile for height dropped a little. He can be short, that's not a huge deal, he just needs to be consistently the same level of short to show that he's growing properly. Alagille's kids do tend to be small, but it's not because of the faulty gene, it's because they have a hard time absorbing fat and vitamins so they have a hard time getting good nutrition. Nothing to get worked up about at this point, just something to remind us to stay diligent.
We also had a renal scan to check on the hydronephrosis of Lincoln's left kidney. The test took about an hour and a half from start to finish and Lincoln was a champ. He napped for part of it and just hung out while John showed him different toys on his phone for the rest.
To do the test they hooked him up to an IV so they could administer what they call nuclear medicine. Basically just medicine that can be followed through his body by the scanning machine. They monitor how long it takes for the medicine to go into his kidneys and then how long it takes to leave the kidneys. We meet with the Urologist on the 14th to officially go over the results and discuss next steps, but we know the test showed that 83% of the work is being done by the right kidney. This means there is a blockage in the left kidney and the last time we met with the Urologist he said if there is a blockage it would have to be surgically corrected. He also told us the procedure is relatively common in the urology world and has a great success rate, so that's reassuring. We're not happy about the results, but if surgery is what has to be done to get this little guy a small step towards better then that's what we'll do.
In other news, MBA classes for both John and I start on the 13th. We'll both have class two nights a week and are pretty nervous about how this is going to go. Luckily Lincoln's Aunt Maria and Uncle Brian are going to babysit one night a week so that will help a ton. Having a babysitter means we'll both be able to take a class on the same night instead of having to alternate class nights. This means we'll be able to see each other 4 evenings a week out of 7 instead of only 3. Honestly, I seriously considered taking another semester off. However, that would mean my loans would come due before I had my degree. I can't stomach that thought, especially since I only have 4 classes to go until I graduate. So the Spurriers are just going to stay energetic and positive and keep on keeping on! Wish us luck!
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