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.
Saturday, February 22, 2014
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.
Subscribe to:
Posts (Atom)