Lincoln videos

I thought I'd post a couple of the recovered Lincoln videos, because they're just so cute! I wish I could post more, but there is a 100mb limit for a single video and the others exceed that. Sorry family, I can show you next time I see you! Also, I can't figure out how to rotate them so some are sideways.

But they are pretty cute! Love and miss this little guy so much every day.

This one is Lincoln at just over 4 months old...

This one is just over 4 months, too, bouncing with daddy...

And here's Lincoln chewing his Sophie giraffe at almost 5 months...

And this is Lincoln at about 7 months, all gross and covered in green beans. I remember wondering if all babies try to sleep eat like this...

Thank you!

The Lincoln Classic was Sunday and I can’t thank everyone enough for making it a success. Thank you Maria and Brian. You’re amazing, and a force to be reckoned with. Unstoppable. Thank you Kerri, Alison, Kristy, Lindsey, Andi, Missie, Bri, Erin, Kelly, Charissa, and everyone else who made the Lincoln Classic possible. For asking businesses for gift cards, for putting together raffle baskets, for sending donations, for taking time out of your day to come play golf and have dinner with us. Thank you John and Brian for doing the public speaking when I couldn’t pull it together to say it myself. Thank you!

We received so much support and love and it’s humbling. The donation amount is not yet final, pending final invoice from the Hamilton Elks (a beautiful golf course, by the way). But it’s safe to say we’re over $8,500! We were told by folks at Children’s that this was not a small event, and it’s not a small amount of money to have raised. We agree! I want to take a moment to tell everyone who participated what you’ve done.

1)  You’ve raised money for the Gastroenterology, Hepatology & Nutrition Department at Cincinnati Children’s Hospital Medical Center. Why is this such a big deal? I have a friend who is always saying “Money buys science, and science saves lives.” I believe that wholeheartedly. By giving resources to the folks at CCHMC you allow them to keep poking and pushing at their boundaries, making them question why the boundaries are there and how to get around them. The more they poke, push and question, the better they get at saving babies like Lincoln. And babies like Lincoln need saving.

2)  You reminded Lincoln’s family that we’re not alone. The grief of losing a child is isolating because it’s a pain most can’t relate to. Which is a good thing. We don’t want anyone else to know the kind of pain we know. But it’s lonely. Seeing so much support from friends, family and even folks we’ve never met reminded us we’re not alone in our grief. People are still standing by us, loving Lincoln.

3)  You allowed us the opportunity to continue to parent Lincoln. The thing is, when you lose a child you don’t lose the need to take care of them. That ache in your heart that says you need to watch over your baby and make sure they’re ok? Still there. But you lose the ability to take care of them the way your heart tells you to. It’s an incredibly helpless feeling. Make no mistake. Maria and Brian did the bulk of the work for the Lincoln Classic. But John and I were able to pick up small pieces of the work and it let us feel like we were doing something to take care of Lincoln. We were honoring him and helping people remember him and it took away a piece of that helplessness.

These things are a big deal. Thank you. You’re amazing. We’re already looking forward to next year’s Lincoln Classic!

Lincoln Classic 2014 - Links for Lincoln

We are so incredibly excited about Lincoln's upcoming event. Lincoln's aunt and uncle, Maria and Brian, have decided to put together a golf outing in his name. They are amazing, and are working incredibly hard to make sure this event is fun for everyone. It's in September, just a little over a week before Lincoln's birthday. John and I expect September to be a pretty difficult month, but this gives us something positive to focus on. Did I mention we're excited??

Proceeds from the golf outing will go to Cincinnati Children's Hospital Medical Center in two forms. Most of the funds will go to the Gastroenterology, Hepatology and Nutrition department (the liver department). Lincoln got help from many departments during our stay there, but the Gastroenterology department was his "home base" and his core team. Our goal is to raise enough money to get Lincoln's name on a wall or plaque somewhere at Children's. He was a really important little guy and a big deal, so that is the very least we can do!

A smaller portion of the funds raised will be allocated to the Child Life department. This is the department providing kids with toys/movies/etc. to use during their stay at the hospital. We're going to buy several "star gazer" machines that Lincoln loved. They're small projectors that display "stars" on the ceiling and walls. They're a bit of a hot commodity because they're one of the more expensive items offered by Child Life so not many are available. In fact, the only time one was available to Lincoln was in the Pediatric ICU. They weren't available on the Liver floor so he didn't get to have one in his regular room there. We were going to buy him one as his coming home present. Since we never got that chance we're going to purchase several that will be specifically for kids on the Liver floor. This is what they look like:
http://www.amazon.com/Laser-Stars-Indoor-Light-Show/dp/B000VBNIP2/ref=sr_1_1?ie=UTF8&qid=1406986044&sr=8-1&keywords=star+light+show

The golf outing offers two options.
1) Golf and dinner. This option is $100, which includes a donation. Singles to full teams are welcome! If you don't have a full team, no worries. We'll be pairing individuals to create full teams. It's a scramble, so no pressure if your golf skills aren't at Tiger Woods level.
2) Dinner only. This option is $25, which includes a donation

The outing will take place at the Hamilton Elks Golf Club at 4444 Hamilton Middletown Road in Liberty Township, Ohio. Shotgun start at 1pm.

Before/during dinner there will also be a raffle. Raffle tickets will be available for purchase, and raffle items have been donated so all raffle proceeds go toward the donation. There are some great items being raffled off! Bengals tickets, Reds tickets, Xavier items, wine and food baskets, restaurant gift cards...it's going to be a lot of fun! And for anyone who likes the photographer who took mine and Maria's wedding photos and the photos taken of me, John and Lincoln on Lincoln's last day before he became an angel, our friend and favorite photographer Tracy Doyle has donated one photo session to the raffle.

I'm sorry I can't attached the flyer or sign up sheet, but both are available here: https://www.facebook.com/#!/lincolnclassic?fref=photo

You can also contact Maria or Brian Huberfeld:

Brian Huberfeld
bhuberfeld@yahoo.com
513-535-6482

Maria Huberfeld
lachermaria@yahoo.com
440-622-0640

This event is going to be a lot of fun, and we hope to see you there!

The Autopsy

We met today with Lincoln's liver doctor to discuss the autopsy results. He told us...not much we didn't know. Everything they thought before was true. Lincoln did not have biliary atresia, the ducts outside of his liver were in place. He had a shortage of bile ducts inside his liver, just like his biopsy he had when he was just a few weeks old showed. His liver was scarred, as the doctor expected. The only unexpected thing about Lincoln's liver was the fibrosis in his liver wasn't what they normally see. I'll try to explain this without butchering the medical profession...Instead of the fibrosis (fibers that form when the liver is scarred) being kind of uniformly going from one particular spot in the liver to another and leaving empty space in the middle, Lincoln's fibrosis was more scattered across that empty space. The doctor said he's seen this before but it's not what they typically see. He has no idea what it means, if anything, other than Lincoln's liver didn't act like anyone expected. But we already knew that.

Lincoln's heart had regrown tissue below his aortic valve and his aortic valve was smaller than it should have been. His left kidney was smaller than it should have been and his right had grown larger to compensate. His lungs showed signs of previous trauma, likely from his fluid retention and difficulty breathing leading to previous trips to the PICU. But the main takeaway was that there wasn't anything they missed while he was still alive. And nothing to provide a diagnosis, other than to verify the "Alagille Syndrome like" symptoms.

The next step is to do genetic mapping. This will involve comparing Lincoln's DNA to mine and John's to see if there are any abnormalities in us or if Lincoln has anything genetic he couldn't have gotten from us. A big question we have is why this happened and whether it would happen again if we have more kids. But unless they find a genetic "smoking gun" they can't answer that question. The doctor said when multiple organs are involved it's almost always "syndromic", or part of an underlying syndrome, which is almost always genetic in some way. That doesn't necessarily mean it's something inherited. Lincoln could have had a random genetic mutation he didn't get from me or John. But it means it was likey in his genes one way or the other. The gene mapping provides a definitive answer 30% of the time and a tentative answer 20% of the time. It takes about 6 months.

My worry of course is that it wasn't something genetic and it's something I did, a random infection maybe. If that is the case we'll never know. I know I shouldn't blame myself, and the doctor and John tell me it's ridiculous to think that. But I think moms are prone to worrying it's their fault. I mean, I grew him, I was supposed to keep him safe. The doctor told me again today it is hugely unlikely it's something I did, but I think unless they find something genetic I'll always wonder. Having something genetic to blame wouldn't necessarily make me feel better, because at the end of the day Lincoln is still gone. But at least I could stop second guessing myself.

Music to my ears

“The sound of my baby's name may bring tears to my eyes, but it is music to my ears.”

I don’t know whose quote that is. I found it yesterday. It’s not mine, but it fits.

I want to start by saying thank you to everyone. It’ll never be enough but I want to say it anyway. I thought about writing thank you notes so everyone who has supported us would know how much we appreciate them but I just can’t find it in me. I wouldn’t know what to write. So for every gift, card, kind thought, dinner, everything, thank you.

If you dropped off dinner and saw the truck in the driveway, we were probably home and couldn’t bring ourselves to face anyone. We probably forgot to thank you. You didn’t get mad or judge us. Thank you for that. It’s still hard to do “normal” things like go to the grocery store or cook and you made it so we didn’t have to.

If you sent a card, email or text you brightened our day by reminding us you care. We might not have sent a response because we’re not really quite with it just yet. You didn’t take it personally or expect anything from us. Thank you for that. They say you find out who your friends are during hard times and I think that’s true.

If you sent money to our family you helped us find Lincoln a beautiful resting place without the financial stress it would have caused otherwise. We find so much comfort in honoring him with his spot and knowing John and I have resting spots right next to him so he won’t be alone. Thank you for that.

We know people wonder how we are and want to know if we’re ok. We’re kind of ok, or some new version of ok. We’re back to work/school/starting a business. I won’t speak for John but personally, some days I’m ok and some days it’s about all I can do to put one foot in front of the other and get through the day. But we’re still here, still love each other, doing the best we can. We try not to be hard on ourselves. If some nights all we have the strength to do is lie on the couch, hide from someone bringing us dinner, and watch TV then that’s all we do.

We also know people wonder if we have any answers about what caused Lincoln’s health troubles. We don’t yet. There was one more genetic test for Alagille’s Syndrome outstanding but that came back negative. That means it either wasn't Alagille's or Alagille's is connected to a gene they haven't linked it to yet. We meet next week with Lincoln’s liver doctor to discuss autopsy findings and next steps. When we were still in the hospital they told us they’d probably do genome mapping for myself, John and Lincoln. My understanding is it would compare Lincoln’s entire DNA to ours to see if anything looks out of place. Everyone has genetic mutations that usually don’t cause any problems, but it’s possible he had one that caused all this. They said 30% of the time they find something definitive, 20% of the time they find something that might have caused problems, and 50% of the time they don’t find anything. So we’re not really counting on getting answers but are hopeful.

Finally, we know people sometimes don’t know what to say to us or are afraid to ask questions. It’s ok. You can ask anything you’d like. Please don’t feel like you have to hedge around the topic to keep from upsetting us. You won’t upset us by talking or asking about Lincoln. We are proud parents and love to talk about our son. Sometimes I might cry, but don’t let that bother you.

“The sound of my baby's name may bring tears to my eyes, but it is music to my ears.”

Visitation & Service

Lincoln Lancaster Spurrier

Age: 0

Born: September 16, 2013

Died: May 14, 2014

Obituary

Lincoln Lancaster Spurrier.         

Born Sept. 16, 2013; passed away May 14, 2014. He is survived by his parents, John Lancaster and Melanie Kathryn Spurrier. Lincoln was a courageous angel who got his wings too early. He is loved and missed, and touched more lives than he ever knew. His smile lit up a room and will continue to shine from heaven. Visitation will be held on Mon., May 19, 2014 from 9:00 am to 12:00 pm with service immediately following at the Gwen Mooney Funeral Home of Spring Grove Cemetery.

Our Angel

Wednesday, May 14th was the day our angel got his wings.

John and I both stayed at the hospital the night before, and slept in until 8am or so. I used to get up earlier so I could have my teeth brushed and be ready to participate in daily rounds, but I hadn't participated in a few days. There didn't seem to be much point, so we just got the summary from the nurse. There wasn't anything new.

The nurse disconnected the CRRT a little after 10 am. They removed one of two PICC lines, then John and I gave Lincoln a bath. Then the ventilator was removed. Lincoln was on high flow oxygen for a few minutes to make sure he was stable enough to go outside, then on a lower flow nasal canula. About 20 minutes after the ventilator was removed we headed outside.

John and I got to take our baby outdoors. Last time we were home it was February and cold, so it had been awhile since we'd been able to. John got to put Lincoln's feet in the grass, and we think Lincoln liked it. He was sedated, but his feet curled around the blades of grass and he didn't pull away. John walked him around, letting him touch leaves and rocks, and showing him just a tiny bit of what we had wanted to show him in a lifetime. I'm unbelievably grateful that we were able to get some beautiful photos of our family.

Being outside with Lincoln was bittersweet. It was wonderful to be with him with no tubes or lines. But in order to keep him comfortable he was pretty sedated, and was clearly sicker than he had been two weeks prior.

We spent the rest of the afternoon in Lincoln's hospital room. Lincoln and I took a nap together and I think it was the most peaceful rest I'd had in a long while. All three of us laid in bed for awhile, then just Lincoln and I again. We watched TV, something we hadn't done in weeks. It was wonderful to just lay next to my baby, with my arm around him and giving him kisses.

John switched places with me and got into bed with Lincoln while I went down to the cafeteria to get some dinner. About 5 minutes after I got back, with his mommy and daddy holding him and rubbing his head, he took his last breaths. We think he was comfortable, and we don't think he realized what was happening. We knew before the doctor came in to check that Lincoln was gone.

We stayed in the room with Lincoln until the nurse came to prepare to take him away, which took about an hour and 20 minutes. During that hour and 20 minutes the nurses and John packed up the room. I wasn't able to do much but sit in bed with Lincoln. I wished we had packed earlier. I spent most of the hour and 20 minutes just staring at Lincoln, wishing it wasn't the last time. I know I'll see him at the funeral home, but I'm afraid by then his skin color will be different. I'm afraid he'll be pale, not his usual "liver kid" bronze, and I'll hardly recognize him. I stared at him, smelling his head, kissing his fingers and cheeks, hoping I could somehow stamp the feel of him into my mind. The nurse removed his second PICC and helped me get him into his pajamas. Blue striped with a little lion on them.

I'd wondered how it would feel to leave the hospital for the last time. The chaplain took us out the back way so we didn't have to pass anyone in the waiting room, but otherwise we left like we would have left any other day. Walking through the lobby I'd walked through so many times on my way to my peanut was heartbreaking. Luckily we have some great friends who came took our things to our house, and who met us at the house so we didn't have to be alone.

We miss Lincoln so much already, and I know that will never go away. He made us better people. He was the best baby anyone could ask for. Taking care of him was never a burden, not for a minute. I am honored to be his mom and I know John is honored to be his dad.

The post I hoped I'd never have to write

I've spent a lot of time looking for blogs written by parents with "liver kids". I wanted to know how their kids were doing compared to Lincoln, how they did post-transplant, how parents felt about the whole process...I felt it helped me prepare for different paths we might take. Inevitably I'd find blogs with posts written when kids weren't doing well. I'd read through the posts, hoping the kids would get better, and I'd come across that post where the parents explained that there was nothing more the doctors could do. I hoped I'd never have to write that post.

But here I am. There's nothing more the doctors can do. Weekend before last Lincoln's kidneys stopped working and he started holding onto too much fluid. It wasn't a ridiculous amount, something his heart should have been able to handle, but his heart didn't handle it well. His heart rate and blood pressure dropped. He had to be intubated. The doctors all met at their weekly transplant meeting the following Thursday and discussed how now that Lincoln was on a continuous dialysis machine (Continuous Renal Replacement Therapy, or CRRT) his heart function was back to how it was before this episode, but they now realized that it wasn't strong enough to make it through a transplant. It also isn't strong enough to make it through another heart surgery. His doctors tried. They talked about combining heart surgery and liver transplant, kidney transplant, everything they could think of. Things that had never been done, but they were willing to explore in every effort to save Lincoln. They felt every option they thought of was futile, and would at best cause Lincoln to die on the operating table and at worst make him suffer before eventually passing away after a surgery.

John and I were first given the choice to preserve as much liver function as possible or to give him any medication necessary to keep him comfortable. We went with comfort. We were then given the choice whether leave him on the respirator and CRRT or take him off. If we left him on he'd eventually pass from liver failure or, more likely, an infection. Then the doctors would be reacting instead of planning ahead, and his pain would be more difficult to manage.

So tomorrow around 10am Lincoln will be disconnected from the CRRT. They'll disconnect all of his lines, clean him up, then remove the breathing tube. Since his kidneys aren't working he'll start to hold onto fluid, and will pass from respiratory failure, although we don't know how long that will take. We asked the hospital to let us take Lincoln outside, somewhere we can sit with him in the grass (assuming it doesn't storm on us). The hospital has been great and is making that happen. We'll also have our friend and photographer Tracy Doyle with us to hopefully get some photos of our precious little man.

The doctors don't know how long it'll be before Lincoln starts to struggle to breath. They don't actually know how well he'll breath on his own once the tube is removed. The PICU attending doctor thinks Lincoln will make it through pictures and his trip outside, but other than that the day is unclear. They said maybe hours, maybe a day or two. No more than two days. We had hoped Lincoln would be able to wake up and look at us, but we won't know until tomorrow whether that'll be possible while still keeping him comfortable.

Lincoln has a pain management team that will be with him when we go outside to make sure he doesn't suffer. They said that families get distressed watching kids in this situation struggle to breath, but that the families don't believe the kids suffer. He'll be on medication that doesn't let him realize he's having a hard time breathing.

The conversations we've had to have over the past week are surreal. We sit there, me usually with a cup of coffee or water so my hands have something to play with, and talk about things we never fathomed having to discuss. Things like how our son will die, and where we want him buried. Whether we want an autopsy. Whether I want to donate the breast milk we've stored. Usually at some point during these conversations I kind of zone out and think about how I can't believe this is really happening and I'm really having these conversations.

Thank you to everyone who's visited, called or sent messages during this journey. You've helped us so much, and we appreciate it.

We're feeling whatever it is you feel when you go past devastated. And the kind words, thoughts and prayers help keep us afloat.

Listing

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!

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!

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.

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.

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. 

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.

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.

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.

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 27^th 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!

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!