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!