This entry may ramble a bit, but I’m going to try recapping the events of the last week.
Last week Sam was scheduled for exploratory abdominal surgery. Prior to this, his respiratory requirements shot up and he was placed back on the ventilator. His oxygen requirements kept creeping up.
As we got closer to his scheduled surgery, it became pretty obvious that he wasn’t going in. The doctors kept looking for signs of infection, sending off blood samples and secretion samples every day, sometimes twice a day. Nothing ever came back positive. But his CRP number was slightly creeping up, and his platelets were dropping, and his oxygen was pretty high.
On Monday Scott asked his doctor if she thought his recent respiratory requirements were cardiac related. She didn’t think so but thought it a valid question, ordered another echo. At this point, we were still waiting to see if Sam would turn it around and he’d still have the belly surgery. Yes, it’s risky, but he needs it. The echo came back and the results weren’t good.
The echo showed Sam’s pulmonary hypertension, which we knew about, but also something more dire: pulmonary vein stenosis. At our meeting on Tuesday, we learned all this, learned that the prognosis wasn’t good. The stenosis isn’t really treatable, is very rare, is recurring. The current treatments available are short-lived. Crap o crap. The cardiologist admitted that she didn’t understand how Sam was doing as well as he was; she freely admitted his condition made no medical sense that she could determine. Part of me was proud since nothing about Sam has been per medical expectations, but another part was scared to death. Still is really.
That night, the next day we started doing research; unfortunately there’s almost nothing out there. A big thanks goes out to Maureen, Paul, Tia and Jamie for also looking, especially Maureen for sending me studies and abstracts!!! This disease is just so rare and so little understood that anything out there tries to determine the cause, not how to treat. Only one hospital even claims to have a program for this, in Boston, so definitely not close. They’re also doing a clinical trial using chemotherapy.
In some of our own, we saw a study that said PVS in 1-2 veins had a 0% mortality rate (awesome!), but in 3-4 veins had 85% mortality. Later the same day all our hope was crushed. We had previously understood Sam had PVS in just 2 veins. Scott talked to the doctor on duty that day, who had talked to the cardiologist to confirm everything before talking to Scott. The doctor told Scott that Sam had PVS in all 4 veins. All hope gone, absolute devastation. My visit at the hospital that evening was just heartbreaking. I held Sam, cradled him, talked to him, kissed him, asked him to keep fighting, to not give up, that Mommy and Daddy were trying to help him. As I was leaving, I ran into the doctor from the previous day. I cornered her, demanded to know how severe the PVS was. She said she understood it was just his left side. Wait, what?! We were told today it was both sides. No. That small glimmer of hope returned. Immediately I called Scott to tell him, to also give him that same hope.
The following day we confirm, the PVS is only on the left side. Prognosis still isn’t good; Sam’s oxygen requirements are going up. We talked to the other cardiologist on duty. We weren’t impressed. He said there wasn’t anything to be done, that no other facility had viable treatments. We brought up the chemo study in Boston, and he acted like he’d never heard of it. Our faith in the cardiologists was quickly diminishing.
Meanwhile his doctor decides to focus on getting Sam stable, increasing his calories, trying to get him big enough to be eligible for any type of procedure for his heart. The next morning I start to question him: are we trying everything, is there something we’re not considering, something we’re missing. He assured me that every doctor there discussed Sam multiple times a day. I asked him to try harder.
Later that day when we returned to the hospital, Sam’s oxygen parameters couldn’t have been more different. Prior to this visit, they had been keeping his oxygen between 50-70, and he was barely keeping his oxygen saturation in the 90s. This visit, his oxygen was at 28, and his saturation was in the 90s. What is the world is going on!? The nurses explanation didn’t make sense, so we waited till the next morning when we could speak to the doctor again.
That day, the doctor called the cardiologist on duty, and they got to talking. Sam’s doctor theorized that maybe keeping his oxygen requirements so high was actually making things worse, that increasing the pressure wasn’t helping. After all, the veins in his heart are narrowed, so no matter how much blood is pushed through at a higher rater, the same amount is going to go through. So by lowering the requirements, lowering the pressure, the blood still goes through just fine. This explanation is horrible — but I’m still wrapping my head around it all. Basically now they’re keeping his oxygen requirements as low as possible, treating the hypertension instead. They can’t treat the PVS, so they’re treating what they can. And so far it’s working.
As the title of this post is, there’s always something.
On Monday night, after I demanded the doctor on duty allow me to hold Sam (sidenote: this hospital doesn’t allow vented babies to be held, but considering Sam’s condition, I’ve been demanding it. Yes it takes a lot of work, it’s risky, but the poor baby needs to be held, needs to know he’s loved dearly. Most doctors/nurses have been on board. The ones Monday night weren’t — but I put up a stink and told them to make it happen), I called around 4am when I was feeding Sebastien to get an update. The nurse couldn’t come to the phone because she was busy with Sam — he’d pulled out his IV. My first thought was to chuckle…that’s my Sam, he’s so strong. A couple hours later we get a phone call. My first thought was, ‘crap, he extubated. but i held him hours ago, so they can’t blame me for that.’ Nope. When he pulled out his IV, he lost a lot of blood. A LOT. I’m not sure what happened exactly, or why it took so long, but I can only assume the nurse had his top down on his bed and wasn’t paying attention. He lost way more blood than anyone his size should ever lose, probably close to actually bleeding to death. He had to get red blood cells, plasma, platelets, other fluids to compensate. He was also given Lasix to help him pee off the excess.
After that crazy night/early morning, Sam still had to have his eye exam (doing fine, responding well to the laser surgery). In addition, he had another echo. The cardiologist expected the pressure to be much higher because of all the fluids he was given; his doctor hoped it would be lower. His doctor won; his PVS was better! How is that possible?! No one has an explanation.
All the cardiologists at the hospital and the pediatric cardiac surgeons sat down and discussed Sam. They can’t figure out how or why he’s doing so well, but they’ve decided that they can and will support him. They want him to get the abdominal surgery, get stable again, and then they’ll work on the cardiac issues. (side note: we requested that the doctors here contact Boston, consult with the folks up there. We also wanted to know if Sam would be a candidate for the chemo study. The doctors here think that Boston would take him in the study, but they’re sending more information so that the Boston people know exactly what type of patient they’d have. Regardless, at least the consultation is happening, maybe the other doctors will have insight — we aren’t to the point where we’d want to pursue chemo, but we need to know all available options). A complete change for them. The neonatologists are now talking to the general pediatric surgeons (one in particular), and they’ll discuss with the anesthesiologists to determine when Sam is ready for exploratory bowel surgery. Everyone has to be on board.
So here we are now, working to stabilize Sam. After all the blood and fluids yesterday, his body needs to sort of return to normal. His respiratory issues need to stabilize. Everything needs to be in his normal ranges. He might get another upper and lower GI. Then we’ll go in, figure out what’s wrong with the belly, fix what needs fixing. Then we’ll stabilize again. Work on the heart issues. From my understanding, we’d first try the catheters, try to expand the veins. From there, I don’t know, I can’t think that far ahead yet.