Mostly Bad Results

Roscoe's big day of procedures came to an end with some good news, but mostly bad news. As an overview, he got the lung scan ("perfusion scan") done from 10-10:30am, then the cardiac catheterization from 3:30-7:00pm. They skipped the bronchoscopy because he had some stability problems during the cardiac catheterization (referred to as the "cath" from now on to save on typing). Read below for the detailed results. It looks like we are facing a dangerous surgery as our only option.

We took Roscoe to the perfusion scan room which contained a long x-ray machine which looked similar to a miniature MRI machine. They injected Roscoe with some radioactive material dissolved in saline solution, so that they could trace the radioactive material travelling through his lungs. The tech accidentally spilled a few drops of the solution onto the floor, so he had to check all of our shoes with a Geiger counter before we left. The scan of Roscoe's lungs only lasted 1-2 minutes, but it showed much more circulation going to his right lung (the good one) than to his left (the one with pulmonary vein stenosis). I only saw the picture, so when they schedule a family meeting to go over the details we'll hopefully find out how abnormal his flow was.

Roscoe getting transferred from his crib to the machine

Roscoe in the machine getting scanned by x-rays

The on-screen output showing the circulation through his left and right lungs (bottom two small patches) and his brain (faded upper patch). The top image is from one side and the bottom image is from the opposite.

Viewing Roscoe through the center of the scanning machine

The Geiger counter they used to scan for radioactive material on the floor 

Roscoe returned to his room and played a little before the cardiac cath. Each passing day he has grown more alert, so it was great to see the old Roscoe start returning. Before he went to the cath lab, they moderately sedated him for transport. Once we arrived at the cath lab (which we first visited back in February) they gave us an overview of the process, got him settled, and then gave us a pager to let us know when the procedure was progressing.

Transferring Roscoe from his crib to the cath lab table

Cath lab equipment, looks super high-tech

Dr. Moore holding Roscoe's pacifier as he explained the cath process to us

While the procedure was happening, we drove to Fisherman's Wharf to get fish & chips and pizza for an early dinner, since normally we wouldn't be able to travel that far. They paged us right as we finished, so we headed back and waited anxiously for the results. They let us know that they'd foregone the bronchoscopy, but didn't say why over the phone.

Dr. Moore met us in the hallway to explain the results of the cardiac cath. Going into the procedure, he expected to see similar results to the first cath he'd done in February, where with one of Roscoe's two left pulmonary veins exhibiting stenosis (narrowing). When he did the cath, he found that both of Roscoe's left pulmonary veins were now exhibiting stenosis. Not only that, but the stenosis had gone from mild/moderate in February to moderate/severe in June. He attributed this to Roscoe's body growing at a rate that was faster than these pulmonary veins. This observed data puts a time limit on Roscoe's ability to survive in his current state, because eventually he will reach a point where his right lung will cease to circulate blood back to the heart (which will cause all sorts of issues, most likely heart failure).

To put numbers on it, the diameter of Roscoe's left pulmonary veins as they enter his heart are both roughly 2mm, when they should be 2.5mm (upper) and 5-6mm (lower). This means that his set of pulmonary veins is only able to deliver about 50% of the blood flow that it should be delivering. It also causes a pressure differential of up to 5x the normal blood pressure that should be in his lungs (pulmonary hypertension). His heart has to work against that higher pressure, which makes the 5x blood pressure a threat of heart failure. As Roscoe continues to grow bigger, his body's needs for oxygen will increase, but his ability to deliver oxygen will either stay the same or decrease.

Dr. Moore said that right now Roscoe's left pulmonary veins are too small to expand with either a balloon or stent. They have stents that will fit, but since they are so small (a wire mesh tube that's ~1mm in diameter) the stent would have limited expandability and couldn't grow with him to support him long-term. Dr. Moore's opinion that the only option to resolve Roscoe's pulmonary vein stenosis is surgery. He explained that surgery would carry significant immediate risk due to Roscoe's instability (ie: there is a chance Roscoe wouldn't survive the surgery). He also said that even if successful, the surgery only works as a long-term solution 50-60% of the time. The rest of the time, the surgery site grows scar tissue and re-narrows the vein opening. We prayed for the doctors to come to the best solution for Roscoe's long-term health, but right now it feels like we're backed into a corner with no other options to save his life.

Right now you might be asking, "So what's the good news?". The good news is that Roscoe's pulmonary vein and vessels don't seem to be narrowed except where they enter the heart. This is good, because it allows for the surgical option and localized treatment. If the veins or vessels were completely narrowed, Roscoe would have no option for long-term treatment. Also he said on multiple occasions that Roscoe is "tough", which is what he'll need if we move forward with the risky surgery. The last piece of good news is that a few hours after the surgery, Roscoe was active and kicking, despite having been briefly paralyzed a few hours prior. He was smiling even more than usual, which gave us a chance to snap this picture as he laughed with Alisha:

The neonatalogist was concerned about Roscoe's pulmonary hypertension causing heart failure over the next 18-24 hours, because the pulmonary hypertension escalated due to the agitation of the cardiac cath (some escalation was expected, but it went further than they thought). She did say it was a great sign that he came out of the paralysis so quickly and was so alert. The neonatalogist added yet another sensor to Roscoe's arsenal of sensors. This time, it's a forehead strap that measures the oxygenation in his brain. If heart failure occurs and they are unable to get blood pumped through his lungs due to the hypertension, this reading will let them know how long Roscoe's brain will have gone without oxygen (brain damage). For now, it just looks funny:

Now that UCSF has all of this information gathered, they will find a time when Alisha and I can meet with all of the experts involved (Dr. Moore for the catheterization, Dr. Fineman for the pulmonary hypertension, Dr. Cohen for surgery) along with their teams to discuss the data and risks of surgery or other treatments (of which I'm not sure there are any). Hopefully this meeting will occur sometime on Friday. Once UCSF arranges the meeting, Alisha and I will know more about the risk level of Roscoe having open heart surgery (90% survival vs 50% survival vs 10% survival), how bad his current state is if we choose to avoid surgery, and any other questions that might arise. It looks like we'll be at UCSF for at least another 1-2 weeks, since they'd want Roscoe as calmed and stable as possible if the surgery were to take place.

One final note: Please do not send text messages or phone calls to Alisha. Messages like "We are praying for you" or "How is Roscoe doing?" are just reminders that her baby is in critical condition in the hospital. Since she always has her phone on her, messages which might be intended as encouraging actually just remind her of the scary situation we're in (when she might be out on a walk, trying to clear her head). She is under a considerable amount of strain having to process all of this removed from the comfort of her home. If you feel like texting/calling, you can try my phone but I most likely won't answer. :)

We tried to implement some stress relief by constructing our own ice cream sandwich from two cookies and ice cream in the UCSF cafeteria