Purgatory

This won't be a fun blog post. Usually I try to keep blog posts upbeat, but this won't be one of those. By reading this, you'll be on the "weep with those who weep" (Romans 12:15) side of things. In short, the past few months of breathing progress that we've seen were somewhat of an illusion. Roscoe was making visual progress, but in ways that hurt his long-term progression. Now he is back where he was in February.

Alisha and I had a meeting with Roscoe's neonatologist (day-to-day doctor), pulmonologist (lung doctor), and cardiologist (heart doctor). This was the first time I remember getting to have all of them in the same room together to ask questions. The subject of discussion was the future plan for Roscoe.

Roscoe has two main issues as I described here previously. These issues are inter-related and effect each other since they deal with his lungs' function of delivering oxygen into his bloodstream:

1. Chronic Lung Disease (lungs which don't work properly due to damage or immaturity)
2. Pulmonary Vein Stenosis (narrowed vein that feeds blood from the lungs to the heart)

Both of these issues have the side effect of pulmonary hypertension (high blood pressure in his lungs). This is a dangerous side effect because it puts extra strain on his heart, because his heart has to push against a greater pressure to get the same amount of blood back from his lungs. Ultrasounds have already shown that his heart's right ventricle wall (which pushes blood to his lungs) has thickened due to the added pressure. In adults this is not reversible, but it might be reversible in kids as small as Roscoe.

Roscoe has been taking Bosentan to make the blood vessels in his lungs expand and grow at an increased rate. Expansion of his lung capillaries (tiny veins in the functional surface of his lungs) is the growth that's needed to resolve issue #1. Bosentan won't resolve issue #1 all by itself, because Roscoe's body still needs to produce growth on its own. Breathing support machines like nasal cannula, vapotherm, and CPAP also help to produce this growth. Issue #2 will only be resolved by growth or a stent (or surgery if he gets big enough).

Roscoe has a 6-month follow-up appointment at UCSF in late July. At that appointment, UCSF will perform another cardiac catheterization to see how much his lungs and pulmonary vein have grown since the first appointment. The main doctor doing this work will be Phillip Moore. It is possible that UCSF may choose to insert a stent, depending upon their findings and the complexity of doing so. The Kaiser cardiologist said that Dr. Moore is one of the best in the world at this procedure. UCSF's main focus is resolving issue #2. Until the late July appointment, it is Kaiser's responsibility to mitigate as much of issue #1 as they can using Bosentan, breathing support, and nutrition. The more that Kaiser is able to resolve issue #1 before late July, the less complications will be put onto UCSF's ability to diagnose and make improvements on issue #2.

The doctors told us that Roscoe's blood results showed low pH (acidic) and high CO2 numbers. They explained that high CO2 in the blood stream causes blood vessels to constrict (issue #2) and that low pH also causes blood vessels to constrict (issue #2). This means that the high CO2 and low pH of Roscoe's blood has been working against the Bosentan medication he's been taking, making it not as effective. Roscoe's oxygenation looked fine, so we thought he was making progress, but really he was working against his long-term growth.

They proposed (and we agreed) to increase Roscoe's breathing support using a bubble CPAP machine. Roscoe was on one of these up until February earlier this year (here's a reminder). Here's a picture of him back on the bubble CPAP today:

Roscoe's blood tests should show improved CO2 and pH numbers within 3-5 days of being on the bubble CPAP. The blood draws from his foot can be inaccurate, so each week he'll get one blood test using his foot and one blood test from his arteries. This will continue until the late July appointment at UCSF. If repeated blood tests show that Roscoe isn't responding to the bubble CPAP support, then Kaiser may have to increase his breathing support using a ventilator. This is something that Kaiser doesn't want to do, since a ventilator is more invasive and damaging to his lungs long-term, due to the higher pressures. Based on what we saw this morning, Roscoe started responding well to the bubble CPAP because his heart rate was easily 10-20 points lower than usual (the green number):

This is where it gets a little queasy as a parent. If Roscoe does respond to the bubble CPAP, then to make things easier on Roscoe they will most likely perform a broncoscopy (using a camera to look at the inside of his throat) and then perform a tracheostomy (inserting a breathing tube into the front of his throat). The bubble CPAP would then be connected to his tracheostomy tube, rather than a mask over his face. This would most likely be accompanied by a gastronomy tube (tube into his belly to allow venting excess air pressure).

You might be sitting and thinking "Easier? How is cutting a hole in his throat easier on him?". I thought those same things. However, the bubble CPAP mask has several developmental downsides. It blocks his vision, interferes with his ability to eat, and is quite a nuisance for various sleeping/playing positions. Though the tracheostomy is very invasive, it would be better for Roscoe overall. The tube would remain in Roscoe's throat until he returned from UCSF, at which point Kaiser would leave it in until Roscoe could be weaned from needing it. Online we've seen several parents say that their kids came home with a "trach tube", so it's possible he'd come home with it.

To summarize, Kaiser is working on Roscoe's chronic lung disease (issue #1) for the next 2.5 months using breathing support and possibly a tracheostomy. UCSF will be working on Roscoe's pulmonary vein stenosis (issue #2) in late July. After that, Roscoe will return to Kaiser in Roseville and depending on how quickly he progresses he will come home. This means that Roscoe probably won't be home until he turns 10 or 11 months old.

Alisha and I are exhausted and frustrated. It really saps our spirits to be guaranteed another 3+ months of being at the hospital every day. Alisha spends 7-9 hours and I spend 2-3 hours each day at the hospital. What makes it extra exhausting is that nobody can lift this load for us. Visitors aren't allowed to be there without us, so nurses are our only option to watch him when we are tired. It is a weary task, and we know that we're the only ones capable of doing it. We know we have everyone's support and will reach out if we find ways for others to help us through this.

Roscoe is the bright spot in all of this. We have him. He is alive, he is happy, and he is growing. We have prayed constantly that God would snap his fingers and resolve all of these issues and bring our boy home. God has not healed him and we don't know why. We feel like we've been forsaken by him after his initial efforts to heal our boy. We know that God hasn't forsaken us or Roscoe, but as sometimes happens in life our emotions and our intellect are at odds with one another. I titled this post "Purgatory" because it feels like we're stuck in a loop of punishment for something we've done, even though we know that's not the case. Hopefully some day we will look back on this and understand why, but for now we are just left confused.

"Be strong and courageous ...
the Lord himself goes before you and will be with you;
he will never leave you nor forsake you.
Do not be afraid;
do not be discouraged."

- Deuteronomy 31:6-8