Hello, friends. Here is the latest update on Gus. Lots to cover in this one. We're catching up on incremental developments over the last few weeks and briefing you all on some new and concerning challenges that have developed. Thanks for reading and praying for us all.
As I've mentioned in previous updates, the day-to-day with Gus has been consistent. The three ongoing objectives have been: 1) weaning his sedation meds, 2) increasing his feed to help him gain weight, and 3) caring for his kidneys with the right fluid balance and ongoing peritoneal dialysis. He is still on hourly passes.
With the first, he's made remarkable progress. He gets all but one of his meds enterally. While he's on a variety of medications, he is close to being completely off the sedatives. It's hard-won progress. It's hard to see him fight through the withdrawals. His team has been aggressive but patient and compassionate. As a result, he's much more alert, and we're working on more developmental and rehabilitation goals.
With the second, it has been mixed. He's still being fed through the nasogastric (NG) tube. His feeds have increased, transitioning from constant feeds to condensed feeds. Unfortunately, he's been throwing up regularly. The bouts of emesis are likely a result of 1) the discomfort that attends the sedation weaning, 2) the adjustment of having food in his stomach - before this, he was being fed with a nasojejunal tube that goes directly to the intestines, and 3) the increased abdominal pressure and discomfort associated with peritoneal dialysis. He's gained weight, but he's not where he should be. He's also working with swallow therapy on the bottle. Feeds are hard for kids in his position.
As for the third issue - the kidneys - the news is less encouraging. Despite some good days, the overall trend is not favorable. His urine output is still quite minimal. His renal function labs show key markers hovering at concerningly high levels. He got another renal ultrasound this week to evaluate how well his kidneys are getting perfused. The results suggest it is worse, not better than it was a few weeks ago.
This leads to one of the more concerning, and certainly one of the more daunting developments we've had in the last month or so. It requires a bit of context.
Since Gus' heart transplant, when his new heart failed to start as expected, our team has been monitoring his heart function closely. While the overall trend has been promising, one area of the heart has concerned us for a while - the tricuspid valve between the right atrium and right ventricle. There have been persistent signs of tricuspid regurgitation (TR). TR occurs when the tricuspid valve between the right atrium and right ventricle doesn't close completely when the right ventricle contracts. This causes blood to flow backward into the atrium making the heart work harder.
There was hope that the TR resulted from fluid balance and systemic pressure issues that would resolve as the right side of the heart recovered and the fluid drainage was addressed on our trip to Vanderbilt. Unfortunately, the TR has not gone away. In his last visit to the cath lab, our team conducted a TEE (transesophageal echocardiogram) for better heart imaging. The findings were discouraging. The TR is primarily a mechanical issue with the heart. The tricuspid valve is damaged - probably from the original trauma the heart went through during the transplant. The little tissue strands that hold the valve's leaflets in place (where the term "heartstrings" comes from) may have been damaged during an episode of ischemia.
It wouldn't be so alarming and so urgent if it wasn't for the kidneys. TR is a problem, but it isn't always an acute problem that demands immediate intervention. However, when you have kidneys on the verge of renal failure, not having good perfusion due to a leaky valve can be devastating in its effect. Of particular concern is that we are now more than halfway through what our nephrologists believe to be a 12-week window in which the kidneys can recover.
What does this mean for Gus? We're uncertain, but the possibilities are daunting. We will know more in the next few days. Our team is continuing to deliberate on what this means and what the best way forward will be. A few weeks ago, we felt like we were on a trajectory that could have us home in a matter of weeks. We know now we are on the verge of what could be many more difficult weeks.
For those of you following our story and praying for us, we ask you to pray with us that: 1) Gus' kidneys would recover and his urine output would increase, 2) that God would grant our team the wisdom and insight they need to address the TR issue, with all the daunting implications it involves, 3) that Gus would continue to gain weight and keep his feeds down, 4) that God would sustain us - five months in and we are burnt out and exhausted. The news about his heart and kidneys took the wind out of our sails.
I don't want to end without saying that God has been kind. He's given us more days with Gus than we would have had if it wasn't for his kind intervention and the amazing team of doctors and nurses he blessed us with. Gus has made incredible progress. He's teething. He is discovering the world with his hands. He's holding his own pacifier in place! God has encouraged us by all he has done to persist in prayer. We continue to come boldly before his throne to cast our anxieties on him, knowing he cares for us. Our greatest desire is that God would be glorified in us.
Thank you for praying with us. As we learn more in the days ahead, I'll provide further updates.