Hope has had a complicated few weeks. She has been fighting off infections and we received a new diagnosis because, hey, we don’t have enough to deal with already, right?
Let’s start with the infections. Hope continues to struggle with a condition called neutropenia, (click link for more info – noo-troe-PEE-nee-uh – is an abnormally low level of neutrophils, which are a common type of white blood cell important to fighting off infections). Neutrophil counts less than 1,000 — and especially counts of less than 500 — are always considered to be neutropenia, where even the normal bacteria from your mouth and digestive tract can cause serious infections. Hope’s labs have been coming back in the 30-170 range. You read that right – not 300-1700. She’s sitting in the thirty – one hundred & seventy range on a scale that should be in the thousands.
Why is this important? It has been practically impossible to keep her healthy. John acquired a nasty cough and despite our mask-wearing & sanitizing efforts, we realized he had shared it with Hope right before Thanksgiving when she started letting out tiny little coughs. We stayed home from all holiday festivities to try to keep her from getting worse, but we were stuck in this strange limbo where we could be at home, but we also needed to be in hyper-vigilant mode because the moment she might spike a fever we would need to make a beeline for Children’s. With her counts so low, she just can’t really mount much of an immune response and basically has the front door wide open for any bacteria, virus etc to walk right in and make themselves comfortable. To that end, she acquired a staph infection from bacteria that is just normal everyday bacteria found on our skin. Wednesday afternoon her mood shifted and we both knew a fever was impending and we started hourly temp checks and by midnight things were escalating and finally the fever peaked at 12:45 am and 102 degrees so we started the process, an awful 4 hour process, of being admitted. This snowballed into two sleepless days for John and I that left us utterly wiped. We got to come home late Friday on a home regimen of daily shots meant to boost her neutrophil count and Monday we will find out if we can stay home and whether her neutropenia will cause a delay to the surgical intervention coming up this Thursday.
The intervention I’m referencing is for the diagnosis we received as a result of findings at her one-year cath/biopsy when they were assessing pressure differences between her heart and lungs. They discovered that one of her pulmonary veins was completely atretic, or closed, and her left lung was only receiving about 10% blood flow. They need to time to investigate and compare her prior cath from December 2017 to more fully understand what we were dealing with, e.g. had it always been closed? We met with the Cath team right before Thanksgiving and left feeling much more educated thanks to their patience. Unfortunately, part of that education was understanding that this is not just a singular blockage to deal with and be done, but a progressive and unpredictable diagnosis: pulmonary vascular disease. This is the condition they hope to intervene on net Thursday *hopefully* – to open this blocked vein. They analyzed her scans from a year ago and it’s clear that the atresia is acquired and was not present last year. The outlook for this diagnosis is confusing. The team said that the intervention will have one of three outcomes 1-makes things better, 2- make things worse or 3- no change at all. Additionally, the next year will be one of “heavy surveillance” as they watch and assess how aggressively it progresses. If they can get this closed vein back open a series of additional procedures to keep it open will be needed in quick succession in the next few months because closed veins also have a propensity to close back up. If they can’t get it open, then we’ll still be under “heavy surveillance”, but only waiting to hear just how bad the picture will look at the end of the year – the same…moderately worse…much worse??? Only time will tell.
Right now the disease is only affecting one lung, but the progressive nature leads the team to believe it could affect other veins and/or lung lobes in the future so they feel a sense of urgency to at least try to open the atretic vein. Her neutropenia could delay things a bit, but we won’t know for sure until after transplant takes a look at her labs on Monday morning.
Oh, and we forget to mention the complication from the blood clot she had last year in her IVC that blocked access and clotted off her femoral veins. This access issue has meant that Hope’s regular caths are done through her neck, but that approach won’t work for this intervention and they will have to access her IVC by going through her liver, more risky than a typical cath. However, our doc reassured us that he is quite experienced with this.
John and I have both been struggling with sharing this news. Honestly, it felt like a real sucker punch to our morale when we heard something new was wrong. After all we’ve been through, we struggled to accept this new part of Hope’s health journey. In an effort not to end the post on a down note, here’s what we are hoping for with all the optimism we can muster.
- We hope the neupogen injections & reduced viral med will help boost her neutrophil count so we can proceed with the procedure on Thursday (and stay out of the hospital between now and then!).
- We hope the Cath team can get through the blockage and successfully balloon or stent her left pulmonary vein allowing her left lung to receive full blood flow.
- Lastly, we hope this intervention will slow or stop the progression of the condition.
Finally, if you are struggling to see the bright side, as we often are, here’s a video of Hope just being herself, totally oblivious and happy as a clam. If this doesn’t bring a smile to your face, check your pulse.
Also, more Hope to share 🙂