07/19/2024
DAY +176 - PREDICTABLY UNPREDICTABLE RESULTS
7:30 AM CST is seriously too damn early for this Mountain Time family but nonetheless, we showed up, on time, at the hospital this morning despite significant grumblings from the peanut gallery, aka Bella.
Growth hormone testing may seem obsolete for a clearly non-adolescent who, despite her petite size, stopped growing several years ago. When Bella was a child, this same growth hormone test determined she was deficient and resulted in a daily injection of human growth hormones for nearly 7 years. As she was a small child, maybe 7 or 8, when these painful injections began, we bribed her with 2 quarters each time she did her shot without crying. She eventually earned enough money to split the cost of an iPad with us, providing a much needed distraction during endless appointments.
Around 16 or 17, we were told Bella's growth plates had closed ending her potential fornfurther growth and to us, it made sense to then stop the daily shots and routine visits to the Endocrinologist as we had bigger issues issues to deal with, ya know, like a bone marrow transplant. However, we recently learned that normal growth hormone levels are necessary even in adulthood as it helps maintain bone health and prevent osteoporosis, especially after a bine marrow transplant.
So, we again found ourselves testing Bella for growth hormone levels this morning - a 3 hour blood test which includes 1 injection of medication into Bella's bicep, 7 blood draws (thank GOD she has her central line still) and multiple blood glucose tests when she became symptomatic. We don't know the results yet, but the medication clearly had some effect on Bella as her glucose eventually dropped below 60, she became overheated and lethargic, and her blood pressure dipped too, resulting in a call to the doc to determine if they should discontinue the test.
Thankfully, just as Bella was hitting a wall, a very familiar face showed up in her infusion suite. It was Shay, one of Bella's very favorite nurses from the 4th Floor! Her surprise visit actually caused Bella's blood pressure to rebound and, we assume because Shay is so sweet, her glucose jumped back up just enough to finish out the test! Thanks for the visit, Shay!
While sitting around waiting for the q30 minute blood draws, Dr. Wagner came in for Bella's 6 month post BMT check up. Overall, he feels things are going well but of course, is concerned about the endocrine and pulmonary issues and the potential for either to be GVHD. He would like to see her T-cell subset counts a bit higher but attributed their slow rise in numbers to a handful of factors including a slight DQ mismatch on the HLA typing (essentially, Kennedy and Bella are a perfect bone marrow match however, one of them somehow inverted their DQ1 antigen), the previous struggles with pneumonia and ongokng lung issues, and perhaps even the Celexa debacle that suppressed her counts for a while. More time, patience, and continued adherence to protocols for keeping Bella away from crowds, germs, and other potential enemies was his recommendation. She gets to decrease the dosage of one medication slightly but overall, the advice is to stay the course.
In the Fall, ther is an Adult FA meeting that Bella would LOVE to attend as it is a unique opportunity to gather with peers from around the world for support, encouragement, and enducation. We discussed Bella's potential attendance, which would mean exposure to hundreds of people. Dr. Wagner didn't say "no" but he did set a high bar which may be unachievable. IF, big IF, her CD4 is above 100 (it's currently 71), we.can discuss it. However, if the number is lower than 100 or if anything at all shows signs of GVHD, it's a hard no.
After wrapping up with Dr. Wagner and the Growth Hormone Testing, we headed up to Endocrine. Bella struggles with maintaining a healthy blood glucose level for unknown reasons. It may be simply an intolerance, could be a sign of potential diabetes in the future, or it could be that she's just sensitive to slight fluctuations. So for the next 10 days, Bella gets to wear a continuous glucose monitor on the back of her left arm that will produce a record of the highs, lows, and normals. She didn't even flinch when the Dexcom pod was applied (it has a small needle in it), and was fascinated by the process as she's seen many YouTubers share their experiences with the Dexacom.
It takes 30 minutes for the sensor to warm up, but as soon as it did, we received an alert that she was "dangerously low" at 42. Eek! This is supposed to be a "blind test" meaning we dont treat the highs or lows but I did give her an apple juice because, seriously, we were already running late for our final appointment if the day and had no time for her to pass out. I'll be interested to see what the 10 day results yield.
Ournlast appointment of the day was a Lipid Clinic because apparently when your cholesterol jumps from normal before transplant to the 300's 6 months later, you earn a VIP slot with Preventative Cardiology Care. Fun.
Normally, Bella would be stated on a stating, like Lipitor, due to the alarmingly high cholesterol spike. However, statins and posaconozole (an antifungal Bella is on for one year post BMT to prevent fungal infections like the very scary aspergillis) are a big no-no as they are contraindicated due to potential for liver and kidney damage. Soooo....there goes that easy fix. Damn.
Other options were discussed including something called PCSK9. Unfortunately, that must be injected once per month which means either we fly up here every 4 weeks so the docs here can do it, or we get a referral for a cardiologist in Colorado and ask them to prescribe it at her initial visit. The problem here is that most cardiologists can take up to 6 months to on-board a new patient.
The doctor required us that in the next 6 months, Bella heart will not explore due to cholesterol overload. The purpose of statins and reducing cholesterol is to prevent long term damage to her heart. So, as the posaconozole will be discontinued in 6 months, this opening Bella up to use the Lipitor, it wa suggested that we make some slight dietary changes (nothing major as we need to maintain her weight) and revisit the Lipitor option at our 1 year visit in January 2025.
And then a funny thing happened on the way home (to Hope Lodge) from the hospital. As mentioned, Bella's overall cholesterol had jumped from normal (158) before transplant to high (333 - anything over 200 is 👎) in June. And the bad cholesterol (LDL) went from 86 (normal) to 218 (over 100 is HIGH). We made a few slight changes (thanks, Stephanie Kagan!) to her diet over the past weeks while we were home but nothing major - more fruits and veggies, less processed food, etc. While discussing the statin conundrum with the doc, results from today's test were still pending so all.of the talk was hypothetical, assuming her cholesterol hadnt changed much from 6 weeks ago. Of course, as we were walking out the door, the numbers populated in her Mychart.
202! Bella's overall cholesterol dropped over 100 points and is just slightly high! And her LDL, the bad cholesterol, is now 99, under the threshold of 100 for a healthy range. We will still follow up in January, but I'll sleep better tonight knowing she's not a ticking time bomb of sticky, waxy gunk clogging up her arteries!
Surgery is scheduled for 12:30 start tomorrow and will include 5 separate procedures performed by 3 different teams of doctors. Congrats to whoever coordinated that circus as I can barely get 3 humans out the door at the same time in any given day. A colonoscopy and endoscopy will be the responsibility of the Gastrointeroligist, a bronchoscopy is on the agenda for the Pulmonologist, and the general surgeon will both remove her central line (she has requested to keep the device after removal) and then remove and FINALLY close, for good, the g-tube site which Bella has resented for as long as she can remember, except of course, for when it was needed to save her life occasionally.
I'll admit to being kind of emotional with tomorrow's remarkable milestone in Bella's life. Bella has, literally, had a g-tube since the day I met her, the day she was born. It's the reason she's alive today. Save for a short stint when she was 5ish when it was removed for about 6 months, the gtube has ALWAYS, ensured we had a way to easily provide nutrition, hydration, and medication, even when Bella was at her most vulnerable, weakest, and sickest. She's most definitely ready for it to go, but I'm not sure I am. No matter though, tomorrow we say goodbye to 2 decades of g-tube reliability and hello to Bella 2.0, who has proven for several years now that the tube was not needed, self-sustaining her body, health, and nutrition, without a fallback plan. Thankfully, she's stronger and more confident than I'll ever be and most definitely, SHE'S GOT THIS!
Please storm the Heavens with prayers for our Fierce FA fighter, Bella, tomorrow as she puts on a brave face to once again enter the OR arena, endure the procedures and pokes and prods and slicing and dicing needed to release her of the constraints her g-tube and central line have on her, tolerate the scopes and cameras necessary for internal imaging, and safely come out on the other side. 🙏 ✌️ ❤️
