July 14: Day +12

The note written on my bag of goodies from RMH today... we need one...

Well, Daylon and Bella just keep getting closer and closer...

Looks like Bella has V.O.D. as well. Hepatic Veno-Occlusive Disease, also called vascular occlusive disease, turns out to be a relatively common side effect in pediatric stem cell transplantation. It is characterized by swollen abdomen (check), swollen liver (check), weight gain due to fluid retention (check), and jaundice from increased bilirubin (not yet). It is a category of disease that does not necessarily have a defined criteria, i.e. not all 4 have to be present, and up till recently, there has been no success in treating the disease, and if it spreads to other organs, it is 80-90% fatal.

At rounds this morning, the nurse and attending resident mentioned that Bella's abdomen looked swollen, as did her liver. Her eyes looked extra puffy this morning as well. Given those signs, they ordered an ultrasound for her liver which was done around 2. By 4, the attending fellow walked through the door.

I could tell what kind of visit it was within about two steps in the door. So, I just let her explain the whole deal about VOD and the experimental drug that she could go on... like dejavu after following Daylon's journey. He was diagnosed on day +10, we were diagnosed on day +12.

There is a drug that has worked well in clinical trials as treatment and as prophylaxis on VOD in transplant patients called defibrotide. It is not yet FDA approved despite successful studies on the drug in the U.S. going back as far as 1998. It is approved in Europe where it has shown great results as a prophylaxis. In addition to lowering cases of VOD, when given preemptively it also lowers cases of GVHD and increases the effectiveness of G-CSF, a "fertilizer" drug used to stimulate the growth of the newly transplanted stem cells (which Bella is on). These results are from only one study from 2009, but show promise. I already forwarded the research to Dr.s Wagner and Tolar and suggested they take a good look at adding it to their treatment protocol given what is happening with Daylon and Bella.

Anyway, regardless of her bilirubin count, I will be asking in the morning to go on the drug as soon as possible. This way, we try to stay ahead of the progression of this potentially fatal disease.

We knew to expect the unexpected, but we need to KEEP expecting the unexpected. I thought there were only 3 major dangers, GF, GVHD, and risk of infection. I either never heard them talk about VOD, or they never did. Whatever. Either way, here it is, and now it's time to do what we can to manage it.

My heart was breaking and aching as the fellow gave her spiel. It was just like when Bella's Dr. called to explain she had recessive dystrophic EB. I felt the pangs of pressure on my heart as the words started to sound like they were emerging out a long tunnel, feebly finding my ear.

Throw on top of all this that Bella has to go down for surgery tomorrow morning for her new Hickman and her G/J tube, AND it's a dressing change day, and tomorrow is going to be all action. My heart hurts just thinking about tomorrow.

I am sad. I do not know where the road before us leads, but it is going to be more windy and bumpy than I hoped. These are the moments where I am grateful for faith. Why? Faith is NOT hoping or believing in something when there are signs and evidence to support you. That is called expectation, or deduction. Faith, on the other hand, is when you stand in the face of no evidence, and still believe in and stand for an outcome. Despite this setback, I still declare that one day, Bella will look back on all this and say, "Yeah, I was born with this rare condition, but when they brought me home from the hospital, it went away."

Mommy and Ali watching "My Little Pony"...

The good news? My mom ("Nanny") comes into town tonight for 2 weeks. We timed it to fall on days +14 to +28 since this is when the *$#t tends to hit the fan in transplantation. Good timing, huh?

Other good news? The numbers for the day are in: Heart rate, creatinine, BUN and CSA are all now under control and stable, so I'm not going to give those.

WBC: 1,400

ANC: 1,000

watching Ali wiggle in mommy's lap while watching "My Little Pony"...

So, despite the side effects, the primary effect of transplant... Bella growing Ali's cells, seems to be moving right along! For this, I am grateful! It is possible to feel sad and grateful at the same time.

My morning walk to the hospital...

I want to close by thanking you for praying for Bella. She needs your prayers; I am consoled by you helping with this most important part of the equation. Ang is caring for Ali, I am caring for Bella, and your prayers sprinkle us all with the extra fertilizer we all need to emerge from this a healthy and intact family. Thanks again for all the prayers, thoughts, wishes, intentions, comments... ENERGY you send our way. Each one makes a difference.

Good night and God bless you.

I leave you with... Ali... "enjoying" her frosted flakes...