Frequently Asked Questions

I spent yesterday afternoon doing some selfish sewing. It was a cold, slow day, and it felt nice to get lost in the creative process. But when I was done, I told Mike, "I probably should have spent my time writing a blog post instead." "Why?" he countered. "There isn't anything going on."

That's kind of true. It has been a slow week. We are in a holding pattern for now as we wait for tests and decisions and hopefully a few more answers. One of the blessings of this not being a malignant disease is that we don't have to rush into a transplant. We can take the time to check off each box and leave no stone unturned. That said, it isn't something that we can put off indefinitely as Aaron is in an extremely vulnerable and fragile state. We, along with Aaron's team of doctors, are not sitting around twiddling our thumbs. We're using the time we have to do as much as we can as we march ever closer to what feels inevitable.

I thought maybe the most helpful thing I could do today is just answer some of the questions I get asked the most. We are grateful for the interest you've all taken in Aaron's health, and I am happy to be open about anything I know the answer to (which, to be honest, often feels like not much). If I've skipped over something you've wondered about, feel free to leave a comment or send me a text.

When is the transplant going to take place? 

We don't have a fixed date yet. When we originally found out that Aaron's bone marrow was failing again, the doctors estimated that they would do another transplant in three weeks. However, at Aaron's appointment this week, they pushed that estimate back to the end of March. There are a number of reasons for this: They started weaning Aaron off steroids last week, but he won't be completely off of them until March 25th. They could do the transplant while he is still on them, but they would prefer not to, so they're going to wait. In addition to that, they decided to finish out his doses of rituximab. He got four total, the final one happening this past Thursday. Between the immunoglobulin, steroids, and rituximab, the doctors want to wait a couple of weeks and give them a chance to make a positive impact on Aaron's bone marrow. Personally, I appreciate them moving forward judiciously and with great care. I am still praying every day for a miracle.

Are you looking for another donor?

Interestingly, this seems to be the number one question that people have, and I think it all stems from one (very short) reference I made to the possibility of another donor in an earlier post. But as of right now, Maxwell is still the foremost candidate (much to his disappointment). It's easy to assume that since the transplant failed, there must have been something wrong with Max's cells or something that made him incompatible with Aaron. However, none of the evidence points to this. Rather, we saw blood counts that were very consistent for a year, no symptoms of Graft-Versus-Host-Disease (GVHD), and reassuring chimersims. It appears that it was Aaron's residual T-cells (not Max's) that wiped out his bone marrow a second time. Our doctors said it wouldn't hurt to look at the bone marrow registry and see if Aaron has another perfect match, but this poses its own set of risks and problems. Even when the HLA-alleles match between unrelated donor and recipient, the DNA material between these alleles will not match as well as it will between siblings. (This is my rather-primitive understanding.) Because the biggest risk with a transplant involves GVHD, they want the new stem cells to look as much like the old cells as possible--something that is much more likely with a matched sibling (as we can attest to from our first experience). The other thing that must be considered is the age of the donor. At almost 11 years old, Maxwell is pretty much the perfect age to be a donor. If we use someone from the registry, they will be an adult. The health of cells gradually decreases with age. If we use Maxwell, we will be putting the healthiest, most vibrant cells possible into Aaron. (Maxwell had a full workup this past week to check for any viruses or other issues that could make him incompatible. We don't have these results back yet.) 

But then, what about the mismatched blood type?

This is what everyone is getting hung up on. Aaron's blood type is A+, Maxwell's blood type is B+. After transplant, Aaron's blood type was supposed to change to B+, but as far as we know, it never did. It can take four months following transplant for it to change, and the last time Aaron got a type and screen (before these most recent ones) was only two months post-transplant. So there's a chance that his blood type actually did change, but then changed back when everything started to fall apart. (Can you imagine how insightful it would be if we had a type and screen from six months ago???) Even if this is what happened, it still leaves a lot of unanswered questions regarding the percentages in his chimerism. How can someone be 100% donor with their red blood cells but not be the donor's blood type? While there is a very slight possibility that the blood type is somehow a clue into the failed transplant, it still seems like using Max for the donor is the safest, smartest choice.

Have you found out anything else from the bone marrow biopsy? 

We finally got the full chimerism report from the bone marrow biopsy. The doctors were most interested in what happened with CD-33 (the antigens on the myeloid cells). In November, the chimerism report showed that three of the five CD-33 markers were 100% donor whereas the other two markers still showed trace amounts of recipient cells. In January, the chimerism report said the CD-33 was 100% donor, which made us all breathe a (premature and false) sigh of relief. The bone marrow biopsy showed that the CD-33 is now 94% donor. The fact that it is dropping is not a good sign and also confirms everything else we've already been learning.

Will you get a second opinion?

We trust Aaron's doctors a great deal and have the highest respect for them. That said, we also think there is value in other perspectives and opinions. We are going to consult with a couple of other bone marrow transplant centers to see if there is anything more that would be helpful to his case. Aaron's doctors are fully supportive of this.  

How often is Aaron at the hospital? 

Aaron has been going to the hospital twice a week: Mondays, just to draw labs, and Thursdays, for labs and an appointment. If he needs a transfusion, these theoretically short visits can stretch to many hours. 

Is Aaron getting any transfusions?

Yes. I mentioned before that his doctors were trying to hold off on transfusions if at all possible, but this past week, it became necessary for Aaron to have both a platelets transfusion (on Tuesday) and a blood transfusion (on Thursday). His platelets have been hovering right around 3 for the past couple of weeks, and he seemed to be doing okay, but then his nose started to bleed a little bit every time he ate. Mike and I were so nervous that we started setting alarms throughout the night to go check on him. Then on Tuesday, Aaron's gums started to bleed. We felt like there were too many red flags, and the team agreed, so he got a transfusion. They didn't draw labs immediately after the transfusion, so we don't know how high his platelets went, but at his appointment two days later, they were at 43, so we took this as a very positive sign that he wasn't killing them off right away. On Thursday, they decided to also give him two units of blood. His hemoglobin was at 7.2, and they didn't want him to drop below 7 over the weekend. Between the rituximab and transfusion on Thursday, Mike and Aaron were at the hospital for eight hours, but it was nice to see Aaron with some color in his cheeks again and a little more energy.

How is Aaron feeling?

His energy is generally pretty low. He spends a lot of time on the couch or in bed. It's hard to say if this is because of his low hemoglobin or because we've restricted a lot of his activities due to his low platelets. I'm often energized when I get up and move my body, and he's not doing a lot of that right now, which I think only exacerbates the fatigue. But really, the thing that is hardest for him right now is that his mouth is full of sores. This happened to him during his first diagnosis as well. Because his body can't really fight infection (due to a low white blood count and no neutrophils), any little bump or cut in his mouth turns into a full-fledged sore. This is agonizing for him. And since food is one of the main sources of pleasure for him these days, it is a real downer that eating is now causing him so much pain. 

How is Aaron's mood?

You might notice that I talk about my emotions a lot in these blog posts, but usually say very little about Aaron's. This is because I am the one writing the posts. I can speak to my own mental health but don't feel like it's necessarily fair or right of me to try to represent him. What I will say is that it's complicated. There is a vast difference between an 11-year-old (when he was first diagnosed) and a 12.5-year-old (currently). This is a really volatile age in general, and probably even more so when you're going through a medical crisis (and on medication that causes mood swings). Our even-tempered, easy-going Aaron is not quite so even-tempered or easy-going at the moment. I don't blame him one bit. 

What do you need? 

At this moment, mostly just prayers, positive thoughts, and lots of hope. Because Mike enjoys cooking, and Aaron loves his dad's food more than anything, we actually prefer to just do our own thing when we can. That certainly might change with a long hospital stay, but we are good for now.  

This is a rare disease with some complex variables, and there are so many answers I wish we had but don't. Hopefully your questions have been answered, but if not, please ask, and I'll do my best to answer them or (more likely) say, "I don't know."