The Long Haul

I don't know how frequently I'll be posting updates on Aaron now that he's home from the hospital and we're settling into the long haul that is "post-transplant." I actually hope that there won't be too much to report on because that will mean that things are going well.

In talking to people over the last week, I know there are some misconceptions about Aaron's recovery, particularly with the timing of it all. We don't expect anyone to fully understand what is required following a bone marrow transplant, so we are never bothered if we need to answer questions--we are mostly so grateful that people are interested in him and care about how he's doing.

But I thought it might be helpful to map out and explain some significant milestones in the process:

April 23, 2021: Bone marrow birthday

May 13, 2021: Engraftment

August 1, 2021: Day +100

October 23, 2021: Tapering of immunosuppressant begins

January 23, 2022: No more immunosuppressant

April 23, 2022: One-year anniversary

Aaron is on a drug called tacrolimus, which I've written about many times before. This drug suppresses his immune system (specifically the T-cells), which helps prevent graft rejection. The goal is to keep the donor (Maxwell's) cells from attacking the recipient (Aaron's) cells. 

Sometimes, even being on an immunosuppressive drug, this can still happen, leading to a host of problems (some short-lived and mild, others severe and long-lasting) known collectively as Graft-versus-host-disease (GVHD).

Our job right now is to keep Aaron as healthy as possible so that his body can work on growing these new cells instead of attempting to fight an infection or illness with a weakened immune system. He is on a low-microbial diet (no restaurant food, soda machines, raw fruits or vegetables that cannot be easily washed, communal bags or jars, etc.). The house must be kept extra clean. He can't work in the garden or be next to a campfire. We can't do any type of home construction/remodeling. He can't be in the same room if someone is dusting or vacuuming. And of course, the most obvious one: no sick people in the house. 

This is a balancing act because we have other children besides just Aaron. We must keep Aaron home and sheltered (except for exciting appointments at the hospital), but the rest of our kids need to still go about their lives to a certain extent. This comes with risk because they could easily bring something home, but we are trying to be as careful as possible. We are carrying on with some of our activities (school, church, small playdates) and avoiding others (family vacations, big parties, tons of extracurricular activities). 

Even though it seems like we're saying no to a lot of things, we actually feel quite fortunate because we're able to be home. Primary Children's takes care of kids from all of the mountain west states. Following a bone marrow transplant, you have to live no more than an hour's drive away from the hospital for the first 100 days. This means that many families have to find other accommodations for another two to three months after being discharged from the hospital. 

The road after transplant is a precarious one. When people ask how Aaron is doing, Mike likes to say, "It's like we're walking on the edge of a cliff." Things are going fine right now, but they could turn quickly and with very little warning, which is why close proximity to the hospital is essential.

100 days post-transplant is significant because several things usually happen: you get to say good-bye to the low-microbial diet; your central line gets removed; you are past the most vulnerable time with GVHD; and you get to start coming off of the immunosuppressive drug . . . or maybe not.

It is my understanding that patients who have leukemia start being weaned off of the immunosuppressive drug around 100 days. I think this is because they need the immune system to start working again as soon as possible to continue to fight the cancer cells. 

But with a non-malignant disease like Aaron's, it is actually better to keep his immune system suppressed for longer to give the bone marrow a chance to become strong and fully-functioning before allowing the T-cells to wake up. Last time they waited until he was six months post-transplant before beginning to take him off of tacro. I have not specifically talked to his doctors about this, but I am assuming it will be the same this time.

I realize I just gave you a lot of boring information, but hopefully it provided a clearer picture of the next few months and explained why Aaron can't resume all of his normal activities even though he has already had the transplant.

The other thing I thought I'd talk about is what our daily schedule looks like. This might be more for me than for you since I hope to be able to look back at it in a few months and see some progress:

8:30 am: Aaron wakes up (or gets woken up by me).

8:35 am: Check blood glucose. Give insulin if needed. Eat breakfast. Count carbs. Record all numbers on spreadsheet.

9:00 am: Take tacrolimus and other meds.

9:05 am: Flush and heparin lock central line.

9:00 am - 12:00 pm: If it is Monday or Thursday, go to hospital for appointment. Get transfusions and/or neupogen if needed. If it is Tuesday, see the home health nurse for a dressing and clave change on his central line. If it is Wednesday or Friday, stay home and try to convince him to do schoolwork.

12:00 pm: Lunch time--do all of the same things we did before breakfast.

2:00 pm: Take mycophenolate (another immunosuppressive drug that he has to take three times a day; he will only be on this one until Day +35).

2:00-4:00 pm: He has usually maxed out on school by this point and reads, watches a show, or builds a Lego set.

4:00 pm: The other boys get home from school. They immediately wash their hands and change their clothes to minimize germs.

5:00 pm: Dinner. Repeat all of the glucose/insulin/carbs steps from above.

7:00 pm: Start magnesium infusion. He gets this every night through his central line. It runs for two hours.

8:00 pm: Start micafungen infusion. This is an anti-fungal medication. He gets it every night through his central line. It runs for an hour. Because he has a double lumen, he can get both this and the magnesium at the same time.

9:00 pm: Take tacro and other medications.

9:05 pm: Flush and heparin lock central line.

9:15 pm: Give him long-acting insulin

10:00 pm: Do one last glucose check. Give insulin if needed.

10:30 pm: Go to bed.

These are the things that stay fairly consistent every day. There are variations (although not too many since we're not really doing anything) in the in-between hours.

So far, we haven't seen too much action with his blood counts. His neutrophils are going a little crazy (1700 on Monday, 600 on Thursday, 3500 on Friday), but that's because he's been on and off and on neupogen, so I feel like I can't really trust them yet. He needed platelet transfusions on both Monday and Friday. He didn't need a blood transfusion this week--his hemoglobin didn't go up but managed to stay fairly steady. 

We had a little bit of drama with his tacro level this week. At each appointment, they check to make sure it's in the range they want (which I think is between 8 ng/mL and 12 ng/mL). When Aaron was discharged last Friday, his tacro level was 16.5, so they told us to skip his night dose and then lower his dose by 0.3 mg. They suspected his central line might have become contaminated, and so they order a peripheral blood draw at his appointment on Monday.

Sure enough, the peripheral draw indicated that his tacro level was less than 2 ng/mL (which meant that the labs from his line had been artificially high), so they immediately increased his dose. I was pretty frustrated by this for a number of reasons: While Aaron as in the hospital and getting tacro through his central line, Mike and I were absolutely militant about checking to make sure it got put into his purple lumen only so that the red lumen could stay clean for blood draws. Somehow the red side got contaminated, and I know for certain this wasn't caused by tacro being put into it. So there has to be another way that tacro can cross over to the red side (maybe through back flow or checking for blood return, although they say they've seen lines get contaminated even after switching to oral tacro). 

This feels like something that would be worth figuring out since it can lead to dangerous situations, as in Aaron's case: They thought his tacro level was high, but when they did the peripheral check, they found out it was actually extremely low. Personally, I feel like they should have done a peripheral check much earlier, even before his level was "high," just to make sure it was matching with the draw from his line. This seems like it should be standard practice, but when I mentioned it to the doctors, they said, "We don't want to subject him to a poke unless it's absolutely necessary," which strikes me as extremely ridiculous since they have no problem drawing more and more labs at any other time. But for some reason, an extra poke seems like a worse tradeoff than GVHD. It makes no sense to me.  

I think one of the hardest things in all of this is watching everyone else come out of the pandemic and quarantine. The masks and restrictions are going away and normal life is being resumed, but not for us. We went into this quarantine state in August 2019. In March 2020, Aaron started coming off of tacro and we were just about ready to reemerge when the pandemic shut everything down. Now the pandemic is coming to a close (hopefully), but we are at the beginning of another transplant. It all just feels never-ending and like we will never get to go places or do things without masks or a sense of caution. 

But enough of all of that. This post has become much longer than I anticipated. I wasn't planning on sharing so many details, but now that they're there, I guess I'll leave them so I'll have them for future reference. 

The truth is that in spite of all of the appointments and blood tests and daily care, we're enjoying life at home so much. It's so nice to be able to go on a date with Mike or eat dinner together as a family or go on a walk around the neighborhood. There are many restrictions, but we still feel free.