Before we start this journal entry, we wanted to say thank you to everyone who has signed the guestbook or sent e-mails of support. We couldn’t imagine trying to go through the last 7 weeks in isolation. Just as we know that we won’t make it through what lies ahead without your continued support. Thanks for being there.
Since last week, many of you have contacted us to let us know that you’re registering as a bone marrow donor or signing up to give blood or platelets. Many of you have asked if you can earmark your registration or donation for Ethan. Unfortunately, I don’t think you can, though it’s not inconceivable that he might end up receiving donated blood from someone who reads this. But even though we can’t be sure that your donations will directly help Ethan, thank you so much for taking those concrete steps to help him or someone else in need.
Last week Ethan went in for an eye exam and Sarah, Ethan and I met with Dr. Nemecek, the head of his bone marrow transplant team.
The eye exam was mostly good news. MPS kids suffer from corneal clouding as the waste product in their cells gets stored everywhere, including their eyes. Ethan does have some clouding—the doctor described it as looking through a window with a film on it. She said it is pretty minor at this point. She also said that he didn’t appear to be suffering from light sensitivity as some MPS kids do. She’s going to evaluate him again after the transplant. If it hasn’t gotten any worse then I don’t think there will be a need for a cornea transplant.
Ethan also had blood drawn for a DNA sequencing to see if they can localize the MPS mutation to enable MPS testing of our extended families to see who else might be an MPS carrier. We should have the results in 6-7 weeks.
Our meeting with Dr. Nemecek was pretty straight forward, I suppose. The most surprising thing was that the doctor said she might look to do Ethan’s transplant as early as mid-October. MPS is a degenerative disease, so the earlier we do the transplant, the earlier he might start producing the enzyme so that it can get into his brain. So earlier is certainly better. Nonetheless, the transplant is when all of this will really get intense, and Ethan may not survive. So part of me wants to push it back more so that we have more time to enjoy what we have.
The specific timing of the transplant will depend on the final outcome of the donor search. The transplant team is pursuing two different options at the moment. They’ve identified a stored umbilical cord blood unit that appears to be a good match for Ethan. They’re doing some secondary testing on it now to confirm the match. They’ve placed a hold on it and if that turns out to be the best match, they can schedule the transplant pretty easily. They completed the secondary testing of the adult unrelated blood samples, and they didn’t get a match on Ethan’s atypical HLA factor. So, they’ve also requested some adult donors come in for secondary testing to see if they can get a match there. They’ve said that the decision to go with a cord blood unit or an adult donor will be based solely on which is the best possible match. So if an adult donor proves to be a better match, the transplant could get pushed back in order to schedule the donation.
We didn’t go into a whole lot of detail beyond the fact that Ethan will be going in for a few days of chemotherapy immediately before the transplant. Dr. Nemecek mentioned that she has worked with about 5 Hurler’s kids. We didn’t talk about the outcomes of those cases. But the doctor mentioned that given the airway issues that MPS kids are prone to, she tends to treat them very aggressively and intubates their airway if there are any signs that the airway might be collapsing rather than waiting for the situation to become more critical. I’m not sure why she brought that up this early in the discussion, except to get us mentally prepared for the image of Ethan breathing through a tube.
Ethan is through week three of his enzyme replacement therapy, and he’s continuing to do well. As we’ve said here before, much of the physical damage that has been caused by his MPS is permanent and will require follow up treatments for the rest of his life. In addition, the doctors have told us that it is still too early to see any results from the enzyme treatments. Nonetheless, we are noticing some improvements!
Sarah believes that Ethan is starting to make more different kinds of sounds and is seeming to be able to eat more different kinds of table foods, which may indicate that his tongue is becoming less swollen. And I think that he’s starting to be able to focus on sounds more. It’s probably impossible to tell if these are the results of the treatments or just developmental steps for Ethan. But progress is progress; we’ll take what we can get.
Ethan is also starting to point at things now, which is fun. He’s still cruising along the walls and furniture, and he’s starting to balance himself in between objects for a half-second or so. MPS kids often have tight tendons in their heels, and I think the fluid in his ears is probably affecting his balance. I’m really hoping that he’ll start walking before his transplant. But given the timing between his ear tube surgery (Sept. 16) and his transplant (mid-October), he might not pick it up in time. And once he goes in for his transplant, I imagine he’ll be confined to bed for a long period of time, so I’m not sure when he might actually start walking.
Weeks 3-6 of the enzyme treatments are also when they keep a sharper eye out for an allergic reaction. Ethan’s body may treat the enzyme as a foreign body and develop antibodies against it. There is a small possibility that Ethan may go into anaphylactic shock. So the nurses are keeping oxygen masks handy and checking in regularly. So far, his infusions have been routine. Let’s hope they stay that way!
As we get closer to the transplant date, Sarah and I are thinking more and more about the logistics of being present at the hospital for Ethan’s care and being home with Caleb.
When Ethan goes in, Sarah and I will probably each have about one month of leave saved. MPS transplant kids are typically in the hospital between two and six months, followed by a period of some months when they’re going back 2-5 times a week for monitoring.
We think that, at least initially, Sarah and I will trade off weeks at the hospital. One of us will be there 24/7, and the other one will be working and then at home with Caleb. We’re fortunate that Sarah’s parents live in the area, and my mother has said that she can come out for an extended period to help as well.
Once our leave runs out, I think what we’ll probably do is turn the daytime monitoring over to the grandparents and we’ll plan to have one of us at the hospital in the evenings and at night time. It’s inevitable that we’ll have to take some unpaid leave, probably a fair bit of it. But the less we have to take the better. I know a lot of you have offered to help in any way you can, and we certainly haven’t forgotten that. The thing is that Ethan’s immune system will be completely gone during the transplant, and will be recovering during his hospitalization. So we think we need to limit the number of people who are in contact with him. We may be able to think about week long daytime rotations of people who could stay with him at various points. But it doesn’t sound like something where we’ll be able to have different people in with him on different days.
There are a couple of things that might make this easier. Sarah’s HR department has told her that she can telecommute from the hospital, as long as she’s not the primary care giver. And I expect that my HR department will say the same thing. That will allow us to be at the hospital to monitor his care and to participate in decisions even after our leave time is gone.
It is also possible that Portland State Univ. might offer leave sharing in the new contract to be signed this fall. I’ve got a number of great colleagues here who have offered to donate some of their leave to help us out. I’ve been in touch with the union representatives who are negotiating this part of the contract, and I’ve told them that, second to health insurance, this might be the most important benefit we could have to help us through this. I don’t know if leave sharing will be part of the final contract. And I don’t know when the contract might come up for a vote—maybe September. But we’re hopeful that some type of leave sharing will be included.
The other thing that Sarah and I have been thinking about is our dogs. We’ve got two humane society dogs, Katie and Mollie, that we love quite a bit. But as we look at our schedules while Ethan is in the hospital, we’re not sure that we’re going to be able to give them the care and attention they deserve. We’ve contemplated asking for help with walking them, but aren’t sure we could coordinate something that extensive for the length of time we’d need help. Another option we’re investigating is finding a temporary home for them for that period. I’ve contacted the Oregon Humane Society and one of the local rescue organizations, but haven’t found any local organizations that offer temporary homes.
If you’ve got any ideas or experience with arranging this sort of thing or are interested in possibly taking one of the dogs while Ethan is in the hospital, please let us know. It would be a big help to us and a big weight off our minds knowing that they’re in good hands. Both of them are good dogs, but they’re both humane society dogs and they have their “issues.” Mollie is a beagle mix and pretty good with kids and cats, though she doesn’t like other dogs. And Katie, is a lab mix who is great with teenagers and adults, but doesn’t like other dogs, cats, or kids she doesn’t know.
That’s the update for now. Hopefully, we’re kind of in a bit of a routine and things will progress pretty smoothly over the next few weeks leading up to Ethan’s surgery.
We’ll keep everyone apprised of developments and the latest with the donor search as we go along.
Thanks for checking in on us!