Sorry it’s been a while since we’ve updated everyone. We’ve been the proverbial ducks on the pond over the last couple of weeks: on the surface, everything going along according to our new routine; underneath paddling furiously towards the transplant.
Ethan is continuing to do great. He’s strong enough now that I have confidence that he can make it through the transplant (assuming the first transplant engrafts). Ethan is scheduled to be admitted on Wednesday, October 21 or Friday, October 23, depending on the schedule of the radiology team for his radiation treatment. Pre-transplant assessments started last week and from then through admittance he’s got 12 additional assessments and consultations in addition to his weekly enzyme appts: these include weekly sessions with the early learning folks at the school system, numerous audiology tests, post op assessments, radiology consults, hospital services consults, transplant doc meetings and a scheduled MRI under sedation to do a brain scan followed directly by an EKG and sedated audiology test.
He had his first visit from the school district Early Intervention specialist on Tuesday. She spent a good hour talking with Sarah and playing with Ethan to show us the kinds of games and activities we can do with him to improve his fine motor skills and his communication skills. Many, if not most, of the activities are ones that will be easily transferable to the hospital (like playing with puzzles that have knobs on the pieces or putting together and pulling apart plastic blocks or learning how to respond to simple requests.) Kathy, his EI provider, was very happy to see him walking and climbing and being interested in drawing with a pencil when she showed him how. She even commented that his face seemed less coarse than a month ago when she last saw him. Perhaps another sign that the ERT is working.
Another bit of exciting news--Ethan had his first ERT with the central line last week and loved the fact that there were no, I repeat NO, needle sticks!! (Though it was a bit daunting to see how much blood they took for testing—they’ll take some each week since there are so many tests they need to run pre-BMT.) Also, during that treatment, we had a visit on the ward from a certified visitation llama, which I don’t have to tell you was weird and cool. Ethan did pet the llama after a bit. But mostly, he just looked at it and tried to figure out why this furry dog looking thing had such a long neck?!
For today’s ERT, we had a brief visit from the hospital social worker—not much going on there. And we had an initial visit with the Dr. Oltmans, a Kaiser pediatrician, who may be Ethan’s new primary care physician after Dr. Liebo retires next month. Dr. Oltmans, Ethan and I went down to a conference room to conference call Sarah in on our initial discussion (read: job interview). I don’t know if Dr. Oltmans knew, or perhaps didn’t care, that she was being interviewed. She seems capable, but came across with that mildly annoying sense that she would always know more than we did and already had the job in the bag. She comes well recommended, and she’s regularly at Doernbecher, Ethan’s hospital, and perhaps I think she’ll be an effective advocate for Ethan, an effective primary care doc for him. So, I think she’ll be alright and we’ll see how the relationship develops.
Things are heating up and shaping up here, and we have definitely turned the corner, both physically and emotionally, and are now pointed directly towards the transplant and hospitalization period. Ethan’s family care team--Sarah and I, Sarah’s parents Bob and Pat, and my mother Lilianne--is in place, as Lilianne arrived from Florida late Tuesday evening. Sarah and I are drafting the initial care schedules/leave schedules that we think we’ll follow (Sarah’s note: Todd does amazing things with a spreadsheet), and we’re working through a whole bunch of pre-transplant assessments.
Lilianne came out early so that she could spend some time with Ethan before he goes in to the hospitall. With her arrival, Ethan's last day at daycare was Wednesday. That was a difficult milestone for us, both because we are immensely grateful for the care and love his teachers have given him, and the support they have given us; and because we know it may be a year before he's ready to go back to school again. But, taking him back to school and returning him to that loving environment next year will be an even more important milestone, becuase it means we'll have gotten through this.
At the moment, I’m over in the family lounge at the hospital getting caught up on work on this journal. And Ethan’s down the hall in the infusion center getting this week’s ERT under the watchful and loving care of 3 grandparents!
In fact, we’re hoping that this is the model that we’ll follow when Ethan is admitted. Sarah and I know that one of us will be in the hospital with him at all times. And we know that we’ll be the ones sleeping here, and that we’ll need to be present and involved in tracking and understanding his condition and options. But we’re also hoping that during the days, one or more of the grandparents can be his primary caregiver so that we can telecommute both to triage things at the office and so that we don’t have to use 40 hours a week of our leave time.
When Ethan goes in, Sarah will have about 90 hours of leave and I’ll have about 190 hours. In addition, I can borrow an additional 69 hours of leave under university policy. If the hospital parent can telecommute something between 2 and 5 hours per day depending on Ethan’s condition, then I think we can stretch the leave out to cover a six month stay in hospital. That assumes that we’ll need to be present for him more over the first couple of months (maybe only working 10 hours per week) and then can scale up (to working 20 hours per week). So one of us would be working at their office and the hospital parent would be in Ethan’s room in the mornings for the daily discussions with the doctors, then telecommuting in the afternoon.
We’re hoping that the grandparents can serve as his primary caregivers for five hours a day during the week, so we can do that. I think that’s manageable. But more than 5 hours a day as the sole family member in his room would be too much. And hopefully, we’ll have two family members in with him in the evening.
About the only change in Ethan’s condition is that he hasn’t been sleeping too well over the last couple of weeks. Really since he got his port placed. He’s waking up 2-3, occasionally four times per night. Usually when you pick him up, he’s asleep again by the time you get him to your shoulder, but occasionally he’ll be up for an hour or so. We don’t know if he’s just sleeping on the port wrong and it’s waking him up, or if he wakes up a little bit and get’s scared in the dark as all kids do, or if there is something else.
There’s another MPS1H kid, named Caleb Waddell (no relation that we know of, but I wouldn’t be at all surprised if we were distant cousins), who recently had a sleep study done. The study showed mild sleep apnea of 10 seconds or so (if I remember correctly), which is considered mild, but the study also showed a lot of sleep interruptions during the night. When Ethan had his sleep study done pre-diagnosis, pre tonsil and adenoid removal, it also showed mild 5 second apnea. But we never saw the actual report, so we don’t know if Ethan had a lot of interrupted sleep episodes at that point and whether or not this is a development of an existing cycle or something new. So we’ve asked for a copy of the report so we can take a look.
For Sarah and me, we are trying to learn how to effective, assertive but respectful advocates for Ethan both in his care and in navigating the obstructionist barriers that our modern healthcare system has inserted between patients and their doctors to ensure that we don’t take up too much of their time. We are really trying to understand Ethan’s treatments and to respect the structures and processes here at the hospital, while at the same time learning how to be effective advocates for him and ensuring that his team understands how MPS patients are different from other BMT patients (e.g. leukemia, anemia, etc.).
When we were in the run-up to Ethan’s surgery, we got the sense that each of the surgeons was focused on their individual procedure, and none of them were taking a holistic view of his care or paying a whole bunch of attention to how the surgical needs of MPS patients are different from typical patients. So we got in the habit of regularly asking for second opinions from Ethan’s primary care physician or the hospital’s MPS specialist.
Now that Ethan is in the transplant pipeline, it appears that Dr. Nemecek, his transplant doc, is taking full ownership of his care, and she seems to be looking both at the transplant needs and at the MPS needs and assessments. So, I’m having to break my habit of immediately going for a second opinion and I first need to understand why Dr. Nemecek is asking for the procedure and what MPS risks and considerations she’s already taken into account before I go ask for a second opinion.
This morning we got hit with some really difficult news that will test how well we can both advocate respectfully and forcefully but that may end being an argument and discussion that we can’t win. We found out this morning that due to H1N1, the hospital is prohibiting any visitors under 12 yrs old on the BMT transplant ward. This is a very, very difficult restriction for us. We certainly don’t want to do anything that would unduly put Ethan’s health at risk or endanger any of the other immunosuppresed kids on the transplant ward. At the same time, our 4-, going on 5-year old, Caleb, is a member of this family and is Ethan’s older brother. We both have an extremely difficult time with the idea that Caleb will not be able to visit Ethan at all for 2-6 months. And if, god forbid, Ethan should die in the hospital that Caleb would not be permitted to have shared that time with us and with his brother.
A month or so after we got Ethan’s diagnosis, the father of an only child MPS kid, suggested that we send Caleb to live with his grandparents for 6 months to a year because kids are exposed to germs and having another kid in the house dramatically increased the risk that Ethan would contract something and die when he was immunosuppressed. I explained that Caleb is a member of our family and that while we would take every single precaution to ensure that Caleb would not pass on an illness, we could not send Caleb away. We do understand that kids get exposed to all kinds of germs and that immunosuppressed kids with older siblings are at a higher risk of contracting a life threatening infection. But we also feel strongly that cutting Caleb out of his brother’s care is likely to be equally traumatic and damaging to Caleb and that Caleb needs to share and learn through his brother’s experience.
We have always felt that Ethan’s fight is our fight and we will do it together as a family. We still feel that is true. We will take every single step that we need to in order to prevent Caleb spreading something to Ethan. If that means removing Caleb from school while Ethan’s in the hospital, monitoring his temperature daily, bathing him daily, or putting him in full mask and scrubs during visits, we’ll do it. But cutting Caleb off from his brother would be very difficult for us.
I talked to a couple of doctors today and one said that this policy was being adopted in pediatric hospitals across the country. And I said, I didn’t really care if it was being adopted widely, I still want and need someone to sit down with me and walk me through every single H1N1 factor and every single possible protection measure and tell me in detail why each one had been considered and discounted. If they can do that, then we won’t have any other choice. But until they can explain and discount every single other option, I’m going to advocate strongly and pretty damn loudly for other options.
We want to send our thoughts and prayers to the Ellis family, whose two beautiful boys Damion Ray (5 yr) and Jacob Jr. (4 yr) were both diagnosed with MPS1 in late August. (http://www.caringbridge.org/visit/damionandjacobjrellis/journal) To their parents, we were there so recently, and I know how easy it is to feel lost and isolated. Never forget that we all walk with you and are here for you whenever you need us.
There are three reasons why I keep this journal and each is equally important. 1) we can’t do this alone and this is the best way to keep everyone informed, including our family and friends back east and in other countries; 2) I hope that Ethan won’t remember any of this when he gets older, but that we’ll be able to read this together with Ethan one day so he knows how strong a fighter he is, and 3) MPS is such a rare condition that each family’s story forms a part of the knowledge base. Learning about other families’ journeys was an important part of our acceptance of Ethan’s diagnosis, and is an ongoing part of our education about Ethan’s treatment. Someday, perhaps, one other parent who has just learned their child has MPS may find something here that helps them on their journey and in their fight.
Thanks for checking in.