We were welcomed to Minnesota by a cold rainy and later on snowy day. It's hard to believe we left 90 degree weather in Phoenix. We arrived in our hotel at midnight and reported for our first day of evauluations, consults and tests by 8:30am. We first spoke to Dr. Whitley, a metabolic geneticist who was here for the first BMT (Bone Marrow Transplant for MPS I) in 1983. He explained the differences in the MPS disorders and the treatment protocols for those that have treatments. He was also the first to explain why MPS I is the only MPS with a BMT treament, which I have been curious about from the beginning. Basically, MPS I is a double edge sword...it has one of the worst prognosis if left untreated, specifically neurologically and life expectancy, however it responds the best to BMT. So though BMT is a drastic procedure, it is worth the risk for MPS I whereas the other treatable forms of MPS respond well enough to ERT (Enzyme Replacement Treatment) alone that they don't warrent the risk of BMT. I don't know if this makes me feel better or worse... which is kind of how I feel after every consult we've had.
After the geneticist we met with the BMT Drs.Orchard and Miller. Coincidentally enough we were on the same flight as Dr. Miller from Phoenix to Minneapolis. He was visting in-laws. Dr. Miller spent about 2-3 hrs. explaining the protocol, the research, the schedule, the risks, the drugs, the bone marrow donor matching process, what to expect post BMT and just about everything else. We are overwhelmed with information. Fortunatly Paul is taking copious notes so eventually we can remember everything we learned.
We then met with with the neurologist who explained the neurological effects of MPS I and showed us Coleson's MRI and explained the risk of stroke.
Coleson had is first EKG on Monday, which of course he didn't like and also had to do more blood work. He has really little veins and thick skin which makes it hard for the lab techs to find a vein. After being unsuccessful locating a vein they had to bring in an imaging device to locate a vein.
Tuesday we woke to a beautiful sunny day that revealved a 4" blanket of snow. It felt like Christmas... but in April!
We first met with the Anesthesiologist who also has been doing MPS BMTs since the beginning. It makes us confident we are at the right place and in the right hands. He also explained the complications of sedation and anesthesia with MPS patients because of their shortened neck, thicker tongues and smaller airways. It is very risky to sedate and especially to put MPS kids under anesthesia but obviously necessary so should only be done by those familiar with MPS. Hopefully after the BMT the airway will open up a bit and the tongue will become smaller and lessen the risk.
Then it was on to an echocardiogram and chest x-rays.
Then we spoke to a nurse regarding his Hickman line which is a temporary port in his chest that they will be able to administer drugs and draw blood from. It will be how he will receive his ERT and chemotherapy among other things. More risks, complications, precautions and care advice.
Lastly we spoke to a social worker who talked to us about "life" during the BMT process.. which is around a 4 - 5 month event. She talked about the financial aspect, the emotional aspect, the family aspect etc. She also took us around the BMT ward... which will be where we set up residence for about 50 days (20 pre-BMT and 30 post-BMT). They are "closed clean" rooms with Hepa filters as the chemo will wipe away any immune system he has. I have to say they really have tried to make it very comfortable for the families. One of us will have to stay with him at all times since he is so little.
We were able to get in to the Ronald McDonald house on Monday which is really fortunate for us. The RMH is AMAZING! I loosly knew about RMHs and have donated leftover change from the McDonald's drive thru on occasion, but I have a new found respect for this charity and the volunteers that make this place a home away from home for families with really sick kids. To be displaced from one's home for months and for the reason being a sick kid is very difficult. This place does a pretty good job at trying to provide a home away from home. And to be able to talk to other families who are going through similar situations is priceless. In fact we met the parents of the only living set of twins with MPS I today. I had read about these kids a week or two ago and to meet them and talk to their parents was inspiring. They were diagnosed and had their BMTs at an earlier age than Coleson but they had significant challenges as well. But their parents were so forthcoming with information and seemed so happy despite having two kids with Hurlers. It gave me a lot of hope.
The RMH is located right near U of Minn campus (in fact right next to a frat house) so we went to Chipotle for dinner. For a minute I was taken back to my college days, listening to the conversations going on around me... but my son quickly reminded me that I am a 30 something year old mommy with a sick kid when he had yet another blowout! He's been having intestinal issues for the past 2 days burning through several diapers and outfits. Fortunately laundry is part of the perks at the RMH so we have done a few sanitary loads already!
Tomorrow is a long day of neuropsych evaluations to understand where he is developmentally and what type of therapy he will need pre and post BMT. And then we meet with the cardiologist in the late afternoon. We are very overwhelmed and tired so hopefully this post makes some sense.
There are actually another set of Identical MPS I twins but they where no transplanted at Mn so thus why you may have been mistakenly told there is only one other set. There are also several sets of us Fraternal twins w MPS I though in my case my brother is un-affected and I am affected (with H/S form but same enzyme and
ReplyDeletechromosome error is affected).