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Welcome to Coleson's blog. We've created it to keep friends and family updated on the journey we are about to embark on.. We appreciate your support and words of hope and encouragement during this time when it matters most.

Thursday, January 30, 2014

T+187 The good, the bad and the Ugly

Again, I realize my updates are not very timely.  I wish I had more time but my life is pretty crazy.    First of all, it was really nice to see all of our friends at the RMH and the Doctors, Nurses, staff, etc. at U of Minn Amplatz Children's hospital but man was it COLD!  I am so thankful that we live in Arizona and Coleson can be running around outside and getting his exercise over the winter months and particularly at this stage of his BMT process when it is so critical to keep his development going, particularly his gross motor skills.  We did suit up and took Peyton sledding one day.  It didn't take long before we were too cold and then she ended up with a cold the next day... figures!

It was a pretty emotional driving into the city (Mpls) from the airport as the magnitude of the past year hit me.  I haven't been able to take much time to reflect on the whole event. From the diagnosis to starting treatment to moving our family and our lives into a small RMH room in a state 1800 miles away...to the chemo, the BMT, the drugs, the mucositis, the days of not opening the blinds, the therapies, the treatments, the vomiting, the g-tube feeds, the TPN, the pericardial effusion the weekly CBCs, transfusions, infusions.... well you can see how overwhelming this process was and still is and it kind of hit me as we were driving back to this place where a majority of the above took place.  We basically took Coleson to the brink of death and then brought him back to life but a new life.  Unfortunately not all survive this process as was the case with our friend Johnny and we just got word of another RMH friend, Gaby who passed away this week.  She had been pretty sick and was never healthy enough to get her BMT but had been in the hospital since October waiting. My heart goes out to her mother and to another one of our RMH friend's Karee Jo (a cancer survivor who is just finishing out her last rounds of chemo) who became really good friends with Gabby. Kids shouldn't have to die or witness their friend's dying.

On the brighter side, Coleson is doing remarkably well.  Dr. Miller (Wes) said he was doing better than he expected given the respiratory issues and the pericardial effusion (fluid around heart) he left with. His effusion is now smaller than when we left Minnesota.   His WBCs and RBCs and platelets are all strong but his hemoglobin is continuing to decline.  Not quite sure why but the Dr.s promise it will correct itself in time. Despite the hemoglobin, the Dr.s decided to start to taper him off his CSA and Steroids, both of which suppress the immune system.  We will need to keep a close eye on his hemoglobin level and pericardial effusion to see how they respond to this taper and we may need to stop the taper if we see a negative response.  The CSA is what makes him so hairy and so hopefully he will start to lose this extra body hair, though I have to admit, I've grown to like it.  However, it will be nice when it's gone and that will be one less thing that makes him look different.  

Even better the neuropsych Drs. thought he had made tremendous developmental progress.  He is "solidely" average in many areas for his age and low average in a couple others.  He still need to work on his gross motor skills, his peer to peer social skills and his enunciation, but typically at the 6 month post BMT mark is when you see the biggest decline so I was ecstatic to hear how close to "normal" he is.  The intrathecal injections (a spinal injection to deliver the enzyme to the brain) which are still in clinical trials, I think have helped halt the cognitive decline in Hurler's kids and I am so grateful especially with how "old" Coleson was when he got the BMT, that he was able to receive these injections.  

The best news we received is that both his T-cells and C-cells are 100% engrafted with Donor cells!  That means there is very little to zero trace of his own cells.  So all of the cells should be adequately supplying the enzyme he needs to halt further progress of the disease.  Time will tell what future surgeries, etc. will be needed (I expect their will be somewhere between 15 and 25 future surgeries) but for now we can celebrate this accomplishment. There is also a chance that his residual cells will reproduce and eventually his engraftement level will fall but hopefully they won't ever be enough to overtake the donor cells.  I think there is very little chance of that happening.

On the dark side of things; we had our diaper bag stolen at the Mall of America.  Which if it were just diapers and clothes, would not have been so bad, but unfortunately we had my IPAD in it (I was afraid with my record of 8 car break ins to leave it in the car) and Coleson's hearing aids.  Both of which are very expensive to replace.  I don't quite understand why things like this happen to me (again I've had 8 car break ins over the years).  Well - it actually happened to Paul.  I took Peyton on the rides and Paul took Coleson to Sears to keep him out of the mainstream.  But first he stopped at Subway. We have gone over all the events in our heads several times and we are pretty sure that that is where it was nabbed.  It is right near the entrance for a quick getaway and this was the only time he was distracted long enough for someone to have taken it.

On the even darker side of things, I ended up taking Coleson to the ER on Monday night after forgetting to prime his tubing for his IV fluid and pumping air into his central line.  Fortunately he coughed it out but it could have gone into his heart or brain.  It was a simple mistake that any busy "Murse" could make but a very serious one and I am just so lucky that his body protected itself.  In Minnesota the IV fluid came pre-primed and I had 1 bag left that I had out to use but at the last minute our home health care delivery came with the new bags (which included potassium as a result of his labs that day) so I made a last minute switch and it didn't dawn on me until he started coughing on what had happened.  So we made it to the ER around midnight was observed until 5:00am when then transferred us to the BMT floor to be observed until 2:00 Tuesday afternoon.  This poor kid has been through so much that I just hate that I had to subject him to yet another awful experience.  He is old to enough that at least in the short term to remember what the hospital is and starts to cry/fuss every time we go to clinic.   Coleson even remembered our old room at the RMH house and one day while we were there and he was tired, took me up the elevators and stood outside our old door wanting to go nite-nite.

We also had drinks with the President of the CCRF (Children's Cancer Research Fund) and met with 2 other CCRF employees.  They are doing a story on Coleson to promote their contributions to non-cancer disorders such as MPS.  MPS is considered a "Rare and Orphan" disease and as I've said before, does not get the funding, grants, publicity, etc. that other more common illnesses get.  It is nice to know the CCRF recognizes this and allocates some of their funding towards such causes.

We also made time to see other friends like Janna & Steve, Nicole and Debbie.  Peyton enjoyed seeing all of her friends at the RMH and didn't want to leave for 100 more days!  Overall, it was a very good trip but I'm glad we got out of there before it turned really cold!