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!

Wednesday, January 8, 2014

T+168 (24 weeks)

Several of you have reached out to me personally over the past couple of weeks for an update as I have not had much time to update my blog.  Typically that means there isn't any bad news to share but I appreciate the personal inquiries.  At least I know I’m not writing into outer space.  We had a quite Christmas with just the 4 of us at home in Phoenix.  We typically travel to Michigan to spend Christmas with my family but obviously were unable to do so this year.  It turned out to be a good year to skip the Michigan trip as we were swimming in our pool while my family had single digit temperatures and no electricity for several days.  My parents were hoping to make it here for a visit but with the bad weather, kept pushing their trip back and now will not make it at all.  Christmas Eve we had to make a quick trip to the emergency room much to my dismay as Monday night Coleson’s G-Tube managed to come out and the balloon that keeps it in had burst.  So while I was able to get it back in and secure it for the night, I did not have an extra G-Tube on hand and since it was Christmas Eve, it was unlikely to get a quick insurance approval for home health care to provide me with one.  So instead they will get an Emergency room bill!  Oh, and I got a Christmas gift from the Insurance company in the form of a 3 inch high stack of insurance claims.  I guess the hospital in Minnesota submitted them all at once.  That stack only covers July – October.  Trust me, if you stack all of the claims for this year, I’m sure it is closer to an 8-10 inch stack. 

In good news, the fluid around Coleson’s heart is decreasing weekly and he has come of the lasix completely and is only on his morning does of Steroid.  However, he is starting to get the steroid puffiness, weight gain and just recently the steroid temper.  He is close to 30lbs now which is getting hard for me to carry.  He can walk – though typically runs – but quite often I have to hold him or carry him for various reasons.  I’m not sure if the temper is just a 2 year old temper or steroid induced but he has gotten into the “Mine, Mine, Mine” stage and “No, No, No” stage.  This is still on an exception basis and most of the time he is still very well behaved and loving.  Many mom’s may be frustrated with this but I rejoice in the fact that he is acting like a “normal” 2 year old.  “Normal” is not a word that I often hear for Coleson… in fact since he was born the most common words I heard was, “it’s not abnormal” which trust me is not the same as “it’s normal”.   It is very wearing to constantly hear/be reminded how different your child is.  At least if I am going to have an abnormal child, it would be nice for him to be a perfectly abnormal child.  What I mean by this is that he obviously has had some complications with his BMT.  First the pneumonia scare, then the periocardial effusion and then the periocardial effusion again in additional throughout this he has had Cytopenia.  Cytopenia basically is the bone marrow not producing the right mix of blood cells.  Another way to say it is a deficiency of one of the blood cell types, either the white, the red or the platelets.  His oscillate so when one goes up, another goes down and when that type comes up, another goes down.  So we keep getting infusions to try to level them all out and hope that they all learn to play nice with each other inside Coleson’s body.   

The bad news is that we have to face the bitter cold in Minnesota in 2 weeks for his 6 month check up.  I would try to postpone this until the weather is nicer but it is important to get his last intrathecal injection (spinal injection of the enzyme) on time.  I am not looking forward to traveling into sub-zero temperatures and going back and forth to the hospital during cold/flu and RSV season.  Makes me especially nervous as steroids further suppress his immune system which is already suppressed by the BMT and other medications.  I had hoped he would be off the steroids by the time we left but due to the cytopenia he won’t be.  We will get to see some of our friends who are still at the RMH even though we most likely will not be able to stay there this time.  The U of Minn Hospital and the RMH is getting to be a pretty popular place and as large as it is, they waiting list is typically double digit long.  I am hoping that perhaps one day they will be able to expand by another 15 or so rooms so again, if you’re looking to donate anywhere, please consider RMH Upper Midwest.  We will have to make the trip on an annual basis and trust me, the costs add up.  Even though Paul and I have good jobs, it is expensive to make these trips.  It’s not like these are vacations for us but they are just as expensive or more so.  To not have to pay hotel costs is such a blessing in addition to all the other benefits the RMH has to offer.  And for all the other MPS families that have to travel there annually as well who might not have the jobs we have.   

Also anyone who has been collecting soda/pop/beer tabs, please bring them by my house in the next week and I will take them with us.  You would be amazed at how much money they make from these!  I have the facts somewhere in my office, maybe someday I will find them and send it out.

I hope all of you had a good new years.  Again ours was quite but my Uncle and Aunt did make it out of Michigan for a visit so we spent it with them and watching my Alma matter Michigan State beat Stanford in the Rose Bowl!  My brother in law and oldest niece went to the Rose Bowl and flew into Phoenix to make the drive so we were also able to spend a day with them.  We would have loved to have gone with them but it was not in our cards this year.  I’m hoping it doesn’t take them another ~30 years to make it back there again!

I’ll try to get more frequent updates out.  I have so much more to say but find very little time to do so.