T+247 Last Dose of CSA

Last Dose of CSA today
Last Dose of CSA
Take .1ml and flush it down
Time to throw the CSA bottle away!

Major Milestone Accomplished.  Today was his last day of steroids and CSA, both immunospressive drugs. Hope that most of the others will follow suit on Monday at our clinic visit.

T+232 One Year Ago

One Year ago, I sat in a Geneticist's office answering medical questions about my son and Paul's and my family history
One Year ago, I held Coleson as she evaluated his head, hair, fingers, spleen, liver and toes
One Year ago, I heard, "MPS, that's what I think this is" as she handed me a kleenex box
One Year ago, I heard that my son's life expectancy could be 6, 8 or maybe 10 yrs.
One Year ago, I held Coleson in my lap as a lab tech searched for a vein to draw blood to no avail
One Year ago, I stuck a urine collection bag on Coleson to confirm the diagnosis and typing
One Year ago, I cried for the rest of the day, asking "why him, why me?"\
One Year ago, I stopped looking at my son as a normal 18 month old, and instead evaluated his every move, his every feature, his every word, comparing him to other kids and looking for signs that he did not have MPS
One Year ago, our journey began...

It really began at birth (actually at conception), though I wasn't informed of such.  Yes, there had been several signs something wasn't quite right, his bi-lateral inguinal hernias, his Pectis (sunken chest), his failed hearing screen, his Dyastasis recti (abdominal muscles not fused)....all things know within the first few weeks of life but did not seem to cause concern to his pediatrician, nor his surgeon.  There were several more subtle signs during that first year; he didn't crawl, couldn't point, didn't kick his legs during diaper changes, and more obvious signs; his head kept growing bigger and his body at a slower pace.  Though I kept questioning these differences, it wasn't until his 15 month well visit that his pediatrician showed the first signs of concern...

And since then, well much of it is in this blog.

One Year later, I wake up with my son in my bed, hooked up to an IV overnight
One Year later, I give him multiple medications morning, day and night
One Year later, we go to the hospital every Monday for blood draws and weekly evals
One Year later, we have therapists and nurses come to the house
One Year later he has hearing aids and glasses (though I haven't gotten the glasses yet)
One Year later, he has dark curly hair instead of straight drk blond hair
One Year later, I occasionally cry and say, "Why him and why me?"
One Year later, I still evaluate his every move, his every feature, his every word, not compared to other kids his age but compared to his "yesterday self".
One Year later, I run after him as he runs down the street excited to get beyond the gate to the main street with lots of vehicles; yellow school buses, red fire trucks, mommy's car (any silver SUV), big trucks
And One Year later, he is still alive and his life expectancy can be into his 20s and possibly longer.

Playing the drums with silverware

The journey is still just beginning and it will be a bumpy road but at least there is a road.















Below is a prior post that I wrote earlier this week but did not Publish yet.

In talking with one of my girlfriend's yesterday she said I woke up this morning and thought, "Oh, Valentine's Day is this week!"... Then she realized it's March!  This is how I feel.  It doesn't seem that 2 or 3 days should make a big difference in a month but February just escaped me.

We had an exciting past 2 weeks.  Aggie, Beth and Mina came to visit and 1 week later Aggie returned with  my Dad.  We got to reciprocate the hospitality that Janna and Steve Laumann have showed us in Minneapolis and had them up to the Ranch for dinner last Friday night.  I told Janna that since we will be in Minneapolis every summer for Coleson's annual checkups (and some times more) that I would like to put on an RMH dinner on or near the anniversary of Coleson's BMT, July 24th.  So all you local Honeywellers and Friends, be prepared for some support come July!  

We had a chance to put on a dinner for the PCH RMH 2 weeks ago.  Rachel organized her friend's and put on a great spread.  It was nice to be on the "other side of the buffet line".  I got a chance to eat with one family and share our stories.  With the different "groups" of friends that I have I hope to be able to do this frequently and especially on Coleson's original Birthday of October 4th.  If anyone is interested in organizing a dinner, I would be more than happy to lend a hand.  

That same weekend we had the Annual Plant sale at the FIGG garden.  We almost tripled our sales from the year before thanks to a lot of hard work by this amazing group of gardeners. I remember when the garden was just an empty lot and wow has it transformed in just 3 years!

Coleson is continuing to taper off steroids and CSA.  I hope by the next blog post that I can say he is off them completely and at the same time hopefully come of the overnight IV magnesium.  He has just blossomed over the past few weeks.  He is talking up a storm, very adventurous and wants to explore everywhere!  He is not satisfied in the confines any longer!  We are also exposing him to more kids (healthy ones) so that he can work on his social skills.  He seems to be playing independently side by side other kids just fine.  

Peyton continues to be a ham.  Her newest saying is "Of course" as in "Of course I like tomatoes, Of course I'd like to play with friends, Of course, I'd like to brush my teeth", it doesn't matter what comes after "Of course", everything is "of course".  And I'm fine with that!  At least it isn't "Of course not!"   She rode her horse, Tony this past weekend and we had plenty of quad rides too.  Coleson loves horses as well so Paul is a very excited that both kids will be able to enjoy this with him.  

T+217 Wear Jeans for Rare Genes on Feb 28th.

As always, I am late with my communications but tomorrow is "Rare Genes day" and to honor those like Coleson with rare genetic disorders, it is wear Jeans day.

Going back a couple weeks...

I had the best Valentine's day this year..  My son gave me the gift of his heart.  We met with the Cardiologist on Valentine's day and he said the fluid around his heart was minimal enough that we no longer have to have weekly echos.  We instead can have them every 3 months from here on out.  Many of you would think having echos every 3 months is not something to celebrate but this is a welcome relief to our busy weekly Dr. schedule.  Peyton gave me the gift of being an exceptional big sister, going to Coleson's visit with him, doing all the routine stuff like Blood Pressure, weight, heart listening, etc. right along side him.  My husband surprised me with an early Valentine's "Grown Up" dinner of Sushi and a movie (America Hustler).  Rachel stayed with the kids all evening.

We received more good news over the past few BMT Dr. visits in that we are continuing to taper the Steroids and CSA, his blood pressure is under control with the new dose of Amlodipine and his Magnesium is finally in the middle of the range.  I have increased his oral Magnesium with veracity in hopes we can discontinue the nightly IV magnesium.  We reduced the IV dose to 800mg this week and I am really hoping that in a week or 2 Dr. Douglas will stop them altogether.  He should be off CSA by March 20th and that is what depletes the Magnesium in the first place.  He's currently at 50% of his original dose.  He will need another dose of IVIG probably next week though and I hope that too will be his last.  I can't wait to get him off all these meds, get his central line taken out (replaced by a port) and get this kid in the pool and more importantly stop the weekly dressing changes which is ripping off a huge sticky bandage off his hairy little chest and under arm area.    We have been trying to swim when he naps because he so wants to swim...

Paul did well at the horse show despite riding very little this past year.  He took Grand Champion in one of his classes, catapulting him out of "Rooky" status.  He is now a Novice.  His trainer Crystal has been amazing support for him this past year and obviously has done great work with his horse, Rafiko or rather "Every Chic's Dream" (his show name which Paul just loves especially when it appears right under his own name!).

Aggie, Beth (Step mom and step sister) and Rachel's sister Mina just left after a short visit with us.  It was nice of Beth to want to make the trip out here to see her niece and nephew.  For those of you who don't know she has Muscular Dystrophy, another inherited disorder, and traveling is not easy on her.  Since we didn't make it home in the past year to see her, she came out to see us.  She and Coleson have so much in common now.

My good friend's mother, Connie, has been sitting with the kids on weekends/nights so I can catch up on "life" and I was able to put together a calendar of pics from the past year.  It is amazing to see Coleson change from pre-BMT, to during BMT to post BMT and to post, post BMT.  He went from dark blond straight hair to bald to curly dark hair.  It was also amazing to see how "sick" he looked after BMT or rather how healthy he looks now.  I didn't recognize it at the time or perhaps I didn't want to recognize it at the time, but now I can clearly see how tired and worn out he was in August, September, October, etc.    A BMT is one of the toughest procedures a body can take and not all of them do, I am fortunate that Coleson did.  I keep my friend's who were not so lucky in my thoughts every day.

T+200 and Leaky Hose

For those of you who are connected to my Coleson's Courage Page on Facebook, this will be pretty much a repeat of my post from this weekend.  For the rest of you, this will be new:

So my new analogy for this BMT rollercoaster ride is that it is like a leaky garden hose.  We tape up one leak just for another leak to pop up.  Last week his hemoglobin dropped to 7.7 so we had to spend another day in the Hospital getting a blood transfusion and IVIG.  Peyton and Rachel went with us as he his tolerance for hospital stays is minimal especially when you have to be cooped up in a hospital room for 5 hrs.  His blood pressure also took an unexpected rise over the past 2 weeks, requiring a couple doses of Irazipine and an increase in his amlodipine.  Also requiring frequent blood pressure checks which is just another thing I have to do "to him".  So far this week his BP has returned to "normal" so I'm hoping we do not have to go to yet another Dr. (Nephrology) He's been congested for the past 2 weeks as well, which just adds to the over all concern.  I think it's allergy related but it does seem to coincide with his steroid tapers.  We all had a bad case of allergies over the weekend (even Paul) so I hope we'll break out of this early allergy season (with the higher than norm temps) soon.
His echo last week was better than the last one we had in Phoenix which was on 1/13, I don't know in comparison to the one we had in Minnesota, but I'm hoping we can start nixing these weekly appts. off our schedule soon.
His platelets and WBC dropped this week compared to last (still within a normal range), probably as a reaction to the increase in hemoglobin and I hope next week all blood components will find harmony.  Dr. Douglas dropped his overnight fluids by 1/2 so he's only on 200ml of fluid overnight.  Now I only have to change his diaper once overnight which is a welcome relief.  We are continuing to taper his CSA which is what depletes his magnesium which is why he's on overnight fluids.  I long for the day we can stop them altogether.  It's a slow taper we started at 1ml/day total and we are not at .7ml/day total and will drop 1 ml per week.
Peyton is enjoying school, music, Spanish and gymnastics but not her new "Ben"...I replaced my stolen IPAD (Peyton's Ben) with a surface 2 (Microsoft tablet).  It doesn't have as good as apps for Peyton to play with but I like the comfort of a windows experience.  She has been such a good girl through all of this.  I constantly watch for clues that she is getting jealous or resentful of the attention Coleson gets and try to do things 1:1 with her.  This Sunday my girl friends took our kids to see the Cat in the Hat.  Kim and I even dressed up as Thing 1 and Thing 2.  

Paul has a bit of work travel this month and the Arabian Horse Show starting this weekend.  I know he doesn't get to ride Rafiko as much as he'd like and hope he can get back into doing so soon.
Aggie and Beth and Rachel's sister Mina are coming for a visit next weekend.  It will be nice to have the company.

And lastly the community garden I was formerly treasure of (no time nowadays) is having their annual plant sale on March 1st. so if you are in the Tempe area, stop by.

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!

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.  

T+151 BMT Jingle Bells

White Blood Cells, White Blood Cells

Donor Cells all the way

Oh what fun, it’ll be to see

My ANC level today

Red Blood Cells, Red Blood Cells

No infusions for me I pray

Can’t wait to see, what my hemoglobin will be

In my lab bloodstream yet today

CSA, CSA

GVHD stay away

Oh what fun, to see my skin again

Once all this body hair goes away!