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!

T+149 I'm Dreaming of a BMT Christmas and other BMT Christmas Songs

What  people may not know (although you are all getting familiar with it by now) is that a BMT is not a one day event, or even a 1 week, 1 month or even a 1 year event.  It is a life event that changes you forever, quite literally in fact.  We are nearing our Coleson's 5 month anniversary now and are so fortunate to be celebrating Christmas with him and at home!  Many of our RMH friend's are still there and will be celebrating Christmas there.  From what I hear Santa is very good to all the RMH kiddos -deservedly so!  Though I am sure they would much rather be at home for the holidays.  Coleson's labs this week were all in "range" with the exception of his Hemoglobin which was slightly lower than last weeks and slightly out of range.  We may expect to get another bump of red blood cells if it doesn't turn around.  Even better news came from the cardiologist in that the fluid around his heart seems to be less than it was when we left Minnesota!  Thus they are tapering off the steroids and lasix.  I am looking forward to him being a little less puffy and heavy!  He's getting to be a big boy to carry at 30ish lbs! He is eating like a horse again and has expanded his diet to Oreos, Goldfish crackers, lots of shaved ham and greek yogurt.  (He doesn't know this is actually good for him!) They also lessened the overnight fluids which is helping the overnight diaper changes a little.  Although last night I went to change him and the cool air must off caught him and before I could get the new one on, he had made our bed a waterbed!  People often ask me when I appear to be so happy how do I do it.. well let me tell you first of all, I have no choice.  It's not like I was asked if I wanted a "normal" child or a "special" child.  Second of all, what would happen to Coleson if I did "do it"?  After all, he's the one that is going to have a tough life, why wouldn't I do everything I can to make it even just a little bit easier on him.  Third, not all days are doom and gloom and when we have a "good" day, we need to celebrate it as if it were his last because the reality is, that it could be.  There are a million things that could change on a dime that could take him away from us, he could stop breathing, he could bleed out, his kidneys or liver could start to fail, his heart could become compromised, he could get a fungal, bacterial or viral infection that he won't be able to fight off.  He could come into contact with a kid who has chickenpox or had a live virus immunization.  The list is really endless, so I've learned to appreciate each and every day with my kids.  Also you have to find some time to laugh and make light of the situation, so while some may not appreciate my sense of humor with this...well, they can simply stop reading my blog!  This is my way of dealing with this and if you think it's inappropriate, that's not my problem!  :)  So in the spirit of Christmas, I've put a BMT spin on some classics. Hope you enjoy...

Twas the night of BMT and all through the hospital room

We were all waiting for the bone marrow to turn up soon

The IV lines were connected to his hickman line with care

In the hopes that new marrow would soon be there

Coleson was snuggled all cozy in bed

The rest of us starting to bow are own heads

And Paul in his BMT day shirt and I in mine too

As well as was Peyton and the rest of our crew

When through the door there came a nurse with the marrow

I looked up from my chair and was brought out of my sorrow

To his bedside I flew and picked up this boy of mine

Hugged him as I created him for the very last time

The glow of the light shone down on his eyes

As mine welled up with tears and cries

When suddenly what to my eyes did appear

The bag of O+ blood cells, along with some fear

The nurse hung it and hooked him up quick

I knew in a moment that this was it.

More rapid than enzyme the blood was infused

His body I’m sure must have been confused

Now faster, now quicker, the cells they did flow

Oh cells, oh cells please do start to grow!

To the top of his head to the bottom of his toe

Grow little cells, grow little cells, grow little cells grow!

Then it was over, the last drop of blood ran through his IV line

And spread into his veins like a wild grape vine

So into his body new marrow was there

With the enzyme he needed which made him so rare

And then briefly it was quiet as the crew did go

And left me alone with my new little boy

As I held him close with the look of amazed

I felt him start to tremor and look kind of dazed

He started to get sick and felt really hot

Surely something was wrong was it not?

The nurses I called and sprang into action

I wasn’t prepared for this kind of reaction

They changed him and washed him and gave him some meds

And he soon settled down as he lay in his bed

His body was still, his butt in the air

What he had been through, truly unfair

His little mouth let out a little cry

And mine, well it let out a deep sigh

His hair lay gently on his cute little head

I knew soon it would be gone, this I did dread

He had a broad face, and a little round belly

That shook when he laugh'd, like a bowl full of jelly:

The donor cells went straight to work that night,

And fill'd his body with platelets and cells, red and white

And soon there were would be enough so he could go outside

The chemo, the ERT, we had been on quite a ride

And laying his finger aside of his nose

With his thumb in his mouth he began to dose

And I heard him coo as he fell asleep that night —

Happy rebirth to you son, I know all will be right!

***Our Christmas came early this year on the night of July 24^th/25^th with the rebirth of our son Coleson via a Bone Marrow Transplant***

On the 12^th day of BMT by Doctors gave to me:

12 pre-BMT ERTS

11 nights of vomiting

10 nights on morphine

9 scans/MRIs/EKGs/Echos/X-Rays

8 days of chemo

7 nights with fever

6 nights with blow by

5 IV pumps a pumping

4 intrathecal injections

3 days of campath

2 hickman line surgeries

And a bag of marrow for my BMT

T+138 Lights, Friends and Hot Chocolate

Coleson was released from the hospital on Thursday afternoon.  The echo from that morning showed that the fluid had decreased a bit.  We went home on Lasix and Steroids and back to weekly echos.  If the fluid doesn't go away, he most likely will have a "window" put in so that the fluid can drain out into his body and won't be able to accumulate again. Labs on Friday were good but it looked like his White Blood Cells and neutrophil count was trending down and he was really lethargic on Friday so I was still a bit worried going into the weekend.

Despite my worries, we had a nice weekend taking a drive on Friday night to see Christmas lights and did some stuff at our Ranch... (I haven't been there in almost 6 months) and stopped to see Paul's horse before coming home and watching Michigan State (my alma mater) beat Ohio State for the Big Ten Championship and a trip to the Rose bowl.  Unfortunately Arizona State did not fair as well against Stanford so I will not get to see both my alma maters duke it out at the Rose Bowl.

Sunday I woke up early to a cold Arizona morning (It was 56 degrees, some of you might not think this is all too cold for a winter morning), and put on my Coleson's Courage T-shirt for the Hot Chocolate 5k with some of my best friends.  The race benefitted the Ronald McDonald House and we got Hot Chocolate and Chocolate fondue with some tasty treats so it was worth the 5:30 alarm clock.    Then Peyton and I headed to good friend Grant's Firetruck Birthday Party and then to the FIGG Garden Social (FIGG is community garden I am (rather was) treasurer of).  A few of my garden friends designed the Coleson's Courage T-shirt and ran as well.

To my relief, today's labs were much better.  His White Blood Cells and neutrophils were back up, his platelets and Red Blood Cells were back up and though his hemoglobin was just slightly low to range, his reticulyte count was strong which should indicate the hemoglobin will be higher next time.  His next echo is tomorrow and I hope the fluid is continuing to decrease.

It's been a week since Johnny passed away and it was a highly emotional week for those of us close to his family.  Reading the posts and "watching" them from afar go through the grieving process and the funeral arrangements, etc. hits very close to home with me. A BMT is a highly risky procedure but for certain diseases and disorders really the only option available to try to save your child's life.  Though Coleson has reached his second "milestone" of being able to come home, he is and far from being risk free and in fact will never be risk-free.  It won't be until his 1 year mark that they will call it a successful transplant (as long as he's stable) and he will be able to resume some of the activities that he is banned from doing today.