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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.

Tuesday, April 15, 2014

T+266 Caught Offguard

"I'm sorry for calling your son a monkey" came the apology from the 10 yr. old girl.  Coleson and I were at McDonalds yesterday while Peyton was at gymnastics.  He hasn't been able to go many places beside children's hospitals and other places were looking different is the norm so I was caught off guard by the apology.  I hadn't heard the girl call him that, but I had witnessed the mother call her down from the play structure and talk to her just minutes before.  I hadn't paid attention to their exchange but then realized the mother must of overheard the kids or at least her daughter making fun of Coleson and made her apologize for it.   I hadn't practiced responses to such interactions.  I have coached Peyton through situations when she is curious about some one else's "owies" and had responded to others when they were inquisitive about Coleson but had not prepared a response for an apology for rude comments.  I fought back tears as I accepted her apology.  I know he must look a bit odd with all his hair and his unusual facial features but I only see a beautiful boy when I look at him and so I guess I was a bit naive to how he looks to others.  The girl went back to playing and I cringed at the lost opportunity to explain why he looked different.  As we were leaving another little girl (probably 5 or 6) asked why he looked so weird and I was able to tell her that he had been sick and the medication made his hair grow but that it was OK to look different.  Her Grandma patted her head and added that she also had unique characteristics since she was a red head.

I know this wasn't the first time he was made fun and certainly won't be the last, and I'm sure I will get used to it, but it took me by surprise for sure.  I won't let another opportunity escape where I can address the reason why he looks different.  Kuddos to the mother and grandmother who addressed the situations as they did.  I know there will be times where mothers won't reacted in the same educational/informative way.

On a happier note, Coleson's BMT doctor reduced some more meds and took him off 1 more as well as changed our appts. to bi-weekly instead of weekly.  I was starting to worry about his hemoglobin level last week as it was steadily marching down week after week and last Monday was at 10.1 (he needs to be at least at 11.5 to be in normal range).  However, all of his counts including his Hemoglobin (10.8) and electrolyte numbers were up this week.  He also had an echo last Thursday and though we do not have the official results, I'm thinking that if there still any concerns with the pericardial effusion that we would have heard by now.  We also talked about his Hickman line removal/port placement around Mid-May.  I'm looking forward to the reduction in hospital trips though I know they need to be replaced with other appts. now.

I also got to see 2 of my BMT mother friend's (Debbie and Kathy) last weekend.  It was nice to be in the company of people who just "know".  Though our kid's illnesses are different, we have the common bond of the BMT and going through such a life altering experience.

I'm hoping too as we round out this year that my focus will be able to shift from Coleson's immediate needs to doing more fundraising/awareness, etc.  I will soon start to utilize some of the other "pages" of this blog that I intentions of doing from the start.  It's amazing what we've been able to do for the MPS society and Ronald McDonald houses in at least 4 different states already without a real focus so I'm excited to start giving back and helping others that come after us!

Sunday, April 6, 2014

T+254 "Well It's Hurlers..."

"Well it's Hurlers" the geneticist said bluntly. The 3 little words I dreaded hearing. It came like a punch in the gut even though I thought I had prepared for it.

In the 3 weeks since first seeing the geneticist I had diagnosed Coleson myself with MPS. There are several symptoms and I could put a check mark next to almost every one. Also, thanks to my boss and her boss, I had gotten in to see another geneticist at Phoenix Children's Hospital, Dr. Aleck who was more experienced in metabolic and lysosomal disorders who also was very confident he had an MPS disorder, but we needed the official lab results for the type.  Despite all this, I still held out Hope that he didn't have MPS and that it was merely coincidence that he displayed some of the symptoms and features.

"Do you have any questions?"
"Hurlers" I said, "that's MPS I, right?" I wanted to make sure of the designation as treatments differed greatly between types.
"Yes"
"No, no other questions".
The geneticist kept speaking but I didn't hear another word.
That phone call that lasted less than a minute would forever change my life.

With those 3 little words I was thrust into the category of "Super Mom", a title reserved for only the bravest of mothers of kids with special needs like Nikki, Laurel and Aggie. I didn't belong in this group. Somehow I had reasoned that their kids were born to them because they possessed super mom powers. I held those mothers on a high pedestal. I did not possess such powers and certainly did not belong on the same pedestal.

There was no time to grieve for the normalcy that was lost. I had done my research in preparation that it was Hurler's and knew that it was critical to get the BMT done before the age of 2 to prevent cognitive decline. There were only 3 hospitals that were experienced in BMTs for Hurler's and U of Minnesota had the most advanced protocol which included an experimental treatment (trials) of 4 inthrathecal injections of the artificial enzyme into the spinal cord in order to get the enzyme into the brain. They also used the other near term treatment of ERT, artificial enzyme replacement therapy, prior to BMT which was not offered at all hospitals. This would immediately get the missing enzyme into his body prior to the BMT, but it would not pass the blood/brain barrier which is why they were trialing the inthrathecal method. Logistically, none of the hospitals were local so we would have to temporarily move for 5 months to have the BMT. There was no time to even worry about this yet. We just needed to get Coleson the Best treatment available.

I emailed Dr. Paul Orchard at University of Minnesota on Saturday and by Sunday he had emailed me back twice, with his entire team on copy. A BMT doctor emailing me on a weekend?! I knew I had made the right choice in Drs./hospitals. Up until then, I had felt all of Coleson's Drs. here had failed us. He had seen several, his pediatrician, his surgeon, his ENT, the first Geneticist (the one who delivered the bad news), none of them had taken Coleson's symptoms seriously or reacted with any sense of urgency. In fact it took 3 weeks to get diagnosed because the geneticist dropped the ball on the lab work (and the MRI). Finally though I felt like we were in the right hands. On Monday, the intake nurse called me at 5:30 am on her way to work to brief me on what I needed to send her so we could arrange the grueling week long baseline testing and evaluation process.

When Coleson was diagnosed he was the only pediatric Hurler case in the valley. Hurlers and MPS belong to the "Rare and Orphan" diseases. The chance of having a child with MPS is 1 in 100,000 to 250,000. In Coleson's case, he was 1 in 5 million living in the Phoenix valley. There were a few other MPS families with other forms but none of the other forms go through BMT and unfortunately some do not have ERT available either. I felt very rare and orphan myself.

Ironically today, on the 1 year anniversary of Coleson's diagnosis, I learned and spoke to another potential Hurler family in the valley. They are in the agonizing waiting-for-the-lab-results stage. Fortunately Dr. Aleck can provide them with a little more guidance than he could us by using Coleson as a reference point.  This is the one of the positives about having a special child, there are numerous ways to give back and help others. Coleson's journey will help others who come after him just as we have benefited from those who have come before us.

I still remember my first phone call with a Hurler dad. Even though we were thousands of miles apart, he was able to give me some hope and lots of info about the journey we were just starting. With that call I felt a little less "rare and orphan". I was also lucky enough to meet him and his boys in person in Minnesota. Thanks Todd for that first conversation and for all the others as well.

This is one reason why I write my blog.  It serves as a historical reference for me and a way to communicate to the masses but also a guide for others.  This is the way the those of us in the Hurler family learn from eachother (blogs and facebook).  This is how I spend my free time, reading blogs and posts from other Hurler and BMT families. We are fortunate to live in a time with these resources and those that came 20, 10 and even 5 years before us did not have these invaluable resources.

Friday, March 28, 2014

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.

Thursday, March 13, 2014

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.  

Thursday, February 27, 2014

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.









Tuesday, February 11, 2014

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.

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!