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, November 13, 2014

My version of a bad day

Just to warn you this is a "venting" post perhaps a rant. You know, I have learned to come to terms with our new "normal", earning frequent "traveler" awards from Phoenix Children's Hospital, the endless Dr. appoints, the mountain high stack of bills and insurance claims, my ear permanently red from all the phone calls scheduling appoints, dealing with the insurance, following up with Dr.s... the list goes on...  And I've adjusted to that normal as stressful as that is but when things with that new normal don't go right, I really struggle with keeping my composure.  

So Coleson gets a weekly Enzyme Replacement Therapy.  It is about a 4-5 hr. infusion of an artificial Enzyme that costs $10,000 a week!  Yes, that is right, the drug alone is over $1/2 million a year. That is why I call him my Million dollar baby.  It's not fun since he has to have a needle stuck in his port and he's hooked up to an IV so he's fairly limited in what he can do.   Typically though he naps for about 2 hrs of it and watches TV once he wakes.   We have been getting them at home since around May.  We had an awesome home health care nurse that gave it to him and she had zero issues.  The only 2 times we have had issues with this is when we've been in Michigan.  The first time, we ended up in the ER because the nurse could not get the heparin in after the infusion (heparin is what prevents clotting in the port).   This was after a few attempts to re-access his port (meaning a few more needle pokes).   The last time we were there for my dad's passing we had to scramble to get things arranged and manged to do so but somehow the pump settings changed during the infusion from ml/hr to mg/hr. So about 2.5 hrs into the infusion, the nurse noticed the bag was still full of drug and it took us a little while to figure out what was wrong.  Again leading to multiple needle pokes.   Yesterday I get a call from a nurse saying she's schedule to do Coleson's infusion today.  She sounded a bit flaky about it so I was already worried about it.  Our regular nurse had not said anything about not coming.  The first thing that went wrong (notice I said the First), was that she forgot to unclamp the line after she accessed him (stuck the needle in him) so she did not get any blood return (indicating she was in fact in the port), so she re-accessed him (a new needle and poke), before she finally figured out what she was doing wrong.  Clamping and unclamping the line should be instinctual for a nurse.  At 5:00ish, I came out of my office to see how close they were to being done when I looked at the drug bag and noticed it was completely full, meaning he had been hooked up for over 4 hours for no reason.  So we tried to troubleshoot why and without knowing the root cause, we re-started the process and kept a close eye on the line to ensure the drug was moving through it.  The initial infusion rate starts at 2 ml/hr which is like watching a snail crawl.  This meant that he would be hooked up for another 4-5 hrs. and we were planning on going out to dinner for Peyton's birthday.  Fortunately our nanny was able to come back to stay with Coleson so Paul and I could take Peyton out still.  When we returned from dinner, I noticed that Coleson's chest was swollen.  This means the fluid is going into his body and not the port (vein).  So I immediately stopped the infusion, told her we were "done" and when she tried to flush his line, the needle practically popped out.  There was no way I was going to allow her to try to put the needle back in so we called the Dr. and he said it should be Ok to leave it until this morning to allow the swelling to go done.  Rachel did tell us after the nurse left that they had already changed his dressing once (the tape that keeps the needle in) which should have been a clear indication to the nurse that the needle was in fact NOT in the port.  At one point I had to play referree b/w Paul and the nurse as Paul cannot tolerate incompetence and the nurse was trying to justify her actions.  Fortunately he does listen to me when I try to diffuse the situation.  So the nurse left, I'm sure she felt awful as well, and I do feel bad for her.  But we just wasted a $10,000 supply of critically important drug and put Coleson through a lot of unnecessary pain and frustration (not to mention me as well).  It's events like these that are to blame for my unusually high blood pressure readings recently.   To top things off, I just ate some apparently bad, though freshly opened, not to expire until December Turkey pepperoni.  There's another $5 down the train for antibiotic free, uncured, turkey pepperoni.  

Thank goodness I have this forum to release my frustrations, my job to keep my mental health in tact, and my friends and all of you to keep me going.  

Wednesday, November 5, 2014

Bittersweet Anniversary

One year ago, after 138 days in Minneapolis, Coleson was given the green light to come home.  It was a day filled with mixed emotions as we said goodbye to the Doctors that had given him a new/better life and the more than amazing friends we made along the way.  During this time frame I watched my son go from a seemingly healthy boy to being totally dependent on tubes, machines and medicine to keep him alive, to a resilient survivor, 

This year in the same time frame I watched my 74 year old dad go from a seemingly healthy cancer survivor to a courageous face death head-on hero to a peaceful angel.  I’m not sure which was a tougher experience. As a parent, one should never have to watch their child go through what Coleson went through and yet as a child you are never old enough to watch your parent die.   

I don’t know that there is a good way to die, unless you are fortunate enough to have lived a good long life, your days no longer have purpose and you pass away in your sleep.  But my dad did it in about as good a way as you can.  It was unexpected, yes, but not immediate like a car accident or heart attack. He had time to say several goodbyes and take care of many loose ends.  It wasn't drawn out so he had little, if any suffering and we did not have to spend many days watching him digress, listening to his every breath, wondering if today would be “his day”.    Once he was given the prognosis, he took matter into his own hands and I believe was in almost total control up until the very last minute.  He did not want us to go through what he went through with my mother.  He was at peace with his destiny, though of course he wished he could live longer, he wasn’t afraid or mad.  He told me he really will miss not seeing Coleson grow up  and see what he will achieve.  I know that to be true with all of his grandkids, but of course, Coleson is special. I know how hard Coleson’s BMT was on him as well.  You never want to watch your own child and grandchild faced with such challenges.   

He passed very peacefully on Monday, October 6th at around 2:30pm.   He waited until we will all there, making sure Grandma (his mother who is 99) got there so she would be with us when he passed.  She had a chance to say goodbye and give him a kiss as we all had several times throughout the day.  Within 5-10 minutes of us leaving Aggie in the room alone with him giving her time to say her final goodbyes and to tell him that she wanted to spend every last second with him, he took 3 quick and final breaths.  It was quick and peaceful.  It happened to rain that day and there was a bought of thunder just after he passed and of course we took that  as his way of letting us know he was knocking on the heavenly gates.  A rainbow appeared thereafter.  

Of course the following days are really too busy for the immediate family to mourn, making funeral arrangements, writing obituaries, putting together picture boards and a video montage of his life.  As we all went through the years of photos, I couldn’t help to think that we should have been doing this for his 75th birthday, not his funeral.  I also noticed that my dad rarely took a bad photo.  I wish I could say the same for me.. He had a natural and warm smile and his eyes were bright.  We had a hard time narrowing it down to less than 60.   Here is the video montage John Lewis Video Montage
and the funeral home tribute:

We had 2 visitations.  I think I already mentioned how he thought no one would show up for his funeral but the line was out the door with friends, family, colleagues, neighbors, church members etc.  What can I say about the funeral itself… I think it’s the most difficult of all the ceremonies, it marks the final goodbye.  A family friend sang a very touching song, The Field Behind the Plow.  www.youtube.com/watch?v=PUM8mXJre1c.  He rode away in an old 1939 Cadillac Hearse, a perfect final ride for him. 

I haven’t had a lot of time to really mourn since but it does find ways to creep in every now and then.  As you know I live a very busy life and we have had several events in the past month including Coleson’s birthday party, the Be the Match race, several friend’s birthday parties, Halloween and preparing for this weekend’s community garage sale and Peyton’s birthday party.  Also tomorrow my friends and Sole Sports are putting on another Ronald McDonald house dinner at Phoenix Children’s hospital.  I think this makes 5 RMH dinner’s this year on behalf of Coleson. 

I want to thank all my friends who sent flowers and plants, cards and gifts, phone calls and texts, and invited me to lunches and dinners.  You are the reason why I am able to continue to face life’s big challenges, you are what keep me going day after day when it life isn’t fair, you are my strength.  Someone asked me how they can help me and it really is little things like these that help me the most.  And just to appreciate life and family and to show compassion for those who are less fortunate.    

Thursday, October 23, 2014

Video of our TV Appearance

Well as it goes with TV, we were actually on Sonoran Living at 9:45am.  It was a 5 minute spot.

Coleson was great and I won't comment much on my own appearance other than the start seemed so dramatic that I got teary right away.  It seems that day sitting in the geneticists office was so long ago and the events that took place afterward were so rapid that I forget how devastating it actually was.


I appreciate all the generous donations and support for Coleson's crew.  We are sitting in 4th place as a team at $2085 and I am sitting in 3rd place as an individual at $1005.00.  Friday is the last day to donate.  The money is used to support research and to help offset the medical costs of the uninsured.

Here is my personal link again (this goes against my individual contributor numbers and the team).
Colesons Crew Be The Match Run/Walk

Wednesday, October 22, 2014

TV Appearance Date

Good News.. thanks to several generous donations yesterday Coleson's Crew is still in 3rd place by a narrow margin for the Be The Match Run/Walk.

Also, It appears I did not provide the date of the TV spot.  It Thursday Oct 23 b/w 8:00am - 9:00am (I suspect in the second half hour but not certain) on ABC 15 in Phoenix.

Monday, October 20, 2014

Be The Match 5K and TV Appearance

As many of you know and most of you probably assumed, my father passed away very peacefully soon after my last blog posting on October 6th.   There is so much more that I want to write about that but my Dad always said, “Life is for the Living” and our life is very busy,  so I will have to find another time to circle back around on that “chapter”….

What is keeping us busy these days is school, birthdays and the Be The Match 5k this Saturday.  First of all, Coleson started pre-school last week.  It was kind of chaotic with us being gone the 2 weeks prior but we managed to get all of the paperwork and IEP done for his first day.  He was a little off the first week on his sleep schedule being on Michigan
time still so the first 2 days he did shed some tears for 20-30 minutes when he was tired. By his 3rd day he only shed tears while dropping off and today was even better.  Drop offs are a bit chaotic and overwhelming so the teacher agreed we could hand him off to an aide who would take him immediately into the classroom so he could start playing  

We also had a joint birthday party for his 3rd and our friend Quinn’s 4th birthday.   We had it at his favorite place, Great Play.  It was amazing to watch him respond to the instructors requests to have the “birthday boys” lead certain activities or to sit in a specific location.  Since part of his developmental needs is in the receptive/responsive communication area, I feel like Great Play is a really good addition to his “therapy” schedule.  He is really thriving.. a far advancement from last year when we were getting admitted back into the hospital for the pleural effusion at this time. 

Lastly, the Be the Match 5k run/walk is this Saturday and Coleson’s Crew is sitting in 3rd place for team fundraising.  I don’t think we’ll reach the number 1 or 2 spot but I am hoping to hold onto 3rd place.  In addition, Be The Match (the bone marrow/cord blood registry organization) is doing a story on Coleson inclusive of a short TV spot on ABC 15.  I’m not sure exactly what time it will air but I think it’s between the 8:00 – 9:00am morning hour on ABC 15, so set your DVRs so you can see us. 

Also, if you are interested in helping us hold on to third place for fundraising, you can donate below:

Or google Coleson's Crew Be The Match.  
(The URL will go to my personal home page which I prefer, google will take you to the team page.)

Monday, October 6, 2014

My First Super Hero

This post is dedicated to the first Super Hero in my life, my Dad.  As I write this, he is fighting his last courageous battle, and soon will raise his white flag.  As with all of my other lost loved ones, Cancer has finally found another unique way to take him.  Three years ago, Bladder Cancer won a battle against my dad, leaving him bladder-less,  but did not win the war.  My dad was wounded but came out victorious. It slowed him down temporarily but he rebounded, changed his life plan and became strong again. Little did we know that Cancer too was re-strategizing.  This time it sent in faster, stronger troops, that could stay undetected for longer, covering more area and had iron clad defense.  They sent in Mesothelioma.  For those of you who are not familiar with Mesothelioma (though you may have seen advertisements on TV for the victims of it), it is a rare form of cancer that is found in the thin lining of the lung, chest and the abdomen and heart, its cause is Asbestos.  It can lay dormant in the body for decades, leaving many of the victims wondering where they might have been exposed to it.  My dad has always been a hard working man and could have been exposed from a variety of sources over his 74 years.  He first passion is farming and he has done so for most of his life.  He held a variety of jobs during his teens and early adult hood including electrical and construction type work.  He spent 34 years as Director of University Services at Michigan State University.  He purchased an old farm in the early 70s and with much love, turned it into our beautiful family home.  The same home where my mom passed from breast cancer 34 years ago and where he too will take his last breath.  I am thankful and I know he is too that he can spend his last days here with all of us as opposed to a hospital.  And I am thankful that we have a chance to say our goodbyes as well.  As so many of his friends have been able to do as well.  The phone has been ringing non-stop and our door barely closes before another friend stops by to deliver food or say their goodbyes.  My dad once joked (modest as he is) that he wasn't sure anyone would come to his funeral so I am glad he knows now just how influential and loved he was.  My dad could be a little rough around the edges, a savvy business man, direct and with little patience but most of all a very hard worker who expected the same of others.  He also was very fair, social and likable guy.  I think this combination of traits earned him a lot of respect.  He was also very smart and wise, wiser than I gave him credit for as a teenager.  I don't think I got away with as much as I thought I did, I think he just picked the times that he thought I needed a little more fatherly direction. In fact some of the stories he shared with us during this past week were some of those times.  One of his favorite stories he has often shared with me was when I was a little girl, perhaps 5 years old, when I said one day, "Daddy, I wish I had a full-time mommy".   While my mother had been sick spending most of my toddler years in the hospital before passing when I had just turned 4, we had several nannies.  How it must have broken his heart to hear me say that.  But it wasn't long after that when my wish came true and he met Aggie. Though they didn't marry until I was 13, she filled that role from the start.  We are all so lucky to have her and I am especially happy that he had 32 wonderful years with her.  I can't and don't want to imagine what it was like for him at the age of 40 left to raise us 3 girls alone.   He always said, "You girls raised me". 
This last battle started for him just before labor day when he went to the ER with chest pains and lack of breath.  He had a Plueral Effusion (fluid around his lung), which they drained over 1 1/2 liters of fluid from only to reveal a mass.  A few days later they took a biopsy of the mass.  A few days later he was released from the hospital but not before seeing the lab diagnosis of the mass as "malignant mesothelioma, sarcomatoid type".  He went home with this information but without being able to talk to an Oncologist.  He had to wait 12 days before seeing his oncologist but in the meantime we had all done some research on Mesothelioma and knew there was not going to be a happy ending but that there may be treatment options including surgery, radiation and chemo.  When he finally got into the Oncologist, they were not certain it was Mesothelioma but rather it could be his bladder cancer had metasticized. Which if this were the case, there was no hope.  They were going to have Mayo take a look at his labs/scans to help confirm the diagnosis.  In the mean time, they had set up an appointment at University of Michigan who were more specialized in Mesothelioma.  That appt was 2 weeks ago Wednesday.  Clarity came with this appt.  They were certain of the diagnosis of Mesothelioma, the rarest, most agressive, most untreatable form... It was too large for surgery or radiation and he wasn't strong enough for chemo.  He was sent home, with the number for hospice care.  
At this point in time he was already weak from his prior hospital stay, but he was finally able to put his mind to rest with the diagnosis and prognosis.  He was at peace with knowing his future and was content with his past.  We didn't know how long he would have but it became clearer every day that it would not be a year.. then it would not be months.. then it would not be weeks and then not even days.  I came home last Tuesday just in time for him to have 2 last days of relatively understandable communication. He was able to talk to all of us girls and be able to say what he wanted to before he went.  The last few days since we have seen him slip away more and more...although did I mention he was stubborn?  He is... and his mind will just not let him go. He still shows us an eye roll or a raised brow when we talk to him.   But it is never easy to watch someone so strong be in pain and not be able to do a whole lot to help.  We have all said our goodbyes and now are just waiting for him to say his.     

This is about all I can write for now.  

Monday, September 1, 2014

Lots of "Firsts"

First of all, notice I did not prefix this post title with the days post BMT.  Now that we have surpassed the 1 year mark, it's time to stop living by the number of days post BMT.  It's also time that my baby becomes a little boy and so we've done several little boy things.  First we went to the dentist.  Peyton loved the X-rays of her teeth...she is fascinated with bones...and despite being a little nervous actually enjoyed getting her teeth cleaned.  I think she liked playing with the water gun and suction more than anything.  Coleson did not enjoy the X-rays but we managed to get 1 of his front teeth which revealed a cavity behind his front tooth.  The Dentist said he would not have caught it without the X-ray but it needs to be pulled.  It's either that or a baby root canal which leaves the possibility of infection which we need to avoid at all costs... in this case a missing tooth until his adult one grows in in about 3-4 years. 


Then it was his first official hair cut.  The only other hair cut he's had was post-BMT when we had to shave it.  There were lots of tears during that one..both his and mine... but this time it was a much better experience for both of us.  He got to sit in a "car" salon chair and watch a movie.  He looks so handsome with his big boy haircut and for those of you worried that his curls would be forever gone... they're not.  He's still got his curls in the front and on top and already his hair is starting to show curl in the back. 

He also started Gymnastics.  A parent and tot class so that I can ensure he doesn't do anything that would compromise his back and neck.  But those of you who know me, know that I want to encourage as much gross motor skill development as possible, even turning my own house into a mini gym.  I am struggling to find a pediatric developmental physical therapy place that my insurance covers which is within a reasonable distance as we spend a lot of time in the car going to appts.  I have found a rehab/physical therapy place that my neighbor Teresa works at which is not necessarily for pediatric development skills but will give him some stretches. 

Lastly he got a big boy room inclusive of a bunk bed and new wall decals of Cars. His sheets are Thomas the Train and Cars.  I think Peyton is equally as excited to play on his new bed.  Peyton got the queen bed that was in Coleson's room before.  She's not sure if she likes the bigger bed as she really like her full bed. 

He has an allergy apt, a new pediatrician, and a cardiologist apt and a dental surgeon coming up in the next couple weeks so we can determine which surgeries (PDA in his heart, tooth extraction) he needs and when.  Also he will start to get his newborn immunizations. 

Paul and I also had our first get away to Sedona for our anniversary.  It was a quick 24 hrs. but we packed as much fun, exercise and relaxation as we could.  We had an amazing room view and some good hikes and pool/hot tub time.  It sure beats last year's anniversary in the hospital. 

We are getting ready for the Be The Match fundraiser on September 27th and the run on October 25th.  They are going to do an article on Coleson.  More details to come in a separate post.

Lastly the Ronald McDonald House's dog Jerry passed away from Cancer.  He spread so much joy to the families at the RMH and ironically helped heal many cancer patients.  It's another sad loss to those of us who knew him. 

As always it's been very busy 'round here.  I may not post as often as I'd like to but being the mother of a very special boy and girl too, while working full time,  is more challenging

Friday, August 15, 2014

Faces of the Ronald McDonald House - Upper Midwest 2013

How does one express what it's like living at a Ronald McDonald House for 5 months?  If I were on Glee I would do it through song.  But since I don't have a good singing voice, I put together this video from RMH Upper Midwest Summer - Fall 2013 inclusive of many of the amazing kids (patients and siblings) and their equally amazing parents. It's 15 minutes long but how do you condense 5 months into much less than that? I will always appreciate the Ronald McDonald House Upper Midwest and the friends I met while there.  

This video contains music so is not available to be viewed on phones. Sorry, you'll have to use your computers!

Monday, August 4, 2014

T+365 +++ Happy Re-Birthday Coleson and our summer Trip

I know you all have been waiting patiently for an update our trip to Minneapolis... I have been pretty sick since we returned home with a Sinus infection and as I am going on day 7 of this crud, I'm only feeling moderately better. I'm even on the z-pack.  What's worse is Coleson has the same thing and even though we are 1 year post transplant I am monitoring him like a hawk.

As I logged on to write this post I realized that I never published my last post from a month ago. So I apologize that it has been 2 months since you've last received an update. 

Well our trip to Minneapolis was a very busy, mostly positive, very emotional one.  We got to spend our first day having lunch with friends Nicole and Kelly and then to Janna and Steve's for a family afternoon and dinner. 

Bright and Early Monday morning kicked off our Jammed pack week of Dr.s visits, procedures and celebrations.  First up with the neuropsych evaluation.  This is never a fun appointment!  After a few hours of testing cognitive, social, speech, fine motor and gross motor skills we were told that Coleson is still testing at the average level for the cognitive, social and speech skills but is slightly below for fine and gross motor skills.   This was to be expected but of course you never want to hear it confirmed by a Dr.  We then went to endocrinology appt. where we heard some average news again.  He did grow in the past year but remained on the same growth curve with is the < 1% compared to kids his age.  We know he will be exceptionally short - hopefully he will make it to the 4 foot range, but I was hoping he has made up some growth as he appears to me to have been growing so fast.  Next we were on to see his Dr. Miller, his BMT Dr. and have an EKG.  This appointment was way too quick.  There are so many questions that I didn't get answered as we were already running behind schedule to get his chest X-rays and to drive to St. Paul to Gillett's Children's hospital to meet with our first orthopedic Dr.  Since he had not had labs drawn yet, this was just a clinical observations but Dr. Miller thought he looked great.  The neurologist quickly assessed him as well though by this time he was sleeping so again, a quick observation/assessment with positive remarks.  We then re-scheduled his chest x-ray and went to St. Paul.  I tell you, it was heartbreaking seeing all the kids with some very serious orthopedic issues.  It was difficult to get a leg X-ray but we managed with both Paul and I holding him.  Dr. Walker said he had typical Hurler hip/leg development with incomplete hip sockets that most likely would need surgery in a couple years to fix.  He is slightly knock kneed but it is too early to tell if this will correct itself or if this too will require surgery in the future.  After an exhausting first day we headed to the RMH for dinner. 

It was great to see the staff and to see some of the renovations that the All-Star organization helped pay for.  We did not expect to see Peyton's friend Coco and Nana to still be there but unfortunately for them, they still were.  We thought they had gotten to go back home to Saudi Arabia (and they thought they were too) but at the last minute, when they pulled Coco's line out, discovered fungus in the line and so she had to stay for treatment.  We also were disappointed to see another family still there... Carter had neuroblastoma and after enduring and surviving what I can only say was a hellish treatment of chemo, radiation and BMT, they had actually gone home, cancer free.  They returned 3 weeks later after he complained of back pain only to find 3 more lumps in his back and were told there was nothing else they could do.  So they were there just for pain management as Carter lived his last days.  The RMH was busy with big families.. more people then were there last year, there was not an empty table for dinner.   Paul unloaded about 25lbs worth of soda tabs that traveled with us from Phoenix.  We filled up half the container.  Thank you to all of you who collected these for us especially Amanda and Charlotte. 

Tuesday was an early morning as Coleson had surgery to repair his hernia and while sedated to get an MRI, EMG (for carpel tunnel) and his chest x-Rays.  I'm not sure it is a good thing when you go back for pre-op and you know the pre-op nurse, the anesthesiologist, both surgeons, etc.  However, it is comforting to have a set of Dr.s who have worked with Coleson before.  After a 4 hr. sedation and procedures, we met back up in Post-op. He was very sore after his hernia surgery and he barely made it up in time for us to make it to his pulmonology appointment at 3:10.  After a painstakingly long appt. which could have been shortened greatly (especially considering what Coleson had been through that day already) we received positive news that his lungs looked good and we could start to wean off the inhalers.  We had a fun dinner with our BMT family (Siegels, Christensens and Bells). 

If you thought our week sounded exhausting already, Wednesday was actually our busiest day.  We kicked it off with an eye exam where again we had slightly positive news in that his nearsightedness and astigmatism mostly canceled each other out so wearing glasses is not necessary, however is good practice if we can get him to put them on occasionally.  We then had Physical Therapy who gave us some recommendations then an echo and cardiology assessment.  It was this appointment that we received the most frustrating news...she wants to fix Coleson's PDA which has been a known issue since before BMT but wasn't worrisome enough to delay BMT.  But now, he should really get it fixed.  Wait, weren't we just in surgery the day before?  I was hoping we were done with surgeries for a while.  And not only would this require 1 surgery but 2 surgeries as he should really get his port removed for this surgery and the replaced after infection risk has passed.  Lovely! 

We then moved on to audiology testing and ENT.  He only lasted so long in the sound booth as by this time he was exhausted.  They observed no change from previous assessments so we need to stick with hearing aids and speech therapy but at least both tubes are still in his ears. 

Finally, we were able to go to the RMH where with the help of our Honeywell family and super boss woman organizer Janna, we pulled off an amazing dinner.  Paul and Jack (Caleb's dad) were the grillers and Cary was the runner with all the chicken and hot dogs.  There were many Honeywellers there to serve and whom made all kinds of salads, beans, cookies, etc.  I am so fortunate to have the support of my work place and to be in a location to have their support.  It was a crazy night for me as I met two new Hurler families that night and we celebrated with our BMT family 4 1 yr. anniversaries (Caleb, Coleson, Lizzie and Ari).  It was a night we had looked forward to for a year and were so appreciative that we got to celebrate it together.  Another Hurler family we are close with was also there that week so we also got to celebrate Braden's 8 year anniversary. 

Thursday was our last day of appointments starting with the Orthopedics for the spine.  We heard fairly positive news thank goodness in that the only issue he really has is the slight kyphosis which is measuring at 42degrees. Otherwise the rest of his spine and neck looked good.  We need to keep him strengthening his back so the kyphosis doesn't worsen and may even get better.  We had our first break in appts so we headed back to the hotel for a nap...and I ran over to the Mall of America for a 2 hr. power shopping.  I only hit 4 stores but it was enough for my credit card company to call me to verify my card had not been stolen!  That tells you how infrequently I shop! 

We finished up our day at the Orthopedics for the wrist where again, we got positive news in that he shows no sign of carpel tunnel or digit trigger issues.  We went back to the RMH to have our last day with our BMT family and to of course to play Bingo! 

Friday Peyton and I went to the water park with the Siegel's and then we all went out to lunch before we said our "see you soon"s.  They will be coming to Phoenix to run the Be the Match 5k in October, a cause so near and dear to us.  (I will posting a blog update on this soon). We made it to Lincoln Nebraska by 9:00 pm and on Saturday to Elizabeth Colorado where Oscar and Charlotte welcomed us into their home for the night with a nice dinner and night of conversation.  Charlotte and Oscar have supported us greatly over the past year in so many ways.  We are so blessed to have them as part of our extended family.
We left Co. at 7:00 am for our longest drive of 12 hrs making a stop at Little Anita's in Albuquerque and then into the up and down of the Northern AZ mountains.  We had amazing weather until we hit ALB and AZ where it down poured and was dark for hours until we hit the valley of the sun.  You would not know we were in the same state.  14 hrs after we piled into the car, we made it home.  Upon which my sinuses started to fill and rendered me fairly useless for most of the week. 

It was an exhausting vacation.. if you can call that a vacation.  I don't know when we will be able to take a real vacation which isn't centered around hospital visits or Coleson's disease.  It was an exhausting year...but we survived it with the support of amazing friends and family. 

Sunday, August 3, 2014

T+331 One Year Ago we arrived in Minneapolis

One year ago today, we arrived in Minneapolis after a 28hr, 1820 mile car ride with stops in Albuquerque, Colorado Springs, Peyton Co., (a special stop just for Peyton) and  Lincoln, NE.  We had no idea what the next 5 months would have in store for us nor did we know if we would be returning with both kids, but we were certain that the bone marrow transplant was Coleson's best chance to extend his life and help stop the progression of his very awful disease. 

A lot has happened since my last post, and this delay in posts means I am simply too busy having fun with my family (not to mention being really busy at work).  The most important event was Coleson's hickman removal and his port placement in mid-may.  This allows him to be submerged in water again and he has thoroughly enjoyed baths and swimming.  Not to mention so has Peyton!  We have been swimming practically every day!  Peyton has been showing him how to swim and even agreed to put on her "hippo" (lifejacket) to show Coleson how he can swim on his own.  He much prefers holding on to mom though!  I think the "hippo" stretches out his shoulders more than he is comfortable with.  He has limited range of motion from the disease.  However, in just a few weeks he has learned to swim (rather jump) from the steps to me standing a couple feet away (underwater of course).  I most enjoy not having to do dressing changes.  I hated how much it hurt him. 

I always hate to send my baby off to surgery(it never gets easier), but he was all smiles and giggles before they took him back and this always helps. 

Peyton has been missing going to school but did go to School camp and Gymnastics camp to help bide her time.  She also started swim lessons.  As mentioned before, she is a little mermaid when it comes to swimming and she could stay in the pool all day long but she needs to learn all actual strokes and kicks.  She is more interested in going down the big slides after swim lessons are over.  Have I mentioned how fearless she is!  She looks like Paul but she is my daughter! 
We also have been busy having play dates with friends and I now can take both kids.  There have been too many to mention but some of the favs over the past month have been the butterfly museum, playing with 10 new pups, and most recently seeing Peter Pan The Musical. 
Tinkerbelle and mommy!

Peyton teaching Coleson how to drive

I finally took Coleson to get his glasses.  Both kids had fun trying on glasses but now that the lenses are prescription strength, he doesn't like them as much.  He much prefers Daddy's aviator sunglasses!   I couldn't get a pic of him fast enough but he is adorable!
By the way, Coleson's hair is getting really curly and long, though thinning out from its thickest point.  I have been tempted to get it cut but want to wait until after we return from his 1 year visit in Minnesota.   Meanwhile the body hair is slowly thinning out as well.  Can't wait until this all goes away!

Lastly, we helped serve dinner at the Ronal McDonald House @ Phoenix Children's hospital this past weekend.  A neighbor organized the dinner in Coleson's honor, Thanks Teresa.  We had lots of food and volunteers. I'm looking forward to serving dinner at the RMH Minneapolis when we are there in July!


Monday, May 19, 2014

T+292 Angel Wings come in 3s

Today is a sad day in the MPS I family as two Hurler kids earned their Angel wings.  One actually was an adult but she had had a transplant as a child.  I didn't know of her but she must have been one of the first to have had a transplant.  She was 27 years old.  It gives me hope that the woman made it into her mid-20s but at the same time it is a reminder that the BMT was not a cure, just a life extender.. and for how long, no one knows.  In fact, I don't know how old the "oldest" Hurler kid has lived to be.  She may have been it.  The other ones that I am aware of that are Hurler and had transplants are in their early to mid-20s.  She passed from complications with surgery, I don't know what kind of surgery.  These kids undergo so much trauma in their life from the BMT to multiple surgeries, sedations, etc.  The body can only handle so much.

The other was a 10 month old baby who was 27 days post transplant at University of Minnesota.  Baby Vincent's death hits me particularly hard as it was just 9  months ago that Coleson was at that mark at the same place.  I was in contact with his family in the beginning and was planning on seeing them when we go for Coleson's 1 year in July.  To see pictures of him, in the same hospital PJs, in the same hospital room, with the similar looking facial features is very surreal.  That could have been Coleson.

As you read this, Coleson will be in surgery to remove his Hickman line and get his port.  Please pray he endures yet another sedation, another surgery.  I remember thinking when he got his inguinal hernias repaired at 10 weeks old that I hoped it was his last surgery.  Boy, how I long for just the "simple" surgeries of a hernia repair!  I wear very different glasses now.

They say things happen in "3s" so in other sad news, a colleague's husband passed away last week after what I can only describe as an extraordinary fight with appendix cancer. He was diagnosed soon after Coleson was born.  There should be a picture of Joe Kahler in the Hall of Cancer Fame.  His Courage, strength, determination and his love for family is what I will remember most about Joe.  He put a check next to many items on his bucket list even after diagnosis that many would not have.  

On a positive note, Coleson was weaned off Potassium today and tapered off 1 dose of his BP medicine. He also has been switched to monthly Dr. consults with bi-weekly labs.  This means I only have to go to PCH once/month and get his labs done at the PCH clinic in the east valley making that trip a lot shorter.

Please hug your kids, your spouses, your siblings and other family members today and be thankful every day for what you have.  I know I am!

Tuesday, May 13, 2014

T+286 Wear Purple on May 15th for International MPS Day

This Thursday is International MPS Day.  Wear purple in support of Coleson and all other MPS families.  
Also, today was Coleson's last dressing change. Hooray!  Next Tuesday (5/20) he goes in for his line removal and port placement.  He will finally be able to take a real bath and go for a real swim!

Here is a short You Tube video narrated by Alec Baldwin on lysosmal disorders.


Here is some info on MPS as provided by another MPS mother.Thanks Amber Mongan

Treatment for MPS I (Hurler syndrome): Successful HSCT (Hematopoietic Stem Cell Transplantation) has been performed for children with Hurler syndrome since 1980. The immediate benefits include correction of the enzyme deficiency and clearance of glycosaminoglycans (GAGs). Long-term benefits include the possibility of long-term survival by protecting the heart, lungs, and brain from the effects of progression of the MPS disorder. Other organs and tissues can also show benefits from the HSCT; these include the eyes and ears, liver, spleen, joints, airway, etc. However, it should be noted that many children are still requiring a variety of orthopedic surgeries despite a successful transplant. While the term “cure” should not be used, HSCT has the longest track record of any effective therapy for Hurler syndrome, including the ability to preserve cognitive function and development in the normal range. 

Brought to you by Be The Match: WWW.BeTheMatch.OrgA bone marrow or cord blood transplant is the only treatment that can stop the effects of Hurler syndrome at this time. A bone marrow or cord blood transplant begins with chemotherapy, with or without radiation, to destroy the diseased cells and marrow. The transplant replaces diseased blood-forming cells with healthy ones.
The type of transplant used for Hurler syndrome is an allogeneic transplant. This type of transplant uses healthy blood-forming cells from a family member, unrelated donor, or umbilical cord blood unit.
For an allogeneic transplant, a patient gets chemotherapy, with or without radiation, prior to transplant to prepare his or her body for the treatment. Then the replacement cells are infused into the patient’s blood stream. From there, the cells find their way into the bone marrow, where they start making healthy red blood cells, white blood cells and platelets. These new cells have the enzyme needed to break down GAG and stop further damage to the body.

It is because of the shortcomings of the current treatments (and the lack of treatments entirely) that organizations such as The National MPS Society, Hunter Syndrome Foundation, Team Sanfillipo, Hunter Syndrome Research Coalition, Ryan Dant Foundation, and numerous other parent based organizations exist. If it were not for the willingness of families to not only say “Something has to change” but also to stand up and do something about it, doctors like Emil Kakkis would have lost funding for research before they were able to bring a treatment to reality for us. These family organizations work not only at funding promising new research, but also at advocating inside of the FDA and congress to educate on MPS, and negotiate the best ways to bring safe and effective treatments through the system as quickly as possible so that families can gain access before it is too late to help their child.
This year International MPS Awareness Day falls on a Thursday (May 15th) so many families will be celebrating with fundraising events today. The success these events translates to saving the lives. It translates to hope. So make sure you ask the MPS Family you love how you can help give them hope today.
“What we needed was one day. Whatever money you could raise in one day, somewhere somebody could use it.” -Mark Dant  MPS I HS Dad

Friday, May 9, 2014

T+283 A Mother's Day Surprise - a Gift from Strangers

I know... you don't hear from me for 3 weeks and now 3 posts in 1 week!  I was just so moved, not once but twice in the past 30 minutes that I couldn't wait to post for fear that I would get too busy again and not share the beautiful gift that Paul and I received today - mostly from strangers!

First a dear colleague of mine (you know who you are) honored me with a donation to the RMH in my name for Mother's day.  It is the best Mother's day gift that I could receive (well, with the exception of a miracle)!

Secondly, an envelope was delivered today and inside several very generous gifts from complete strangers, spearheaded by one of Paul's dearest friends (again, you know who you are!)  I am literally shaking from the generosity of these people who have not met us nor Coleson, but only know us through the stories of our dear friends (and perhaps/hopefully this blog).  And these friends have already done so much for us, in so many different ways.  If those 16 families read this blog, I sincerely thank you, and to O&C, words cannot express how appreciative we are.  You have made this Mother's day, the best I've ever had.

To all you mothers, I hope your special day is/was as good as mine.  I have met some amazing mothers this past year that will stop at nothing (even the US Gov't and health care system) and who sacrifice everything for their children.   And of course, I already was surrounded by amazing mothers both professionally and personally that do the same for their children day in and day out.

Happy Mother's Day!

Thursday, May 8, 2014

T+282 Oh Peyton

It's time I dedicate one of these posts to my other Hurler kid, because when you have one special needs kid, all of your kids have special needs.  You have to work extra hard as a parent to ensure that all of your kids feel like they receive the same amount of attention as the one that requires the special attention.  I have to say, Peyton has been the best daughter and big sister in the world...of course I'm biased! Precocious Peyton, aka Miss P., aka Sweet P, aka.. many other special nicknames, has taken all of our life adjustments in stride. She is curious about all of the medical stuff and loves to go to the hospital, play with the OT, Connie, get her blood pressure taken, her heart rate and all the other medical things that Coleson "gets" to do.  She is sad when she doesn't "get" to do them with Coleson.  
Besides being the best big sister patient ever (and wanting to be a nurse or a doctor), she has become an amazing swimmer, gymnast and horseback rider!  Since heating the pool this winter (knowing this is one thing Coleson will eventually be able to enjoy), she has become a fish!  Or rather a mermaid.  She swims back and forth the length of the pool, under water the short ways and above water the long ways.  She can dive down and get a small key ring off the bottom of the floor as well.  
She has gotten to "ring the bell" at gymnastics a few times recently and her fearlessness enables her to try all the scarier tricks.  You should see her bounce around our house from the balance beam to the back of the couch to the trampoline to the crashpad.  Our house has become a min-gym, mostly to help Coleson's gross motor skills but Peyton obviously loves the new equipment as well!  I think I've already mentioned how she has aced the monkey bars and has the blisters to prove it!
She also is starting to ride her horse, Tony, all by herself; completing turns and stops all on her own, much to Paul's delight!  We don't own Tony but I can predict we'll be buying another horse in our future.  A new horse is nothing to Coleson's yearly medical bills after all!   
And lastly she is just finishing her first year of school where she has made several friends.  She aced all of her objectives and the teacher commented that she is very compassionate.  And I agree.  She always sticks up for her friends (in a nice way) and mediates any disputes.  She shares very well.  She also wears her heart on her sleeve and does get very hurt, "Mommy my heart hurts" when someone says something mean to her or doesn't want to play with her.  She will do anything for her friends.  Her teacher also mentioned what a good story teller she is.  And she is, although she likes to tell stories when she really shouldn't as well.  She does have quite an imagination though.  And the things she says continue to crack me up.  Sometimes I have to laugh before addressing the reason she's upset from some of her comments.  If my memory was better, I'd give you more examples, but it's pretty much turned to mush!  She also sometimes breaks out in a British accent like Peppa Pig.  She always sounds much more polite when she does this!
Thanks for letting me post (Ok, Brag) about Peyton.  Don't get me wrong, she does have her moments, but overall I am so proud of her.  I know how hard it was for me as a little girl to adjust to some of my life's big adjustments and though she does get frustrated with Coleson every now and then (what siblings don't?!), she really has weathered this storm very well.  I hope she continues to do so as the storm ebbs and flows for the rest of Coleson's life.  

I don't condone climbing on play structures!
Everything is subject to climbing!

T+280 40 Weeks - the first and the most recent

40 weeks... The gestation period for humans.  A time in expecting parents life that is riddled with emotions, anxieties and excitement.  I have to admit my first 40 weeks with Coleson were a lot easier than these past 40 weeks.  I was, in my opinion, a pretty relaxed expectant mother with my second pregnancy.  I naively wasn't worried about the baby being OK, we hadn't known the sex of our baby, wanting it to be a surprise. My second pregnancy was much like my first, both were born a little over 1 week early, they were within 1 oz. of each other, Coleson being bigger.  Of course they were very different babies.
This past nine months however have been a different story. I am no longer "naive" about what can happen to innocent children.  Now I am quite aware of the tragedies that can happen to children, either at birth, as toddlers or as adolescents or teenagers.  I see it day in and day out, from living at the Ronald McDonald House or going to the hospital or reading on facebook.  I've seen all kinds of cancers and anemias, babies born severely pre-maturely, kids who need organ transplants who know that for them to live, someone else must die.   "Butterfly" kids (EB) who have to be bandaged day in and day out, meningitis and SIDS survivors and others.  Kids with all kinds of hardware and tubes attached to them keeping them alive.  Bald heads and heads covered with hats or spunky colored wigs.  Wheelchairs and braces supporting them.  Kids who face chemo and radiation, and even isolation. Surgeries, BMTs, Transfusions, therapies and medications.   Kids who survive and some that don't... all equally courageous as they put up the fight for their life.. quite literally.
I wasn't a Facebook fan until this all happened with Coleson and now I find myself swiping through my news feed reading up on all my new special friends looking for something I know I won't find... a miracle.  I swipe with vigor hoping to find a post of a Hurler child who has not had dozens of surgeries, hospital stays, therapies etc.  I wish I had more time to enjoy the everyday posts but right now I'm focused on connecting with all the other Hurler families and learning from them.  As well as keeping up with good lifelong friends we've met along our way.
Speaking of, two of our RMH families who both had been residents of the RMH for  2 years (yes, 2 years) are home or going home soon.  Cash was 5 weeks old when he was found not breathing in his crib and was resuscitated.  He spent 690 days in the hospital before finally returning home just in time for his 2nd birthday.  Another family, here from Saudi Arabia for their eldest daughter's cancer.  Coco and younger sister Nana were Peyton's best friends at the RMH house.  They arrived in the US in January of 2012 and to the RMH in March of 2012.  They have just been given the green light to return home.  Peyton will be sad that they won't be there when we return in July but I am so happy for them.  Now what does that say about the value of a RMH!  I still say it's priceless!  And oh, BTW, both families gave birth to another child during their stay. On a side note, I currently have nearly 20 lbs of soda tabs ready to deliver to the RMH.  Thanks to all for collecting them!  We leave in early July for our trip to Minnesota so please collect and drop off as much as you can before then. We'll be driving so we can actually take them to "our" RMH.

Coleson has had C-Dif for the past couple of weeks so he's back on antibiotics but otherwise his counts remained fairly stable from 2 weeks ago. We are making progress on weaning off some meds.  He is scheduled for surgery on May 20th to remove his hickman line and replace it with a port so he can finally bathe and swim in our pool.  This makes surgery/procedure #6 (although some of his surgeries were multiple procedures in 1 so it's really higher than that).  As always the scariest part for a Hurler kid with a narrow airway is the sedation.
March's Meds
May's Meds
Lastly, MPS Awareness day is May 15th.  I'll blog more about this specifically over the next few days.

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.
"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.