Happy Holidays

Happy Holidays to one and all!

 

We have had a good 4^th quarter here at chez David.  We started the quarter with a trip home in October for fall break.  October 6^th was the anniversary of my father’s passing and we buried his ashes (those that weren’t spread elsewhere) next to my mother.  Aggie had a very nice headstone with a barn made, very apropos for my dad.  It of course brought all the sadness of it all back to the surface but at the same time at least for me, it also gave me a sense of peace with it all as well.  I have been visiting that cemetery for 35 years now since my mother passed away and I find comfort in knowing she is watching over him (showing him the ropes so to speak)!   My Aunt Rose took our family pictures at the farm while we were there. 

 

The rest of the quarter was inundated with birthday parties, including Peyton’s 6^th.  She has lost her 2 bottom teeth already.  She is doing very well in school but longs for the Pre-K days when school was only half a day and there was no homework!  Her freedom is getting squashed at Kindergarten, she doesn’t have as much time to play at the park these days.  I thought she would enjoy being at school with friends all day but I think being at school with friends and playing at the park with friends is very different.  Despite this though, she is exceling academically and behaviorally. 

 

Coleson is also doing quite well.  In fact, I think he has had only 1 sick day from school, not because he was sick but because there was strep throat in the Pre-school classes and I didn’t want to chance it.  After we got back from home, we enrolled him in a Sensory Learning Program.  Think of it like exercising the neuron pathway to the brain that controls focus, stability, attention and communication.  He had to lay flat on a table that moved in a circular motion while staring at a flashing light and listening to headphones with music that would oscillate through the decibels.   He had to do this 2x/day for 30 minutes ea. for 12 days straight.    By the time we got him into it, I wasn’t quite sure that he really needed it as he has progressed to be in the average range in every area.  However, I figured it could only help him and so we went through with it.  Thank you to those who donated to Coleson’s cause so that we could afford to send him there!  He actually did quite well despite it being a pretty intense 12 days and I have seen a difference in his attention, focus and his communication skills.  He still remembers going and everyday says, “No Sirri”, which is the name of the place we did this at.   Rachel did most of the weekday sessions with him and I think she says the same thing! 

 

We have scheduled his surgery for next year for May 24^th and I booked a house for the entire summer.  I really think this will be better for us all.  I still don’t know how to break the news to him as he is very much aware of what happens at hospitals now and this surgery will only make that nervousness worse.  He has gotten a few shots lately (vaccines and flu) and though he handled them all quite well (better than Peyton),  there is no reason in the world that a 4 year old will understand why he is able to run into the hospital fine but come out with a double cast and in a lot of pain. 

 

We took the kids to Polar Express this weekend with some neighbor friends and I think Coleson really understands Santa now.  Nothing like meeting him in person!  I hope all of you have a happy holiday season.  If you are thinking about ways to help out others during the season, I have 2 recommendations:

 

1.  If you are in Arizona, you can make a dollar for dollar tax credit on your Arizona income taxes by donating to Waggoner Elementary School of up to $200 single/$400 joint.  You don’t have to have kids in school.  It is a direct $ for $ so why wouldn’t you want this money to go to a school instead of to the government.  Kyrene.org/taxcredit, select Waggoner Elementary. 
2. Also, you can sign up on Be The Match   (www.bethematch.org) as a bone marrow donor (or if you don’t qualify, you can donate directly).  It’s very simple.  This video, a parody of Taylor Swift’s “Bad Blood”, puts a light hearted spin to the Bone Marrow Transplant process.        A Bone Marrow Transplant parody of Taylor Swift's Bad Blood

Hope your holidays are merry and bright!

From Be The Match to Walk For Wishes

It came as a surprise and with much disappointment that I learned the Be The Match run/walk event has been discontinued in Phoenix.  Even though you all helped me raise over $1000 last year, it wasn’t enough for them to return this year.  However, I am very excited for another equally worthy charity and event this year, Walk For Wishes.
 

I am on the Phoenix Chapter of the LGR Diversity and Inclusion Network at Honeywell.  The purpose of our chapter is to provide professional development and networking opportunities for our Phoenix LGR community and to engage in at least 1 community service event per year. As our launch event, we have formed a team to Walk for Wishes on Sunday October 4, 2015 in support of Make a Wish Arizona.

 

As you may know, MAW grants wishes to children with life-threatening medical conditions.  Coleson will eventually be granted a wish from this wonderful organization at a point in time he is able to decide for himself what that wish will be.  This year’s event just so happens to fall on Coleson’s 4^th birthday.   I wonder what Coleson will wish for as he’s blowing out his candle this year. 

 There is no cost to walk with the team, but any and all donations are welcome! The event consists of a 3K fun run/walk and a 1 mile fun run/walk. We will incorporate some networking before or after the event with details to follow. Please join the team and bring your families to this great event! Unfortunately, we have tickets to go to Michigan for fall break and unless we switch our plane tickets will not be at the event ourselves but there are already quite a few people signed up.

 

To join our team and/or donate to MAW, click the link below: Walk for Wishes Donation Page

 

Wheels on the Bus....

For Peyton, the wheels on the bus going round and round are just another exciting part of her daily school routine.  She started Kindergarten this year and loves every bit of it, including her transportation home.  She is making lots of new friends and is very well behaved in school.  Each day she is so excited to share how many "Paw-sitives" she has to bring home or what level she clipped up to and if she got a blue or purple for her daily behavior rating.  I am so proud of her.  Despite the attention that we have to give Coleson and what our family has been through, she has turned out to be one of the biggest hearted big sisters and loves her family and friends very much.  She behaves and listens at school and always stands up for her friends or gives them a hug if they are having a bad day... and it's all genuine.  The other day I took the kids to Great Play for open gym and while Peyton is older than many of the kids she took 3 little 18mo girls (2 were twins) and started leading them to different play areas, picking them up if they fell down, asking them what toys they wanted, etc.  I can't believe my fearless little girl who would rather ride the scariest ride than play with dolls has also found her inner nurturer.  Couldn't be prouder.
















For Coleson, on the other hand, the wheels on the bus have stopped going round and round (at least home from school, he still sings the song constantly).  After a couple weeks of school (second year pre-school) I thought he would enjoy riding the bus home 2 days/week.  He loves vehicles and buses and rode one last year as a day trip with his class and loved it.  Well, I don't think the first day went well as he was the first one on the bus the first day and didn't know what was going on.  It was scary for him and after 2 weeks of riding the bus home he started having some behavior issues at school, particularly those days he was going to ride the bus home.  So I canceled the bus and he has gone back to his usual happy self.  We'll try again next year maybe.  Otherwise I had his IEP review with the school and he is doing great.  Progressing in all areas with still some underdeveloped areas to focus on, but not major ones.   His teacher has seen a tremendous difference in his interaction with his peers and his openness to new things.  His class is smaller than last year and is more his age so I think this helps as well. 

After much hesitation, we decided to have tubes put back in his ears.  These are not the normal PE Tubes but T-Tubes which are a little bigger and are supposed to last longer.  I got a lot of feedback from the Hurler community and several Hurler kids have had multiple sets of PE Tubes, adenoids removed and other things to help the ears so I felt like we had to do it.  My contemplations for not wanting to do them was because there is a lot of opinion out there that they shouldn't swim with tubes and this is one of the best and most enjoyable things Coleson can do and does do almost daily.  The other is just for the mere fact that this was another procedure and another sedation and he is getting older and realizes what is about to happen which makes doing these things a lot harder.  So far, his emotional well being has stayed strong but with every poke, Dr. visit, surgery, etc. I just fear that he will start to get frustrated by his condition.  But I knew it was for the best so we arrive at 6:00am and were home by 9:00am and he was off and running once we got out of the car.  He is such a trooper.  Unfortunately, he won't be able to be "off and running" after his next surgery and that is when I fear he will start to realize the unfairness of his life. 

Speaking of unfair...his favorite place, Great Play, is closing next week.  He goes to Great Play 3x a week.  It is our version of "Physical Therapy" and the timing is perfect as Rachel can take him right after school 2 days a week and I take him on Saturdays.  He loves Buddy (the mascot) and looks forward to going every day.  It's how we get him excited to go to school on Monday and Wednesdays!   We held an early birthday party for him there last night just to get as much Buddy time in.  I do not know how to break the news to him.  Fortunately we will be heading off to Michigan the first week's it's closed and I hope that that helps the transition.  I can not find anything similar that works well with his school.  Other places have more morning classes when he's in school.  Anyone want to open a Great Play in the Tempe/Chandler area?  :)

The kids and I and Rachel made a last minute trip to Rocky Point with our friend Kim and her kids and nanny.  Paul was on a golf trip with his buddies and after several years of Kim inviting us, I happened to ask her what she was doing for the weekend and when she invited us to go, I did not have a good excuse not to, even Rachel could come with so I had help.  Coleson is healthy right now, and doesn't have any near term surgeries, appts. etc. so we went.  It was a nice getaway and the kids loved it.  Peyton was so excited when she got of the bus to learn we were packing up to go with her friends on a trip. 

The Good, the Bad and the Ugly

I believe I had a prior post with the same title… C’est la Vie! 

As promised, a medical run down of our week in Minneapolis.  You’ll know why I separated these posts about half way through it. 

 The Good

Our week started with fasting labs followed by a consult with Dr. Wes Miller, Coleson’s BMT Dr.  I feel consult is a more appropriate description for out visits and none of our appts. are straight forward.  There is a lot of back and forth and decision making that happens within these windows of time and I need to take full advantage of the time we have with these Dr.s in person.  It’s hard to have your medical “team” be 2000 miles away from you.  We do have local counterparts here for many of the areas but the Hurler experts are in Minnesota.  Remember Coleson was the first BMT Hurler patient in the valley in the last 20 years. 

Wes (yes, we are on a first name basis with our Dr.s) thought he looked great! His chemistry was all in “normal” range. It will be a few weeks yet before we get his engraftment and enzyme levels which I am always anxious to receive.  Last month we saw our local Phoenix geneticist and they took GAG & Antibody levels.  These 4 tests are the key indicators of how Coleson is doing from a “Hurler”/BMT view.

Wes Miller - much better photo than 2 years ago.

Engraftment - means what percent of “Jacob cells” (I no longer need to refer to them as donor cells) he is producing.  He was 100% last year and of course what we hope continues.

Enzyme levels – the amount of enzyme he is producing (0% = Hurler), and he was within normal range last year at 49%.  The higher the better for this normal to eat away at some of the GAG (build up in his body that occurred pre-transplant that wreaks havocs on joints, bones and muscles).

GAG – As mentioned above the amount of “Stored” build-up in his body.  He was at 85 last year and post-transplant has held steady at 5 for the past 2 years.

Antibody Level – Is he building antibodies against the enzyme, rendering the transplant not effective?  This came back negative (which is good).

 We talked about continued Enzyme Replacement Therapy and he felt that is still very beneficial and according to a recent study that U of Minn participated in, the more enzyme circulating in the blood stream the better.  So for now, he will continue.  We even got a half way decent photo with Wes, nurse coordinator Teresa and Paul Orchard, the head Hurler BMT Dr. at the U and is highly involved in the clinical trials and advancements in Hurler and Storage disorder treatments.  I will never forget his email response to me on that Sunday afternoon!

Paul Orchard, BMT, Paul and Coleson with his Recycle Truck

We had X-Rays, EKG and Echo next.  I say “we” as I often have to hold Coleson during everyone one of these procedures to keep him calm.  He really is a trooper no doubt but can you imagine, we are already at our 5^th “procedure” on our first day and it’s not even noon!

 

 

 

 

 

 

We will miss Teresa!  Good Luck at NP school.

In the afternoon we had neurology and cardiology consults.  Neurology was painless but the wait was not!  We were in the room for 45 minutes before thankfully a music therapist showed up and Coleson got to take out some much needed energy on the drums!  He loves the drums.  Another 15 minutes later, the neurologist arrived and gave him a gold start.  He doesn’t show any signs of nervous system or brain impact.  He actually is quite smart!

The cardiologist consult was the last of the day and overall his heart condition was great!  He has minor thickening of the mitrial valve but it is not impacting his heart function…but

 The Bad

… I was hoping his PDA had closed on its own but this was wishful thinking as she confirmed it was still there, not surprising to her, and we were still on schedule for his heart surgery to fix the PDA on Wednesday.

Tuesday was an earlier morning and I pulled Coleson right out of bed and into the car where he promptly said, “No hospital, No hospital!”  Broke my heart and we were only starting day 2!   I promised him no “owies” though I am not sure how honest I was being considering we started the day with a 4 hr. neuropsych evaluation.  It is one of the most dreaded appts. for me, nothing like over analyzing everything your kid does/doesn’t do.  However, for him, hopefully it feels more like playing.

 
The Good
However, I guess the 4 hr. duration was worth it as he made progress in every developmental area, even his receptive/expressive communication which is the one area he had been a little lagging in.  They stressed the continued need for speech therapy for his enunciation and OT for his fine motor skill development.  He scored average in both areas while his cognitive scores were still above average (he can count to 100 already!)  I often wonder what he would have been like had he not had Hurler but these are fleeting thoughts as there is no purpose on dwelling on the what ifs.  We quickly squeezed in a pulmonary consult which happens to be on the same floor where which he sailed through with flying colors, despite the respiratory scare we had the week before. Then we were off to St. Paul to Gillet’s Children’s hospital for a hand ultrasound (to check for carpel tunnel) and a consult with the hand Ortho.

 Technical Solutions Difficulties (the bad)

When we checked in, we were handed a tracking device and a buzzer similar to what is used for restaurant wait lists.  As the newly named Director of Contracts Technical Solutions, I am always impressed with new technology that better enables a process.  After waiting 45 minutes without a "buzz", I waited in line for the receptionist.  There was only 1 of her and 3 of “me” in line so it took 30 minutes to get to her.  By that time we had missed the consult with the Dr. and when I handed her my tracking device and buzzer, she informed me that the buzzer had not been assigned correctly!  As you just read, we had already been through 5 hrs. of appts.,  travel to 2 hospitals and an 1:15 hr. wait. I was not happy to say the least!

Customer Service (the good)
However, the receptionist provided great customer service which always makes these things a little easier - right HW colleagues?  J and got us right into the Ortho for our consult and based on her range of motion exercises with Coleson determined that his wrists, elbows and shoulders had actually improved and we could skip the ultrasound!  I guess it was worth the wait to skip another “picture taking” session!

Are you exhausted yet?  We are only on Tuesday people!

The Ugly
Wednesday started early, with a 5:00am wake-up call and a 6:00am check-in time.  After the usual 1:30mins of pre-op, meeting with the Dr.s, nurses and anesthesiologists (who I have the utmost respect for!), he was taken back to the OR.  It never gets easier no matter how many times we have been through this routine.  It is comforting to see familiar faces, although I would be happy if I never saw any of the surgical team ever again!  J  Its funny how even the smell of the surgical ward is familiar... and we have our typical routine.  I struck a conversation with another mother whose son was getting his adenoids out, a first timer.  I feel like a pro at the waiting room protocol and let her discuss all of her anxieties, nervousness, etc…  Only then did I tell her that this was at least number 10 for Coleson (I have to finish documenting all of his surgeries to be sure and how do I count multiple procedures at one time?)   

 He was supposed to have an MRI after the procedure but they failed to realize that they would be inserting a metal coil into him for his PDA and putting him into a gigantic magnet right afterward may not be the best idea!  I thought perhaps we would escape the MRI this year but since he is participating in a trial for the intrathecal enzyme therapy, it is required… after all the intent of the intrathecal is too deliver the enzyme to the brain.  So unfortunately we will have to follow up in Phoenix with an MRI after the tissue has formed around the newly placed coil (6-8 weeks).  Though the procedure itself is fairly low risk, the recovery from it is difficult.  He has to lie still, strapped to a board for 4 hrs. while the blood clots around the incision areas (one in the vein and another in the artery in his right groin).  It took him longer to wake up from this surgery than prior surgeries and I was getting anxious to get back to him post-op.  I always forget how cold it is in the PACU and we were there for 4 hrs.  Longer than required as they needed to one last echo to ensure the coil stayed in place and though they called the echo tech @ 1:00, they didn’t arrive until 2:00, just my luck the tech was training so not only did we have to wait for her to do the echo, he got a “bonus” echo by the trainee (Just our luck).  No problem if this had occurred during the 4 hr. lie motion less post-op but this was hr. 5 and all 3 of us were getting a bit restless.   We still had to be careful of his incisions and as we arrived at Janna and Steve’s for dinner and he comes running out of the car I mention how I have to ensure blood doesn’t start gushing out of his incisions!  You would have never known that he had spent from 6:00am to 3:00 in the “surgical process” besides the gnarly bruise and the new “beauty marks” (scars)!

What day are we on again?!

The Bad
Ah yes, another pull him out of bed right into the car for the audiology testing.  I was hoping for good results since the ABR he had in December showed normal hearing activity and his vocabulary and communication skills have improved greatly.  However, the pressure in both ears was not equalizing and he does have fluid in his left ear (his eustachian tube is not as vertical as it should be).  So they only got one recordable event which happened to be normal range but recommended he get tubes in his ears again (3^rd time is a charm)!

Next stop endocrinology with a new Dr.  Dr. Polgreen who had seen the Hurler kids for years left to go to California to do research and clinical trials for Hurler.  I was skeptical to see Dr. Miller (Brad– not Wes) but was pleasantly surprised and really like him.  He was so good with Coleson and explained everything very thoroughly, though it did confirm that the thyroid medicine that Coleson has been on for about 3 months is keeping his TSH levels in normal range (which is a good thing) but just confirms that he has hypothyroidism.  I was hoping his TSH would be too low and would indicate Thyroid medication is not needed.  It’s not the end the world but it is a daily medication… for which I have found a new transport vehicle… Reese’s Peanut Butter Cups!  The crushed pill sticks to the PB and he is none the wiser!    

 The Good

Other than the above news, he is growing according to the chart and actually gained a %.  He is now on the 2% growth curve!  We talked in great detail about Growth Hormone, a controversial topic and one that we do not need to get to deep into yet.  We traveled to the Spine Center to see Dr. Schwender, neck and spine ortho.  This was probably one of the most positive consults.  After more “pictures”, Coleson’s kyphosis had improved from 42% arc to 15% arc.  That’s from all that swimming and climbing!  I tell you, he tries to do everything his sister does and she’s a daredevil herself!  Not sure if that is a good thing or not!  His spine and neck looks great and foresees no spine surgery in his future much to my relief… however, he also was formerly the hip and knew ortho and he prepared us for what Dr. Walker, our next appt. would be telling us, and what I knew but was hoping for a miracle would not be required…

 

The really UGLY
Dr. Walker (appropriately named considering he is a hip and knee specialist) was the appointment I was dreading the most.  Yes, even more so than surgery and neuropsych eval.  After another round of “pictures” (we’re getting good at bribing) we had a “extended consult” with the Dr.  It’s never good when your appointment is labeled, “Extended”.  We spent nearly 2 hrs. Discussing the surgery Coleson will need next summer, Pelvic Osteotomy and Proximal Femoral Osteotomy.  In layman’s terms, Hip Reconstruction, on both hips.   Basically, they will cut his hips and create a wedge and cut off another piece of his hip to insert into these wedges so that it completes the hip sockets.  At the same time, they cut his femurs and reposition the thighbone into the hip socket.  They screw a metal plate into his femurs (which requires another surgery to remove).  Sounds fun, huh?  Oh, then he will need to wear a cast from his thighs to below his knees for at least 4 weeks.  Ideally they will do both sides and both procedures at the same time.  These surgeries are fraught with risk.  They are cutting the very place where bone marrow is made with high risk of blood loss and transfusions required, potential of the bone dying (and therefore not ever growing), spinal injuries (from being sedated & intubated for so long), infections, etc.  They have 2 surgeons working on him at the same time to reduce the surgical time and hook him up to electrodes to monitor his central nervous system.  Did I leave anything out?  Oh, yeah, that outside of the BMT, this is probably going to be the most stressful, worrisome procedure he will need to have.  And we will do this in Minnesota so we will be living away from home for the summer again.    Though I love the RMH, I do not want to live there again necessarily.  It has its pros and cons and it was there when we needed it most, but I think for this procedure, it is better for Coleson and our whole family to be somewhere I can protect him better.

Anyone have a rental house in the Minneapolis area?  J

A Brother from a Different Mother & 2 Year Re-Birthday!

Usually this is said figuratively but in Coleson's case, this takes a quite literal sense, in fact twins!  The highlight of our trip to Minnesota was meeting Coleson's Donor, Jacob and his family.  It is an exceptional experience and one that does not happen that often for various reasons.  We are so very grateful for Jacob and his family who were faced with the decision to help try to save a life of someone he didn't even know.  I'm not even sure what to call such a person.  Somehow adjectives like generous and unselfish do not seem adequate.  We call him our hero!  I'm not sure what gift we could give him in return other than a heartfelt thank you and to meet Coleson in person. 


Can't you see the resemblance?!

During the transplant, you may recall I mentioned a physic reading I once had where the physic said I was going to have twins.  Well I guess I don't have twins but rather a twin, genetically speaking.  Hopefully he inherits Jacob's height as Paul and I didn't give him much to work with in that category!  Also, we now have a new namesake for the J. in Coleson J. David.

 

 

Happy 2 year Re-birthday Coleson (July 24th)!

 





Peyton just can't resist climbing on RM

Finally one without climbing!



I can't believe it's been 2 years since Coleson's BMT.  We just got back from our annual tour of Minneapolis hospitals where Coleson had no less than 16 different "procedures" including consults, x-rays, surgery, labs, etc.  It is an exhausting and overwhelming week where you hope for the best of news and end up walking away with some mixed news.  It was no different for us this year... I will fill you in on all the medical details tomorrow as I want to share the positive aspects of our trip.  I will mention though that it is nice to see "old" friends.  Lizzie and Caleb were not able to make it at the same time this year but we got to see Brayden, Maddie, Tia and Ari.  We didn't get to see Maddie last year so it was nice to get to see her this year.    We celebrated at the RMH house on Bingo night so the house was full of people including 2 Hurler families who both have been there way to long.  One little girl failed transplant - never engrafted and had an emergency second transplant and the other family with fraternal twins, girl & boy, both have Hurler.  The girl is doing well but the boy is starting to reject his donor cells and will need another transplant as well.    Ironically the former little girl also is a fraternal twin but her twin is unaffected. My heart goes out to these two families as they are into the 200 day range already and their journeys are far from over. 

Ari, Tia, Brayden, Coleson

Peyton had a fun week bonding with Grandma.  Aggie flew into Minneapolis while we drove so she could play with Peyton.  Peyton is not one for Hospitals and to try to keep our Minneapolis trips as positive experience as possible for the entire family, Grandma thought it best they play.  And play they did.  We stayed at a hotel with a big indoor park/play center.  If you know Peyton, she is high energy and loves physical play.  She loves to swim, play at the park, gymnastics, dance, etc.  She played all day long at that play center and even most of the night.  Tuesday the hit the rides at Mall of America (Nickelodeon Universe) where she rode no less than 30 rides over an 8 hr. timeframe.  She even got Grandma on a roller coaster.  Paul, Coleson and I met them after our exhausting day (more details tomorrow) and for the first time since BMT, Coleson got to ride the rides too.  And he loved them just as much as Peyton!  Especially the Wonder Pets ride which he rode on 5 times in a row and had this same reaction each and every time! 

 

 

 

 

The next day they took it easier and played in the pool at the hotel and then after Coleson got out of surgery (Yes, another one bites the dust), we went to my Honeywell friend, Janna's for dinner.  One of her sons and his family was there and they have a little girl a year younger than Peyton so she was happy.  Although she and grandma had shrimp for lunch and for the 3rd time, she got sick a few hours afterward.  I am convinced she is allergic to shrimp now.  Thursday was all day at the Waterpark of America with Brayden and Ari and their families with the birthday celebration mentioned above at night.  Friday we met up with my friend Nicole and her daughter for a park play date and then to the RMH where my Minnesota Honeywell family donated, volunteered, cooked and served dinner.  Another awesome dinner served! 

 

We also stopped to see a dear friend, Karee Jo, who is in the hospital enduring another 20 rounds of chemo.  She was here in 2013 where she went through her first 20 rounds and a surgery to remove the tumor and half of her lung.  It returned 9 months into remission and she is back at it again.  This little girl defines the word, "tough" and the only time I saw her break down was when she was told she might not be released in time to make it to the One Direction Concert on Sunday.  Which she did and she got to meet them backstage.  We love you Karee Jo and can't wait until you are back in remission. 

I'll give you all the gory medical details tomorrow.  It's time I put my kids into bed!

 

Graduations and Celebrations

Not sure any actually made it into his mouth!

Well we pulled off the big surprise by showing up unexpectedly to schools and homes last weekend.  My Grandma looked great for any age, much less 100.  It was a short weekend but packed full of fun, so much so that Peyton with that huge heart of hers, did not want to leave her cousins!  The first time that she was in tears leaving.   It was fortunate (and just coincidental) that my niece Katie was "too sick" to go to school on Friday and when she showed up at Grandma's house in the morning to find us eating breakfast her jaw hit the floor.  She wasn't too sick to spend the day playing with Peyton and Coleson though and so we went to Mooville for some ice cream.  Then we showed up at the High School to surprise my oldest sister and my other niece.  Jaw dropping must run in the family because they both had the same reaction.  Then we headed over my nephew's school to surprise my other sister and the boys.  They didn't necessarily have the same reaction (they are boys and play it a bit cooler) but none the less were happy to see Peyton and Coleson.  All my nieces and nephews are so good with my kids.   They spent the evening schooling Paul at basketball and playing on their scooters.  We also surprised my grandma at her place on Friday as Saturday we predicted would be a bit overwhelming. 

Looking Great at 100!

Saturday was spent playing on the farm and Aunt Lorin's house until Party time.  Lots of people showed up Hope Landing to celebrate both 100 birthdays.  I don't think age is a topic they talk about much at Hope Landing so there were quite a few residents who were equally surprised by the special day.  Saturday night was another basketball schooling and family fun.  Sunday was short as we had to leave for the airport at 11:00. 

Grandma Great and her Greats!

And the with the Grands!



Cool in Daddy's Shades!

Did someone say Cake?!

Coleson's last day of his first year of Pre-school was this week.  Since there are kids moving on to Kindergarten next year they had a graduation and all the kids regardless of age/grade got to attend.  I didn't use to be such a softy but I had tears in my eyes as they played the traditional graduation song and he accepted his diploma!  Which he immediately tore.  Peyton's last day of pre-school was last Thursday.  They didn't have graduation but I too felt sad that she was leaving such a great school, Day Spring, and will be heading off to Kindergarten in the public school system next year.  When we went to enroll her last week, she said she was very nervous about going to her new school.  If I have to be honest, so am I!  Looking forward to the 3 day weekend. 

Coleson's story was featured on the ABC 15 website this past week to promote Be The Match Donor Registry.  http://www.abc15.com/news/be-the-match/blood-transplant-was-phoenix-3-year-olds-only-hope .  I also attended a lecture from a Neuropsychologist sponsored by Genzyme, the company that distributes the Drug (Aldurazyme) that Coleson is infused with weekly.  I got to meet another MPS I mother that I have only emailed with until now.  Her son has the adolescent onset form of the disease and an adult women who has the adult onset form of the disease.  It was good to connect with others in the valley (there are so few of us) that are living with disease.    Well, that was our week!  Hope you all enjoy or enjoyed your holiday weekend.

Birthdays, Donor and MPS Awareness Day

Birthdays - Some big ones coming up.  First would have been my Dad's 75th on May 8th.  The year of "firsts" is always hard and this will be no exception.    I made this for Dad when I was in 5th grade.  There were typically school projects for "Mother's Day" and it was convenient that my Dad's Birthday was always the same weekend so I could modify the projects for my Dad's Birthday. (My dad and Aggie didn't marry until I was in 7th grade).  It's hard to read but it say's,
 I Love You Because...

Family Leader
A nice guy
Tough and Always Working
Hoping we will be right
Eager to get the job done
Really interested in what we do

I might not have been a poet at 10 years old but it was pretty accurate.  Still don't believe that he is not here with us to celebrate this milestone birthday....
Speaking of which, my Grandma is turning 100 on May 19th.  We are flying home to surprise her.  It's about the only thing a 100 year old would want on her birthday.  Aggie and my Dad threw her a big party last year just in case she didn't make it to 100.  We never expected it was because my dad wouldn't make it.  My Grandpa's Birthday is on May 9th, though he has been gone for 21 years now.  I think Grandma is really looking forward to the day that they can celebrate their May birthday's together again. And to be honest I hope they are together for it next year.  Not because I don't want my Grandmother here but when you have lost everyone in your immediate family, and you're 100, the days must tick by so slowly. 

On April 1st, almost exactly 2 years after we received Coleson's Diagnosis (April 2nd, 2013), I received his marrow Donor's contact information.  It was the day I had been waiting for since July 24th, 2013.  I was finally able to thank the man who gave my son a new opportunity at life.  His name is Jacob and he's from Wisconsin.  Coincidentally, Coleson's middle name is J.  Just "J."  Originally it was for John (my Dad) and Joyce (Paul's mom) but I can't help but think there was another reason for naming him just "J."  He seems to be a good guy (being a donor take's a special person) with a lovely family full of boys(3). He responded back to me that he felt a special connection as one of his own is Coleson's age.  I hope to thank him in person some day.   



International MPS Awareness Day is on May 15.  Wear purple to spread awareness and help make a difference in the life of a child with MPS or related disease! If anyone is interested in Donating to the National MPS Society, to help find a cure for my son and others with this illness, here is the link:  http://mpssociety.org/

We are making our annual summer trip plans including our week long tour of the Twin Cities Children's Hospitals.  I think we have to go to 4 different ones to see all of his specialists. He may have his heart surgery while we are there and hopefully we will hear that he will not require any other surgeries in the short term. We are also excited to help host the 2nd annual RMH dinner with the help of my Minneapolis based coworkers! On July 24th again as luck would have it!

We held a Kentucky Derby/Fight Night celebration at the Ranch over the weekend.  My dad's truck proved to be one of the more entertaining "toys" to play on.  I think my Dad would be happy that it is bringing so much joy to my husband and my kids! (Which in turn, makes me happy).

 Another bit of bright news in April was that I received a promotion at work.  My new title is Director of Contracts Technical Solutions!  I am so thankful to work for such a great company and a great function and a great team.  I need to finish building out my staff but then hope that to make some significant and exciting achievements.   

No News is Good News

Many of you have called to check up on Coleson (and the rest of us J) recently so I am making time to post.  The saying goes, “No news is Good news” and for now, I would say that is pretty accurate. 

 

We have enjoyed the last few months, being somewhat “normal” enjoying the Arizona winter outdoors and in the pool.  Last weekend we spent most of the weekend in the pool and Coleson, who is fearless around water just like his big sister, is making great progress.  He can swim under water the width of the pool, he can get his head above water briefly and knows to turn and swim to the stairs when he jumps in and yes, he does jump in!    

 

We are staying healthy for the most part despite the measles outbreak and the flu vaccine not totally hitting the mark.  Both Coleson and Peyton had some bad intestinal issues a couple weeks back and I thought not only did Coleson have a  C.Dif flare up but that Peyton got it too, but his labs came back negative so I think we determined it was due to lemons.  AZ Citrus has had some “bugs” this year and I think we were impacted by it.   I also had quite a scare after Coleson got his second round of DTAP and Prevnar vaccines.  That night he couldn’t walk on one of his legs and really had me concerned. Wouldn’t you know that Paul happened to be on business travel that night.  Thankfully a good friend and neighbor, Teresa a Physical Therapist, was home and she evaluated him.  Without knowing he had had a vaccine, she confirmed it was his right leg that he was favoring and not his back or hip (which I was fearful of).  We gave him some Tylenol and by the next morning he was fine.  He has never had a bad reaction to a vaccine before but he was pretty tense by the second shot which was in his right leg.  I know there is a lot of controversy around vaccines and with both kids I was slow to immunize.  Neither had a negative reaction until now. Unfortunately, Coleson has to get them all over again since his BMT.  We have been to allergists, ENTs, Endocrinology, dentists and others over the past few months.  Contemplating putting tubes in his ears again, since both have fallen out now, watching his thyroid level and determining when to get his PDA (his hole in his heart) fixed.  I’m waiting for University of Minnesota to schedule all of his appts to determine if he will need to be sedated there and if so, probably just wait to do the PDA there in July. 

 

Coleson is also starting to potty train.  I’ve decided to do the “3 day Challenge” with him but I never have 3 days to devote to doing it.  Last weekend we gave it a strong effort (in between pool time that is) and he seems to have made progress even going on the potty at school twice.  We didn’t have much opportunity this past weekend so we are targeting Easter weekend to give it another solid try. 

 

I’ve done some travel.  First to Puerto Rico in January to see my team that lives there.  It has been 4 years since I’ve been there and it was very good to do some team building with all my team members (9 in Puerto Rico). I also got to see Pat and Theresa (neighbors from above story) who were there for winter baseball (Pat is a baseball player).   Also, Paul and I took a surprise vacation to Cabo with my sister and brother-in-law in February.  I’m not sure I was ready to “plan” a trip so far away from the kids but this literally just fell into place last minute after Lorin got a call a week before saying they had overbooked her hotel and they were moving her to another hotel.  At first she was skeptical but after talking to some family friends found out it was a 3 bedroom Villa at a 5 start resort.  She called us  and before we knew it, we had nanny coverage for the weekend and our tickets booked.  It was a nice long weekend and this resort was within the same family of resorts we stayed at when I was 6 months pregnant with Peyton (and loved by the way).   Had I had months to think about the trip, I would have found excuses not to go but literally it was too good a deal to pass up. 

 

Grandma has also visited twice.  The first time she drove my dad’s, “Big Red Truck” down here with a friend to give to Paul.  It was the one thing we asked my dad for prior to him passing and he gladly gave us.  Paul’s truck (F250 King Ranch) was stolen from the Honeywell parking lot 6 years ago (again when I was pregnant with Peyton) and the Honda Accord he got in replacement has not had the same feel.  I know my Dad always wanted Paul to have a truck again and to know his truck is at “The Ranch” I think made my dad happy… and it makes Paul very happy too.  He loves to drive the truck and the kids love to ride in the, “Big Red Truck” too.

 

Her latest trip was just last week, the normal time she and Dad would come to AZ.  This time a bit more emotional as Dad was traveling with her via vials.   One to be spread at the Ranch and one to be spread in Sedona.  It’s ironic that my dad was not a traveling man, that is not until later in life when Aggie finally got him to go on adventures with her, that he wanted a little bit of him to be spread at his favorite places including The Ranch, Sedona and Tibey Island.  We just felt a bit rushed to try to do this during this visit though and decided to wait until her next visit over Christmas.  So for now, Dad is now watching over us for a while here.

 

Peyton is doing well, still enjoying gymnastics, swimming, horseback riding and school.  We have now added dance to this mix as well.  Freestyle – which is more suited for her personality.  She is 5 ¼ now as she will tell you and looking forward to going to Kindergarten at Waggoner next year.  She is such a good big sister which Aggie and I got to witness when Coleson went with her to Dance class.  She made sure that Coleson was included in every way.  I think she was more focused on Coleson than herself.  She is so proud of him and I of her.  She often likes to sleep in his room with him (that is when he actually sleeps there himself).  After potty training, these will be the next 2 things we tackle with him. 

 

Paul did well at the Arabian horse show this year, getting 3^rd place in the Novice division overall.  We’ve also been able to spend time with some friends this year (date nights even) as we don’t have to be as protective of Coleson’s health like we were last year.  I’m still experiencing some funny sensations in my heart.. I don’t know if there has ever been a time I have been fully confident that my pacemaker is working as well as I’d like it to.  A close family friend recommended a cardiologist and after a stress test, he thought perhaps my sinus node is starting to give out.  There are 2 “nodes” in the heart and currently my pacemaker is only acting on behave of the AV node (it’s what carries the current from the top heart chambers to the bottom chambers).   I am currently on a 30 day holter and event monitor.  So I am wired up and hoping that it is not my sinus node giving out.  I am fully dependent on the pacemaker now so I don’t know why I am fearful that my sinus node isn’t working, I guess I have a false sense of security with only 1 node being broken.  If for whatever reason my pacemaker gave out though I would be a world of hurt. 

 

I’m looking back at the pictures from last year that make up my 2015 calendar, I had titled March, “Clowning Around” for the picture of the kids with Ronald at the RMH next to the ambulatory building at PCH. We spent almost 1 full day/week there last year at this time getting his infusions and his weekly checkups.  After 3 months going around in circles with the insurance company and his Physical Therapy and Occupational Therapy providers, I finally closed out ~$2600 in unpaid Physical Therapy bills.    I said this last year but I really hope 2015 is less eventful than 2014.   

 

BMT Day Video Post - Link

For some reason, the video link did not appear in the email that came out so I am reposting the URL:

https://www.youtube.com/watch?v=DJcLoBmFp4o 

Sorry, I'm not an expert on Blogging and YouTube!  :)

Coleson's BMT Day Video

It's taken me a long time to put this video together of Coleson's BMT day.  It's 9 minutes long and cannot be played via iPhone, so it needs to be viewed from a computer.  The first half is filled with all of our BMT day supporters (friends, family, colleagues) in their Coleson's Courage T-shirts which was organized by one of my best friends, Emily.  I was surprised to start receiving these pictures the day of his BMT and it helped knowing there were so many people lifting us up on that day.  If there is a missing photo of a Coleson supporter, please let me know and I can easily add it to the video.   The second half of the video is the BMT and post-BMT days.  The actual transplant itself is a blood transfusion so though significant, there isn't much to it.  It's the days of Chemo and other protocol leading up to it, its the dark days afterward where his own cells are no longer reproducing yet his new cells aren't either, it's the days, weeks, months, years after BMT that make his one of the most incredible journeys. 



So much has changed since this day, mostly for the better, but as I play it, I see two angels who were with us that day but are not today.  A reminder that life is precious no matter how old you are, no matter what your battle. 

Surviving Disney World, MPS Conference and the Holidays

Several of you have mentioned my blog recently and it’s been a while since my last post.  I decided to try to take as real of a vacation as I could this Christmas and that included a vacation from social media, technology, etc.  Most of my vacation time is spent in waiting rooms, Dr.s Office, hospitals, etc. and after the past year (past two years really), I needed to focus on my family and myself. 

I never updated the results of the “Be The Match” run/walk.  Thanks to many of you, I was the second place individual fundraiser with $1245 and Coleson’s Crew came in 4^th overall with $2450.  We had a good team of walkers join us during the race including Coleson’s BMT brother Caleb and his family from Illinois.  It was a pretty warm day and Coleson decided he didn’t want to be in the stroller so we carried him the 3.1 miles!  In November my good friends Jenn and Janice along with Sole Sports put on a Ronald McDonald House dinner, (I think that makes 5 RMH dinners in Coleson’s honor in 2014) and Sole Sports wants to be involved in next year’s Be The Match run/walk.  I’m looking forward to their support.      

We had a fairly good November (as I recall it went by so fast).  We started out with the neighborhood garage sale and I finally said good bye to all my infant stuff, a bittersweet event.  Most of it I ended up donating to Maggie's Place, a home for pregnant and new mom's who have no other place to go.  I also donated some of my work clothes as Maggie's place helps them find jobs and they need professional attire.   That same day we had Peyton's 5th Birthday Party @ Pump It Up, a bounce house place that seems to be "The Place" for birthday parties this year.  We've gone to about 6 or 7 in the last 6 months.  I can't believe she's 5 already!  She got to bring "Socko" the class sock monkey home for the weekend and we enjoyed several adventures including quad riding, horse riding, eating out (I posted about that awful day).  We also go to enjoy Thanksgiving with our good friends the Richters.

In December, I did the annual "Hot Chocolate Run/Walk" with some of my good friends.  The run's charity is the Ronald McDonald House so it's another way we are able to "give back" and at the same time have a couple of hours of much needed girl time!  Although we have learned that only $.75 goes of the race fee actually makes it back to the RMH which is a bit disappointing.

Coleson had his 4 top front teeth extracted.  Going through the BMT process and the chemo makes the kiddos teeth weak, thin and prime for cavities.  Through much of the BMT, their gums are swollen such that you can barely see the teeth, much less brush them.  So this Christmas, he really did want his, “two front teeth” plus the next two as well!  Peyton was curious if the tooth fairy was going to come and how much money she was going to leave him.  However, I did not want to take the excitement away from Coleson of losing his first tooth and putting it under the pillow so I told Peyton that I was going to ask the Tooth Fairy to wait until he did lose his first tooth naturally and then we would put all of them under the pillow.  It was funny to watch Coleson explore his gums with his tongue and to watch all the silly faces that resulted from it.  He had a hard time keeping binky in his mouth too so I was hoping we would be able to get rid of it for good.  However, he’s gotten used to the gap now and has found a way to keep binky in, although it does fall out more at night.  He doesn’t look that much different since his teeth were so little and still partially in the gums yet anyway.

He had to have it done at Phoenix Children’s Hospital under sedation and so it was a very nerve wracking procedure for me.  I actually went into the OR room and held him as they masked him which was a bit tough for me.  However, he is starting to remember what is about to happen when the nurse brings out the Purple or blue hospital PJs for him to change into and I hope it comforted him just a little to have me hold him during that scary time.  During the holidays different organizations and groups sponsor and decorate Christmas trees in the hospital.  We were in a different OR than the normal OR and there was only a single sponsored Christmas Tree.  As luck (or fate) would have it, it was the Sole Sports sponsored tree.  It made the process a little easier knowing our friends were “there” with us! 

And then there was Disney World! (And the MPS Conference too.)  We had a great first day at Disney World with the kids.  Peyton got to ride Splash Mtn. and Thunder Mtn.  (but not Space Mtn. since she wasn’t tall enough).  The kids also got to meet Cinderella, Rapunzel and Tinkerbelle (Paul’s favorite) but if you ask Peyton her favorite part was going to Bippity Boppity Boutique where she was made over into a princess.  I don’t know how she sat still through the hair as she doesn’t sit that well for me when I do her hair… She however embraced the part and curtsied thereafter to anyone who spoke to her.  I was feeling like we conquered the first day pretty well and were right on track to make it back to the hotel to check in for the conference on time until she wanted to go on the new 7 Dwarf’s mine ride.  She and Paul left Coleson and I at the Dumbo play structure (a hidden gem we discovered way too late in the day).   1 hr and 15 mins later… needless to say, we were a little late to the dinner and missed getting back out of the hotel for Mickey’s Christmas.

The conference came and went so fast and like Disney world, I think you have to go there once to determine how to get the most out of it in future years.  We got to reconnect in person with some of our MPS friends which being that we are all so far apart was probably the best thing about the conference.  I also enjoyed meeting all the rest of the MPS 1 BMT families that I did not yet know and hearing their stories.  I also enjoyed the talks from the Doctors and Researchers about new trials and advances in the MPS world.  Being an “orphan” disease means MPS does not get a lot of funding and grants and I am sad to hear that much of the research/trial funding is soon to expire.  Donating to the National MPS Conference is one way you can help keep these trials and research alive.   The conference is a very overwhelming and heartbreaking experience at the same time.  Not all of the MPS diseases have treatments, some treatments have just become available for some of them and in general no matter what type of MPS one has, it is a nasty disease.  We are in the lucky group… MPS 1 kids who have BMTs.  (If you can imagine we are lucky).  There are families there who have lost their kids or who are watching their kids regress day by day knowing that one day the regression will stop forever (but not in the positive way).  I too know that Coleson’s time here is shortened than the average person but we have hope that he will make it to his 20s and maybe into his 30s.   Of course we still have hope that it will be much longer than that but we don’t have any evidence that that is possible yet.  So going to an MPS conference comes with mixed emotions. 

Also at the conference the 24 hr stomach flu was going around.  Coleson got it Thursday night but was good to go by noon the next day while Paul got it Saturday morning and couldn’t make it out of bed the entire day.  I took the kids and met my family at Magic Kingdom but by noon I wasn’t feeling so good myself and left Peyton with my family and Coleson and I spent the rest of the day in bed as well.  I didn’t have it as bad as the rest but it made packing for our 5:00am departure time a tremendous task. 

Then we headed to Michigan for Christmas.  The first without my Dad.  And though we carried on just as if he was there, there was obviously a big void.  We made it through without a lot of tears but my sister, Jae, made us memory books of Dad and Aggie had Teddy Bears made out of my Dad’s flannels and overalls for all the grandkids which made holding back the tears pretty difficult.  Included with the bears were pictures of each kid with Grampa wearing the clothing the bear was made from which made the bears extra special. 

 

We made it back to AZ to enjoy a week’s worth of vacation and I feel like I was able to get a lot of things done that I’ve had to put off over the years.  We were blessed with more gifts from “Coleson’s Friends” which is very helpful when we are approaching the new year and a new set of medical deductibles.  We basically hit the Maximum out-of-pocket costs within the month of January so we are very appreciative of all of Coleson’s Friends, especially O&C who have become our extended family and biggest supporters. 

I realize this blog has gotten too long to include the events of January so I will have to pick this back up in another week or so.