T+7 Superstar Coleson

Today the Dr. gave Coleson his Superstar award of the BMT ward.  He really has been doing well; getting out of bed to play everyday and still drinking and eating at least one little thing a day.  Yesterday it was a Cheeto, the day before a gummy bear.  However,  T+7 thru T+21 are also notoriously known to be the hardest days.  The new marrow has not yet engrafted and his own has been depleted.  His white blood cell count is less than 0.1 (normal range is 6.0 - 17), platelets are at 16 (normal is 150 - 450).  Needless to say he is petering out a bit.  He has lots of energy for cuddling but not much more than that however he still managed some play time today.  This is the critical time in the BMT process so we really need your prayers over the next couple weeks. 
My best friends (Emily, Nikki and Jaimee) from home (Michigan) came for a short visit and we pampered Peyton taking her to lunch and her first haircut and mani-pedi.  She wanted a short haircut (but not as short as Coleson's) but I only let them take 2 inches.  Other than that I fear it's the calm before the storm and someday soon we he will start to get more physically sick. 

Bald and Beautiful!

Today we had to shave Coleson's head.  It was his first haircut and he didn't like it at all!  I think he wears it well though!  He's been doing great though a bit more restless the past 2 nights and just seemed a bit more uncomfortable so we upped the Morphine.  We also got a bit of a jolt from our post BMT bliss when his CSA levels came back abnormally high.  CSA is the graft vs. host disease drug cyclosporine.  So they had to redraw labs and try to take a peripheral lab straight from a vein...which is difficult due to his tiny veins and thick skin.  It took about 15  to 20 minutes to due a toe prick and slow drop by drop collection to get 1 ml of blood.  Turns out the lab came back in normal range which means most likely it was a nursing mistake on the original lab draw.  She must have taken it from the line where the meds go through.  I'm so glad I put my son through  20 mins of pain for a nursing mistake!  We had a Skype night tonight with Cathie, the Nelson's, Lauri, Kim, Suzette and kids, and then grandma and grandpa.  Coleson's got all fired up and played hard for an hour!  Who forgot to tell him that he just had a BMT?!

T+3 So good to be on the "+" side of things!

Had some good days since the BMT.  He has slept well and hasn't gotten sick.  He's even gotten up and played a few times and enjoyed some breadsticks and apple juice last night.  We were waiting for him to show us his dinner again last night but he held it down like a champ.  Docs say he is doing well.  It takes about 7 days for the new cells to start growing so they won't even start taking blood cell counts until next Wednesday.  I am so proud of my baby boy. His hair is starting to fall out on his pillow and I know the day will be coming soon that we will have to give him his first hair cut.. I love his hair and hope that it comes back in the same color.  (He could pick up the color of his donor or a a mixture). 

Peyton misses home and all of her friends.  I showed her the BMT day pics of her friends and she got a little sad.  It will be a long 100 days for her.  There are a lot of kids at the RMH but some are only here for short stays, some are in & out of the hospital and some are just not as outgoing and independent as Peyton.  I hope she meets a friend soon that she can look forward to seeing regularly.  We are going to my friend Nicole's house this afternoon to play with her girls and jump in a bounce house that her neighbor is putting up. 

Grandma and Grandpa left late last night.  Things are going to seem a little calmer for them considering they've been with Peyton for the past 4-5 weeks. 

Paul got to play golf yesterday (for work) but it was probably the ugliest day we've had here since arriving.  It was cold and rained off and on all day.  Today has returned to it's sunny self, a bit colder but sunny. 

The support from all of you has been fantastic.  I wish I could bottle up all the prayers and love that all of you are sending and have him drink it down.  He would be the healthiest little boy alive.  Don't be afraid to call us.  If we can't take your call, we'll listen to your voice mail and it will brighten our day.  We're also setting up a few Skype (& facetime) dates with friends. It's a good way to get to see Coleson!

My phone number is 480-321-9473 and Paul's is 602-284-9271 Skype:  PaulandCaraDavid

T=0 and T+1 Our little boy becomes a new man!

At 1:42am (11:42pm Phoenix Time) our little boy became a new man..quite literally (the donor was a 39yr. old male but that is all we know about him).  I'm not sure which "birth" I cried more at and I didn't even take any pain meds for his first birth.   It was a long day of waiting as we were originally told it would be at 1:00pm but by yesterday morning it had changed to 4:00 - 5:00pm.  Then it changed to 7:00 - 8:00pm.  We were getting nervous when they couldn't even verify that the marrow had arrived in Minneapolis.  Due to donor confidentiality the process of donating, transporting and transfusing blood marrow is topic secret.  I don't even think the Government knows the details of these logistics!  So we waited, and waited and waited some more.  Fortunately by the afternoon he was playful and happy again.  Finally at 1:00am they said that they had the blood at the hospital.  It was a very high concentration of blood cells and so they actually were able to store 2 more bags of it that Coleson didn't need and hopefully will not need in the future.  There is a chance they may have to transfuse more if it doesn't engraft but we are praying this will not be the case.  That is the one benefit of marrow vs. cord blood that you can transfuse more if needed.  The transfusion was over by about 2:30am.  He had a rough rest of the night; running a fever, vomiting, bad diapers, and overall just seemed in pain.  They are starting morphine today.  Thank you to all our Coleson Courage Supporters who sent us pictures in your BMT Day T-shirts.  Thank goodness the marrow arrived when it did or those t-shirts would have been outdated!  (We are using Arizona Time). We finally got to hang the medal of honor (The BMT sign) on our door. 

Grandma and Coleson waiting for blood marrow

T-1 and The ABCs of MPS

Tomorrow is the big day @ 1:00pm...  Unlike a surgery, it's a pretty uneventful blood transfusion.  We could use uneventful as the chemo is finally kicking in and he is getting sick multiple times a day. It takes about 7 - 14 days for his body to accept and start reproducing the donor cells (engraftment).  These will be are hardest days so we will need your prayers over the next 2 weeks more than ever. 
He is less active but did rally when he saw his sister for the first time in 3 weeks.  Peyton just brightens the days.  She may be his best medicine!

We have also acquired a new language.. here are our new ABCs: My spelling may not be accurate!

A is for Aldurazyme (The Enzyme Drug)
B is for Bone Marrow & Blood
C is for Chemo (Campath, Cytoxin and Busulfan) & Cyclosprine (Anti-rejection med)
D is for Donor (who we are forever grateful for) and Dressing Changes & DNA (His will change)
E is for Enzyme (what he lacked naturally) & Engraftement
F is for Fluconazole (Antifungal drug) & Flushing (g-tube and Hickman)
G is for Genetics & G-Tube & Graft vs. Host Disease
H is for Hickman line and Hurlers (MPS I) and Hemoglobin
I is for Intrathecal (Spinal injection) & IVs
J is for Juice (specifically apple which Coleson has grown fond of)
K is for Keppra (anti seizure med)
L is for Lysosomal Diseases (storage diseases ~50 rare inherited metabolic disorders)
M is for mucopolysaccarides (MPS) & MRIs
N is for Neuropsych Evaluation (Developmental Specialist)
O is for Orphan Disease (like MPS those that are so rare they do not get a lot of funding) and O+ (His new blood type)
P is for Protonix (Acid reducing med) & Platelets & Paul Orchard (Sr. Dr. of BMTs for MPS)
Q is for quiet (the hallways of the BMT ward since patients are confined to their rooms)
R is for Red Blood Cells & Rare disease
S is for surgeries (I've already lost count and there will be more in the future)
T is for TPN (Feeding through IVs) & Transplant
U is for Ursodiol (Liver protectant drug) & University of Minnesota - Amplatz Childrens  Hospital
V is for Veno-occlusive disease of the liver (Possible complication of Chemo)
W is for White Blood Cells & Wes Miller (our BMT Dr.)
X is for X-rays
Y is for "Y?"
Z if for Zofran (Antibiotic)

T-3 Starting to slow down...just a little

Doses of chemo to date = 17 
Doses of chemo left = 6
BMT in 3 days

Coleson is starting to slow down, just a bit.  He's gotten sick the last 2 days but seems to bounce back afterward and it is usually predicated on something in his stomach, either food or meds.  So he is being a cuddlebug today (which I don't mind) but still smiling and playing as well.  We know the worst is yet to come and it typically is 7 - 14 days after BMT when the new marrow hasn't engrafted yet and he doesn't have his own anymore.  We are kind of anxiously awaiting this day just for it to be behind us.  I am glad though that Peyton will get to see her brother feeling and looking good before she sees him at his worst.  My parents are bringing her "home" tomorrow.  Last night Rachel stayed the night with Coleson and Paul and I stumbled upon fireworks marking the end of the Minneapolis Aquatennial.  We were in the hospital for July 4th so it was nice to get to see them this year, though it is really hard to celebrate much these days.    I skyped with Janice (AZ friend) today which was a nice break from my day.  If anyone else would like to Skype us, we are at PaulandCaraDavid. 

T-7 Coleson 3, Chemo 0

Coleson has had 3 doses of the chemo Cytoxin and so far is doing well.  He is still energetic during the days and seems to be feeling good with the exception of 2 small vomiting episodes.  He is sleeping well at night as well. He is starting to slow down on eating and drinking and I'm sure we will be switching to g-tube feeds within a few days.  I think this is the "honeymoon" period of chemo and it will take a few days for the side affects to kick in.  It is a gradual build up which is welcomed after the immediate in your face side effects of the Campath.  Tomorrow is his last day of Cytoxin and then we switch to 4x daily doses of Busalfen.  These are 2 hr transfusions @ 4am/pm and 10am/pm. 
Rachel arrived last night and is going to stay with Coleson this evening so Paul and I can go for a run.  Speaking of which I need to send a big Thank You to Sole Sports or the wonderful care package they sent full of toys, games, puzzles, crafts, etc.  and some running maps/shirts for us.  This was an amazing surprise.  I'm sure Claudia had something to do with this as well so I thank her as well.  We continue to get support from colleague and friends via emails, cards, etc. and each one is very appreciated.  I am finding little time to update my blog much less send personal thank yous so I hope you all know that we do appreciate each piece of support no matter what form it takes.  I am really focused on keeping Coleson's development going while he has the energy to do so when he is awake it is mostly play time, singing and dancing and reading.
Today was Peyton's last day at Aunt Ems where she had so much fun.  Less than 1 week until Grandma & Grandpa bring her back... and I can't wait, although I know it will be hard for her to see Coleson in the hospital.  1 week from today is his transplant day.  The procedure itself is pretty uneventful but the significance unquantifiable.

T-10 Checked in to our new pad!

Today we checked into our new place - Room 4138.  We decked it out with his Coleson banner and his UmiZoomi stickers to make it more "homey".  I also have my "good day" button and my "30 Days of Inspirations" cards sitting next to my couch/bed.  Thanks to everyone who has been so thoughtful to give these items to us.
Over the past 4 days we've been to neuropsych to determine that he is still testing at the average level for his age for the most part. He has progressed in his gross motor skills but is still testing a little low for the responsive communication (things like "go get the ball" or "please bring me the ball").  At his ophthalmology appt. we learned he is nearsighted and astigmatisms with some cornea clouding and retina damage so he will need glasses.  I really wasn't prepared to hear that since he "seems" to see fine.  But this disease hides out in every inch of the body so I guess I shouldn't be too surprised.  I'm thankful his heart and lungs are so strong.
Friday he had surgery again to replace his central line.  Wayne and Jim (colleagues from HW) stopped by to spend time with us while he was in surgery and we learned a lot about the U of M campus and Minneapolis in general. 
Saturday he woke up full of energy and also missing one of his line caps!  It must not have been tightened enough coming out of surgery (as there is no way he can get it off himself).  We were fortunate the line was clamped (and those clamps don't always stay shut) as he could have bled out and I don't know if I would have woken up in the middle of the night to realize it.  Lesson learned...double check the Dr.s and Nurses work!
Janna (another colleague) and her husband Steve came over and brought us some dinners and a "Cars" book for Coleson and stayed for lunch put on by one of the many organizations that donate their time to feed us.  Watch out Honeyweller's, Janna got inspired while she was at the RMH!
Then we got an unexpected (but welcomed) visit from Cary (another colleague) Saturday afternoon.  We spoke about the "Minneapolis" stats like the 2nd most congested city and the 2nd most "fit" city.  I failed to tell him I also learned it was also rated the 4th most "Snobbiest" city behind Boston, San Fran and Seattle.  I'm surprised by this last stat as we've only met friendly people here.  Although it is mostly Dr.s and Nurses!  We so appreciated our visits over the weekend, it definitely helps to know we have friends nearby.
After Cary left we toured the surrounding area and on a recommendation from Janna & Steve, we drove through Como Park.  What an amazing park! It has an amusement park, zoo, lake, pool, etc.   We didn't get out as there were a lot of people there and with Coleson's compromised immune system, we didn't want to risk it.  We definitely will take Peyton there when she gets here. 
Today he was full of energy again, unfortunately we spent much of the day in the hospital.  They just started his antibiotics, antifungal meds and will start his "IV Flush" at 10:00pm.  We start Chemo #2 (Cytoxin) tomorrow which can form crystals in the bladder so they have to pump him full of fluids over the next 5 days and we have to change his diaper every 2 hrs. to ensure he is peeing it out. 
Peyton has been having so much fun at Aunt Em's house.  She's gone to the pool, Lake Michigan, Aunt Nik's house, McDonald's etc.  She is having fun sleeping over and from what I hear Lincoln's bunk beds are full of all the kids.. and Aunt Em.  I'm afraid she will disappointed to come her next week.  Although she does get "Sleepy" when we Skype with her which I think is code word for "I miss you and I'm sad that Coleson's owies aren't all gone yet."

T-14 Another day in the life

After enjoying our Sunday back at RMH, we had his inthrathecal injection and ABR sedated hearing test on Monday.  We got some bad news in that he has mild hearing loss in both ears and needs hearing aids.  He can't hear high pitched sounds which include the isolated letters/sounds of S, Th, Sh, Ch and F.   Kids some times have a heard time understanding plurals if they can't hear the S sound.  As we suspected, due to his narrow airway, they had problems intubating him and I know his throat must hurt afterward.  We had a longer than expected recovery (4 hrs.) wait due to having his first round of chemo and having a suppressed immune system.  Tuesday we actually had a free day from the hospital which we needed.  Today was his ERT day and we started off with a clogged line.  After x- rays and a 2 hr. delay they determined that the line was still in place so they ran an anti-clogging med through his line and it cleared it.  However late in the day the radiologist looked at the line and since the "cuff" that was supposed to be under the skin had been exposed (pulled out) the line has to be replaced before we do chemo again.  So another surgery and a another sedation scheduled for Friday.  I'm not sure which is worse.
I met another Hurler family tonight at RMH.  The mother had emailed me a while back and her son was diagnosed/ had his BMT at the same age as Coleson.  He is 7 years out and looks great but has undergone numerous surgeries and is back for another hip replacement surgery.  As I've said, this BMT is only the beginning....  It is always so nice to meet another family and Hurler kid.  It truly gives me hope, which I needed today.
Before I forget, we had several packages delivered to us today at the hospital and I want to thank all of you for the care packages, cards, gifts, Banners, "support", :) etc.  We have the best of friends and are very thankful.   Tomorrow is Neuropsych so no needles, blood, tubes, sedation, or enzymes!  Just "play", to see where he stands developmentally.
  Also, we've met some wonderful families and kids here, all with there own story/battle.  We also have a colleague/friend that has been battering his own cancer journey for the last 18 months.  All of which could use your thought, prayers tonight.

T-17 An Extra Night's stay but Temporarily Going Home Today

 Well we bought ourselves an extra night's stay at the hospital after Coleson spiked a fever (100.7) after his last round of chemo 2 nights ago.  It was in the morning and we have to wait 24hrs. after a fever to get released.  He was feeling back to "normal" yesterday and was a ball full of energy running around his hospital room.  He later took a 3 hr. in the early evening and thus stayed up to 1:00am.  He thankfully though slept until 9:00am so I was able to catch up on some much needed sleep. His blood pressure is running high which it typically does in Hurler's kids but it came down after a dose of BP meds.  Oh, and we discovered during his line dressing change that he slightly pulled out his line exposing the protective cuff that is supposed to prevent the line from coming out. 
Todd - I thought of you and Ethan, although after an x-ray it is still in position thankfully. 

Tomorrow he has to be sedated for his intrathecal (spinal) injection of the Enzyme and an ABR and hearing aid fitting.  It will be a tough day of sedation so please send your love our way!

Peyton is having a lot of fun in Michigan.  She is playing with cousins and friends, swimming, washing pigs (my nieces and nephew show them at the county fair this week), jumping in the hay bails, and attending a George family Reunion yesterday.  I heard she hung out with cousin Rob, caught 2 fish, played with Caden explaining they were cousins and wanted to jump off the high dive (I think it's like 20 ft up?)  Again, Tenacious P!  She misses her brother and wants to know if his "owies" are gone.  I hate to keep telling her "not yet". 

Here are our addresses, please feel free to send us cards, letters, etc.  Peyton loves to get mail!

University of Minnesota Amplatz Children's Hospital
2450 Riverside Ave.
BMT Ward 4 - Coleson David
Minneapolis, MN 55454

Ronald McDonald House
(Our Name) RM 441
621 Oak St. SEMinneapolis, MN 55414

T-20 Happy and Peaceful 4th of July!

We can see the fireworks from our 4th floor room with a view (the windows are full length).  Thankfully Coleson is having a peaceful and restful second dose of Campath after having his ERT today.  He didn't eat much today and was overall lethargic but I'm hoping it means he will sleep better tonight.  He usually does after ERT but then again, he usually doesn't have a chaser of chemo! 
We made it into the RMH today and met another Hurler couple who we have been following.  There is also another Hurler child still in his post 30 day stay who I hope we will also get to meet.  I hope you all enjoyed your 4th.  I look forward to next year's when we can watch fireworks as a family hopefully over Lake Michigan. 

The Countdown Begins! T-21

Wow, That Sucked!  I wish I had more positive things to post about our first night of chemo but there really isn't any except that it is over.. and this was supposed to be the easier one.  We got admitted at 10:30am and didn't start the chemo until after 9:00pm.  I thought perhaps this wouldn't be so bad as maybe he would sleep through most of it but boy was I wrong.  I'm about to get graphic so if you have a weak stomach you may not want to read further.  However this blog serves as my historical record and also for future Hurler BMT parents and so I need to put it all out there. 
As I was saying, we started the Campath soon after 9:00 and within 15 minutes he had awoken from his sleep so I put him on my lap.  Within maybe 10 more minutes, just as the nurse left, he started vomiting. So I called her in and she cleaned him up and I cleaned myself up.  We left him in his bed as he had fallen back asleep.  He again was restless after another 10 or so minutes and I tried to soothe him but again he vomited.  After another round of clean up he wasn't fallen back asleep so I held him in the chair again.  The nurse asked if I wanted any anti-nausea medication and I said lets just give it a few minutes and see if he falls back asleep.  She left and almost immediatly he acted like he was going to get sick again but he didn't have anything left in him. So we gave him the anti-nausea and he finally was able to fall asleep.  Near the end of the 2 hr infusion, just when I thought we were in the clear, my lap became very wet.  So we had stopped it from coming out the top but it now moved to the bottom.  Again a ring to the nurse and I had to break into the "Comfy" clothes that my college girlfriends had sent all of us and were waiting in our room when we arrived.  Then the shakes started.. and the fever.  I really wasn't prepared for the shakes which lasted over an hour.  This was probably the hardest part as they wouldn't stop no matter how much I tried to hold him close and I know that how tight shaking makes your body.  At about 1:00am those finally stopped and again we took position in the recliner.  At about 2:30am I put him back in his bed and got some sleep myself, about 3 hrs worth.   During that time they did give him more Tylenol and an Antibiotic which the Drs. said would most likely be needed since kiddos almost always have a fever.
Today we have ERT on top of the Campath and a sleep study tonight.  It will be an interesting mix.
In other news - Paul wasn't feeling well yesterday so he went back to the hotel at around 2:00 so he missed the whole thing.  Which is good since he doesn't do well with the gorey stuff and I will need a reprive today to do the laundry and make get a good nap in.  We also got a call about noon yesterday saying we got into the RMH.  What timing!  So Paul is moving our stuff there this morning.  Fortunately we have a bit less to move and more room in the car since Peyton is in Michigan and we are here. 

By the way T=0 is the actual transplant day (July 24th) and we count down so as of yesterday we had 21 days to BMT day and then we start count up afterward.

The Week in Review: Unplugged and Busted!

Sorry I haven’t sent an update in the past week.  I just needed a break from my so called blogworthy life and unplug from the digital matrix inclusive of work, emails, phone calls, facebook, my blog, etc. and just enjoy some time with my family to be frank, just in case… 

My vacation time has been spent at hospitals this year and this past week was the last time that we were able to spend some time together prior to Coleson’s admittance to the hospital.  Amongst, X-rays, EKGs, Echos, consults and the rest, we sprinkled in a trip to the mall of America, some pool time, some walks around a nearby lake, and some play time at a park nicknamed “Chutes and Ladders” for the numerous rope walls, slides, tunnels, etc..  The weather has been beautiful our entire trip; we were fortunate to leave AZ just before the record breaking heat.  Our drive here wasn’t as bad as one would think.  We ended our road trip with a stop in Clear Lake, Iowa and were fortunate to stumble upon a lake and park which reminded me of Grand Haven for you Michiganders.  We stopped for 2 hrs to enjoy the sun and the beach and some live music in the park.   We have not gotten into the RMH yet and looks like there is a bit of a wait list so we are staying at a hotel about 10 miles from the hospital.  We did stop there one night for dinner to meet up with another Hurler family that was in town for a 2-year checkup and to discuss participation in a new trial for post-BMT ERT.  It was really beneficial to meet another family and it gave us hope and inspiration. I have been communicating with the father since receiving the diagnosis and he has been very helpful. 

We got the green light to proceed with the BMT and we signed the consent forms but not before we were told no less than 3 times of the risks, possible complications, side effects, and review all the drugs: Immunosuppressant, chemo, anti-nausea, antibiotics, antihistamines, antiseizure and pain reliever (do you think he might need this last one!).  I’m sure I’m forgetting some.  The doctors said something that really resonated with me about BMTs for Hurlers kids.  It’s different than other BMTs as Hurler kids appear “Healthy” and you purposefully make them sick in order to make them better, whereas most BMTs the kids are already sick.  Not that either situation is easy but it is difficult to think we are purposefully giving a “healthy” child chemo. Don’t get me wrong, we don’t really have a choice it’s just hard to think that in just 2 days he will go from being a vibrant little boy to a very sick one.

Lastly I turned a year older yesterday.  It wasn’t the best birthday I’ve had but we did have lunch at my close grad school friend, Nicole’s house and Peyton got to play with her 2 girls.  She even got me a birthday cookie.  Then we had to drop Peyton and Grandma off at the airport and I had to say goodbye to my little girl for 2-3 weeks until they return before BMT day.  I missed her instantly and don’t know how I will make it for that long of time with her.  Not to mention having Grandma around was nice too.  She takes good care of us. 

Lastly if you are still reading this we has some excitement at the hotel last night.  We had some noisy neighbors right below us for several nights and after getting a glance at them and their decked out hummer with gold bling we were convinced they were dealers… and not the blackjack kind!  Well, after I awoke at 1:00am to the sounds of yelling and up and down the stairwell right beside us I hear a police bullhorn say “Put your hands up and get out of the car”.  We witnessed the arrest of 6 people and saw the cops pull some baggies (white not green) and guns out of the hummer.  We are not in a bad part of town or a bad hotel but I guess they have the money to stay in nice places!  Needless to say, I hope next year’s birthday is a lot better.  In fact, I could use a much better year!