Answers Come in Many Different Ways

We went to Minneapolis seeking answers and at first I felt like we weren't getting any, but by the end of the trip, I realized we really had after all.

First let me not leaving you all hanging in regards to Coleson's surgeries.  His ear tube surgery went well, though we were delayed an extra hour in the pre-op room which to an already "nervous" patient just adds to the anxiety.  He didn't want to put on the gown but was very good all the way to the operating room.  He was only going under the gas mask as far as sedation so there were no pre-meds.  I held on to him as they put on the mask which he immediately began to fight.  Despite the Dr.s asking what "flavor" the patient wants the mask to be, nothing is worse than the smell of that gas!  I got quite a bit myself as I was trying to hold on to him.  And worse, the smell sticks to you like glue so I got wiffs for the rest of the day.  Coleson woke up extremely sad and mad and almost on cue started pulling out his IV line and EKG stickers.  He said how scared he was of the mask which I couldn't blame him for and I just dreaded having to do it all again the next day.

As per his usual "MO", he recovered quickly and we were off to Crayola Experience for a few hours of making colors come to life.  I almost thought we weren't going to fit this important activity in with the added surgeries but this would have made him more mad than the surgeries themselves!

Though I would have rather been able to "plan" for the leg surgery, I guess the one good thing about it being more of an emergency was that I didn't have much time to dread it.  It also helped make some of our decisions in regards to "where" and "when" we were going to do it and if we were going to try to get his port out at the same time.  After using the sterile soap to wash two nights in a row and getting very little sleep, 5:00am came and we were off to the hospital again.  The one thing I love about these hospitals is that they understand Hurler kids and how much they go through and they do everything they can to try to make it as easy as possible on the kids.  For instance, I knew Coleson was not going to put on the gown and allow us to wipe him down with the sterile wipes.  I knew if he saw a mask that he would get superman strength and fly off the bed so fast.... so they didn't make him do any of that.  Instead we hooked his line up to his port, gave him some meds to put him to sleep and then they took him back.  I don't think he even knew what was about to happen.  He had 1 1/2 hr MRI first and expected about another 1 1/2 - 2 hrs to remove the plates.  Right on schedule, he was done about 11:00.  Dr. Walker said he did really well, no complications and there did not appear to be any infection but that it would take 48-72 hrs for the cultures to grow to know for sure.  So we got settled into our room.  Coleson was in a lot of pain but didn't seem to complain about the surgery.  It was almost as if he knew that the Dr. was fixing his legs again and that he knew he would be able to walk again soon.  I feel like he grew up a little during this trip... his birthday was October 4th (7 years old, can you believe it?) and he just seemed "older".

Paul and I were contemplating different scenarios of how we were going to get back home if Coleson ended up needing to stay as the Dr. wanted us to.  Paul had a work trip he needed to fly out on Monday morning and I wouldn't be able to keep Peyton with me at the hospital, but the thought of trying to fly home with Coleson was intimidating as well.  I just hoped that Dr. Walker would release us on Saturday, which he reluctantly did after I pleaded my case and his cultures showed no sign of infection yet.  I was so ready to come home for many reasons, including the weather.  I was really looking forward to a mid-west fall but what we got seemed like winter instead. It rained almost every day we were there, hovering between the 30s and 40s and I did not see the sun once!   We got home late Saturday night only to find ourselves locked out of our house.  Apparently we had another big rain storm on Saturday knocking out our electricity and wiping out our garage entry.  I won't tell you how we finally broke in but it was dark, cold and the kids were scared on top of the pain Coleson was in.

Coleson wasn't able to walk at all on Sunday but by Monday he was starting to stand and as the day progressed he took a few and then even more steps.  By Tuesday he was getting around by himself fairly well.  He went to school on Wednesday and even though the plan was to pick him up after a couple of hours, he lasted the whole day.  They get out 2 hrs earlier on Wednesday anyway.  By today he was ready to get back to his usual Friday routine of going to soccer after school, but his legs still need 6 weeks to heal so we will be cutting out soccer and gymnastics until January. 

As mentioned in the beginning of this blog, we went to Minneapolis seeking answers to some of our questions and I felt like we had not really received any answers in regards to continuing his weekly infusions, and his surgeries but I guess we did after all.  Part of our consideration was whether we were going to stop ERT and try to get his port out with his leg surgery (so we could reduce the number of surgeries) but with that no longer being an option, I feel like there is no rush now to get his port out.  And if we aren't getting out, we should continue to use it, which means we will continue ERT.  We spent a lot of time talking about Coleson's GAG levels.  GAG is the gunk that builds up in the body due to the missing enzyme which impacts the skeletal and organ development.  Coleson's GAG levels are fairly low, even within normal range for someone without Hurlers.  What I didn't understand and learned this trip is that this is the GAG level that is in his blood cells and with him having a BMT, you would expect the cells would have a normal GAG level.  What it isn't measuring is the amount of GAG in his joints, organs, etc. which could only be achieved through biopsies.  With this new realization that we really don't know how much GAG is still in his body and that ERT only helps try to get rid of this, we will continue ERT until new information suggest otherwise or Coleson starts to put up a fight about it.

Expect the Unexpected

You would think we would have learned this lesson well enough by now (to expect the unexpected) as it seems like every time we come for checkups, something unexpected pops up.  This trip it was 2 unexpected surgeries that popped into our schedule just during the first day of appointments.  We started with the typical lab work and BMT clinical visit.  No surprises here, though I was hoping for more guidance on whether we should continue weekly ERT, there is just not enough data out there to lead us in either direction.  So we are left to contemplate this important decision on our own.  This was the only expected part of our day. 

We then went had neurology.  I guess if I were keeping better records it would be 3 unexpected add-ons to our schedule.  Neurology added a full spine MRI to his existing brain and cervical spine MRI just to rule out any neuro related issues affecting his toe-walking and wetting problems.  I really think the toe-walking is related to his hip/femur surgery but I guess having a full MRI does have its benefits.  I didn't count this as an additional item since he was already scheduled for an MRI already. 

We then headed over to Gillette's to see Dr. Walker, his ortho surgeon.  He is healing very well from his hip/femur surgery, however, it appears the bracket on the left leg is starting to come through the other side of the bone and it appears cloudy around the top part of the bracket that sticks into the bone which may indicate an infection. The screws also look like they may be coming loose so long story short, we decided it best to remove the brackets as soon as possible which happens to be this Friday. Normally, this would't be that big of a deal as we knew we had to get the brackets out eventually but  there is an "unknown" to this equation in that we don't know for sure if there is an infection and if there is, what kind it is and whether or not we will have to be admitted to the hospital for overnight or even longer.  Normally the bracket removal can be outpatient but he won't know until he sees the bracket/bone on whether he wants to start him on IV antibiotics or not.  You would think if there was an infection that he would show other symptoms such as a fever but then again this is Coleson and he is a good "hider".  He rarely shows clinical symptoms.  So we asked for his MRI to be moved to this hospital so we could avoid back to back sedations or so we thought.

Then we went to Audiology and ENT.  We actually got mixed results during his hearing test.  His left ear has improved from last year, while his right ear has worsened. Traditionally his left ear was worse than his right but the right tube is still in but seems to be plugged which is affecting his hearing.... so when we saw ENT right after, he suggested we put in new tubes in both ears.  So we scheduled this for Thursday.  Unfortunately these are 2 different hospitals so we can't do this all in 1 shot.  But tubes are fairly quick and are a light sedation so I guess that gives me a little relief.  Coleson is going to hate having these back to back but I am hoping praying that this is it for him....

Today we got confirmation of his ADHD.  This is pretty typical in all MPS patients no matter what type so they were going to provide us with better tools to implement into his IEP at school to hopefully help while we contemplate medicating.  The only other thing we had on the schedule was his bone density and DEXA scan which requires him to lay still for 4 minutes.  We haven't been successful doing this in prior years. 

Tomorrow is Endocrine and ophthalmology.  We hope to get more clarity around his thyroid medication with Endocrine and don't know what to expect from ophthalmology.   Will try to update as we learn more. 

It's All Relative...

Wow – where do I even begin?!  Maybe with an apology for leaving everyone hanging for the past year….  It wasn’t my intention but for several reasons, I just never found time to update my blog.   It was a very busy year but also somewhat “normal” relatively speaking.   I will try to briefly summarize it below:

We survived the remaining two weeks of Coleson’s braces with relative ease.  I stress the word “relative” as having a 5 year old in braces during the summer isn’t easy at all.  I do have to gloat for a minute about my son (as if I don’t always)… he did not complain AT ALL during the 5 weeks he was in the braces.  Outside of the initial pain of surgery and the catheter, once we made it back home he was a trooper.  However, I don’t think we prepared him well enough for the first few days once we got the braces off.  We kept telling him that he would be able to walk and run again, but we failed to tell him that it would not immediately after getting the braces off.  Needless to say he was quite confused and scared when we took the braces off and he couldn’t walk and we handed him this contraption (walker) to use.  It didn’t take him long though (probably 2 weeks) before he was up and walking around and by the time school (Kindergarten) started (August 3^rd) he was walker free.

Fast forward 1 year and he is now a 1^st grader.  Completely blew over his Kindergarten year.   We still have some residual side effects of the surgery.  His feet are a little turned out (apparently, due to his abnormal bone growth, his tibia is rotated out on his left leg).  His left leg is also ½ in shorter than his right leg and so he has to have a shoe lift.  Unfortunately, it isn’t one that goes in the shoe therefore we have to have it mounted on the bottom of each shoe, costing $170 a shoe!  Fortunately, he is a boy and doesn’t have a big shoe wardrobe.  None the less, it is a pain to deal with.  And he has developed into a toe walker.  The ortho surgeon failed to communicate that we should watch out and try to prevent this from happening.  The feet are definitely an underappreciated body parts.  So we have added daily stretching to our routine.  Otherwise medically speaking we are contemplating stopping the weekly Enzyme Replacement Therapy and also considering timing of getting the hardware out of his femurs as well as contemplating removing his port.   I am also starting to suspect he has ADD or ADHD based on his short attention span and lack of focus.  He is a really good kid but struggles a bit with following directions the first time and staying focused on task.  I used to chalk this up to being a 6 year old boy but after talking to some friends, I am starting to question whether or not there is something else at play.    We skipped going to Minneapolis this summer as I thought we could all use a break but I have come to the conclusion that we have to go.   I have too many questions that just can’t wait.  I have to say Coleson was pretty upset when he learned we were not going to Minneapolis this summer, which says a lot that he remembers more of the fun we have than the Doctors appts and hospital visits.  He wants to go to Crayola Experience and Mall of America.  So we will be jetting off on his birthday and as an extra fun surprise we are going to stay at the Great Wolf Lodge the first weekend.  We will move into the RMH for the work week so that we can navigate all of the medical appointments.   He did have a significant anniversary this summer, his 5 year transplantiversary.  I can’t believe it has been over 5 years already but am grateful for every year!  He is in gymnastics and hip hop and started boy scouts this year.  He is taking after school soccer and golf (which Paul is happy about). Not sure how long he will be able to do any of these sports so am encouraging him to do all he can while he can. 

Peyton continues to be a great big sister and just a great person overall.  I admire her big heart and though I do get nervous that it can easily be broken, I appreciate how thoughtful and caring she is.  She is almost 9 already, being only 3 when Coleson had his transplant and only 5 when my dad passed.  Both events having a significant imprint on her.  Thankfully she is a good student and is very respectful of rules and teachers.  I rely on her to counterbalance the worry I have with Coleson.  I don’t take her for granted though as I know she still needs my attention and quality time.  She picked up cheerleading last year which is a perfect sport for her due to her nature of supporting and encouraging others.  She is the “tumbler” on the squad being able to do both back and front handsprings.  She is still in gymnastics and we added hip hop as well as Girl Scouts to the mix.  Are you starting to understand why I am so busy?  She had her first “major” (well to her it was Major but again, it’s all relative) surgery the first day of summer after falling on the slide in our pool and cutting open her chin.  She has severe anxiety when it comes to pain but after numbing the area and 9 stiches later, she was showing off her injury and recounting how brave she was. 

We suffered another death in the family this past November (just before Peyton’s birthday) with my Grandma passing away at 102 ½  years of age.  After 100, the ½ years become relevant again!  She had a stroke and briefly regained consciousness but died within 2 days.  My sisters and I spoke at the funeral remembering all of her great qualities and all of her “hidden” tissues which she pulled out at the first sign of a sniffle, a tear or a dirty face!   When we cleaned out her apartment we discovered lots of these tissues hidden in sleeves, pants pockets, etc.  Enough to fill a trash bag. 

Part of the reason why we have been so busy is that we ended up buying a new house (in Tempe) while we were in Minneapolis… sight unseen.  We had been considering moving to a bigger home in our neighborhood (really just one with a bigger yard) and the prefect one came up for sale about a week after we left for Minneapolis.  Thanks to great neighbors and friends and FaceTime (not to mention timing), we cut our Minneapolis stay 2 weeks short and signed a week later.  Thankfully Rachel had been in between jobs and helped pack up our house and the Ranch.  Yes, with this purchase, Paul had made the dissection to sell Rancho Costa Lotta.  I know it wasn’t an easy decision but he swears he doesn’t regret it.  It is an end of an era but we are making our new home just as unique and special to our whole family.  It has a big back yard with an awesome pool (with a big slide and waterfall) and we have had more get togethers here already than we had at the old house, including a surprise birthday party for Paul!

Paul got a new job (at Honeywell) in July.  He is getting back into Sales where he spent most of his career.  I think this is more his calling and although he is selling a different product line to different end users, you can see he is enjoying it.  Believe it or not, I am celebrating my 20^th anniversary at Honeywell.  My job has undergone some recent changes as well and I am finding more balance in my life as a result.  We also celebrated our 10^th wedding anniversary this past August.  I used to tease Paul before we were married how his life changed once he met me, but wow, the past 10 have been eventful! I really hope the next 10 are a little more relaxing.  I’m sure Paul will be retiring in that time so it should be for him at least. 

I will post again once we get through our week in Minneapolis.  Oh, and I will try to find time to update pictures.  Wish us luck (on both)!

3 Down, 2 to Go!

Weeks until braces off that is... 


At least according to Leo the Ninja Turtle that is.  Leo is our official holder of our countdown chain (an idea from a good friend and Hurler dad, Todd.  Leo was a gift from his friend Eli.  It is a cardboard cutout and stands 6 ft. tall.  He not only makes a good chain holder but also can be used as an anti burglary device as well!  (Think "Home Alone" style).


Coleson seems to be holding up fairly well, both physically and emotionally.  I think his emotions are following a bell curve (can you tell I'm in business) as he is starting to ask more frequently about the number of days.  Not unlike my state of mind when I was running marathons... you start off thinking 1 mile down 25 to go,  a milestone so far off that you don't even want to watch the mile markers... you ask yourself, why am I doing this again?  Then you find your rhythm and you zone out for a while and you think, "I got this... Now I remember why I'm doing this", I feel great like I can do and achieve anything!  Then you hit mile 20 (the "Wall") for some and you again start asking, "Why am I doing this again?".  Self-doubt on being able to finish the last 6 miles sets in, you start to feel pain in various places in your body.  You start rationalizing that you've already run 20+miles and isn't that a big enough accomplishment... no one will know if you walk the rest of the way in...  the miles are counting down quite as quickly as they were before... But before you know it, you look up to see that you are already at mile 25...and you get your adrenaline back... but that last mile.2 feels like the prior all of the previous 25 miles combined.  Somehow the additional .2 feels like so much more.  But alas you cross that finish line and later you can reflect on the entire race and soon enough similar to giving birth without any meds, you think... that wasn't too bad, I can do that again!    I don't think Coleson will ever want to go through this again, but hopefully he will come to the same conclusion that this wasn't so bad and he can accomplish anything!




We are finding ways to get through the days/weeks.  Thankfully we have quite a few friends both work and fellow Hurler families here.  We've been to the RMH a few times for bingo and hanging out with the other Hurler families.  They changed the BMT protocol for Hurler kids a couple of years ago to replace a couple of the harsher chemo drugs in lieu of a couple others that are less invasive on the body.  However it seems like the kids are having a harder time keeping the transplant and many are finding that they have to go through 2 BMTs and other complications.  There is a family from Michigan here right now who adopted their Hurler boy and he is on the brink.  I can't even say (or write) what he is on the brink of because it is too hard.  The mother sent out a FB plea to the birth mother (whoever she is) to connect with her so that perhaps he can receive an immediate donation of a sibling or from her just so he can survive.  They aren't even worried about the Hurler syndrome gene at this point.  It is heartbreaking.    It is families like these that make me grateful for what I have and how relatively speaking, Coleson has run his marathon with relative ease. 


We still have a few more appointments to go, Endocrine, Spine Ortho and Wrist/hand Ortho.  I already know his TSH levels are going up so I think he will need an increase in his thyroid meds.  I hope that we continue to get good news from the other ortho docs as we have in prior years.  Then finally we get the braces off and start physical therapy. 


I am looking forward to going to Prince's Paisley Park in a couple weeks with another Hurler family.  I was a huge Prince fan in Jr. High/High School!  Minnesota has so much to offer!  However today it offered us a hail storm this morning and a possible tornado this afternoon.  I don't mind the storms but don't like the prospect of riding out a tornado in the basement.  Peyton is already preparing what she will take down with her if a watch or warning pops up.  She wants to bring all of her clothes, games, stuffed animals, etc. with her!  I tried to convince her that buying new clothes wouldn't be all bad if we had to.  She wasn't buying it!   She gets so attached, she doesn't want to see anything get "lost". 




Meanwhile today is going to be an indoor day which gives me time to post, organize pictures on my computer and do other things that I never find time to do as we don't get too many "indoor" days in Arizona and even if we do, there are too many other things to do around the house. 

Oh, and today is my sister, Beth's, 40th birthday!  Talk about someone who has run a marathon... well I guess she "wheeled" her way through it.  She has defied the odds... Happy Birthday Beth!  Also, my grandmother turned 102 in May.  Another women who seems to be running a marathon!

 

 

 

 

 

 

 

 

I've Got That Power

After 9 hrs. of surgery and only 3 nights recovering in the hospital we were able to bring Coleson home on Friday, a whole 4 days earlier than expected.  I am biased but my kid has super hero strength to recover from surgeries.




Monday evening's "talk" about the surgery was uneventful.  Peyton and I told him that his hips were not formed correctly and the Dr. needed to fix them and he would be in a wheelchair afterward.  Peyton had twisted her ankle on Monday and was hoping on one foot and she thought she needed x-rays and possibly a wheelchair as well so she explained that to him as well.  Tuesday's drive to the hospital reminiscent of last years drive, albeit a different route and this time all of us went.  The pre-op routine was very familiar, as we've experienced this at least 15 times before between surgeries, MRIs, ear tubes, etc.  This time though, they access his port and but the happy time cocktail in his port in a stealth like fashion which also had the benefit of having almost instant impact.  He immediately started to smile and act goofy but he didn't fall asleep so alas I had to "suit up" in the paper jumpsuit and mask and make the uncomfortable walk to the OR room.  The walk to an from where you try to fight back the flood of tears that are begging to flow out.  If you've never seen an OR room, it is what I can only describe as "eerie".  It feels like a cold garage, made sterile and filled full of medical gadgets, instruments and machines and of course the bright lights. 


It was a long 9 hrs. but the nurse called us every few to let us know everything was going OK and as more time past the better as that indicated to me that they were able to proceed with all of the surgeries.  There were actually 6 procedures, the cutting of the femurs and drilling the plates to secure the pieces together, shaving off part of the pelvic bone which would be used as the wedge that was needed for the hip socket and then the actual cut in the hip socket to make it more round by placing t previously cut hip bone into the now vacant space in the socket.    As Dr. Walker explained to us after the 9 hr. day, there were only 2 minor issues but otherwise the surgery went perfectly.  The first was that one of the electrodes that were placed into Coleson's skulp had inadvertently came loose and ended up poking the anesthesiologist which meant both Coleson and the anesthesiologist had to have their blood testes to see if Coleson had any viruses or otherwise communicable diseases. He also had to have 1 pint of blood but that is pretty typical.




Reminiscent of last year, Coleson again demonstrated his superhuman ability to rebound from surgery.  He was on quite a bit of pain medicine with a self controlling pump that would administer addition pain meds as he needed.  A 5 year old obviously cannot be responsible for administering his own pump so the nurse asked if I would like to do it or have the nurses.  After not sleeping for 2 nights already, I was in no shape to be making those types of decisions.  I could barely form intelligible sentences at that point.  So I signed off on having the nurses do it the first night.  Coleson was very anxious every time he woke up that night and I went from trying to sleep on the couch to the chair to eventually in his bed with him.  Fortunately we are both small enough to do that and the hospital allows that.  It's been really cold and rainy here in Minnesota since we have arrived and the hospital room was pretty cold so snuggling up to him kept me warm. 




Coleson wasn't very happy every time he woke due to the cast that ran from his thighs and covered his feet, the catheter and all the IVs in him.  He also doesn't like the EKG stickers nor the pulse ox that is stuck to his toe to measure oxygen levels.  As true Coleson fashion, he started lobbying to get things "out" and "off" every chance he could get.  He was able to get one of the IV lines out of him that was used to measure his blood pressure during surgery.  He had to wean off the pain pump and take meds orally to be able to get much of the rest off and out.  Well Coleson never needed more than 1 additional "pump" that first night and was comfortable with just the normal dosage of pain meds/relaxers every 4 hrs.  So by mid-day Wednesday we shut off the pump.  The Catheter could be removed 6 hrs. after this happened but I said we might as well wait til the morning since that would put us in the middle of the night. He had tried to pull on it several times and with his strength and will power I was afraid that if he found the opportunity, he would pull it right out.   He also started asking to go to the bathroom.  Another milestone a patient has to achieve before getting released.  He was pretty scared of the wheelchair like potty that he had to use since the casts prevented him from using a real potty.  However we were able to coax him into it which is a process when you have casts on both legs but to no avail.  I was hoping for a little more sleep that night but Coleson was determined to go to the bathroom and get the cath out.  He couldn't wait til morning so it seemed we tried every hour throughout the night.  Finally at 2:00am, we had achieved the necessary "wait" time after the pain pump was shut off and the removed the cath.  An hour later he finally was able to go to the bathroom.  Another milestone achieved.   




The next morning the Drs. came in and said they would be fitting him with his braces that day.  A day earlier than expected!  I started thinking that we could actually get out of here before spending the whole Memorial Day Weekend in the hospital. So we started the process of removing the casts and getting his new blue braces and his wheelchair was delivered so once we were done with that we were able to take him out and about.  He had also started taking his pain meds by mouth instead of IV so he was only getting the saline in his IV.  Another milestone achieved.  More lines came of him, he was mobile (wheelchair assisted) and now we were just waiting to be released.  I was able to sneak away on Thursday afternoon for a few hours to shower while Aggie, Paul and Peyton stayed with Coleson.  They took him outside and our friends Tasha and Braden along with Tasha's mom, Betty, came for a visit.  Braden is 12 and has Hurlers too.  We met them our first year here in 2013 during BMT.  He also was getting this same surgery.. .again.  Braden has been through variations of this surgery twice before and was back again this summer.  I can't imagine going through this again and hope that we do not have to.




The next morning we got the fabulous news that Coleson could go "home".  I put it in quotes as we are renting a place here so it really isn't our home but it is a "home" none the less and much better than being in the hospital.  The release process always takes a while but by noon we were heading out of the hospital just as Aggie arrived back in Michigan.  Paul had dropped her off at the airport on Friday morning after a quick visit with us. 




So we were able to enjoy Memorial Day weekend at home, learning how to take care of our boy who can't bend his legs.  He is not enjoying his wheelchair but we brought a big running stroller with us and his seems to like that better.  The weather still isn't cooperating much but we have had a few hours of sun so that we could take him outside and play for a while.  We have to do some Range of Motion exercises with him 4x a day so his legs don't get too stiff which he doesn't mind since we have to take the braces off.  He has weaned off all meds already.  I didn't think we were going to get released from the hospital until now and he is already off all his pain meds! 


So it is back to work and reality with some adjustments to our normal life and we anxiously await June 26th when we return to the Dr.s to remove the braces and start to learn how to walk again!  I think the song that Coleson likes to sing, "That Power"  (I've got that Power) sure applies in this case as Coleson certainly has some time of Power to endure surgeries and hospitals and Dr.s time and time again.   

Here We Go Again!

One of Coleson's favorite songs to sing and dance to is "That Power" or as he calls it, "Math Power" by Will.I.Am.  He mistakenly sings the refrain, "Here we Go, Here we Go, Here we Go Again", which is kind of how I felt talking to Dr. Walker, the orthopedic surgeon who will be performing 1/2 of Coleson's surgery tomorrow (this procedure takes 2 surgeons working at the same time).  Hopefully this time it will "Go" as planned and we will be on the other side of this in about 24 hrs. from now.  Probably about the same time you all are reading this. 


Sorry this is my first posting since we have arrived but we had a summers worth of fun to pack in before today and that is exactly what we did.  What does that look like you ask... well it entails a whole day each dedicated to our favorite places starting with the Edinborough Indoor Play Park and Gym on Tuesday, 7 hrs. of amusement park fun at Nickelodeon Universe on Wednesday, 5 hrs. at Crayola Experience on Thursday, 5 hrs. of waterpark fun on Friday and a trip to Minnesota's Largest Candy Store and 3 hrs. at Dave and Busters on Saturday. Fortunately or unfortunately, the weather was cold and rainy all last week so we didn't feel like we were missing the outdoors at all.  We are however done with all the indoor fun and would like nothing but sunshine the rest of our stay.  Yesterday I picked up Grandma early in the morning from the airport and we had a much needed day to recoup before we started into this week.


As far as all of his appointments went last week, we had nothing but "stable" news, which is kind of the best you can hope for with Hurler kids.  We started off with our favorite of the bunch, a visit to Dr. Miller at the BMT clinic and then to Pulmonary, Cardiology and lastly Audiology. Both Pulmonary and Cardiology said he is looking really good and Audiology said his hearing tests have improved from last year slightly but still some mild to moderate hearing loss particularly in the right ear.  Of course, all of these clinical visits require a battery of tests, labs, EKGs, Echo, X-rays, vitals, etc. which Coleson hates to do but he handled most of them like a champ.   We still have endocrinology, spine and hands and wrist Dr.s left which will all be post surgery.


So the day has come that I have to break the news to Coleson that we have to go back to the hospital yet again tomorrow but this time there will be "owies".   Sill not sure what I am going to say or when but I am sure I will find the strength somehow.  I don't think there is any better way to do it so that it won't make him sad and scared so it probably doesn't matter what I say but that I just make sure we give him as much love as possible when I do it.  Peyton of course has been a big help at all the Dr.s appointments especially today for the x-rays which took quite a bit of encouraging for him to do.  He did not want to lay down on the table for the hip x-ray as I am sure he associates laying down with surgery. 


So as you read this, I am hoping we will nearly be out of surgery and that I will soon be following this up with good news.  We could still use all your thoughts and prayers and appreciate all of them we have received along our journey thus far. 

We Have a Date!

For surgery that is...I was both elated and felt my heart sank all at the same time when after 5 months of weekly harassing two different Dr.s offices, I heard the news that Coleson was finally approved for surgery.  Elated as it meant that his skull and spine surgery of last summer was dissipating the syrinx in his spinal cord and anxious as it meant we would soon be leaving for Minneapolis to have the bi-lateral pelvic and femoral surgery (the one he was supposed to have last summer).  The only date that we was available at that point was May 23^rd unless we waited to mid-July.  It means leaving 2 weeks earlier than had hoped and missing Coleson’s pre-school graduation but also 2 weeks more of recovery before he starts Kindergarten.  Peyton of course does not mind one bit that we are taking her out of school a week and a half early.  It also means I will be narrowly missing a visit from one of my best friends, Christina.  I am hoping that we have less Minneapolis visits in the future and maybe we can actually take a family trip to California.  Coleson is so excited to go to Minnesota… fortunately for now he associates positive experiences with Minneapolis.  I hope this summer doesn’t change all that.  The hardest part for me as a mom is not the actual surgery itself as that is up to Dr.s and higher powers, and all of you help get me through the day, but it is telling him that he has to have surgery and what the recovery entails… cast, braces, pain, etc.  Harder this year than last as he has “grown up” so much this past year and is just so much more “aware”.  Of course, I won’t tell him until probably the day or two before and won’t go into all of the details but I need to prepare him that he will be in a wheelchair for a while.  Fortunately, Peyton does know and they have discovered a new iPad game called Roblox which has a game that takes place in a hospital and you can have your characters play doctors and patients and get casts and be in wheelchairs.  She is such a good big sister and is teaching him that it’s OK to have surgery and be in a wheelchair through this game.  Sometime you have to appreciate technology!  Speaking of how grown up my kids are getting, Coleson still loves to sing but has matured his play list from Twinkle Twinkle and Wheels on the Bus to more a more eclectic collection.  He loves to sing and dance to “Just Dance” videos on YouTube.   From boy bands, (“Best Song Ever”) to classics and 80s like (YMCA, Final Countdown) to Pop like Katy Perry (Dark Horse), to Black Eyed Peas (“I’ve got the Power”).  However, I think our theme song this year is “Fight Song” by Rachel Platten.  He really belts out this one. 

You might have noticed that this is my first blog entry in a while.  Life has been just so “normal” for the past few months which has been a welcome relief.   Let me see if I can recap the past 6 months:

 

I continue to develop my career at Honeywell.  My team is constantly growing in size as a result of organizational changes.  My team is over 40 employees now and I continue to expand my responsibility into more processes.  As the saying goes, the one constant is change and I can attest to that!  I have been getting back into running but have no ambitions to run marathons again.  I also do Zumba and Pound at the gym.  We have gotten more involved with local organizations like HopeKids AZ which is a non-profit that provides free fun events for families with kids with terminal diagnosis.   As there are only a handful of other MPS families in the family, we have really appreciated being a part of a bigger organization and are enjoying meeting other families who are going through similar experiences.  

Paul also continues to expand his career at Honeywell.  He was promoted last summer and now is responsible for a global team which means he is back to traveling overseas.  His most recent stress reliever is refurbishing his ’72 Corvette.  I can’t remember when he first started this project but our garage was starting to bulge out with 3 cars and an entire car’s worth of parts.  Fortunately he made great progress and many parts are back into the car including the wheels and engine and in fact the entire car is at the paint shop getting its original Bryer blue paint. 

 

Peyton is still doing well in school, although she would prefer to stay home and play all day.  She continues to enjoy gymnastics and also added violin, acting and drawing classes this year.  She dropped dance as ballet was not her style.  She is much more a contemporary and hip hop dancer and gets lots of practice alongside Coleson with the Just Dance videos.  She also spends probably too much time on her iPad mostly playing Minecraft or Roblox and watching YouTube.  She also can’t seem to walk without doing a cartwheel, a summer salt or some other parkour type of move.  She is older than her years and continues to be best big sister with the biggest heart.  She loves all animals, literally won’t even let me swat flies, and wants to be a pet sitter when she grows up.  I’m thinking this is more of a teenage job for her instead!  I definitely don’t think she will follow in our footsteps into the business world.  She is such a free spirit and has a knack for the stage and an audience so she may go in a completely different direction! 

 

As mentioned, this was a big year for Coleson as far as just “growing”.  He still swims like a fish and even dives down to collect items off the pool floor.  He still enjoys gymnastics and KTR (a parkour type of gym).  He is graduating pre-school after 3 years of the same school/teacher and transitioning to Kindergarten at Peyton’s school.  This will be a big transition for him.  I have been having the regular IEP meetings with the school and he actually is scoring in the average range for almost all of the disciplines.  In fact the only service he will be getting next year is speech and I think this is mostly due to the fact that he hasn’t had his upper 4 teeth since he was 2!  Hard to say “Fs” and “S”s with a window in your mouth.  Of course, we don’t know what to expect his recovery to be like after surgery and we may have to add on Physical Therapy for a while.  He wants to be a doctor, a firefighter or a construction worker.  I think he could also be a baker as he and Peyton both love to bake!  Cupcakes are their specialty.  

 

We have added a new addition to the family this year, our Yorkie Terrier mix, Bella.  She is a rescue pup who was rescued from a kill shelter in Texas.  She is the most people loving pup but a bit protective of us around other dogs.  I am taking her to training so we can train her to be more mindful and give her more appropriate puppy social skills.  Everyone that interacts with her agrees she is the just the best puppy.  We really did luck out with her.  

 

We have had a change with our nanny situation.  Rachel graduated and took a full time job in an old care facility.  Fortunately she works afternoons/evenings so she is still able to come in the mornings and help get the kids off to school.  I don’t know what we’ll do next year when hopefully she gets a job on the management side of a care facility and probably will work more “regular” hours.  We did find a college student, Jaclyn who has been coming in the afternoons.  She has been a fun (youthful) addition to the family. 

 

Will continue to update as we start our travels.  Thoughts and prayers are appreciated.

Back to Reality

We are back to Arizona and back to “normalcy” (whatever that means)!  Things didn’t go as planned this summer but I think they went as God intended.  We ended up having a really good summer (minus the few hospital days) packed full of friends, family and fun.  In fact, so much fun that the kids were completely bored returning to the 115 degree weather here in Arizona.   They are constantly asking to go to, “more fun places” and “what friends are we going to see next”?  I knew we were spoiling them but knowing what next summer is going to be like, I wanted to make sure that Coleson especially looks forward to going to back to the “blue house”.   

 

The kids are back to school, Peyton in 1^st and Coleson in Pre-K.  Coleson loves to go to school while Peyton tolerates it.   The song, “Girls Just Wanna Have Fun” was written for her.  She can play hard all day long but going to school for 7 hrs. is not much fun (except for recess/lunch times).  I try to remember what I was like at her age and life was very different for me at age 6.  It always is from generation to generation but at the age of 6 my mother had been gone for 2 years and my dad had just met Aggie. I can tell you my life was not about going to “the next fun place”.   Both kids have decided to focus on gymnastics this year and rock climbing for Peyton.  Peyton and I were watching the Olympic Gymnastics and swimming events and I asked her if she thought she could do that one day.  True to her big heart she said, “If I were beating someone, I would stop to help them so we could both win”.   She is going to be the best kind of friend.  With her big heart though comes big sadness when saying goodbye to friends and family or getting her heart hurt easily and I dread her teenage drama years. 

 

Coleson has rebounded from surgery and is becoming just as dare devilish as Peyton.  He wants to go on roller coasters and water slides, he’s flipping into the pool.  This makes me more nervous because he doesn’t understand his limitations yet...which is good in some ways but not so good in other ways.  He makes me nervous for Kindergarten when he is with a bigger population of kids.   He is enjoying gymnastics and school and is doing quite well in both.   We have a few appts. coming up, his repeat sedated MRI to see if his surgery was successful and if he will be approved for next year’s surgery as well as ENT.  We can never get a good audiogram on him since his ears are constantly draining from his tubes.  I know the ENT wants to remove his adenoids but I am really trying to prevent another surgery.  I’m hoping as he gets bigger the drainage will reduce in his ears.  He went through a bout of ear infections before we left for Minneapolis but now they just drain occasionally.   Otherwise all of his other appts. went well.  Cardiology said she wouldn’t even have known that his heart was a “Hurler heart”, and pulmonary went well too.  Ophthalmology said his eyes are holding steady with a slight near-sidedness, retinal atrophy and astigmatism, but as long as they are stable he is doing well.  He is still 100% engrafted with donor cells at 3 years out and I expect will remain unchanged.  He is developing at a more age appropriate level in almost all areas but is still behind in gross motor skills (the test doesn't ask about flipping into pools!) so we need to continue with therapies.  They also mentioned we need to work on his attention span and focus so it doesn’t start to impact his schooling.  His spine looks great with only a 17% kyphosis in his lower back and his neck still looked really good (minus the cyst in his spinal cord).  All in all, we received very positive results in almost all areas.  Most Dr.s said they did not need to see him again next year which is good because it is going to take all we have to make it through the pelvic and femur surgeries and I don't want any additional stress on him. 

I turned 1 year older this summer and got to celebrate it three times, 1x in MN, 1x in MI and 1x in AZ.  I think it's official now!  Paul has resumed working on his corvette and parts are actually starting to go back into the car as opposed to out.  Soon tires will be put back on and it will sent out for nice new paint job. 

 

As mentioned, we had a great summer full of friends, family and fun in both Minneapolis and in Michigan.  I appreciate and thank all of you who came to visit, had us over, took us out, let us store stuff, just had fun with us.  We didn’t get to see everyone we intended to see as we still have to work while we are there.  We really missed seeing some of our other BMT family that we typically get to see but we did get to spend some time with some other Hurler families this year and that was nice too.  

 

We are about to kick off the busy Fall season full of holidays and birthdays.. more fun times filled with friends and family.  Thanks for being part of our journey. 

Surgery, What Surgery?!

For those of you on Coleson's Facebook page, some of this will be repetitive, but I tend to be more detailed in my blogs than I am on FB.  For those not on FB, I apologize for the lack of updates.  I actually tried to post on Thursday but blame it on the lack of sleep, that I wasn't successful.




You would never know by Coleson's behavior that just 5 days ago he was in a very risky and lengthy surgery!  Aside from the stiff neck and the stiches (roughly 30) that resemble Frankenstein, he has not once complained of pain and has been running around playing since Thursday when he was released.  Yes, he was released on Thursday morning, a full 2 days earlier than expected much to his delight and amazement from the surgeon and his physical therapist.  I'll back up a few days for you all.




Surgery, Suboccipital craniectomy with C1 laminectomy and dural splitting (trying saying that 3 times fast!), went extremely well, just as it was supposed to.  Coleson was both scared and mad to awaken to all the IV lines in his hands/arms and feet and promptly starting requesting they be removed.  We had a bit of a scare on Tuesday night as he was striderish meaning his airway was swelling from being intubated.  They had to administer steroids to reduce the swelling.  By Friday morning he was breathing good but had a "silent seizure" where his heart rate dropped and he was unresponsive.  My heart rate did just the opposite as I pleaded with him to say something.  It lasted only a minute (but to me it seemed like forever) and came out of it.  They thought it was due to coming off the sedation meds and fortunately he did not have any more.  He drank a whole bottle of ensure for me and after a couple of hours of being stable, they started removing most the IVs and moved us to the recovery floor.  Oh the memories of being cooped up in these rooms for months during transplant.  Fortunately the Children's recovery wings are new (I'd say 5 years old) and they are very nice...for hospital rooms.  After being stable for a few hours on the recovery floor they removed the last IV, he still had his port accessed and a big bandage on his arm where his arterial line was.  He was fairly cranky until Peyton arrived to cheer him up with Ninja Turtle Playdough.  His whole demeanor changed as they played together.  About 6:00pm,  his physical therapist asked for us to try to get 30 minutes of unsupported sitting that night. So at 7:00, we asked if he wanted to sit in his special blue chair, which he loved to sit in when he was here for BMT.  He sat and played with us for about an hour and then he was feeling frisky and wanted to stand up/walk.  He needed to get weighed anyways so we walked him over to the scale.  He didn't stop there, we opened up his door and walked up and down the hallway.  This lasted for another hour for a total of 2 hrs of unsupported (neck) movement.  I was feeling confident that we may get released a day early at this rate.  He had only been on Tylenol for pain most of the day.




He was adamant he was not going to sleep in the hospital bed that night (it's too scary) and fell asleep on the couch (my bed) a little after 9:00.  After allowing what I hoped was enough time that I could move him without waking, I picked him up and laid him down on the bed...I didn't wait long enough!  He began to get upset and I laid with him for another hour about no more pokes.  The thing about sleeping at a hospital is they have to come at shift change and do vitals and then again at 4:00am. (A practice that is being challenged by the medical industry as being disruptive to the healing process and I hope gets changed in the future).  At this point any single type of status check was scary to him and he know if he was in that hospital bed, they would be in to touch him.  He stayed asleep until 3:00am when he woke and cried for me.  I promptly moved into bed with him again and had another 1 hr. talk about  how scary the hospital is and his "inside" owies and that he was done with the hurt part and he just had to get strong enough to go home.  I called the nurse to come do vitals early and give him his meds since he was awake and I could help him through it.  I had just gotten back to sleep it seemed when he woke for the morning at 6:00.  "Is it daytime?" he asked, he knew day time meant possibly going home, even though I knew we had at least 1 more day.   He was again pretty cranky, not wanting to do anything but go down the elevator... "go down 2 elevators mommy", he says, knowing that it takes 2 elevators to get to the car in the parking garage.  "No up elevators", he's too smart!  Daddy showed up at 8:30 and somehow convinced him to get out of bed and walk the hallways.  Within 30 minutes, the neurosurgeon saw him walking around and with a look of surprise asked if was ready to go home which Coleson promptly answered, "Yes!"  Paul said the physical therapist also did a double take when she saw him and was amazed he was up and walking.  It was Coleson's sheer determination and fear of the hospital.  He was not about to stay in that hospital any longer than necessary, and I don't blame him.  I too was exhausted from 3 nights of no sleep and anxiety and wanted to get home too.  Paul says it was all his and Coleson's plan as he knew it was Paul's night to sleep at the hospital.  At any rate, he was a free man and he returned to his normal happy self.  He even said to my friend Nicole who had stopped by to see us and helped us carry our things down to the car, "Thanks for riding down the elevator with us and thanks for carrying our bags".  Where did that come from?! 




Since returning home, he as been as joyful and funny as normal.  He has not slowed down at all and we spent all day exploring the train museum and science museum on Saturday.  It was rainy so a good museum day.  Today we went to Nicole's for lunch and friend time.  Then we stopped for "Izzy's Ice Cream" an awesome ice cream joint near our place which reminds of our good friend Issy from Phoenix.  Then to Choo Choo Bob's for some toy train play time.  Even after all that he was not ready to come home but after too full days, Paul and I were ready. 




We return to somewhat of a normal life again, we have all his usual appointments to contend with over the next several weeks but the only one that will require a "procedure" is the EKG.  I would ask them to skip it but with my own heart condition, I can't risk it.  Otherwise, they drew all the blood they required (which is like 13 vials) and I asked the spine surgeon to get the scans from Dr. Walker.  Us Hurler parents get to be pretty savvy at "combining" and "reusing" tests for his other specialists. 

We have a couple Hurler family friends arriving this week and will try to see them as well. I'll update again as we get through the upcoming appts. 




I can't thank everyone who supported us over the past 2 weeks.  It makes such a big difference, I can't even put it into words.  A simply Thanks doesn't seem enough but that is about all I can give as well as letting you into our personal world. 

Paul says' Chicks will dig this scar when he's older!  This chick hopes it disappears!

Quick Update

Surgery went well and after 7 1/2 hrs., we were able to see him again.  He's in pain, swollen in the neck from the intubation tube and neck from the surgery.  His airway started to close a little last night so we were watching him closely but he's paste the worst of it now.  He's scared mostly as he's worried there are more pokes and owies to come.  He's got IVs in both feet and one in his artery in his right hand which is boarded up to protect it.  Hopefully we'll get released from the PICU today to be moved to the general surgery recovery floor.  We are watching Team Umi Zoomi (reminiscent of BMT days) and he drank a little apple juice.  I'll post more details later.   

New Surgery, New Plans, New Attitude

HOne of the hardest parts about this process is the complete loss of control over the well being of your child.  You get hit with this overwhelming information, just enough to fill you with anxiety but not enough to put all the plans in place or put you at ease.  Once you have all the information you start to take comfort in the "game plan".  I feel like that is where I am at today. 


We met with the neurosurgeon yesterday.  There was no denying once he threw up the pictures of the MRI that the cyst exists and is big.  Even to my untrained eye I knew immediately where it was and that it was big (I'm sure you will see it too in the picture below).  He explained the root cause of the cyst was due to a malformation of the skull which creates a "pooling" of spinal fluid in the base of his skull.  This in turn creates and imbalance of fluid flow along the spinal chord and brain.  Think of it as a river, and his skull in creating somewhat of a dam which affects the rest of the flow of the river (aka his spinal chord) which causes other "pools" to form along it.  Fortunately it is only the 1 and there are not others.  Bone malformation is one of the most common Hurler traits and is the reason for the hip surgery.  Hurler Syndrome is a "storage" disorder meaning substance (called gag) that should get processed and expelled by the body rather does not and instead stays in the body in places where there is room, mostly the joints.  Because this gag material is sitting in places where bones would normally develop, it causes the bones to develop abnormally.  It is a total body affect but each Hurler kid's bones develop different depending on where most of the gag goes in the body and in Coleson's case it had a big affect on the skull. 


It is big enough that they need to address it.  To fix it, they have to break down the dam so to speak.  So next Tuesday, the 31st, he will have surgery to remove some bone at the base of the skull  which will allow the fluid to stop pooling in the skull which naturally (over the coarse of a year) should reduce/eliminate the pooling in the spinal chord.  It is too dangerous to do surgery on the cyst itself as that would be require surgery on the spinal chord.  We will be back at the University of Minnesota for this surgery so back to our normal stomping grounds.  I asked if this surgery could be combined with a new port placement and was happy at least to hear that it could be and in fact is scheduled with the same general surgeon who put in in his central line 3 years ago.  It does mean another 6 hr. surgery and 4 night stay in the hospital but at least he will walk out of the hospital.  We have some follow up appts and MRIs over the next year but we should be able to plan the hip surgery for next summer. 


Though I was hoping he would not have any more "Major" surgeries after the hip surgery, I guess, technically, this isn't after the hip surgery.  It is unexpected and a risky procedure itself, but it allows Coleson another year to comprehend the hip surgery which may not be a bad thing.  It does mean another traumatic and scary experience for Coleson however and though I was hoping as he got older, he would just "get used" to all the pokes, prods, medicines, IVs, X-rays, EKGs, MRIs, echos, blood pressures, pulse ox..... you get my point, I think he is getting more afraid of them.  We will have to take extra care in tackling each procedure as simple and nonintrusive as it may seem to be. 


So we have some re-arranging of plans left to do but it also allows us to get home around mid-July instead of at the end of July so that we can get settled before school starts on August 1st. I do believe that everything happens for a reason, so while I still don't know the reason why kids are born with such difficulties, I do believe we were not yet ready for the hip surgery and needed another year to prepare.


After the appt. yesterday, we all went to Mall of America and spent 6 hrs. riding rides.  Paul and I even got to ride the adult rides for once since Grandma was with us watching the kids.  Peyton is still not big enough to ride those yet but maybe, just maybe by next year she will be.  She can't wait.  Coleson too loves the excitement of the stomach turning rides and unfortunately will have quite a few more years before he will be big enough to ride them. 


So think of last Tuesday as a trial run for this Tuesday and next year.  The outpouring of support has really kept me going these past couple of days and I cannot thank you all enough for that. 

The white is the pooling fluid and the "dam" where the skull meets the chord.

It's What's on the Inside that Counts!

I was hoping to start this entry with news of a successful surgery today but as one Hurler mother recently said, we need to be prepared to expect the unexpected.  I thought I had accounted for the unexpected by reserving a house for the summer but nothing prepared me for the events over the last 24 hrs. 

We had a relatively uneventful first couple days getting settled here in our temporary home.  Our place is nestled in a very nice neighborhood with a park 3 blocks away.  Our landlord is very accommodating and has a girl close in age to Peyton who only lives 1 block away.  Dairy Queen and a very nice local grocery store are within 3 blocks, the river across the street.  The weekend weather was gorgeous and without the distractions of every day life, we actually were able to relax on Sunday. 

The knots in my stomach didn't start to occur until we were on our way to the pre-op apt on Monday.  Coleson did well during the Child Life discussions and started out being a big boy for the x-rays but after 3 of them started to melt down and I had to help him through the other 4 or 5.  Our consult with Dr. Walker went really well and I was actually feeling very confident about the surgery.  The last item of the day was to get labwork.  We started off in the lab, which we found out could not do lab draws from his port.  We have not tried an non-sedated blood draw since he was first diagnosed and didn't even want to try.  So we went back up to the Dr.s office for a nurse to do the draw.  After about 15 minutes and several  pokes and prods, they were unable to get a blood return from the port.  There must either be clotted blood around it or a collapsed vein, regardless it needs to be replaced.  Next they brought in a PICU nurse to try an entry into a hand or foot.  By this time, Coleson had already had enough and even with 4 us holding him down, the nurse could not get a needle in a vein in either hand.  So they called in "Dee Dee" who for sure would be able to do it.  So again with the attempts but now on his feet.  Again we all had hands and legs secured and he was lying in my lap with me shielding his chest holding his head and he still wiggled and whaled rendering any attempt useless.  For a 4 year old with Hurler's he has superman strength!  We had to get the blood draw so they could get the right kind of blood (with the proper antibodies) prepared for today's surgery.  So Plan D was to use nitrous.  After 3 hrs and with the help of nitrous they finally got enough blood.  It was a horrible experience and not the way I wanted to start this surgical procedure. 

Today actually started off better, except for the complete lack of sleep for me, Coleson didn't mind the 5am wake up call and immediate bath with the special soap (which we also had to do last night as well).  He didn't start to get nervous until we were in the parking garage and refused to get out of the car.  However, once we got into the waiting room and play area, he was OK.  He was even OK up to the point that they masked him for his MRI.  Prior to the MRI, Dr. Walker said based on the X-rays that he consulted with a neurologist and they thought his neck was strong enough to perform the surgery as intended, with the femurs being done while he was facing down on his belly and then the hips with him lying on his back.  Last night we had talked about doing the entire procedure on his back, though a little more complicated for the surgeon, less risk for the patient.  This I felt was actually good news and was looking forward to getting this behind us.

We didn't receive the bad news until Dr. Walker came out after the MRI to give us an update.  I knew something was wrong when he wanted to talk to us privately.  He reassured us immediately that Coleson was OK but that the MRI revealed a cyst at the base of his brain on his spine.  This cyst was putting pressure on the spine and it would not be safe to perform the surgery.  The cyst, though currently was presenting no signs it was there externally, would need to be taken care of before it started to effect his balance and cognitive ability.  The next hour was overwhelming and all I wanted to do was to hold Coleson again but they wanted to perform more MRIs to ensure there were no other cysts.  Fortunately there was no more.  But now we are facing another surgery, plus we need to get the port replaced and who knows what will come of the leg/hip surgery. 

On the outside you would never know that Coleson even needed any surgery.  He climbs like a champ and even started doing flips in the pool.  He is super smart (ask him anything about the planets) and his communication is even maturing.  His latest IEP was good in every area.  But on the inside, you never know what exists and with a Hurler kid, it could be just about anything.  It's a total body condition and each kid presents differently.  So, another challenge for us and for him.

Two of my closest friends sent me this same message (separately coincidentally), "You never know how strong you are until being strong is the only choice you have."   I have never had a choice to be strong, it started when I was 4 and lost my mom.  I feel like life has challenged me in so many ways  it is the only choice I have and I will continue to be.  I've said this before but it remains true, I can only be this strong with the love and support of friends and family and yet again, you all pulled through for me today.  Our appt. with the neurosurgeon is tomorrow (today by the time you read this) and I'll hopefully have a better idea of what our new plans will be. 

Continue the prayers and wishes and this truly is hard on Coleson who knows enough to be scared but not enough to believe everything will be OK.  I really am concerned for his mental well being along with of course his physical well being.  I'll update as soon as we know more. 

Help Support MPS Research with Triple Match Now - 3 Weeks til Surgery

Its 3 weeks and counting until Coleson’s surgery.  Each day brings more and more anxiety.  I recently learned that a young girl who had the same surgery as Coleson cannot feel anything from her chest down.   Her surgery was more than 10 days ago.  This is the risk/reality that we face when we send our kids back to the operating room and wait for 6-8 hrs. and then even longer for them to wake up from sedation and try to wiggle their toes.   My heart sunk when I read the update and I cannot express the sorrow I feel for Brynlee, her brother and her parents.  I know they have not given up hope yet that she will regain feeling and so if you feel so inclined to pray or send a good thought to them, I know they could use the support.  There are quite a few families having the same surgery this summer, but I think Coleson is the next in line, and I know all of us are diligently waiting for updates as each one occurs.   Brynlee’s mother posted a saying, “Sometimes the best thing you can do is not think, not wonder, not imagine, not obsess.  Just breathe, and have faith that everything will work out for the best.”   If she can live by this, then I should be able to as well.

 

With that said, there is a great opportunity to support MPS research (something that doesn’t get a lot of attention) and have it TRIPLE MATCHED.   If you ever have thought about donating to MPS Research this the time, triple matches don’t come often!  Triple Match ends this Saturday.

               

Donating to #TeamMPS is easy! There are three ways to help us reach our $50,000 goal. Remember, all donations are triple matched, so a $10 donation becomes $30!

1. Text the word "courage" with a donation amount to 41444.
2. Donate through Penn Medicine to #TeamMPS. -  This is the best way so you can learn more about MPS and this
3. Donate through PayPal.
    
   May 15^th is MPS Awareness Day.  So please wear your purple in Coleson’s honor.   I’ll be updating this blog frequently over the next several months as we climb this next mountain.

Here is a flashback from 3 years ago of Peyton Singing the "Brave" song for Coleson during BMT.

The Countdown Begins (30 days & counting)

30 days until we leave for Minneapolis and 34 before Coleson has his surgery (May 24^th).  Coleson will be having 4 surgeries in 1, double pelvic and double femur osteotomies.  The pelvic osteotomy is to correct a “shallow” socket that if left uncorrected will eventually lead to the femur “ball” slipping out of socket.  The femur osteotomy is to correct the alignment of his femurs into his hip socket.  Two surgeons will operate on him at the same time and the surgery is expected to last ~6 hrs.   It is a high risk surgery and one that all of us hurler parents dread, though almost all hurler kids have to have it done.  It is high risk as they are cutting into the femur (which is where bone marrow originates) and so there is potential for lots of blood loss.  In addition, there is potential for nerve damage due to the length of the surgery and the short necks and spinal curves our kids.  Needless to say, I will need all of your thoughts and prayers that day (May 24^th) as it is an excruciating wait.  Feel free to send texts/facebook posts/emails, etc. that day as it will be a good distraction from staring at the clock.  He is expected to be in a spica cast for at least 4 weeks and then we start physical therapy and rehab.  We are planning to stay in Minneapolis for the summer just in case anything goes wrong  and to ensure he is well on his way to recovery as well as go to all the other annual appts.  So Minnesota friends, we'll be in touch.  I hope to get to the office there a few times as well. 

 

Otherwise we have been enjoying the winter/spring here in Arizona relatively healthy.  Coleson has been battling chronic ear infections for the past 2 months.  We went to a new ENT and he suctioned out the blockage in his ear tubes.  Hurler kids tend to have thicker ear secretions and the antibiotic drops weren’t able to penetrate through it to clear up the infection.  Coleson didn’t like the suctioning at all but I could tell a noticeable difference in his behavior afterward.  He is a happy go-lucky kid in general but he was even more spirited afterward.  The ENT did plant that seed that we may need to look at removing his adenoids.  I am not in favor of having any unnecessary surgeries so hoping this won’t be necessary.  Peyton too has been fairly healthy with the exception of a cold/ear infection that happened to occur when my sister and niece were visiting.  I haven’t had any major ailments lately myself (knock on wood) however Paul came down with a serious case of bronchitis a couple weeks ago.   He was in bed for several days and we even took a trip to the ER.  by habit I almost drove to Phoenix Children's hospital.  It's been a long time since I've had to go to an adult ER!  He is still recuperating but is expected to feel the effects for another couple weeks.  Had a bit of a scare last week when Rachel called me from the park to say that Peyton fell off the monkey bars and wasn’t moving.  Luckily I had just taking a CPR and first aid class and was able to run through the checklist to assess the situation.  Fortunately she was breathing, just needed to calm down, and she didn’t have a concussion, but did have a sore back for a day or two. 

 

We have been enjoying the warm weather, plenty of pool time, gymnastics and dance.  I took Peyton on her first hike with some good friends and after our second hike, I think she has had enough.  Just not exciting enough for her… she takes after me, I’d rather be on a bike on a mountain rather than my legs.  Speaking of bikes, Peyton is proud of her newest accomplishment, riding her bike without training wheels.  She didn’t like riding it much with the training wheels so it didn’t get a lot of use. One day she got on a neighbor's smaller bike without training wheels and figured out she could do it. So we took them off her big bike and away she went!  The bike gets a lot of use now.  She has even rode it to school a couple times.  Coleson continues to be a climber, climbing anything and everything including the pantry and his closet to pick out his shirt for the day.  He is particular in what he likes/doesn’t like and that includes what he wants to wear.   We took the kids to a place with warp walls and trampolines and he did great!  He even somehow got up on the big warp wall (perhaps someone helped him).  He escaped the "6 and under" area without me knowing and when I couldn’t find him, my eyes raced around the gym and spotted him way up at the top of the big walls.  I really hope this surgery doesn’t set him back too far.   He is so adventurous and agile, it’s hard for me to think in just 1 month, he will be in a cast and wheelchair. 

 

I’ve been trying to stay as healthy as possible with my eating and my workouts. I’m been trying to get to Yoga or Zumba 4-5 times a week.  Paul can “handle” the kids now at night on his own so it allows me to get to the gym.  It’s amazing how much better I feel and how much more energy I have.  The kids have starting waking up earlier than usual (not sure why) and fortunately I’ve been able to adapt.  It’s amazing how much more I can accomplish with an extra hr. in the morning. 

Lastly, I took 66 lbs. of pop tabs to recycling this weekend for the RMH.  This is in addition to the nearly 100lbs from last fall.  The RMH will certainly appreciate this donation.  If anyone else has any pop tabs, please let me know. 

I'll probably be publishing some frequent updates for the summer as we tackle this next journey.  Thanks for reading!    Here are some recent pics and videos.