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)!