Never a dull moment!

Early on in Simon’s diagnosis, another MPS mom who I had a chance to meet (who is now a close friend) warned me that this journey would be a relentless and wild roller coaster – with highs and lows and everything in between. She said that there would often be periods of intense medical interventions, appointments and decisions and then somehow, life would return (temporarily) to a relatively uncomplicated and ‘normal’ feeling state. This advice rings so true for our family. I’ve learned that being aware of and making the most of the simpler times allows us to fill our cups and build up our resilience for the next wave of complexity . . .  when the inevitable brutal reality of MPS comes rushing back again. 

This summer was one of those complicated periods of time – interspersed with vacations and summer camps. Every August, the provincial government reviews whether or not they will continue to fund Simon’s life sustaining enzyme treatment (those are his weekly infusions). To make their assessment, they require a long list of annual assessments – most of which involve trips to BC Children’s Hospital:

  • Pulmonary function test
  • 6-minute walk test
  • Gross motor physio evaluation
  • Fine motor physio evaluation
  • Audiology report
  • Ophthalmology report
  • Overnight sleep study
  • Cardiology test
  • Bloodwork
  • Physical exam

It’s essentially a full time job to take the time to coordinate all of these appointments and make sure they’re completed on time. And they require this every. single. year.

All of that, on top of his weekly home infusions and regular appointments to see his physiotherapist and orthopedic surgeons.

To give you a sense for other MPS related complexities this summer, I’ll share a little bit of the whirlwind with you . . . just before summer vacation started at the end of June, Simon went under general anesthesia for a simple procedure to place tubes in his ears – he was a champ and bounced back pretty quickly. Just a few days later, he missed another day of school so that we could head over to Vancouver for an overnight sleep study . . .  where they hook him up to over 20 wires to monitor his breathing while he sleeps overnight in the hospital (I get to be there right alongside him). Neither of us slept very well but were happy to get out of the hospital around 6 am and so grateful for our friends for hosting us for coffee and a shower first thing in the morning.

We then headed back to the hospital to meet a new orthopedic surgeon – Dr. Mulpuri, a hip specialist – to discuss Simon’s decreasing mobility and increasing hip pain. He made a plan to follow up again in 6 months and monitor progression. That sounded simple enough . . . though also didn’t leave us with much of a plan. It turned out that Simon’s hip pain increased substantially in the weeks that followed and on August 1st we were able to get Simon back in to see Dr. Mulpuri. We discussed the advice we received from Dr. Mackenzie at Nemours Children’s Hospital in Delaware (our March 2023 trip to Philly / Delaware) and in the end, Dr. Mulpuri decided to put Simon on the list for a major double hip reconstruction in early 2024.  Woah, how quickly things changed. All of a sudden we were grappling with how our busy life as 2 working parents and 3 energetic young kids could handle such a disruption. We managed to distract ourselves with summer fun through the rest of August, though we felt the heaviness of what lies ahead impacting us all in all sorts of different ways. 

We’re still in limbo about specific timing of the surgery, but we do know that it will involve a week in the hospital following a 3+ hour surgery, then 4-6 weeks with Simon casted from his chest all the way to his ankles, and then a couple of months of in-patient rehab in Vancouver at Sunny Hill before returning home to Victoria for more rehab through the rest of the year. We can’t picture how we’re going to manage all of this, but we are feel strong and entirely focused on making sure we’re all feeling mentally strong and physically healthy – filling our cup with the things we love. . . lots of time in nature, good food, family time and a bit of travel. More to come as we know more!

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Read our story about MPS Type 4A
and how it has changed our lives.

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