Connected to his shadow for the day, a skinny little IV pole, Simon Hoskins cracks jokes at the expense of his siblings.
He’s a pretty funny guy for six, and chipper for someone who’s received enzyme transfusions weekly for pretty much the latter half of his life.
The three Oak Bay kids – Spencer, 9, and twins Isabel and Simon, 6, – are also pretty comfy with the term mucopolysaccharidosis (MPS) though the family uses the specific term Morquio. It is part of the standard lexicon for the three Willows Elementary students.
It explains why Simon uses a scooter to get around; why his bone structure looks different than other kids his age; and why he spends Thursdays connected to an IV.
MPS is a rare progressive genetic disease caused by missing or malfunctioning enzymes that break down molecules called glycosaminoglycans. Over time, the glycosaminoglycans collect in the cells resulting in permanent, progressive cellular damage. Type IV, or Morquio, mainly affects the skeleton.
Once a week he spends the day receiving a synthetic enzyme that helps replace the missing.
“It’s great. And it’s not a cure,” mother Becs Hoskins said. “Success is slower progression. We’ll take it.”
With 52 infusions per year, by the time he’s 10 Simon will have endured more than 350 days hooked up to an IV pole. He spends those days hanging out with a nurse – he absolutely plays favourites and is a bit of a gossip hound – doing everything from screen time to music games or even baking.
These days, he’s also working out how to give speeches, with a plan to address his peers at Willows asking them to wear blue for International MPS Awareness Day on May 15.
In an interesting twist, when young Simon was diagnosed at two-and-a-half, neighbours and friends already had a baseline understanding. Another Simon in the community had laid the groundwork, and many people had heard of MPS, Becs explained.
Simon Ibell was 39 when he died in 2017. He had lived with MPS type II, or Hunter syndrome, since a young age.
A St. Michaels University School grad, as is dad Trevor Hoskins, Ibell was active in raising funds and awareness. In 2002, Ibell biked the length of Vancouver Island with NBA icon and fellow SMUS grad Steve Nash, raising $250,000 for Bike 4 MPS, founding the Be Fair 2 Rare outreach program, and founding the iBellieve Foundation. SMUS still celebrates IBell each spring with the iBellieve Right to Play event.
The advocacy work IBell did set young Simon up for the treatment he receives, and the family up for a community with a base knowledge of MPS.
“It rooted us. It was such a gift to start somewhere,” Becs said.
The web page for iBellieve no longer exists and social media accounts appear inactive since 2017. But not long after young Simon’s diagnosis, mom Becs scooped the website curemps.ca with a plan.
“We could start off where Simon Ibell left off,” she said.
As a family they decided that now, settled into a routine and with International MPS Awareness Day on May 15, is the time to share their story.
Visit curemps.ca to learn more.