Doug Penner will hopefully be feeling a little more energetic by the time Sunday’s motorcycle ride in his honour kicks off.
The 14-year-old was diagnosed with Duchenne muscular dystrophy (DMD) when he was two. It’s a commonly fatal genetic disorder that affects approximately one in every 3,500 to 5,000 male children.
For the past 11 years now, Doug’s father Cam – and his entire family – have hosted the Ride for Doug each June in Langley.
This ride has raised more than $175,000 for Muscular Dystrophy Canada and been a constant morale booster for Doug.
Meanwhile, Doug has continued battling his disorder, with a number of ups and downs – through the years.
The past year has been particularly hard on him, explained his father, Cam.
Doug was part of a drug trial for Drisapersen since 2011, but last year that medication was rejected by the Food and Drug Administration. Consequently, when the study was discontinued and the medication was no longer available, Doug’s health began to deteriorate, Cam said.
“For the past yar, we have watched Doug begin a decline in strength and endurance. His muscles are weakening, despite receiving the best care possible. The one medication that seemed to make a difference sitting on a shelf, slowly expiring,” explained Dad.
But as of Tuesday, the situation has changed. While the drug has still not been approved, and is not expected to be, whatever quantity was left from the study has been transferred from the pharmaceutical company to an organization called CureDechenne.
“Despite everyone involved really wanting to make this abandoned drug available, the paperwork and approvals process was formidable,” Cam said. “Thanks to a small but very dedicated Duchenne community we received word that the drug was going to be made available again.”
Now, after continued lobbying for access, those leftover drugs are being made accessible to just a few of the past recipients. And Doug is among them.
“But not every boy with DMD can access this. There are probably only five boys in Canada who are eligible to participate,” Cam added.
Doug received his first treatment earlier this week.
“What does this mean to us?” Cam said. “There are so many different emotions. Relief that the long waiting and uncertainty have ended. The frustration that Doug lost an entire year’s worth of mobility while waiting. And, lots of hope that we will once again see Doug stabilized like he did all those years on the medication.”
Based on their experience the last time the drug was halted for a year (a few years back), the Penners expect that during the next three to four months Doug’s declining strength will stabilize.
“We anticipate him having longer endurance and better balance. To feel more energetic. We don’t expect to see a lot of strength increases – that doesn’t seem to be how this drug works – but with DMD a significant slowing of decline is a major victory,” Dad said.
Practically speaking, it means the Penners will be taking Doug into BC Children’s Hospital once a week for treatment, where he’ll be hooked up to an IV for several hours as the infusion takes place.
That’s a lot of missed school, Cam admitted. But it seems a small price to pay.
“I don’t know exactly how much of the drug is left,” he said, noting a year’s worth has already been transferred to BCCH for Doug. Cam hopes there’s more to follow.
“Hopefully, researchers can make more progress on finding a more permanent cure,” he said, admitting he’s burned out emotionally from this fight but remains constantly optimistic.
“New technologies are always on the cusp of something great. This special access program buys us some of that time.”
In the meantime, he’s now gearing up for this Sunday’s (June 4) ride that is expected to see about a hundred motorcycle enthusiasts leave the South Langley Church (20098 22nd Ave.) at exactly 1 p.m. and take a three to four-hour ride through the rural areas of the Fraser Valley before returning to the Langley church for a barbecue.
For more information or registration, visit: www.ridefordoug.com.
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