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Ever wonder what it would be like to never have to go back to work again, to put up your feet and have time for every book in the world that you’ve ever wanted to read?
Only about two or three times a day, I’ll bet.
Well, Guy Ashford-Smith used to do that. Now he says — be careful what you wish for.
When the 50-year-old Lisgar resident went to his family doctor for his annual bout with pneumonia in the winter of 2001, he never imagined where it would lead: to an instant trip to the emergency ward at Credit Valley Hospital, where a nurse’s eyes got big as saucers when she took his blood pressure.
He was asked to lie down and take oxygen. “That’s the last thing I remember,” he said Wednesday in the living room of his home on a lovely sunny winter afternoon. “I woke up three weeks later. The doctor told my wife he didn’t think I’d survive.”
Ashford-Smith had only 7 per cent lung capacity and obvious respiratory problems. Doctors thought he might had muscular dystrophy. Fortunately an intern at the hospital who had been studying MD, and a quite similar condition called Pompe’s disease, suggested they test for it.
Sure enough, Ashford-Smith learned that he is one of the unfortunate 10,000 or so people in the world who have Pompe’s, a degenerative, neuromuscular disease for which there is no cure. “I’m missing an enzyme that helps me use the energy in my muscles. I store the energy in the muscles and when enough energy stores up, there’s a sort of explosion.”
The graduate in civil engineering from Humber College, where he met his wife, now has about 30 per cent lung capacity. He tires easily. “Some days I come downstairs and have a cup of coffee and that’s it, I’m done for the day.”
He has very little strength in his upper leg muscles, making it difficult for him to
get out of many chairs. He uses an elevated chair that sits in four plastic cups so he can slide out of it without too much trouble. He has to be very careful not to fall because he can’t get himself up. His wife and three daughters have a tough time getting the 220-pounder vertical again.
This all sounds quite depressing until you chat with Ashford-Smith for a while. He is one of the lucky ones when it comes to adult-onset Pompe. The 50-year-old is not in a wheelchair or on a respirator yet.
He is obviously happy there is good news to share about a potential pharmaceutical breakthrough against Pompe.
A new enzyme replacement therapy called Myozyme, manufactured by Mississauga-based Genzyme Corporation, was approved by Health Canada last year. When given to the infants who are born with the disease, and who rarely survived in the past for more than a year, they have now all survived at least 18 months.
The release of the drug prompted Genzyme to bring together the 13 adult Canadians who have Pompe. That, in turn, prompted them to form the Canadian Association of Pompe, of which Ashford-Smith is the president (www.pompecanada.com).
“Myozyme is helping stop the progression of the disease so far. People have more energy,” says Ashford-Smith. “It’s kind of holding things in place until something better comes along. It’s not a cure.”
Earlier this week, the Mississaugan met with Ontario Health Ministry officials on behalf of the association to press them to fund the $400-$500,000 annual per patient cost of Myozyme.
The association is about both public education (especially of doctors) and about the camaraderie of communicating with the only other people who can really understand what living with Pompe is like.
Ashford-Smith is a house-husband now. His wife used to do accounting from home but now she has her dream job teaching in a Brampton private school which their youngest 14-year-old daughter attends.
He can’t do the heavy housework but Ashford-Smith wields a mean barbecue fork and does most of the family’s cooking, which is a challenge because his wife has Celiac disease and can’t consume wheat gluten.
He needs lots of protein so he’s getting good at soy and tofu. He has to avoid carbs, so he loves to work with fresh herbs and vegetables. He is an experimenter in the kitchen, and loves it when a new dish solicits raves from the family.
He misses the pleasure of his work at CTS in Streetsville where he would go into companies from whom the firm bought supplies and work with staff there to improve the process and save both partners money and grief. “I enjoyed my job. My brain is wired for working,” he says.
When well enough, he makes models and reads military history. He spends an awful lot of time sliding out of his chair to let Lacey, the family’s deaf-from-birth dog, into the back yard.
One thing still intact is Ashford-Smith’s sense of humour. He reflects on the fact that he has a lot of time on his hands, just like a lot of people dream of.
“Yeah,” he says. “I guess this is my lottery ticket.”