Ottawa

Ethical questions cloud lottery for $2.8M gene therapy

For parents whose children are suffering from spinal muscular atrophy, a Swiss drug maker's lottery for free doses of a miracle drug with an astronomical price tag is a slim chance at hope. But a CHEO clinician-scientist questions the company's true motive, and the wider impact on drug pricing.

CHEO expert questions Swiss drug maker's giveaway of prohibitively expensive treatment

Four-month-olf Eva Batista has been diagnosed with spinal muscular atrophy, a rare, potentially deadly genetic disorder that weakens her muscles, making it difficult to breath at times or eat without the aid of tubes. (Paul Smith/CBC)

It's a high-stakes lottery where the prize is a potentially life-saving drug.

But a clinician-scientist at CHEO is questioning the motives of the drug company behind the draw, even as he acknowledges the potential benefits for individual families.

The drug, for very young children diagnosed with spinal muscular atrophy or SMA, is called Zolgensma. It's a one-time, "one-and-done" dose with a $2.8 million price tag.

Last week, Swiss drug maker Novartis launched a lottery, offering up the treatment for free to 100 babies and toddlers living in countries where the drug is not yet approved. 

'Like a candle going out'

Dr. Alex MacKenzie, a clinician-scientist at CHEO with experience in the treatment of SMA, described it as "a poignant disorder" that causes "very cognitively normal, happy children [to] slowly waste away … in the first two years of life … like a candle going out."

But the gene therapy drug appears to be the spark doctors and families have been looking for. 

"It looks like we might be able to use the word 'cure' here," MacKenzie told CBC Radio's Ottawa Morning. "You go from what is a fatal disease to a baby who can sit and even walk. So it really is a bit of a poster child for effective therapy."

CHEO clinician-scientist and researcher Dr. Alex MacKenzie is questioning the motive behind a drug company's lottery for free treatments of a prohibitively expensive gene therapy treatment for spinal muscular atrophy, a disease that causes young children to 'slowly waste away ... like a candle going out.' (Stu Mills/CBC)

At least one couple in Toronto is hoping to win the lottery. Jessica and Ricardo Batista's four-month-old daughter, Eva, has SMA, which weakens her muscles and makes it difficult for her to breathe or eat without the help of tubes.

"If there is something available, why do we have to wait till she's failing and in her last moments?" Ricardo Batista asked in December.

Lottery 'a sideshow'

But MacKenzie wonders whether Novartis has an ulterior motive with the lottery.

"This may be cynical, but it may be a means of letting the wide world know how effective this therapy is, as well as addressing those of us who feel the price is too high," he said.

"I don't think even Novartis would say this is unvarnished altruism on their part. I think it's a bit of a sideshow. I think the bigger challenge is how we're going to address the price for all Canadians."

MacKenzie said there are 3,000 SMA cases in Canada alone. "So the 100 doses worldwide … is a drop in the bucket."

Parents Ricardo and Jessica Batista want a shot at winning a free single dose of the one-time gene therapy injection that could potentially cure their daughter's life-threatening disease. (Paul Smith/CBC)

Another concern that MacKenzie has is the precedent the drug's astronomical prices would set.

"The tail is wagging the dog to some extent. The prices of these drugs seems to be fuelling price increases more broadly," he said.

For the families who are taking part in the Novartis lottery, it's a slim chance at a free cure. But MacKenzie said there's still a cost.

"Free for the families. But free for society? Obviously not. And so there's the very hard calculus. Health ministers and others have to contend with this, and I don't envy them this choice."