Teen spared cystic fibrosis death sentence

Larissa Rueckenbach and her family can breathe a little easier. After three tries, the family finally got Vertex Pharma to add their daughter to the company's compassionate care program.

Larissa Rueckenbach and her family can breathe a little easier.

After three tries, the family finally got Vertex Pharma to add their daughter to the company’s compassionate care program. That means Larissa will get a lifesaving cystic fibrosis drug for free, instead of the $349,000 per year price they’d originally been faced with.

“I was on my way to the bank to set up a donations account and start fundraising when the cystic fibrosis clinic in Vancouver called to say Larissa was on the program,” Larissa’s mother, Heike, said.

“I was in tears. Larissa asked, ‘Mom, why are you crying,’ but I was just so happy,” she said.

A week ago things were much more grim. Larissa has an extremely rare variant of cystic fibrosis called the G551D strain. The genetic disorder causes mucus build up in her lungs, painful arthritis aches and cystic fibrosis diabetes.

She has trouble breathing and is sometimes bedridden with arthritic pain. She missed so much school this year that she’ll have to do Grade 8 over again next year, but if her new medication works as expected, she won’t have to sit out of gym class any more.

Kalydeco has been hailed as a miracle drug for Larissa’s form of cystic fibrosis. It treats the cause of the disease directly, and while it can’t reverse the damage already done, studies show it has the potential to stop CF progression in its tracks. Other patients who are on the drug, which costs upwards of $400 per pill, say that within days their lungs begin to dry out and pain subsides.

But for months, Larissa’s family was stuck in limbo, waiting for the Pan-Canadian Pricing Alliance to finish haggling with Vertex over the price.

Earlier this week Ontario’s Health Department announced that the bargaining was almost over. A tentative deal had been reached that would allow provinces and territories to cover the expensive drug under public health care plans.

Pat Living, a spokeswoman for Yukon’s Health Department, confirmed the news.

“We have a price now, we’re just waiting for the formal agreement. We’re really quite pleased,” she said.

Last month Yukon Health Minister Doug Graham teamed up with his Albertan counterpart, Fred Horne, and met with Vertex top brass, ramping up the pressure to reach an agreement.

“The ministers went down to Toronto to explain the impacts on us in a Canadian context and give the company an explanation of the expectations from Canada,” Living said.

“We needed to make sure we were getting good value for the drugs.”

In a statement, the company cautioned that even though a tentative deal has been reached, the work is still not done.

“Once an agreement is finalized, we stand ready to work as fast as the individual provinces can to put in place the product listing agreements that will allow eligible people with CF across Canada to begin receiving Kalydeco under public reimbursement. Until all provinces finalize their listing agreements, our work is not done,” Vertex said.

While the negotiations had been going on, the Rueckenbachs had applied for Vertex’s compassionate care program twice. The family’s doctor at B.C.‘s cystic fibrosis clinic wrote letters of recommendation, pleading for Vertex to cover the drug and laying out how badly she needed it.

They were turned down twice. Now, days after a tentative deal to cover the drug’s cost publicly was announced, Larissa finally got the OK from the company.

Heike said the timing is a little suspicious, but regardless the family isn’t complaining. They’re just happy Larissa will get the help she needs.

And she plans to make the most of it. This summer is already filling up with events for the spunky 13-year-old.

She’ll be going to Calgary for a weeklong horseback riding camp where she’s hoping to learn how to jump. She’s also planning to spend as much time as she can with her horse, Fancy.

“I’m really excited, and so is she,” Larissa said, stroking Fancy’s white mane.

“We really love to run together.” If Larissa’s new medication works they way it’s expected, the two will be doing a lot more running in the weeks and years to come.

Contact Jesse Winter at

jessew@yukon-news.com

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