LEANDER, Texas (KXAN) — $272,000 a year.

That’s how much a drug called Orkambi is listed for, and some researchers say that’s four times its value. 

Orkambi is for people with cystic fibrosis, which is a genetic disease that affects your lung functions. It’s one of the first drugs to treat the underlying cause of the genetic disorder, rather than only treating the symptoms.

The drug can break down mucus built up in the lungs to prevent infections, helping people live longer. 

Lora Moser, who lives in Leander, told us she was diagnosed with cystic fibrosis when she was 2 years old.

“When I was diagnosed in 1979, the average life expectancy was about 14,” she said. “So my family made it their mission to do everything they could to extend my life.”

That included raising money for research and development of drugs like Orkambi.

“My family has spent endless hours fundraising for these life-saving drugs,” she said. “My family alone has raised three-quarters of a million dollars in order for these drugs to be developed, and now here I am, the drug has been priced out of my reach.”

Moser told us in January, she learned she no longer qualified for a grant that used to help pay for the co-pay.

Under Medicare, Moser was still responsible for a $15,000 co-pay for Orkambi, which is one of about a dozen medicines Moser needs every day. So without the grant, Moser’s family said they cannot afford the drug. 

“There are thousands of people on Medicare that are in the same circumstance that I am, whether it be cerebral palsy, multiple sclerosis, diabetes or heart disease,” she said. “There are Medicare patients out there that are unable to obtain grants and are struggling to find ways to pay for these excessively expensive life-saving drugs.”

“All of these families, they’re like, most of them are like us,” said Casey Moser, Lora’s husband. “We’re not asking for a handout. We’re willing to pay a fair market price for the drugs, but I mean, $70,000 a year, for co-pays and things like that, hardly anybody can afford that.”

Eight months without Orkambi led to Lora Moser losing 26 percent of her lung functions. Two weeks ago, she ended up in the hospital. 

“It’s been an extremely frustrating process, scary to say the least,” she said. “I feel like the situation is out of my hands, and my life is truly in the hands of tech pharmaceuticals.”

Moser said she asked Orkambi’s manufacturer, Vertex Pharmaceuticals, for help, but she’s still without the medicine. 

“I want to see his kids graduate. I want to see them get married and have babies,” she said. Moser told us the average life expectancy for cystic fibrosis is now about 40.

Her 40th is coming up in a few months. “Here I am, I’ve made it this far, and to watch my lung functions deplete so rapidly because I’m unable to obtain this drug, it’s maddening. It’s absolutely maddening,” she said. 

Vertex Pharmaceuticals sent KXAN this statement:

The list price for Orkambi in the U.S. is $272,000. Importantly, that is not the price patients pay. Patients in the U.S. today have broad access to our medicines (over 99% coverage) and we have a number of financial assistance programs in place to help those who are eligible and need support beyond insurance. While we can’t comment on individual patient cases, I can tell you that we evaluate each on an individual basis, and do review our assistance programs regularly to help as many patients as possible.

According to the Institute for Clinical and Economic Review, researchers believe the price for Orkambi should come down by about 75 percent.

“Discussions at our meeting highlighted that, while these therapies offer important benefits for those living with CF, the $300,000 annual price is far too high to pay year after year, harming patients and families today while threatening the health care system’s ability to maintain access to important future clinical advances,” noted Dan Ollendorf, PhD, ICER’s chief scientific officer.

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