For Paul Rinderknecht, living with ALS, a 'right to try' is a chance to live

Paul Rinderknecht can walk, still, yet each step is labored. His right fingers curl into his palm. A machine keeps him breathing through the night.

Two years with Lou Gehrig’s disease has taken much. But terminal illness also forged a purpose: To see a day when he can exercise the right to bet his life.

“I accept my fate,” Rinderknecht said. “But I won’t stop fighting for the future.”

The Springfield Township husband and father, 44, has amyotrophic lateral sclerosis, a neural disease with a life expectancy of three to five years. About 20,000 Americans have the disorder, and 6,000 are diagnosed with ALS every year. Discovered in 1869, the illness is nicknamed for its most famous sufferer, the legendary New York Yankees baseball player of the 1920s and 1930s.

ALS has no cure and only two U.S.-approved treatments. In the summer of 2014, the grim situation prompted a goofy fundraising campaign that became an internet phenomenon. The Ice Bucket Challenge raised more than $115 million for ALS research and patient support. In August 2016, The Enquirer wrote about Rinderknecht laughing his way through a personal ice bucket challenge with three Cincinnati Bengals players. He had just started on an experimental drug that was showing promise.

Hundreds of Rinderknecht’s relatives and friends coalesced a support network called Team Smilin’ Paul. A year ago at the Pebble Creek Country Club, the team organized a massive benefit that raised $75,000. The silent auction at the Colerain Township club featured hundreds of fancy gift baskets, expensive dinners, wine, sports tickets and, as grand prize, a trip to France.

Rinderknecht’s wife, Leslie, 39, said, “The day he was diagnosed, we promised each other and our family that we would do whatever we could to keep him here. He’s still my husband. He’s still young. I want him here as long as possible. It’s a huge financial burden, but I’m not worried about it. We will figure it out.”

The Rinderknechts enlisted in a small but growing national movement. The political advocacy organization the Goldwater Institute has been pushing to give people with terminal illnesses, such as ALS or cancer, the right access to experimental drugs that have entered but not passed through the years-long approval process of the U.S. Food and Drug Administration.

Ohio, Kentucky, Indiana and 35 other states have enthusiastically enacted “right to try” laws. But drug approval is a federal task, so the state laws have no effect – unless Congress also acts.

The Rinderknechts have gone to Washington to ask lawmakers to vote on a federal right-to-try bill. The U.S. Senate passed a version last summer, and two similar House measures await action. So the Rinderknechts was startled and delighted last month when President Donald Trump in his State of the Union address named only one legislative proposal, the right-to-try measure. Paul Rinderknecht said the speech “did kind of kick-start a conversation, and that’s all I want.”

But Trump’s mention also kicked loose a landslide of opposition from scientists, researchers, ethicists, clinical-care doctors, even patients. It’s a comforting concept to say yes to a dying person who wants a treatment and can pay the formidable cost.

The FDA already has a compassionate-use provision for terminally ill patients, and the experts said a right-to-try law would endanger the nation’s drug-testing regimen to little general benefit. A significant problem with right-to-try laws is that they do not require manufacturers to provide experimental drugs just because a patient asked.

Within a week of the State of the Union address, more than 300 experts warned Congress, “This legislation sells vulnerable patients and families false hope at the expense of weakening the FDA’s critical role in making sure that all Americans can have confidence in the safety and effectiveness of our medical products.”

Rinderknecht said he appreciates and even agrees with the medical and scientific community. But ALS has made those arguments a luxury. In September, the illness forced him to leave his job as a Johnson & Johnson sales representative. In November, the maker of the experimental ALS drug that Rinderknecht took for more than a year pulled the product as a disappointment.

Rinderknecht recently started on one of the two FDA-approved ALS drugs. It’s too early to see results. His difficulty breathing at night means ALS is moving faster than expected.

“People get the impression that we’re trying to make a willy-nilly grab at the shelf,” he said. “If I were, then I’d be in Thailand at some stem-cell laboratory.”

But, “we’ve got to look at this differently. I don’t even want to circumvent the FDA. But it should be a patient’s choice to use a drug, even when it’s just in the early stage. There will always be a person who will say: 'I’m not scared of death. I’ll try that.' ”

Rinderknecht said he is that person, for the chance at another day with son Leo, 8, and daughter Nora, 6. He said he is lucky that, unlike many terminally ill people, he’s got the stakes to put on the table. The $75,000 that Team Smilin’ Paul raised at the silent auction “is my right to try.”

Paul and Leslie Rinderknecht plan a March trip to Washington to lobby Congress once more. They want to take the kids camping this summer. First, they are embracing a new ALS fundraising challenge, not to take on buckets of ice but to bite into hot peppers.

“We’ve got the jar of them already,” Leslie Rinderknecht said.

“I’m excited about it,” said Smilin’ Paul.

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What is the 'right to try'?