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.

Thu., Feb. 15, 2018: Ann Spears gives her son-in-law, Paul Rinderknecht, a dose of edaravone for the treatment of ALS. Edaravone had been used in Japan since 2015, but was only FDA-approved in May of 2017 -- making it only the second drug on the U.S. market to treat the disease. The first had been approved more than two decades ago. Rinderknecht has become an advocate for federal right-to-try legislation, which would allow terminally ill patients to have access to products that are not yet FDA-approved. (Photo: The Enquirer/Carrie Cochran)

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.

excerpt © 2018 Cincinnati.com. All rights reserved.