Last Friday, we highlighted how PSC tragically struck down NFL hero Walter Payton in his prime. Walter Payton is just one of many amazing people we have lost to PSC. Today is the tenth anniversary of the death of Chris LeDoux, another American legend who died as a result of bile duct cancer associated with PSC.
Chris LeDoux was a national rodeo champion, as well as a star in the country music world owing to the songs he wrote about his cowboy lifestyle. LeDoux is famously mentioned in the debut single from Garth Brooks, “Much Too Young (To Feel This Damn Old)”. Following the release of this song, Brooks and LeDoux became good friends. They performed a duet on the title track for LeDoux’s golden album “Whatcha Gonna Do With a Cowboy”, a song that was a top ten country music hit in 1992. The two singers were so close that when LeDoux was diagnosed with PSC in 2000, Brooks volunteered to donate part of his liver to LeDoux.
Garth Brooks’s liver was an incompatible match for LeDoux. However, a matching donor was found, and unlike Walter Payton, Chris LeDoux managed to receive a liver transplant to treat his PSC. Unfortunately, like Walter Payton, Chris LeDoux also became one of the ten percent of PSC patients to develop bile duct cancer, or cholangiocarcinoma, which took his life ten years ago today. His death in his home state left a “hole in Wyoming’s heart”, and in Kaycee, Wyoming a weekend of Chris LeDoux Days are celebrated each year with rodeos and music. The city also hosts a Chris LeDoux Park with a magnificent “Good Ride Cowboy” monument at its entrance (pictured). As the monument and memories of Chris LeDoux stand strong and cherished, the risk of developing cholangiocarcinoma is still the source of tremendous anxiety for present PSC patients and their families.
Ten years since LeDoux passed away, and over fifteen years since Payton passed away, liver transplantation for cholangiocarcinoma has come a long way. In the old days, cholangiocarcinoma in the setting of PSC was a death sentence: patients were generally considered ineligible for liver transplantation. But a protocol was developed at the Mayo Clinic in Rochester to give PSC patients with cholangiocarcinoma a chance at liver transplant and survival: patients undergo radiation and chemotherapy, and if their disease can be contained, they undergo liver transplantation when a donor organ becomes available. As data accumulate to support this approach, more centers are beginning to offer this treatment option to patients facing this deadly disease.
Leading PSC clinicians are also working to incorporate risk stratification for cholangiocarcinoma into ongoing clinical assessments of PSC patients, which is important for identifying patients who should be screened more frequently or more thoroughly. Here at SAVE JON one of our main objectives is to provide those screening tools by finding new ways to detect the development of cholangiocarcinoma as early as possible; one example is our partnership with Perspectum Diagnostics. Another example is a recent study in the American Journal of Gastroenterology, highlighting multifocal polysomy as an important predictor of risk for cholangiocarcinoma; this study was conducted by researchers at the Mayo Clinic, including Dr. Lazaridis, a SAVE JON advisory board member. Meanwhile, new molecular studies continue to emerge that may shed light on the origins of bile duct cancer: one example published in this month’s issue of the Journal of Gastroenterology and Hepatology by Dr. Hiroshi Matsushita and colleagues suggests that the development of cholangiocarcinoma may be caused by an insufficient immune response through a protein called NLRP3 in bile duct cells. This points to a potential target for new drugs designed to prevent cholangiocarcinoma in PSC patients. However, much more work needs to be completed before we can eliminate this devastating complication for PSC patients.
Like many PSC patients, LeDoux left a string of achievements in the wake of his premature passing, as well as a personal example for us to follow. He is listed in the ProRodeo Hall of Fame for both bareback riding and his contribution to the sport through music. More importantly, his bio on the official Chris LeDoux website states that he “helped teach us to believe there is a lot of good out there we can do for others while at the same time enriching our own souls beyond measure”. At SAVE JON, in the memory of Chris LeDoux, we aim to do for others what sadly could not be done in time for him.