Charcot’s disease: death of Pete Frates, who popularized the “Ice Bucket Challenge”

In 2014, they were among the most popular on platforms like YouTube: the Ice Bucket Challenge videos, in which stars like Bill Gates, Mark Zuckerberg, David Beckham, Anna Wintour, Oprah Windrey and even the American President of then, Barack Obama, took up the challenge of pouring a bucket of iced water over his head to raise awareness of Charcot’s disease.

If so many celebrities have lent themselves to the game, it is partly thanks to Peter Frates. Himself affected by this rare pathology, this former professional baseball player then popularized the Ice Bucket Challenge and made it a viral challenge.

He died Monday, Dec. 9 at the age of 34 at his home in Beverly, Mass., his family said in a statement.

A disease with imperceptible symptoms

Also called amyotrophic lateral sclerosis (ALS), Charcot’s disease is a degenerative genetic disease that affects up to 500,000 people worldwide. In France, it is estimated that 5000 to 6000 people are affected. Between 800 and 1,200 cases are diagnosed each year.

In many patients, the onset of Charcot’s disease is almost imperceptible. It is characterized by regular muscle contractions and cramps, muscle stiffness, muscle weakness in an arm or leg, but also slurred speech and difficulty chewing and swallowing.

“These conditions gradually turn into bouts of weakness that become more and more evident, possibly leading to atrophy, which leads the doctor to suspect Charcot’s disease,” explains information site dedicated to Charcot’s disease. Symptoms can occur in the legs, hands, or arm. The patient realizes this when he regularly stumbles or can no longer perform simple everyday gestures, such as turning his key in the lock.

Most often affecting people aged 40 to 60, Charcot’s disease can also occur in younger subjects, of all origins and all socio-professional categories. If 5 to 10% of the cases are hereditary, the disease appears in 90% of the cases by chance, without associated risk factor. Death usually occurs three to five years after diagnosis.

Five new genes discovered thanks to the Ice Bucket Challenge

Diagnosed in 2012, Pete Frates has since never ceased to fight against the disease and to make it better known to the general public and thus to advance research.

According to the ALS Association, donations related to the Ice Bucket Challenge totaled $115 million. These funds were used in particular for the discovery of five new genes linked to the disease and the financing of new clinical trials.