The topic of the day for Winnfield Rotary Club’s meeting on November 30, 2022, was RideAtaxia Dallas, the annual long distance bike ride to raise money for Friedrich’s Ataxia Research Alliance [FARA], an organization dedicated to researching treatment and a cure for Friedrich’s Ataxia [FA]. Rotarian of the Day Shea Sanders introduced the speaker, John “Bo” Walker, who told the audience of Rotarians about the success of the fundraiser, as well as the success of his granddaughter, Kate Walker, and her team, Kate’s Krewe, in raising money for the cause.
According to Mr. Walker, Kate was the second-highest individual fundraiser for this year’s RideAtaxia Dallas, raising $22,048, with the aid of her father, Jason Walker, her grandparents, Bo and Chris Walker, and Dr. Jimmy and Helene Walker, all residents of Winnfield for many years. Kate’s Krewe was the highest team fundraiser for the ride this year, with $29,344, with the collaboration of many friends and relatives adding to Kate’s personal fundraising efforts. Kate herself raised $4000 through a FaceBook fundraiser, while Laure Derr, along with her friends at Our Lady of Lourdes Catholic Church in Winnfield, generated donations of $3500 with a spaghetti dinner, and Kim Caldwell raised donations of $900 with a bake sale. All of these funds went to FARA via RideAtaxia Dallas. The organizers of the Ride set a goal of $175,000, and at the time of the Rotary meeting on November 30, the amount raised was a little over $168,000, leaving little doubt that as the final donations arrive, the event will meet and exceed the goal.
So what is Friedrich’s Ataxia, and why do so many people in Winnfield care about it? FA is a relatively rare disease which affects only approximately 5,000 people in the United States and about 20,000 in the world. The condition is caused by lack of or mutations in the FXN gene on chromosome 9, which makes a protein called frataxin. When the body’s cells produce less frataxin, the lack of frataxin causes degeneration of nerve tissue in the spinal cord. This causes the ataxia, that is, lack of voluntary coordination of muscle movements. The degeneration of sensory neurons in the spinal cord essential for directing muscle movement of the arms and legs through connections with the brain are particularly affected. The spinal cord becomes thinner, and nerve cells lose some of their myelin sheath, which is responsible for insulating the nerve cells and increasing the rate at which electrical impulses pass along the connected nerve cells.
The low levels of frataxin means that the electrical impulses that fire to cause movement in the body pass along the nerve cells more slowly or sometimes not at all. Thus, muscle movement which most of us take for granted, particularly of the arms and legs, become slower and eventually impossible in many persons affected by the disorder. FA is one of a larger group of disorders known as muscular dystrophy.
Initial symptoms include difficulties with balance and motor movements. For example, one of Kate’s first symptoms was that she was not able to achieve adequate balance to ride a bicycle. She was diagnosed with cardiomyopathy, hardening of the heart muscle, at the age of eight, but it was not until 2016 when Kate was 13 years old that a doctor suggested she be tested for FA, which revealed that Kate has the genetic abnormality that causes low levels of frataxin. Her parents both underwent genetic testing that revealed they both have the gene that causes a lack of frataxin. The disease only occurs in children whose parents both have the defective gene.
Persons affected by FA generally have a life expectancy only into the mid-20’s, but some people can survive years longer. The effect on the heart is the main reason for the low years of life expectancy. One of the men with FA who began the bike rides to raise money for research, Kyle Bryant, is currently in his late 30’s. The symptoms progress at different rates in different people, and the mid-20’s is only an average of all people known by FARA to have suffered from the disorder.
Some of Kate’s symptoms have progressed since her diagnosis in 2016, while others are currently unchanged over the past year. She was unable to walk within four years of her diagnosis with FA, and she has begun to develop scoliosis (curvature of the spine) but is not experiencing difficulties because of that condition yet. For the last year, her cardiomyopathy has remained unchanged.
Despite the challenges presented by FA, Kate is thriving in many ways. As a sophomore at the University of Arkansas in Fayetteville, she lives on campus, attending class and getting where she needs to go via wheelchair and a van with hand controls. She works with a personal trainer to improve her upper body strength, which aids her in transfers to and from her wheelchair. She is socially as well as educationally active, and is a tireless advocate for those with FA. She has a vlog on YouTube to which she posts videos about her activities when she has time. One may also follow Kate on FaceBook and Instagram.
“So how does a person who can’t walk ride a bike for miles and miles?” you ask. The bicycles are designed specifically for person with low levels of lower body strength. They have two wheels on the back and one on the front. The seat is recumbent, meaning that the rider is essentially lying on her back, almost straight out, in the seat of the bike, and is thus able to achieve enough leverage with the body that the legs can move the wheels on the bike to move forward.
Kate and her family and friends have been supporters of FARA since her diagnosis with FA in 2016. Over the six years, Kate and her Krewe have donated $159,576 to the organization to help fund research into FA, its causes, and drugs which can retard the progress of the disorder’s symptoms, as well as seeking a cure. As a charitable organization, FARA is rated 4 out of 5 on its ratio of administrative expenses to funding of research. Four percent of the money received by FARA goes to administration, which is 6 or 8 people in a small office in Philadelphia, and the other 96% is invested in research. A promising drug which appears to be able to retard progress of the symptoms of FA is in development by Reata Pharmaceuticals in collaboration with FARA, but the FDA did not consider it in the last round of approvals. But it is highly possible the drug might be FDA-approved in the first quarter of 2023. Unfortunately, Mr. Walker pointed out, drugs for diseases which affect only a relatively small number of people in the United States get little attention from the FDA as it spends most of its time on drugs for diseases which affect many people, and drugs for FA do not get much attention.
In addition to supporting FARA to stimulate research on drugs for treatment and a cure for FA, Kate’s parents formed a foundation called Make It Happen—Cure FA a couple of years ago. Contributions to this foundation are used to held young person with FA obtain the equipment and aids they need to maintain as much independence as possible, including electric wheelchairs, hand operated vehicles, service animals, walking aids such as canes and walkers, and other aids to help with dressing and other activities of daily living.
For anyone who would like to donate to FARA, the website for RideAtaxia Dallas is still available through which you may make your donation. Please be sure to select Kate as the individual and Kate’s Krewe as the team on whose behalf you are making the donation so it will be properly credited to her fundraising efforts. Even the smallest amount can make a difference, added to many other small amounts.
After Bo answered all questions, the meeting was adjourned, as customary, with the Rotary motto, “Service above self!”