A Cecilia resident has made it her mission to raise awareness of Huntington’s Disease, a rare neurological disease from which she suffers, and she is taking her message to Congress.
With color-coded notes in hand, Kala Booth testified virtually Thursday during an Energy and Commerce Committee’s Health Subcommittee hearing. Invited by Rep. Brett Guthrie, R-Bowling Green, who serves as the Republican leader of the subcommittee, Booth shared her family’s battle with Huntington’s Disease in a hearing titled “The Path Forward: Advancing Treatments and Cures for Neurodegenerative Diseases.”
“This allowed me to tell Congress what we need as an HD community,” she said. “It lets me be a voice for people who don’t have a voice.”
The hereditary disease is a progressive brain disorder caused by a defective gene that was identified in 1993. It causes changes in the central area of the brain, which affect movement, mood and thinking skills, according to the Alzheimer’s Association.
The gene has a 50% chance of being passed on to children. Booth’s grandfather and mother were diagnosed with the disease, which is fatal and has no cure.
Booth, 34, said she believes being tested for the gene is a decision each person must make on their own. She was tested early and the diagnosis allowed her to make “very big life choices.”
“It helped me be part of clinical trials which helps bring hope to so many people,” she said. “It gives me purpose and gives me hope for me and my family.”
Booth’s testimony advocated for the passage of House Resolution 2050, the Huntington’s Disease Parity Act. The act would eliminate waiting periods for Huntington’s patients for Social Security Disability Insurance and Medicare.
“Huntington’s is very different from other neurological disorders,” Booth said. “There’s already some diseases, like ALS, already had their waiting times waved.”
Unlike other neurological diseases, Booth said symptoms begin affecting quality of life in Huntington’s patients by their early to mid-40s, not later in life.
By the time the disease progresses and the patient can no longer work, they lose employer provided health insurance and have no financial recourse for health care within the six-month waiting period for Social Security disability and two-year waiting period for Medicare.
“So they’re losing income, the medical bills are now coming in and now the family is dealing with this progression (of the disease),” she said. “It’s just hit after hit after hit.
“So two years in a Huntington patient … they’re pretty far advanced,” she added. “Two years is just too long.”
Booth said it took longer than the six-month waiting period and several court appearances before her mother was able to get Social Security disability.
“Mom started her SSI app in Nov. 2019 and wasn’t awarded until March 2021,” she said.
Booth hopes her testimony will help shed light on this disease so care can be improved to help advance treatment options and avoid misdiagnosis, which both have caused stays in mental health facilities for her mother and grandfather.
“I want to bring awareness so I don’t get stuck in the system and so that there isn’t that stigma,” she said. “It brings knowledge to people because they don’t know how to help and they don’t know what they’re treating.”
Guthrie said Booth’s testimony was important for a number of reasons, but mostly to bring a “unique perspective” and show “that anyone can testify before Congress.”
Guthrie said it also allows government officials, Congress and those serving in various agencies such as the Food and Drug Administration, to put a face to the problems they are working to address.
“It hopefully gives a mission of what they’re there for and who they’re serving,” he said. “I think a lot of times agencies and members of Congress, they get inside a bubble and you look at studies, you look at numbers, you look at dollar signs and you got to remember there are real people that are affected by the decisions that we make.”
Guthrie said he wants to capitalize on Operation Warp Speed, where the COVID-19 vaccine was manufactured, tested and approved by the FDA.
“(The FDA) didn’t cut corners for safety,” he said. “They ensured we had a vaccine that is 99% effective and they were able to get it approved in record time with no sacrifice for safety.”
Some neurological disorders, such as ALS, patients don’t have four years to participate in a clinical trial because their life expectancy isn’t long enough to endure that.
“I’ve talked to my colleagues on both sides of the aisle,” he said. “We’re going to continue to push the FDA to be safe, we want it to be safe, but also want to be reasonable, particularly when you have the situations we were talking about (in the hearing).
“When you have a promising drug that needs a four-year study, if you have three to four years to live, four years is a lifetime,” he said. “What we’re trying to do is allow people to make decisions.”
While Booth was nervous about her appearance, she appreciated the chance from Guthrie to share her story.
“Public speaking is not at the top of list of things to do,” she said. “It’s one of those things though that it’s such an honor.”
“I appreciate everybody that has taken the time to hear my story and share my story and to help raise awareness,” she said. “I’m really appreciative of that.”
Gina Clear can be reached at 270-505-1418 or firstname.lastname@example.org.