After seven months, new drug helping slow progress of ALS patient's decline
Rene Fogarty's voice is no more than a whisper these days.
The 52-year-old Turtle Creek man, first diagnosed with amyotrophic lateral sclerosis in May 2017, needs a walker to get around. There are stairlifts to get him between the floors of his home.
“I still feel pretty good,” Fogarty says to a visitor while he was receiving his Radicava medication intravenously. “But this drug does affect my balance.”
For now Fogarty — the first ALS patient in the United States given Radicava — will take the balance issues in exchange for the drug slowing down the disease that will eventually rob him of his ability to perform everyday tasks, like buttoning his shirt or clearing away the dinner dishes. It will kill him.
“You have to keep on living life,” Fogarty says. “I don't think about things I can't control.”
Fogarty began taking the newly approved drug in August .
“His disease has progressed. His breathing has stayed fairly stable, but he is steadily getting weaker in his muscles,” says Sandeep Rana, Fogarty's doctor and director of Allegheny Health Network's ALS Center. “Our goal is to slow down the progression. … Radicava has been well tolerated. But, it can cause some unsteadiness while walking. Some bruising too.”
But the medication has helped. Fogarty and his wife, Linda, were able to visit their native Chile, where they knew each other in high school. They later reconnected when both were living in the United States. They got married seven years ago.
“It took a lot of years for us to get together,” Linda says. “He was kind of nerdy, but we laugh a lot.”
Fogarty continues to work full time as a sales coordinator for the vacuum parts supplier Leybold USA Inc. in Export. He drives too.
“His right leg has not been affected,” Linda says.
Fogarty says he is experiencing some weakness in his left hand that had not been there before. He also goes to therapy to help with his breathing and swallowing. Because he has trouble with “thin” liquids, Linda makes his liquids thick.
“Nectar consistency,” she says.
Amyotrophic lateral sclerosis, or ALS, is a nervous system disease with no known cause that weakens muscles and impacts functions that many of us take for granted, such as breathing and swallowing.
ALS was first described in 1869 by French neurologist Jean-Martin Charcot. But the disease itself did not get national attention until 1939 when New York Yankees first baseman Lou Gehrig abruptly retired from baseball after being diagnosed with ALS.
Normally, motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body, according to als.org.
In ALS patients, the progressive degeneration of the motor neurons eventually leads to their death. When the motor neurons die, the brain's ability to initiate and control muscle movement is lost forever.
With voluntary muscle action progressively affected, patients in the later stages of the disease may become almost totally paralyzed, such as physicist Stephen Hawking, who died last month.
Fogarty says he knew something was unusual when he began experiencing the weakness in his legs while on a trip to New York City. When he came home to Pittsburgh, he went immediately to his primary care physician who then sent him to a neurologist. A myriad of tests were done and everything but ALS was ruled out.
“There are no tests to diagnose whether a person has ALS,” says Fogarty, who receives Radicava treatments daily for two weeks, then cycles off the treatments for two weeks. He'll repeat that routine for the rest of his life, Rana says. Each treatment costs $1,100, and the doctor estimated an annual cost of $145,000. His medication costs are covered by both the manufacturer and insurance.
The disease itself is rare. It typically strikes people between the ages of 40 and 70, and as many as 30,000 Americans have the disease at any given time. Besides Lou Gehrig, ALS has cut short the lives of Hall of Fame pitcher Jim “Catfish” Hunter, U.S. Senator Jacob Javits, actor David Niven, “Sesame Street” creator of Jon Stone, and composer Dimitri Shostakovich, to name a few.
For every 100,000 people, two or three people will develop ALS, Rana says. Slightly more men than women have ALS, he says. Life expectancy for an ALS patient after diagnosis is two to five years, he says.
While Fogarty has made changes in his lifestyle to accommodate the progression of his ALS, he is adamant he will not live like Stephen Hawking, who lost his ability to speak when he underwent an emergency tracheotomy in 1985 because of pneumonia.
“He chose to be kept alive,” Fogarty says. “I would never be like him.”
The Fogartys will attend the 2018 National ALS Advocacy Conference May 13-15 in Washington, D.C. They plan to meet as a group with members of Congress to talk about the need for additional ALS research funding.
Suzanne Elliott is a Tribune-Review staff writer. She can be reached at email@example.com, 412-871-2346, or Twitter @41Suzanne.