By Matthew Santamaria (firstname.lastname@example.org)
In 1971, Colleen Walsh-Barnes was at a gas station near her house. She would soon meet Ralph Walsh as Colleen helped him check her transmission fluid.
“We chatted and we flirted in the way that lust filled young people do,” said Colleen. “Ralph suggested I wait for him to finish his shift and we would go for a ride together. I pulled my car over and waited with great anticipation. I genuinely believed the universe was smiling down on me.”
By 1977, Ralph and Colleen were married with two children, Myles and Jason and another on the way, Nicole. She noticed that Ralph was developing some ticks including an eyebrow twitch and jerking legs.
“As he drove home from work down our narrow Paterson, NJ streets he was side swiping cars albeit doing very minimal damage,” said Colleen.
These are just a few of the symptoms of Huntington’s disease (HD), Ralph’s father died due to complications from HD at the age of 47-years-old. This is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person’s physical and mental abilities usually during their prime working years and has no cure.
However, Ralph began showing symptoms of HD in his mid-twenties.
HD is described as having ALS, Parkinson’s and Alzheimer’s diseases – simultaneously. Symptoms include personality changes, mood swings, depression, forgetfulness, impaired judgement, unsteady gait, involuntary movements, slurred speech, difficulty in swallowing, and significant weight loss.
One year later, their daughter Nicole was born. Ralph’s symptoms started to worsen. In order to to get to the bottom of what was going on with him, they went to New York-Presbyterian/Columbia University Medical Center
. This is one of the 50 HDSA Centers of Excellence that provide an elite team approach to HD care and research. Professionals have extensive experience working with families affected by HD and work together to provide families the best HD care program.
Ralph would then go through the testing process, which at that time consisted of a clinical evaluation. Ralph was diagnosed with having HD. Colleen remembers it like it was yesterday.
“On March 3, 1979 we drove to New York City to get the results of what had been almost a year long process,” Colleen explains. “The memories of that day are as clear to me as if it happened yesterday. It was a dark cold rainy day. As we entered the building’s lobby, I saw Robin, genetic counselor, come out of the stairwell door headed to the elevators in the lobby. She saw us and her face gave away Ralph’s diagnosis. My heart sank, I felt dizzy and nauseous, but I did not let on to Ralph. Perhaps he also saw her. I guess we decided to shield each other for our last fifteen minutes of a somewhat normal life.”
Years later, Ralph’s children would have the option to be genetically tested to know if they were HD positive. The decision to get genetically tested is difficult to make. Each year, 5-10% are tested. It is never the right or wrong decision to be tested. There are people that see no benefit in knowing that they will develop the disease while others want to know in order to make informed choices about their future. It can take up to several weeks to receive your results from the genetic testing center.
They tried to live a normal life despite the devastating news. When Ralph went back to work, his boss questioned him about his day off. Ralph told him the reason why and was let go due to safety concerns as a steelworker. Ralph received six weeks of disability pay as he was in the United Steelworkers Union.
“I had driven him to work that day,” said Colleen. “So, I piled the three kids back in the car and picked him up. He cried all the way home. By this time, my family knew the outcome of our appointment and loved ones called and offered support.”
Colleen’s family helped the family anyway they could as Colleen was the only one working in the family. She was a substitute van driver for the Wayne Board of Education.
“My husband’s great uncle by marriage brought us milk left over at the end of the week in the school cafeteria,” Colleen explains. “My family would come to check in on us and leave money under the sugar bowl. Pasta, PB&J and powdered iced tea mix were dietary foundations. Sunday dinners sent home with leftovers from Ralph’s mom and Nana gave us much needed nutrition.”
During this time, Ralph would apply for disability. Social Security Disability is a federal benefit program designed to provide long-term financial and medical insurance assistance. It is a work-based program that employees pay into the system in order to be eligible.
On HDSA Disability Chat, HDSA’s Manager of Disability Programs Allison Bartlett (email@example.com) talks in-depth about what the HD Community should know about Social Security Disability.
To watch HDSA Disability Chat, click here
“I was trying to explain to a cold-hearted woman what was wrong and what was going to happen,” said Colleen. “Ralph looked young and healthy and HD did not fit the boxes on the form. It took contacting Representative Phil Roe to get Ralph’s claim processed. It was eighteen months before we started to receive social security benefits.”
Ralph and Colleen wanted to find hope. They heard about an organization that could help and went to the New York City office. The organization was called the Committee to Combat Huntington’s Disease (CCHD). It was founded by Marjorie Guthrie in 1967 and would later become the Huntington’s Disease Society of America (HDSA)
Marjorie was the wife of iconic folk musician Woody Guthrie who died due to complications from HD. Before he died, she promised to Woody that she would devote her life to finding answers.
“I was told of a meeting of HD affected family members that would be taking place, they were known as the Kitchen Table group,” Collen explains. “It was mostly a group of middle aged and older people. I can still feel the tension in the room and shock on people’s faces when we said Ralph had been diagnosed with HD. They were all there concerned about their spouses and not their children. Turned out we were their worst nightmare: a person under 30-years-old, a agemate of their children or grandchildren who had active HD. How was it possible when the literature and medical books said (then as well as now) that HD struck in middle age. Even though both Ralph and I wanted to just disappear or escape we stayed. They were kind to us and shared information about a conference that was to take place in Philadelphia.”
One week later, she received a phone call from Fran Needles. Fran offered her comfort and told her that Colleen was what the organization needed.
“She encouraged me to return with Ralph to more meetings,” Colleen explains. “If not for her, I don’t know if we would have stayed involved. I felt so unsophisticated, ashamed of our poverty, ashamed to be 26-years-old with three kids and no degree or career, ashamed to have three kids at risk to HD. Ralph wanted to be involved he needed hope wherever it could be found. He needed me to drive him, so we went to Philadelphia.”
One of the presentations they attended was Nancy Wexler. She would later provide the genetic material that led to the discovery of the gene that causes HD.
“The Venezuela Project filled me with the hope that the gene would be found in the near future and a treatment would quickly follow,” said Colleen. “My overwhelming feeling was that my kids stood a chance to have a normal life.”
During that same presentation, Ralph and Colleen met Marjorie.
“She reached up took Ralph’s face in her hands,” Colleen explains. “He bent down to her and she kissed him on both cheeks. She brought with her such positive energy, she moved with exquisite grace and had a peaceful demeanor and I was awe struck. If she had managed and survived the horror of living with a spouse with HD, I could too.”
Colleen would later become the secretary for the New York Chapter.
“Being involved gave me purpose and gave Ralph hope,” said Colleen. “I still hold that decision to stay involved as one of the best decisions I made in my whole life.”
One year later, the couple moved to Andover, New Jersey. Even though they paid their rent, Ralph’s Uncle Frank (also landlord) told them to move because he could not handle Ralph with HD.
“Andover was a rural community and offered my children the distractions they needed to cope with their dad’s progression in HD,” Colleen explains. “They spent time in the woods, swam in our lake, rode their bikes with abandon and participated in town sports.”
The couple still had hope for a cure as Ralph would take any experimental drug available but there were never any improvements physically or mentally.
“With each hundred-mile round trip to Columbia Presbyterian, Ralph’s condition worsened, my hope dissipated, and my children became more fearful,” said Colleen. “Four years after pursuing every option, hoping for the best and trying desperately to create a normal life for our children, Ralph went to live in a nursing home.”
The last ride the family took together was to the Jersey Shore as Ralph loved the beach and ocean. She wanted Ralph to see the beautiful views of the beach and ocean one more time as he was now unable to walk.
“I will never forget the look of joy on his face as the wind blew his hair not the look of shame, pain and fear as he lost his bowels,” Colleen explains. “I found a way to clean him and change him that day, it was also the day that I accepted I could not take him out for our pleasant couple rides anymore.”
On April 16, 1987, Ralph died at the age of 37-years-old due to complications from HD.
“I have never once regretted meeting and marrying that extraordinary beautiful soul.”
Ralph’s and Colleens’ three children all elected to get tested for the HD gene in their mid-twenties. Myles and Jason tested HD positive and Nicole HD negative.
Jason elected not to have children and Myles’ son Kai was born using Pre-implantation Genetic Diagnosis (PGD) In Vitro Fertilization (IVF). PGD testing can be used with IVF that makes sure any fertilized egg implanted does not have the abnormal gene. For their family, HD will not be passed down.
Tragically, Myles died of HD in 2013 at the age of 39-years-old and Jason died in 2019 at the age of 45-years-old.
Huntington’s disease is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person’s physical and mental abilities usually during their prime working years and has no cure. Every child of a parent with HD has a 50/50 chance of inheriting the faulty gene that causes Huntington’s disease. Today, there are approximately 41,000 symptomatic Americans and 200,000 at-risk of inheriting the disease. In less than 10% of cases, juvenile Huntington’s disease (JHD) affects children & adolescents. JHD usually has a more rapid progression rate than adult onset HD; the earlier the onset, the faster JHD progresses. HD is described as having ALS, Parkinson’s and Alzheimer’s diseases – simultaneously. HD is characterized by a triad of symptoms, including progressive motor dysfunction, behavioral disturbance and cognitive decline.
The Huntington’s Disease Society of America is the premier nonprofit organization dedicated to improving the lives of everyone affected by HD. From community services and education to advocacy and research, HDSA is the world’s leader in providing help for today and hope for tomorrow for people with HD and their families.
To learn more about Huntington’s disease and the work of the Huntington’s Disease Society of America, visit www.HDSA.org or call 1(800)345-HDSA.
This is a story featuring a personal experience with Huntington’s disease. If you would like to have your story told please contact Matthew Santamaria at firstname.lastname@example.org