Not all family traditions are positive; inherited conditions like Huntington’s disease can devastate generations.
Named after the doctor who discovered it in the late 19th century, Huntington’s disease is a fatal disorder that causes the progressive breakdown of nerve cells in the brain, according to James Duffy, MD, a neuropsychiatrist with the Chase Family Movement Disorders Center, part of the Hartford HealthCare Ayer Neuroscience Institute.
“It’s what we call an autosomal dominant disorder. You only need one gene from one parent to inherit the disease,” Dr. Duffy explained.
As Huntington’s causes nerve cells in the brain to deteriorate, it can affect the individual’s motor movement, leaving some writhing, and target fine motor skills, cognition, impulse control and metabolism. People with Huntington’s disease, as a result, have a higher risk of diabetes, depression and severe shifts in their personality.
“The disease affects every aspect of who we are – how we relate to others socially, how we maintain relationships and how we stay in jobs,” Dr. Duffy said.
He works on the team at Chase Family Movement Disorders Center to help unravel the disease and its impact on patients and their families, who are greatly affected by the changes. The team approach – which includes a neurologist, neuropsychiatrist, neuropsychologist and social worker – focuses on minimizing the disease’s effect on the person’s life, while treating the movement disruptions.
“It takes a team to help these families,” Dr. Duffy admitted.
Typically, symptoms of Huntington’s develop in a person’s 50s or 60s, depending on the degree of genetic mutation. Genetic counseling can help pinpoint the disease, but he said it should only be done after a patient has a care team lined up for much-needed support.
“Families have been carrying this torment around for generations. Part of our job is to reassure them that we will treat them with compassion. Let’s do all we can to slow it down and, hopefully, something will come out to stop its progression,” Dr. Duffy said.
Treatment for people with Huntington’s addresses the movement disorder, depression and some impulse control issues. Lifestyle changes like regular mild exercise, good sleep habits, healthy diet and eliminating sugar and alcohol can also help combat the disease, slowing its onset and progression.
“The gene is your potential but not necessarily your ultimate destiny,” Dr. Duffy noted. “It’s like any gene – you have this vulnerability, therefore, you do everything you can to minimize the effects of the gene.”