Imagine what a world without Huntington’s disease (HD) would look like if a cure were found. It would be a world free from the suffering caused by symptoms like chorea and cognitive, emotional, and behavioral changes. There would be no more swallowing difficulties, weight loss, or pneumonia attributed to the disease. Genetic counseling and testing wouldn’t be required. There would be no need for specialized medicine, equipment, or interventions. Huntington’s disease has affected several generations of my family, taking the lives of my dad, granddad, and great-grandmother. While I tested negat ..read more

The U.S. Food and Drug Administration (FDA) has approved Neurocrine Biosciences’ oral granules formulation of Ingrezza (valbenazine) — called Ingrezza Sprinkle — for treating chorea, or involuntary movements, in adults with Huntington’s disease. The new formulation, taken once per day, is designed to provide an easier administration option for patients experiencing swallowing issues due to chorea. A previous survey showed that 62% of 78 Huntington’s patients with chorea and their caregivers reported such difficulties, according to a press release from Neurocrine announcing the therapy’s ..read more

Jennifer Ushe’s grandparents, Jean and Edward Moshier. (Photo courtesy of Jennifer Ushe) This is Jennifer Ushe’s family story: Both of my grandparents died 10 years before I was born: Grandpa, unexpectedly and tragically, of a heart attack, and Grandma, expectedly but no less tragically, of Huntington’s disease. My Aunt Kathy tears up as she recounts the day Grandpa died. She and her siblings expected Grandma to go first. When Grandpa died, on Jan. 2, 1974, he left behind six children, including my dad. Grandma had spent two years at the Hudson River State Hospital in New York, which offe ..read more

May is Huntington’s Disease Awareness Month, and this year the focus is on the patient experience. Huntington’s disease (HD) is a neurodegenerative disorder thought to affect 4.9 per 100,000 people worldwide, including about 41,000 U.S. residents, and can impact many aspects of everyday life. While each patient’s journey is different, the progressive disease is characterized by symptoms including involuntary jerking known as chorea, walking problems, and issues with speech and swallowing. People with HD may experience cognitive impairment and have difficulties in planning, thinking, and ..read more

As my wife, Jill, and I recently made our way through Ronald Reagan Washington National Airport in Arlington, Virginia, I saw her glance longingly at a Starbucks we passed on the way to our gate. We were headed to Boston to visit family but were running a little late. However, a flight delay enabled me to return to the coffee store to pick up some peach tea that brings Jill much joy. Thanks to mobile ordering, I don’t have to worry about getting her the wrong thing. Traveling is stressful and has become more difficult for Jill over time. Despite the progression of her Huntington’s disease sym ..read more

Some of my closest friends are musicians who love the work of Bob Dylan. During a recent conversation, Dylan’s visit with ailing singer-songwriter Woody Guthrie came up. My friends hadn’t realized that Guthrie had Huntington’s disease — which they knew as the same disease that’s affected several generations of my family. (I recently tested negative for Huntington’s, thankfully.) As a young man, Dylan read Guthrie’s 1943 autobiography, “Bound for Glory.” The older singer behind “This Land Is Your Land” was born on July 14, 1912, in Okemah, Oklahoma, and grew up to become Dylan’s idol and inspi ..read more

Daily treatment with Ingrezza (valbenazine) led to sustained reductions in chorea — characterized by involuntary, unpredictable body movements — for nine months among the small group of Huntington’s disease patients simultaneously using antipsychotic medications in the Phase 3 KINECT-HD2 trial. In addition, improvements in chorea were also sustained for up to a year of the oral therapy, developed by Neurocrine Biosciences, among the overall trial population, as previously reported. Those were the findings presented by the study’s principal investigator, Erin Furr-Stimming, MD, of the Universi ..read more

Ah, spring has finally sprung. Plants are blooming, trees are budding, and the weather is warmer. As a result, my wife, Jill, and I recently decided to take advantage of the beauty exploding around us, along with the good weather, by spending the day in the nation’s capital, close to where we live. Under a cloudless sky, we walked down Washington D.C.’s Pennsylvania Avenue and were surrounded by throngs of tourists. Among them, we saw a middle-aged woman slowly pushing an elderly man in a wheelchair. “Carlos,” Jill said. “Do men talk about caregiving like women do?” Her question struck a chor ..read more

Psychosis causes a person to lose a degree of contact with reality. According to the National Health Service, three main symptoms are linked to a psychotic episode: hallucinations, delusions, and confused and disturbed thoughts. As a 2019 Huntington’s Disease News article noted, “Psychosis is defined as experiencing hallucinations — things that are not really happening — and delusions, or believing things that are demonstrably untrue. Psychiatric symptoms are estimated to affect up to 11% of people with Huntington’s.” Alcohol and drug abuse can also trigger psychosis, and people with psy ..read more

A type of brain nerve cells called layer 5a pyramidal neurons — especially those projecting into the striatum, a brain region greatly affected in Huntington’s disease — are lost early in the course of Huntington’s, a study found. Excessive CAG repeats in the HTT gene, the cause of Huntington’s, were detected in these vulnerable neurons, as well as neighboring neurons that remained resilient, or alive, during these early phases of the disease. This, along with findings of altered neuronal communication in layer 5a pyramidal neurons, suggests that both CAG repeat expansion and changes to how th ..read more