Huntington's Disease Clinic at University Hospitals Offers Needed Expertise
August 19, 2022
Multidisciplinary approach sets it apart
UH Clinical Update | August 2022
Diagnosing and managing patients with such a devastating and life-changing genetic condition as Huntington’s disease is a complex endeavor. It requires years of clinical neurological expertise with the disorder in order to distinguish the condition from those that mimic it, as well as leading-edge genetic testing and the involvement of social workers and psychiatrists to monitor symptoms and support patients in their “new normal.”
Such is the approach at the monthly Huntington’s Disease Clinic at University Hospitals. The multidisciplinary team at UH diagnosing and managing these patients includes movement disorders neurologist Steven Gunzler, MD, geneticist Suzanne DeBrosse, MD, and psychiatrist Rajeet Shrestha, MD, as well as social workers Beth Wachter and Lauren Somple. Patients being evaluated for Huntington’s disease or being managed for the condition come to one location and see all their scheduled providers on one day, easing their burden.
Multidisciplinary care: “We all have an opportunity to discuss the patient toward the end of the clinic,” Dr. Gunzler says. “So not only can we see the patient, we can then have an opportunity to discuss in person and formulate a plan.”
Expertise matters: Dr. Gunzler has been diagnosing and treating Huntington’s disease patients as a movement disorders neurologist at UH for 15 years, including the last 10 years in the Huntington’s Disease Clinic. He also recently completed data collection for an NIH-funded study aimed at discovering mitochondrial metabolomics biomarkers for Huntington's disease, gathering samples from patients with the disease and normal controls – a project done in collaboration with Xin Qi, PhD, from Case Western Reserve University.
“These are small molecules that may look different in somebody with Huntington's compared to somebody without Huntington's, but also may show whether the patient has active Huntington's disease as opposed to carrying the gene but not yet having active disease,” he says. “How long it takes for the person to develop symptoms is very difficult to predict, so there is interest in developing a test that will tell us that the person may develop Huntington's disease symptoms in the next several years. That prodromal stage of HD would be an opportune time to enroll the person in a neuroprotective study of a drug, to try to slow its progression. So there’s a lot of research focus on not only identifying people early, but maybe even identifying them before they even develop symptoms.”
Treatment options available at UH: Clinical trials to date have not identified a neuroprotective compound that successfully slows down the progression of Huntington’s disease, although research is ongoing, Dr. Gunzler says. However, he and his colleagues are able to offer their patients therapies to lessen their symptoms:
- Tetrabenazine or deutetrabenazine can be prescribed for symptoms of chorea.
- Physical, occupational and speech therapy can help with physical function, speech, swallowing and everyday tasks.
- Psychiatric medications and therapy can ease depression and anxiety.
All patients welcome: Dr. Gunzler says many of the patients seen in the UH Huntington’s Disease Clinic are those with or without family history of the condition, but who are experiencing symptoms such as the trademark abnormal movements, or chorea. However, the clinic is also fully equipped to manage patients with current Huntington’s disease, as well as those with a family history of the condition but no current symptoms.
For all these patients, sensitivity around the role of genetic testing is paramount.
“Psychiatry not only screens carefully for psychiatric symptoms, but also determines if the person is psychiatrically and behaviorally fit for testing, to see if the person can emotionally handle a positive or negative test result,” Dr. Gunzler says. “And then our geneticist provides very extensive pre-test counseling.”
Ensuring access in the wake of COVID: Like many other services at UH, the Huntington’s Disease Clinic has seen fewer patients since the pandemic. However, Dr. Gunzler and his colleagues are determined to reverse this trend and get the word to their fellow clinicians at UH about the value they can provide for Huntington’s disease patients.
“We’re concerned that people with Huntington's disease are not having the same access to care that they have had prior to COVID-19,” he says. “We know we can help with that.”