Huntington’s Disease and Patient Care

by James Rioux, Director of Classification and Treatment

On Wednesday, March 9th a multidisciplinary team of professionals at Bridgewater State Hospital took part in a thoughtful, educational and enlightening discussion about Huntington’s Disease (HD).  Allison Howland, who is employed by Massachusetts Partnership for Correctional Health (MPCH) as a Mental Health professional in one of the maximum security units of the hospital coordinated this discussion to facilitate education and awareness around this often unfamiliar disease.  She invited Jim Pollard, a national expert on HD, and well-known patient advocate to speak. Howland works directly with a patient who is afflicted with HD and mental illness.  She states that she was determined to gain more knowledge of the disease and educate staff on how to better assist her patient and other patients who may develop HD in the future.  Although Howland’s main role is to help patients understand the legal process, educate them about their mental illness, and help them to engage in treatment, she admits that managing someone with HD presents a set of unique challenges which require the support of correctional and medical staff.

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Mr. Pollard educated staff on the cause, symptoms, and stages of Huntington’s Disease.  According to the Huntington’s Disease Society of America, Huntington’s Disease (HD) 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 during their prime working years and has no cure. HD is known as the quintessential family disease because every child of a parent with HD has a 50/50 chance of carrying the faulty gene. Today, there are approximately 30,000 symptomatic Americans and more than 200,000 at-risk of inheriting the disease. The rate of disease progression and the age at onset vary from person to person. Adult-onset HD, with its disabling, uncontrolled movements, most often begins in middle age. Some individuals develop symptoms of HD when they are very young, even before the age of twenty.

During the discussion, Mr. Pollard played a pivotal role in providing staff with helpful suggestions on how best to interact with HD patients, improve the quality of their life, and allowed staff to participate in exercises that would simulate the struggles someone from HD experiences in order to give them a deeper more intimate understanding of disease’s progression.

Symptoms of Huntington’s Disease include changes in thinking, movement, and mood.  The order and progression of these symptoms are not always the same for all HD patients.  Some of the cognitive limitations include slower thinking, difficulty staying focused, and difficulty organizing information or answers.  Pollard recalls working with a patient named, Tommy who he had met after HD began to affect his speech and thinking.  “One evening he was reminiscing about his family with me.  He told me about his daughter’s childhood. He spoke one sentence at a time with extended pauses before he shared his next thought.  As tactfully as I tried to keep the conversation going, I sensed that Tommy was becoming increasingly exasperated with me.   Unnerving tension began to replace the warmth of his reminiscences.  He told me about his daughter’s first day of school which occurred decades ago. He paused.  I waited for his next thought.  I waited a bit longer but not long enough.  At the moment that he began speaking again, so did I.  As soon as he heard me speak, he stopped.”  Pollard states when he interrupted Tommy his brain essentially hit the ‘reset’ button and prevented him from resuming where he left off in their conversation, a common problem for patients living with HD.  Pollard adds that he still has difficulty waiting through silent pauses in conversations but realizes that listening and patience are your best attributes when caring for someone with HD.

Symptoms of HD are not limited to cognition.  The physical and movement elements of the disease include dystonia or weakness in facial muscles, difficulty changing posture, and impaired balance or depth perception to name a few.  Patients with HD commonly lean or slouch, their head may fall down to their chest, and they may exhibit bursts in movement or in voice.  Although it may be difficult to witness some of these physical movements, Pollard suggests that providers should allow the patient some freedom in determining the type and level of exercise they can tolerate.  Pollard believes that despite HD being a fatal disorder, providers should whenever possible strive to create the least restrictive environment, one that makes the patient feel part of a larger community and improves their overall quality of life.

The third component that is affected by Huntington’s Disease is mood.  HD patients often present with irritability, fatigue, and apathy.  These symptoms are also present in someone who is depressed.  Pollard explains, “They may appear disinterested, angry or resistant and actually be thinking and feeling very differently. It’s challenging for providers, despite our best efforts and wealth of experience, to look beyond their appearance and behavior, to see through this “disguise,” that the cognitive and physical features of HD present in the person”.

Understanding the conditions and challenges of each patient at Bridgewater State Hospital is a key component to positively and proactively managing the care of our community.  The staff at BSH is always learning more about the individual challenges both physical and mental that each individual patient faces on a daily basis.  Knowledge is critical to helping the population of BSH live a safe and well-managed life while at the hospital.  BSH would like to acknowledge Ms. Howland for her recognition of the need to educate not only herself, but the staff and BSH community to better understand HD, and for facilitating this discussion.   The Bridgewater State Hospital community would like to extend their sincere thanks to Mr. Pollard for sharing his experiences and knowledge about Huntington’s Disease and helping the staff more effectively meet the needs of its patient population.  If you would like to learn more about HD visit http://www.hdsa.org.

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