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Huntington’s Disease

Huntington’s Disease is a progressive neurological disorder. The disease is genetic, has no cure, and is fatal.

  -Age of onset is usually between age 30 and 50.

  -Life expectancy after onset is 10 to 25 years, with death usually occurring due to a complication such as heart failure.

  -Everyone who has a parent with Huntington’s Disease has a 50/50 chance of inheriting the genetic mutation that causes the disease and everyone who inherits the gene will develop the disease.

Musician Woody Guthrie passed away from complications of Huntington’s Disease in 1967. His wife Marjorie Guthrie spearheaded the movement to research for a cure for Huntington’s Disease.

Characteristics of Huntington’s Disease

According to the Huntington’s Disease Society of America, Huntington’s Disease can be described as having ALS, Parkinson’s Disease, and Alzheimer’s Disease, all at the same time. The following chart provides an overview of the primary characteristics of Huntington’s Disease.

Motor Control Symptoms Cognitive Symptoms Psychiatric Symptoms
Chorea – abnormal involuntary movements Loss of executive functioning Depression
Dystonia – involuntary muscle contractions Difficulty with multi-tasking Anhedonia – loss of sexual libedo
Motor impersistence – the inability to maintain postures or positions without prompting Rigid, inflexible thinking Suicidal ideation
Dysarthria – difficulty controlling the muscles used for speech Poor concentration, easily distracted Social isolation
Dysphagia – difficulty swallowing Impaired visual-spatial abilities Disrupted sleep patterns
Poor posture Impaired impulse control Delusions
Incoordination Diminished vocabulary Hallucinations
Delayed initiation of movement Poor insight Paranoia
Impaired balance and gait Short term memory loss Irritability

Implications for Occupational Therapy Treatment

People with advancing Huntington’s Disease have difficulty completing any functional activities independently. The involuntary muscle spasms make it hard for patients to do much with their hands. Patients also have difficulty maintaining healthy body weight due to unknown metabolic issues as well as difficulty chewing, swallowing, and handling utensils.

This video includes an interview between an occupational therapy student and a neurologist regarding occupational therapy intervention for people with Huntington’s Disease, as well as a simulated patient evaluation and training in the use of adaptations to compensate for impaired cognitive skills.

Task Interventions Example
Eating -Work with speech therapy to modify food and liquid textures to increase ease of swallowing
-Provide adaptive dishes and utensils to encourage self feeding for as long as possible.
-Encourage a modified eating environment with minimal distractions.
An occupational therapist help a man with Huntington’s Disease to feed himself by training him in the use of a plate guard, a swivel spoon and a non-skid placemat.
Bathing and Hygiene -Provide grab rails and adaptive seating for toileting and bathing.
-Make sure that adaptive seating is of a type that will not cause injury if a patient has sudden total body extensor tone.
An occupational therapist recommends a tilt in space shower chair for a nursing home patient with Huntington’s Disease who repeatedly slides out of a regular shower chair due to extensor tone.
Dressing -Recommend loose fitting clothing with elastic waistbands and front closures, as well as supportive, low heeled shoes.
-Provide zipper pulls for jackets and coats.
-Recommend that clothing be laid out for patients who cannot organize and initiate dressing.

A woman with Huntington’s Disease wants to continue to wear her favorite pair of jeans but she cannot button the waistband. The occupational therapist adapts the closure with an elastic tab to convert the waistband to pull-on style.
Grooming -Recommend adaptive grooming devices, such as electric razors or beard trimmers, electric toothbrushes.
-Recommend using salon services for hair styling, manicures and pedicures.
An occupational therapist recommends an electric toothbrush to a man with Huntington’s Disease who cannot hold on to a manual toothbrush to brush his teeth.
Household management -Provide adaptive equipment and assistance with kitchen reorganization to improve safety during meal preparation.
-Recommend pre-packaged microwavable meals.
-Clearly label cupboards and closets.
-Help patients locate assistance with tasks that they can no longer complete, such as shopping, heavy cleaning, financial management, etc.
An occupational therapist labels the cupboards and drawers in the kitchen of a woman with Huntington’s Disease so that she can remember where food, dishes and utensils are located.
Mobility -Assist patients in removing safety hazards from living areas, such as throw rugs and small, unnecessary pieces of furniture.
-Train patients in methods of carrying items to keep hands free while walking so that hands may be used to stabilize on grab rails or furniture. Using a shoulder bag is an example.
-Recommend methods of emergency communication in the event of a fall, such as carrying a cell phone or wearing an emergency communication button.
An occupational therapist recommends that a man with Huntington’s Disease carry his cell phone in his shirt pocket at all times so that he can call his neighbor for help if he falls.
Cognitive Skills -Teach patients and their caregivers how to use planners, calendars, alerts on electronic devices, and similar memory aids.

-Break down tasks into smaller steps.

-Help patients to establish a daily routine and to stick to that routine.

-Ask direct questions and offer specific choices rather than asking open ended questions.

-Give patients time to respond to questions or instructions as response time can be delayed.
An occupational therapist assists the caregiver of a woman with Huntington’s Disease in setting up a calendar and alert system on the woman’s computer to help her remember her daily routine.
Psychosocial Issues -Educate the patient, family and caregivers in how to keep the patient’s environment calm and consistent.
-Discuss stressors that can trigger frustration, irritability, or outbursts and help patients and caregivers to avoid those situations.
-Avoid confrontational situations with patients. Instead, attempt to redirect patients to other topics or activities.
An occupational therapist provides treatment to her patient with Huntington’s Disease in the later afternoon when the therapy clinic is quiet.

References

Pedretti, L. W., & Early, M. B. (Eds.). (2001). Occupational therapy: Practice skills for physical dysfunction (pp. 3-12). London: Mosby.

“Huntington Disease.” Genetics Home Reference. N.p., n.d. Web. Retrieved from http://ghr.nlm.nih.gov/condition/huntington-disease on February 26, 2018

Cook, C., et. al. (2012). Occupational Therapy for People with Huntington’s Disease: Best Practice Guidelines. European Huntington’s Disease Network. Retrieved from file:///C:/Users/meyda/AppData/Local/Packages/Microsoft.MicrosoftEdge_8wekyb3d8bbwe/TempState/Downloads/Occupational%20Therapy%20for%20people%20with%20Huntingtons’s%20Disease%20best%20practice%20guideline%20(2).pdf on March 10, 2018.

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