Lisa Pezik, RN BScN

Clinical Educator

A Review of Huntington’s Disease

Discuss the physiology and incidence.

Review signs and symptoms at all stages.

Discuss treatment options (pharm/non-pharm).

Objectives

A complex disorder

A genetic, progressive brain disorderParent has 50% chance of passing on the HD

geneAffects the basal ganglia

CaudatePutamenGlobus pallidus

Voluntary movementsEmotionLearningReasoning

Physiology

Appear between the ages of 30-50.Emotional

Depression IrritabilityAnxietyObsessiveness

Cognitive Inability to focus and planTrouble making decisions

PhysicalDifficulty

WalkingTalkingSwallowing

Onset and Symptoms

Death usually occurs 15-25 years after onset/diagnosis

Diagnosis:Blood test for HD gene Neurological testingPsychological testingReview of family history

Diagnosis of HD

Stages of Progression

Cindy was admitted into LTC three years year:Having frequent fallsLosing her mobilityUnable to cook meals for herself.Became suspicious of familySuspicious of meals on wheelsRefused visits from family MD

What stage of Progression of HD was Cindy three years ago?

Case Study: Cindy- 3 Years Ago

Cindy’s family is coming in for a care conference today.

The goals is to discuss treatment options for Cindy’s frequent aspiration pneumonias.

Cindy also frequently becomes anxious and refuses baths from unfamiliar staff.

Cindy refuses all programming as she doesn’t want to spend time with “old people.”

At times, Cindy’s spastic movements result in hitting staff. Her medications must be reviewed.

Case Study: Cindy-Now

What stage in the progression is Cindy now?

Case Study: Cindy- Now

Treatment: Not a one-stop-shop

No known cure at this time for HDIn 2011, Canadian researchers were able to

successfully reverse the physical symptoms of the disease in a mouse model.

Goals is symptom reduction and comfortInvoluntary movementsAnxietyDepressionObsessiveness

Treatment of HD

AntipsychoticsTreat the chorea and help with involuntary movementsWorsen symptoms and rigidity

HaldolRisperadolSeroquelClonazepamKlonipin

AntidepressantsStabilize mood and obsessivenessNausea, weight gain, low BP, drowsiness

CelexaZoloftDepacon

Pharmacological Treatment

Speech TherapyRecreation TherapyPhysical Therapy/Occupational TherapyVolunteers

Non-Pharmacological Treatment

Huntington’s Society of Canada. www.huntingtonsociety.ca

References