Huntington Disease

PREPARED BY : MUHAMMAD MUIZUDIN BIN KHALIZAKI

(A155427)

Introduction

Huntington disease is a chronic, progressive, heredity disease of the

N.S.

Result in:- progressive involuntary choreiform movement and

dementia.

The disease effect approximately 1 in 10 000 men and female

It is transmitted as an autosomal dominant genetic disorder

A child of a parent with Huntington disease has 50% risk of

inheriting the disorder

A genetic marker have been produced to detect the disease but there
is no hope for cure so far

Pathophysiology

It involve premature death of cells in the striatum (caudate and

putamen) of the basal ganglia

-region which involve in the control of movement

Cell also lost in the cortex, which associate with thinking, memory,
perception, and judgement.

Cerebellum which control the involuntary muscle activity

Clinical manifestation

The most prominent clinical feature of the disease are chorea

(abnormal involuntary movement), intellectual decline, and often
emotional disturbance

Speech become slurred, hesitant, often explosive, and unintelligible

Chewing and swallowing are difficult and there is a constant danger

of choking

Progressively patient will depend on wheelchair to move and soon

CRIB when have no bowel and bladder control.

Disease onset usually between 35 and 45y/o. although about 10% of

patient are children.

Patient succumb(death) 10-20 years to heart failure, pneumonia,

infection, falling and even choking

Assessment and diagnostic finding

Same as clinical manifestation

Management

No cure

Medication given to reduce chorea

-Thiothixene hydrochloride n Haloperidol deconate (block dopamine

receptor)
-levodopa (antiparkinson)
-anti depression drug (emotional disturbance)

Teaching patient self care

-manage symptom such as swallowing and poor bladder/bowel control

Refer patient occupational therapist, psychological, and audiologist

to tackle with patient problems

Nursing diagnosis
Alteration in swallowing
Outcome
-patient can consume food without difficulties
-aspiration will not occur
Intervention
-asses patient ability to swallow food, to figure out type of food is suitable
-place patient fawlers position during eating, for better food passage
-dont give large amount of food at a time to prevent choking
-give patient watery and soft diet, so patient can swallow easier
-ask doctor to apply ryles tybe for patient is needed.

Impaired thought processes; dementia / forgetfulness

Outcome
-Know / oriented towards people's time and place.
-Perform daily activities optimally.

Intervention
-Asses patient memory by asking his name, age and etc
- use a large clock, a calendar that has a large sheet of paper per day with.
-Encaurage patients to mention his name and closest family members
-Give the opportunity for clients to know where it is located.
-Give praise if the patient when the patient can answer correctly.
-Observation of the patient's ability to perform daily activities
-Help the patient to perform activities that is difficult for them
-Give praise if the patient can perform activities.
-Ask if the patient feel able to perform its activities .

Risk for injury from falls related to constant movement

Outcome
-Patient remains free of falls
-Patient remains free of injury related to constant movement
-Patient understands the rationale behind nursing interventions
Intervention
-determine risk for falling by using an assessment tool, and update frequently as clients condition
changes
-pad the sides and head of the bed
-pad furniture frequently used by client
-provide non-restrictive elbow/knee/wrist padding as needed to prevent client injury depending on
movement
-provide assistance with ambulation
-provide cues to encourage client to ask for assistance (call light within reach, bed and chair
alarms)
-provide observation and assistance with ADLs
-provide secure, padded protective devices on patients wheelchair

Reference

Smeltzer, Bare, Hinkle, Cheever, (2010) .Medical-Surgical Nursing . Lippincott

Williams and Wilkins

https://www.youtube.com/watch?v=DU_xkl-DO-Q