huntington's chorea syndrome classroom

8/8/2019 Huntington's Chorea Syndrome Classroom

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Huntington's DiseaseHuntington's Disease

13 Woody Guthrie Arlo Guthrie


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Concept Map: Selected Topics in Neurological Nursing

PATHOPHYSIOLOGY

Traumatic Brain Injury

Spinal Cord Injury

Specific Disease Entities:

Amyotropic Lateral Sclerosis

Multiple Sclerosis

Huntingtons DiseaseAlzheimers Disease

Huntingtons Disease

Myasthenia Gravis

Guillian-Barre Syndrome

Meningitis

Parkinsons Disease

PHARMACOLOGY

--Decrease ICP

--Disease /

Condition

Specific Meds

ASSESSMENTPhysical Assessment

Inspection

PalpationPercussion

Auscultation

ICP Monitoring

Neuro ChecksLab Monitoring

Care PlanningPlan for client adls,

Monitoring, med admin.,

Patient education, morebased

On Nursing Process:

A_D_P_I_E

Nursing Interventions & Evaluation

Execute the care plan, evaluate for

Efficacy, revise as necessary


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Degenerative disease

Progressive

Fatal (10 20 years)

Inherited (Autosomal Dominant)

Somewhat rare


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Gradual loss of

Motorcoordination and

Mental function


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Whats in a Name ?Whats in a Name ?

ChoreaChorea" comes from the Greek word for

"dance" and refers to the incessant quick, jerky,involuntary movements that are characteristic of

Huntingtons ChoreaHuntingtons Chorea


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Destroys neurons in areas of the brain involved

in the emotions, intellect, and movement

ATROPHY Brain Cell Death

PathophysiologyPathophysiology


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Which ExplainsWhich Explains

Jerking uncontrollable movement of the limbs,

trunk, and face (chorea)

Progressive loss of mental abilities

Development of psychiatric problems


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CharacteristicsC

haracteristics Usually develops at age 35 45 (+ or 17 years)

10% in children

Men and women equally

Younger people with Huntington's disease often

have more severe case, and symptoms may

progress more quickly


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Cause and Risk FactorsCause and Risk Factors

Having a parent with Huntington's is the risk factor. A child of

an affected parent has a 50% chance of inheriting the disease

The genetic mutation that occurs in gene IT-15, located on

chromosome 4, alters the huntington protein, which is present

in all human beings, and causes Huntington's disease. How

the mutation of gene IT-15 alters the function of the protein is

not well understood


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PrePre--Symptomatic TestingSymptomatic Testing

A positive test result can have profound,

unanticipated impacts on patients andtheir families


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PrePre--Symptomatic TestingSymptomatic Testing

Can be performed on adults, children, and even fetuses in thewomb. Genetic testing of a fetus holds special challenges and

risks, and some testing facilities choose not to do it

At-risk couples wanting to have children may choose toundergo in vitro fertilization with pre-implantation screening. Inthis procedure, embryos produced from the couple's spermand eggs are screened to identify one that is free of the HD

mutation, which is then implanted in the woman's uterus


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Types of SymptomsTypes of Symptoms

1. Movement

2. Cognitive

3. Psychiatric

* Chorea can become more intense when theperson is anxious or upset


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CognitiveCognitive

As Huntington's disease progresses, the ability to

concentrate becomes more difficult

May have difficulty driving, keeping track of things,

making decisions, answering questions, and may

lose the ability to recognize familiar objects.

Over time judgment, memory, and other cognitive

functions begin to deteriorate into dementia


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PsychiatricPsychiatric

Early psychiatric symptoms of Huntington's disease are

subtle, varied, and easily overlooked or misinterpreted

Depression is the most common psychiatric symptom

and often develops early in the course of the disease.

Signs of depression include:

- Hostility/irritability

- Inability to take pleasure in life (anhedonia)

- Lack of energy


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Continued

May exhibit psychotic behavior:

- Delusions

- Hallucinations

- Inappropriate behavior(eg unprovoked agression)

- Paranoia


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ComplicationsComplications

Lack of physical activity, dietary problems, and eating

and swallowing problems can cause constipation,

incontinence, and weight loss

Psychiatric and cognitive problems can lead to social

isolation and deep depression


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CT ScanCT Scan

Often shows shrinkage of the brain:

Actually lose about 30% of brain weight


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Genetic TestingGenetic Testing

Genetic testing may be required from a closely related affectedrelative, ideally a parent

This helps confirm the diagnosis and is important if the family'shistory is in any way unclear, uncertain, or unusual

Persons who test positive and are considering pregnancy areadvised to seek genetic counseling before they conceive


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TreatmentTreatment

There is NO CURENO CURE for Huntington's disease

Collaborative goals focus on:

- Reducing symptoms

- Preventing complications

-Providing support and assistance to the patientand significant others


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MedicationsMedications

Medications are available to help manage themanage the

signs and symptomssigns and symptoms of Huntington's disease, but

treatments can't prevent the physical and mental

decline associated with this condition


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MedicationMedicationAntipsychotics (hallucinations, delusions, violent outbursts):

haloperidol, chlorpromazine, olanzapine (contraindicatedif patient has dystonia)

Antidepressants (depression, obsessive-compulsive behavior):fluoxetine, sertraline hydrochloride, nortriptyline

Tranquilizers (anxiety, chorea): benzodiazepines, paroxetine,venlafaxin, beta-blockers

Mood-stabilizers (mania, bipolar disorder): lithium, valproate,carbamazepine

Botulinum toxin (dystonia, jaw clenching)


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NutritionNutrition

Some HC patients need a lot of time for meals becausethe loss of coordinated movement makes it difficult forthem to swallow or feed themselves

Minimize Risk ofChoking

- Cut food into small pieces, softened, or pureed to makeswallowing easier

- Swallowing therapy can help if started before there is seriousdifficulty

- Avoid dairy products because they tend to increase the secretionof mucus, which can increase the risk for choking


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Continued

Important to consume enough calories to maintainadequate body weight

- Number of daily meals may have to be increased

- Vitamins and nutritional supplements recommended- If eating and dietary problems become severe, may needfeeding tube

Requires large quantities offluids (especially during hotweather to avoid dehydration)

- Bendable straws make drinking easier

- Liquids may have to be thickened with additives to theconsistency of syrup before drinking is possible


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Physical ActivityPhysical Activity

Should walk as much as possible, even if assistance is necessary

Daily exercise promotes physical and mental well-being

Falls are always a risk, keep surroundings free of hard, sharpobjects

Wearing special padding during walks helps protect against injuryfrom falls

Small weights worn around the ankles and sturdy, well-fitting shoesthat slip on and off easily can improve a patient's stability


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Social ActivitySocial Activity

Unless and until the disease's progression prohibits it,

should participate in outside activities, socialize, and

pursue hobbies and interests

These activities also give family members and

caregivers valuable time for themselves


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PrognosisPrognosis

The bedridden patient in the final stages of Huntington's

disease often dies from complications such as heart

failure or pneumonia

Juvenile Huntington's disease (16%) runs it course

comparatively fast, with death typically occurring in about

10 years


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Affairs in OrderAffairs in Order

While it may be emotionally difficult, it is important for patients andcaregivers to make informed, carefully considered decisionsregarding the future while the patient is capable of making his or hercontribution to a planned course of action

Patients and their family members should discuss and considerissues such as legal concerns, home care, assisted care, andinstitutionalization

Draw up wills and other important documents as early as possible toavoid legal problems later on, when the patient may be unable torepresent his or her own interests

Legal assistance may be necessary if the patient encountersdiscrimination over insurance or employment.

huntington's chorea syndrome classroom

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