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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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Huntington's DiseaseHuntington's Disease
Degenerative disease
Progressive
Fatal (10 20 years)
Inherited (Autosomal Dominant)
Somewhat rare
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Huntington's DiseaseHuntington's Disease
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.