What Is Huntington'S Disease?
What Is Huntington'S Disease?
What Is Huntington'S Disease?
TRANSMISSION
HAPPYLETSJ
Basal ganglia: brain in control of movements DIAGNOSTICS
Cortex: thinking, memory, perception, judgement
- Diagnosis is based on clinical manifestations and family history
Cerebellum: coordination, voluntary muscle movements 1. Genetic marker
- Identified through use of recombinant DNA technology
CLINICAL MANIFESTATIONS
- Confirms the diagnosis
1. Chorea/choreiform movements 2. Genetic testing
- Abnormal involuntary twitching movements - Available through both blood and amniotic fluid
- Choreae: Greek word meaning dance - Can be on fetal cells by amniocentesis or chorionic biopsy
- Begins at the face and arms then the involvement of the entire - Test if a carrier or not
body with facial grimace, tongue protrusion, jerky movements of arms 3. Pre symptomatic testing
and legs and arrhythmic lurching gait resembling the face - No test is available to predict when symptoms will develop
2. Constant writhing, twisting of face and movement involving the speech, - Asymptomatic and (+) in family genetics = dilemma on testing or not
uncontrollable movement involving entire body
3. Chewing and swallowing is difficult, patient become emaciated & THERAPEUTICS
exhausted then cachexia
- May cause aspiration and nutrition problem - No treatment stops or reverses the process, but there are medications
4. Gait deteriorates and ambulation becomes impossible to control chorea (symptomatic)
5. Intellectual decline, emotional disturbance and problems with Pharmacologic management
concentration, emotional ability, and psychotic behavior
- Most devastating 1. Thiothixene HCl (Navane) , phenothiazines and haloperidol decanoate
6. Bowel and bladder control is lost (Haldol)
7. Death may occur 10-20 years after the onset of symptoms and occurs - Blocks dopamine receptors improving dyskinesia
due to medical problems associated with HD such as infection, choking 2. Riluzole (Rilutek)
or aspiration pneumonia
8. Speech becomes slurred, hesitant, often explosive - Slow down worsening of disease and prolong survival
9. Cognitive function is affected with dementia - Reduces chorea
10. Personality changes 3. Antiparkinson medication (levodopa)
- Nervousness, irritability and - For hypokinetic motor impairment
impatient behavior - Amino acid precursor of dopamine
11. Judgement and memory are impaired
- Most effective and commonly used drug
12. Hallucinations, delusions and paranoid thinking maybe present
13. Mental and emotional changes that occur maybe more devastating
- Given with combination of carbidopa
o Inhibitor of L amino acid, decarboxylase, plasma
to the patient and family than the abnormal movements
enzyme that metabolizes levodopa proliferate
HAPPYLETSJ
4. Antipsychotic: phenothiazines and butyrophenones - Pad the sides and head of the bed, use padded heel and elbow
- AKA neuroleptics protectors
- Manage psychosis including delusions, hallucinations, paranoia, o To ensure that patient can see over the side of the
disordered thoughts bed
5. Antidepressant medications: fluoxetine (Prozac); paroxetine, Paxil, - Keep skin meticulously clean and apply emollient cleansing
agent and lotions frequently
Sertraline (Zoloft)
o Use soft sheets and beddings
o Football padding and other forms of padding
NURSING MANAGEMENT
- Encourage ambulation with assistance to maintain muscle tone
1. Program combining medical, nursing, psychological, social, occupational, o Secure patients only when necessary in bed or with
speech and physical rehabilitation services and palliative care needed padded protective devices
to help the patient cope o Making sure that its loosened frewuently
2. Re motivation and Milieu Therapy
- improve physical, mental and social functioning 2. Imbalance Nutrition: Less than body requirements due to
3. Family education about the condition and medications inadequate intake & dehydration resulting from swallowing or
4. Teaching plan for managing chorea, swallowing problems, limitation in chewing disorders and dangers of choking and aspiration
ambulation and loss of bowel and bladder function
5. Independent ambulation is encourage as possible
- Give phenothiazines as prescribed before meal to calm patient
- wheelchair may become necessary who has difficulty ambuliting - Elevate the head of the bed/ use of pillow
6. Genetic counseling - Talk to patient before mealtime to promote relaxation, show the food
and enumerate the food whether it is hot or cold
- Patient of childbearing age may seek information about
transmitting the disease - Do not interpret stiffness, turning away or sudden turning of the head
7. Consultation to a speech therapist as rejection (chorea) so just continue feeding
- Alternative communication strategies - Increase calorie requirements and avoid milk because it can increase
8. High calorie, high protein diet mucus production
- Prevent weight loss from constant involuntary movement - Use long-handled spoon (ice-tea spoon) and place on the middle of the
9. Support group tongue and put slight pressure
- Huntington’s Disease Foundation of America - Place bite-sized food between patient’s teeth, serve stews, casseroles,
thick liquids and avoid too many milk drinks
NURSING DIAGNOSIS - Give between-meal feedings
- Develop skills in Heimlich maneuver in the event of choking
6. Risk for Injury from falls and possible skin breakdown from constant
movement 3. Disturbed thought process and impaired social interaction
- Have clock, calendar and wall posters in view for orientation
HAPPYLETSJ
- Interact in a creative manner and do only one to one contact
- Don’t abandon a patient because the disease is eventually terminal
o patients are living until the end
- Continue therapeutic techniques even client may not be responsive
- Refer patients to Huntington’s Disease Foundation
NOTABLE SUFFERER (pake ko ani nila? Lol pero gitype lang gyapon)
1. Woody Guthrie
- Journalist, physician
- Died at 55
2. Olivia Wilde
- American actress and film maker
3. Marianna Palka
- Celebrity actress, screen writer, producer, director
4. Trey Grey
- Drummer
5. Charles sabine
- 2 decades: television reporter in MBC covering wars, revolution and
natural disasters
- Also his brother john with cognitive
- From father and uncle
HAPPYLETSJ
- mathematical determination of the size or number of something
NCM 116: MEDICAL SURGICAL NURISNG - an assessment of the risks, possibilities, or effects of a particular
ALZHEIMERS DISEASE situation
- includes solving mathematical problems
- comparing assessment of situations to promote safety and determining
possible results in life situations
WHAT IS ALZHEIMERS DISEASE? - important in our engagement with the world
4. visual-spatial perception
- Neurodegenerative disorder marked by Marked by cognitive and - ability to recognize end objects physical location as well as the physical
behavioral impairment that significantly interferes with social and relationship between objects
occupational functioning of our clients - ex: patient with Alzheimer's disease will question the presence of the
- an irreversible, progressive brain disease that slowly destroys memory computer mouse place beside the laptop
and thinking skills and eventually even ability to carry out simplest task o client can no longer associate the role of the mouse in the easy
- In most people with Alzheimer’s, symptoms first appear after age 60 usage of the computer
- 1-10% have onset in middle age 5. Judgment
- most common cause of dementia among older people
- Ability to make considerable decisions or come to sensible conclusions
- Dementia involves progressive decline in cognition
o Loss of cognitive functioning, thinking, remembering and
6. Abstraction behavior
reasoning
o Interferes with daily life and activities - Ability of a person to function to adapt behavior to the transformation
- Degenerative disorder of cerebral cortex especially the frontal lobe of the environment
o Frontal: more than half of cases of dementia - environment may contain certain abstract notions such as loudness,
- Primary form of dementia stretching, a sound in time
o Prognosis is poor!! - those who have problems in abstraction behavior cannot adapt to a
loud sound during party in the house because of the loss of association
that there is a need to transform an environment to fit an event or
AREAS OF COGNITION activity such as a party
ETIOLOGY
PATHOLOGIC CHANGES
- Unknown
o Knowledge of hereditary links are growing
- Destruction of brain cells
- Pathologic changes occur 5-20 years before evidence of symptoms
- Genetic component
RISK FACTORS
Primary Caregiver
- ensuring appropriate care for pt with dementia
- meeting all the health and personal needs of the person with dementia
- often a family member
- often neglect their own care
- many caregivers report high levels of stress
- 40% suffer a form of depression
*To care for the primary caregiver, look for signs of caregiver burnout, treating
medical problems, and referring to support services
ETIOLOGY
PARKINSON’S DISEASE
a. Viral
b. Chemical toxicity
- It is a progressive neurologic disorder resulting from
c. Cerebrovascular disease
degeneration of basal ganglia in the cerebrum.
d. Effects of drugs such as major tranquilizers and
- 4th most common neuro-degenerative disease
reserpine
- Men > Women
The degenerative or idiopathic form – most common
- Symptoms usually appear during 50s
Secondary form with a known or suspected cause
- Cases have been diagnosed as early as 30 years of age
- It is a brain disorder that leads to shaking, stiffness and difficulty Several causative factors (acc. to research):
with walking, balance and coordination.
- Genetics
- Parkinson’s symptoms began gradually & gets worse with time.
- Atherosclerosis
- As the disease progresses people may have difficulty walking
- Excessive accumulation of oxygen free radical
and talking.
PATHOPHYSIOLOGY
When PD occurs, degenerative changes are found in the area of
the brain known as: Substantia nigra, which produces DOPAMINE - Associated with decrease level of dopamine resulting from
80% appear disruption of pigmented neuronal cells in the substantia
nigra in basal ganglia region of the brain
Dopamine is a chemical substance that enables to move normally
- Depletion of dopamine --- Diminishes normal NM inhibiting
and smoothly.
mechanisms ---- neurologic deficit
The loss of dopamine stores in this area of the brain results in more
excitatory neurotransmitters than inhibitory neurotransmitters
leading to imbalance that affects voluntary movement.
CLINICAL MANIFESTATIONS Others
- Flexed posture of the next, trunk and ,limbs - Reducing excessive influence of excitatory
- The patient stands with the head bent forward and - Acting on other neurotransmitter pathways
- Levodopa (Dopar, Larodopa) is the most effective
walks with a propulsive gait.
6. Freezing Movement agent and the mainstay of treatment. Always given in
combination form with Carbidopa to avoid Monoamine Oxidase Inhibitors (MAO Inhibitors)
metabolism of levodopa before it can reach the brain o Drugs used to treat depression by inhibiting
2. Anticholinergic Therapy monoamine oxidase and allow neurotransmitter to
- Biperiden, and Benztropine mesylate) remain in the brain
- Effective in controlling tremor and rigidity Catechol-O-methyltransferase (COMT) Inhibitors
- Drugs that oopose the effects of acetylcholine at o Act by extending the duration of action of levodopa
receptor sites in the substantia nigra and corpus thus, improving the amount of time a patient can
striatum thus, helping to restore chemical balance experience from the benefits of levodopa
3. Antiviral Therapy Antidepressants
- Amantadine hydrochloride (Symmetrel)
Note:
- Helpful in levodopa – induced dyskinesia
- Antihistamine agents - Promote measures to maintain adequate airway
i. an antiviral agent used in early Parkinson’s - Provide semisolid diet
treatment to reduce rigidity, tremor and - Maximize functional abilities
bradykinesia.
SURGICAL MANAGEMENT
ii. It releases dopamine from neuronal storage
site Intractable tremor or diskinetic movement may be ameliorated
SIGNS ND SYMPTOMS
- Cervical discs are the cushions between the vertebrae in the
1. Pain above the heel upper back and neck
2. Snapping or popping sound upon rupture - Herniation of the disc occurs when the gelatinous inner disc
material, the nucleus pulpous, ruptures, or herniates, through
3. Difficulty flexing or extending the foot
the outer cervical disc wall
CAUSATIVE AGENT Surgical Treatment
1. Excessive stress being placed on the disc from lifting heavy 1. If pain from a cervical disc herniation lasts longer than 6 to 12
objects or other damaging motions weeks, or if the pain and disability are severe, spine surgery may
be a reasonable option
SIGNS AND SYMPTOMS 2. Anterior Cervical Discectomy and Fusion (ACDF)
1. Pain that radiates down the arm to the hand or fingers 3. Cervical Spinal Fusion
2. Numbness or tingling in the shoulder, arm, or hands
NURSING MANAGEMENT
3. Weakness of hand and or arms (upper extremities)
4. Paresthesias 1. Bed rest on a firm mattress
5. Certain positions or movements of the neck can intensify the pain 2. Heat or ice massage on the affected area
6. Often resemble other disorders such as carpal tunnel syndrome,
3. Cervical collar or cervical traction
problems with the rotator cuff, and gout
4. Educate client regarding lifestyle changes (smoking cessation,
A cervical herniated disc can cause spinal cord compression. increase
Symptoms include:
5. activity and weight loss)
7. Awkward or stumbling gait 6. Provide instructions regarding back anatomy and physiology.
8. Difficulty with fine motor skills in the hands and arms
7. Teach patient about proper body mechanics.
9. Tingling or "shock" type feelings down the torso or into the legs
8. Encourage good nutrition and proper rest to reduce risk of
MEDICAL MANAGEMENT recurrence.
Non-surgica lTreatment
● De enerative disk disease is the term used to re er to symptoms o pain caused by wear-and-tear
on a spinal disc.
● ‘It pertains to the presence o osteophytes, loss o disc hei ht and reduced si nal intensity on MRI.
Causes
● Drying out of the disc. When we are born, the disc is about 80 percent water. As we a e, the disc
dries out and doesn't absorb shocks as well.
● Crack. Everyday movements and minor injuries can be sources o stress which can cause tiny tears
in the outer wall, which contains nerves. Any tear near the nerves can become pain ul.
● Inflammation. As the proteins in the disc space irritate the surroundin nerves—both the small
nerve within the disc space and potentially the lar er nerves that o to the le s (the sciatic nerve).
● Abnormal micromotion instability. When the outer rin s o the disc, called the annulus fibrosus, are
worn down and cannot absorb stress on the spine e ectively, resultin in movement alon the
vertebral se ment.
Si ns and symptoms.
I. Nonoperative Therapy
● Back education and back school
● Exercise
● Medications: muscle relaxants, nonsteroidal anti-inflammatory dru s (NSAIDs), and anal esics
● Physical modalities: ice packs, heatin pads, electrical stimulation, phonophoresis, iontophoresis,
relaxation, and bio eedback.
Lumbar discectomy - a sur ery to remove a herniated or de enerative disc in the lower spine. The
incision is made posterior, throu h the back muscles, to remove the disc pressin on the nerve.
Discectomy may be recommended i physical therapy or medication ails to relieve le or back pain or i
you have si ns o nerve dama e, such as weakness or loss o eelin in the le s.
Lumbar Spinal Fusion Surgery – this is the standard sur ical treatment or lumbar de enerative disc
disease. In this procedure, two vertebrae are ra ted to ether. The oal o usion sur ery is to reduce
pain by eliminatin motion at the spinal se ment.
Total disc arthroplasty (replacement) – an alternative to lumbar usion and has been used or lumbar
disko enic pain, with and without radicular symptoms. The main objective o an artificial disk is to
replace a pain ul disk while maintainin the natural anatomic structure o the spine.
● Cervical Procedures
Anterior cervical discectomy - involves per ormin a decompression o the nerve roots throu h an
anterior discectomy.
Anterior cervical discectomy and fusion – a ter the discectomy is per ormed throu h the ront o the
neck, a bone ra t is implanted in the space where the disc was removed. Hardware, such as a ca e or
plate, is also installed to hold the adjacent vertebrae in place and help acilitate the bone ra t to row
and eventually use these two vertebrae into one solid bone.
Cervical Artificial disc replacement - the disc is removed the same as in ACDF, but ADR replaces the
dama ed disc with an artificial disc to maintain more o the normal spinal motions rather than settin
up a solid usion.
● Assess vital si ns
● Use pain scale to assess the patient’s pain
● Administer prescribed medications
● Provide rest, heat or cold therapies.
● Educate the patient about appropriate exercises, proper body mechanics, and wei ht control.