What Is Huntington'S Disease?

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o Gene mutilations in chromosome 4 = genetic disorders and

NCM116: MEDICAL SURGICAL NURSING cancer


HUNTINGTON’S DISEASE ▪ Ex: neurological, neurodegenerative dx
• Parkinson’s, Huntington’s, Narcolepsy
WHAT IS HUNTINGTON’S DISEASE? ▪ Happens when cells divide abnormally during
reproduction
- AKA “Huntington’s chorea” - Each person has two copies of the gene and a person will not develop
- Chronic, progressive (slowly) hereditary disease of the nervous system HD if both copies of the gene are normal
resulting in involuntary choreiform movement and dementia - If an individual has one mutated copy, he or she is destined to develop
o due to degeneration of the basal ganglia progressing to HD
cerebral cortex (Burrel, 1997)
- Affects men and women of all races
- transmitted as an autosomal dominant genetic disorder, each child of a
parent with HD will have a 50% risk of inheriting the illness
- Discovered by George Huntington in 1872
o American physician
- Affects 1 in 10,000 individuals
- Onset is ages 35-45 yrs old
- they may unknowingly pass the disease before symptoms appear
- Diagnosis in the past was based on family history and clinical symptoms,
however, since the gene for Huntington’s Disease has been discovered, PATHOPHYSIOLLOGY
one can now be tested with the presence of the gene
- People who are asymptomatic but has a positive family history of
Huntington’s Disease will face the dilemma on whether they will be
tested or not
- (+) test = the person will develop Huntington’s Disease but when and to
what extent the disease develops cannot be determined

TRANSMISSION

- Transmitted as an autosomal dominant genetic disorder: Chromosome


#4
o One of 23 pairs of chromosomes
o Normally, people has 2 copies of these chromosomes

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

1. Memory DEMENTIA INVOLVES DECLINE IN TWO OR MORE OF THE FOLLOWING AREAS OF


- refers to the processes that are used to acquire store retain and later COGNITION
retrieve information
2. Language
- principal method of human communication consisting of words used in
a structured and conventional way
- conveyed by speech, writing, and articulation for the receiver to have
3. Calculation
PATHOPHYSIOLOGY
STATISTICS

- Currently affects as many as 4-5 million people


- Characterized by intracellular neurofibrillary tangles and senile plaques
- As society ages, more and more people will have Alzheimer’s disease - Senile plaques cause AD or whether they are a byproduct of the AD
(AD) process, this remains unkown
- By 2030 AD will affect >8 million and by 2050 >14 million - presence of abnormal clumps (neuritic or senile plaques) and tangled
bundles of fibers (neurofibrillary tangles) in the brain.

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

1. Family history of dementia


2. Family history of Down syndrome
3. Family history of Parkinson’s
4. Head trauma (with LOC)

COMPARE NORMAL BRAIN


TO ALZHEIMER’S DISEASE

- Decrease in mass and


size
- Change in size
ALZHEIMERS DISEASE - the intricacies of the mechanism have not been clearly defined
- this lack of understanding of the intricacies of the mechanism regarding
the progression of the pathogenic process = reason for the non-
A= CEREBRAL CORTEX availability of effective treatment which can prevent onset and
- involvement in conscious thought and progression of the disease
language - we have some medications given by our experts, but this does not stop
the pathogenic process
B= BASAL FOREBRAIN o it only addresses the functions that are being affected
- has large number of neurons containing o it continues progressively in its pathophysiologic events
acetylcholine
- acetylcholine: chemical in nerve transmission DIAGNOSTIC EXAMINATION
in memory and learning
- usually diagnosed clinically through pts history
C= HIPPOCAMPUS
- plaques develop here 1. patient history, collateral history from relatives
- structure in brain that encodes memories 2. Clinical Assessment
- essential to memory storage - During consultation
- earliest signs of AD found in this area 3. Assessment of intellectual functioning including memory testing
- Using some tools by the neurologists
4. computed tomography (CT)
PROGRESSION OF ALZHEIMERS DISEASE - Used to exclude other cerebral pathology problems or dementia
5. magnetic resonance imaging (MRI)
- Used to exclude other cerebral pathology problems or dementia
6. positron emission tomography (PET)
- exclude other cerebral pathology
7. Genetic testing for the ApoE gene
- Helpful in DX but further studies are still ongoing to confirm the
reliability of this genetic testing not so popular

IN DEMENTIA CT AND MRI MAY BE NORMAL BUT IN LATER STAGES MRI=


DECREASE IN CEREBRAL CORTEX SIZE OR AREA IN BRAIN WHICH RESPONSIBLE
FOR MEMORY OR HIPPOCAMPUS
CHARACTERISTICS/ SIGNS AND SYMPTOMS - Urinary or fecal incontinence
- Lint-picking motion
Stage 1: Predementia - Chewing movements
o Recent memory loss - Progression through stages 2 and 3 varies from 2-12 yrs
o Increased irritability
o Impaired judgement Stage 4: Advanced
o Loss of interest in life
o Decline of problem-solving ability
- Masklike facial expression
o Reduction in abstract thinking - No communication
- Apathy
- Remote memory and neurological exam may remain unchanged - Withdrawal
- Changes are mostly considered as natural occurrence - Eventual immobility
- Seen as normal manifestation of an older person - Assumed fetal position
- No appetite
Stage 2: Early - Emaciation
- Lasts approximately 1 yr
- Lasts approx. 2-4 yrs
- Decline in ability to manage personal and business affairs
- Inability to remember shapes of objects
HOW DOES ALZHEMER’S LEAD TO A PERSON’S DEATH?
- Repetition of meaningless word or phrase (perseveration)
- Aspiration pneumonia
- Wandering at night
- Bedsores
- Circular speech patterns
- Sepsis infections from undiagnosed UTI
- Restlessness
- Infections in general
- Depression, anxiety
- Injuries from falls
- Intensification of cognitive & emotional changes
- Malnutrition and dehydration
*vast majority of those with Alzheimer’s die from aspiration pneumonia
Stage 3: Moderate *the disease does not kill the person, complications do
- Impaired ability to speak (aphasia)
- Inability to recognize familiar objects (agnosia)
- Inability to use objects properly (apraxia)
- Inattention
- Involuntary emotion outbursts
MEDICAL MANAGEMENT - Enhance mood and behavior
- Foster a safe environment
- There is no cure - Promote social engagement
- Available tx offer relatively small symptomatic benefit but remain
palliative Priority Nursing Diagnosis
- Initial management begins with education of the family and caregivers
1. Self-care Deficit r/t impaired cognitive and motor function
Pharmacologic Treatment 2. Risk for injury r/t
a. unable to recognize/ identify hazards in the environment
Focuses on symptoms with an attempt to maintain cognition b. disorientation, confusion, impaired decision making
c. weakness, the muscles re not coordinated, the presence of
1. Donepezil, Rivastigmine, Galantamine seizure activity
o Cholinesterase inhibitor
o Elevates acetylcholine conc in cerebral cortex
o Slowing degradation of ACH Nursing Interventions
2. Antidepressants: SSRI
o Inc activity of serotonin in the brain - establish an effective communication system with pt and his family
o Treats depression, anxiety, and irritability - provide emotional support
3. Cerebral Vasodilators - administer ordered medications
o E.g. Ergoloid, mesylates, isoxsuprine, and cyclandelate
- protect the pt from injury
o To enhance cerebral circulation
4. Psychostimulant
- provide rest periods between activities
o E.g. methylphenidate - encourage the pt to exercise, as ordered
o To enhance the pt’s mood - encourage pt independence
5. Anticholinesterase agents - encourage sufficient fluid intake and adequate nutrition
o E.g. Tacrine - assist with hygiene
o To help improve memory deficits - frequently check v/s
- monitor fluid and food intake
NURSING MANAGEMENT - inspect skin for evidence of trauma
- encourage the family to allow as much independence as possible while
Goals ensuring safety
- promote bowel and bladder continence
- Maintain quality of life - high fiber diet
- Maximize function in daily activities - encourage to stay awake during the day so they can sleep at night
- Enhance cognition - avoid overstimulation at bedtime
*all interventions boils down to maintaining and maximizing the remaining
functions of the clients while keeping them safe

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

6 CARDINAL MANIFESTATIONS Shuffling gait


Fatigue and muscle weakness
1. Tremor
- Early sign MEDICAL MANAGEMENT
- Slow, unilateral resting tremor
Treatment is directed towards controlling the symptoms and
- Rhythmic, slow turning motion (pronation-supination)
maintaining functional independence, because no medical or
of the forearm and the hand and a motion of the
surgical approaches in current use prevent disease
thumb against the fingers as if rolling a pill between
progression.
the fingers
2. Bradykinesia (akinesia, dyskinesia) Care individualized in each patient based on presenting
- One of the most common features of Parkinson’s symptoms and social, occupational and emotional needs.
disease
Pharmacological management – mainstay of treatment
3. Rigidity/Cogwheel rigidity
- Passive movement of an extremity may cause the MEDICATIONS
limb to move in jerky increments, referred to as lead-
pipe or cogwheel movements 1. Antiparkinsonian Medication

4. Stooped posture - Increasing striatal dopaminergic activity

- Flexed posture of the next, trunk and ,limbs - Reducing excessive influence of excitatory

5. Loss of Postural Reflexes cholinergic neurons on the extrapyramidal tract

- 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

OTHERS Legible Candidates: Idiopathic Parkinson’s disease who are taking


maximum doses of antiparkinsonian medications
Dopamine Agonists
o Drugs that increase the release of dopamine at 1. Thalamotomy and Pallidotomy
receptor sites - Effective in relieving many of the symptoms of
o More effective than anticholinergics in treatment of Parkinson’s disease.
Parkinson’s disease
- Thalamotomy: stereotactic electrical stimulator destroys 3. If the patient is unresponsive or intolerant to
part of the ventrolateral portion of the thalamus in an pharmacotherapy, Electroconvulsive therapy is indicated.
attempt to reduce tremor 4. Suicidal precautions to be followed, if the patient exhibits
- Pallidotomy: Involves ventral aspect of the medial any suicidal ideas.
globus pallidus through electrical stimulation in patients - Sedatives are used if there are sleep problems
with advanced disease. 5. Patients should not be forced into situations in which they
2. Deep Brain Stimulation feel ashamed of their appearance.
- An electrode is placed in the thalamus and connected in 6. Encourage the patient to participate in moderate exercises
a false generator implanted in the subcutaneous, - Daily Range of Motion exercises to avoid rigidity and
subclavicular or abdominal pouch. contractures
- The electrodes block nerve pathways in the brain that 7. Instruct the patients to speak slowly and clearly, and to
cause tremors. pause and take a deep breath at appropriate levels.
8. Advise the patient to organize thoughts before speaking and
NURISNG MANAGEMENT
encourage the client to use facial expression and gestures if
Nursing attention should focus on the physical and psychological possible to assist with communication.
deficit. (Health Assessment, Medication, Monitoring, Education) - If possible, used mechanical boards, computer or
electronic typewriter is advised
1. Observe the patient's mood, cognition; organization and
9. Patients are taught how to initiate raising from a chair by
general well being
placing their hands on the arms of the chair.
- Observe for features of depression (treatment:
10. Relaxation therapy
tricyclic antidepressants)
2. Avoid frequent change in the environment
- To minimize confusion
- Put signboards
SIGNS AND SYMPTOMS
NCM 116: MEDICAL SURGICAL NURSING
DEGENERTIVE NEUROLOGIC DISORDERS
- onset of ALS can be so subtle that the symptoms are overlooked
but gradually these symptoms develop into more obvious
WHAT IS MYOTROPHIC LATERAL SCLEROSIS?
weakness or atrophy that may cause a physician to suspect ALS
- Some of the early symptoms include:
- unknown cause o fasciculations (muscle twitches) in the arm, leg, shoulder,
- there is a loss of motor neurons (nerve cells controlling muscles) or tongue
o muscle cramps
in the anterior horns of the spinal cord and the motor nuclei of
o tight and stiff muscles (spasticity)
lower brainstem o muscle weakness affecting an arm, a leg, neck or
- often referred to as Lou Gehrig’s disease diaphragm
o after the famous baseball player who suffered from the o slurred and nasal speech
disease o difficulty chewing or swallowing
- As motor neuron cells dieà the muscle fibers that they supply
DIAGNOSTICS
undergo atrophic changes à Neuronal degeneration may occur
both the upper and lower motor neuron systems
- leading theory held by researchers is that overexcitation of nerve 1. Electromyogram (EMG)
- doctor inserts a needle electrode through your skin into various
cells by the neurotransmitters glutamate = cell injury and
muscles
neuronal degeneration - evaluates the electrical activity of your muscles when they
- Cases of ALS arise sporadically, 5% to 10% of cases are familial contract and when they're at rest
- Abnormalities in muscles seen in an EMG can help doctors
CAUSATIVE AGENT diagnose or rule out ALS
- EMG can also help guide your exercise therapy
2. Nerve conduction study
1. Autoimmune disease - Measures your nerves' ability to send impulses to muscles in
2. free radical damage different areas of your body
3. oxidative stress - can determine if you have nerve damage or certain muscle or
- Several risk factors have been identified, but the exact cause is nerve diseases
still unknown 3. MRI

- Using radio waves and a powerful magnetic field, an MRI


produces detailed images of your brain and spinal cord
- can reveal spinal cord tumors, herniated disks in your neck or 1. Breathing care
other conditions that might be causing your symptoms
- You'll eventually have more difficulty breathing as your muscles
4. Blood and urine tests weaken
- Analyzing samples of your blood and urine in the laboratory might - Doctors might test your breathing regularly and provide you with
help your doctor eliminate other possible causes of your signs and devices to assist your breathing at night
symptoms - You can choose mechanical ventilation to help you breathe
- Doctors insert a tube in a surgically created hole at the front of
5. Spinal tap (lumbar puncture
your neck leading to your windpipe (tracheostomy that connects
- involves removing a sample of your spinal fluid for laboratory
to a respirator)
testing using a small needle inserted between two vertebrae in
your lower back
6. Muscle biopsy 2. Physical therapy
- If doctor believes you may have a muscle disease rather than ALS,
you might undergo a muscle biopsy. While you're under local - physical therapist can address pain, walking, mobility, bracing and
anesthesia, a small portion of your muscle is removed and sent to equipment needs that help you stay independent
a lab for analysis - Practicing low-impact exercises can help maintain your
cardiovascular fitness, muscle strength and range of motion for as
MEDICAL MANAGEMENT long as possible
- Regular exercise can also help improve your sense of well-being
- Appropriate stretching can help prevent pain and help your
Medications: muscles function at their best
- A physical therapist can also help you adjust to a brace, walker or
1. Riluzole (Rilutek) wheelchair and might suggest devices such as ramps that make it
- taken orally, this drug has been shown to increase life expectancy easier for you to get around
by three to six months
- can cause side effects such as dizziness, gastrointestinal 3. Occupational therapy
conditions and liver function changes
2. Edaravone (Radicava) - An occupational therapist can help you find ways to remain
- given by intravenous infusion, has been shown to reduce the independent despite hand and arm weakness
decline in daily functioning - Adaptive equipment can help you perform activities such as
- effect on life span isn't yet known dressing, grooming, eating and bathing
- Side effects can include bruising, headache and shortness of - An occupational therapist can also help you modify your home to
breath allow accessibility if you have trouble walking safely
- This medication is given daily for two weeks a month
4. Speech therapy
Therapies: - A speech therapist can teach you adaptive techniques to make
your speech more understandable
- Speech therapists can also help you explore other methods of 3. Altered bowel function
communication, such as an alphabet - Provide dietary instruction regarding the importance of fiber, use
board or pen and paper of bran cereals, prune juices, etc., unless contraindicated because
- Ask about the possibility of borrowing or renting devices such as
tablet computers with text-to-speech applications or computer- of swallowing ability
based equipment with synthesized speech that can help you - Daily use of stool softeners and Metamucil are often
communicate recommended
o Laxatives may be used, but chronic use may be harsh on
5. Nutritional support the bowel
- team will work with you and your family members to ensure
o Mineral oil should not be used because of the danger of
you're eating foods that are easier to swallow and meet your
nutritional needs aspiration
- might eventually need a feeding tube 4. Nutrition for patient w/ Dysphagia:
- Discuss techniques for protecting airway
6. Psychological and social support o i.e., sitting up straight, putting chin on chest when
- team might include a social worker to help with financial issues,
swallowing, concentrating while eating
insurance, and getting equipment and paying for devices you
need - Avoid increased stimulation
- Psychologists, social workers and others may provide emotional o i.e., talking at mealtime
support for you and your family 5. Respiratory
- Maintain airway patency
NURSING MANAGEMENT - Maintain adequate ventilation
1. After each change of position, check for redness over bone
- prominences, and provide an eggshell or circulating mattress WHAT IS MUSCULAR DYSTROPHIES?
when immobility prevents independent repositioning
- An Achilles tendon is a sturdy fibrous cord that links the muscles
- Repositioning in the wheelchair based on the patient’s skin
at the back of the calf to the heel bone
tolerance
o It is also considered to be the largest tendon in the
- Use of a wheelchair cushion to prevent skin breakdown
human body
- Proper positioning when ambulating or in a wheelchair
o This tendon allows the toes to point towards the floor or
o i.e., use of a sling for a weak upper extremity
above it
- Promote adequate nutritional intake
- some people most especially athletes may unconsciously
2. Urinary Function
overstretch this part of the lower extremity that causes it to
- Encourage daily fluid intake of 2,500cc’s per day unless
rupture or tear either partially or completely
contraindicated because of swallowing ability
o This may also be caused by a sudden forceful movement 4. Swelling at the heel area
that stresses the calf upon doing a particular motion 5. Inability to stand on toes on the injured legs
- Risk factors may include, landing or moving in an uneven or rough 6. Inability to bend the foot downward
surface, overtraining, walking or running technique, weight, age 7. Feeling of having been kicked in the calf
and wearing the wrong footwear may also be a risk factor 8. Difficulty walking
o Upon tearing it, a pop may be heard followed by a sharp
pain on the ankle that may radiate towards the lower MEDICAL MANAGEMENT
part of the leg
1. Surgical repair of the rupture tendon
o Upon diagnosing it, a physician would inspect the tendon
2. Prescription of anti-inflammatory and pain medications
and may even palpate it to check if it has ruptured
3. Casting of the foot
completely or partially
4. Wearing of brace
o The physician may even ask the client to lie on the
5. Percutaneous techniques (less invasive procedure)
examination table and would squeeze the calf muscle,
usually the foot will automatically flex, but if doesn’t, NURSING MANAGEMENT
then it would mean that the tendon has ruptured
1. Application of cold compress on affected area.
CAUSATIVE AGENT 2. Demonstrating to the client on proper crutch walking
3. Instruct the client to rest the affected foot.
- bears along the movement that an individual does
4. Emphasize to the client to avoid strenuous activities for 3-5 weeks
o Anatomically, the tendon is located between the heel
or until advised by the physician.
and the calf where it connects the two and upon its
5. Educate the client on strict adherence in wearing the foot brace if
range of motion, it can be observed that it could only
prescribed.
support a specific range when the foot is flexed or 6. Educate client on weight management techniques if overweight.
extended
o during strenuous activities, an individual may overstretch
it or may do a sudden forceful movement that causes the WHAT IS HERNIATION OF A CERVICAL
Achilles tendon to rupture and cause a very sharp pain INTERVERTEBRAL DISK?

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

1. Physical therapy and/or exercises for exercises to help relieve the


pressure on the nerve root
2. Medications, such as non-steroidal anti-inflammatory drugs
(NSAIDs) to reduce swelling and pain and analgesics to relieve
pain
3. Epidural steroid injections and nerve root injections to help
reduce swelling and treat acute pain that radiates to the hips or
down the leg
De enerative Disk Disease

● 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.

● Moderate, continuous low back pain.


● Local tenderness.
● Le pain
● Givin out symptoms. eelin o sudden weakness or instability can happen as the disc
becomes weaker, creatin a sensation that the low back will “ ive out” with sudden movements.
● Pain with sittin .
● Exacerbated pain with bendin or twistin .
● Pain relie when walkin or chan in positions.
Medical and Nursin Mana ement.

A. Medical Mana ement

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.

II. Surgical Therapy


● Lumbar Procedures

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.

Nursin Mana ement

● 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.

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