Degenerative diseases

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NEURODEGENERATIVE DISEASES

VIRGINIA DAMMANG

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LEARNER’S OBJECTIVES: Identify different types of

neurodegenerative disorders. Able to determine individual’s needs

according to each diseases. Able to manage the diseases according

to their manifestations. Educate the patient and family related

to the disease process.

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Neurodegenerative Diseases

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TOPICS:

Multiple Sclerosis Alzheimer’s Disease Huntington’s Disease Parkinson’s Disease

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MULTIPLE SCLEROSISMS is a autoimmune disease where the

myelin surrounding the nerve pathways, which transmit signals through the brain and spinal cord, are slowly diminishing. This is a process called demyelination. After this occurs the body produces scar tissue or plaque around these nerve pathways. Due to this, the body is unable to transmit information quickly.

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SYMPTOMS OF MS Symptoms of MS often occur in

episodes or attacks. Although occasionally the person with MS may experience an attack due to certain triggers most episodes are most likely not expected to the person with MS but instead happen for no reason at all.

The following may contribute to attack: Spring/summer Infections Stress Pregnancy Symptoms will vary from each person with

MS. It depends on the stage of the disease process and the location of scar tissue within the body.

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3 TYPES OF THERAPY Interventions to treat symptoms Drugs to treat the attack Drugs that modify the course of the

disease

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DRUGS: IV Corticosteroids- not considered long

time solutions-have no proven long-term efficacy and multiple side effects can develop

Adrenocorticotropic hormone (ACTH) Disease-modifying agents-Fingolimod-Teriflunomide oral -Dimethyl fumarate-Interferon-beta-Glatiramer acetate intravenous-Natalizumab

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In 2014, a pegylated form of interferon beta-1a was introduced with the brand name Plegridy, which is available as a subcutaneous injection.

This peginterferon beta 1-a attaches polyethylene glycol to the interferon molecules allowing longer lasting biological effects in the body while decreasing the frequency of administration to once every two weeks

Interferon beta balances the expression of pro- and anti-inflammatory agents in the brain, and reduces the number of inflammatory cells that cross the blood–brain barrier

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DISEASE MODIFYING AGENTS Reduce frequency of attacks Help reduce the severity Slow progression of disability Reduce lesions responsible for relapses

These agents should be started following the diagnosis of active relapsing

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Alzheimer’s Disease (AD)

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Alzheimer's disease is the most common cause of dementia, which is the loss of intellectual and social abilities severe enough to interfere with daily functioning.

Dementia occurs in people with Alzheimer's disease because healthy brain tissue degenerates, causing a steady decline in memory and mental abilities.

Alzheimer’s Disease

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The causes of Alzheimer's are poorly understood, but its effect on brain tissue has been demonstrated clearly. Alzheimer's damages and kills brain cells.

Studies of plaques and tangles from the brains of people who have died of Alzheimer's suggest several possible roles these structures might play in the disease.

Cause of AD

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Medical History

Basic Medical Test (e.g. blood tests)

Mental Status Evaluation

Neuropsycological Testing

Brain Scans

Assessment and Diagnosis

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Stage 1. Mild memory lapses

Stage 2. Obvious short- term memory lapses

Stage 3. Disintegrations of personality

Stage 4. Terminal Stage – physical and mental deterioration

AD’s Different Stages

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Clinical Manifestations

Disorientation

Loss ofJudgement

Difficulty FindingThe Right Word

Difficulty PerformingFamiliar Tasks

Difficulty withAbstract Thinking

Personality Changes

Increasing andPersistent

Forgetfulness

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Currently, there is no cure for AD. Medications are prescribed to offer relatively small symptomatic relief.

Management of AD

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NURSING ASSESSMENT Activity/restSigns: anxiety, helplessness, sleep pattern

disturbance, lethargy and impaired motor skills.Symptoms: feeling melting Ego integritySigns: hide incompetence, sit down and watch the

other, the first activity might accumulate objects are not moving and emotional stability

Symptoms: suspicious or afraid of the situation / person fantasies, misperceptions of the environment, loss of multiple.

EliminationSigns: Incontinence of urine / fecesSymptoms: The urge to urinate

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NURSING CARE PLAN Change the thought process related

to:Irreversible neuronal degenerationMemory LossPsychological ConflictSleep deprivation

Changes in sleep patterns related to:

Changes in sensoryPsychological pressureChanges in activity patterns

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Risk of trauma related to:The inability to recognize / identify hazards in

the environmentDisorientation, confusion, impaired decision

makingWeakness, the muscles are not coordinated, the presence of seizure activity.

Risk for Injury related to:Unable to recognize / identify hazards in the environment.Disorientation, confusion, impaired decision

making.Weakness, the muscles are not coordinated, the presence of seizure activity.

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ALZHEIMER’S DISEASE DRUGSGalatamine inhibitors Cholinesterase inhibitors

Donepezil (Aricept)- Alantamine (Reminyl)

Rivastigmine (Exelon) Tacrine (Cognex)

Side effects: GI erosion and hepatotoxicity

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MEMANTINE (NAMENDA)

The first drug approved to treat moderate to severe stages of Alzheimer's, memantine (Namenda), protects brain cells from damage caused by the chemical messenger glutamate.

It sometimes is used in combination with a cholinesterase inhibitor.

Most common side effect is dizziness, and may increase agitation and delusional behavior.

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Huntington’s disease is a chronic, progressive, hereditary disease of the nervous system that results in progressive involuntary choreinform movement and dementia.

It is transmitted as an autosomal dominant genetic disorder.

Also called Huntington's chorea, “chorea” comes from the greek word meaning “to dance” and refers to the incessant quick, jerky, involuntary movements that are characteristic of this condition.

Each child of a parent with Huntington’s disease has a 50% risk of inheriting the illness.

It affects men and women.

Huntington’s Disease

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Huntington's disease is an inherited condition caused by a single abnormal gene.

Because only one copy of the defective gene, inherited from either parent, is necessary to produce the disease.

Because signs and symptoms typically first appear in middle age, some parents may not know they carry the gene until they've already had children and possibly passed on the trait.

Cause of HD

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CT scan and MRI may show atrophy of the Caudate Nuclei once the disease is well established.

A genetic marker for Huntington’s disease has been identified through the use of recombinant DNA technology. As a result, researchers can now identify presymptomatic individual who will develop this disease.

Assessment and Diagnosis

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Abnormal involuntary movements (chorea), intellectual decline, and emotional disturbances.

Constant writhing and uncontrollable movements of the entire body occur as this disease progresses.

Facial movements produce ticks and grimaces; speech becomes slurred, hesitant, open explosive, and then eventually unintelligible.

Chewing and swallowing are difficult, and aspiration and choking are dangers.

Gait becomes disorganized, and ambulation is eventually impossible; patient is eventually confined to a wheelchair.

Clinical Manifestations

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Medications

Tranquilizers such as clonazepam (Klonopin) and antipsychotic drugs such as haloperidol (Haldol) and clozapine (Clozaril) can help control movements, violent outbursts and hallucinations.

While these medications can be helpful, a common side effect is sedation, and in some cases, these medications may cause additional stiffness and rigidity.

Various medications, including fluoxetine (Prozac, Sarafem), sertraline (Zoloft) and nortriptyline (Aventyl, Pamelor), can help control depression and the obsessive-compulsive rituals that some people with Huntington's disease develop.

Medications such as lithium (Eskalith, Lithobid) can help control extreme emotions and mood swings.

Management

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Physical therapy can help keep muscles stronger and more flexible, which helps maintain balance and may lessen the risk of falling.

Occupational therapy can help make your home safer and give you strategies for coping with memory and concentration problems.

Later in the disease, occupational therapy can assist you with eating, dressing and hygiene challenges.

Physical and Occupational Therapy

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Though the signs and symptoms vary from person to person, vital functions, such as swallowing, eating, speaking and walking, usually degenerate over time.

Many people with Huntington's disease develop depression, and some are at risk of suicide. However, death generally occurs as a result of complications of the disease, such as an infection or a fall.

Complications

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Reinforcement the understanding that Huntington’s disease takes emotional, physical, social, and financial tolls on every member of the patient’s family.

Encourage genetic counseling, long term psychological counseling, marriage counseling, and financial and legal support.

Teach patient and family about the medication, including signs indicating the need for change in dosage or medication.

Nursing Management

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Address strategies to manage symptoms (chorea, swallowing problems, ambulation problems, or altered bowel or bladder function).

Emphasize the need for regular follow-up.Refer for home care nursing assistance,

respite care, day care centers, and eventually skilled long-term care to assist patient and family cope.

Provide information about the Huntington’s Disease Foundation of America, which gives information, referrals, education, and support for reaserch.

Nursing Management

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Parkinson's disease is a disorder that affects nerve cells in the part of the brain controlling muscle movement.

People with Parkinson's disease often experience trembling, muscle rigidity, difficulty walking, problems with balance and slowed movements.

Symptoms usually develop after age 60, although some

people affected by Parkinson's disease are younger than age 50.

Although Parkinson's disease may eventually be disabling, the disease often progresses gradually, and most people have many years of productive living after a diagnosis.

Parkinson’s Disease

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Most people with Parkinson's disease are described as having idiopathic PD (having no specific cause).

There are far less common causes of PD including genetic, toxins, head trauma, cerebral anoxia, and drug-induced PD.

Causes

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Past episodes of head trauma are reported more frequently by sufferers than by others in the population.

A methodologically strong recent study found that those who have experienced a head injury are four times more likely to develop Parkinson’s disease than those who have never suffered a head injury.

Head Trauma

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Antipsychotics, which are used to treat schizophrenia and psychosis, can induce the symptoms of Parkinson's disease (or parkinsonism) by lowering dopaminergic activity.

Drug Induced PD

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Clinical Manifestations

Tremors

Bradykinesia Rigid Muscles

ImpairedBalance

Dementia

DifficultySwallowing

ImpairedSpeech

Loss of Automatic

Movements

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Medications

Anticholinergics

Cathechol – o –Methyltransferase

SelegilineEldepryl

DopamineAgonist

Levodopa

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Surgical Management

Pallidotomy

Deep BrainStimulation

Thalamotomy

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Difficulty chewing and swallowing.

Urinary problems.

Constipation

Sleep problems

Sexual dysfunction

Complications

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Provide information and support regarding Parkinson’s Disease.

Health teaching, diet, high in fiber, increase oral fluid intake to prevent constipation.

Promote relaxation exercises to promote proper sleep.

Improving mobility.Enhancing self-care activities.Improving bowel eliminationImproving nutrition.Enhancing swallowing.

Nursing Management

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NURSING CARE PLAN1.Fatigue2.Self care deficit3.Low self-esteem4.Powerlessness/hopelessness5.Risk of ineffective coping6.Ineffective family coping7.Impaired urinary elimination8.Knowledge deficit9.Risk for caregiver role strain

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1. FATIGUEMay be related to Decreased energy production, increased energy requirements to perform

activities Psychological/emotional demands Pain/discomfort Medication side effectsPossibly evidenced by Verbalization of overwhelming lack of energy Inability to maintain usual routines; decreased performance Impaired ability to concentrate; disinterest in surroundings Increase in physical complaintsDesired Outcomes Identify risk factors and individual actions affecting fatigue. Identify alternatives to help maintain desired activity level. Participate in recommended treatment program. Report improved sense of energy.

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4. POWERLESSNESS/HOPELESSNESSNursing Diagnosis Powerlessness HopelessnessMay be related to Illness-related regimen, unpredictability of disease Lifestyle of helplessnessPossibly evidenced by Verbal expressions of having no control or influence over situation Depression over physical deterioration that occurs despite patient compliance with

regimen Nonparticipation in care or decision making when opportunities are provided Passivity, decreased verbalization/affect Verbal cues Lack of involvement in care/passively allowing care Isolating behaviors/social withdrawalDesired Outcomes Identify and verbalize feelings. Use coping mechanisms to counteract feelings of hopelessness. Identify areas over which individual has control. Participate/monitor and control own self-care and ADLs within limits of the individual

situation.

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2. SELF-CARE DEFICITMay be related to Neuromuscular/perceptual impairment; intolerance to activity;

decreased strength and endurance; motor impairment, tremors Pain, discomfort, fatigue Memory loss DepressionPossibly evidenced by Frustration; inability to perform tasks of self-care, poor personal

hygieneDesired Outcomes Identify individual areas of weakness/needs. Demonstrate techniques/lifestyle changes to meet self-care

needs. Perform self-care activities within level of own ability. Identify personal/community resources that provide assistance.

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3. LOW SELF-ESTEEMMay be related to Change in structure/function Disruption in how patient perceives own body Role reversal; dependencePossibly evidenced by Confusion about sense of self, purpose, direction in life Denial, withdrawal, anger Negative/self-destructive behavior Use of ineffective coping methods Change in self/other’s perception of role/physical capacity to resume

roleDesired Outcomes Verbalize realistic view and acceptance of body as it is. View self as a capable person. Participate in and assume responsibility for meeting own needs. Recognize and incorporate changes in self-concept/role without

negating self-esteem. Develop realistic plans for adapting to role changes.

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5. RISK FOR INEFFECTIVE COPINGRisk factors may include Physiological changes (cerebral and spinal lesions) Psychological conflicts; anxiety; fear Impaired judgment, short-term memory loss; confusion; unrealistic perceptions/

expectations, emotional liability Personal vulnerability; inadequate support systems Multiple life changes Inadequate coping methods

Possibly evidenced by Not applicable. A risk diagnosis is not evidenced by signs and symptoms, as the

problem has not occurred and nursing interventions are directed at prevention.

Desired Outcomes Recognize relationship between disease process (cerebral lesions) and

emotional responses, changes in thinking/behavior. Verbalize awareness of own capabilities/strengths. Display effective problem-solving skills. Demonstrate behaviors/lifestyle changes to prevent/minimize changes in

mentation and maintain reality orientation.

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6. INEFFECTIVE FAMILY COPINGMay be related to Situational crisis; temporary family disorganization and role changes Highly ambivalent family relationship Prolonged disease/disability progression that exhausts the supportive capacity of SO Patient providing little support in turn for SO SO with chronically unexpressed feelings of guilt, anxiety, hostility, despair

Possibly evidenced by Patient expresses/confirms concern or complaint about SO response to patient’s illness SO withdraws or has limited personal communication with patient or displays protective

behavior disproportionate to patient’s abilities or need for autonomy. SO preoccupied with own personal reactions Intolerance, abandonment Neglectful care of patient Distortion of reality regarding patient’s illness

Desired Outcomes Identify/verbalize resources within themselves to deal with the situation. Express more realistic understanding and expectations of patient. Interact appropriately with patient/healthcare providers providing support and

assistance as indicated. Verbalize knowledge and understanding of disability/disease and community resources.

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7. IMPAIRED URINARY ELIMINATION

May be related to Neuromuscular impairment (spinal cord lesions/neurogenic bladder)

Possibly evidenced by Incontinence; nocturia; frequency Retention with overflow Recurrent UTIs

Desired Outcomes Verbalize understanding of condition. Demonstrate behaviors/techniques to prevent/minimize infection. Empty bladder completely and regularly (voluntarily or by catheter

as appropriate). Be free of urine leakage.

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8. KNOWLEDGE DEFICITMay be related to Lack of exposure; information misinterpretation Unfamiliarity with information resources Cognitive limitation, lack of recall

Possibly evidenced by Statement of misconception Request of information Inaccurate follow-through of instruction; development of preventable complications Inappropriate or exaggerated behaviors (e.g., hysterical, hostile, agitated,

apathetic)

Desired Outcomes Participate in learning process. Assume responsibility for own learning and begin to look for information and to ask

questions. Verbalize understanding of condition/disease process and treatment. Initiate necessary lifestyle changes.

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9. RISK FOR CAREGIVER ROLE STRAINRisk factors may include Severity of illness of the care receiver, duration of care

giving required, complexity/amount of care giving task Caregiver is female, spouse Care receiver exhibits deviant, bizarre behavior Family/caregiver isolation; lack of respite and recreation

Desired Outcomes Identify individual risk factors and appropriate

interventions. Demonstrate/initiate behaviors or lifestyle changes to

prevent development of impaired function. Use available resources appropriately. Report satisfaction with plan and support available.

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SUMMARY There is increasing research to help

those suffering with this this deadly disease.

Worldwide quest is on their way to stop slow or even prevent this diseases.

Monitoring and treating the disease is very important in identifying the effectivity of the medications and if the condition is improving.

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REFERENCE:

Rubinsztein DC (October 2006) “the roles of intracellular protein-degradation pathways in neurodegeration” Nature 443 (7113)

Varkey J, Isas JM, Mizuno N, et al (October 2010) “Membrane curvature induction and tubulation are common features of synucleins and apolipoproteins” The Journal of Biological chemistry 284 (12)

 Peginterferon beta-1a description National Multiple Sclerosis Society (15 August 2014). Retrieved on 27 October 2014

Kieseier, Bernd C. (1 June 2011). "The Mechanism of Action of Interferon-β in Relapsing Multiple Sclerosis". CNS Drugs 25 (6)

Arnon, R., & Aharoni (2004). "Mechanism of action of glatiramer acetate in multiple sclerosis and its potential for the development of new applications". Proceedings of the National Academy of Sciences of the United States of America 101

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Thank you!!!