parkinsons disease 1

Overview of PD

What is PD? History of PD

Cathi A. Thomas, MS, RN, CNRNDepartment of Neurology

Boston University Medical Center

What is Parkinson’s Disease (PD)?

• PD is described as a chronic, progressive neurological condition.

• Second most common neurodegenerative disease following Alzheimer‟s disease

• PD is a “hypokinetic” movement disorder.

• Movement Disorders are a group of conditions that cause abnormal movements usually “too much” or “too little”.

Parkinsonism

• IDIOPATHIC PARKINSON’S DISEASE.……....85%

• Neuroleptic-induced parkinsonism…………7% - 9%

• Multiple system atrophies (MSA)……………….2.5%

• Progressive supranuclear palsy (PSP) ………..1.5%

• Vascular parkinson syndrome…………………….3%

• MPTP, CO, Mn, recurrent head trauma………....rare

• Postencephalitic parkinsonism……none since 1960s

Historical Perspectives

• James Parkinson (1755-1824) published An Essay on the Shaking Palsy, 1817, London

Described:

• “Involuntary tremulous motion, with lessened muscular power, in parts not in action and even when supported; with a propensity to bend the trunk forwards, and to pass from a walking to a running pace: the senses and intellect being uninjured.”

History (cont’d)

• 1960‟s - Discovery of the neurochemical basis of PD; use of L-dopa (precursor of dopamine) by Dr. George Cotzias

• 1990-2000 “Decade of the Brain”

• 21st Century - PD now considered a multi-system disorder

PD - Who is affected?

• Recent study (Willis AW, et al, 2010) identified demographic and environmental factors of PD

• Used extensive Medicare data set from over 10 years

• 1.6% of U.S. population over 65 has a dx of PD

PD - Who is affected? (cont’d)

• Approximately 130,000 people newly diagnosed each year in US

• Men slightly more likely to have PD

(1.55 male : 1 female ratio)

• Caucasian men in the U.S. have up to double the rate of PD as compared to African Americans or Asians

• Asian women have lowest rate of PD in the U.S.Reference: Willis AW, et al. Geographic and Ethnic Variation in Parkinson Disease:A Population-Based Study of US Medicare Beneficiaries. Neuroepidemiology 2010;34:143-151

PD - Who is affected? (cont’d)

• Diagnosis typically occurs in the 5th and 6th

decade of life (average age 63)

• 5-10% of people have symptoms < 45

• Projected number of people with Parkinson‟s disease in the most populous nations will double by 2030- 4.3 million to 9.5 million worldwide.

E.R. Dorsey et al, Neurology 2007;68:384-386

Economic Burden

• Staggering Costs

• The combined direct and indirect cost of PD including treatment, social security payments, and lost income is estimated at 25 billion in the US

• Medication costs average $2,500 annually

• Surgery costs average $100,000(PDF Fact Sheet, 2007)

• 2-7% of individuals in long-term care settings have a diagnosis of PD (Caring for the Ages - AMDA, 2003)

Introduction to the Role of the Nurse in PD Care

Cathi A. Thomas, MS, RN, CNRNDepartment of Neurology

Boston University Medical Center

Delivering Care• The delivery of quality care to a patient/family living with

Parkinson‟s Disease is complex and requires multiple disciplines working collaboratively to reach the best possible outcome

• Quality Care is supported by care that is patient-centered, evidence based, and delivered by interdisciplinary teams

• The nurse is a core member of the interdisciplinary team.

Hickey, J., The Clinical Practice of Neurological &Neurosurgical Nursing, 6th

edition, 2009 Wolters Kluwer Health, Philidelphia,PA.

• Nurses play an important role in care delivery across the continuum from diagnosis to end of life

• Nurses encounter PD patients in many clinical settings

• Increased utilization of nurses as disease progresses

Nurse Specialists in PD (U.S.)

• Work in Movement Disorder Centers

• Neurology Practices with large PD populations

• Neurosurgical Practices with DBS Programs

• Information and Referral Centers funded by lay organizations including APDA, and NPF

• VA PADRECC Centers

• Nurse Practitioners

• Clinical Nurse Specialists

• Nurse Clinicians

• DBS Nurses

• Research Nurses

• Coordinators supporting the Community at large (National organizations; APDA/NPF)

• And more

These Nurses function as:

• Long Term Care Nurses (AMDA 5-10%)

• Rehabilitation Nurses

• Home Care Nurses (VNA, Parish Nurse, etc.)

• Adult Day Health / Assisted Living Nurses

• Geriatric Treatment Program Nurses

• And more

“Frequent-Encounter” Nurses

• Nurses in Acute Care Settings (ER‟s, medical-surgical areas, orthopedic units, critical care units, psychiatric units, perioperative settings)

• In the neighborhood….

“Chance-Encounter” Nurses

Clinical Care• Assess signs and symptoms of an individual’s

disease process and response to treatment over time (i.e. medication, bowel, bladder…)

• Assess impact of these human responses on an individual‟s quality of life

• Provide patient/family focused care

• Assess patient/family coping strategies, define un-met needs, and provide support in accessing resources

Clinical Care (cont’d)

• Provide patient/family education *medication*

• Provide ongoing assessment and support to patients in between visits via telephone/e-mail

• Implement center protocols to increase communication between patient/family and other disciplines (telephone triage, diary training)

• Develop programs that enhance the delivery of care (day evaluation program)

Education and Support

• Patient/family education

• Healthcare professionals

• Community at large

Patient/Family Education and Support

• Disease process, targets of therapy, medication management, DBS, self management of activities of daily living, safe mobility, coping strategies

• Development of educational programs and symposia for patients/families in a practice or community (newly diagnosed, young onset, family caregivers)

Research

• Nurses participate in PD research in a number of ways:

• Investigator exploring models of care evidence-based practice

• Consultant to other disciplines conducting research “providing the nursing perspective”

• Clinical Trial Coordinator (Parkinson Study Group, NET PD, Industry trials)

Thank You To All Of The Nurses Who Have Supported Me Along The Way. A Special Thanks To The Nurses Who Have Joined Us Today.

Happy Neuroscience Nurses Week!

Overview of Pathogenesis and Epidemiology of PD

Susan Heath, MSN, RNMovement Disorders CNS

San Francisco VAParkinson‟s Disease Research, Education and

Clinical Center(PADRECC)

Objectives

• Describe current evidence and theory of the pathogenesis of idiopathic Parkinson‟s Disease.

• Discuss genetic and environmental evidence and theory into the cause/s of Parkinson‟s Disease.

Pathogenesis: What happens to the brain in PD?

Olanow, C. W. et al. Neurology 2009;72:S1-S136

Parkinson’s as we were taught

Lewy Bodies

• Alpha –Synuclein are proteins found inside Lewy Bodiesand these are the pathologic hallmark for PD.

• Unknown if LB‟s are the toxin to the cells causing PD?

• Or are LB‟s the end result or „trash can‟ response in dying cells?

Olanow, C. W. et al. Neurology 2009; 72:S1-S136

Parkinson’s as we think about it now

Lang & Lozano (1998)

Multiple Sites of Neurodegeneration in PD

•Dopamine (DA)– red

•Norepinephrine – green(May precede loss of DA: Associated with brain functions such as: sleep, memory, learning and mood)

•Serotonin – orange(May precede loss of DA: Associated with mood, anxiety, appetite, GI function and pain)

•Acetylcholine – blue (Associated with memory and learning)

Theoretical Causes of Idiopathic PD

• Idiopathic PD is 90-95% „sporadic‟ and only 5% familial.

• Etiology of sporadic form of PD is unknown.

• Several single gene mutations are identified in familial PD but only a minority have a clear familial pedigree

• More common in 1st degree relatives by 2-3 fold• PD twin study showed no sig. concordance of PD

except if onset before age 50. • Thus young onset PD is more genetically determined.

Genetic Causes of PD =

Parkinson’s genes have given clues to the multiple paths to cell death:

• Mitochondrial and oxidative stress• Protein degradation malfunction (trafficking)

Parkinson’s Disease & the Pesticide Link

From: Web Ross MDSlides used with permission

Environmental toxins (fungicides, herbicides and pesticides) are actively being investigated

• In a recent review, over 24 of 31 case-control studies have shown an association of PD and pesticides. • PD risk is 1.6 to 7 times higher in pesticide exposed• The higher the exposure the greater the risk

BUT

• Broad chemical categories

• Few specific agents identified

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••

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Brown et al, 2006

From: Web Ross MDSlides used with permission

Summary: Pesticide - PD Link

• Association does not prove „cause and effect‟ and the evidence that pesticides cause PD is still not definitive.

• National Academy of Sciences Institute of Medicine determined that “there is limited or suggestive evidence of an association between exposure to the compounds of interest and PD”

Summary: What we know about the Pathogenesis of PD

• Causes of PD remain elusive. (Tanner, 2010)

• PD is more complex, gone is the simple notion that PD is a simple lack of dopamine.

• Sporadic PD is diagnosed after the alpha-synuclein pathology has reached an advanced stage. (Braak et al, 2003)

• Genetic mutations may contribute to one‟s susceptibility.

• Most cases of sporadic PD are thought to be caused by an interplay of environment and genetics.

“The Parkinson Umbrella”Importance of Differential Diagnosis

Gwyn M. Vernon, MSN, CRNP

University of PAParkinson‟s Disease and Movement Disorder Center

and School of Nursing

• Speaker‟s bureau

• Ipsen

• Teva

• Medtronics

Disclosures

Objectives

• Briefly, identify how the diagnosis of PD is established

• Compare and contrast conditions and considerations in the differential diagnosis, ie. secondary parkinsonism and atypical parkinsonian syndromes

PD can be misdiagnosed

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Clinically Autopsies

How is PD diagnosed?

• Prodrome phase

• Depression, anxiety, non-specific cognitive changes, chronic constipation

• Initial clues may be non-motor

• Pain/sensory complaints, urinary symptoms, lethargy, visual symptoms

Non-Motor Symptoms (NMS) at initial presentation

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THE DIFFERENTIAL DIAGNOSIS:PARKINSON’S AND

“Parkinsonisms”

• Idiopathic Parkinson‟s disease

• Secondary Parkinsonism

• Atypical Parkinsonism

DIAGNOSIS OF PD:Motor Characteristics of IPD

• Two of the following:

• Rest tremor, cogwheel rigidity, bradykinesia

• Asymmetric presentation

• Robust response to levodopa

Additional Differential Diagnostic Considerations

• Secondary causes ruled out• Often treatable or reversible

• Atypical parkinsonian features not present early • Neurodegenerative, progressive

Secondary “Parkinsonism”

• Metabolic (Wilson‟s disease)

• Vascular lesions (Multi-infarct states)

• Structural lesions (Hydrocephalus)

• Drug induced

Drug-Induced Parkinsonism

• Typical neuroleptics

• Such as haloperidol (Haldol)

• Metaclopramide (Reglan)

• Prochlorperazine (Compazine)

• Promethazine (Phenergan)

• Atypical neuroleptics

• Risperidone (Risperdal), olanzapine (Zyprexa)

Atypical Parkinsonism or “Parkinson-plus” syndromes

• Neurodegenerative disorders

• May mimic PD early on

• Progressive; treatments limited

Common Atypical Parkinsonisms: Approximately 15 % of presenting “PD” cases

• Corticobasal degeneration

• Dementia with Lewy bodies

• Autopsies commonly show

• Multiple system atrophy

• Progressive supranuclear palsy

• Alzheimer‟s pathology

American Academy of Neurology

Practice Guideline (2006)

• Symmetry of signs and symptoms

• Lack of tremor

• Poor response to levodopa

• Falls early in course

• Dysautonomia early

• Rapid progression

• Characteristics supportive of other parkinsonian syndromes

Corticobasal Degeneration

• Parkinsonism; +/- tremor, bradykinesia

• +/- dystonia, myoclonus

• Pronounced asymmetry

• “Alien limb”, apraxia

• Speech and sensory abnormalities

• As name implies, degeneration of multiple cortical areas, especially frontal-parietal and basal ganglia; cause unknown, possible abnormality of “tau” protein

Dementia with Lewy Bodies (DLB)

• Parkinsonism – bradykinesia and rigidity

• Concurrent cognitive decline with

• FLUCTUATING ALERTNESS AND ATTENTION

• VISUAL HALLUCINATIONS

Multisystem accumulation of abnormal protein deposits (Lewy bodies) in brain stem, basal ganglia and cerebral cortex………

Multiple System Atrophy

• Shy-Drager

• Striatal-nigral degeneration

• Olivopontocerebellar atrophy

• Autonomic degeneration

• MSA – P

• MSA -C

Multiple System Atrophy

• Autonomic and Urinary Dysfunction• Orthostasis > 30 mmHg systolic or > 15 mmHg diastolic, or

• Urinary incontinence, or

• Both

• Parkinsonism (MSA-P)• Bradykinesia plus one of rigidity, tremor or postural instability

• Cerebellar (MSA-C)• Gait ataxia, plus one of : dysarthria, limb ataxia or sustained

gaze evoked nystagmus

Progressive Supranuclear Palsy (PSP)

• Parkinsonism: Bradykinesia, rigidity

• Severe, early imbalance and falling

• Slurred speech, dysphagia

• Myoclonus

• *Inability to gaze downward

• “A tauopathy” characterized by abnormal accumulations of tau protein in cerebral cortex especially frontal areas, basal ganglia, cerebellum and spinal cord

Alzheimer’s disease with motor features

• May appear to have severe bradykinesia

• May have apraxia, visuospatial issues

• Probably have:

• Severe psychomotor slowing

• Major depression (22.5-54.4%)

Diagnosis of Parkinson’s disease

• Difficult; delayed; often misdiagnosed

• Based on history and clinical findings; no laboratory testing

Parkinson’s vs. ParkinsonismCommon differentials to consider

• Idiopathic, typical Parkinson‟s disease

• Secondary parkinsonism• Metabolic, Vascular, Structural, drug induced

• Atypical parkinsonisms• Corticobasal degeneration, Dementia with Lewy bodies, MSA,

PSP, Alzheimer‟s pathology

Differential Diagnosis Resources

• Locate a specialist:

• Parkinson’s Disease Foundation national hotline 800-457-6676; or “ask the expert” at www.pdf.org

• National Parkinson Foundation

• www.parkinson.org; click on “find resources”

• American Parkinson Disease Association

• www.apdaparkinson.org; click I and R centers

A correct diagnosis

Leads to improved patient care

Lessens unnecessary interventions

Gives patient and family confidence and support

Review of Lisette Bunting Perry’s PD Model of CareLisette Bunting-Perry, Ph.D., R.N.

Conflicts of interest: None

Lecture Outline

• The Parkinson‟s Disease Model of Care

• Why develop a model for nurses?

• U.S. Demographics

• What is palliative care?

Why Develop a Model for Nursing Care in PD?

• Nursing as a science

• Chronic disease – Parkinson‟s disease

• Nursing care specific to PD

• Palliation as a philosophy of care across the lifespan

• Need to frame our work – the science of caring

Bunting-Perry, L. (2006). Journal of Neuroscience Nursing, 38(2), 105-112.

Parkinson’s Disease Model of Care

Diagnosis of

Parkinson’s Disease

Treatment of PD (Prolongation of Life)

Death

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Palliative Care (Relief of Suffering)

Early P.D.

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Theoretical Modelsand Nursing Science

• A theoretical framework allows for the structure of scientific inquiry, the framing of research questions, and the explication of relationships among important variables and outcomes.

• Few theoretical models have been proposed to guide care for patients with PD and their family.

www.capc.org

The Demographic Imperative:U.S. Projections

• People age 65 and over is projected to increase from 39 million in 2010 to 69 million in 2030.

• The 85 and older population is expected to more than triple, from 5.4 million to 19 million between 2008 and 2050..

• The aging of the population will increase the annual number of deaths by over 70%, from 2.3 million in 1995 to 4.0 million in 2050. (US Census Bureau, 2008)

U.S. Life Expectancy: Sex

U.S. Projections

Medicare: Personal Care

US Data: Memory Impairment

Incidence of Parkinson’s Disease: Variation by Age, Gender, and Race/Ethnicity

Van Den Eeden et. al, 2003

• Goal: was to estimate the incidence of Parkinson‟s disease by age, gender, and ethnicity

• Findings: gender-adjusted incidence rate of 13.4 per 100,000 cases

• Age: Incidence increases over the age of 60 years

• Sex: 91% higher incidence in males as compared to females

• Race: Highest among Hispanics: 16.6/100,000

• Non-Hispanic Whites: 13.6/100,000

The Reality of the Last Years of Life: Death Is Not Predictable

(slide courtesy of Joanne Lynn, MD Rand Corp.)Covinsky et al. JAGS 2003;

Lynn & Adamson RAND 2003.Morrison & Meier N Engl J Med 2002.

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CHF, dementia

The Cure - Care Model: The Old System

Life

Prolonging

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Disease Progression

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Palliative/

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www.capc.org

New ModelCenter to Advance Palliative Care

Palliative Care

Modern

MedicineHospice

www.capc.org

Diagnosis of serious illness

Death

Palliative Care’s Place in the Course of Illness

Center to Advance Palliative Care

Life Prolonging Therapy

Palliative CareMedicare Hospice

Benefit

Bunting-Perry, L. (2006). Journal of Neuroscience Nursing, 38(2), 105-112.

Parkinson’s Disease Model of Care

Diagnosis ofParkinson’s Disease

Treatment of PD (Prolongation of Life)

Death

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Palliative Care (Relief of Suffering)

Early P.D.

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Unified Parkinson Rating Scale V: Hoehn & Yahr Score

Stage 0 = No sign of disease

Stage 1 = Unilateral Disease

Stage 1.5 = Unilateral Plus axial involvement

Stage 2 = Bilateral Disease, w/o impairment of balance

Stage 2.5 = Mild Bilateral disease, with recovery on pull test

Stage 3 = Mild to moderate bilateral disease

Stage 4 = Severe disease

Stage 5 = Wheelchair bound or bedridden

Unified Parkinson Rating Scale IV:

Schwab and England ADL Scale

Measure of ADL’s

Scored from:

100% = Completely independent

to

0% = Reflecting vegetative functions

Bunting-Perry, L. (2006). Journal of Neuroscience Nursing, 38(2), 105-112.

Parkinson’s Disease Model of Care

Diagnosis ofParkinson’s Disease

Treatment of PD (Prolongation of Life)

Death

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Palliative Care (Relief of Suffering)

Early P.D.

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Why Palliative Care?

Questions

• What do persons with Parkinson‟s disease say they want from our healthcare system?

• What is the impact of Parkinson‟s disease on families?

Answer

• Palliative care promotes concordance with patient and family wishes

What is Palliative Care?

“Palliative care is interdisciplinary care focused on the relief of suffering and

achievement of the best quality of life for patients and their family caregivers”

(Morrison and Meier, 2003)

WHO Definition of Palliative Care

“An approach which improves the quality of life of the patient and their family‟s facing

life-threatening illness, through the prevention, assessment, and treatment of pain and other physical, psychosocial and

spiritual problems” (World Health Organization 2002)

Palliative Care is Family Care

Who is the family?

“Family is anyone who shows up when illness strikes and

stays on to help”

Carole Levine, 2003

WHO 2003

Goal of Palliative Care• Provides relief from pain and other distressing

symptoms

• Affirms life and regards dying as a normal process

• Neither hastens or postpone death

• Integrates psychological and spiritual aspects of patient care

• Offers support to families

• Enhances Quality of Life

Conclusion

• The Parkinson‟s Disease Model of Care

• Presented the Parkinson‟s Disease Model of Care

• Reviewed U.S. aging demographics

• Defined palliative care as a philosophy of care