Early Onset Parkinson s Disease: What NPs need to know

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Early Onset Parkinson s Disease: What NPs need to know PATRICIA H. COX, DNP, MPH, FNP-BC ASSISTANT PROFESSOR, UNIVERSITY OF PORTLAND VISITING NURSE FACULTY, PARKINSON S FOUNDATION MELODY RASMOR, EdD, FNP, ARNP-BC, COHN-S ASSISTANT CLINICAL PROFESSOR, WASHINGTON STATE UNIVERSITY VISITING NURSE FACULTY, PARKINSON S FOUNDATION

Special thanks The Parkinson s Foundation is working toward a world without Parkinson s disease. Formed by the merger of the National Parkinson Foundation (NPF) and the Parkinson s Disease Foundation (PDF) in August 2016, the mission of the Parkinson s Foundation is to invest in promising scientific research that will end Parkinson s disease and improve the lives of people with Parkinson s, and their families, through improved treatments, support and the best care.

Disclosure statement: We have nothing to disclose during our presentation.

Objectives Define Parkinson s and describe prodromal symptoms Describe the pathophysiology of early Parkinson s Discuss and analyze case studies of early prodromal Parkinson s Explore early interventions that NPs can use to assist their patients Identify the elements of a Parkinson s neuro assessment

Parkinson Disease Defined A neurodegenerative movement disorder- It presents with motor deficits, cognitive, affect challenges, and autonomic nervous system dysfunction. Dopamine depletion, is the hallmark of its motor presentation. This presentation will hopefully provide a better understanding of the pathophysiology of Parkinson and early recognition of the disease.

Facts 4,500 years ago Ancient Indian medicine kampavata 1817 James Parkinson - the shaking palsy PD is the 2 nd most common neurodegenerative disorder after Alzheimer s Disease 1-2 million people in the U.S. 10 million people worldwide The 14th leading cause of death Occurrence expected to increase two-fold by 2005-2030 in individual aged > 50 years

Costs Exceeds 14.4 billion dollars in U.S. More than 6 billion dollars in direct medical costs Direct medical costs per patient estimated 10-14,000 dollars annually Direct + Indirect costs approximately 22,800 dollars annually

Disability Reduced quality of life Impairment of activities of daily living (walking, dressing, shopping) Impairment of instrumental activities of daily living (meal preparation, money management, medication management) Social isolation (embarrassed over physical symptoms of disease)

Demographics Gender differences- Men>women Younger age of onset- more symptoms Age not related to severity or progression 2-3% increase in older adults >65 years

Risk Factors Advancing Age Men more likely than Women Heredity- Having a close relative with Parkinson's increases the chances that you'll develop the disease. Risks still small unless you have many relatives in your family with Parkinson's. Environmental Exposure- herbicides and pesticides may put you at a slightly increased risk of Parkinson's disease.

Risk Factors

Risk Factors

Parkinson s Definition Neurological Motor Disease Affects Dopamine Neurons in Substantia Nigra Pars Compacta Symptom onset correlates with advanced neurodegeneration

Parkinson s Disease-4 Cardinal Symptoms Tremor (resting) Muscle Rigidity Bradykinesia Postural Instability

Parkinson Disease Motor Clinical Manifestations Decreased arm swing when walking Micrographia (small hand writing) Hypophonia Masked face, decreased blinking Shuffling, slow gait Stooped posture

CARDINAL SYMPTOMS OF PARKINSON S DISEASE https://www.youtube.com/watch?v=d0nyc1jicl A

Pathophysiology The substantia nigra pars compacta is located deep in the mid brain. These neurons are large dopamine neurons that degenerate in PD Literally all patients receive a late diagnosis since motor symptoms, required for diagnosis, are manifested when approximately 60% of the neurons of the substantia nigra pars compacta (SNpc) are lost

SUBSTANTIA NIGRA PARS COMPACTA

How Is Prodromal (Early) Parkinson s Disease Different? Non-Motor Neurological Disease Preceded PD Diagnosis Affects Dopamine Neurons in the SNpc Characterized by premotor PD features Onset prior to advanced neurodegeneration

Life course of disease and need for early diagnosis

Why isn t it identified earlier? Mean delay 3-6 years from first symptoms, some for 10+ years Underreporting & Under-recognition of NMS Lack of PCP systematic questioning & knowledge Lack of inter-professional collaboration I m just getting old. The great copycat Knowledge inadequacies

Prodromal Symptoms Olfactory impairment(hyposmia)-90% REM Sleep Behavior Disorder Autonomic Dysfunction Anxiety/Depression/Apathy Mild Cognitive Impairment (MCI) Fatigue Hallucinations Restless leg (Akathisia) Diffuse Pain Seborrhea/melanoma Decreased blink response

Movement Disorder Society (MDS) Prodromal PD Criteria Diagnosis of prodromal PD relies on clinical nonmotor markers Speed of progression from prodromal PD to PD is unpredictable Criteria is based on estimated risks and symptoms Criteria development relied on prospective studies Estimates criteria predicts prodromal PD with >80% likelihood

Prodromal PD in Primary Care Increased Awareness of Prodromal PD Put Algorithm here Evidence is only informative Diagnosis still relies on MDS Clinical Criteria for PD Proposed Algorithm

Why NPs need to know more Early Parkinson s-vague symptoms Patients frustrated with multiple diagnosis Complex disease with no diagnostic test Lack of diagnosis, delayed referrals, delayed treatment

Why should we evaluate patients with Non-motor symptoms? Time to plan Physiotherapies to prolong quality of life NPs incorporate patient s feelings and thoughts Early referral allows for neurology evaluation and possible treatments/studies

How can NPs evaluate patients early to make a difference? Know your patient s normal to detect subtle changes Broaden your differential for olfactory/gustatory changes Mimics other disease processes: Sleep disturbances, IBS, essential tremor, stress incontinence, depression, dementia, metabolic diseases, cerebrovascular disease, Lewy body disease, Huntington disease, multisystem atrophy-so think Parkinson s

Time for a Case Study So you have a 50 something year old white female coming to see you and she discloses she is in the process of getting a divorce and she is depressed. She reports that her boss has noticed some postural changes and shuffling. She has no tremor, no constipation and she does Jazzercise 2-3 x week. She has HTN, Thyroid Disease, Metabolic syndrome, is Vitamin D deficient and she is postmenopausa1. She works full time, is a non-smoker and drinks a glass of wine per night.

The Michael J Fox Foundation https://www.michaeljfox.org/foundation/newsdetail.php?watch-our-latest-video-what-isparkinson-disease

Here is the Neuro Exam Conduct a dexterity test-ask patient to finger tap. Maintained speed over 10-15 seconds and maintained amplitude. Slowing speed, decreasing amplitude, pauses/arrests in finger and foot taps are consistent with bradykinesia Observe walking-gait, ataxia, freezing, limitations in turning Observe for reduced arm swing Observe rising out of a chair. Perform the Pull Test. www.toolkit.parkinson.org/sites/toolkit.parkinson.org/files

Questions?

Acknowledgements Gwyn M. Vernon, M.S.N., R.N., C.R.N.P. Co-Founder and National Director, Parkinson's Disease Foundation The Edmond J. Safra Visiting Nurse Faculty Program Nikkiel Lefebre, RN, BSN, MN, FNP Elizabeth Teeling, BSc, RN-BSN, MN, FNP

References Ackley, B. J. & Ladwig, G. B. (2014). Nursing Diagnosis Handbook: An evidence-based guide to planning care. Maryland Heights, MO: Mosby-Elsevier. Bach, J., Ziegler, U., Deuschl, G., Dodel, R. & Doblhammer-Reiter, G. (2011). Projected numbers of people with movement disorders in the years 2030 and 2050. Movement Disorders, 26(12), 2286-2289. Berg, D., Postuma, R. B., Adler, C. H., Bloem, B. R., Chan, P., Dubois, B., &... Deuschl, G. (2015). MDS research criteria for prodromal Parkinson's disease. Movement Disorders, 30(12), 1600-1611. doi:10.1002/mds.26431 Chen, H., Burton, E., Ross, W., Huang, X., Savica, R., Abbott, R., Zhang, J. (2013). Research on the premotor symptoms of Parkinson s disease: clinical and etiological implications. Environmental Health Perspectives, (121)12: 1245-1252. Cronin-Golomb, A. (2013). Emergence of nonmotor symptoms as the focus of research and treatment of Parkinson s disease: Introduction to the special section on nonmotor dysfunctions in Parkinson s disease. Behavioral Neuroscience, 127(2), 135-138. doi: 10.1037/a0032142 Foster, E. (2014). Instrumental activities of daily living performance among people with Parkinson s disease without dementia. American Journal of Occupational Medicine, 68(3), 353-362. Hoyert, D. L., & Xu, J. (2012). Deaths: Preliminary Data for 2011. National Vital Statistics Report 61(6), 1-52. Retrieved from: http://www.cdc.gov/nchs/data/nvsr/nvsr61/nvsr61_06.pdf Kieburtz, K. & Wunderle, K. (2012). Parkinson s disease: Evidence for environmental risk factors. Movement Disorders, 28(1), 8-13. Kowal, S. L., Dall, T. M., Chakrabarti, R., Storm, M. V. & Jain, A. (2013). The current projected economic burden of Parkinson s disease in the United States. Movement Disorders, 28(3), 311-318.

References Parkinson s Disease Foundation (2016). Diagnosis. Retrieved from: http://www.pdf.org/en/diagnosis Postuma, R., Berg, D., Stern, M., Werner, P., Olanow, C., Oertel, W., Deuschl, G. (2015). MDS clinical diagnostic, criteria for Parkinson s disease. Movement Disorders, 30(12), 1591-99 Postuma, R. B., Pelletier, A., Berg, D., Gagnon, J. F., Escudier, F., & Montplaisir (2015). Screening for prodromal Parkinson s disease in the general community: A sleep-based approach. Sleep Medicine 21(2016), 101-105. doi: 10.1016/j.sleep.2015.12.016 Shulman, L. (2010). Understanding disability in Parkinson s disease. Movement Disorders, 25(1), 131-135. Tolosa E, Gaig C, Santamaría J, & Compta Y. (2009). Diagnosis and the premotor phase of Parkinson disease. Neurology, 72, S12 20 1p. http://doi.org/10.1212/wnl.0b013e318198db11 University of Oxford (2014). Healthtalk.org. Parkinson s disease: Path to diagnosis. Retrieved from: http://www.healthtalk.org/peoples-experiences/nerves-brain/parkinsons-disease/path-diagnosis U.S. Department of Health and Human Services Centers for Disease Control and Prevention, 2012 Wright-Willis, A., Evanoff, B. A., Lian, M., Criswell, S. R., & Racette, B. A. (2010). Geographic and ethnic variation in Parkinson disease: A population-based study of US Medicare beneficiaries. Neuroepidemiology, 34(3), 143-151. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/pmc2865395/

Melody with Parky and Nikkiel Lefebre