1. Amyotrophic Lateral Sclerosis (ALS) Jason Conk

2. ALS- Lou Gehrig’s Disease • FACTS • Progressive neurodegenerative disease affecting nerve cells in the brain and spinal cord. • Death of motor neurons leads to loss of muscle movement. • Progression of disease eventually leads to total paralysis. • Progressive degeneration of the motor neurons eventually leads to death. • PURPOSE • The purpose is to evaluate the best available Evidence-Based Practice (EBP) to guide fundamental nursing care in order to provide the most comprehensive care possible for individuals living with ALS.

3. Evidence for Care • Stats • 30,000 Americans affected • Can affect anyone • Caucasians at highest risk • Onset 40-60 y/o • No risk factors • Riluzole only drug for care • Average lifespan only 2-4 years • Manifestations • Muscle Weakness • Fasciculation & cramping of muscles • Difficulty projecting speech and dysphagia • Hyperreflexia • Incoordination • Muscle atrophy

4. Nursing Theory l • Hildegard Peplau • Theory of Interpersonal Relationships • The Seven Nursing Roles • The Stranger Role • The Resource Role • The Teaching Role • The Counseling Role • The Surrogate Role • The Active Leadership Role • The Technical Expert Role

5. Evaluation of the Evidence • Methods • Longitudinal Study • 9 month study • Zung Depression Scale (ZDS) • McGill Quality of Life (MQoL) • Caregiver Burden Inventory (CBI) • Self-Perceived Burden Scale (SPBS)

6. Evaluation of the Evidence • Methods • Longitudinal Study • 9 month study • Zung Depression Scale (ZDS) • McGill Quality of Life (MQoL) • Caregiver Burden Inventory (CBI) • Self-Perceived Burden Scale (SPBS) • Results • ZDS • Patient= Even • Caregiver = Increase • MQoL • Patient= Increase • Caregiver= Decrease • CBI • Patient= Increase • Caregiver= Increase • SPBS • Patient = Increase • Caregiver= Increase

7. Evaluation of Evidence • Article Critique • Not a nursing journal • Not performed by nurses • Is applicable to nursing • Ordinal Level of Evidence • QSEN Level 2 • Threats to validity: Testing, Maturation, Mortality, Hawthorne Effect • Limitations • Small sample size • Many threats to validity • Deterioration of mental function

8. Patient Care Design • Patient • Nurses • Nurse Aides • PCP • Neurology • Radiology • OT • Psychiatry • Respiratory Therapists • Social Workers • Clergy • Hospice/ Palliative Care

9. Case Study • A 51-year-old woman developed an unusual gait related to "calf stiffness." After several months of progressive weakness, she developed a right foot drop. Within 15 months, she also described difficulty in writing, weakness of the right hand and arm, and diffuse muscle twitching with painful muscle cramps. Assessment confirmed weakness of all limbs, more marked on the right side, with muscle atrophy. Reflexes were hyperactive and fasciculations were observed in the right leg. • Within two years, the patient became severely disabled because of generalized weakness. She experienced no sensory problems, intellectual deterioration or skin breakdown in spite of her degree of immobility. Difficulty in swallowing necessitated the use of a gastrostomy tube for feeding. The patient died of respiratory failure approximately three years after reporting the initial symptoms.

10. Patient Care Design- TRUEPIC

11. Patient Care Design- TRUEPIC cont.

12. Patient Care Design • Primary Nursing Diagnosis: • Death anxiety r/t impending loss of function leading to death. • Impaired swallowing r/t weakness of muscles involved in swallowing. • Overall approach to care: • Palliative/ hospice care is the primary care. • Referrals for spiritual/ psychiatric care. • Suctioning and tube feeding in later stages of disease.

13. Advocate Role • Riluzole • Suctioning • Tube feeding • Respiratory care • Palliative care • Referrals • Emotional Support

14. References • Colley, S. (2014). Nursing 441: Nursing Theory [Syllabus] p. 6. • Gauthier, A., Vignola, A., Calvo, A., Cavallo, E., Moglia, C., Sellitti, L., Mutani, R., Chiò A. (2007). A longitudinal study on quality of life and depression in ALS patient–caregiver couples. Neurology, 923-926. • Ladwig, G.B., & Ackley, B.J. (2011). Mosby’s guide to nursing diagnosis (3rd ed.). Maryland Heights, MO: Mosby Elsevier. • Smeltzer, S. C., Bare, B. G., Hinkle, J. L., Cheevver, K. H. (2010). Brunner & Suddarth’s textbook of medical-surgical nursing (12th ed.). Philadelphia, PA: Wolters Kulwer Health Lippincott Williams & Wilkins • Taylor, C., Lillis, C., LeMone, P., and Lynn, P. (2011). Fundamentals of nursing: The art and science of nursing (7th ed.). Philadelphia, PA: Lippincott Williams & Wilkins