Amyotropic Lateral Sclerosis (ALS)

1. AmyotropicLateral Sclerosis(ALS) Jerry Carley RN, MSN, MA, CNE AH II Summer 2010

2. Concept Map: Selected Topics in Neurological Nursing PATHOPHYSIOLOGY Traumatic Brain Injury Spinal Cord Injury Specific Disease Entities: Amyotropic Lateral Sclerosis Multiple Sclerosis Huntington’s Disease Alzheimer’s Disease Huntington’s Disease Myasthenia Gravis Guillian-Barre’ Syndrome Meningitis Parkinson’s Disease ASSESSMENT Physical Assessment Inspection Palpation Percussion Auscultation ICP Monitoring “Neuro Checks” Lab Monitoring PHARMACOLOGY --Decrease ICP --Disease / Condition Specific Meds Care Planning Plan for client adl’s, Monitoring, med admin., Patient education, more…based On Nursing Process: A_D_P_I_E Nursing Interventions & Evaluation Execute the care plan, evaluate for Efficacy, revise as necessary

3. ALSis also known as Lou Gehrig's Disease, after the famous baseball player who died of the disease in 1941

4. Characteristics of ALS • Disease of the motor system • Progressive muscle atrophy • Fatal (2 – 5 years) d/t respiratory failure

5. Characteristics • Most commonly diagnosed in 40 - 70s • Affects men more often than women • The disease has no racial, socioeconomic, or ethnic boundaries

6. What’s in a Name ? • Amyotrophy refers to the atrophy (progressive muscle wasting) • Lateral sclerosis refers to demyelination followed by hardening of the spinal column from buildup of scar tissue (sclerosis = scar) • As the disease progresses, it will move up the affected leg or arm until eventually all muscle groups become involved. This spread into all muscle groups is the defining characteristic of ALS

8. Why ? ... Etiology….. • Mitochondrial dysfunction? Genetic defect (chromosome 21) suggests the disease is inherited and accounts for 5 to 10% • Environmental factors, since the disease tends to cluster in geographical pockets? (Extremely high incidence of ALS has been observed in Guam and the Trust Territories of the Pacific ) • Free radical damage? • Glutamate excitotoxicity? (Apoptosis / Programmed Cell Death)

9. Signs and Symptoms • Difficulty swallowing (dyphagia) • Slurred speech (dysarthria) • Fatigue • Fasciculations of tongue (twitching) while at rest • Usually the first muscles affected are those in the arms and legs (Walking or climbing stairs may be difficult, may drop things, fall, experience muscle cramps. The arms and legs may feel especially tired. If the hands are affected, may have difficulty picking up small objects or turning keys)

10. Diagnostics • EMG / NCV studies - NCV is administered before EMG and measures the speed at which nerves transmit electrical signals - EMG measures nerve impulses within the muscles • Muscle Biopsy • Tests to rule out other neurological disorders - MRI may be used to rule out spinal cord diseases - Blood tests may be done to detect the presence of heavy metals such as lead, abnormal proteins or hormone levels associated with other neurological diseases - Lumbar puncture to analyze the cerebrospinal fluid for genetic abnormalities (e.g., viral, autoimmune, neurotoxic)

11. Renowned scientist Stephen Hawking suffers from amyotrophic lateral sclerosis

12. Late Stage… • As disease progresses and more muscle groups are affected, the person becomes progressively incapacitated • When respiratory muscles weaken, the client will require a ventilator • Percutaneous Endoscopic Gastrostomy (PEG) or feeding tube • ALS patients often experience fear, anxiety, & depression ** Ability to think or reason remain intact !

13. Collaborative Goals • Focus on maintaining quality of life • Control symptoms • Prevent complications • Provide adaptive devices to increase mobility and self-care

14. Collaborative Team • Physical therapy helps to relieve cramping and muscular pain. Passive stretching helps to avoid permanent contraction of muscles (contractures) that may cause joint problems • Dietician ensures diet of high-energy foods that are easy to swallow • Splints, braces, and wheelchairs are used to help with mobility • Occupational and Speech therapy as their motor control gradually deteriorates

15. Medication Riluzole (Rilutek®) is one of the few drugs effective against ALS and may prevent progression and prolong life for a few months or so…

16. Medication Antispasmodics: - Baclofen (Lioresal) - Damtrolene sodium (Dantrium) - Diazepam (Valium)

17. Meds… • Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen may relieve general discomfort • Tramadol (Ultram®) is often prescribed for pain relief

18. Depression • Very common • Antidepressant medication and counseling can help patients and their families cope

19. Affairs in Order… • While it may be emotionally difficult, it is important for clients and caregivers to make informed, carefully considered decisions regarding the future while the patient is capable of making his or her contribution to a planned course of action • Patients and their family members should discuss and consider issues such as legal concerns, home care, assisted care, and institutionalization • Draw up wills and other important documents as early as possible to avoid legal problems later on, when the patient may be unable to represent his or her own interests • Legal assistance may be necessary if the patient encounters discrimination over insurance or employment.

20. Prognosis • Fifty percent of patients die within 3 years of diagnosis • 20% live 5 years • 10% live 10 years • Hospice care can provide comfort and dignity to patients and their loved ones