ALS

(Amyotrophic Lateral Sclerosis)

Lou Gehrigs Disease

What is ALS?
A neurodegenerative disease. Progressive, rapid, fatal neurological disease.

Motor neurons are responsible for muscle movement.

Motor neuron degeneration leads to paralysis and eventually death. Death 2-6 years after diagnosis.

 ALS causes rapid degeneration and death of both upper & lower motor neurons. Motor neurons are located in the brain, brainstem and spinal cord. Once the neurons die, they are unable to send messages to the muscles.

Unable to function, the muscles slowly weaken, waste away (atrophy) and twitch.

Who Gets ALS?

Every year, approximately 5,000 people in the US are diagnosed with ALS. People of all races and ethnic backgrounds are affected. Most commonly affects people between 40 and 60 years old. More commonly seen in men. In 90%-95% of all ALS cases, the cause is idiopathic. 5%-10% of all ALS cases are inherited (specific genetic defect that leads to mutation of the enzyme known as superoxide dismutase 1 or SOD1).

Steven Hawking

Signs & Symptoms

Early symptoms may include: Twitching Cramping Muscle stiffness or weakness Slurred speech Difficulty chewing or swallowing Exaggerated reflexes (gag reflex) As the disease progresses:
Dysphagia

Dysarthria
Impaired mobility Difficulty breathing due to diaphragm and intercostal muscle weakness (respiratory paralysis)

Clients with ALS will need mechanical ventilation

Treatment
Currently, there is no cure for ALS.

ALS patients have higher levels of glutamate in the serum and spinal fluid.
Riluzole (Rilutek) decreases the release of glutamate. Has been shown to lengthen survival by several months. Prolongs the time before a client needs ventilation support.

 Possible Nursing Diagnosis:

Nursing Care

Ineffective airway clearance related to impaired or absent gag reflex (or absent swallowing reflex)

Assessment:
-Assess gag reflex before giving food or fluid P.O.

Interventions & Planning:

-Elevate the head of the bed -Rest periods before meals -Reduce the risk of aspiration -Deep breathing exercises and use of an incentive spirometer -Maintain on respirator if necessary

Goal:
-Client will demonstrate effective coughing and deep breathing exercises by the end of my 12 hour shift.

Nursing Care
Possible Nursing Diagnosis:
Risk for injury related to use of assistive devices secondary to lower extremity paralysis.

Assessment:
-Assess skin daily -Assess degree of immobility

Interventions & Planning:

-Encourage patient to change positions every two hours -Check for redness of skin after position changes -Assist patient with ROM exercises -Promote exercise program to strengthen unaffected muscles -If patient is confined to a wheel-chair, maintain proper positioning and provide cushion to prevent skin breakdown -Promote sufficient nutritional intake

Goal:
-Client will demonstrate correct use of assistive device(s) by the end of my 12 hour shift.

Article
Pain in Amyotrophic Lateral Sclerosis: A Neglected Aspect of Disease
Chalonda R. Handy, Christina Krudy,Nicholas Boulis, and Thais Federici http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3135011/pdf/NRI2011-403808.pdf

Pain isnt generally associated with ALS. Approximately 70% of ALS patients report pain. Pain thought to be underrated and undertreated. ALS pain is thought to be caused largely by immobility. NSAIDs and Opioid analgesics

References
Feldman, E.L., (2007). Amyotrophic lateral sclerosis and other motor neuron diseases (23rd ed.). Philadelphia, PA: Saunders Elsevier. Handy CR, Krudy C, Boulis N, Federici T. Pain in amyotrophic lateral sclerosis: a neglected aspect of disease. Neurol Res Int. 2011;2011:403808. Epub 2011 May 3. PubMed PMID: 21766021; PubMed Central PMCID: PMC3135011. Lewis, S. L., Heitkemper, M.M., Dirksen, S.R., OBrien, P.G., & Bucher, L. (2010). Medical-surgical nursing: Assessment and management of clinical problems (8th ed.). St. Louis, MO: Mosby Elsevier. www.alsa.org