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amyotrophic

lateral

sclerosis

Sarah Sward

A progressive neurodegenerative disease that affects neurons in the brain and spinal cord that is likely caused by genetic mutations.

Examples of clinical situations involving

ALS

Initial/ Early Onset:

- Primary Health Care Physician visit for examination of muscle weakness

- Neurologist consult for further investigation

- Emergency Department after fall or injury because of muscle weakness

Late Stages:

- During homecare visits when ADLs can no longer be completed

- In Hospice at end of life with possible pain management and aspiration precautions

Clinical Manifestations

Objective

Subjective

- Fatigue

- Muscle weakness

- Depression

- Anxiety

- Fatigue

- Muscle atrophy (including tongue)

- Twitching of face and tongue

- Dysarthria ( Difficulty speaking due to slurred speech)

- Dysphagia ( Difficulty swallowing)

- Stiff and clumsy gait

- Abnormal reflexes

Pathophysiology

of ALS

Chronic neurologic disease of unknown cause (genetic and environmental factors identified) causing progressive muscle weakness and wasting, leading to paralysis of respiratory muscles

Diagnostic Testing and Labs

Urine collection for heavy metals

abnormal results: Protient called p75ECD elevation

Spinal tap

abnormal results: elevated levels of neurofilaments; usualy done for patients who have abnormal ALS symptoms to exclude inflammatory nerve conditions

X-rays, including magnetic resonance imaging (MRI)

abnormal results: used mainly to rule out pressure on the spinal cord or major nerves, MS or tumors

Muscle and/or nerve biopsy

abnormal results: Shows level of degeneration of muscle tissue allowing for stage diagnosis of ALS; ALS patients tend to show more severe myopathic changes over a shorter period of time

A thorough neurological examination

abnormal results: weakened or decreased reflexes and muscle strength

Blood and urine studies, including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour

abnormal results: elevated or decreased T4 ( thyroxine), Hyperactive parathyroid hornmone, elevated Creatinine kinase, HIV positive

Electrodiagnostic tests, including electomyography (EMG) and nerve conduction velocity (NCV)

abnormal result : spontaneous electrical activity when the muscle is at rest

Nursing Interventions

- Encourage daily fluid intake of 2,500cc’s per day unless contraindicated because of swallowing ability.

- Discuss techniques for protecting airway, i.e., sitting up straight, putting chin on chest when swallowing, concentrating while eating. Avoid increased stimulation, i.e., talking at mealtime.

- Elevation of the head of the bed with a foam wedge or hospital bed to ease shortness of breath and provide comfort during sleep.

- Encourage realistic expectations of the disease whenever possible.

Medications

Riluzole / Rilutek: used to slow the disease.

Action may be related to:

Inhibition of glutamate release,

Inactivation of sodium channels or,

Interference with neurotransmitter binding at receptor sites.

Side Effects:

- Dizziness

- Weakness

- Abdominal Pain

- Nausea

- Hepatotoxicity

- Interstitial lung disease

- Anaphylaxis

Health Promotions

-Provide intellectual stimulating activities, because the client typically experiences no cognitive deficits and retains mental abilities.

-Promote client and family coping as the client and his family deal with the poor prognosis and the grieving process

- Maximize functional abilities

Prevent complications of immobility

Promote self-care

Maximize effective communication

- Prevent respiratory complications

Promote measures to maintain adequate airway

Promote measures to enhance gas exchange, such as oxygen therapy and ventilator assistance.

Promote measures to prevent respiratory infection

Resources

(2023, October 17). Amyotrophic lateral sclerosis ALS. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis als#:~:text=A%20neurologic%20examination%20will%20test,help%20support%20an%20ALS%20diagnosis.

Barbieri, E. (2022). How does an ALS muscle biopsy help researchers? Target ALS Foundation. https://owl.purdue.edu/owl/research_and_citation/apa_style/apa_formatting_and_style_guide/reference_list_electronic_sources.html

Ignatavicius, D. D., Workman, M.L., Rebar, C.R., & Heimgartner, N.M. (2021). Medical-surgical nursing: Concepts for interprofessional collaborative care. Elsevier Inc. ISBN: 978-0-323-61242-5.

Ozkaya, O. (2017, February 24). Simple urine test could help diagnose, track ALS progression. ALS News Today. https://alsnewstoday.com/news/urine-test-als/#:~:text=ALS%20currently%20has%20no%20validated,the%20urine%20of%20ALS%20patients.

Vallerand, A.H., & Sanoski, C.A. (2023). Davis’s Drug Guide for Nurses, 18th ed., F.A. Davis.

https://www.drugguide.com/ddo/view/Davis-Drug-Guide/51666/all/riluzole#5 .

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