Treatment 

No cure is available for ALS. However, riluzole is believed to reduce the damage to motor neurons by decreasing the release of the excitatory neurotransmitter glutamate. Another drug, edaravone, is a free radical scavenger that is thought to decrease oxidative stress on neurons in ALS. Both drugs claim to slow the progression of ALS, and studies reveal modest results.34,35

Symptom management and palliative care

A neurologist and a multidisciplinary team are required for the management of ALS. Whenever possible, primary care clinicians should refer the patient to a specialized ALS clinic. Treatments to manage the various symptoms can improve the quality of life (Table 2). Medications to reduce fatigue, relieve muscle cramps, control spasticity, and reduce excessive oral secretions can be beneficial. Gentle, low-impact aerobic activity and range-of-motion and stretching exercises can help to prevent painful muscle spasticity and contractures. When respiratory muscles weaken, intermittent positive pressure ventilation may be used to ease the work of breathing during sleep. Most patients remain asymptomatic until the forced vital capacity (FVC) is less than 50% of the predicted level as a result of respiratory muscle weakness. The patient’s preferences regarding life-sustaining treatment and approaches to palliative care should be discussed soon after the diagnosis of ALS, and any decisions should be reconsidered on several occasions before respiratory failure occurs. 

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Conclusion

The primary care clinician may be involved early in the ALS disease process. Often, the diagnosis is delayed because the clinical presentation is unclear at an early stage. The primary care clinician may be involved in managing the symptoms of a patient with ALS and serve as a source of support for the caregiver(s). Patients require multidisciplinary care and rehabilitative interventions; maximizing independent function and quality of life are the goals. Family members, particularly spouses, commonly care for patients with ALS. The caregiver role frequently causes strain, which generally increases as the disease process progresses. Continued support and education from the primary care provider are essential for caregivers. Currently, numerous investigations into the genetic basis of ALS and the development of drugs to diminish the progressive degeneration associated with this disease are under way. 

Teri Capriotti, DO, MSN, CRNP, is a clinical associate professor, Erin Donnelly is an honors student, and Simone Brissenden is an honors student at Villanova University, PA.

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