Treating amyotrophic lateral sclerosis (ALS)

There is no definitive treatment for amyotrophic lateral sclerosis (ALS). Most treatment regimens are supportive and symptom-relief oriented with a focus on airway management and feeding.

Medications to prevent the decline

While most treatments for ALS only relieve symptoms, riluzole is a bit different. Riluzole is recommended for ALS and can somewhat slow disease progression, potentially extending life by a few months to a year. Riluzole acts to diminish glutamate release by blocking specific sodium ion channels. Glutamate is a neurotransmitter that is cytotoxic to nervous system cells in increased concentrations.

Other experimental medications are on the horizon. Several researchers seek to alter glutamate availability and toxicity through various mechanisms.

Edaravone has also been invoked in the care of patients with ALS and should be considered. This medication is a free radical scavenger and is thought to slow functional decline in patients with ALS.

Medications for stiffness and cramping

Stiffness and cramping are common complaints from patients with ALS. These symptoms can be treated with various muscle relaxants such as baclofen and tizanidine.

Medications for depression

Depression is common in patients with ALS, and selective serotonin reuptake inhibitors (SSRIs) should be strongly considered. The disease carries a level of certitude and hopelessness both for the patients and their loved ones.

Other therapies on the horizon involve the removal or suppression of mutated superoxide dismutase 1 (SOD1) proteins in motor neurons. Research has also been conducted on the replacement of lost motor neurons with stem cells.

Taking another approach, brain-digital interfaces may promise some hope by allowing patients with ALS to control robotic exoskeletons with thought. For the time being, however, most interventions for ALS are supportive.

If available, ALS is ideally treated in specialized multi-disciplinary clinics that offer tracheostomy, mechanical ventilation, and feeding tube support. Aggressive physical, speech, and occupational therapy should be employed to optimize the patient’s mobility and activities of daily living.

Respiratory complications are the main cause of death for these patients. Both respiratory drive and airway clearing are compromised by ALS. Consider noninvasive ventilatory support, such as a continuous positive airway pressure (CPAP) machine, particularly at night for patients beginning to exhibit respiratory failure.

Tracheostomy and mechanical ventilation may have to be considered with the progression of ALS. Some authors recommend a relatively early transition to tracheostomy and mechanical ventilation. However, many patients elect not to resort to this level of support and dependency.

Lower cranial nerve dysfunction leads to increasing difficulties with swallowing. Therefore, aspiration becomes a persistent challenge and many patients tend to become poorly nourished. Good nutrition is therefore a mainstay of supportive care.

Consult nutritional services early and consider placing a feeding tube to maintain good nutrition in your patient. A feeding tube should be considered relatively early in the disease progression. Percutaneous endoscopic gastronomy (PEG) tubes are preferred since nasogastric tubes increase the chances of aspiration in patients with ALS.

Psychosocial and behavioral difficulties are also common with ALS and may include inappropriate behaviors, impaired decision making, involuntary laughing, and crying. Reach out to psychosocial support systems early to initiate psychiatric and psychological support.

Other management challenges must be considered as the disease progresses and additional symptoms arise:

• Hypersalivation and drooling
• Thickened secretions
• Limb stiffness
• Anxiety
• Pain
• Skin breakdown
• Anorexia
• Sleep difficulties

Nursing care facility placement may become necessary. Families caring for a patient at home will need extensive support. Social services should become involved early in the course of the disease.

Sadly, most patients with ALS die within 3–5 years. Therefore, it is incumbent on the provider to encourage contact with end-of-life services and experts relatively early in the course of the disease.