Invited Review: Amyotrophic Lateral Sclerosis and Palliative Care: Where We Are, and the Road Ahead-31Amyotrophic Lateral Sclerosis and Palliative Care

31Amyotrophic Lateral Sclerosis and Palliative Care

Pdf Summary

Amyotrophic lateral sclerosis (ALS), a progressive neuromuscular disorder, presents high symptom burdens for patients including pain, fatigue, dyspnea, and sialorrhea. It also takes its toll on family caregivers who struggle to meet the physical, financial, and emotional demands associated with the illness. While multidisciplinary care has improved the quality and length of life for ALS patients, there is still much to be done in terms of symptom management. Pain, in particular, remains poorly studied in ALS patients. Palliative care specialists should be involved in the ALS multidisciplinary team to further improve the quality of life for patients and their families. <br /><br />Some of the common symptoms present in ALS patients include pain, depression, anxiety, and sialorrhea. Pain is caused by muscle atrophy and weakness, as well as muscle cramping and spasticity. Depression and anxiety can be caused by physical symptoms, and patients may also experience pseudobulbar affect, which includes episodes of laughing and crying. Sialorrhea, or excessive drooling, can cause discomfort, embarrassment, and complications such as aspiration of saliva. <br /><br />Treatment options for these symptoms include non-pharmacological methods, such as positioning and suctioning, as well as medication. Anticholinergic medications are commonly used to treat sialorrhea, but they may have side effects such as sedation and delirium. Botulinum toxin injections into the salivary glands and low-dose radiation therapy have also been shown to be effective in treating sialorrhea in some cases. <br /><br />Non-invasive positive pressure ventilation (NIPPV) has been proven to improve quality and length of life in ALS patients with severe dyspnea, and opioids such as morphine can be used to manage severe dyspnea in patients who do not use NIPPV. LTMV (long-term mechanical ventilation) can also prolong life for more than 10 years in ALS patients, but patients must decide if life prolonged in this manner is worth living. Communication and discussions about end-of-life care and treatment options are crucial in helping patients and their families make informed decisions. Palliative care specialists can provide education, support, and symptom management throughout the course of the illness.

Keywords

Amyotrophic lateral sclerosis

ALS

neuromuscular disorder

symptom burdens

pain

fatigue

dyspnea

sialorrhea

family caregivers

multidisciplinary care

amyotrophic lateral sclerosis

ALS