Amyotrophic lateral sclerosis and palliative care: Where we are, and the road ahead

Abstract

Patients with amyotrophic lateral sclerosis (ALS) have high symptom burdens, including pain, fatigue, dyspnea, and sialorrhea, and they must make difficult decisions about the use of life-prolonging therapies, such as long-term mechanical ventilation. The impact of ALS is also felt by family caregivers who often struggle to meet the heavy physical, financial, and emotional demands associated with the illness. Expert multidisciplinary care may improve both quality and length of life of patients with ALS. However, although advances have been made in the treatment of some symptoms, others, including pain management, remain poorly studied. Involvement of palliative care specialists as part of the ALS multidisciplinary team is recommended, as we continue to work toward improving the quality of life for patients and their families.

Hypnosis-based psychodynamic treatment in ALS: a longitudinal study on patients and their caregivers

“…Potential executive dysfunction has been described, susceptibly to demographic variables (Palmieri et al, 2014), in a relevant proportion of cases (Abrahams, 2013). The disease is also featured by secondary symptoms such as pain (Pagnini et al, 2012a), sleep disorders (Blackhall, 2012), emotional lability (Palmieri et al, 2009) and fasciculations (Rana et al, 2009), that generate from mild to severe discomfort in everyday living. In the late stages of the disease, progressing muscular degeneration can evolve in a “locked-in” state in which conscious patients becomes progressively paralyzed and voiceless (Borasio et al, 2001).…”

Efficacy of Incobotulinumtoxin Type A (Xeomin®) in the Management of Sialorrhea in Neurodegenerative Diseases

“…Also the caregivers benefit from treatment because the sialorrhea may require a continuous health care, so a distress for patient and for those who takes care of him [31,32].…”

A Review of Options for Treating Sialorrhea in Amyotrophic Lateral Sclerosis

“…Notwithstanding this isolated finding, nearly one third of treated patients do not respond to anticholinergic drugs, and even in the presence of an adequate response in the earlier phases of the disease, for a majority of patients, these medications are often not a safe or sustainable therapy for drooling. 14,41 Additionally, all anticholinergic drugs are contraindicated in the presence of heart diseases, glaucoma, pyloric stenosis, prostatic hypertrophy, and hepatic and/or renal insufficiency, and particular care must be taken in elderly patients, thus limiting their widespread adoption for the treatment of sialorrhea in ALS.…”

“…In 14 subjects treated with a single fraction of 7.5 Gy, Neppelberg et al 60 observed a 50% reduction in salivary secretion after 2 weeks and a 20% reduction after 3 months. It has been suggested that a dose of 8 Gy delivered in a single fraction may be as effective and safe as higher fractionated doses, 14 and increasing the dose did not improve initial achievement. 61 A review of published studies on BoNT and radiotherapy for sialorrhea in ALS attempted to evaluate and compare the effectiveness and side effects of the 2 treatments.…”

“…[11][12][13] About half of all patients affected by ALS report significant sialorrhea or drooling at some point during the course of the disease, and ϳ20% of them have moderate-to-severe symptoms. 14 There are different scales for the assessment of sialorrhea. The revised Amyotrophic Lateral Sclerosis Functional Rating Scale, a widely used and validated instrument to monitor disease progression in ALS, contains 3 items assessing the function of bulbar muscles, including one evaluating sialorrhea.…”