Amyotrophic Lateral Sclerosis (ALS) – Care Guideline

The revised guideline Care for Patients with ALS (2009) was updated and reissued in 2022. It was developed based on challenges experienced by healthcare professionals, patients, and their families, as well as scientific literature on the topic. The multidisciplinary working group and patient representatives formulated additional recommendations tailored to practical needs. The Dutch Integral Cancer Center (IKNL) served as a process facilitator, as it does for palliative care guidelines, including this one.

About ALS

Amyotrophic Lateral Sclerosis (ALS) is a progressive disease where motor neurons in the spinal cord, brainstem, and brain gradually die. These neurons control the body’s muscles. As the neurons lose their ability to transmit signals, the disease causes increasing muscle weakness. Early symptoms often include reduced strength in the arms or legs or difficulty speaking, swallowing, or breathing. With reduced signals from the neurons, muscles begin to atrophy. The disease leads to growing functional limitations. When the motor neurons have completely degenerated, the brain can no longer control the muscles, resulting in paralysis.

Although ALS typically leads to death within three years of symptom onset, about 20% of patients survive five years or longer. Life expectancy is shorter in older patients and in cases with a bulbar onset compared to younger patients with a spinal onset of ALS.

Purpose

This guideline aims to improve care for people with ALS by providing clarity on how care should be delivered and organized. It focuses on how to best support patients across the four dimensions of care: physical, psychological, social, and spiritual.

Target Population

The guideline addresses (palliative) care for people with ALS throughout the diagnostic, supportive, and end-of-life phases. It also includes, where possible, care for family members, although they are not the primary target group. The recommendations may also apply to patients with Progressive Spinal Muscular Atrophy (PSMA) and Primary Lateral Sclerosis (PLS). However, ALS generally progresses more rapidly than PSMA or PLS. Healthcare providers must assess whether the recommendations are applicable to conditions other than ALS.

Target Audience

This guideline is intended for a wide range of professionals involved in ALS care, including neurologists, rehabilitation physicians, social workers, chaplains, respiratory physicians, nurses, nurse practitioners, caregivers, physician assistants, psychologists, neuropsychologists, general practitioners, gastroenterologists, geriatric specialists, pulmonologists, physiotherapists, occupational therapists, speech therapists, dietitians, palliative care consultants, volunteers, and volunteer coordinators working in palliative and end-of-life care.