New ALS guideline establishes national standard for managing neurodegenerative disease

Nov 17, 2020

The first Canadian guideline for the care and management of patients with amyotrophic lateral sclerosis -- Lou Gehrig disease -- recommends a patient-focused approach, with attention to holistic and emotional aspects of well-being.

The guideline, published in CMAJ (Canadian Medical Association Journal), is intended for amyotrophic lateral sclerosis clinicians, allied health professionals and primary care providers, and contains an easy-to-reference table with comprehensive recommendations. As new evidence may change management, the guideline will be updated every 5 years.

"These best practice recommendations are an important step forward in improving the lives of people living with amyotrophic lateral sclerosis across the country and supporting their caregivers by addressing important issues," says Dr. Christen Shoesmith, neurologist and Motor Neuron Diseases Clinic director at London Health Sciences Centre in London, Ontario, and chair of the amyotrophic lateral sclerosis guideline working group. "The emphasis on expert consensus relative to evidence-based recommendations highlights the need for more research in amyotrophic lateral sclerosis management and underscores the challenges amyotrophic lateral sclerosis clinicians face in managing patients with this complex and devastating disease."

About 3000 Canadians live with amyotrophic lateral sclerosis, a debilitating degenerative disease affecting the brain and spinal cord that ultimately results in paralysis. Amyotrophic lateral sclerosis has no cure, and four out of five people with amyotrophic lateral sclerosis will die within five years of being diagnosed.

The guideline is based on the best available evidence as well as expert consensus when evidence is lacking. It is intended to guide Canadian clinicians through issues unique to Canada and to update previous guidelines published in the United States and Europe.

Communications -- tailor the initial discussion about diagnosis to the patient's needs; information about amyotrophic lateral sclerosis prognosis does not need to be provided at the time of diagnosis

Disease-modifying therapies -- have an open discussion with patients about the potential risks and benefits of both approved and unapproved therapies

Multidisciplinary care -- refer patients to specialized amyotrophic lateral sclerosis multidisciplinary clinics for optimized health care delivery

Caregivers -- be attentive to the needs of caregivers and involve them in care planning

The guideline provides detailed recommendations for managing symptoms including pain, cramps, sleep disturbances, depression and anxiety as well as recommendations for respiratory management, nutrition, exercise, medication alignment, palliative care and more.

The authors emphasize the need for more research into amyotrophic lateral sclerosis treatment and the challenges in caring for people with this disease.

Funding for the guideline was provided by the ALS Society of Canada and the Canadian ALS Research Network (CALS).

Listen to a podcast https://soundcloud.com/cmajpodcasts/191721-guide.

"This guideline will enable amyotrophic lateral sclerosis clinics across Canada to meet a common national standard, and to adapt as this standard continues to evolve over time. In doing so, amyotrophic lateral sclerosis clinicians can offer the best possible care to their patients and help them to navigate this exceedingly complex and devastating disease," conclude the authors.

Source: News Release
Canadian Medical Association Journal
November 16, 2020