Peer Professional Workgroup on Amyotrophic Lateral Sclerosis (ALS)

Field of Work: End-of-Life Care

Problem Synopsis: Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurodegenerative disease that affects about 4,600 Americans each year. There is no cure for ALS, which typically leads to death three to five years after symptoms begin. As the disease progresses, most people suffering from ALS and their families must make decisions about whether and when to forgo active treatment or life-sustaining care. Most ultimately decline aggressive treatment, making palliative care essential.

Synopsis of the Work: The Amyotrophic Lateral Sclerosis (ALS) Workgroup of RWJF's Promoting Excellence in End-of-Life Care national program sought to catalyze rapid change, resulting in improvement in the care of patients dying of ALS and their families. Workgroup members addressed issues related to the end of life in ALS within 12 domains: general psychosocial care; bereavement; spirituality; quality of life; caregiver issues; communication skills; ethics; decision-making; symptom management; access to care; costs of care; and knowledge/education. In each domain, members identified the current state of knowledge, determined markers of ideal care, recommended ways to close the gap between current and ideal care and identified tools for clinicians and other staff.

Key Recommendations

  • The ALS Workgroup recommended:

    • Clinicians should use the following triggers as criteria in deciding to initiate discussions with patients about end-of-life care:
      • The patient or family asks for information or help in addressing the disease or its symptoms.
      • Severe psychological, social or spiritual distress or suffering.
      • Pain requiring high dosages of analgesic medications.
      • Need for a feeding tube.
      • Difficulty breathing or symptoms of hypoventilation.
      • Loss of function in two body regions, such as the arms, legs or facial muscles used for speech or swallowing.
    • Because ALS progresses over time, family members often assume large burdens in caring for people with ALS. Therefore:
      • Clinicians should interview caregivers to identify concerns that require attention.
      • Researchers should develop an instrument to measure caregiver burden. They should then study whether that burden can be reduced by offering services to caregivers before they become overburdened with worry and responsibility.
      • Policy-makers should improve financial and insurance coverage for home-based care.
    • Decisions about forgoing or withdrawing life-sustaining interventions often involve considering beliefs that patients or families may not have previously explored. Therefore:
      • Doctors or others working with families should identify and monitor conflicting issues associated with end-of-life care, including conflicts within families and between patients, families and health care providers.
      • Researchers should study the effects of end-of-life decisions on families and caregivers and on the overall quality of life during the terminal phase of ALS.