Amyotrophic Lateral Sclerosis

ALS is a rare, fatal progressive degenerative disease that affects pyramidal motor neurons, usually begins in middle age, and is characterized especially by increasing and spreading muscular weakness. (Also called Lou Gehrig’s disease)


Patients will be considered to be in the terminal stage of ALS if they meet the following criteria (must fulfill 1, 2, or 3):

1. The patient must demonstrate critically impaired breathing capacity with ALL of the following characteristics in the past 12 months preceding initial hospice certification:

  • Vital capacity (VC) <30% of normal
  • Significant dyspnea at rest
  • Requiring supplemental 02 at rest
  • Patient declines artificial ventilation

OR

2. Patient must demonstrate BOTH:

A. Rapid progression of ALS as demonstrated by ALL of the following within the 12 months preceding initial hospice certification:

  • Progression from independent ambulation to wheelchair or bed-bound status
  • Progression from normal to barely intelligible or unintelligible speech
  • Progression from normal to pureed diet
  • Progression from independence in most or all activities of daily living (ADLs) to needing major assistance by caretaker in all ADLs.

B. Critical nutritional impairment as demonstrated by ALL of the following within 12 months preceding initial hospice certification:

  • Oral intake of nutrients and fluids insufficient to sustain life
  • Continuing weight loss
  • Dehydration or hypovolemia
  • Absence of artificial feeding methods

OR

3. BOTH of the following:

A. Rapid progression of ALS (2.A. above)

B. Life-threatening complications as demonstrated by ONE of the following within the last 12 months preceding initial hospice certification:

  • Recurrent aspiration pneumonia (with or without tube feedings)
  • Upper UTI, e.g., Pyelonephritis
  • Sepsis
  • Recurrent fever after antibiotic therapy
  • Decubitus ulcers, multiple, Stage 3-4

In end-stage ALS, two factors are critical in determining prognosis: ability to breathe, and to a lesser extent, ability to swallow.

If a patient meets the medical criteria above, they are by definition eligible to receive hospice services. Some patients may not meet the criteria, but may still be eligible for hospice care due to comorbidities or rapid functional decline. Contact your local Compassus program for further information.