DISEASE INDICATORS

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Indicators and limitations of coverage and/or necessity

Medicare Qualifications

Patients will be considered to be in the terminal stage of ALS (life expectancy of six months or less) if they meet the following criteria (must fulfill 1, 2 or 3).

  1. The patient must demonstrate critically impaired breathing capacity:
  2. Critically impaired breathing capacity as demonstrated by all of the following characteristics occurring within the 12 months preceding initial hospice certification
  3. Vital capacity (VC) less than 30% of normal
  4. Significant dyspnea at rest

iii. Requiring supplemental oxygen at rest

  1. Patient declines artificial ventilation
  2. Patient must demonstrate both rapid progression of ALC and critical nutritional impairment:
  3. Rapid progression of ALS as demonstrated by all of the following characteristics occurring within the 12 month preceding initial hospice certification:
  4. Progression from independent ambulation to wheelchair or bedbound status
  5. Progression from normal barely intelligible or unintelligible speech

iii. Progression from normal to pureed diet

  1. Progression from independent in most or all activities of daily living (ADL) to needing major assistance by caretaker in all ADLs.
  2. Critical nutritional impairment as demonstrated by all of the following characteristics occurring within the 12 months preceding initial hospice certification
  3. Oral intake of nutrients and fluids insufficient to sustain life
  4. Continuing weight loss

iii. Dehydration or hypovolemia

  1. Absence of artificial feeding methods
  2. Patient must demonstrate both rapid progression of ALS and life-threatening complications:
  3. Rapid progression of ALS, see 2a above
  4. Life-threatening complications as demonstrated by one of the following characteristics occurring within the 12 months preceding initial hospice certification
  5. Recurrent aspiration pneumonia (with or without tube feedings)
  6. Upper urinary tract infection, e.g. pyelonephritis

iii. Sepsis

  1. Recurrent fever after antibiotic therapy

Medicaid Qualifications

ALS tends to progress in a linear fashion over time. By the time patients become end stage, muscle deterioration has become widespread, affecting all areas of the body, although initial predominance patterns do not persist. In end-stage ALS, two factors are critical in determining prognosis: ability to breathe, and to a lesser extent ability to swallow.

The patient must demonstrate critically impaired breathing capacity. Critically impaired breathing capacity as demonstrated by all of the following characteristics occuring within 12 months preceding initial hospice certification:

  1. Vital capacity (VC) less than 30% of normal
  2. Continuous dyspnea at rest
  3. Requiring supplemental oxygen at rest PO2<55 or O2 Sat <89% so that Medicaid supplemental O2 criteria is met
  4. Patient declines artificial ventilation. Patient must demonstrate both rapid progression of ALS and critical nutritional impairment
  5. Rapid progression of ALS as demonstrated by all of the following characterisitics occurring within the 12 months preceding initial hospice certification:

i. Progression from independent ambulation to wheelchair or bed bound status

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