ALS tends to progress in a linear fashion over time, and the overall rate of decline in each patient is fairly constant and predictable. However, no single variable deteriorates at a uniform rate in all patients, so multiple clinical parameters are required to judge the progression of ALS.
Although ALS usually presents in a localized anatomical area, the location of initial presentation does not correlate with survival time. By the time a patient is end-stage, muscle denervation is widespread , affects all areas of the body, and initial predominance patterns do not exist.
Progression of the disease differs widely from patient to patient, therefore the history of the rate of progression in the individual patient is important to obtain to predict prognosis. In end-stage ALS, two factors are critical in determining prognosis: ability to breathe and, to a lesser extent, ability to swallow.
Examination by a neurologist within three months of assessment for hospice is advised, both to confirm the diagnosis and to assist with the prognosis.
To qualify for hospice, patients must have:
Critically impaired breathing by evidence of all the following characteristics within the 12 months preceding initial hospice certification:
- Vital capacity < 30% of normal
- Significant dyspnea at rest
- Requiring supplemental oxygen at rest
- Patient declines artificial ventilation
Patients must demonstrate rapid progression of ALS and critical nutritional impairment
- 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 to needing major assistance by a caretaker
- Oral intake of nutrients and fluids insufficient to sustain life
- Continuing weight loss
- Dehydration
- Artificial feeding methods declines
Patients must demonstrate rapid progression of ALS and life-threatening complications
- 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 to needing major assistance by a caretaker
- Recurrent aspiration pneumonia
- Urinary tract infections
- Sepsis
- Recurrent fever after antibiotic therapy