Prognosis Of Amyotrophic Lateral Sclerosis Based On Hospice Admission Criteria

What follows is the prognosis for Amytrophic Lateral Sclerosis based on hospice admission criteria. If a patient meets these criteria he or she is eligible for hospice care which means that he or she has an estimated six months or less to live.

To meet the CMS Disease Specific Criteria For terminal (6 months or less prognosis for) Amyotrophic Lateral Sclerosis, the patient must meet the following:

A patient is eligible for hospice services if he meets these three criteria:
1) has a Palliative Performance Scale of less than 70% [Resource (2)]
2) is dependent on at least two Activities of Daily Living [Resource (3), and
3) meets the Disease Specific Guideline for Amyotrophic Lateral Sclerosis [Resource (1)]

Amyotrophic Lateral Sclerosis

General Considerations:

1. ALS tends to progress in a linear fashion over time.
Thus the overall rate of decline in each patient is fairly
constant and predictable, unlike many other non-cancer
diseases.

2. However, no single variable deteriorates at a uniform
rate in all patients. Therefore, multiple clinical parameters
are required to judge the progression of ALS.

3. Although ALS usually presents in a localized
anatomical area, the location of initial presentation does
not correlate with survival time. By the time patients
become end-stage, muscle denervation has become
widespread, affecting all areas of the body, and initial
predominance patterns do not persist.

4. Progression of disease differs markedly from patient
to patient. Some patients decline rapidly and die
quickly; others progress more slowly. For this reason, the
history of the rate of progression in individual patients is
important to obtain to predict prognosis.

5. In end-state ALS, two factors are critical in determining
prognosis: ability to breathe, and to a lesser extent
ability to swallow. The former can be managed by artificial ventilation, and the latter by gastrostomy or other
artificial feeding, unless the patient has recurrent aspiration
pneumonia. While not necessarily a contraindication
to hospice care, the decision to institute either artificial
ventilation or artificial feeding may significantly alter six
month prognosis.

6. Examination by a neurologist within three months of
assessment for hospice is advised, both to confirm the
diagnosis and to assist with prognosis. Patients are considered eligible for hospice care if they do not elect tracheostomy and invasive ventilation and display evidence of critically impaired respiratory function (with or without use of NIPPV) and / or severe nutritional insufficiency (with or without use of a gastrostomy tube).

Critically impaired respiratory function is as
defined by:

1. FVC <40% predicted (seated or supine) and 2 or more of
the following symptoms and/or signs:
• Dyspnea at rest;
• Orthopnea;
• Use of accessory respiratory musculature;
• Paradoxical abdominal motion;
• Respiratory rate >20;
• Reduced speech / vocal volume;
• Weakened cough;
• Symptoms of sleep disordered breathing;
• Frequent awakening;
• Daytime somnolence / excessive daytime sleepiness;
• Unexplained headaches;
• Unexplained confusion;
• Unexplained anxiety;
• Unexplained nausea.

2. If unable to perform the FVC test patients meet this criterion
if they manifest three or more of the above symptoms/signs.
Severe nutritional insufficiency is defined as: Dysphagia
with progressive weight loss of at least five percent of
body weight with or without election for gastrostomy tube
insertion.

These revised criteria rely less on the measured FVC, and
as such reflect the reality that not all patients with ALS can
or will undertake regular pulmonary function tests.