Amyotrophic Lateral Sclerosis (ALS)

Rapid progression of ALS in the preceding 12 months evidenced by:

• 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 ADLs to needing major assistance by caretaker in all ADLs

At least one of the following must also apply:

• Critically impaired breathing capacity evidenced by:

• Vital capacity (VC) < 30% of normal

• Significant dyspnea at rest

• Requires supplemental oxygen at rest

• Patient declines artificial ventilation

• Critical nutritional impairment evidenced by:

• Oral intake insufficient

• Continuing weight loss

• Dehydration or hypovolemia

• Absence of artificial feeding methods

• Life-threatening complications:

• Recurrent aspiration pneumonia

• Upper urinary tract infection (pyelonephritis)

• Sepsis

• Recurrent fever after antibiotic therapy

• Clinical findings of malignancy with widespread, aggressive or metastatic disease or

• Decline in performance status and/or significant unintentional weight loss

NOTE: The patient may still receive disease-specific treatment if it is palliative.

The Following Information Will Be Needed If Available:

• This patient has evidence of malignant histopathology (define the cell type)

• A neoplastic histopathology is not available (describe circumstances and basis for presumptive diagnosis)

• Poor response to optimal treatment with diuretics and vasodilators, including angiotensin converting enzyme (ACE) inhibitors and

• The presence of significant symptoms of recurrent CHF at rest and classified as New York Heart Association (NYHA) Class IV (inability to carry on any physical activity without discomfort, symptoms of heart failure or angina at rest or increased discomfort even with minimal exertion).

Supporting Documentation:

• Ejection fraction < 20%

• Treatment resistant symptomatic supraventricular or ventricular arrhythmias

• History of cardiac arrest or resuscitation

• History of unexplained syncope

• Brain embolism of cardiac origin

• Concomitant HIV disease

• Patient suffers from pain or discomfort with minimal activity (New York Heart Class IV).

• Recurrent symptoms of CHF, even when using drugs designed to relieve CHF symptoms (vasodilators, diuretics)

  • Co-conditions that further decrease survival:

  • History of patient blacking out due to lack of oxygen in the brain (history of unexplained Syncope)

  • History of cardiac arrest

Supplemental Indicators:

• When a patient's heart is not circulating more than 20% of its blood with each pump (Ejection Fraction of 20 percent or less)

• Functional decline with change in condition over the past three to six months

• Multiple diagnoses without any one being the primary diagnosis (CHF, diabetes, etc.)

• At least one co-morbidity in the past 12 months

• Examples include:

• Recurrent fever after antibiotics

• Urinary tract infection

• Upper respiratory infection

• Decubitus ulcer

• Sepsis

Supplemental Indicators:

• Unintentional weight loss of ≥ 10% of body weight over the last 6 months

• Emergency room visits or hospitalizations

• Desire for palliative care

• Speech limited to six or less intelligible words

• Incontinent bowel and bladder

• Increased need of assistance for ADLs

• Unable to ambulate

• Unable to dress self

• Unable to bathe self

• Associated diseases or infections:

• Aspiration pneumonia

• Urinary tract infection (UTI)

• Unintentional weight loss of ≥ 10% of body weight over the last six months

• Stage seven or beyond according to the Functional Assessment Staging Scale

• Unable to ambulate without assistance

• Unable to dress without assistance

• Unable to bathe without assistance

• Urinary and fecal incontinence, intermittent or constant

• No meaningful verbal communication, stereotypical phrases only, or ability to speak is limited to six or fewer intelligible words and

• Patients must have had one of the following within the past 12 months:

• Aspiration pneumonia
  

• Pyelonephritis or other upper urinary tract infection
  

• Septicemia
  

• Decubitus ulcers, multiple, stage 3-4
  

• Fever, recurrent after antibiotics
  

• Inability to maintain sufficient fluid and calorie intake with 10% weight loss during the previous six months or serum albumin < 2.5 gm/dL

• Prothrombin time (PT) more than 5 seconds over control, or International Normalized Ratio (INR) > 1.5

• Serum albumin < 2.5 gm/dL and

• One or more of the following conditions:

• Ascites, refractory to treatment or patient non-compliant

• Spontaneous bacterial peritonitis

• Hepatorenal syndrome; elevated creatinine and BUN; oliguria {< 400 ml/day}; urine sodium concentration < 10 mEq/l; cirrhosis and ascites

• Hepatic encephalopathy, refractory to treatment or patient non-compliant

• Recurrent variceal bleeding, despite intensive therapy

Supporting Documentation:

• Progressive malnutrition

• Muscle wasting with reduced strength and endurance

• Continued active alcoholism (> 80 gm ethanol/day)

• Hepatocellular carcinoma

• HBsAg (Hepatitis B) positivity

• Disabling dyspnea at rest or with minimal exertion and little or no response to bronchodilators, resulting in decreased functional capacity, fatigue, and cough and

• Progression of end-stage pulmonary disease, as evidenced by prior increasing visits to the emergency department or prior hospitalizations for pulmonary infections and/or respiratory failure and

• Room air findings of hypoxemia, as evidenced by pO2 < 55 mmHg and oxygen saturation < 88% or hypercapnia, as evidenced by pCO2 > 50 mmHg

• Cor pulmonale and right heart failure (RHF) secondary to pulmonary disease (e.g., not secondary to left heart disease or valvulopathy)

• Unintentional progressive weight loss greater than 10% of body weight over the preceding six months

• Resting tachycardia > 100/mm

• Unintentional weight loss of ≥ 10% of body weight over the last 6 months

• Malnutrition or nutritional impairment

• Disability indicated by Karnofsky ≤ 40%

Supplemental Indicators:

• Anorexia

• Weakness

• Memory loss

• Depression

• Change in ability to perform ADLs

• Dizziness related to:

• Hypotension

• Electrolyte imbalance

• Anemia

• CD4 + Count < 25 cells/mcL or

• Persistent viral load > 100,000 copies/ml plus

• At least one of the following:

• Wasting (loss of 33% lean body mass), untreated, or not responsive to treatment

• Mycobacterium avium complex (MAC) bacteremia, untreated, unresponsive to treatment, or treatment refused

• Progressive multifocal leukoencephalopathy

• Systemic lymphoma

• Visceral Kaposi's Sarcoma unresponsive to therapy

• Renal failure in the absence of dialysis

• Toxoplasmosis, unresponsive to therapy

• Karnofsky Performance Status (KPS) < 50%

• Advanced AIDS dementia complex

Critical breathing capacity with the following findings:

• Dyspnea at rest

• The requirement of supplemental oxygen at rest

• The patient declines artificial ventilation or

Critical nutritional impairment evidenced by:

• Oral intake of nutrients and fluids insufficient to sustain life

• Continuing weight loss

• Dehydration or hypovolemia

• Absence of artificial feeding methods or

Rapid disease progression or complications in the preceding 12 months evidenced by:

• 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 independent in most or all activities of daily living (ADLs) to needing major assistance by caretaker in all ADLs

Life-threatening complications in the preceding 12 months as evidenced by one or more of the following:

• Recurrent aspiration pneumonia (with or without tube feedings)

• Upper urinary tract infection (pyelonephritis)

• Sepsis

• Recurrent fever after antibiotic therapy

• Stage 3 or 4 decubitus ulcers

• Shortness of breath while at rest

• Frequent and increased visits to the ER for respiratory infections and respiratory failure

• Poor responsiveness to drugs designed to help patients breathe easier (bronchodilators)

• Lack of oxygen throughout the body (hypoxia) even while on supplemental oxygen, evidenced by a standing oxygen saturation test of ≤ 88%

Supplemental Indicators:

• Resting heart rate of over 100 beats per minute (tachycardia)

• Unintentional weight loss of ≥ 10% of body weight over the last six months

• Karnofsky Performance Status Scale from ≤ 70%

• Palliative Performance Scale ≤ 70%

• Body Mass Index < 22

• Unintentional weight loss or weight gain

• Creatinine clearance < 10 cc/min or < 15 cc/min for diabetics

• Serum creatinine > 8.0 mg/dl or 6.0 mg/dl for diabetics

• Oliguria: Urine output < 400cc/ 24hr

• Uremia: Clinical symptoms of renal failure

• Confusion

• Nausea/vomiting

• Generalized pruritis

• Restlessness

• Not seeking dialysis, needed transplant or is discontinuing dialysis

• If on dialysis:

• Has a prognosis of ≤ 6 months, if the illness runs its course despite dialysis

• Karnofsky Performance Status Scale from ≤ 40%

• Palliative Performance Scale ≤ 40%

• Body Mass Index < 22

• Unintentional weight loss (despite tube feeding) 10% in 6 months or 7.5% in last 3 months

• Dysphagia without tube feeding

• Pulmonary aspiration not responsive to speech pathology intervention

• Serum albumin ≤ 2.5 gm/dl

• Age > 70

• Post stroke dementia, with FAST score of ≥ 7C

• Medical complications related to progressive clinical decline over past 12 months

• Aspiration pneumonia

• UTI's

• Sepsis

• Skin breakdown, decubitus ulcers, refractory stage 3-4