End Stage Neurological Disorders – Amyotrophic Lateral Sclerosis 肌萎缩侧索硬化 (ALS) (Learning about Hospice)

ALS is a rapidly progressing degenerative neuromuscular disease with an unknown origin, results in muscle weakness, disability, respiratory insufficiency, and eventually death. The median survival duration for patients is approximately 3 years, yet 10% will survive for > 10 years.

1. Muscle weakness
   • A major symptom in ALS, progressive muscle weakness, should be managed by regular exercise
   • Physical therapy to the point of exhaustion is considered counterproductive, however, the main focus should be on maintaining mobility and the highest possible degree of independent.
   • As weakness progress, passive physiotherapy gains importance in preventing contracture and stiffness.
2. Dysarthria 构音障碍
   • Speech impairments affect up to 3/4 of ALS patients and frequently cause major distress for them and their caregivers.
   • Patient should be introduced early to alternative communication devices such as alphabet boards and computer-based systems that can be operated by hand or eye control.
3. Sialorrhea 流涎
   • Sialorrhea – Drooling which is caused by a combination of facial muscle weakness and a reduced ability to swallow.
   • Sialorrhea has been linked to aspiration pneumonia
   • Treatment with anticholinergic agents is generally tried first (Atropine, glycopyrrolate, and etc.)
4. Pseudobulbar affect (Emotional lability 不稳定)
   • Excessive laughing and crying affects 20-50% of patients with ALS.
   • Emotional lability is caused by bilateral corticobulbar tract degeneration and is not considered to be an emotional disorder per se.
5. Muscle spasticity 痉挛
   • may cause painful spasms (Muscle relaxants: Baclofen and Tizanidine)
6. Muscle cramps
   • Quinine is considered the most effective treatment option but should not be used routinely because of the potential for toxicity
   • The EFNS guideline also recommends the use of levetiracetam based on a small open label trial
   • vitamin E, gabapentin, and magnesium have been used frequently but have failed to demonstrate efficacy in small clinical trials, whereas Quinine, calcium channel blocker (diltiazem), and naftidrofuryl have been shown to be beneficial.
7. Pain
   • Many patients suffer from pain secondary to muscle spasms, cramps, and contracture, as well as musculoskeletal pain caused by reduced mobility.
   • Frequent changes in position are essential to prevent the skin from breaking down.
   • Physical therapy might help to avoid joint stiffness.
8. Psychosocial symptoms
   • approximately 10% are estimated to develop major depression
   • both patients and caregivers should be offered counseling, and pharmacological treatment should be considered
9. Insomnia
   • causes: anxiety, depression, dysphagia, dyspnea, and the inability to change posture, which can result in discomfort and pain.
   • Identification and treatment of the underlying causes is crucial to treating the insomnia
10. Dysphagia
    • difficulty swallowing is one of the most common symptoms of ALS, and patient should be screened for symptoms in the office setting at least once every 3 months
    • Aspiration precaution
    • Chocking due to food intake or weight loss of 10% or more of body weight should trigger a conversation about the placement of a percutaneous endoscopic gastrostomy (PEG) tube. (Studies have shown that PEG tube placement prolongs survival, but there are insufficient data on its impact on quality of life.)
11. Dyspnea
    • At the onset of respiratory symptoms indicating hyperventilation, or when a patient’s forced vital capacity (FVC) drops below 50%, the patient should be counseled about noninvasive mechanical ventilation as well as the terminal phase of the disease. — risk of sudden death or ventilator dependence
    • If patients decline noninvasive ventilation or do not tolerate it, options such as invasive ventilation or hospice referral should be discussed at this point.

***The main area of involvement is the motor neurons of the brain and spinal cord. Approximately half of those patients presenting with ALS will have difficulty swallowing as their first symptom. Other patients will experience distal weakness. As the disease progresses, weakness affects both the upper and lower neurons. Death generally results from respiratory failure due to weakness in the diaphragm along with decreased laryngeal and lingual functionality. Swallowing and oral nourishment are of high concern for these patients. Loss of motility in the tongue and hypopharynx result in the loss of ability to manipulate food as well as creating speech and communication barriers. ***

Hospice referral:

Two factors are critical in determining prognosis: ability to breathe and, to a lesser extent, ability to swallow. 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 (should fulfill 1, 2 or 3):

1. Critically impaired breathing capacity
   • Vital capacity (VC) less than 30% of normal, if available
   • Dyspnea at rest
   • Declines artificial ventilation
   • External ventilation used for comfort measures only
2. Rapid progression of ALS and critical nutritional impairment
   • Within the 12 months preceding initial hospice certification, progression from:
     • Independent ambulation to wheelchair to bed bound status
     • Normal to barely intelligible or unintelligible speech
     • Normal to puréed diet
     • Independence in ADLs to needing major assistance in all ADLs
   • Critical nutritional impairment as demonstrated by all the following characteristics occurring within the 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, sufficient to sustain life, but not for relieving hunger
3. Rapid progression of ALS and life threatening complications
   • Recurrent aspiration pneumonia (with or without tube feedings)
   • Upper urinary tract infection (pyelonephritis)
   • Sepsis
   • Recurrent fever after antibiotic therapy
   • Stage 3 or 4 decubitus ulcer(s)

References:

Corridor (2006). Hospice Quickflips: A Guide for Hospice Clinicians

Yennurajalingam, S., & Bruera, E. (2016). Oxford American Handbook of Hospice and Palliative Medicine and Supportive Care (2nd. Ed.)