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Amyotrophic Lateral Sclerosis (ALS) (cont.)

Medical Treatment

No cure is available for ALS. However, a medication called riluzole (Rilutek) has become available.

  • This medication apparently slows damage to motor neurons by decreasing levels of a substance called glutamate that is thought to have a role in motor neuron degeneration.

  • This drug has been shown to prolong life or delay ventilatory failure by two to three months in patients with ALS.

  • It does not reverse damage already done to motor neurons.

The remainder of medical treatment is focused on relieving symptoms, maintaining function for as long as possible, and coping with loss of function.

  • Regular exercise and a physical therapy program can strengthen muscles and reduce spasticity. This helps maintain movements for as long as possible.

  • Speech therapy can help maintain speech for as long as possible and introduce other methods of communication. Computerized devices may help you communicate when you can no longer speak.

  • Medications such as baclofen (Lioresal) and tizanidine (Zanaflex) may be used to relieve severe spasticity.

  • Medications also may be prescribed to relieve pain (if any), depression, anxiety, sleep problems, and constipation.

  • Diet needs to be adjusted to maintain adequate nutrition. Percutaneous tube feedings to the stomach are recommended when eating becomes an arduous task (takes more than an hour to complete the meal, or patient gives up before finishing), or when patients find they are choking on their food, or if they begin to lose weight rapidly.

Breathing support becomes necessary when you encounter symptoms of ventilatory failure. Often these result first in disrupted sleep. A patient may become aware of this first by noticing excessive sleepiness, or tiredness, during the day, or by waking up with headaches. Later, patients may become short of breath if they lie down or with the smallest of activities. Non-invasive ventilatory support (Bi-PAP) can be used to support breathing during the night, and also, in more advanced disease, to give the breathing muscles a rest during the day. 

Nutritional support and judicious use of non-invasive ventilatory support may extend life by up to 12 months on the average and result in improved quality of life.

Invasive ventilatory support is required when patients can no longer breathe without assistance or if excessive secretions preclude the use of non-invasive ventilatory support.

  • Usually a mechanical ventilator is connected to the trachea (airway) through a small, permanent hole in the neck called a tracheostomy. The tracheostomy is performed by a surgeon.

  • The biggest problem for a person on a ventilator is keeping the airway clear of natural secretions such as saliva and mucus.

  • If not cleared, these secretions can be "aspirated," or breathed into the lungs. There they cause problems such as infections and pneumonia.

  • A respiratory therapist can help prevent aspiration by regular suctioning and chest percussion.

  • These secretion management techniques can be learned by caregivers and home health aides.

Only a small minority of patients choose to receive invasive ventilatory support because of its requirements for 24-hour care in the context of a lifestyle that most, but not all, patients do not choose for themselves. However, some patients on long-term permanent ventilatory support have reported meaningful quality to their lives.

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