What is the role of respiratory therapy in the treatment of amyotrophic lateral sclerosis (ALS)?

Updated: Jan 02, 2020
  • Author: Divakara Kedlaya, MBBS; Chief Editor: Ryan O Stephenson, DO  more...
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Patients with ALS who predominantly have limb and respiratory weakness, accompanied by limited bulbar involvement, may prevent respiratory complications and placement of permanent tracheostomy through aggressive pulmonary care. Respiratory therapists can play a critical role in the management of these patients. [19, 20]

Recommend that patients perform routine measures of assisted peak cough flows to evaluate bulbar muscle function. Continued attainment of adequate peak cough flow is the most important goal for preventing serious pulmonary complications.

Routinely measure vital capacity in sitting and recumbent positions. Usually, recumbent vital capacity is the first to decline, resulting in nocturnal hypoventilation.

Oxyhemoglobin saturations (SaO2) should be monitored, especially at night in patients complaining of nocturnal awakenings or sleep difficulties. Decreased overnight SaO2 indicates nocturnal hypoventilation and the need for noninvasive IPPV at night. Despite low SaO2, do not administer oxygen without invasive ventilation. In patients without invasive ventilation, oxygen administration decreases the respiratory drive and may thereby result in exacerbation of hypercapnia.

Respiratory therapists also are involved in educating patient and caregiver. They can instruct caregivers on proper performance of assisted cough techniques and teach submaximal respiratory strengthening exercises to the patient. Respiratory therapists also can provide instruction for proper use of expiratory muscle aids, such as the intermittent abdominal pressure ventilator and mechanical insufflator-exsufflator. In addition, they can teach patients and caregivers to use and manipulate inspiratory muscle aids, including noninvasive and invasive ventilation.

Finally, respiratory therapists can teach patients glossopharyngeal breathing. This involves gulping boluses of air into the lungs to add to the user's deep breath and can be useful in patients with nonbulbar ALS who do not have a tracheostomy. One glossopharyngeal breath contains 6-9 gulps and can provide the patient with weak inspiratory muscles and no vital capacity a safe way to breathe if, for any reason, a ventilator cannot be used or there is sudden ventilator failure.

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