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University of Pittsburgh

Breathing Better

As the muscles that expand the lungs begin to weaken, respiratory failure becomes a concern for people with ALS. Even though the lungs may function normally, the progression of ALS makes the process of breathing more difficult. Sometimes, the first symptom is difficulty sleeping due to problems breathing while lying flat.

Respiratory management in ALS is essential to comprehensive care. Pulmonary consultation should be arranged soon after the diagnosis is made and then should continue as part of multidisciplinary support. Patients are usually referred to the Comprehensive Lung Center / Sleep Center at UPMC, under the direction of David Kristo, MD and Euhan (John) Lee, MD. At the Comprehensive Lung Center, a chest X-ray and pulmonary function tests will be done as part of the initial evaluation. Information about the use of various types of non-invasive (BiPAP/AVAPS) and invasive mechanical ventilation and advanced directives are discussed in detail so that patients can make informed decisions about their care. Patients are also evaluated for cough assist devices.

General Recommendations to Improve Breathing

  • Do not lie down immediately after eating.
  • Avoid eating large meals that can increase abdominal pressure.
  • Elevate your head with pillows from 15-30 degrees to keep abdominal organs away from the diaphragm.

Respiratory Assistive Devices

The following assistive devices may be prescribed to help people with ALS breathe easier.

  • Non-invasive ventilatory assistance (BiPAP such as AVAPS)—to rest the lung muscles, provides comfort during sleeping and improve performance during the day. This can be achieved with a nasal or facemask that is connected to a machine test makes breathing easier.
  • AVAPS is a new type of BiPAP that is better for ALS patients. Sip and puff ventilation may also be useful and does not require a tracheostomy.
  • Suction machine—to clear the mouth and throat of secretions, if patients are having difficulty coughing or swallowing these secretions on their own.
  • Cough-assist device – a non-invasive portable, electrically powered device used to provide negative and positive pressure that causes airflow from the lungs to stimulate a cough.
  • Diaphragm Pacing System - an electrical stimulation system in which electrodes are placed in the diaphragm muscle via a laparoscopic surgical procedure performed by our colleagues in Thoracic Surgery. An external device generates the impulses. A clinical study concluded that diaphragm pacing prolonged survival by 9-16 months in combination with BiPAP. The diaphragm must have some activity for diaphragmatic pacing to be effective. To be eligible, patients must have some evidence of decreased ventilation by various tests that can be performed in clinic or in the pulmonary function testing lab. http://www.synapsebiomedical.com/als/neurx-als.shtml

Tracheostomy/Mechanical Ventilation

A small percentage of ALS patients elect to undergo these procedures. MDA has two videos entitled “Breath of Life” and “Breathe Easy” that are recommended for viewing (www.mdausa.org). Discuss this issue with your family, neurologist and pulmonologist, and determine your insurance coverage and home care options.

The Comprehensive Lung Center, phone (412) 648-6161.