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. 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 and to help clear the small airways and reduce the chance of developing pneumonia. In general, the device should be used 2-4 times a day and adjusted by a respiratory therapist for comfort. Click here for an example of a device.
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.
When verbal communication becomes difficult, some patients, who are able, prefer to write. A Boogie board is more efficient than a tablet and is preferred by many.
For patients with an ipad, the addition of the app Proloquo2Go can create a professional type augmentative communication device (about $250 for the app).
With regard to speech-language pathologists with expertise in ALS, we refer patients to the ICAN Talk Clinic See http://www.icantalkclinic.com/
For an appointment call 412-489-5527
Katya Hill, Ph.D., CCC-SLP or Michael O’Leary, M.S., CCC-SLP from the ICAN Talk Clinic attend multidisciplinary clinic and follow patients in the AAC Institute in Carnegie, a full-service speech and language pathology outpatient center with a specialty in providing professional services to individuals with severe communication disorders, particularly those who rely on Augmentative & Alternative Communication (AAC) systems or benefit from other assistive technology to support treatment. AAC systems include eye gaze and brain computer interface (BCI) access.
We also refer patients to the University of Pittsburgh Center for Assistive Technology (CAT).
They have an augmentative communication clinic in addition to clinics for computer and technology assistance and mobility (wheelchairs).