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Sean M. Healey & AMG Center for ALS
ALS research at Mass General
ALS causes weakness in many muscles in the body, including the muscles involved in breathing and swallowing. Weakness in these muscles can cause respiratory problems, making breathing more difficult.
Research nurse Darlene Pulley teaches a patient to use an assistive breathing device.
Early Respiratory Issues and ALS
ALS causes weakness in many muscles in the body, including the muscles involved in breathing and swallowing. Weakness in these muscles can cause respiratory problems, making breathing more difficult. Respiratory problems are among the most serious symptoms of ALS and should not be ignored. The ALS Multidisciplinary Clinic offers pulmonary care services to help treat respiratory issues. If you begin to have difficulty breathing or swallowing, tell your physician right away. You should also inform your doctor right away if you develop a fever or if you having a change in the color of your phlegm, as these can be early signs of respiratory infection.
Early Signs of Respiratory Problems
Shortness of breath may be the first respiratory symptom that you experience. You may notice shortness of breath when you are engaged in vigorous physical activities like running, exercising, walking long distances, or climbing stairs. You may also have difficulty breathing at night which can interfere with your sleep and cause fatigue or morning headaches. You may find yourself waking frequently throughout the night. Difficulty breathing at night occurs when your chest muscles relax too much to assist with breathing during the dreaming phase of sleep.
Problems with swallowing can also interfere with breathing. When the swallowing muscles are weakened, food and saliva are more easily inhaled (aspirated) into the windpipe which leads to choking. Aspiration of food or saliva can also introduce bacteria into the lungs, increasing the risk of respiratory infection and pneumonia. Fever can be a sign of respiratory infection and should be reported immediately so your doctor can prescribe a course of antibiotics.
Treating Early Respiratory Symptoms
If you are experiencing any difficulties with breathing or swallowing, inform your physician. A breathing test, called a vital capacity (VC), will be used to measure the amount of air you can blow out. Most ALS clinics routinely administer this test to monitor any changes that may occur.
Another common breathing test performed in the clinic is the maximum inspiratory pressure (MIP), which measures the strength of your breathing muscles. Additional tests that may be performed include oxygen saturation levels of your red blood cells (called pulse oximetry) using a small probe placed on your finger throughout the night at home, an overnight sleep study in a sleep lab, or a blood sample to measure your oxygen and carbon dioxide levels.
The results of these breathing tests are used to decide when to recommend a treatment called non-invasive positive pressure ventilation (NIV). This therapy uses a ventilator which is called either BiPAP or VPAP, depending on the manufacturer. Research has shown that using NIV when the VC is at or below 50% of normal improves the quality of life and prolongs survival for many people with ALS (Bourke S, et al. 2006). Newer studies (Carrat`u P, et al.,2009) would support introducing it earlier. The question when to the best start using NIV, continues to be studied.
NIV is often applied by putting a mask over your nose or over your nose and mouth. There are, however, a variety of appliances that can be used and our goal is to find the one that works best for you. NIV assists weakened breathing muscles by using forced air pressure to help fill your lungs with air. NIV may be uncomfortable at first and it may take some time to get used to wearing the mask. Your respiratory therapist may initially recommend using it while relaxing during the day to help with this adjustment. A heated humidifier with the NIV prevents dryness of the mouth, nose, and throat which are commonly reported side effects. The goal of using NIV is to use it a minimum of four hours a night. As you get used to the device, most go on to use it throughout the entire night.
As the respiratory muscles weaken, coughing becomes more difficult making it difficult to expel phlegm from your lungs. If you have difficulty coughing effectively, a cough-assist machine may be helpful. This device, sometimes called a mechanical insufflator-exsufflator (MIE), works by producing a deep breath of air followed by negative pressure (suction) which helps you cough up phlegm. Using a cough-assist machine can help prevent lung infections by keeping the lungs clear of mucus
If you develop a fever, notice a change in the character of your sputum, or have an acute change in your breathing, contact your physician immediately. These can be symptoms of respiratory infection. If you have a respiratory infection, the best success to clear it quickly is to begin a course of antibiotics immediately.
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