MDA Engage: Patients can take steps to improve breathing issues
Pulmonologist details ways to boost quality of life
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- Noninvasive ventilation, cough-assist devices, and breathing exercises are among ways ALS patients can ease breathing issues.
- Early signs of weakening breathing function can be subtle.
- Patients should get regular vaccinations to minimize the risk of respiratory infections.
A progressive decline in lung function is unavoidable for many people with neuromuscular conditions such as amyotrophic lateral sclerosis (ALS), but there are steps that patients can take to ease breathing issues and improve quality of life.
“We can’t really change the progression,” said James Rowley, MD, a pulmonologist at Rush University Medical Center in Chicago. “But we can try to make the quality of life and the quality of your breathing as good as possible, for as long as possible.”
Rowley, a lung specialist who treats many people with ALS, recently led a session at the MDA Engage: Chicago symposium explaining how patients can breathe easier as their muscles weaken. The Sept. 25-26 event brought together people living with neuromuscular diseases and their caregivers, as well as clinicians and advocates, for education and community support. Rowley’s session was titled, “Taking Charge of Your Breathing Health.”
Tracking breathing function
ALS is a neurodegenerative disease marked by progressive weakening of different muscles throughout the body, including those involved in breathing. When these muscles weaken, patients can develop respiratory issues and may require supportive treatment to help them breathe better.
According to Rowley, early signs that the muscles used for breathing may be weakening can be subtle. They can include shortness of breath while walking, needing extra pillows to sleep, daytime fatigue, or morning headaches.
Doctors often track two measures of breathing function: forced vital capacity (FVC), which shows how much air a person can forcefully exhale after taking a deep breath, and maximal inspiratory pressure (MIP), which measures the strength of the muscles used to breathe in.
James Rowley, MD, a pulmonologist at Rush University Medical Center in Chicago, speaks at MDA Engage. (Photo by Douglas Backstrom)
In Rowley’s experience, breathing muscle strength may decline before FVC falls. He said that when FVC falls below 60% of the predicted value for a person of a specific age, gender, and height, or MIP drops below -60 centimeters of water, it may be time to discuss noninvasive ventilation (NIV) and insurance coverage with a care team.
NIV provides breathing support through a mask or mouthpiece rather than a breathing tube. It is often used during sleep to help breathing muscles rest.
For people with ALS who feel short of breath despite normal FVC and MIP values, an overnight oximetry test may reveal problems earlier, he said.
“That’s where you basically wear a finger probe throughout the night,” Rowley said. “We measure your oxygen levels. In ALS patients, the overnight oxygen is actually abnormal before the other two values are abnormal.”
Rowley said these numbers can also guide feeding tube decisions. With a predicted FVC of 50% or higher, placement is usually a safe, same-day outpatient procedure. Below 30%, patients may need a breathing tube during the process, so Rowley encourages the conversation early.
Everyday steps that can help
Even as breathing function declines, people can change how breathless they feel, Rowley said. He recommended walking daily if possible, “even if it’s just a block,” and sitting up as much as possible to keep the lungs open. He also recommended using an incentive spirometer — a handheld device that encourages people to take deep breaths and helps maintain the lungs open, reducing shortness of breath during the day — and breath stacking, a technique in which a person takes several breaths on top of one another before exhaling.
People who struggle with the NIV mask at night can ease in gradually, he said. First, wear it during the day without the machine, then connect it. Then repeat those same steps at bedtime.
“When you’re able to fall asleep with something on your face at night, then you can connect it back to the machine,” Rowley said. “And hopefully that would allow you to be able to use it pretty well.”
A weak cough is also a major concern for people with neuromuscular conditions. Mucus can block the airways, contributing to respiratory infections and potentially requiring emergency care. A cough-assist device can help clear secretions when cough muscles are too weak. Some people who use NIV also may need cough assistance, Rowley said. Staying hydrated, using a humidifier, reducing dairy intake, and maintaining oral care may also help manage secretions, he said.
Rowley also recommended that people with neuromuscular disease get a flu shot every year, along with the COVID-19 vaccine, to minimize the risk of respiratory infections. Older patients should also get the RSV and pneumonia vaccines, he said.
ALS is the condition Rowley’s team at Rush treats most often, he said, and the research behind much of his advice is strongest for ALS. However, many of the same approaches may also help people with other neuromuscular diseases.
Note: The ALS News Today team is providing coverage of the MDA Engage Symposium. Go here to see the latest stories from the conference.
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