The Role of a Respirologist on the ALS Care Team

Amyotrophic lateral sclerosis (ALS) commonly affects the respiratory system, as the disease progressively impairs the nerves that control the breathing muscles and the muscles become weak.^[i]

“Similar to how the muscles in the legs, arms or neck can get weaker, the muscles that help you breathe, as well as the muscles that help you cough and protect your airway can also get weaker,” explains Dr. Anu Tandon, Head, Division of Respirology and Medical Director, Pulmonary Function Lab at Sunnybrook Health Sciences Centre in Toronto.

Dr. Tandon is part of a multidisciplinary team at the ALS clinic at Sunnybrook Health Sciences which includes neurologists, a palliative care physician, a respiratory therapist, an occupational therapist, a registered nurse, as well as other healthcare professionals. When patients display symptoms of breathing muscle weakness, or diagnostic testing indicates it is present, they are referred to Dr. Tandon for evaluation and support.

The main role of a respirologist in the ALS clinic is to help identify and manage breathing muscle weakness.

For ALS patients, symptoms related to breathing can start early, and for others, these symptoms can start much later.^[ii] Symptoms that may indicate weak respiratory muscle involvement can include: ^[ii]

• • Air “hunger” (gasping, labored breathing) with or without activity
  • Fatigue
  • Waking in the morning with a headache or fuzzy headed feeling (morning confusion) 

According to Dr. Tandon, symptoms may also include sleepiness during the day, awakening frequently during the night with shortness of breath, difficulty breathing when lying flat and difficulty coughing or chest congestion.

Tests are often done at the early stage of ALS to help establish baseline measurements to which later breathing tests can be compared, helping track the progression of the disease. Commonly performed tests include: forced vital capacity (FVC); maximum inspiratory force (MIP), maximum expiratory force (MEP) and blood oxygen saturation. A respirologist may also teach ALS patients assisted coughing techniques and deep breathing exercises and identify treatment and therapy options that suit the individual person’s needs.^[iii]

It’s important for patients to know if they do develop respiratory muscle weakness as part of ALS, we have options available to help manage these symptoms.

There are several devices to help support breathing and clearing of the lungs for people living with ALS. Devices to help support breathing, or ventilation can be delivered either non-invasively or invasively. Other devices can help improve the ability to cough and clear mucus from lungs.^[ii]

Non-invasive ventilation, which usually consists of a mask over the nose, or nose and mouth,^[iii] has been shown to improve survival, slow decline in lung function and increase quality of life in ALS patients.^[iv]

One common option is the bilevel (BiPAP) device, which a patient wears over their nose and mouth when they sleep at night to help rest breathing muscles, thereby reducing shortness of breath during the day.^[v]

“While the non-invasive supports may take some getting used to, they are very beneficial,” says Dr. Tandon. She also suggests ways patients can support breathing or airway clearance on their own:

• • Stay as active as you can
  • Wash or sanitize hands and get vaccinated to reduce the risk of infections
  • Maintain weight as much as possible

As a patient’s respiratory symptoms progress, decisions are made regarding their advanced directives and goals of care. During the later stages of the disease, it is important to understand if a patient wishes to move to invasive ventilation in the event that they can no longer breathe on their own or with the support of a BiPAP machine.^[vi]

With invasive (mechanical) ventilation, a tube is inserted into the windpipe through the neck (in a procedure called a tracheostomy) and connected to a ventilator machine that assists with breathing.^[iii]

Dr. Tandon explains that for some patients, total ventilatory support can provide a quality of life they enjoy.  For others, they may choose to forgo invasive breathing support and instead let the disease take its course, focusing on comfort and symptom management. In that case, the respirologist would work very closely with palliative physicians to help manage their care.

“These are very difficult decisions for patients as you can imagine, and not everybody knows what they want right away. Often the family, and other values the patient holds dear, play a role in that decision,” says Dr. Tandon. “My job is to provide information and support for whichever route they choose to take.”