Ask the Doc: Q & A with Edward Kasarskis, MD, PhD

Edward Kasarskis, M.D., Ph.D. is Director of the multidisciplinary ALS Center at the University of Kentucky Neuroscience Center in Lexington, Kentucky, professor in the Department of Neurology at the University of Kentucky, and Chief of Neurology at the VA Medical Center in Lexington KY. 

Dr. Kasarskis

Q:  My mother-in-law has bulbar ALS and, now that it’s winter, we’re concerned about her contracting a respiratory virus or other infection. The problem is that she lives quite far away from the Clinic and family, and insists on going to her small town family practitioner. She recently had pneumonia and was given a shot and antibiotics. She no longer speaks, so her communication with the practitioner is fairly limited. How much can we trust the diagnosis and treatment she is getting from this practitioner? And can you clarify why the concern is so high for the risk of respiratory infections in ALS patients?
      -- Shilo, Lees Summit, Missouri

A: ALS and respiratory infection: this is a difficult situation in many ways. Even without an infection, it’s hard enough with ALS to take a deep breath, cough effectively, and deal with secretions. With a cold or the flu, the challenges inherent in dealing with ALS are magnified with fatigue, thick mucus, cough, dehydration and fever adding to the difficulties and risks. Usually it takes more than just a simple antibiotic and frequently an ALS patient needs hospitalization to treat pneumonia effectively. Fortunately, it sounds like your mother-in-law's case was not too severe.

So, the first and most important approach is actually an admonition: people with ALS should do everything they can to prevent a respiratory infection. In its simplest terms, that means the airway has to be kept clear and the lung expanded. To do this, you have to prevent as many infections from bacteria and viruses as possible and also head off infections from aspiration of mouth contents (saliva, food, drink) into the lungs. Keeping the lungs expanded may involve noninvasive ventilation (BiPAP) and exercises for the respiratory muscles.

ALS patients should get the pneumococcal vaccine (Pneumovax) and the annual flu vaccine; keep away from friends and family who are sick; use a hand sanitizer; get as much sleep as possible; and get the nutrition necessary to maintain optimal health (through a PEG tube into the stomach, if necessary). If you smoke, quit now! There is no point in fighting two problems (ALS and lung damage from smoking). If you have asthma, make sure your treatment is tuned up to the max -- usually in consultation with a pulmonologist.

People with ALS who have trouble dealing with their secretions and saliva when they’re healthy are likely to have real challenges managing if they have a respiratory illness. It’s important to talk with a neurologist about medications and treatments that can reduce drooling and the build-up of saliva in the mouth. Good management of these issues can also help reduce the risk of getting pneumonia. People with bulbar ALS are especially prone to these issues.

But sometimes, despite best efforts at prevention, a respiratory infection strikes. When it does, there are a few things that should be considered during the acute phase and recovery:

• Frequently, antibiotics alone are not enough to clear pneumonia. The ALS patient needs adequate hydration and deep coughing to expand the lungs and clear the airway. This may require intense therapy and hospitalization.
• For the long term, two devices may be needed to mobilize secretions and clear the airway. A Cough-Assist device (generically known as a "Mechanical Insufflator-Exsufflator") can be ordered by a physician, and a respiratory therapist would typically provide help and instruction about how to use it. The device mobilizes secretions and helps cough them out. There is also an external vest that vibrates, helping to release tenacious mucous; it’s often used in addition to a cough-assist device.
• Mucous-busting medications such as Mucinex help thin secretions and are available over the counter. Robinul (glycopyrrolate) helps reduce the amount of secretions and requires a prescription.

Can your family practitioner handle all this? It obviously depends on the expertise of your local physician. A respiratory infection can pose significant risk and considerable discomfort for the person with ALS and may ultimately require specialized care from an ALS Center with your neurologist and pulmonologist. But your first point of contact will always be your local physician, an important member of your health care team. Keep the physician up to date on the status of your mother-inlaw’s ALS so that if pneumonia strikes, he or she will be ready to initiate treatment in consultation with her ALS neurologist and other specialists.

If you would like to submit questions for a future Q & A, please send your questions to Amber Walters at awalters@alsa-national.org. Please understand that we won’t be able to address all questions and we won’t be able to respond to individuals personally.