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ALS Multidisciplinary Clinic
Clinic: 617-724-3914 Neurology Access Center: 1-855-MGH-NEUR (644-6387)
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ALS research at Mass General
Treating Advanced Respiratory SymptomsAs ALS progresses, respiratory problems often worsen. Shortness of breath may occur more often, including while at rest during the day. When this happens, it can be helpful to start using NIV during the day as well as at night. Your respiratory therapist can help you with alternative appliances for daytime use, such as a mouthpiece.
When it becomes necessary to use NIV for most of the day, it is time to consider a tracheostomy. This involves a surgical procedure to create an opening into your windpipe (trachea) through which a plastic tube is inserted. This tube allows air to pass directly into your trachea. Unlike the mask used for NIV which can be taken off, this tube stays in place at all times. A ventilator can be attached to the tube for breathing assistance as needed. This tube can also be used to clear phlegm from your lungs and to prevent food or saliva from going into your lungs by accident.
A ventilator with a tracheostomy may add years to your life as it can fully support your breathing. It will not, however, reverse the progression of your disease or prevent further weakening of the muscles throughout your body. Some people with a tracheostomy are able to speak, but this varies among individuals. Similarly, some people are able to eat and swallow with a tracheostomy while others cannot. For people who cannot eat or take in all of their nutrition by mouth, a feeding tube is inserted into the stomach for nutrition.
Whether or not to have a tracheostomy and to use a ventilator is an individual decision. You can discuss any questions or concerns you may have with your physician.
Discontinuing VentilationChoosing to use a ventilator, whether it is with NIV or with a tracheostomy, does not mean that you must continue to use breathing assistance indefinitely. As your ALS progresses, you can decide to discontinue ventilation and allow nature to take its course. Ventilation can be discontinued at home with hospice care to ensure that you are comfortable and do not experience any breathing distress. Sedation is provided before the ventilator is removed and is continued until breathing stops naturally. Usually, family and friends are present so that they can say goodbye and witness their loved one’s passing. This can be a very peaceful and loving experience.
It is always a good idea for people with ALS, especially those on ventilation, to appoint a healthcare proxy who can make medical decisions on their behalf if they become unable to make or express decisions themselves. You should make your wishes about important aspects of treatment and end-of-life decisions known to your healthcare proxy. It is important to keep an open, continuing dialogue with your healthcare proxy as your thoughts and feelings may change over time.
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