By Kelly Berg
I am a 37-year-old female and I have Congenital Muscular Dystrophy. With CMD, the degree of respiratory involvement varies. My respiratory weakness began in early childhood and has slowly progressed as I’ve gotten older.
When I was a young child, a normal cold virus often turned into an upper respiratory infection or pneumonia. At age eight, I came down with a severe case of pneumonia; had to get a tracheostomy; and was hospitalized for two months. Eventually my condition got to the point where whenever I got a cold, I was put on antibiotics so it wouldn’t turn into an upper respiratory infection or pneumonia.
I had scoliosis surgery at age 17. My doctors recommended I get a trach at the same time because they anticipated me having trouble getting weaned off the ventilator after the operation. They were right; I was on the ventilator for two months after surgery. During this time, several unsuccessful attempts were made to take me off the ventilator. It was awfully scary to feel like you can’t breathe. I eventually knew when I was strong enough to be weaned from the ventilator. At that point, I didn’t panic at all. I was finally strong enough to breathe on my own. My trach was removed after I was off the vent for several days. It was during that same hospital stay (3 months in total) that I was put on a bipap at night via a nasal mask.
At first, I did not like the bipap at all. After getting used to it, though, I grew to love it. Using the bipap during the night made me feel so much better in the morning. I was on nighttime ventilation until I was 26.
It was at this time that I began to get headaches and feel very fatigued in the afternoon. This was caused by my CO2 levels rising as the day went on. At first, I just had to go on my bipap machine for an hour or so to alleviate these symptoms. Eventually, however, I got an LP-10 volume ventilator, which I used non-invasively (without a trach) through a mouthpiece.
Non-invasive ventilation has worked well for me and is what I prefer. There is less risk of infection and less care without a trach. Currently, my respiratory equipment includes a Cough Assist and Trilogy ventilator. Cough Assist is a great machine for people with weak respiratory muscles. It makes your cough stronger and more productive. Since I got a Cough Assist, I haven’t had any upper respiratory infections.
I got my Trilogy ventilator eight months ago, and it, too, has made my life so much better. Before the Trilogy, I had a bipap and separate volume ventilator, which were each double the size and weight of the Trilogy. Since the Trilogy has both bipap pressures and volume ventilator settings, I only need one machine. The Trilogy has the option of dual settings so I have my volume (primary) setting during the day and my bipap (secondary) setting at night. Bipap pressure settings are more comfortable for me to use with a nasal mask at night and volume settings seem to work better with a mouthpiece during the day.
Another advantage of the Trilogy is that it is very portable, as it weighs only 11 lbs. Since it is lightweight and also has a carrying bag accessory, I can hang it over the back of the chair on my travel scooter when I go out. There is a three-hour internal battery, and each external battery lasts three hours as well. The external battery is small and snaps into the back of the Trilogy. In addition, it has an optional rolling stand accessory. This works well for me, as I live independently. I can easily wheel my vent anywhere in my apartment.
Non-invasive ventilation has worked for me, thanks to a Cough Assist and Trilogy. I am currently able to be off-the-vent for short periods of time during the day. On a good day, I can be vent-free for up to an hour-and-a-half, with the total time off of around 4 hours a day. While the freedom is nice, my voice is pretty weak without the vent and gets weaker the longer I am off it, so I feel better and more comfortable when I am on the ventilator.
Despite having CMD and its associated respiratory weakness, I have been able to accomplish the things I have wanted. I earned an Associate’s Degree in Business Administration and have a Bachelor’s Degree in Accounting. I do accounting work for a local business and I have my own handmade greeting card business. As I am limited physically, I really enjoy the time I spend making cards and other crafts.
Although my respiratory weakness has gradually progressed, I have just adapted along the way. Sometimes I think back to when I only needed nighttime ventilation, and I wish it were still like that. For the most part, I have learned to live in the moment. In fact, I love the following quote by Eleanor Roosevelt: “Yesterday is history, tomorrow is a mystery, and today is a gift; that is why they call it the present.” All things considered, I am very blessed with great family and friends, and am very thankful for all I have done and for all I can still do.