The long road to breathing well
with Karen Roth and Linda (April 2000)

Linda here - I’ve known Karen Roth of Texas for years and recently asked her to tell me the story of how she got to be the dynamo in a wheelchair that she is. Here is her story:

I was three years old living in Waurika, Oklahoma, when I got the flu, and my right foot started turning in. My parents took me to Dallas to the Carroll Clinic. There had just been an outbreak of polio and that’s what they thought was wrong. They really didn’t know. My foot didn’t get much worse, but at the age of seven, I had tendon transplants done. In grade five, I noticed my right hand started getting a little weak.

I had played the piano since age four, and at 10 I noticed my right hand was weak as I played. Things levelled out, but each time I got the flu, I got worse, and I began to notice steps were beginning to be hard to climb.

In high school, I played tennis and basketball a little. I went on to Midwestern University in Wichita Falls, Texas, where I studied piano and voice. Things seemed to level out again. In my junior year, I had a bad case of the flu, fell down some steps and ended up in the hospital with a crushed foot. It was then I had an ankle fusion done on my right foot. It didn’t heal, and it had to be done again months later. In spite of all that, I entered graduate school, Texas Women’s University in Denton, Texas, to get my Master’s degree in music. School went well but the foot didn’t really heal. It never healed completely, and I began using a walker then because I couldn’t put all of my weight on it.

During my final year, I married a young man I had met back at Midwestern. I was going to sing at my own wedding but I had what I thought was a cold. We honeymooned in the Caribbean, and when we returned my voice was much worse. It was at that time that I was diagnosed with a paralysed vocal cord. That’s also when I thought my world came to an end because I loved to sing.

I got sick with the flu again and was sent to National Institutes of Health (NIH) in Bethesda, Maryland. They didn’t know what I had, so I came back after nine weeks there and set up housekeeping and got on with newly married life.

We moved to Plano, Texas, in 1968, and adopted two daughters, one in 1968 and one in 1973, and a third daughter much later in 1989.

In 1985, I was at the mall with my daughters, and there was a health fair going on. Just for fun, I took the breathing test they offered. They had a fit and told me I needed to go to the doctor immediately, my numbers were very low. I didn’t do anything, I felt great.

Now, I wonder if my having to go back to bed after getting the girls off to school was a sign that my breathing was bad, and I was experiencing fatigue.

Through Linda and CMT International, I found Dr. Charlie Chan in Toronto and went to see him. After all these years, he was the one who diagnosed me as having CMT, and he also told me that I had severe phrenic nerve involvement causing diaphragm weakness. He took me on a tour of the wards with people who had waited too long to get help for breathing problems. They were just sitting there with their breathing machines attached to them. It was awful. It really scared the hell out of me.

I came home, went to Dallas Rehab Institute and had more breathing tests done. The head of the pulmonary department said to me, “Karen, you want to live or die?” I stayed three or four weeks at DRI getting used to my PLV100 ventilator. It was awful at first. I didn’t have a good seal between my mask and face. There were many times I just wanted to give up. A dentist came in and made me a mask that hooked on to my mouth and my teeth, and it was awful, but I got through it and went home. I could only use it about three hours a night. I used to call it Darth Vader, I hated it so much.

Then, I went back to DRI for a checkup, and there was Richard, a new respiratory therapist. He said, “Karen, I think I can help you with your mask,” and oh, did he! He made me a customized mask to fit my face. It is very lightweight, has a good seal, is comfortable, and of course, the rest is history. I’ve done very well.

I use the machine every night with the mask Richard made me, and during the day, if I feel tired, I let it breathe for me about 10 times, and then I’m good late into the night. If it wasn’t for his mask and my machine, I don’t think I would be here today. It is my lifeline, I have energy and feel good most of the time. My vocal cord is no longer paralysed, and even though I now use an electric wheelchair, I’m able to sing in the Christ United Methodist Church Sanctuary Choir, which I love, and I’m still able to teach piano, harpsichord and voice.

Linda here - To find out the other side of treatment, Richard Hodgkins, Karen’s respiratory therapist who now works out of Silver Leaves Nursing Centre in Garland, Texas, spoke to me recently.

“At the time I was at the Dallas Rehab Institute, the program I worked in was based around neuromuscular diseases. People have to understand that the level Karen was at then and is at now is one where daily activity is exercise. Breathing itself becomes exercise. In order to achieve a proper healthy stasis, we need adequate nutrition, appropriate exercise and effective rest. This can be accomplished by nocturnal ventilation where you knock out all muscle use.

“There are two ways to provide ventilation, either invasive which is a tracheostomy or noninvasive which is a mask attached to a ventilator.

“Once the body reaches the point where breathing is exercise the chronic stress stimulates a high metabolism. It is like a long distance runner that gets no rest. You are always under chronic physical stress.

“You have to institute a phase of rest to extend life expectancy. Karen had difficulty singing and was experiencing difficulty with her quality of life. If done properly energy levels may improve.
“I learned from Dr. Viroslav and Sue Sorter of the Dallas Rehab Institute.

“The effect of the mask places the air directly into the nostrils and I make sure there is an anatomically correct surface area over and around the nose which ensures air going directly into the nostrils rather than blowing air at the nose hoping it goes in the nostrils.

“The mask is made out of silicone impression material. It is what a dentist would use to make impressions of the teeth.

“Karen originally has been on an oral/nasal mask because she was leaking air out of the mouth. On her second evaluation, we found that with the new type of nasal mask and her improved strength, due to her time on ventilation, we might be able to use just nasal ventilation for comfort and improved patient compliance. When you get down to three hours a night, you aren’t really using it; we had to find something Karen would and could use. It turned out that the nasal mask worked fine for her. The machine attached to the mask eliminates the use of the respiratory muscles completely giving her effective rest so she has improved energy during the day.

“To improve or ensure effective ventilation, you should have good chest wall mobility. If the chest is stiff from shallow or little movement, it resists air movement and it takes great pressure to put air into the lungs. A mask works best at low pressures, so a program of chest stretching can be effective to add mobility and reduce ventilating pressures. This is achieved with IPPV, intermittent positive pressure ventilation breathing, a machine used by mouthpiece set at a pressure prescribed by a physician, usually 40 centimetres of water pressure. This mechanically stretches the chest and should be done daily.

Another way of visualizing this would be if you had your arm in a sling. When you take the sling off, the arm is stiff. A physiotherapist would have to slowly stretch that arm out. The same works with the cartilage and muscles of the chest. The constant and consistent application of pressure gradually adds mobility to the chest wall. The effectiveness of the mask is improved with the mobility of the chest. Karen practises a daily regimen of chest wall mobility to keep her chest walls flexible.

“Anyone with neuromuscular disease should be careful how much activity they experience daily. It isn’t your lungs, it is the muscles you use to breathe and the energy you expend to breathe. That’s the problem. Remember, daily activity is exercise and breathing is exercise.

“It is difficult to recognize the benefit of ventilation and providing rest and breaking the cycle of chronic stress but it is very important that it be recognized.

“If you experience chronic fatigue with your CMT and feel you have breathing problems, try to identify if breathing itself is a burden. Relieving that burden periodically through nocturnal ventilation which gives your breathing muscles total rest may see you begin to thrive.”