Life on the Vent

The other side of the mountain

by Erin Brady Worsham on June 1, 2001 - 9:47pm

Macy's Thanksgiving Day Parade, 1997

Erin Worsham's self-portrait "Breathtaking Metamorphosis" depicts her new life with a ventilator.

The Macy's Thanksgiving Day Parade has long heralded the start of the Christmas shopping season. In 1997, it marked the beginning of a new life for me. It had been just over three years since my husband, Curry, and I received my diagnosis of amyotrophic lateral sclerosis (ALS) and I was given three to five years to live.

I lay in bed in my Nashville home that morning, as I had done the two previous days, too exhausted to get up, and watched the televised parade. Helium-bloated Garfield, Bullwinkle and Blue from "Blue's Clues" floated ponderously down the streets of New York.

An unnatural stillness in my body suddenly made me conscious that my heart was pounding madly. I had stopped breathing, just like that. After three years of watching and waiting for this moment, death was slipping past my vigilant guard.

My body rejected death's invitation to rest and fought for every breath.

Curry was in the room when I went into respiratory arrest. I couldn't find the breath to tell him I couldn't breathe. He initially left the room and called the nurse who handled our case. But then, seeing my blue lips, he called 911 and the paramedics were there in five minutes.

Our 2-year-old son, Daniel, was bundled off to the neighbor's house. I wondered when would I see him again.

After their record-setting arrival, the paramedics took their time setting up. They certainly weren't panicking — why should I? They started me on oxygen and transported Curry and me to Vanderbilt Hospital. As the oxygen flowed, my fear melted and I gave thanks.

The plan goes forward

What happened that Thanksgiving morning may have surprised us, but how we chose to proceed had been in the planning for a long time. We'd already discussed with my pulmonary doctor, James Snell, the time frame for my getting a tracheostomy and ventilator. He must have known it wouldn't be long when he told Curry to call 911 if anything happened over the holiday.

Three years earlier, I wasn't sure I wanted to continue living with ALS. Following my diagnosis, I staunchly declared that I would not live on a machine.

That was before we discovered I had gotten pregnant the day after my diagnosis. For six years we'd tried to have a family. How could I think of dying while carrying such a precious gift inside me? So I decided to use whatever means possible to stick around and watch this miracle child grow up.

That meant getting a feeding tube, a tracheostomy and a ventilator when the time was right for each. Curry supported this decision. We made sure everyone working with us was aware of our choices.

I got my feeding tube in June 1997, when eating and drinking became too much of an effort and I lost 20 pounds. It took some time for my stomach to adjust, but then my body blossomed with the balanced nutrition and unlimited fluids I could have without fear of choking.

For three years I had climbed the mountain of ALS. My muscles weakened from the exertion and eventually gave out. The longer and higher I climbed, the thinner the air became, until I reached the summit on that Thanksgiving Day and couldn't breathe at all. The next day I fell gently to sleep under the anesthesia and awoke on the other side of the mountain.

A new therapy

Breath in, breath out, breath in, breath out, breath in, breath out. Six times a minute the ventilator filled my lungs. The rhythmical whoosh of the ventilator was now my constant companion. I soon found I didn't mind its steady cadence.

I naively thought that once I was plugged into the ventilator, I could go home and resume my life with my family. But there were arrangements to be made for my homecoming, Curry needed training with the ventilator, and I needed rehab.

Curry, Erin and their son, Daniel

Rehabilitation isn't usually associated with ALS, the logic being that there's no going back after the muscles have atrophied. No one told the therapists this at the Vanderbilt Stallworth Rehabilitation Hospital. They took me on as their first ALS patient.

People with ALS and other neuromuscular diseases have healthy lungs. What we lack is the diaphragm power to operate them properly. As the diaphragm (a muscle) weakens, it becomes harder to breathe deeply and cough effectively. Secretions build up in the lungs, which can lead to pneumonia and death. When I went on the ventilator, I was using only 8 percent to 10 percent of my lung capacity.

For two weeks the staff of respiratory therapists, under the direction of Stephen Lampe and Brenda Butka, worked me over mercilessly. I knew I was in trouble shortly after my arrival when two therapists came into the room with an Ambu bag, a balloonlike device that's used to pump air into the lungs for resuscitation or suctioning.

"You're not going to like this," they said. "But when we get finished with you, you will love it!"

Stallworth uses a very simple, but effective, therapy that was developed at Dallas Rehab (now part of Healthnet Hospital). The therapist covers the exhaust valve of the Ambu bag and hyperinflates the lungs. Then with the butt of the palm of one hand, he delivers a powerful push to the diaphragm as the air is released. This causes secretions from deep in the lungs to come flying out of the trachea. Saline helps loosen the secretions.

The first time I received this therapy, the pain was excruciating. The walls of my lungs had grown stiff from disuse and protested mightily at the hyperinflation. But each day they became more pliable and my capacity increased. I remember the morning the last big plug flew out and made my therapists very happy. As they had predicted, I soon began asking for the therapy.

Healing and grief

Though my lungs healed, my emotions remained in turmoil. Everything turned me into a puddle. Curry, who was staying with me in the hospital, would go and fetch Daniel every day. It comforted me to watch them eat supper together, or to have Daniel lie down and nap with me. I didn't want him to be afraid of his mama's new equipment.

From the moment we're born and take our first breath, breathing seems to be our God-given right. Having to rely on a machine for every breath, I relinquished that right forever. And so I grieved for that loss and began the process of learning to trust my ventilator and accept it as a part of my body.

One hurdle remained before I could go home. I had to get out of bed. It sounds simple enough, but after three weeks of lying down, I was terrified. It took four people to transfer me and then the ventilator to the wheelchair. For the next few days Curry wheeled Daniel and me about the halls of Stallworth.

An outstanding moment in our Stallworth stay was when I rediscovered my voice. Before having the tracheostomy and ventilator, I'd lost the ability to speak for lack of air. My new trachea was equipped with a small, inflatable collar, called a cuff. When inflated, the cuff prevented my making any sound, and it was kept inflated for some days after the surgery.

When the therapists at Stallworth deflated the cuff, my voice erupted from my throat in a loud, liberating shout! We called my family in Louisville, Ky., and blew them away when I said "hi."

During our two weeks at Stallworth, Curry closely watched the therapists as they worked. They began allowing him to do the therapy and the suctioning while they observed. His confidence was growing.

As the ambulance pulled into our driveway, my heart pounded, this time in happy anticipation of our homecoming. The EMTs wheeled me into the house on a stretcher. Standing in the living room, without a bauble on it, was the prettiest Christmas tree I'd ever seen. Curry had slipped out of the hospital and gotten it. We were home for Christmas!

The search for Florence Nightingale

We thought we had a good plan for my nursing care. I'm covered by the state-subsidized Tenncare. Snell prescribed 12 hours of nursing care a day to give my husband a rest. We had no reason to think this would be denied, but it had not been approved by the day I was released from Stallworth.

This necessitated our getting financial help from the family to hire people on our own. Though we talked to everyone we met in the hospital, trying to enlist nurses on our own was a bust. We couldn't afford to hire a nurse through an agency, so we had to settle for hiring a sitter. At least she could wake Curry up if there was a problem.

Our first sitter arrived at our house dressed in a leopard-printed outfit and confidentially told me this was her very first job as a sitter. My heart sank.

During the night, I awoke in a panic because the air was escaping from my open mouth as I slept. In my fear, I could make little sound. My guardian angel must have roused Curry, for he suddenly woke up. He set things to rights and went into the living room, where he found the sitter curled up and sleeping like a baby on the couch.

Incidentally, we solved the problem of the escaping air. Had I had my cuff inflated, it never would have happened, but I'm not comfortable with the idea of not being able to make any sound. We came up with a low-tech alternative. Now we leave the cuff deflated and I sleep with a washcloth between my teeth and cotton in my nose to prevent air from escaping.

Eventually, our social worker told us our nursing care had been denied. But we never received any direct notification from the state, nor any word about how to make an appeal. For six months we paid for a sitter out of pocket. The social worker encouraged us to get a lawyer and contest the state's decision.

Michelle Johnson with the Tennessee Justice Center thought we had a good case. She wrote a powerful letter to the state and we won, without ever going to court. Our case became part of a class action that resulted in the state's having to change its appeals process.

Being eligible for 12 hours of nursing care a day and actually filling those hours with nurses are two different things. Good nurses are a treasure!

We've been blessed with some excellent nurses over the years, but keeping our schedule filled is an ongoing battle. It's a fight we're more than willing to wage, because their help is indispensable in our lives.

Epilogue: The big, blue van

It's been more than three years since that Thanksgiving morning when I confronted my own mortality and chose to fight for my life. Daniel is an impudent young man of 5, who shamelessly bosses his mother around. Our big, blue van takes us everywhere around Nashville, Middle Tennessee, and as far away as Virginia and Michigan.

It hasn't all gone smoothly. There was the time Curry forgot the canister on the suction machine and had to fashion one out of a Styrofoam cup and duct tape. It was nothing to write the patent office about, but it temporarily did the job until we got home.

And who can forget the day we went to the Russian exhibit at the museum and my ventilator tube caught on the van's wheelchair lift and popped apart? Curry truly amazed me that day.

While "bagging" me with the Ambu bag, he lowered me on the lift and tried to reconstruct my tube. A fierce storm was blowing up around us. Curry took me inside the museum, put together my spare ventilator circuit and got me back on track, with time left to see the exhibit. It's always frightening when you can't breathe, but each crisis teaches us more about the ventilator and how to solve various problems.

Using the Stallworth therapy, I've remained free from pneumonia. Before leaving Stallworth, Lampe gave us some good advice: Keep it simple!

My ventilator and feeding tube keep me going, and my Liberator communication device interfacing with our computer allows me to speak, write and draw. I was an artist before I became sick. After the diagnosis, I lost the heart to create. When I found it again, I'd lost the muscle! With the Liberator, I can create again.

The other side of the mountain is more beautiful than I could ever have imagined. I wouldn't have missed this adventure with Curry and Daniel for the world.

People are mistaken when they say they can't live hooked to a machine. Curry likes to remind me that I'm not hooked to the ventilator, it's hooked to me! Together, we're living a good life.

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