- Posted January 31, 2014 by
San Diego, California
This iReport is part of an assignment:
Living with a rare disease?
Lindsay Shipp....Running Towards a Dream
When I was a baby and well into eating solid foods, I would eat, and then I would cry. My post-meal cries were raucous and all-too-consistent, and this troubled everyone around me because I was, by all accounts, an easy-going, happy infant. Not a “crier.” It was 1984. My mom spoke with her brother Barry, to see if he could provide any wisdom into what was happening with her youngest child. Barry, who occasionally donated his time helping a local chapter of the Cystic Fibrosis Foundation, suggested she get me tested for the disease--because he knew that children with Cystic Fibrosis had difficulty digesting food. Why not order a sweat test--elevated sweat chloride levels diagnose Cystic Fibrosis--and rule it out, he said. I was nine months old when I was diagnosed with Cystic Fibrosis.
In the early 80s, a diagnosis of Cystic Fibrosis unequivocally meant a difficult life and an early death. Cystic Fibrosis, or “CF,” is a genetic disease that primarily effects the lungs and digestive tract. It causes the production of abnormally thick, sticky mucus in the body, which leads to blockage in the digestive tract and infection in the lungs. The majority of Cystic Fibrosis patients die from lung failure. In 1984, there were no significant treatments for CF, and the life median expectancy was less than 20 years. My parents were overcome with helplessness and grief. But out of their despair, would become the Madison chapter of the Cystic Fibrosis Foundation in Wisconsin, and fundraising would be our lives’ work. Selling root beer floats and having yard sales to raise money for CF research may have seemed like a small gesture, but to us it was a religion. Seemingly no one knew about this disease, and we were determined to change that.
I became the national postal child, or “the face” of Cystic Fibrosis for many years. I was the child on the penny jar by the store clerk. Due to my mom’s tenacity, I was featured in a piece on the CBS Evening News. My first day of kindergarden made the front page of the Wisconsin State Journal. But as I began school, my parents decided that it was time to let Lindsay define her own future, rather than Cystic Fibrosis defining it. My parents enrolled me in dance classes, and I sang and acted in plays in my spare time. Beginning at age 5, my school allowed me to administer my own digestive enzyme pills, which I had to take anytime I ate so I could absorb my food. But aside from this, I was a regular kid.
In high school, I discovered the healing potential of music. I was always a shower singer or a backup singer, but was too shy to advertise my singing voice to anyone interested. I took a leap and auditioned for a school musical at age 16, and once my teachers learned I had singing talent, they encouraged me to keep at it. I became a dedicated music student, and soon enough I was auditioning for--and landing--lead roles in musicals. But being a soloist had its challenges. I had a great instrument, but I didn't have the breath support that my peers did. I had to work extra hard to hit that high note. And what's more, exerting my lungs through singing was a form of chest physical therapy--meaning, it actively released mucus that was trapped in my lungs. This is good! But in front of an audience with a microphone on, I had to learn how to suppress this mucus and put a pause to my persistent cough, so that I could sing unhindered. By the time I had graduated college, I was singing regularly in shows and charity events, joined a professional choir and I even auditioned for American Idol at age 27.
But around the time I auditioned for the show, my declining lung function and increased mucus production in my body made singing more difficult than ever. The daily struggle to breathe without my body’s objection was foremost on my mind. Petite and frail due to my inability to absorb food, my childhood where CF was primarily a digestive disease seemed a world away. I was hospitalized approximately 4-6 weeks annually for respiratory infections. I devoted two hours daily to breathing treatments, including nebulized antibiotics and a percussive vest device that literally shook my chest, prodding the mucus out. I was taking between 20-30 pills daily, depending on what foods I ate. I worked a regular job, but it never occurred to me to pursue a "real" career. CF would put a swift end to these plans, anyhow.
In 2009, at the age of 25, I learned that CF patients with a rare genetic mutation (affecting 3-4% of CF patients) were being recruited for a promising Phase III, three-year-long clinical trial. I possessed this mutation, and I was one of two people who participated in the study in my city. The treatment being studied was a pill, and there was a 50 percent change that patients would receive a placebo. But what on earth did I have to lose?
By early 2010, my difficulty breathing significantly decreased. I couldn’t believe what I was feeling. At that time, I couldn’t go two months without getting a lung infection, and I had made it from January until summer, infection-free. I steadily gained weight. I fell in love and got married. I started to entertain the idea that I could have children of my own, and live a full life.
But I needed to get in shape. Even though I was a dancer in childhood, I was largely sedentary in adulthood. Difficulty breathing was one thing--but the regular stares of people at the gym as they watched me cough uncontrollably at my feeble attempt at exercise was another. But that all changed in early 2012, when I decided to give my “new” lungs a test. I stepped on a treadmill only to run a quarter-mile in fifteen minutes before I was too tired to continue, but I was so proud of what I’d accomplished. I wanted to keep at it--to see how far these lungs could go. In September 2012, I ran my first 5K race. In May 2013, I ran my first 10K. In August 2013, I ran my first half-marathon in under two hours. Now I’m running a half-marathon every 6-to-8 weeks, on average.
These days, I run about 30 miles a week. When I’m training for a race, that number increases to about 50 miles. In early 2012, my clinical trial concluded when the study drug, Kalydeco, became FDA approved. As I knew all along, it was confirmed to me that I was taking the “real” drug since I enrolled in the study in 2009. These days, my nebulizer and vest therapies have been supplanted entirely by running. I still have years of built-up bacteria and scarring in the darkest corners of my lungs, and I still get the occasional lung infection. But my persistent cough is no more, my ambitions are unbound, and my future is absolute.
There are several cyclists and runners that I see regularly on the trails in my neighborhood. When I tell them my finish time for a race, they’ll say, “so, you must’ve ran track in high school, right?” And I just giggle. If only they knew.