Alumni couple cope with the challenges of cystic fibrosis

Rebecca Martello Poole and Ray Poole

For Rebecca Martello Poole, a 2000 graduate of the Diagnostic Genetic Sciences Program in the Department of Allied Health Sciences, genetic diseases are not just a professional concern. Rebecca was diagnosed at the age of six months with cystic fibrosis (CF) and has suffered from the debilitating effects throughout her life.

Rebecca met her husband Ray Poole, also a 2000 UConn graduate, in mechanical and materials engineering, while at UConn, and the two moved from Connecticut to Wisconsin, then Kentucky, where they currently make their home. Together they’ve traveled a harrowing path between life and death as Rebecca spent almost two years enduring medical procedures. It started in October of 2014 during a weekend visit to Colorado. She fell ill, landing in the hospital, septic and in heart failure, fighting for oxygen. She continued to decline and on New Year’s Eve, she went into respiratory failure and was placed on life support. At that point the doctors told the couple that she was in end stage CF, and time was not on her side.

Rebecca needed a lung transplant but was too sick for the surgery. She was in a chemically-induced coma for six weeks, spent six months on a ventilator and finally grew strong enough to become a lung transplant candidate. After the transplant, she still had to undergo abdominal surgery, then months of recovery that included hours of physical therapy.

Today, Rebecca is almost two years post lung transplant, and while she has her difficult days, she’s feeling better. “It’s amazing to be able to walk or take a small hike and not cough incessantly,” Rebecca says.

The CF continues to affect her sinuses and GI tract and she suffers from CF-related diabetes. Rebecca must take a “handful” of pills four times a day, in addition to medications at mealtime, but her lungs are relatively healthy, and unless she is fighting a cold, she needs breathing treatments only twice a day every other month, rather than three to four times a day. Additionally, the daily percussive breathing treatments are a thing of the past.

While at UConn, Rebecca was a dedicated student. In addition to a major in DGS, she minored in molecular and cell biology. She was a University Scholar, Presidential Scholar and the recipient of various awards including the Allied Health Sciences Outstanding Senior Woman Student and the Courage Award. She also served as a Husky Ambassador and was a member of the Voices of Freedom gospel choir.

Rebecca speaks fondly of her time at UConn, saying that all her professors as well as the department support staff were very understanding when she was experiencing CF symptoms.

“Rebecca was a dynamo to work with as a student,” says Judy Brown, director of DGS. “She always arrived to lab class with a smile, but was focused and driven with a clear path toward her career milestones. Rebecca had a scientific mind with a hidden creative side—she was a talented country music karaoke singer!”

Prior to her lung transplant, Rebecca worked as a diagnostic scientist and clinical researcher. She loved the field, but due to her compromised immune system, she is currently unable to work in medical settings.

Ray quit his job in product management to care for his wife while she was gravely ill. How he dealt with the months of overwhelming stress is documented in his self-published book, Lessons From A CF Cornerman: 38 Lessons I Learned During My Wife’s Illness and Lung Transplant. He wrote it to help others going through critical medical challenges. His website chronicles their many struggles during that time.

Listening to the pair speak of Rebecca’s CF trials, one wonders how they stay so positive. “We appreciate today,” Ray says. “When we got together, we didn’t expect Becca to survive to her 30s. We are thankful for our time together.”

“I didn’t really think about what was ahead,” Rebecca says. “I wanted to be here for my husband. He sacrificed years of his life and he left his job, so I thought I just couldn’t quit. I focused on the here and now. I took it day by day, hour by hour.”

“We tried to keep things positive,” Ray says. “Every day gave us a chance to laugh or connect.”

Cystic fibrosis is a genetic lung disorder that affects the pancreas and other organs, such as the liver and gastrointestinal tract. Rebecca speaks excitedly about the advances in treating CF and hopes that the next generation of people with CF will live healthier lives. “The new FDA-approved medications don’t simply treat symptoms,” she explains. “They target the underlying cause—a faulty chloride channel protein.”

Rebecca goes on to explain that in a healthy cell membrane, chloride flows in and out of the cells within channels, with water following the chloride. In these faulty CF channels, chloride cannot flow out of the cells into the space between the cells, which means water cannot flow either. Consequently, the mucus between the cells is extra thick, which leads to many of the symptoms of CF.

“Much more research is needed,” Rebecca points out. “The last two medications address two classes of channel mutations. There are six classes and the medications are mutation specific.”

“The Cystic Fibrosis Foundation has supported drug development,” Ray says. “In Rebecca’s lifetime, life expectancy has grown from the teens to early forties.” That gives CF sufferers and their family one thing they need most: hope.

By Kim Colavito Markesich