Advances in Cystic Fibrosis Treatment Help Patients Live Longer and Better

October 07, 2019

University Hospitals Rainbow Babies & Children'sExperts in Children's Health

Fifty years ago, children diagnosed with cystic fibrosis (CF) had little hope of living long enough to become an adult. But amazing advances in research and treatment for the disease have dramatically changed that outlook. And UH Rainbow Babies & Children’s Hospital has played an integral role in those advances for decades. Now, children born with CF can live well into adulthood. Jennifer Wuersig is a shining example.

At 9 weeks old, Ms. Wuersig was diagnosed with CF. Her parents were told she would not live to age 12. But now 47, Ms. Wuersig is thriving, thanks to world-class, specialized care at UH Rainbow Babies & Children’s Hospital’s Adult Cystic Fibrosis Program.

“My clinical team at UH Rainbow offers a one-stop shopping approach to care for all my needs from ensuring I’m taking the right vitamins and meeting my exercise performance targets to managing my medications and addressing my other health issues," Ms. Wuersig says. "They are my lifeline.”

Industry Leader

UH Rainbow has been a leader in CF research and treatment for the past 60 years, says Kimberly McBennett, MD, PhD, Director of the Adult Cystic Fibrosis Program at UH Rainbow Babies & Children’s Hospital.

“As one of the first accredited CF centers in the country, we introduced multidisciplinary care for CF patients and have been involved in clinical trials that have led to major advancements in this disease, allowing patients to live longer lives,” Dr. McBennett says. “Today, more adults are living with CF than children. The Adult Cystic Fibrosis Program was created approximately 15 years ago to care for this growing population.”

Multidisciplinary Treatment

CF is a progressive disease for which there is no cure. People born with CF lack a needed protein that allows mucus to thin. As a result, sticky mucus clogs the lungs and other organs, leading to inflammation, infection and other problems. The disease requires ongoing treatment and care to prevent complications and keep individuals feeling well.

Through UH Rainbow Babies & Children’s Hospital’s Adult Cystic Fibrosis Program, adults with CF are mostly cared for by the same CF Center team as children with the disease, providing continuity of care and expertise. The program’s multidisciplinary team of CF specialists includes respiratory therapists, dietitians, pharmacists, nurses, social workers, physical therapists and others. The patient’s physicians only change from pediatric- to adult-trained specialists when he or she transitions to the adult program.

“Continuity of care is so important. Patients who begin care here as a baby often see the same specialists throughout their life,” Dr. McBennett says. “We are able to focus on the whole health of these individuals, not just their CF.”

CF patients also have the opportunity to participate in the program’s clinical trial opportunities, offering access to innovative treatments. At any given time, the program is involved in 20 to 25 active clinical research studies. And if patients require hospitalization, the hospital offers them a dedicated, adult inpatient unit.

A Full Life

A longtime patient of Dr. McBennett, Ms. Wuersig has never let CF hold her back. Growing up, she was involved in soccer and basketball and participated in the marching band. After high school, she went on to graduate college. She married her husband, Bob, and had two children despite being told it wasn’t possible. She currently volunteers as the communications manager for a local ministry, writes a blog detailing her faith journey as she overcomes the challenges of CF, maintains her children’s busy high school schedules and recently celebrated 21 years of marriage.

“In my lifetime, I’ve experienced the evolution of life-changing medications and therapies for CF. They are no longer focused on keeping me alive, but improving my quality of life,” c says. “For the most part, I’ve been blessed to lead a normal life despite my CF.”

With the help of her clinical team, Ms. Wuersig successfully manages her CF, participating in several hours of self-care therapies daily, such as wearing an airway clearance vest and inhaling needed medications. Every few months, she visits the KC and Ginny Bryan White Pulmonary Diagnostic Center at UH Rainbow for lung function testing, blood work and consultation with her CF team to ensure her treatment program is on track. Periodically, she requires hospitalization to receive IV antibiotics and aggressive airway clearance to keep her healthy.

“Dr. McBennett and all my team members are absolutely phenomenal. They understand what I’m going through and help me live my best life,” Ms. Wuersig says. “When I was younger, there was no such thing as an Adult CF Clinic. Children didn’t live that long. I’m so thankful to have benefited from such proactive care.”