Bright futures ahead: Young Patients Thriving Into Adulthood Thanks to Advancements in Cystic Fibrosis Care

Bright futures ahead: Young Patients Thriving Into Adulthood Thanks to Advancements in Cystic Fibrosis Care

Inspired by the lifelong cystic fibrosis care she received at Rainbow, 18-year-old Zoe Watterson selflessly used her wish from the Make-A-Wish Foundation to renovate the Cystic Fibrosis Center’s activity room. The center’s legacy of advanced discoveries in the treatment of cystic fibrosis is helping patients like Zoe thrive. Learn more at RainbowBabies.org/Zoe.

Before Zoe Watterson turned 1 week old, she had been diagnosed with cystic fibrosis – a rare and chronic disease that causes life-threatening lung and digestive problems. When Zoe’s parents learned that their newborn daughter had cystic fibrosis, they moved across the country so that Zoe could receive the highest level of care at University Hospitals Rainbow Babies & Children’s Hospital – a world-renowned leader in the treatment of cystic fibrosis. At the time, many children did not live to see their teenage years, but advances developed by her doctors are a big reason why the 18-year-old is thriving today and heading to college in the fall.

“UH Rainbow’s Cystic Fibrosis Center has an amazing history. Much of how the disease is treated in this country is because of the work of our center,” says Kimberly Spoonhower, MD, Director of the LeRoy W. Matthews Cystic Fibrosis Center at UH Rainbow Babies & Children’s Hospital. “We stand on the backs of giants and continue their advances in this important field.”

Leader in cystic fibrosis treatment

The Cystic Fibrosis Center is credited with many advances. The hospital was among the first to implement a multidisciplinary care model for cystic fibrosis patients, bringing together physicians from different specialties for the collective care of the patient. This unique treatment approach, developed in the 1960s, is now considered the standard of care in the U.S.

The center has also been at the forefront of research to develop new therapies and techniques to treat the disease. These advances have helped expand the length and quality of life for cystic fibrosis patients in the past few decades.

“The expected survival rate of children born with cystic fibrosis in the United States has tripled in the past 30 years. And our patients live longer than the national average,” explains Michael W. Konstan, MD, pediatric pulmonologist and former director of the Cystic Fibrosis Center at UH Rainbow Babies & Children’s Hospital.

Innovation and focused care

Dr. Konstan attributes the hospital’s higher-than-average patient survival to the center’s focused expertise, a more aggressive approach to care and better access to promising therapies through a strong clinical research program. Dr. Konstan and his team developed the only anti-inflammatory therapy currently recommended by the Cystic Fibrosis Foundation. This therapy benefited Zoe, who has taken it since infancy.

“Our center is now at the forefront of conducting clinical trials that target the genetic defect in CF,” explains Dr. Konstan. “We are getting closer and closer to a cure every day.”

A changing population

Treatment success and advances in life expectancy happily prompted the Cystic Fibrosis Center to add to its focus recently. In addition to treating children, the center has a large adult-focused program.

“Our patients are growing into adulthood, graduating from college, getting jobs, having families of their own. They needed a team that understands their unique health needs as adults,” explains Dr. Spoonhower. “We treat their cystic fibrosis as well as overall health care needs.”

Evolving spaces, inspiring minds

In addition to expanding their program focus, the Cystic Fibrosis Center is also expanding its space. This will allow improved access for patient care and ongoing clinical trials. And because of Zoe, part of UH Rainbow Babies & Children’s Hospital’s fifth floor – where cystic fibrosis patients are treated – recently received a major facelift.

As Zoe spent time on the floor receiving lung treatments, she began thinking the space could use some renovations. The teen asked Make-A-Wish, a nonprofit organization that grants wishes to children with life-threatening illnesses, for help.

As a result of Zoe’s unselfish wish, the fifth floor activity room was completely updated to be a fun and uplifting place for kids of all ages. And patients not only have newer facilities, but also a role model. Zoe, who is headed to Oregon State University in the fall, encourages others battling chronic illness. “Do not let it get you down. Be happy and stay positive,” she says.

Seeing Zoe’s bright future makes Dr. Konstan and the cystic fibrosis team even more focused: “It is our patients who inspire us to do what we do. They face their illness with unbelievable hope and courage,” says Dr. Konstan. “Zoe is a perfect example of a teenager who does just that.”

Zoe’s wish

See how Zoe Watterson made a difference for cystic fibrosis patients at UH Rainbow Babies & Children’s Hospital at RainbowBabies.org/Zoe

Milestones in cystic fibrosis care

The LeRoy W. Matthews Cystic Fibrosis Treatment and Research Center is founded.

1957 The LeRoy W. Matthews Cystic Fibrosis Treatment and Research Center is founded, providing the first comprehensive and effective therapeutic approach to cystic fibrosis treatment.

Carl F. Doershuk, MD, receives the nation’s first Core Center Grant for cystic fibrosis research from the National Institutes of Health.

1983 Carl F. Doershuk, MD, Chief of the Pulmonary Division, receives the nation’s first Core Center Grant for cystic fibrosis research from the National Institutes of Health.

Michael W. Konstan, MD, publishes a study demonstrating ibuprofen’s profound effect on cystic fibrosis patients’ pulmonary function, leading to its widespread acceptance as the first effective therapy to suppress inflammation in cystic fibrosis patients.

1995 Michael W. Konstan, MD, publishes a study in the New England Journal of Medicine demonstrating ibuprofen’s profound effect on cystic fibrosis patients’ pulmonary function, leading to its widespread acceptance as the first effective therapy to suppress inflammation in cystic fibrosis patients.

 Cystic fibrosis clinician-researchers conduct a first-of-its-kind gene therapy trial involving compacted DNA technology.

2002 Cystic fibrosis clinician-researchers, based on the research of Pamela B. Davis, MD, PhD, Dean and Professor of Pediatrics at Case Western Reserve University School of Medicine, conduct a first-of-its-kind gene therapy trial involving compacted DNA technology.

Michael W. Konstan, MD, coauthors a study on a new oral medication that provides major, sustained improvements in the treatment of cystic fibrosis.

2011 Michael W. Konstan, MD, coauthors a study published in the New England Journal of Medicine on a new oral medication that provides major, sustained improvements in the treatment of cystic fibrosis.

Kimberly Spoonhower

KIMBERLY SPOONHOWER, MD
Director, LeRoy W. Matthews Cystic Fibrosis Center, UH Rainbow Babies & Children’s Hospital
Assistant Professor, Case Western Reserve University School of Medicine

Michael Konstan

MICHAEL W. KONSTAN, MD
Pediatrics Department Chairman, UH Case Medical Center
The Austin Ricci Chair in Pediatric Pulmonary Care and Research, UH Rainbow Babies & Children’s Hospital
Chairman and Professor of Pediatrics, Case Western Reserve University School of Medicine

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