Pediatric Cystic Fibrosis
Overview
Cystic fibrosis (CF) is a complex, life-threatening, inherited disease that primarily affects the lungs and gastrointestinal system. Children and adolescents with cystic fibrosis have difficulty breathing and may have recurring lung infections. CF can also affect other organs, such as the pancreas, where it prevents the release of digestive enzymes that enable your child’s body to absorb vital nutrients.
CF is a genetic disorder that is passed on when a child inherits two copies of a defective CF gene, one from each parent. Most people with CF are diagnosed at birth with a screening test performed on all newborn babies across the country. After the initial screening, diagnosing CF is a stepwise approach that includes a sweat test, genetic testing, and a clinical evaluation at a Cystic Fibrosis Foundation-accredited site.
Today, the median predicted survival age is close to 47, according to the Cystic Fibrosis Foundation. Early diagnosis and treatment can significantly improve life expectancy and lead to fewer complications.
Our approach
Founded more than 50 years ago, the Minnesota Cystic Fibrosis Center within M Health Fairview is one of the oldest, largest, and most experienced CF centers in the world. Thanks to early detection, ongoing management and newer therapies, care for people with cystic fibrosis has changed dramatically. In recent years, the number of hospitalizations for children with cystic fibrosis has gone down considerably. At the same time, life expectancy continues to rise. Read more about these advancements in care from Samuel Goldfarb, MD, a pulmonologist, professor at the University of Minnesota Medical School, and center co-director.
Our center is home to a renowned multidisciplinary team that provides compassionate, comprehensive care to patients to help them achieve the highest quality of life possible. Our staff includes physicians, nurse practitioners, nurse coordinators, respiratory therapists, dietitians, social workers, and genetic counselors. We also work closely with pediatric specialists from other fields who are widely regarded as international experts in CF-related diabetes, CF-related sinus disease, and gastrointestinal complications of cystic fibrosis.
We begin working with patients when they’re very young, and help educate families from day one, so that they are empowered, can become their own advocates and – at an appropriate age – begin to take care of themselves.
Research and Innovation
Many of the physicians at our center are also researchers developing breakthrough therapies for CF, or leading studies to better understand and control potential complications of the disease. We provide our patients access to a variety of clinical trials and encourage you to take advantage of them for your child. As the first center in the United States to apply preventive care successfully to CF patients, our cystic fibrosis patients have a longer life expectancy than any others in the United States.
The Minnesota Cystic Fibrosis Center is proud to participate in leading edge cystic fibrosis research to improve care and outcomes in our CF patients, and we are actively involved in research studies in patients from 0-24 years of age. Over the years our center has participated in over 100 research studies for cystic fibrosis involving both children and adults. We have advanced testing techniques including the multiple breath washout test, which measures a patient’s lung clearance index (LCI). This testing device has been used in studies over the past decade with newer modifier medications that have improved patient outcomes. Our center is also leading pediatric research into airwave oscillometry — a test that measures lung function in patients as young as 3 years old. Both devices are noninvasive and require a patient to breath normally for as little as 30 seconds, or, in some cases, up to 2-3 minutes. This new method could help us identify lung disease in CF even earlier.
Former University of Minnesota expert Warren Warwick, MD, invented the percussive vest, also known as the Vest Airway Clearance System. The wearable vest inflates and deflates rapidly, causing a high-frequency vibration that loosens mucus that builds up in the lungs of cystic fibrosis patients. The vests, which are still used today, have helped our patients lead healthier, more independent lives.
