Cystic Fibrosis

Cystic fibrosis is a chronic disease that affects the respiratory and digestive tract. A genetic disease typically diagnosed in childhood, the focus for cystic fibrosis treatment is to manage symptoms in a way that ensures an optimum outcome.

Living with the symptoms of cystic fibrosis as an adult requires a knowledgeable multidisciplinary care team. At The University of Kansas Health System, our cystic fibrosis program has been recognized as a Center of Excellence by the Cystic Fibrosis Foundation in recognition of our skilled doctors and commitment to outstanding care.

Cystic fibrosis is a lifelong condition that requires ongoing treatment and support. The symptoms of cystic fibrosis affect the respiratory tract, digestive tract and even reproductive function. You can only get cystic fibrosis if you carry certain genes from your parents.

Most people are diagnosed with cystic fibrosis in infancy or early childhood. In some cases, symptoms may not be identified as cystic fibrosis until adolescence or adulthood. Because there is currently no cure for cystic fibrosis, adults living with cystic fibrosis focus on effectively managing their symptoms to enjoy a higher quality of life.

Often, cystic fibrosis is diagnosed through a screening in infancy before any symptoms are apparent. When symptoms do appear, they can be many and varied:

• Chronic cough that often includes coughing thick mucus
• Greasy stools from undigested fat
• Inability to gain weight
• Increased salt content in sweat
• Intestinal pain, cramping, gas or constipation
• Persistent sinus issues
• Recurrent respiratory tract infections
• Wheezing or other breathing problems

Over time symptoms can improve, worsen or change. For adults with cystic fibrosis whose condition went undiagnosed in childhood, symptoms may be milder.

The primary risk factor of developing cystic fibrosis is having 2 parents who each carry the gene that causes the disease. Only those who inherit the gene from both parents will develop cystic fibrosis. People who inherit the gene from only one parent become carriers of the disease but will not show symptoms themselves.

Today, newborn babies receive cystic fibrosis screening within their first 2 weeks of life. For people who were born before newborn screenings were implemented, other tests may be needed to confirm a cystic fibrosis diagnosis:

• DNA test to confirm a cystic fibrosis diagnosis
• Sweat test to measure the amount of salt present in the sweat
• Tests to evaluate stools for fat absorption

Living well with cystic fibrosis requires an individualized treatment plan that's customized to each person's needs. Your care is managed through a clinical nurse coordinator who provides a single point of contact as you work with cystic fibrosis doctors and specialty clinicians. Those specialties include critical care support, infectious disease, pharmacy, psychology and pulmonology, as well as:

The mission of the adult cystic fibrosis team at The University of Kansas Health System is to grow old with our adult patients by providing outstanding care, educating others regarding cystic fibrosis management and looking for treatments of tomorrow by participating in clinical trials today. For example, our organization was 1 of 115 participating sites in a clinical trial that led to FDA approval of a breakthrough drug. We were among the nation's first providers of Trikafta™, which dramatically improves quality of life for many patients managing cystic fibrosis.

The University of Kansas Health System is a nationally accredited cystic fibrosis care center that cares for both adult and pediatric cystic fibrosis patients. The adult cystic fibrosis clinic was founded in 1993 and was accredited by the Cystic Fibrosis Foundation in 1996. Additionally, for those requiring inpatient services, dedicated pediatric and adult specialty care is available.

We have built a cystic fibrosis treatment program of excellence that is focused on patient care. We have grown to become one of the largest cystic fibrosis adult treatment centers in the Midwest region. As the only adult center in the Kansas City metropolitan area, we serve an extensive patient population in western Missouri and eastern Kansas.