Cystic Fibrosis

A multidisciplinary approach

Cystic fibrosis can affect many aspects of your health and quality of life. We’ll personalize your care plan based on your distinct needs. This starts with determining how cystic fibrosis is affecting your:  

• Airways: Repeated lung infections can lead to lung damage, causing breathing problems and difficulty getting enough oxygen into the body. 

• Liver: Since thickened bile in the liver and gallbladder has difficulty moving into the intestine, it may cause inflammation and scarring of the liver.  

• Pancreas: Because pancreatic secretions are abnormally acidic, digestive enzymes cannot reach the intestine, leading to incomplete digestion and poor absorption of nutrients.  

• Gastrointestinal system: This can start with the first stool (meconium) after birth. The thicker and stickier meconium blocks part of the small intestine. As you get older, you may experience various GI issues like constipation or thick stools.  

• Reproductive system: 97% of men and 20% of women with CF are infertile. Thick secretions in the sperm canal can block sperm from getting through. Women are often able to conceive but may have decreased fertility because of thick cervical mucus blocking the entry of sperm. 

At UI Health Care, you’ll have a team of specialists working together on a care plan that addresses your unique needs. These experts may include:  

• Pulmonologists  

• Nurse coordinators  

• Genetic counselors  

• Respiratory therapists  

• Social workers  

• Dietitians  

• Physical therapists  

• Psychologists  

• Endocrinologists  

• Gastroenterologists  

The transition of adult cystic fibrosis treatment

Our specialists work together to provide individualized care that best supports your unique medical needs from childhood into adulthood.  

We have one central location for our pediatric and adult clinics. That allows you to transition from pediatric to adult care seamlessly. Additionally, our adult and pediatric teams meet regularly to keep you informed and cared for at every point of the transition.  

Most importantly, we help adult patients with cystic fibrosis as their needs change. Whether you need support with fertility challenges, sexuality, or mental health, our team will help you navigate this new phase. 

At UI Health Care, we help you change, update, and manage your new goals as you transition from childhood to adult cystic fibrosis care. 

Cystic fibrosis drug breakthrough

Mutations in the CF gene cause cystic fibrosis (CF). Various drugs treat this, including TRIKAFTA® which became available in 2019. 

TRIKAFTA® (elexacaftor, tezacaftor, ivacaftor) targets the CFTR protein defects to help them function better. You and your provider can discuss if this treatment is right for you.  

This groundbreaking medication was a result of extensive research, in which University of Iowa professor Michael J. Welsh, MD, played a pivotal role. In fact, he won the 2022 Shaw Prize in Life Science and Medicine for his contribution.  

Cystic fibrosis airway clearance

Cystic fibrosis treatment relies on effective airway clearance therapy (ACT), also known as chest physical therapy. ACT helps break up mucus obstructions and reduces your risk of infection or inflammation in the airways. 

Our UI Health Care providers work closely with our respiratory and physical therapists to maximize the effectiveness of ACT treatments.