Most patients begin cystic fibrosis (CF) treatment with our pediatric cystic fibrosis program, an accredited Cystic Fibrosis Foundation care center. University of Iowa Health Care is the only program in Iowa with an accredited adult CF center under the same system. This provides a seamless transition for adults who have cystic fibrosis.
Our goal is to help you live a full life with CF. With personalized care plans and access to new treatments through clinical trials, we’ll help you manage your cystic fibrosis.
What is cystic fibrosis?
Cystic fibrosis (CF) is a chronic, inherited condition that causes sticky, thick mucus to build up in your body. This leads to problems with your airways, organs, and reproductive system.
Cystic fibrosis symptoms
Cystic fibrosis symptoms can vary and may worsen or improve over time. Depending on the severity of the disease, you may not notice symptoms until your teenage or young adult years. These symptoms may include:
Persistent cough with thick mucus
Recurring lung infections
Recurring sinus infections
Foul-smelling, greasy stools
Chronic or severe constipation
Poor weight gain and growth
Cystic fibrosis diagnosis at UI Health Care
Most cystic fibrosis patients are usually diagnosed within the first month of life with newborn screening, before symptoms develop.
However, some adults and older children who did not get screened at birth may be diagnosed later. At UI Health Care, we’re able to diagnose older children, teens, and adults with a sweat test and genetic testing.
Cystic fibrosis treatment from UI Health Care
While there is no cure for cystic fibrosis, we offer the best possible treatment options to help ease and manage symptoms.
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:
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.
The future of cystic fibrosis starts at UI Health Care
You may have access to CF therapies not yet widely available through clinical trials and research at UI Health Care.
Cystic fibrosis clinical trials
UI Health Care participates in clinical trials through the Cystic Fibrosis Therapeutics Development Network, the world’s largest CF clinical trial network. The goal of these trials is to find innovative therapies that can further expand and improve treatments for cystic fibrosis.
Cystic fibrosis research
Our renowned University of Iowa research has paved the way for CF treatment and management for decades.
Some of our research focuses on:
Gene therapy for cystic fibrosis
Preventing mucus strands in the airway
New therapies for treating cystic fibrosis
Slowing the progression of cystic fibrosis
Understanding the biomedical cause of the disease
Cystic fibrosis treatment through molecular therapies