Cystic fibrosis (CF) is a life-shortening, inherited condition that causes bodily secretions to become thick and sticky. While these fluids would normally act as lubricants, the secretions in people with CF tend to plug up their body's ducts and passageways.
The CF program at University of Iowa Stead Family Children’s Hospital is an accredited Cystic Fibrosis Foundation care center, and the only program in Iowa that has an accredited adult CF center under the same health care system.
With an experienced, multidisciplinary team of providers and a robust research history, our pediatric and adult centers offer the most advanced cystic fibrosis care available from birth through adulthood.
Our specialists work together to provide comprehensive, individualized care in one central location so that we can best support each child's unique medical needs.
Cystic fibrosis symptoms
Symptoms of cystic fibrosis can vary and may worsen or improve over time. Depending on the severity of the disease, some people may not notice symptoms until they are in their teenage or young adult years. These include:
- Salty-tasting skin
- Persistent cough with thick mucus
- Recurring lung infections
- Recurring sinus infections
- Nasal polyps
- Foul-smelling, greasy stools
- Chronic or severe constipation
- Poor weight gain and growth
The effects of cystic fibrosis
- Airways: Thick, sticky mucus clogs air passages, preventing mucus and inhaled contaminants from being removed from the airway. Repeated lung infections can lead to lung damage, causing breathing problems and difficulty getting enough oxygen into the body.
- Liver: Bile in the liver and gallbladder is thick and has difficulty moving into the intestine, potentially causing inflammation and scarring of the liver.
- Pancreas: Because pancreatic secretions are abnormally acidic, digestive enzymes are unable to reach the intestine, leading to incomplete digestion and poor absorption of nutrients. Lack of nutrition prevents normal growth in babies and children.
- Intestine: The earliest clinical manifestation of CF, occurring in 13 to 17 percent of infants with CF, is meconium ileus. This bowel obstruction happens when the meconium (the first stool after birth) in a child’s intestine is even thicker and stickier than normal meconium, creating a blockage in the ileum, a part of the small intestine.
- Fertility: 97% of male CF patients and 20% of females 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.
- Sweat glands: The sweat of people with CF has a high concentration of chloride and sodium. This effect is not harmful but was an indicator in the early days of diagnosing CF.
How UI Health Care experts diagnose cystic fibrosis
In 2006, a cystic fibrosis screening was added to the required Iowa Newborn Screen for every newborn. Now, cystic fibrosis is usually diagnosed within the first month of life, before symptoms begin to develop.
If your child's screen comes back positive for CF, they will be referred to an accredited CF center for a confirmatory test called a sweat test. Additional testing is sometimes required to confirm a diagnosis:
- Diagnostic sweat testing: Testing the salt levels of the child's sweat
- Nasal potential difference: Testing for potential differences in nasal measurements
- Genetic evaluations: Testing is completed to identify CFTR mutations
Sweat testing and genetic evaluations may also be needed for persons who have signs and symptoms of CF regardless of their newborn screening results. Adults and older children may not have received screening at birth.
Cystic fibrosis treatment and services from UI Health Care
There is no cure for cystic fibrosis, but symptoms can be eased or reduced with treatment.
Our center’s multidisciplinary specialists provide comprehensive care to address any symptoms or complications that may arise, aiming to improve the quality of life of our patients.
Outpatient treatments include:
A variety of drugs may be used to treat symptoms of CF as well as medications specifically designed to target the gene mutation that causes CF.
Airway clearance techniques
Otherwise known as chest physical therapy, these techniques help break up mucus obstructions, helping to reduce the risk of infection or inflammation in the airways.
Cystic fibrosis specialists
Cystic fibrosis affects many areas of a patient’s health; we provide comprehensive, expert care that addresses the unique needs of each patient. You’ll be cared for by:
- Nurse coordinator
- Genetic counselor
- Respiratory therapists
- Social workers
- Physical therapy
- Child life specialist
- Research coordinator
In addition to our primary team, we have access to specialized pediatric services to meet your child’s needs:
- Sleep medicine
- Pain and palliative medicine
- Interventional radiology
Cystic fibrosis research opportunities
In addition to clinic visits, you and your child will have opportunities to take part in innovative clinical trials that help advance the treatment of cystic fibrosis. You are welcome to speak to your provider if your family may be interested in participating in a clinical research opportunity. Or you can search for and enroll in a clinical trial on our clinical trials site.
Cystic fibrosis mucus defect present at birth
Impaired detachment of mucus from airway glands reduces the ability to clear particles and germs out of lungs.
University of Iowa cystic fibrosis research team awarded $11.5 million grant
University of Iowa researchers have been awarded a grant to help research cystic fibrosis treatment using molecular therapies.
UI strikes agreement with Pfizer Inc. to develop potential cystic fibrosis gene therapy
The University of Iowa Research Foundation recently finalized a license and sponsored research agreement with Pfizer Inc. to support the development of potential gene therapies for cystic fibrosis (CF).
A cystic fibrosis mystery solved
UI study identifies protein that causes problems for CF lungs
Sleuths on the trail of cystic fibrosis
Decades of research make Welsh a national leader in the fight against cystic fibrosis
Daughter helped by drugs tested on mother's lung tissue
Mother of children with CF's donated lung tissue would help create new treatment.
Move aside, mouse model, and bring on the pig
Researchers developed a pig model of cystic fibrosis, moving them closer to a more complete understanding of CF and its effects on the whole body.
Gene therapy gets another look
Exploring advancements in using gene therapy to treat CF.
Resources for families
The University of Iowa Stead Family Children’s Hospital Cystic Fibrosis Family Advisory Board is a group of volunteers who meet often with clinic staff to help improve the clinic and cystic fibrosis care. Composed of parents and children seen at our center, they are available to connect with you about any of your CF questions and concerns.
Cystic Fibrosis Foundation: A nation-wide organization that advocates for persons and families with CF.