Cystic Fibrosis (Pediatric)

Cystic fibrosis is a genetic disease causing mucus in the body to become thick and sticky. This glue-like mucus builds up and causes problems in many of the body's organs, especially the lungs and the pancreas. There is no cure and the disease generally gets worse over time; however, thanks to screening and new treatments, people with cystic fibrosis can live into their 40s and longer. At the Cystic Fibrosis Center at the University of Michigan, we have the largest program of its kind in the state, offering a multidisciplinary approach for complete care, along with an active research program with access to cutting-edge treatments as they become available.

In 2007, our program ranked first in the country for average forced expiratory volume in 1 second (FEV 1), age 6 to 17 years – an important measure of lung function. Since then, we have continued to rank in the top tier of programs nationwide. As a result of our clinical and research efforts, our patients’ expected life spans have steadily increased. On this basis, we created an adult program to support patients over 21 years of age.

People with cystic fibrosis can experience chronic, serious breathing problems, lung disease, digestion issues, growth problems and reproductive issues.

Cystic Fibrosis Symptoms

  • Breathing problems, coughing and/or wheezing
  • Delayed growth
  • Salty skin
  • Belly pain/distended belly
  • Loss of appetite and weight loss
  • Recurrent episodes of pneumonia, lung infections or sinus infections
  • Foul-smelling stools and/or diarrhea
  • No bowel movements due to an internal blockage in newborns

Most states screen newborns for cystic fibrosis. If the disease is suspected, other tests are order to confirm the diagnosis. Diagnosis generally includes a blood test and a sweat test – to measure the amount of salt that is in the sweat.

Early diagnosis and an individualized treatment plan are the keys to ensuring the best quality of life for people with cystic fibrosis. Treatment varies depending on the health issues the patient is experiencing as a result of cystic fibrosis and how the treatments affect your body. Generally, patients will be given a mix of medications (from antibiotics to inhalers for opening the airways) and therapy (breathing exercises, methods to clear mucus and sometimes oxygen therapy), along with diet, exercise and lifestyle recommendations. For severe cases, surgeries including lung transplant and a feeding tube may be necessary.

Cystic fibrosis is an inherited disorder. Both parents must be carriers of the gene in order to pass on the disease to the child. Our genetic counselors are available to educate you and answer your questions about genetic testing. They will also help you understand your risk of getting a disease related to genetics or of having a child with an inherited disease.

Schedule an appointment by calling us at 734-764-4123 for pediatrics or 734-647-9342 for adults.