Cystic fibrosis (CF) is a lifelong genetic condition. Children with CF carry a damaged gene that causes their bodies to make abnormally thick and sticky mucus. This mucus clogs airways and damages the lungs. The mucus also builds up in the digestive system and pancreas. Children with CF may experience serious lung infections and issues with nutrition and growth.
The symptoms of cystic fibrosis vary with each patient. Patients with CF may experience some or all of the following:
Cystic fibrosis occurs in about 1 in every 3,500 live births. About 1,000 new cases of CF are diagnosed each year in the U.S. A child with the condition inherits two copies of the CF gene; one from each parent.
When both parents carry the gene for CF, each pregnancy has a 25 percent chance the child will have CF, a 50 percent chance the child will be a carrier of the gene for CF but not have the condition and a 25 percent chance the child will not have CF and will not be a carrier of the CF gene.
About 10 million Americans carry the CF gene but do not have the condition.
Living with CF means following an aggressive care plan to manage symptoms so that children stay healthy and active.
Many children are diagnosed with cystic fibrosis as infants during a newborn screening, which is completed within the first 48 hours of life in Indiana.
If your older child shows symptoms of CF, a pediatric gastroenterologist or pulmonologist at Riley at IU Health can perform the following exams and tests to make a diagnosis:
Treatment for cystic fibrosis includes:
The Cystic Fibrosis Foundation recommends that children with CF complete at least four clinical visits each year. Each patient’s visit will be different based on his or her specific needs. Children and their families can expect the following during regular appointments:
At each appointment, you and your child will meet with the following medical professionals to discuss and review your child's healthcare plan:
More than 25 years of research has given the cystic fibrosis specialists at Riley at IU Health a better understanding of germs in CF, how germs are spread and the relationship between germs and lung health in CF.
Germs in CF can spread several ways, including:
The following guidelines help control infection:
All staff at the Cystic Fibrosis Program at Riley at IU Health wear gowns and gloves. This is to protect you and your child from indirect contact with secretions. Patient rooms are cleaned between each patient, including all chairs, tables and desks. Patients must wear masks upon entering healthcare settings to comply with infection control recommendations.
Visit the links below to find support groups and services and learn more about cystic fibrosis.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
This foundation researches and develops new medicines to fight cystic fibrosis and improve the quality of life for those with CF. Call the Indiana Chapter at 317.202.9210.
The Cystic Fibrosis Foundation provides this useful resource about treatments and therapies, including airway clearance techniques and chronic medicines to maintain lung health.
The CYACC at Riley at IU Health provides transition consultation, education and care coordination across all diagnoses. The team works with children and their families to create a portable medical summary and transition plan.
This is a nonprofit organization that helps patients with cystic fibrosis afford the medicines and devices they need. Many medicine manufacturers also offer patient assistance programs to help people without insurance afford their medicines. Each program has different standards for the eligibility, enrollment and approval processes. In most cases, a doctor or caregiver must start the enrollment process.
CF Living is an educational program designed to offer information and support for those living with or caring for someone with cystic fibrosis.
Helpful Information About Living With CF
Airway Clearance Technique Information
Nutrition Care Information
The following PDFs from the Cystic Fibrosis Foundation provide useful information about cystic fibrosis and nutrition based on your child's age:
How to Increase Calories
Calorie and Protein Content of Foods
Picky Eaters and Problem-Eating Behaviors
Pancreatic Enzyme Replacement
Cystic Fibrosis-Related Diabetes
The pediatric gastroenterologists and pulmonologists at Riley at IU Health participate in research on the treatment of cystic fibrosis. Speak with your child's doctor if you would like to know more about our clinical research studies.
Mallory Smith was smart, beautiful, accomplished and kind. She also had cystic fibrosis. Diane Shader Smith came to Riley to share her daughter’s insights into CF and to present, in the young woman’s words, “Salt in My Soul: An Unfinished Life.”Continue reading