CF is a genetic, chronic disease. It affects the lungs and digestive system. A defective gene and its protein product cause the body to produce thicker and stickier mucus than normal.

The mucus:

• Clogs the lungs and leads to life-threatening lung infections
• Blocks the pancreas and stops natural enzymes from helping the body break down and absorb food

When the disease was first identified, children often died young. As research and treatments have advanced, people with CF are living longer.

Symptoms of CF

People with CF can have a variety of symptoms, including:

• Very salty-tasting skin
• Persistent coughing, at times with phlegm
• Frequent lung infections
• Wheezing or shortness of breath
• Poor growth or lack of weight gain despite a good appetite
• Frequent, greasy, bulky stools or difficulty in bowel movements

Quality and outcomes

Gaining weight and growing at a normal rate can be hard for kids with CF. They usually have better lung function if their BMI percentile is higher. BMI is a measure of a person's size based on their weight and height. If the number is too high or too low, it may cause health problems.

Treatments help you manage your child’s CF. Our care team will provide the support you need every step of the way. And they’ll help you find the right care options for your child.

Medicines

Medicine is a key way to control symptoms. Medicine can help keep your child out of the hospital and improve their quality of life.

Common types of medicine include those that help:

• Open the lungs and relax the airways
• Thin mucus
• Relieve pain
• Treat or stop infections
• Stop swelling in the airway and a drop in lung function

If your child has problems taking their medicine, talk to your care team. Report any side effects your child is having — the care team can help address them.

If your child has an immediate, severe reaction to the medicine, seek medical attention right away. This includes swelling, sudden breathing trouble (especially if using a nebulizer), rashes or itching.

Clinical trials 

Clinical trials are the studies of new ways of treating CF. Kids who take part may receive new types of care, such as medicine or devices. We compare these to the ‘standard’ care options.
We may ask if you’d like your child to take part in a study at our center. You can also use the Clinical Trial Finder to search for studies based on location, age or mutation.

Children’s Wisconsin and Froedtert & the Medical College of Wisconsin are part of the Milwaukee Cystic Fibrosis Care Center. We are accredited by the Cystic Fibrosis Foundation. That means the center meets rigorous national standards. We give people with CF high-quality care tailored to their condition and needs.

We’re also a Cystic Fibrosis Therapeutics Development Center. That means we conduct clinical research. We are a part of a national network designed to support and run clinical trials for new CF treatments.

Research studies

All our CF doctors take part in research. They work to improve diagnosis and treatment for kids with CF.

Our research includes:

• Clinical trials: Studies of new medicines or devices to treat CF.
• Observational studies: Researchers watch health outcomes in groups of people. They do this to learn about their health over time.
• Translational research: Researchers use lab findings to develop potential treatments for diseases.

Each research study has guidelines for who can join. Researchers base these on factors like genes, age, or treatments. You can ask your care team about studies that may be a good fit for your child.

Cystic fibrosis registry

We participate in the Cystic Fibrosis Foundation Patient Registry. This is a way we gather data on the health of people with CF. It is up to you if you want to take part in the registry.

The data we collect helps create care guidelines, assist care teams and helps improve care. Researchers also use this registry to study CF treatments and outcomes, and design CF clinical trials.

At the Cystic Fibrosis Center, our goal is to make treatments for Cystic Fibrosis more effective.

Partner with the Cystic Fibrosis Foundation: We are fully accredited for care, teaching and research by the foundation. The center's clinical outcomes have remained outstanding for many years.

Award-winning care: U.S. News & World Report often ranks pulmonary care at Children's Wisconsin among the best in the nation. Because of its impact on the lungs, Cystic Fibrosis care is pulmonary care.

Outstanding quality: We received the 2015-2016 Quality Care Award from the Cystic Fibrosis Foundation. This award recognizes our team for our efforts to always be improving patient care and results.