Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disorder that affects mucus and sweat glands. It causes thick mucus to accumulate in the lungs, pancreas and other organs. The disease impacts breathing and causes an increased risk of frequent lung infections. Cystic fibrosis also affects the digestive system, sinuses, sex organs, bones and joints.

There is no cure, but improvements in diagnosis and treatment have greatly improved the outlook for people with cystic fibrosis. MU Health Care has pediatric and adult cystic fibrosis centers to help people manage their symptoms, improve their quality of life and live longer.

Diagnosis

Cystic fibrosis is usually diagnosed very early in life through a multistep process.

Prenatal genetic test: Expecting mothers can choose to have prenatal genetic testing, which will tell them with a high degree of certainty whether their fetus has CF. A thin needle is used to draw a sample of amniotic fluid for testing.

Newborn screening: Babies in Missouri have a few drops of blood drawn within the first few days after they’re born. The sample is mailed to a state laboratory to be tested for various conditions, including cystic fibrosis. If a child tests positive for an elevated level of a specific pancreas secretion, the sample’s DNA is tested to see if it has any mutations of the genes commonly associated with cystic fibrosis.

Sweat test: Babies who test positive for cystic fibrosis in the newborn screening or in a prenatal genetic test should have a sweat test performed at a CF Foundation-accredited center, such as MU Health Care’s Pediatric Cystic Fibrosis Center. A small amount of sweat is collected, and a high concentration of chloride in the sweat confirms a diagnosis of CF.

While CF is typically diagnosed very early in childhood, there is a version of the disease called atypical cystic fibrosis that often doesn’t cause symptoms until a person reaches adulthood. It can be diagnosed through a sweat test and genetic testing.

Symptoms

People with cystic fibrosis can have a variety of symptoms that include:

Salty-tasting skin
Persistent cough
Wheezing
Repeated lung infections
Stuffy nose
Slow growth
Low energy
Gastrointestinal issues

Treatment services

MU Health Care offers treatment for patients of all ages. We are the only health system in mid-Missouri whose cystic fibrosis clinics have been certified by the CF Foundation, meaning our centers offer the highest level of care for patients.

People with CF live longer, healthier lives if they receive consistent care from a team of specially trained providers in different specialties. Our team includes doctors and a nurse, dietitian, social worker, respiratory therapist and a pharmacist. They all work together to cover every aspect of your care.

Our combined pediatric and adult programs work together to provide outstanding ongoing care. We are happy to provide more information about our patient outcomes and clinical trials participation.

Pharmacy

Our Mizzou Specialty Medicine pharmacy provides excellent service in CF specialty medicine to all patients. We can help review your medication list, arrange prescription refills and work with you to obtain prescription renewals or prior authorizations, all in a timely manner.

You can reach our pharmacy by calling 573-882-8288.

Outpatient clinics

Pediatric Cystic Fibrosis Center: For more information, please call the Pediatric and Adolescent Specialty Clinic at 573-882-6921. MU Health Care also offers comprehensive care in pediatric pulmonary medicine for many other lung and breathing conditions.

Adult Cystic Fibrosis Center: If you are new or considering moving to the area, please call 573-884-2591 and a member of our team will speak with you about cystic fibrosis treatment at MU Health Care, answer your questions and help schedule appointments.

Evidence has shown patients who get frequent, quarterly follow-up care have better long-term outcomes. During our appointment scheduling process, we strive to make all your quarterly appointments at once for your convenience.

The adult outpatient clinic meets from 1-5 p.m. on Tuesday afternoons at University Hospital’s Medicine Specialty Clinic. We know your time is important, so we strive to make your appointment as efficient as possible.

Your visit to the adult CF clinic

Two weeks before your visit, you’ll receive an electronic form that will prompt you with suggestions of topics you might want to discuss with different team members

Plan to arrive 30 minutes prior to your visit time.
Patient registration is in the main lobby at the central check-in desk.
Prior to clinic time, a member of the team will inform you of any testing needed for the appointment. All are located near the clinic.
Pulmonary Function Test (PFT) is completed with each visit.
A chest X-ray is completed yearly.
Blood work is completed a minimum of once yearly.
Expect a visit to last a minimum of 1 hour.

More information about the adult CF clinic

Our doctors and our clinical nurse specialist use the online MU HealthConnect patient portal. You can find information there about previous visits and test results whenever you need it.

If you are sick and think you might need inpatient care, you can call the center at 573-884-2591 from 8 a.m. to 4:30 p.m., Monday-Friday. On the weekend or after hours, you can call University Hospital at 573-882-4141 and request the CF doctor on call.