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Medical College of Wisconsin Cystic Fibrosis Program

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What is Cystic Fibrosis?

Cystic Fibrosis (CF) is an inherited disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections, obstructs the pancreas, and stops natural enzymes from helping the body to break down and absorb food. People with CF tend to have a lot of sinus problems.

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Cystic Fibrosis Information

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Symptoms

People with CF can have a variety of symptoms including:

  • Very salty-tasting skin
  • Persistent coughing (at times with phlegm)
  • Frequent lung infections
  • Wheezing
  • Shortness of breath
  • Poor growth/weight gain in spite of a good appetite
  • Frequent greasy bulky stools
  • Difficulty in bowel movements
  • Sinus issues
Diagnosis

Most people are diagnosed with CF at birth upon newborn screening, which became a nationwide standard in 2010. On occasion, someone is missed by newborn screening. A physician who sees signs and symptoms will order either a sweat test or a genetic test to confirm the diagnosis.

A sweat test is the most common test used to diagnose cystic fibrosis. A small electrode is placed on the skin (usually on the arm) to stimulate the sweat glands. Sweat is then collected and the amount of chloride, a component of salt in the sweat, is measured.

In a genetic test, a blood sample, or cells from the inside of the cheek are taken and sent to a laboratory to see if any of the various mutations of the CF gene are found. A genetic test is often used if the results from a sweat test are unclear or if more detail is needed to prescribe the best medication for the patient.

Important Links

Cystic Fibrosis Foundation
The Cystic Fibrosis Foundation is the world's leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.

Cystic Fibrosis
CysticFibrosis.com supports the largest and oldest global Cystic Fibrosis community on the web, founded in 1996, comprising patients, caregivers, healthcare professionals and industry service providers.

 Providers

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Julie A. Biller, MD

Chief, Professor

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Rose Franco, MD

Professor

Brooke Foster, PharmD, BCACP
Pharmacist

Pat Pfahler, RN, BSN
Adult Cystic Fibrosis Coordinator

Julie Siegel, MSW
Master's of Social Work

Michelle McDonagh, RD, CD
Registered Dietician

Tracy Zimmerman, RRT
Registered Respiratory Therapist