Adult Cystic Fibrosis Center

Bruce B. Ludwig, MD, FCCP, FACP
Carol D. Hawkins, FNP
Michael T. McCormack, MD, FCCP

The Heart Lung Vascular Institute at the University of Tennessee Medical Center developed the Adult Cystic Fibrosis Center in conjunction with the pediatric program of East Tennessee Children's Hospital to provide quality, multidisciplinary care for adult patients with cystic fibrosis. The purpose of the Adult Cystic Fibrosis Center is to provide a transition for pediatric patients to more age-appropriate care.

The after-house clinic is held once a month in the University Pulmonary and Critical Care offices of the the Heart Lung Vascular Institute (E-210). A multidisciplinary team made up of the following providers collaborates to ensure adult patients receive quality, comprehensive care:

• Physicians
• Nurse practitioners
• Registered Nurse
• Social workers
• Dietitians
• Respiratory therapists

What is Cystic Fibrosis?

Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is one of the most common chronic lung diseases in children and young adults, and may result in early death.

Causes and Risk Factors

Cystic fibrosis (CF) is caused by a defective gene which causes the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food.

Millions of Americans carry the defective CF gene, but do not have any symptoms. That's because a person with CF must inherit two defective CF genes -- one from each parent. An estimated 1 in 29 Caucasian Americans have the CF gene. The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It's more common among those of Northern or Central European descent. Most children with CF are diagnosed by age 2. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease.

Signs and Tests

A blood test is available to help detect CF. The test looks for variations in a gene known to cause the disease. Other tests use to diagnose CF include:

• Immunoreactive trypsinogen (IRT) test is a standard newborn screening test for CF. A high level of IRT suggests possible CF and requires further testing.
• Sweat chloride test is the standard diagnostic test for CF. A high salt level in the patient's sweat is a sign of the disease.

Other tests that identify problems that can be related to cystic fibrosis include:

• Chest x-ray or CT scan
• Fecal fat test
• Lung function tests
• Measurement of pancreatic function
• Secretin stimulation test
• Trypsin and chymotrypsin in stool
• Upper GI and small bowel series

Calling your health care provider

• Call your health care provider if an infant or child has symptoms of cystic fibrosis. If you do not have a doctor, allow us to help you find one.
• Call your health care provider if a person with cystic fibrosis develops new symptoms or if symptoms get worse, particularly severe breathing difficulty or coughing up blood.
• Call your health care provider if you or your child experiences:

- Fever, increased coughing, changes in sputum or blood in sputum, loss of appetite, or other signs of pneumonia

- Increased weight loss
- More frequent bowel movements or stools that are foul-smelling or have more mucus
- Swollen belly or increased bloating

For more information on cystic fibrosis nutritional consideration please click here.

To schedule an appointment, call 865.305.5888.