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Pulmonary and Critical Care Medicine

Cystic Fibrosis (CF)


Cystic fibrosis is a relatively common cause of bronchiectasis. It is an inherited disease, usually diagnosed in the first months of life. Cystic fibrosis can also lead to sinusitis, asthma and a poorly working digestive tract due to problems in the pancreas. Milder cases can sometimes be missed and are only diagnosed in adolescence or adulthood.

Causes
Cystic fibrosis is inherited, which means the disease is passed from parents to children through genes. The parents will most likely not have CF, but rather, a defect in the CFTR gene that causes cystic fibrosis. Therefore, if a child inherits a faulty CFTR gene from each parent, they will have CF.

Symptoms
Symptoms of cystic fibrosis vary from person to person and may change over time. One of the first signs or symptoms of cystic fibrosis is that a child’s sweat may taste very salty. However, respiratory symptoms are most the common signs of CF. These may include frequent coughing, thick sputum/mucus, sinusitis, bronchitis and pneumonia. Digestive symptoms may include diarrhea, constipation, stomach pain and discomfort.

Diagnostic Testing
The two main tests for diagnosing cystic fibrosis include a sweat test and a blood test to look for the CF gene. Other testing may include chest or sinus X-rays and lung function tests. Prenatal CF screening can be performed, and in New York State, routine newborn screening looks for the presence of cystic fibrosis. In addition, because cystic fibrosis can affect the reproductive system, an evaluation for cystic fibrosis is often done as part of a fertility evaluation.

Treatment
There is no cure for cystic fibrosis. Therapy is tailored to each case and is aimed at preventing and controlling lung infections, reducing the amount of sputum/mucus production, preventing and treating intestinal blockages, and preventing dehydration.

Albany Medical Center is one of approximately 125 specialized Cystic Fibrosis Centers across the country accredited by the Cystic Fibrosis Foundation. Because Albany Medical Center is a Cystic Fibrosis Center, we are able to borrow many of the cutting-edge therapies developed for cystic fibrosis in treating other forms of bronchiectasis.

The Cystic Fibrosis Foundation has been the driving force behind research over the last several decades that has dramatically improved the quality and length of life in patients with cystic fibrosis. The model of care involves an entire team approach—a physician, a nurse, a dietitian, a social worker, and a respiratory therapist who see patients regularly. This team also works closely with the pediatric specialists of the Children’s Hospital at Albany Med who specialize in the care of infants and teenagers with cystic fibrosis.

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