Cystic Fibrosis

Overview

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system, which causes the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections, obstructs the pancreatic ducts and stops natural enzymes from helping the body break down and absorb food.

UW Medical Center is the only adult cystic fibrosis center in Washington, Alaska, Montana and Idaho region accredited by the Cystic Fibrosis Foundation. As one of 13 Cystic Fibrosis Foundation translational research centers in the United States, UW Medical Center can give its patients access to the latest treatments available for treating the disease.

The cystic fibrosis clinic provides a multidisciplinary approach to care, which means that a patient will be able to see providers managing many different parts of their health care during the same clinic visit in the same location. Those health-care professionals could include doctors, nurses, respiratory therapists, nutritionists and social workers.

The clinic also works with a network of providers throughout UW Medical Center to provide patients with access to specialists in many disciplines, such as otolaryngology, hepatologists, liver care, male fertility and obstetrics.

Symptoms

People with cystic fibrosis have a variety of symptoms, such as:
  • Persistent coughing, at times with phlegm
  • Frequent lung infections
  • Wheezing or shortness of breath
  • Poor weight gain
  • Frequent greasy, bulking stools or diarrhea

Causes

Cystic fibrosis is caused by a defect in the CF gene. The condition is inherited when a child receives both parents’ recessive genes. In the United States, one in 30 people carry the gene that causes cystic fibrosis, and one in 3,000 babies born in the United States have the disease.

Risk Factors

The biggest risk factor for cystic fibrosis is having parents who carry the gene for cystic fibrosis. The disease is most common among those of northern European decent.

Diagnosis

Cystic fibrosis is diagnosed by measuring the amount of salt in sweat and by testing DNA found in blood tests. Providers perform chest X-rays, lung function tests, phlegm tests and evaluate levels of fat-soluble vitamins. As of 2008, all newborns in the United States are screened automatically at birth for the disease.

Complications

People with cystic fibrosis often suffer from respiratory infections and have poor weight gain.

Recovery

People with cystic fibrosis should visit an accredited cystic fibrosis center every three months to get help managing their disease. They should take their medications as directed by their provider. Good nutrition and eating right are critical for those with cystic fibrosis.
Self care for those with cystic fibrosis takes about two to three hours every day.

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