Cystic Fibrosis Overview

Cystic fibrosis (CF) is a potentially life-threatening disease that causes thick, sticky mucus to build up in the lungs and digestive tract, as well as the sinuses and sex organs. This build-up causes life-threatening lung infections and serious digestion problems. Read more in this patient education guide.

CF also causes loss of large amounts of salt in the sweat, which upsets the balance of minerals in the blood. This imbalance can lead to dehydration, increased heart rate, fatigue, weakness, low blood pressure, heat stroke, and death (rarely).

CF is an inherited disease. In the United States, CF is present in approximately 1 in 3500 births. It is most common among Caucasians of Northern European descent and least common among African Americans and Asians. Because CF is present at birth, it is typically diagnosed in children before age 2 years, but milder forms of the disease may not be diagnosed until early adulthood. CF has many different symptoms depending on age and the organs affected.

Symptoms in newborns:

  • Delayed growth
  • Not gaining weight at a normal rate during childhood
  • No bowel movements within 24-48 hours after birth
  • Skin that tastes salty

Symptoms in adulthood:

  • Respiratory symptoms
  • Men: infertility
  • Women: difficulty getting pregnant
  • Repeated episodes of pancreatitis (inflammation of the pancreas)

Symptoms related to bowel function:

  • Severe constipation
  • Stomach pain
  • Gas, bloating, or a belly that appears swollen
  • Nausea
  • Poor appetite
  • Stools that are pale or clay colored, foul smelling, have mucus, or that float
  • Weight loss
  • Lack of appropriate weight gain and growth in children

Respiratory symptoms (i.e. lungs and sinuses):

  • Coughing or increased mucus in the sinuses or lungs
  • Fatigue
  • Nasal congestion caused by nasal polyps
  • Recurrent episodes of pneumonia
  • Sinus pain or pressure caused by infection or polyps

 

Written by Laurie LaRusso, MS, ELS

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