What You Need to Know About Cystic Fibrosis

Cystic fibrosis (CF) is a chronic disease of the mucus glands which normally affects the respiratory and digestive systems.  CF is a genetically inherited disease and is not contagious.  Each year about 1,000 people are diagnosed with cystic fibrosis.  The disease occurs more commonly in Caucasians than any other race.  About 1 in 3000 Caucasian babies are born with cystic fibrosis while only 1 in 17,000 African American babies and 1 in 31,000 Asian American babies are born with the disease.  More than 70% of all patients are diagnosed by age 2.

A healthy body produces a slippery mucus that acts as a lubricant to protect the linings of airways, organs and tissues.  However, people with CF produce unusually thick and sticky mucus.  Since the mucus is so thick and sticky it can obstruct the very airways it is supposed to protect.  This can cause breathing problems and bacterial infections in the lungs.  The infections result in coughing, wheezing, and inflammation.  Chronic accumulation of mucus and repeated bacterial infections in the lungs can result in scar tissue, cysts and permanent lung damage.

The thick and sticky mucus which damage the lungs, commonly impedes the function of the pancreas.  One job of the pancreas is to produce enzymes which aid in food digestion.  In cystic fibrosis patients the mucus clogs the ducts of the pancreas, preventing essential enzymes from reaching the intestine.  Without those enzymes the digestive system cannot break down food so it can be absorbed.  This can cause diarrhea and malnutrition which leads to poor growth and weight loss.

People with CF suffer from various symptoms such as:

  • Shortness of breath
  • Wheezing
  • Chronic cough (with or without mucus)
  • Frequent respiratory and lung infections
  • Weight loss despite normal eating habits
  • Skin with a salty taste
  • Difficulty with bowel movements

In the 1950s a childhood diagnosis of cystic fibrosis used to be a death sentence.  In 1955 the Cystic Fibrosis Foundation was established and they were dedicated to finding a cure for the disease.  Great strides in research and medical advancements have led to treatment protocols which allow people with the disease to live much longer lives.  More than 45% of CF patients are over the age of 17 and the average patient now lives to be in their mid thirties.  There are people with the disease who live to be 40 and even older.  While no cure has been discovered yet, continuing advancements provide for a better quality of life for people with the disease.

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