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What is Pancreatic Cystic Fibrosis?

By Stephany Seipel
Updated May 17, 2024
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Pancreatic cystic fibrosis, also called fibrocystic disease of the pancreas or cystic fibrosis of the pancreas, is a type of cystic fibrosis. The symptoms range from mild to severe, depending on the patient. Although once considered a fatal childhood disease, specialists manage the condition through physical therapy and medication.

A genetic mutation causes pancreatic cystic fibrosis. Both parents must have the defective gene to pass the condition down to their children. Children can inherit the disorder even if the parents do not have the disease themselves.

Healthy people have thin, slippery mucus that lines many organs in the body and keeps them from drying out. Cystic fibrosis patients have thick, gluey mucus. The substance clogs up passages and ducts and interferes with their ability to function normally. Cystic fibrosis can affect other parts of the body besides the pancreas, such as the sinuses, lungs and liver.

The pancreas has the twofold job of producing specialized enzymes that break food down into nutrients and releasing insulin into the bloodstream. Pancreatic cystic fibrosis blocks the pancreatic ducts, preventing the enzymes from reaching the small intestine. People who have this condition become malnourished and suffer from vitamin deficiencies. Many patients also have constipation, gas and stomach pain.

People who suffer from pancreatic cystic fibrosis are often underweight. As a result, their bodies cannot produce the energy that they need to battle the antibiotic-resistant lung infections that are common in patients who have cystic fibrosis. Some people benefit from tube feedings as a supplement to their regular diet. Many patients eventually develop insulin-dependent diabetes.

Doctors diagnose cystic fibrosis by performing genetic tests that screen babies at birth. They conduct a blood test to see whether the baby's pancreas works as it should. Doctors confirm the diagnosis by using a sweat test, which is a test that measures the amount of salt in the baby's sweat.

There had been no cure discovered for pancreatic cystic fibrosis by early 2011. In the past, most people with this disease died in childhood. By 2011, advanced treatment methods had improved the life expectancy, so with proper management, many patients were able to survive well into their adult years.

Patients who have cystic fibrosis occasionally suffer from complications from the disease as it progresses. Some individuals develop pancreatitis, which is a painful inflammation of the pancreas. Many people are susceptible to respiratory infections such as pneumonia, sinusitis or bronchitis. Other conditions include lung collapse, infertility, osteoporosis and nasal polyps.

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