• Cystic Fibrosis

What is Cystic Fibrosis?

It is a non-infectious genetic disease that affects the secretory glands that produce sweat and mucus in the body. It belongs to a group of rare diseases. It affects the lungs, liver, pancreas, intestines, sinuses, and genitals and is potentially fatal. Cystic fibrosis affects the cells that secrete mucus, sweat, and digestive juices. These secreted fluids are thin and slippery in their normal state but in cystic fibrosis, secretions become thick and sticky, and then begin to accumulate and collect in the ducts, particularly in the pancreas, lungs, and intestines.

Cystic fibrosis is the most common autosomal recessive disease among people of European or Caucasian ancestry. 70,000 people worldwide have cystic fibrosis.

According to local statistics in 2007, there were approximately 600 infected people in Jordan, in addition to the occurrence of new infections of approximately 55 children with the disease each year.
According to the same statistic, Cystic Fibrosis, also known as 65 Roses by some patients, kills 80% of our sick children before they can light their first candle.

Historically, the disease was first discovered in 1938 by American physician Dorothy Andersen, when the average age of an infected child was estimated to be only six months and pneumonia was the only cause of death. Following that, scientists continued to study the disease until they developed a method for examining sweating in 1953, which is a method for measuring the percentage of sodium chloride in sweat, as high levels of salinity confirmed the diagnosis of this disease. Six years later, this examination became the primary method for detecting cases of mild illness. Since that time, clinical research has begun to yield more diagnostic tests and positive therapeutic developments, resulting in a patient’s life expectancy of 30 years or even 60 years if the patient receives the proper care and treatment.

Although science has not yet discovered a cure, the patient can live a normal life if he adheres to the available treatment and leads a healthy lifestyle that includes good nutrition, light exercise, physical therapy, and vaccinations

What happens in the lungs?

The mucus (sputum) that is produced by the lungs of a normal person, is thin, but the mucus produced in the lungs of a CF patient is very thick and sticky. It sticks to the lungs, causing blockage to some of the breathing tubes (airways) If the mucus stays inside the lungs it will clog the tiny airways and make it easier for microorganisms (bacterial infections) to grow there. That is why physiotherapy is so important to clear the airways.

The Cystic patient usually coughs a lot to try to remove the sticky mucus

What happens to the pancreas?

The pancreas is a very important organ for the digestion of food because it produces many substances called enzymes that help to break down the food we eat. Once the food is broken down, the intestines can absorb the digested foods Into the body.

In persons with CF the pancreas is also blocked up by thick juices, As a result, digestive enzymes cannot reach the food that we have eaten and so it is not digested.

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