Cystic fibrosis (CF) is an incurable, hereditary condition.
In several parts of the human body, glands with cells that produce mucus can be found. The location of the glands and mucus largely determines the function. In the lungs, it is necessary to remove inhaled particles or bacteria (by coughing up mucus). In the digestive tract, mucus is needed for transporting digestive enzymes. The mucus in your mouth serves as a lubricant and it facilitates talking and swallowing.
In people with CF, the mucus is thick and sticky because of an alteration in the DNA. Depending on the alteration a chloride channel in the cell membrane is either present but with inadequate or no function, or it can be completely absent. Chloride plays an important role in the transport of water across cells. When this transport is disturbed, the mucus from the glands does not contain enough water, making it thick and sticky.
Frequently, the first symptoms occur in the respiratory and gastrointestinal tract. People with CF often get bronchitis or pneumonia. The duct from the pancreas to the intestine can become clogged causing a disturbance of digestion. As a result, fat absorption in the digestive tract is reduced. This can also cause problems with the absorption of important vitamins that only dissolve in fat. This in turn leads to an impaired physical condition. People with CF are encouraged to eat calorie rich foods to maintain their body weight.
In the Netherlands CF was included in the neonatal screening program in 2011. CF may be noticed even before the results of the screening are known. One of the earliest symptoms of the disease is already seen in a newborn baby, namely an obstruction of the bowel. This is called meconium ileus.