What is cystic fibrosis?

Cystic fibrosis is an inherited disease that has its main effects on the digestive system and the lungs. It is usually diagnosed soon after birth and symptoms occur throughout life. Nowadays thanks to improvements in dietary supplements and better treatment, most people with cystic fibrosis can lead a fairly normal life, However to achieve this they need daily treatment, consisting of chest physiotherapy, various medicines and pancreatic enzyme capsules taken with their food. In addition they have to pay special attention to their increased dietary needs.

The name cystic fibrosis CF describes the changes that occur at an early age in the pancreas of CF patients. The pancreas a major organ in the body, manufacturing digestive enzymes and other important compounds. The part of the pancreas that produces digestive enzymes proteins that fluid filled spaces cysts.

Another common feature of CF is unusually sticky or thick or thick mucus secretions in the lungs and digestive system. In the jungs the presence of this thick or viscid mucus makes chest infections more severe. In the digestive system it may damage the pancreas, both directly and indirectly by blocking the ducts that would usually form an open channel for digestive enzymes to reach the gut. This viscid mucus is the origin of another common name for CF. muco-viscidosis.

Until the beginning of this century doctors did not recognize CF as a disease in its own right. The various symptoms of CF were merely seen as separate, unrelated infections. Part of the reason for this was that before the advent of antibiotics, chest infections which are a major feature of CF were common in many diseases. The first recognition of CF came through another feature of the disease, namely steatorrhoes. Steatorrhoca means literally fatty stools, and is a condition characterized by the passage of pale bulky smelly faces. Thus in 1912 the London physician Archibald Garrod described the occurrence of steatorrhoes in several members of the same family. The description, with hindsight, was almost certainly of CF.