What you should know about cystic fibrosis

Cystic fibrosis is a genetic disorder which can affect many different parts of the body. Its name is derived from some of its key symptoms. It causing cysts and scarring, or fibrosis, in the pancreas. Recognised as far back as the 1930s, its most critical factor is its chronic affect on the lungs.

The symptoms of cystic fibrosis

Firstly, the good news. Around 20 to 30 years ago, individuals suffering from cystic fibrosis were not expected to survive into adulthood. With medical advances, many patients are not only living to become adults, they may even have a normal life expectancy. This is down to the improvements in the available medications, patient care and an increased knowledge of the disease.

However, it is important to focus on the serious nature of cystic fibrosis. Chronic lung disease and breathing difficulties are potentially life threatening. The way that cystic fibrosis effects the lungs is that a defective gene causes the body to create unusual mucus. This is sticky and thick, clogging the lungs. The common bacteria that are instigated in the process include Staphylococcus. The excess mucus in the lugs can lead to infections developing. It also obstructs the pancreas, preventing natural enzymes doing their crucial job of breaking down food.

When the lungs become clogged, bacteria normally cleared by airways can build up. Up to 20% of children with the disease develop nasal polyps. These are tiny bumps of tissue from the nose lining that must be surgically removed. As well as problems with lungs, affected children also have a high incidence of sinus infections.

Cystic fibrosis and diabetes

Another way that cystic fibrosis strikes is that it can lead to diabetes. This happens when the issues with blockage affect the pancreas to the extent that cells are destroyed. This prompts glucose intolerance, as well as insulin-dependent diabetes. This diabetic condition will develop in around 35% of sufferers in their 20s. By the time they reach their 30s this rises to 40%.

Cystic fibrosis affecting the digestive system

Finally, the defective gene also controls the flow of salts and water throughout body cells. While the pancreas usually secrets substances to help digestion, with cystic fibrosis these become too thick, blocking the passages between organs. When this happens there are fewer enzymes, so a child will have problems absorbing crucial nutrients.

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