I saw a piece on the television recently about a young woman who had Cystic Fibrosis and had been having treatment for all her life. She had decided to become a doctor and was just about to go to university. It made me think that I did not know too much about this condition so I looked it up and this is what I found:

Cystic fibrosis (CF) affects more than 8,000 people in the UK, making it the most common inherited disorder. It's a multi-system disease, which means it affects many organs in the body. However, most of the symptoms affect the lungs and digestive system. If you have healthy lungs, there is a constant flow of mucus (a slimy liquid) over the surfaces of the air passages in the lungs. This removes debris and bacteria. If you have CF, this mucus is excessively thick and sticky, due to an altered balance of salt and water in the lungs.

CF also affects the digestive system. In a healthy person, the pancreas produces enzymes (chemicals) that pass into the gut. These enzymes break down the fat, carbohydrate and protein in food. If you have CF, your pancreas still produces these enzymes, but the duct which takes them to the gut is blocked by thick and sticky mucus.

I saw that programme! That young woman was a great example of someone overcoming adversity.

Life can be very touch if you have CF, as the thick mucus is difficult to cough up and provides an ideal environment for bacterial growth, leading to chest infections. Over time, repeated infections can damage the lungs and a sufferer from CF may become more breathless and prone to further chest infections. Treatment with antibiotics can help treat chest infections and prevent damage to the lungs.

In people who have CF, enzymes produced by the pancreas can't reach the gut because the flow is blocked. Without these enzymes not enough nutrients are absorbed, particularly fat, and a CF sufferer may find it difficult to gain weight. Faeces may contain excess fat making it oily, smelly, and difficult to flush away. But having said all that anyone I have known with the condition has always seemed remarkably cheerful and were just getting on with it!

It is perhaps not so well know that there are other condition that are associated with CF and these can include:
CF-related diabetes, caused by chronic damage to your pancreas
nasal polyps (small growths in your nose)
CF-related liver disease
osteoporosis (thinning of your bones)
constipation leading to bowel obstruction, known as distal intestinal obstruction syndrome (DIOS)
male infertility, because the tube that carries sperm, the vas deferens, becomes blocked
fertility problems in women, because your cervix becomes blocked with thick mucus
These symptoms aren't always due to CF but if you have them, you should always visit your doctor for advice.

I am a great fan of on-line health sites but there is a danger in self diagnosis and it is always better to see a doctor with any symptoms you have. You can then go to the Internet once you know what you are dealing with!

I always used to wonder what caused a child to have this condition, I knew it was genetic but not really how that worked. I found this useful explanation on the CF site:
[url]http://www.cftrust.org.uk/aboutcf/whatiscf/whatcausescf/[/url]


CF is an inherited condition that is caused by a faulty gene called the CF transmembrane conductance regulator (CFTR gene).
In order to develop CF you need to inherit two CF genes, one from your mother and one from your father. If you inherit only one CF gene, you're called a carrier and won't have symptoms.


Illustration showing how cystic fibrosis is inherited
When both parents are carriers, with each pregnancy there is a:
one in four chance of having a child with CF
two in four chance of having a child who is a carrier
one in four chance of having an unaffected child
There are more than 1,000 genetic mutations associated with different degrees of severity of the disease.

Sadly, there is currently no cure for CF. There is a lot of ongoing research to try to find a cure for it through gene therapy or drug therapy to target the CFTR gene. Many of these treatments are undergoing clinical trials.

However in the mean time current treatment are designed to treat chest infections and prevent further damage to the lung and to improve nutrition by providing supplements containing enzymes to help digestion.
Before each meal or snack, CF sufferers need to take replacement enzymes such as pancreatin (eg Pancrex or Creon). These supply the missing pancreatic enzymes and allow proper digestion of food. Vitamin and mineral supplements are also necessary.

There are a range of possible treatments, according to the condition of the CF sufferer which may include, regular antibiotics (tablets and/or nebulised antibiotics) to counter lung infections. Mucolytics such as dornase alfa (Pulmozyme) to make the sputum (mucus from the lungs) less sticky. Asthma therapy, such as bronchodilators, to help treat blocked airways and breathing problems that may have been caused by infection. Then there is insulin therapy for diabetes, to help the body break down sugar properly so that a healthy weight can be maintained.