What is Cystic Fibrosis?
- Cystic Fibrosis (CF) is one of the UK's most common life-threatening inherited diseases.
- Cystic Fibrosis affects over 9,000 people in the UK.
- Over two million people in the UK carry the faulty gene that causes Cystic Fibrosis - around 1 in 25 of the population.
- If two carriers have a child, the baby has a 1 in 4 chance of having Cystic Fibrosis.
- Cystic Fibrosis affects the internal organs, especially the lungs and digestive system, by clogging them with thick sticky mucus. This makes it hard to breathe and digest food.
- Each week, five babies are born with Cystic Fibrosis.
- Each week, two young lives are lost to Cystic Fibrosis.
- Only half of those living with Cystic Fibrosis are likely to live past their late 30s
Cystic Fibrosis is caused by a single faulty gene that controls the movement of salt in the body. In people with Cystic Fibrosis, the internal organs become clogged with thick, sticky mucus resulting in infections and inflammation making it hard to breathe and digest food.
For a baby to be born with Cystic Fibrosis, both parents must be carriers of the faulty CF gene. The diagram shows how CF is inherited. Where both parents carry the faulty gene, each child has a one in four chance of having CF, a two in four chance of being a carrier and a one in four chance of not having any CF genes.
Symptoms of Cystic Fibrosis
Cystic Fibrosis causes the body to produce thick secretions that particularly affect the lungs and digestive tract.
Symptoms of CF can include a troublesome cough, repeated chest infections, prolonged diarrhoea and poor weight gain. These symptoms are not unique to Cystic Fibrosis.
Cystic Fibrosis affects a number of organs.
Lungs
It is common for people with CF to encounter some difficulties with their lungs. A combination of physiotherapy and medication can help control lung infections and prevent lung damage. To avoid the risk of cross-infection, it is recommended that people with CF do not come into close contact with others with Cystic Fibrosis.
Digestive system
Cystic Fibrosis affects the pancreas, which makes it difficult for people with CF to digest food. This can cause malnutrition, which can lead to poor growth, physical weakness and delayed puberty. There is medication that can compensate for the failure of the pancreas.
In older patients, insulin production can become deficient due to increasing pancreatic disease. Some develop CF related diabetes mellitus and their blood sugar levels are no longer controlled. This rarely happens to children with Cystic Fibrosis.
Common symptoms of diabetes include thirst, hunger, weight loss and excessive need to urinate, but some people do not show obvious symptoms of diabetes.
Other Affected Organs
In every ten babies born with CF, one is ill in the first few days of life with a bowel obstruction called meconium ileus. In these cases, the meconium (a thick black material present in the bowels of all newborn babies) is so thick that it blocks the bowel instead of passing through. Babies with meconium ileus often need an urgent operation to relieve and bypass the blockage.
People with CF are prone to developing bone disease (thin, brittle bones) due to the nutritional and other problems involved with the disease. Adults with CF are at an increased risk of bone disease because of the adverse effects of steroids taken to control lung disease.
Although Cystic Fibrosis does not cause sexual impotency, it can lead to fertility problems. In most men with CF, the tubes that carry sperm are blocked, which causes infertility. Because underweight women are more likely to have irregular menstrual cycles, the nutritional problems associated with CF may affect fertility. Women with CF do produce healthy, fertile eggs so effective contraception is necessary.
Cystic Fibrosis can cause the blockage of small ducts in the liver. This only happens to approximately 8% of people who have CF, but it is a serious health risk and may necessitate a liver transplant
