Today was the CFF (Cystic Fibrosis Foundation (Barbados) 5K walk/run. Yellow T-shirts walked, blues ran. BH & BD wore yellow. I’ve copied and pasted the below information on cystic fibrosis from the website: http://patient.info/health/cystic-fibrosis-leaflet but there are many sites that can provide information.
What is cystic fibrosis?
Cystic fibrosis is a condition which mainly affects the lungs and pancreas but can affect other parts of the body, including the liver, nose and sinuses and sweat glands. Normally, cells in these parts of the body make mucus and other watery juices and secretions.
In people with cystic fibrosis, these cells do not function correctly and make mucus and secretions which are thicker than normal. This can cause various symptoms and problems (which are described below).
What causes cystic fibrosis and how common is it?
Cystic fibrosis is a genetic disorder. A genetic disorder is one that can be passed on from your parents through your genes. If you have cystic fibrosis, one of your genes does not work properly. This is known as a CFTR gene which is on chromosome 7. There are different errors that can occur in this gene and this means that there are different severities of cystic fibrosis that occur.
The CFTR gene helps to control the way the cells handle salt (sodium and chloride ions). The error in the gene results in the cells being unable to handle sodium and chloride properly.
As a result, cells in affected organs have a fault in the way sodium and chloride travel in and out of the cells. Basically, too much sodium travels into the cells. Water follows the sodium, which leaves too little water outside the cells. This causes the mucus or watery secretions outside the cells to be too thick (for example, in the airways of the lungs).
The cells that line the airways of the lungs make sputum (mucus) that is thicker than normal and which is not cleared from the lungs easily. This can trap bacteria in the small airways and lead to infection and inflammation. So, symptoms which typically develop include:
- Persistent cough which typically produces a lot of sputum.
- Shortness of breath and breathing difficulties.
- Recurring chest infections. These can be severe, such as pneumonia. Repeated infections and inflammation can damage the lungs and lead to poor lung function.
The pancreas normally makes digestive juices which contain chemicals (enzymes). The digestive juices normally flow out from the pancreatic duct into the duodenum and digest food.
In people with cystic fibrosis, thickened secretions block the normal flow of the digestive juices from the pancreas. This can result in food not being digested or absorbed properly – in particular, fatty foods and fat-soluble vitamins (vitamins A, D, E and K).
This can cause:
- Malnutrition leading to poor growth and poor weight gain (even if you have a good appetite and eat a lot, as the problem is with digesting and absorbing the food).
- Large, smelly, greasy, fatty stools (faeces) occur in about a third of cases.
- Bloated tummy (abdomen).
In about 3 in 10 cases the pancreas functions well and there are no, or minimal, gut symptoms and mainly just lung symptoms.
Symptoms sometimes occur at birth
About 1 in 10 children with cystic fibrosis are diagnosed shortly after birth. This is due to a condition called meconium ileus where in some cases the gut becomes blocked with meconium. Meconium is a thick, dark, sticky substance which is made by the baby’s gut before being born. Urgent surgery may be needed to relieve the blockage.
Other symptoms and complications
Other organs may be affected which may cause various other problems in some cases. Also, the pancreas and airways may become severely affected. Therefore, other problems which may also occur in some cases include:
- Repeated sinus infections.
- Growths (polyps) forming in the nose.
- Infertility (especially in males, as the tubes which carry the sperm can become blocked).
- Damage to the liver, which may lead to ‘scarring’ of the liver (cirrhosis). Liver damage occurs in about 1 in 12 cases (if the small ducts in the liver become blocked or damaged).
- Diabetes. (Special cells in the pancreas make insulin. If the pancreas becomes severely damaged over time then insulin levels go down and diabetes may develop.) This is rare in children but is more common in adults who have had cystic fibrosis.
- Inflammation of the pancreas (pancreatitis).
- Rectal prolapse.
- ‘Thinning’ of the bones (osteoporosis) may develop due to poor absorption of certain foods and, in particular, vitamin D which is needed to maintain healthy bones.
- The sweat tastes very salty.