About cystic fibrosis

FACTS AND FIGURES


  • Cystic fibrosis is a life-shortening inherited disease, affecting over 10,000 people in the UK. 
  • It is non contagious, so you cannot catch or develop it.
  • Cystic fibrosis is caused by inheriting 2 faulty genes (one from each parent) 
  • These genes controls the movement of salt and water in and out of your cells, so the lungs and digestive system become clogged with mucus, making it hard to breathe and digest food.
  • Most babies are diagnosed with the Guthrie test results taken at birth.
  • Each week five babies are born with cystic fibrosis, and two people die.
  • More than 2.5 million people in the UK carry the faulty gene, around one in 25 of the population.
  • A simple mouthwash test can determine if you are a carrier of the defective gene that causes cystic fibrosis. This is important if your partner is a known carrier, or if a relative has cystic fibrosis or is a carrier.
  • Here is a diagram below showing how if both you and your partner have the CF gene there is a 1 in 4 chance that each baby you have with have Cystic Fibrosis.



LUNGS
  • It is common for people with cystic fibrosis to experience some problems with lung function.
  • A combination of physiotherapy and medication can help control lung infections and prevent the build up of mucus that damages the lung.
  • To avoid the risk of cross-infection people with cystic fibrosis should not meet or come into close contact with one another.
  • For people with cystic fibrosis, cross-infection can be very harmful and poses a particular threat.
  • People with cystic fibrosis are vulnerable to different bacteria or 'bugs', which grow in their lungs. While these bugs are usually harmless to people who don't have cystic fibrosis, they can settle in the lungs (colonise) and be harmful for those who do. These bugs can be easily transmitted from one person with cystic fibrosis to another.
DIGESTIVE SYSTEM
  • Cystic fibrosis affects the pancreas because a build up of thick, sticky mucus blocks the ducts, reducing the amount of insulin produced and stopping digestive enzymes from reaching the intestines to aid digestion. 
  • Medication can help compensate for the failure of the pancreas, such as digestive enzymes that most people with cystic fibrosis take with each meal to help digest food.

OTHER BODY PARTS
  • Adults with cystic fibrosis also have an increased risk of bone disease, which is a side effect of the steroids taken to control lung disease.
  • Cystic fibrosis can cause the blockage of small ducts in the liver, leading to liver disease.
  • In older people with cystic fibrosis insulin production can become deficient due to increasing pancreatic disease. Some people develop cystic fibrosis related diabetes mellitus and their blood sugar levels are no longer controlled.

THERE IS NO CURE FOR CYSTIC FIBROSIS. IT IS EXPECTED THAT JUST OVER HALF THE CF POPULATION WILL REACH 41. 

TRANSPLANT
  • Usually someone would be referred for transplant assessment when their lung function falls to about 30%.
  • The transplant process is a lengthy one, and patients and their families can often face a range of physical and emotional issues. 
  • There is a shortage of suitable donor organs, and approximately a third of all those on the waiting list for a lung transplant will die before they receive a donor lung.

All information from http://www.cysticfibrosis.org.uk




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