Cystic Fibrosis causes the mucous glands to produce abnormally thick, adherant mucus and the sweat glands produce excessive salt. The two main areas of the body involved are the lungs and the pancreas (part of the digestive system. The mucus is involved for the principal conditions

In cystic fibrosis the lungs are normal at birth but become susceptible to bacterial infection and damage. The thick mucus collects in the lungs blocking some airways and resulting in damage caused by the infection. Much of this damage can be prevented through adequate treatment of infections.

In the pancreas the small channels (through which the enzyme produced in the pancreas flow to reach the intestines) become blocked with mucus. This results in cysts being formed and these lead to fibrosis in the pancreas. The enzymes produced by the pancreas are vital for normal digestion. Digestive enzyme preparations can replace most of the digestive enzymes produced by the pancreas

Insulin is also produced by the pancreas. However, in most cases of cystic fibrosis, the pancreas usually manages to produce enough insulin in childhood but diabetes is increasingly common in adults.

Cystic fibrosis is a life-threatening condition. 75% of affected children now survive to young adulthood and the average survival is around 30 years.

Inheritance patterns: Cystic fibrosis is the commonest inherited life-threatening disorder amongst Caucasians in the UK today. Inheritance is autosomal recessive.

Pre-Natal Diagnosis: Diagnosis can be made by chorionic villus sampling in the tenth to twelfth weeks of pregnancy. A later test (amiocentesis) is available at 16 weeks. Screening for carriers of the CF gene is now possible.

Further information available from:

Support services,
Cystic Fibrosis Trust
11 London road
Bromley
BR1 1BY
(Tel: 020 8464 7211  Fax: 020 8313 0472)

e-mail: enquiries@cftrust.org.uk

Return to the Medical Index

The Cystic Fibrosis Trust http://www.cftrust.org.uk is a National Registered Charity No. 281287. It was founded in 1964 and offers:

• Support and advice to families with CF
• Funds for research
• Educational materials

Activities

• Trained volunteers to support parents of children with cystic fibrosis
• telephone advice to families and the general public
• Financial support to families facing financial difficulties
• Support for people waiting to transplant.
• Campaigning for improved services
• Conferences and seminars

The trusts members include 4000 affected children and 3000 affected adults