About Cystic Fibrosis

 

WHAT IS CYSTIC FIBROSIS?

Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive system.

A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that: 

  • clogs the lungs and leads to life-threatening lung infections; and

  • obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. 

WHAT CAUSES CYSTIC FIBROSIS?

Cystic fibrosis is an inherited disease. 

People with cystic fibrosis have two copies of an altered gene (CFTR gene), one inherited from each parent. 

WHAT ARE THE SYMPTOMS?

The symptoms and severity of cystic fibrosis vary from person to person. 

The faulty CFTR gene causes the body to produce a thick, sticky mucus that damages organs, particularly the lungs and digestive system. A build up of mucus makes it easier for bacteria to grow and cause infections. 

Early symptoms of CF include failure to gain weight, despite a good appetite, chest infections and breathing difficulties.

TREATMENT AND MANAGEMENT

Early diagnosis and careful management of the condition is transforming the outlook and more and more children with CF are now emerging into adult life with relatively little lung damage. Treatment and management of CF includes:

  • Physiotherapy and Exercise to clear thick mucus from the lungs
  • Nutritional management - enzyme supplements must be taken with each meal and vitamin intake should be monitored
  • Medication involving long-term antibiotic treatment
  • Inhalation therapy

IS THERE A CURE?

There is no known cure for cystic fibrosis. However, medical and scientific research has greatly improved the treatment of the disease in recent years. Research is ongoing in the endeavour to find a cure.

Life expectency and quality of life has increased steadily over the past 20 years thanks to research and improved management of the disease.

CYSTIC FIBROSIS IN IRELAND

Ireland has the highest incidence of cystic fibrosis in the world, with approximately 1,300 children and adults living with the condition.

Approximately 1 in 19 Irish people are said to carry one copy of the altered gene that causes cystic fibrosis. These people do not have cystic fibrosis. Both copies of the gene must be altered in order to have cystic fibrosis.

The incidence of cystic fibrosis in Ireland is almost 3 times the average rate in other EU countries and the United States.