Worldwide Cystic Fibrosis (CF) Day takes on 8th September, an event which aims to internationally spread information and education about cystic fibrosis and improve the lives of people born with this inherited disorder.

Cystic fibrosis causes severe damage to the lungs and digestive system and affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery, acting as a lubricant. But in people with cystic fibrosis, a defective gene causes the secretions to become thick and sticky. These fluids clog the internal organs, especially the lungs and digestive tract, which makes it very difficult to breathe and digest food.

Cystic fibrosis is caused by a mutation in a gene that changes a protein that regulates the movement of salt in and out of cells. The result is thick, sticky mucus in the respiratory, digestive and reproductive systems, as well as increased salt in sweat.

It is an inherited disorder. Children need to inherit one copy of the gene from each parent in order to have the disease. If children inherit only one copy, they won’t develop cystic fibrosis, but will be carriers and possibly pass the gene to their own children.

Cystic fibrosis is the most common life-shortening recessively inherited disorder of Caucasian people affecting as many as 1 in 2,000 newborn infants in some countries. It is estimated that there are between 70,000 and 100,000 people with cystic fibrosis worldwide, although a majority of countries with cystic fibrosis patients have no registries or collected data from health facilities.

Nowadays this condition can be diagnosed in newborns within the first month of life, before symptoms develop. If cystic fibrosis is diagnosed at birth and preventive treatment begun early, patients can live longer and have a higher quality of life. Average life expectancy is 35 to 40 years old in Europe and USA. In countries such as El Salvador, India and Bulgaria, life expectancy drops to below 15 years old.

For people born before newborn screening was performed, it’s important to be aware of the signs and symptoms of cystic fibrosis:

  • High level of salt in their sweat
  • Persistent cough that produces thick mucus
  • Wheezing
  • Breathlessness
  • Exercise intolerance
  • Repeated lung infections
  • Inflamed nasal passages or a stuffy nose
  • Foul-smelling, greasy stools
  • Poor weight gain and growth
  • Intestinal blockage, particularly in newborns (meconium ileus)
  • Severe constipation
  • Pancreatitis
  • Diabetes
  • Infertility

cystic fribosis day

Although cystic fibrosis requires daily care, people with this condition are able to attend school and work, and have a better quality of life than in previous decades, due to improvements in screening and medical treatments.

Worldwide Cystic Fibrosis Day brings the international CF community together to help fight for better standards of care, equal access to care and necessary medication and equipment and support research for a cure.