Cystic Fibrosis (CF) is a inherited disease, that affects mainly the lungs and the digestive system. In the lungs, CF causes severe breathing problems. A build-up of thick mucus makes it difficult to clear bacteria and leads to cycles of infection and inflammation, which damage the delicate lung tissues.

In the digestive tract, CF makes it extremely difficult to digest and absorb adequate nutrients from food. Thick mucus also blocks the ducts of the pancreas, preventing enzymes from reaching the intestines to digest food. Therefore, persons with CF must consume a large number of artificial enzymes (up to 40 pills a day) with every meal and snack, to help them absorb adequate nutrition from their food. They must also follow a demanding daily routine of physical therapy to keep the lungs free of congestion and infection.

How Many Canadians have Cystic Fibrosis?

It is estimated that one in every 3,600 children born in Canada has CF. At the present time, approximately 3,400 children, adolescents, and adults with cystic fibrosis attend specialized CF clinics.

What Are the Signs and Symptoms of Cystic Fibrosis?

• constant cough which expels thick mucus;
• excessive appetite, with weight loss;
• bowel disturbances;
• skin which tastes salty;
• repeated or prolonged bouts of pneumonia.

CF was first described as a disease in the late 1930s. At that time, it was usually recognized only after a child had died, often as a result of malnutrition or pneumonia. Medical awareness of CF has increased tremendously over the years. Nevertheless, cystic fibrosis can still be confused with other common diseases -- such as asthma, chronic bronchitis or pneumonia, and celiac disease.

What Causes Cystic Fibrosis?

People are born with cystic fibrosis; it is a genetic disorder.

Approximately one in every 25 Canadians is a carrier of the gene responsible for CF. A carrier has only one copy of the gene. A carrier does not have cystic fibrosis, and can never get the disease. In most cases, they are not even aware that they are carriers, because they do not have cystic fibrosis, or any of its symptoms.

• a 25% chance that the child will be born with cystic fibrosis;
• a 50% chance that the child will not have CF, but will be a carrier;
• a 25% chance that the child will not have CF, and will not be a carrier.

With each pregnancy, the risks are exactly the same. Two carrier parents may have several children with CF or none at all.

How is Cystic Fibrosis Diagnosed?

If a physician suspects CF, he or she will probably suggest a "sweat test". This simple and painless test measures the amount of salt in the sweat. A high salt level, along with other symptoms, points to the presence of cystic fibrosis.

Genetic tests are also being used to diagnose the disease.

When is Cystic Fibrosis Diagnosed?

Currently, 60 per cent of children born with CF are diagnosed in the first year, often after symptoms are spotted and lung infections may have occurred. In March 2007 Alberta became the first province in Canada to test newborns for Cystic Fibrosis as evidence shows early detection of the genetic disease can lead to better health and quality of life.

How is Cystic Fibrosis Treated?

Treatment programs are tailored to individual needs and depend upon the stage of the disease and which organs are affected. Treatments followed at home generally include:

• tapping or "clapping" the chest and the back vigorously (percussion) or PEP (positive expiratory pressure) Mask Therapy to help loosen the mucus which clogs the lungs;
• taking pancreatic enzymes with all meals, to aid digestion;
• taking nutritional supplements and vitamins to promote good nutrition;
• taking antibiotics in pill or inhaled form, to ease congestion and protect against and fight lung infection;
• exercise.

How does Cystic Fibrosis affect daily life?

For persons with CF, life includes a daily routine of therapy and periodic visits to a CF clinic. Otherwise, most individuals with cystic fibrosis lead normal lives, for many years, in terms of education, physical activity, and social relationships. Eventually, however, lung disease places increasing limits on daily life.

Thanks to the advances in research and clinical care, growing numbers of children with CF are surviving into adulthood. In 1960, when the CCFF was founded, a child born with cystic fibrosis rarely lived four years. Today, in Canada, half of all persons with CF are living into their mid-thirties and beyond.

Over 47% of all Canadians with CF are over the age of 18 years. These men and women are pursuing post-secondary education, careers and many are having families of their own; a tremendous accomplishment, but not enough.

Is there a Cure for Cystic Fibrosis?

As yet, there is no known cure for CF, but there is real hope.

Comprehensive treatment programs have dramatically extended the life expectancy of persons with CF and many are living into their 20s, 30s and beyond. As of 2002, the median age of survival of a person with cystic fibrosis is over 37 years of age. The median age of survival is the age beyond which half of the CF population can be expected to live. Recent years have seen remarkable progress in CF research. Since 1989 when Canadian researchers discovered the gene responsible for CF, global research to find a cure for the disease has brought us closer and closer to a solution. The astonishing pace of CF science suggests that there is good reason to feel optimistic about the future.