Cystic Fibrosis--Frequently Asked Questions

What is Cystic Fibrosis?
How many Canadians have Cystic Fibrosis?
What are the signs and symptoms of Cystic Fibrosis?
What causes Cystic Fibrosis?
How is CF diagnosed?
When is Cystic Fibrosis diagnosed?
How is Cystic Fibrosis treated?
How does Cystic Fibrosis affect daily life?
Is there a cure for Cystic Fibrosis?
Statistics

What is Cystic Fibrosis

Cystic Fibrosis (CF) is the most common, fatal genetic disease affecting young Canadians. CF is a multi-organ disease affecting primarily 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. They must follow a demanding daily routine of physical therapy to keep the lungs free of congestion and infection.

In the digestive tract, CF makes it extremely difficult to digest and absorb adequate nutrients from food. Thick mucus 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 (on average 20 pills a day) with every meal and snack, to help them absorb adequate nutrition from their food.

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 3,500 children, adolescents, and adults with Cystic Fibrosis attend specialized CF clinics.

What Are the Signs and Symptoms of Cystic Fibrosis?

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

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 carries a defective version of the gene responsible for CF. A carrier has only one copy of the gene responsible for CF. Carriers do 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.

When two people who carry a defective version of the gene responsible for CF have a child, there is:

• 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 CF 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.

Increasingly, genetic tests are being used in the diagnosis of the disease. Genetic tests are also used to diagnose CF prenatally.

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 CF 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 or other forms of chest physiotherapy 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, intravenous (IV), and or inhaled forms, 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 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, half of all Canadians with CF are expected to live into their late-thirties and beyond.

In 2006, for the first time, more than 50% of all Canadians with CF were 18 years and older. 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 lives of persons with CF and many are living into their 20s, 30s and beyond.

As of 2002, the median age of survival of Canadians with Cystic Fibrosis is 37 years of age. The median age of survival is the age beyond which half of the CF population can be expected to live.

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 pace of CF science suggests that there is good reason to feel optimistic about the future.

Statistics

The Canadian Cystic Fibrosis Patient Data Registry (CPDR) collects annual data on patients with Cystic Fibrosis attending 38 specialized clinics throughout Canada. The CPDR monitors important epidemiological trends in the population and has helped direct therapeutic advances responsible for improved survival.

Originally established in the mid-1960s, the CPDR continues to collect vitally important health information from CF clinics. The clinics submit clinical data to a database protected with passwords and encryption codes. The CCFF maintains this information in a comprehensive, secure database protected by a high-end firewall, and intrusion detection systems, in addition to network and application level security features.