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What is Cystic Fibrosis?
Cystic fibrosis (CF) is the most common, fatal genetic disease affecting young Canadians. CF affects mainly the lungs and the digestive system. In the lungs, CF causes severe breathing problems. A buildup 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 (on average 20 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?
The symptoms of CF may differ for each child. Infants born with CF usually show symptoms within the first year. Some children, though, may not show symptoms until later in life. The following symptoms may indicate CF, and infants having these signs may be tested for CF:
- diarrhea that does not go away
- foul-smelling stools
- greasy stools
- frequent episodes of wheezing
- frequent episodes of pneumonia
- persistent cough
- skin tastes like salt
- poor growth
- chronic sinus infection
The symptoms of cystic fibrosis may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
Medical awareness of CF has increased tremendously over the years. Nevertheless, cystic fibrosis can still be confused with other children's 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?
Approximately 60% of patients are diagnosed in the first year of life, and 90% by 10 years of age.
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 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.
More than 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 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.
For more information about research and clinical care, click here.
For more general information about CF click here.
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ads to cycles of infection and inflammation, which damage the delicate lung tissues. 