ConnectABILITY

Cystic Fibrosis

Fact Sheet

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 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 (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 is it manifested?

  • 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

Who is affected?

It is estimated that 1 in every 3,600 children born in Canada has Cystic Fibrosis. CF is a genetic disease that occurs when a child inherits an abnormal gene from each parent. When both parents are carriers, there is a 25 percent chance that their child will be born with Cystic Fibrosis. There is also a 50 percent chance that the child will be a carrier and a 25 percent chance that the child will neither be a carrier nor have cystic fibrosis.

How is it diagnosed or detected?

If a physician suspects CF, he 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.

Approximately 60% of patients are diagnosed in the first year of life, and 90% by 10 years of age.

Additional Resources:

Cystic Fibrosis Canada (Formerly known as the CANADIAN CYSTIC FIBROSIS FOUNDATION) (CCFF)www.cysticfibrosis.ca
Cystic Fibrosis Canada is a Canada-wide health charity with more than 50 volunteer chapters. The Foundation’s primary objective is to fund Cystic Fibrosis research and care. The Canadian Cystic Fibrosis Foundation is one of the world’s largest non-governmental granting agencies in the field of Cystic Fibrosis research. Videos are available from Cystic Fibrosis Canada.

CYSTIC FIBROSIS PARENTShttp://groups.yahoo.com/group/cfparents/
Cystic Fibrosis Parents is a Yahoo discussion group that focuses on issues specific to raising children who have been diagnosed with Cystic Fibrosis. It is a place for parents of children with CF to visit for support and information.

CANADIAN ORGANIZATION FOR RARE DISORDERS (CORD)www.raredisorders.ca
CORD is Canada’s national network for organizations representing all those with rare disorders. CORD provides a strong common voice to advocate for a health policy and a healthcare system that works for those with rare disorders. CORD works with governments, researchers, clinicians and industry to promote research, diagnosis, treatment and services for all rare disorders in Canada.

CORD will link other families and individuals with the same rare disorder(s) through a positive and productive networking system. Together they can exchange ideas on how to plan their daily lives, what they can expect to encounter, and offer emotional support so that they can lead productive lives.

The content contained in this document is for general information purposes. It is not intended to diagnose or treat a child.