Cystic Fibrosis

cystic fibrosis

Medical care is provided by a specialized team of health care workers. They work with CF patients and their significant others/ family to assist them in managing their disease and achieve a healthy balance between their CF and non-CF daily living. Continuity of care is provided by the CF team for both outpatient, scheduled clinic appointments and inpatient care when required.

What is CF?

Cystic Fibrosis is the most common, fatal, autosomal recessive genetic disorder in Canada. It is characterized by the presence of thick, sticky secretions mainly in the lungs and digestive tract.

Who gets CF?

Approximately 1 in 25 Canadians is a carrier of the defective gene with approximately 1 in 3600 live births diagnosed with cystic fibrosis. Carriers are not affected themselves by CF. However, if their partner is also a carrier, there is a 1 in 4 chance with each pregnancy that their child will be born with cystic fibrosis. Thirty years ago, children born with CF would not be expected to survive to start school. Today, with the advances in research and medical care, the median age of survival in Canada is now more than 40 years of age.

What happens?

The thick secretions in the lungs provides an ideal medium for the growth of bacteria which leads to frequent infections for persons living with CF. Inhaled medications and chest physiotherapy routines are part of a daily regime to loosen the sticky secretions and slow down the disease process in the lungs. Many persons with CF require frequent admissions to hospital for two or three week of intravenous administration of antibiotics to try and control the infections. In spite of treatments, the lungs eventually become chronically infected with bacteria, irreversibly damaging the airways, and ultimately resulting in respiratory failure and death.

In the digestive system the thick secretions prevent normal production and transport of pancreatic enzymes to the gastrointestinal tract. These enzymes are necessary for the break-down and absorption of the nutrients found in food. Persons with CF must consume multiple, artificial digestive enzyme pills, vitamins and minerals each time they eat to aid with the digestion of food

Is there a cure?

There is no cure for CF. Eventually, the effects of the damaged lungs increasingly limits their opportunities to live a normal life. For those at the end stage of their disease, the only hope of survival is to receive a double lung transplant. Unfortunately, the demand for donor lungs far exceeds the availability. Many people on the waitlist for new lungs will die before organs are available.


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