Cystic Fibrosis

What is Cystic Fibrosis?

Cystic fibrosis (CF) is the most common fatal genetic disease affecting Canadian children and young adults. There is currently no cure.

CF has various effects on the body, but mainly on the digestive system and lungs. The range and severity of symptoms differ from person to person. However, most patients suffer from a build-up of mucus in the lungs making it very difficult to breathe. This build-up also causes persistent and ongoing lung infections which eventually destroys them and their function, causing death in the majority of people with the disease.

Typical complications caused by cystic fibrosis include: difficulty in digesting fats and proteins, vitamin deficiencies due to loss of pancreatic enzymes, and progressive loss of lung function.

It is estimated that one in every 3,600 children born in Canada has cystic fibrosis. Almost 4,100 Canadian children, adolescents, and adults with cystic fibrosis attend specialized CF clinics.

For more information about disease management, please visit Living with Cystic Fibrosis on the Cystic Fibrosis Canada website.

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How is it Treated?

PEP (Positive Expiratory Pressure) therapy is an airway clearance technique for CF patients. Children like Bella do this physiotherapy twice a day, for around 30 minutes each time. While it's a very boring and repetitive task, it can greatly extend the lifespan of patients with CF.

PEP therapy works by having individuals breathe through a face mask attached to a piece of equipment called a resistor, which builds up pressure in the lungs. This is measured by a manometer (pressure gauge). The pressure keeps the airways open, allowing air to get behind the mucus. The mucus can then be moved to the main airway and be cleared by coughing.

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How do the games help with the prevention of Cystic Fibrosis symptoms?

The Bella Project aims to create games using the air input created during PEP therapy as an input for games. But why make them?

Let’s make therapy fun. PEP therapy is generally done at home, and in the case of children, with parental supervision. It's repetitive and boring, and patients often begin to neglect it – but it's ultimately a very key aspect of managing cystic fibrosis. Why not help make this necessary part of life fun?

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