Cystic Fibrosis

• How is cystic fibrosis diagnosed?

• How is cystic fibrosis treated?

• Where can I learn more about cystic fibrosis?

• What are the signs and symptoms of cystic fibrosis?

• What about the patients affected with cystic fibrosis?

How is cystic fibrosis diagnosed?

Newborn screening for CF is now conducted in Alberta since April 2007, and Ontario is slated to begin a newborn screening program for CF in early 2008, other provinces are also encouraged to implement this program. The test requires that a doctor or a nurse takes a small blood sample from the heel of the baby’s foot, which will be evaluated in a laboratory.
In provinces without newborn screening for CF, most people with the disease are not diagnosed until they show symptoms. By that time, early damage to the lungs and digestive system may be difficult (if not impossible) to reverse. If a physician suspects cystic fibrosis (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.

 

How is cystic fibrosis 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)and/or 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 ensure adequate nutritional intake
• taking antibiotics in pill, intravenous (IV), and/or inhaled forms, to treat infections, preserve lung functions, and slow the progression of the disease
• exercise

 

Where can I learn more about cystic fibrosis?

Visit www.cysticfibrosis.ca

Visit www.cysticfibrosis.com

 

What are the signs and symptoms of cystic fibrosis?

May have a range of symptoms, including:

• difficulty breathing
• constant cough which expels thick mucus
• salty-tasting skin
• persistent coughing
• wheezing
• repeated or prolonged bouts of pneumonia
• poor weight gain (despite a normal or large appetite)
• failure to thrive

CF was first described as a disease in the late 1930s, where at the time children were diagnosed with malnutrition and pneumonia. Even though, the medical community is aware about CF, it is commonly confused with other common diseases such as asthma, chronic bronchitis or pneumonia, and celiac disease.

 

What about the patients affected with cystic fibrosis?

For persons with CF, life includes a time consuming daily routine of therapy and periodic visits to a CF clinic. Most individuals with cystic fibrosis lead normal lives, for many years, in terms of education, physical activity, and social relationships. Eventually, however, lung disease progression increasingly interferes with daily life.

• Affected by cystic fibrosis to various degrees of severity
• Undergo rigorous physiotherapy programs every day to relieve congestion in their lungs
• Consume enzymes every time they eat to help them absorb adequate nutrients from food
• Are living longer than ever before
• May experience various CF-related diseases as they age, such as:
• diabetes
• osteoporosis
• arthritis
• congenital bilateral absence of the vas deferens (CBAVD)
• chronic pancreatitis
• liver disease
• nasal polyps
• allergic aspergillosis

 

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