What is Cystic Fibrosis?
Cystic Fibrosis (CF) is the most common fatal genetic disease affecting Canadian children and young adults. At present, there is no cure.
CF causes various effects on the body, but mainly affects the digestive system and lungs. The degree of CF severity differs person to person. As recently as 1960, most children with CF did not live past kindergarten age, however, due to improved treatments and care, the median age of survival of people with CF in 2015 was 52 ears old. Over 60 per cent of Canadians with CF are adults.
It is estimated that one in every 3,600 children born in Canada has CF. More than 4,100 Canadian children, adolescents and adults with cystic fibrosis attend specialized CF clinics.
What Causes Cystic Fibrosis?
CF is a genetic disease that occurs when a child inherits two defective copies of the gene responsible for cystic fibrosis, one for each parent. CF is not contagious; it is a disease people are born with.
What Are the Signs and Symptoms of Cystic Fibrosis?
CF is a multi-system disorder that produces a variety of symptoms including:
- a persistent cough with productive thick mucus
- wheezing and shortness of breath
- frequent chest infections, which may include pneumonia
- bowel disturbances, such as intestinal obstruction or frequent, oily stools
- weight loss or failure to gain weight despite increased appetite
- Salty tasting sweat
- infertility (men) and decreased fertility (women)
How is Cystic Fibrosis Treated?
Although there is still no cure for CF, there are effective methods of treatment. People with CF attend CF clinics multiple times per year where they work with members of a multidisciplinary healthcare team including a doctor, nurse, physiotherapist, dietitian, social worker and pharmacist. In addition to working with their healthcare team, people with CF typically perform over two hours of treatment per day to maintain their health.
Individual treatment plans generally include:
- chest physiotherapy to help clear mucus from the lungs, typically done two to three times per day
- inhaled antibiotics and other medications to manage lung infections
- pancreatic enzymes, taken with meals to help digest food
-
nutritional supplements and adherence to a special diet higher in calories and fat
Tips for Educators Related to Cystic Fibrosis
- Cystic fibrosis has no effect on intelligence
- Emotionally, a child with CF has the same needs as other children, however, CF may make the child feel different, even though it can be "invisible" for the most part
- Coughing helps clear mucus from the lungs and should not be discouraged
- Children with CF may take pancreatic enzymes with their meals and snacks to help with digestion
- Keeping a box of tissues and garbage can nearby as well as a bottle of water is encouraged
- Special considerations should be taken to avoid unnecessary contact with anyone who has a cold or contagious illness
- Exercise helps clear the mucus from the lungs however, the child's extent of participation and tolerance level fro physical activity may vary from time to time
- Chest physiotherapy is typically done two to three times per day and some children may benefit from having chest therapy done at school. Talk with the children's parents who can consult on this option.
- It is helpful to have discussions about CF and peer teaching is also encouraged. Consider using books with characters with CF as a teaching tool.
- Sometimes a child with CF may find it difficult to "fit in." For example, shortness of breath may limit physical activity in the playground.
- It is important to strengthen the child's self image and encourage them to do their best
- Remember that whatever the condition, the child is an individual first, not a disease
RESOURCES
For more information and resources on cystic fibrosis, please contact:
Cystic Fibrosis Canada
2323 Yonge Street, Suite 800
416-485-9149
info@cysticfibrosis.ca
www.cysticfibrosis.ca