Docs Tag: breathing

Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis or CF is a disease that mainly affects the lungs and digestive system (or digestive tract). Although CF can be treated and CF patients can usually lead fairly normal lives, there is no cure for CF. Today, half of Canadians with CF live into their 40s and beyond.

Cystic fibrosis

Stomach, pancreas, small intestine and lung identified in upper body of a child, with a close-up showing mucus in the airways
In cystic fibrosis, mucus in the lungs and digestive tract is stickier than normal. Mucus builds up and causes lung congestion and problems with digestion.

CF affects the lungs

Normal mucus is thin and slippery. It keeps the lungs clean by removing dirt and germs from the lungs’ airway tubes. In CF, mucus is sticky and clogs the tubes. This can make breathing difficult. Bacteria can collect in the tubes because the mucus cannot clear as quickly as it should. This leads to cycles of infection and inflammation (swelling in the airway tubes). These infections can damage the lung tissues.

CF may also affect the digestive system

CF may also affect the digestive system, especially the pancreas. The pancreas is an organ just below the stomach that makes enzymes to help digest food in the small intestine. Enzymes help with digestion and break down the food particles small enough to be absorbed. In CF, mucus blocks the ducts (tube-like channels that carry fluid) of the pancreas.

When the ducts from the pancreas to the small intestine are blocked by mucus, the enzymes cannot reach the small intestine. This means food is not properly digested. When this happens, a child with CF does not get enough nutrition from their food. As a result, a child with CF may take replacement enzymes in order to grow normally and may have to eat a bit more food.

Information taken from https://www.aboutkidshealth.ca/Article?contentid=882&language=English


Typically somewhere between 12 to 14 years, CF patients begin to undertake more responsibility for their own care. The following is advice targeted to this age group.


Adolescence is a time when you are finding your own identity. It is natural that you want more independence, but it is also important that you take the right steps to keep healthy; this includes working cooperatively with your caregiver/parent and CF clinic team. Cystic fibrosis is a progressive disease, which makes staying on top of your health extremely important.

To begin the journey to more independence, it is essential that you are informed and learn to “take charge wisely”. The following are suggestions to help you get started:

Ask questions about your care such as why certain medications or treatments are completed.
Begin to more actively participate in your treatment e.g. “lay out” your medications and “set up” your equipment for treatment (for your caregiver’s review). This will build confidence and is an important step in the transition to self-care.
Research cystic fibrosis and what the future will bring.
Book your own CF clinic appointments. Get organized before appointments and make a list of questions.
Take the lead in communication with the CF clinic team members.


By now you’ve probably heard how important it is to eat high-calorie foods and take your nutritional vitamins and supplements. Adolescence can be a difficult time as the social stresses of media tells young adolescents that they must look a certain way. It’s imperative you talk with your CF nurse or dietitian to discuss ways to maintain your weight and adequate nutrition.

Below are a few documents to review, published by cystic fibrosis dietitians across Canada:

Contributions from: Daina Kalnins, MSc, RD and Janey Hughes, P.Dt, CDE Information taken from https://www.cysticfibrosis.ca/about-cf/living-with-cystic-fibrosis/adolescents

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