Canadian CF treatment options

Living with cystic fibrosis requires a daily treatment routine to maintain optimal health. In the sections below you can learn more about CF treatment options available in Canada.

Treatment resources

Airway Clearance Resources

Cleaning Nebulizer and Airway Clearance Equipment at Home (This document was written by the Physiotherapy Advisory Group)

Canadian CF physiotherapists follow the IPG-CF blue booklet on physiotherapy techniques available to members through the European Cystic Fibrosis Society.

Medications and CFTR Modulators

Medications are a crucial part of cystic fibrosis treatment and care. This includes highly effective modulators (such as Trikafta), enzymes, antibiotics and more. In this section, we will provide a general overview of the types of medications someone with CF may take in their day-to-day life.

CFTR Modulators

CFTR modulators are becoming an increasingly common aspect of CF care. Learn more about types of modulators, how they work and how you can access them.

Antibiotics

Antibiotics are an important piece of maintaining your health for a person with a chronic illness. With cystic fibrosis, antibiotics are used to reduce bacteria in the lungs. They can be taken in three ways, through inhalation, orally and intravenous (IV).

Antibiotics are also used during a pulmonary exacerbation (or when you may have more lung symptoms).

RESOURCE: Medications / Drug Access - Toronto Adult Cystic Fibrosis Centre

Enzymes

For people with cystic fibrosis, the pancreas does not make enough digestive enzymes to break down and digest foods. This can cause poor nutrition and limit normal growth. An important medication for people with CF is pancreatic digestive enzymes in pill form. The enzymes work to help the body digest fats, proteins and complex carbohydrates.

RESOURCE: What are Enzymes and How do They Work?

Vitamins

In addition to enzymes, people with CF take vitamins to help absorb fats from foods. Fat soluble vitamins such as vitamin A, D, E and K are essential to a healthy cystic fibrosis diet.

RESOURCE: Living with CF - Nutrition

Inhaled Medications

There are other inhaled medications that work to support treatment in people with cystic fibrosis. Some of these inhaled medications work to increase the size of air passages in the lungs, or to reduce inflammation.

Mucolytics

Mucolytics are drugs that either breakup mucus or make mucus thinner to prevent blockage of the airways. These medications are delivered (inhaled) using a nebulizer.

Corticosteroids

Corticosteroids are used for treating the inflammation of tissue. In people with cystic fibrosis, this may be for lung inflammation and mucus plugging.

This information has been compiled from the Toronto Adult Cystic Fibrosis Centre page.

The Toronto Adult Cystic Fibrosis Centre provides a detailed overview of different types of inhaled medications, mucolytics and more

RESOURCE: Living with CF - Medication & Drug Access

Active Cycle of Breathing Techniques (ACBT)

ACBT is a cycle of breathing control, chest expansion exercises, and the forced expiratory technique or huffing. Breathing control and chest expansion exercises allow air to travel deep into the lungs and get behind thick mucus, which can then be expelled by huffing.

Autogenic Drainage (AD)

This technique uses one’s airflow while breathing out to move mucus from the small to larger airways where it can be cleared. To achieve this, the level of breathing is adjusted, from low lung volume breathing, progressing to mid lung volume breathing and then high lung volume breathing.

Oscillating positive expiratory pressure (PEP)

This technique involves breathing repeatedly through a device which causes pressure and vibrations in the airways to help loosen and move mucus out. There are different oscillating PEP devices on the market, check with your clinic to determine which one they recommend.

Positive Expiratory Pressure (PEP)

Individuals breathe through a face mask or mouthpiece attached to a piece of equipment called a resistor, which causes pressure to build up in the lungs. The pressure keeps the airways open, allowing air to get behind the mucus. Mucus can then be moved to the main airway and cleared.

Postural drainage and percussion (PD&P), commonly known as chest physiotherapy

This technique is commonly used in infants and young children. An individual is placed in a variety of positions to drain mucus from different parts of the lung. Chest clapping, or percussion, followed by vibrations, is added, along with deep breathing exercises, huffing (actively exhaling but less forceful than coughing) and coughing to help clear mucus from the airway. Modified non-head down positions are now used as the standard of care.

Note from Canadian physiotherapy chairs

Based on current evidence, high frequency chest wall oscillation (HFCWO or “vest therapy”) is not the preferred airway clearance technique in Canada at this time. A Canadian multi-centre study using exacerbation rate as the primary outcome, showed that there was a significant reduction in pulmonary exacerbation in people using PEP (positive expiratory pressure) compared to those using HFCWO. Consultation with your cystic fibrosis clinic is recommended as individual preference and health status (lung function, developmental stage and respiratory symptoms) should always be considered when selecting an airway clearance technique.

Clinical guidelines for cystic fibrosis

Cystic Fibrosis Canada is aiming to expand the reach of clinical knowledge within our specialized CF Clinics, so that every Canadian living with cystic fibrosis receives the care they need when they need it.

Through Canadian cystic fibrosis clinicians, CF Canada works to endorse and update clinical guidelines to ensure consistent care across Canada. We are currently in the process of developing Canadian Consensus Care Guidelines, which will be available when they are finalized.

Guidelines & Standards of Care

Airway clearance techniques

Airway clearance is very important in cystic fibrosis care as it helps to prevent a build-up of thick mucus in the lungs, reduce chest infections and improve lung function in people living with the disease.

In Canadian cystic fibrosis clinics, a physiotherapist will work with people with CF and their caregivers to choose the evidence-based technique or combination of techniques that work best for each individual. The physiotherapist will also recommend how often airway clearance therapy should be performed and will work with other clinic team members to determine the optimal timing of airway clearance when taking certain medications.

For more information about airway clearance options and which ones are right for you, please contact your local cystic fibrosis clinic.

Find a Canadian CF Clinic