Physiotherapy in cystic fibrosis is a diverse field, and CF physiotherapists offer help with a number of areas, including airway clearance, recommending exercise, sinus management, treating joint, back and continence problems, helping with inhaled treatments and prescribing medicine.
Airway clearance helps to stop thick mucus from building up and blocking airways in the lungs, reducing infections and preventing lung damage.
There are many different techniques, and your specialist CF physiotherapist will work closely with you to find the technique, or combination of techniques, that works best for you. Everyone’s lungs are different and the amount of airway clearance needed will vary from person to person. No one technique has been shown to be better than another, and this may be because each person’s chest can behave very differently, and people may prefer different techniques at different times in their life. It is important that you are happy with the technique you’re using as, despite it being essential, physiotherapy to clear the chest can be a treatment that people struggle to do regularly.
Some airway techniques need no equipment, and focus on breathing exercises to clear mucus:
- Active Cycle of Breathing Techniques (ACBT) – a cycle of deep breathing, huffing, coughing and relaxed breathing to move mucus.
- Autogenic Drainage (AD) – moves mucus from smaller to bigger airways using controlled breathing at different levels. Uses fast flow rates as you sigh out but ensures the airways are kept open.
Other techniques use a device to help to clear mucus. Some use positive pressure to hold open the airways and some also create vibrations within the airways:
- Positive Expiratory Pressure (PEP) – breathing out through a mask or mouthpiece against a resistance to build up pressure behind mucus to move it.
- Oscillating Positive Expiratory Pressure e.g. Flutter®, Acapella® - breathing out against a resistance to create PEP and also vibrate your airways to move mucus.
- High Frequency Chest Wall Oscillation (HFCWO) – an electric air pulse generator that connects to an inflatable jacket (vest) to vibrate the chest. Read a statement by the Association of Chartered Physiotherapists in Cystic Fibrosis, regarding the current evidence behind the VEST.
There are other devices not named above. Be sure to discuss any new airway clearance technique that you would like to try with your CF physiotherapist. They will discuss whether there is good research for the device and whether there are any positives and/or negatives to consider. It is also important to know that airway clearance devices are included in the funding that CF centres get and if your CF physiotherapist has recommended that you try a particular device, then this should be provided for you.
The length and number of airway clearance sessions will vary from person to person, and may need to increase if you have a chest infection.
For few or no lung secretions, treatment sessions may only need to last 10–15 minutes, but if there are many it could take 45–60 minutes. Most people do two treatments a day, but the number and length of your sessions may need to increase depending on the volume of secretions your lungs are producing. If no mucus is present, exercise may play a greater role in your airway clearance techniques. Your CF physiotherapist will be able to advise what is suitable for you.
A specialist CF physiotherapist should assess all newly diagnosed people with CF and advise when and how they should start doing airway clearance. This may not be immediately in screened infants with no symptoms, and exercise/activity may play a greater role. At all ages, the advice will be based on an individual assessment.
The families of babies with CF will be taught how to do airway clearance with their child. As you grow older with CF, your physiotherapist will aim to find a technique that you can carry out independently so that you do not have to rely on someone else to help. Breathing exercises can be introduced from the age of two to three in the form of a game. Older children can start doing airway clearance themselves with supervision. Most teenagers and adults become completely independent, and only need help if they have increased mucus.
Exercise helps with general fitness, health and well-being, and bone health. Improved fitness has been shown to be important in the long term prognosis of CF, and people with CF should aim to maintain an active lifestyle. Physical exercise is also often used as part of an airway clearance regime. Exercise can help loosen mucus in the lungs and make airway clearance techniques quicker and easier. Your CF physiotherapist should be able to answer any questions about the type and intensity of exercise suitable for you.
As you get older it is easy to adopt poor postures, which can lead to back pain and impact upon lung function. It is therefore important for people with CF to maintain good posture. Your physiotherapist will be able to offer advice and help to assess and treat any joint or back problems you may have. Download our leaflet on how to improve your posture here.
Some people with CF may develop problems with urinary or faecal incontinence (leaking wee or poo) when coughing and/or exerting themselves. If this should happen to you it is important to tell your physiotherapist as soon as possible and not be embarrassed, as they can teach you pelvic floor exercises to help. They may also refer you on to a specialist. For more information see our factsheet.
Many people with CF will take inhaled medications as part of their treatment. This may be to loosen the mucus, hydrate the airways, open up the airways, reduce inflammation of the airways and/or to deliver antibiotics to treat infections. These medications may come in nebulised (inhaling a mist) or dry powder form, and often the timing of these alongside airway clearance and exercise is important to ensure your treatment is as effective as possible. Please ask your physiotherapist for advice. There are many different nebuliser devices available and more information can be found in our inhaled therapy factsheet.
We have worked with the Association of Chartered Physiotherapists in Cystic Fibrosis (ACPCF) to produce a series of leaflets to complement the advice and teaching physiotherapists give people with cystic fibrosis. Head over to our physiotherapy leaflets page to download the leaflets.
You may also find these leaflets useful: