Background

Cystic fibrosis (CF) is a progressive and life-limiting genetic condition that affects many systems in the body, especially the lungs. In the UK, 1 in every 2500 babies is diagnosed with CF. Physiotherapy is a very important part of treatment for people with CF. The two main goals of chest physiotherapy are to clear sticky mucus from the airways to prevent repeated infections and lung damage and to maintain physical fitness through exercise.

Chest physiotherapy can help prevent poor health and hospital admissions and may contribute to improving quality of life and life expectancy, but it is often considered a burden. People with CF may have to complete seven or more chest physiotherapy treatments a day, which can be time-consuming, tiring, and disruptive. As a result, people with CF may complete as little as 30% of their chest physiotherapy sessions, with rates often dipping during some stages in a person's life (such as adolescence). Although exercise is often viewed more positively by people with CF, they may not always carry out their exercises because of tiredness, feeling unwell, or a lack of time.

We wanted to explore what we can do to encourage people with CF to complete more of their planned chest physiotherapy or exercise. We were interested in a range of possible strategies including leaflets, videos, apps, rewards, motivational tools, exercise games, diaries, and text messages.

What did we do?

We looked for high-quality studies of people with CF (aged seven years and upwards). We planned to include any strategy aimed at improving adherence to chest physiotherapy or exercise compared to no strategy, usual care, or another type of strategy.

What did we find?

We found only two studies that we could include in our review. One was an educational study to improve adherence to exercise, and the second was a motivational study to increase adherence to chest physiotherapy.

In the exercise study (34 participants), those who received a written manual seemed to be more physically active at the end of the study, compared to those who were given verbal advice only, but we are not certain whether this was due to the manual. There were no other differences between groups in terms of quality of life, lung function, or exercise capacity.

In the chest physiotherapy study (43 participants), we are uncertain whether listening to specially-composed music during chest physiotherapy leads to more time spent completing treatments. We did not find that listening to music people chose themselves while doing physiotherapy led to completion of more treatment compared to not listening to any music. We did not find any real difference in hospital admissions due to chest infection between any of the groups.

What are the limitations of the evidence?

In both studies, the groups were very small and there were problems in trial design and reporting. This means that the reliability of the results might be affected. As a result, the evidence for strategies to improve adherence to physiotherapy and adherence to exercise was very uncertain. Whilst these studies suggest that some strategies may be helpful, future research needs to consider ways to accurately measure adherence. In the meantime, physiotherapists treating people with CF should follow local guidelines and national standards of care related to CF and treatment adherence.

How up to date is the evidence?

The evidence is current to 28 March 2023.

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