Key messages
In the short term, digital technology may help people with cystic fibrosis (CF) adhere (or 'stick to') their inhaled treatments, but have little or no effect on lung function or pulmonary exacerbations (flare-ups of disease).
In the medium term, combining digital monitoring with tailored support via an online platform probably encourages people to adhere to inhaled treatment and reduces treatment demands on people with CF made by their healthcare needs and the impact this has on their well-being, but without improving their quality of life.
Future research should assess digital technology for helping children and adults with CF adhere to their inhaled treatments and consider how this affects other areas of their treatment.
What is CF?
CF is a life-threatening, inherited condition where sticky, thick mucus builds up in the lungs and digestive system. Over time, the lungs become damaged and may eventually stop working properly.
How is CF treated?
There is currently no cure for CF, but treatment helps control symptoms and reduce complications. Treatments include antibiotics to prevent and treat chest infections, and medicines to thin the mucus in the lungs, making it easier to cough up. People with CF use inhalers and nebulisers to deliver medicines quickly to the lungs. Nebulisers are small machines that change liquid medicine into a mist which is inhaled through a mouthpiece or mask.
Treating CF is complicated and time-consuming; people with CF typically spend 2 to 2.5 hours daily on treatment.
People increasingly use digital technologies to monitor their health and fitness via activity trackers and mobile phone applications. People with CF use digital technologies for tracking and improving how they manage their treatment. Some technologies allow information to be uploaded to the internet, so individuals and their healthcare teams can immediately use this information to monitor and improve treatment.
What did we want to find out?
Can digital technology help people with CF stick to their inhaled treatments?
Do digital technologies for monitoring adherence have any unwanted or harmful effects?
What did we do?
We searched for studies examining any kind of digital technology for monitoring adherence to inhaled treatments for CF. We summarised the results of the studies and rated our confidence in the evidence, based on factors such as study methods and sizes.
What did we find?
We found two studies, with 628 people aged five to 41 years, using different digital technologies. We could not combine their results and analysed them separately. Both studies put the people taking part into one of two groups at random, with equal chances of being in either group.
One small study compared two breathing modes of a digital nebuliser in 20 children aged 5 to 16 years (the nebuliser delivered an antibiotic as an inhaled mist). One mode encouraged children to take longer, deeper breaths via an adapted mouthpiece; the other mode consisted of the usual breathing pattern. The study lasted for 10 weeks.
The larger study involved 608 people aged 16 years and older. It compared a data-tracking nebuliser paired with a web-based platform called CFHealthHub to the same nebuliser used without the web-based platform. CFHealthHub gave participants access to their adherence data and individual support from study authors, who collected data for 12 months.
Key results
The study comparing inhalation modes found that the children using the digitally enhanced breathing mode recorded higher adherence to nebuliser treatment than the children using the usual breathing mode. However, this may make little difference to lung function. There were no adverse (harmful or unwanted) effects from using the different inhalation modes.
The second study showed that combining an online programme with a data-tracking nebuliser probably improves adherence compared to just using the nebuliser. The combination of nebuliser plus online support also probably lowers the 'burden of treatment'. However, it probably makes little or no difference to quality of life or the number of flare-ups of disease. The group using the online platform reported slightly more adverse events, but we found no difference between groups in adverse effects we considered directly related to treatment (measured using anxiety and depression scores).
What are the limitations of the evidence?
Our confidence in the evidence from the smaller study is limited because it was very small and focused on children, whereas our question was broader. It also only reported on two of our planned outcomes.
Our confidence in the evidence from the larger study ranged from low to moderate. This study was larger and looked at more of our outcomes. However, this study only included people aged 16 and older, so we do not know whether its findings apply to younger children.
Current evidence for how digital technology can improve adherence to inhaled treatments is limited. Future research should assess how technology can improve adherence to inhaled treatments in children and adults, and consider how this affects the total treatment plan (adhering to inhaled treatments may decrease time spent on other treatments).
How up to date is the evidence?
The evidence is current to 27 February 2025.
Read the full abstract
Improved understanding and treatment of cystic fibrosis (CF) has led to longer life expectancy, which is accompanied by an increasingly complex regimen of treatments. Suboptimal adherence to the treatment plan, in the context of respiratory disease, has been found to be associated with poorer health outcomes. With digital technology being more accessible, it can be used to monitor adherence to inhaled therapies via chipped nebulisers, mobile phone apps and web-based platforms. This technology can allow monitoring of adherence as well as clinical outcomes, and allow feedback to both the person with CF and their healthcare team.
Objectives
To assess the effects of using digital technology to monitor adherence to inhaled therapies and health status in adults and children with CF.
Search strategy
We searched the Cochrane CF Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched Embase and three clinical trial registries, and checked the references of included studies. The date of last search was 27 February 2025.
Selection criteria
We searched for randomised controlled trials (RCTs) looking at the effects of a digital technology for monitoring adherence of children and adults with CF to inhaled therapies.
Data collection and analysis
Two review authors screened the search results for studies eligible for inclusion in the review and extracted their data. We used Risk of Bias 2 for assessing study quality. We assessed the overall certainty of the evidence using GRADE.
Main results
We included two studies in our review, with 628 participants aged five to 41 years. There was one study each for two different comparisons.
Nebuliser target inhalation mode versus standard inhalation mode
The included parallel study was carried out over 10 weeks after a run-in period of four to six weeks. The study compared the effects of a digitally enhanced inhalation mode (target inhalation mode) for nebulised antibiotics compared to standard mode in children attending a regional CF clinic in the United Kingdom. The study's primary outcome was the time taken to complete the inhaled treatment, but investigators also reported on adherence to therapy. The results showed that there may be an improvement in adherence with the target inhalation mode when this intervention is used (mean difference (MD) 24.0%, 95% confidence interval (CI) 2.95 to 45.05; low-certainty evidence). The target inhalation mode may make little or no difference to forced expiratory volume in one second (FEV1) % predicted (MD 1.00 % predicted, 95% CI -9.37 to 11.37; low-certainty evidence). The study did not report on treatment burden, quality of life (QoL) or pulmonary exacerbations.
eNebuliser with digital support versus eNebuliser without support
One large multicentre RCT monitored adherence via data-tracking nebulisers. The intervention group also receiving access to an online web-based platform, CFHealthHub, which offered tailored, flexible support from the study interventionist as well as access to their adherence data, educational and problem-solving information throughout the 12-month trial period. We graded all evidence as moderate certainty. Compared to usual care, the digital intervention probably improves adherence to inhaled therapy (MD 18%, 95% CI 12.90 to 23.10); probably leads to slightly reduced treatment burden (MD 5.1, 95% CI 1.79 to 8.41); and may lead to slightly improved FEV1 % predicted (MD 3.70, 95% CI -0.23 to 7.63). There is probably little or no difference in the incidence of pulmonary exacerbations or QoL between the two groups.
Authors' conclusions
Digital monitoring plus tailored support via an online platform probably improves adherence to inhaled therapies and reduces treatment burden (but without a corresponding change in QoL) in the medium term (low- and moderate-certainty evidence). In a shorter timeframe, technological enhancement of inhaling antibiotics may improve adherence to treatment (low-certainty evidence). There may be little or no effect on lung function with either intervention, and online monitoring probably makes no difference to pulmonary exacerbations.
Future research should assess the effect of digital technology on adherence to inhaled therapies in both children and adults. Consideration of adherence to the total treatment regimen is also important, as improved adherence to inhaled therapies could come at the cost of adherence to other parts of the treatment regimen.
Funding
The original review was supported by Cochrane Infrastructure funding from the National Institute for Health Research (NIHR). The review was updated with Cochrane CF funding from the CF Foundation and the UK CF Trust.
Registration
Protocol (2020) DOI: 10.1002/14651858.CD013733
Original review (2023) DOI: 10.1002/14651858.CD013733.pub2
