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Review
. 2023 Mar 29;3(3):CD013766.
doi: 10.1002/14651858.CD013766.pub2.

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis

Sophie Dawson  1   2 , Carla-Jane Girling  3 , Lisa Cowap  2 , David Clark-Carter  2
Affiliations
Review

Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis

Sophie Dawson et al. Cochrane Database Syst Rev. .

Abstract

Background: Adherence to treatment, including inhaled therapies, is low in people with cystic fibrosis (CF). Although psychological interventions for improving adherence to inhaled therapies in people with CF have been developed, no previous published systematic review has evaluated the evidence for efficacy of these interventions.

Objectives: The primary objective of the review was to assess the efficacy of psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (CF). The secondary objective was to establish the most effective components, or behaviour change techniques (BCTs), used in these interventions.

Search methods: We searched the Cochrane Cystic Fibrosis Trials Register, which is compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched databases (PubMed; PsycINFO; EBSCO; Scopus; OpenGrey), trials registries (World Health Organization International Clinical Trials Registry Platform; US National Institutes of Health Ongoing Trials Register ClinicalTrials.gov), and the reference lists of relevant articles and reviews, with no restrictions on language, year or publication status. Date of search: 7 August 2022.

Selection criteria: We included randomised controlled trials (RCTs) comparing different types of psychological interventions for improving adherence to inhaled therapies in people with CF of any age, or comparing psychological interventions with usual care. We included quasi-RCTs if we could reasonably assume that the baseline characteristics were similar in both groups.

Data collection and analysis: Two review authors independently assessed trial eligibility and completed data extraction, risk of bias assessments, and BCT coding (using the BCT Taxonomy v1) for all included trials. We resolved any discrepancies by discussion, or by consultation with a third review author as necessary. We assessed the certainty of the evidence using GRADE.

Main results: We included 10 trials (1642 participants) in the review (children and adolescents in four trials; adults in five trials; and children and adults in one trial). Nine trials compared a psychological intervention with usual care; we could combine data from some of these in a number of quantitative analyses. One trial compared a psychological intervention with an active comparator (education plus problem-solving (EPS)). We identified five ongoing trials. Psychological interventions were generally multi-component and complex, containing an average of 9.6 BCTs (range 1 to 28). The two most commonly used BCTs included 'problem-solving' and 'instruction on how to perform the behaviour'. Interventions varied in their type, content and mode of delivery. They included a problem-solving intervention; a paper-based self-management workbook; a telehealth intervention; a group training programme; a digital intervention comprising medication reminders and lung function self-monitoring; a life-coaching intervention; a motivational interviewing (MI) intervention; a brief MI intervention (behaviour change counselling); and a digital intervention combined with behaviour change sessions. Intervention duration ranged from 10 weeks to 12 months. Assessment time points ranged from six to eight weeks up to 23 months. Psychological interventions compared with usual care We report data here for the 'over six months and up to 12 months' time point. We found that psychological interventions probably improve adherence to inhaled therapies (primary outcome) in people with CF compared with usual care (mean difference (MD) 9.5, 95% confidence interval (CI) 8.60 to 10.40; 1 study, 588 participants; moderate-certainty evidence). There was no evidence of a difference between groups in our second primary outcome, treatment-related adverse events: anxiety (MD 0.30, 95% CI -0.40 to 1.00; 1 study, 535 participants), or depression (MD -0.10, 95% CI -0.80 to 0.60; 1 study, 534 participants), although this was low-certainty evidence. For our secondary outcomes, there was no evidence of a difference between groups in terms of lung function (forced expiratory volume in one second (FEV1) % predicted MD 1.40, 95% CI -0.20 to 3.00; 1 study, 556 participants; moderate-certainty evidence); number of pulmonary exacerbations (adjusted rate ratio 0.96, 95% CI 0.83 to 1.11; 1 study, 607 participants; moderate-certainty evidence); or respiratory symptoms (MD 0.70, 95% CI -2.40 to 3.80; 1 study, 534 participants; low-certainty evidence). However, psychological interventions may improve treatment burden (MD 3.90, 95% CI 1.20 to 6.60; 1 study, 539 participants; low-certainty evidence). The overall certainty of the evidence ranged from low to moderate across these outcomes. Reasons for downgrading included indirectness (current evidence included adults only whereas our review question was broader and focused on people of any age) and lack of blinding of outcome assessors. Psychological interventions compared with an active comparator For this comparison the overall certainty of evidence was very low, based on one trial (n = 128) comparing an MI intervention to EPS for 12 months. We are uncertain whether an MI intervention, compared with EPS, improves adherence to inhaled therapies, lung function, or quality of life in people with CF, or whether there is an effect on pulmonary exacerbations. The included trial for this comparison did not report on treatment-related adverse events (anxiety and depression). We downgraded all reported outcomes due to small participant numbers, indirectness (trials included only adults), and unclear risk of bias (e.g. selection and attrition bias).

Authors' conclusions: Due to the limited quantity of trials included in this review, as well as the clinical and methodological heterogeneity, it was not possible to identify an overall intervention effect using meta-analysis. Some moderate-certainty evidence suggests that psychological interventions (compared with usual care) probably improve adherence to inhaled therapies in people with CF, without increasing treatment-related adverse events, anxiety and depression (low-certainty evidence). In future review updates (with ongoing trial results included), we hope to be able to establish the most effective BCTs (or 'active ingredients') of interventions for improving adherence to inhaled therapies in people with CF. Wherever possible, investigators should make use of the most objective measures of adherence available (e.g. data-logging nebulisers) to accurately determine intervention effects. Outcome reporting needs to be improved to enable combining or separation of measures as appropriate. Likewise, trial reporting needs to include details of intervention content (e.g. BCTs used); duration; intensity; and fidelity. Large trials with a longer follow-up period (e.g. 12 months) are needed in children with CF. Additionally, more research is needed to determine how to support adherence in 'under-served' CF populations.

PubMed Disclaimer

Conflict of interest statement

SD is employed by Nottingham University Hospitals NHS Trust to work on the CFHealthHub Data Observatory, which aims to embed objective adherence data into routine CF care using quality improvement cycles. She did not have any involvement in either the CFHealthHub feasibility study or RCT included in this review.

CG is employed by the University of Sheffield, Clinical Trials Research Unit, and previously managed a clinical trial related to supporting people with CF with adherence behaviours "CFHealthHub Data Observatory" (IRAS 216782 portfolio 33331); the funding was from NHS England.

LC declares no potential conflict of interest.

DCC declares no potential conflict of interest.

Figures

1
1
Study flow diagram.
2
2
Risk of bias summary: review authors' judgements about each risk of bias item for each included study.
1.1
1.1. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 1: Adherence ‐ percentage of prescribed inhaled therapies taken (objective recorded measures)
1.2
1.2. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 2: Adherence ‐ percentage of prescribed inhaled therapies taken (objective recorded measures ‐ adjusted mean difference)
1.3
1.3. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 3: Adherence ‐ percentage of prescribed inhaled therapies taken (subjective measures)
1.4
1.4. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 4: Adherence to dornase alfa
1.5
1.5. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 5: Adherence to inhaled antibiotics (mean score on MMAS‐8)
1.6
1.6. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 6: Adherence to inhaled steroids (subjective measure)
1.7
1.7. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 7: Adherence to hypertonic saline (subjective measure)
1.8
1.8. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 8: Adherence to bronchodilators (subjective measure)
1.9
1.9. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 9: Anxiety (endpoint data)
1.10
1.10. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 10: Anxiety (adjusted mean difference)
1.11
1.11. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 11: Depression (endpoint data)
1.12
1.12. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 12: Depression (adjusted mean difference)
1.13
1.13. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 13: QoL: Quality of Well‐being scale (mean score)
1.14
1.14. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 14: QoL: Physical functioning (endpoint data)
1.15
1.15. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 15: QoL: Physical functioning (adjusted data)
1.16
1.16. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 16: QoL: Emotional functioning (endpoint data)
1.17
1.17. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 17: QoL: Emotional functioning (adjusted data)
1.18
1.18. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 18: QoL: Social functioning (endpoint data)
1.19
1.19. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 19: QoL: Social functioning (adjusted data)
1.20
1.20. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 20: QoL: Treatment burden (endpoint data)
1.21
1.21. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 21: QoL: Treatment burden (adjusted data)
1.22
1.22. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 22: QoL: Role limitations (endpoint data)
1.23
1.23. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 23: QoL: Role limitations (adjusted data)
1.24
1.24. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 24: QoL: Body image (endpoint data)
1.25
1.25. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 25: QoL: Body image (adjusted data)
1.26
1.26. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 26: QoL: Vitality (endpoint data)
1.27
1.27. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 27: QoL: Vitality (adjusted data)
1.28
1.28. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 28: QoL: Eating disturbance (endpoint data)
1.29
1.29. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 29: QoL: Eating disturbance (adjusted data)
1.30
1.30. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 30: QoL: Weight problems (endpoint data)
1.31
1.31. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 31: QoL: Weight problems (adjusted data)
1.32
1.32. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 32: QoL: Respiratory symptoms (endpoint data)
1.33
1.33. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 33: QoL: Respiratory symptoms (adjusted data)
1.34
1.34. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 34: QoL: Digestive symptoms (endpoint data)
1.35
1.35. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 35: QoL: Digestive symptoms (adjusted data)
1.36
1.36. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 36: QoL: Health perceptions (endpoint data)
1.37
1.37. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 37: QoL: Health perceptions (adjusted data)
1.38
1.38. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 38: QoL: Treatment issues (endpoint data)
1.39
1.39. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 39: QoL: Career concerns (endpoint data)
1.40
1.40. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 40: QoL: Interpersonal relationships (endpoint data)
1.41
1.41. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 41: QoL: Concerns for the future (endpoint data)
1.42
1.42. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 42: FEV1 % predicted (endpoint data)
1.43
1.43. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 43: FEV1 % predicted (adjusted mean difference)
1.44
1.44. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 44: Number of pulmonary exacerbations
1.45
1.45. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 45: Pulmonary exacerbations incidence rate (unadjusted data)
1.46
1.46. Analysis
Comparison 1: Psychological interventions versus usual care, Outcome 46: Pulmonary exacerbations incidence rate (adjusted data)
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References

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Abbott 2015 {published data only}
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    1. Cummings E, Hauser J, Cameron-Tucker H, Fitzpatrick P, Jessup M, Walters EH, et al. Enhancing self-efficacy for self-management in people with cystic fibrosis. Studies in Health Technology and Informatics 2011;169:33-7. [CENTRAL: CN-00806050] [CFGD REGISTER: MH24e] [PMID: ] - PubMed
Czajkowski 1985 {unpublished data only}
    1. Czajkowski DR. The use of psychosocial competency/coping skills model to assess and predict the medical compliance of cystic fibrosis adolescents. www.opengrey.eu (accessed 14 November 2020).
Daniels 2013 {published data only}
    1. Daniels T, Mills N, Whitaker P. Nebuliser systems for drug delivery in cystic fibrosis. Cochrane Database of Systematic Reviews 2013, Issue 4. Art. No: CD007639. [DOI: 10.1002/14651858.CD007639.pub2] - DOI - PubMed
Davis 2004 {published data only}
    1. Davis MA, Quittner AL, Stack C. Controlled evaluation of the STARBRIGHT Explorer Series CD-Rom program for children and adolescents with cystic fibrosis. Pediatric Pulmonology 2002;34 Suppl 24:351. [CENTRAL: CN-00404026] - PubMed
    1. Davis MA, Quittner AL, Stack CM, Yang MC. Controlled evaluation of the STARBRIGHT CD-ROM program for children and adolescents with cystic fibrosis. Journal of Pediatric Psychology 2004;29(4):259-67. [CENTRAL: CN-00482821] [CFGD REGISTER: MH10b] [PMID: ] - PubMed
Dodd 2000 {published data only}
    1. Dodd ME, Webb K. Understanding non-compliance with treatment in adults with cystic fibrosis. Journal of the Royal Society of Medicine 2000;93(38):2-8. - PMC - PubMed
DRKS00027569 {published data only}
    1. DRKS00027569. Applying the "MukoHelp" manual to improve psychopathological symptoms and therapy adherence among affected persons with cystic fibrosis. trialsearch.who.int/Trial2.aspx?TrialID=DRKS00027569 (date of registration 26 January 2022).
Duff 2014 {published data only}
    1. Duff AJ, Latchford G. Adherence in cystic fibrosis; care teams need to change first. Lancet: Respiratory Medicine 2014;2(9):683-5. [DOI: 10.1016/S2213-2600(14)70162-6] - DOI - PubMed
Elkins 2006 {published data only}
    1. Bye P, Elkins M, Robinson M, Moriarty C, Rose B, Harbour C, et al. Long-term inhalation of hypertonic saline in patients with cystic fibrosis- a randomised controlled trial. Pediatric Pulmonology 2004;38 Suppl 27:329. [CENTRAL: CN-00507890] [CFGD REGISTER: BD114a]
    1. Elkins M, Robinson M, Moriarty C, Sercombe J, Rose B, Harbour C, et al. Three methods of monitoring adherence in a long-term trial in cystic fibrosis. Journal of Cystic Fibrosis 2006;5 Suppl 1:S111. [CENTRAL: CN-00593155] [CFGD REGISTER: BD114d]
    1. Elkins MR, Robinson M, Moriarty C, Sercombe J, Rose B, Harbour C. Three methods of monitoring adherence in the national hypertonic saline trial in cystic fibrosis. Pediatric Pulmonology 2005;40 Suppl 28:268. [CENTRAL: CN-00593023] [CFGD REGISTER: BD114c]
    1. Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. New England Journal of Medicine 2006;354(3):229-40. [CENTRAL: CN-00532707] [CFGD REGISTER: BD114b] [EMBASE: 2006038601] [PMID: ] - PubMed
    1. Ratjen F. Inhaled hypertonic saline produces small increases in lung function in patients with cystic fibrosis. Journal of Pediatrics 2006;149(1):142. [CENTRAL: CN-01253122] [CFGD REGISTER: BD114e] [PMID: ] - PubMed
Fischer 2003 {published data only}
    1. Fischer R, Naehrig S, Schelling JS, Horn M, Huber RM. Telemonitoring in adult cystic fibrosis patients. Pediatric Pulmonology 2003;36 Suppl 25:371. [CENTRAL: CN-00451898] [CFGD REGISTER: MH11]
Geirhos 2022 {published data only}
    1. DRKS00016714. Feasibility trial of an online intervention for symptoms of depression and anxiety in adolescents with chronic medical conditions (youthCOACH-CD). trialsearch.who.int/Trial2.aspx?TrialID=DRKS00016714 (date registered 25 March 2019).
    1. DRKS00017161. Effectiveness and cost-effectiveness of guided internet-and mobile-based CBT for adolescents and young adults with chronic somatic conditions and comorbid depression and anxiety symptoms (youthCOACHcd): a multicentre randomized controlled trial with a 12-month follow-up. trialsearch.who.int/Trial2.aspx?TrialID=DRKS00017161 (date registered 17 September 2019).
    1. Geirhos A, Domhardt M, Lunkenheimer F, Temming S, Holl RW, Minden K, et al. Feasibility and potential efficacy of a guided internet- and mobile-based CBT for adolescents and young adults with chronic medical conditions and comorbid depression or anxiety symptoms (youthCOACH CD): a randomized controlled pilot trial. BMC Pediatrics 2022;22(1):69. [DOI: 10.1186/s12887-022-03134-3] - DOI - PMC - PubMed
    1. Lunkenheimer F, Domhardt M, Geirhos A, Kilian R, Mueller-Stierlin AS, Holl RW, et al. Effectiveness and cost-effectiveness of guided Internet- and mobile-based CBT for adolescents and young adults with chronic somatic conditions and comorbid depression and anxiety symptoms (youthCOACHCD): study protocol for a multicentre randomized controlled trial. Trials 2020;21(1):253. - PMC - PubMed
    1. Lunkenheimer F, Domhardt M, Geirhos A, Kilian R, Mueller-Stierlin AS, Holl RW, et al. Effectiveness and cost-effectiveness of guided Internet- and mobile-based CBT for adolescents and young adults with chronic somatic conditions and comorbid depression and anxiety symptoms (youthCOACHCD): study protocol for a multicentre randomized controlled trial. Trials 2020;21(1):253. Online Supplementary Materials: Additional file 1 SPIRIT 2013 Checklist. - PMC - PubMed
Geller 2011 {published data only}
    1. Geller DE, Madge S. Technological and behavioral strategies to reduce treatment burden and improve adherence to inhaled antibiotics in cystic fibrosis. Respiratory Medicine 2011;105(S2):S24-31. - PubMed
Goldbeck 2013 {published data only}
    1. Goldbeck L, Hebestreit H, Junge S, Sens B, Smaczny C, Frank M, et al. Vemse-CF - A prospective controlled care-research study investigating the effects of a comprehensive psychosocial intervention. Pediatric Pulmonology 2013;48 Suppl 36:430. [ABSTRACT NO.: 608] [CENTRAL: CN-00875655] [CFGD REGISTER: MH39]
    1. NCT03423576. Effects of a comprehensive patient-centered health service in cystic fibrosis (VEMSE-CF) [Effects of a comprehensive patient-centered health service with a focus on psychosocial services for patients with are diseases using cystic fibrosis as an example (Evaluation Eines Ganzheitlichen Patientenzentrierten Versorgungsmodells für Patienten Mit Seltenen Erkrankungen Unter Besonderer Berücksichtigung Der Psychosozialen Versorgung am Beispiel Mukoviszidose)]. clinicaltrials.gov/ct2/show/NCT03423576 (first posted 06 February 2018).
Goodill 2005 {published data only}
    1. Goodill SW. Dance/movement therapy for adults with cystic fibrosis: pilot data on mood and adherence. Alternative Therapies in Health and Medicine 2005;11(1):76-7. [CENTRAL: CN-00502675] [CFGD REGISTER: MH16] [DOI: 10.1016/S0954-6111(11)70024-5] [PMID: ] - DOI - PubMed
Grossoehme 2020 {published data only}
    1. Grossoehme DH, Cole AG, Lewis K, Stamper SM, Teeters A, Joseph PM. Adults with cystic fibrosis: spiritual coping with lifelong disease. Journal of Health Care Chaplaincy 2020;26(2):45-57. [CENTRAL: CN-02098160] [EMBASE: 631260321] [PMID: ] - PubMed
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Hagelberg 2008 {published data only}
    1. Hagelberg M, Dooley MJ, Poole SG, Leung D, Bailey M, Finlayson F, et al. Direct dispensing of dornase alpha improves adherence and lung function in cystic fibrosis. Journal of Cystic Fibrosis 2008;7 Suppl 2:S27. [CENTRAL: CN-00651989] [CFGD REGISTER: MH25]
Hatziagorou 2017 {published data only}
    1. Hatziagorou E, Chrysochoou EA, Kirvassilis F, Tsanakas J. Home visits vs. telephone monitoring among patients with cystic fibrosis: which is superior? Journal of Cystic Fibrosis 2017;16 Suppl 1:S50. [CENTRAL: CN-01411946] [CFGD REGISTER: MH54]
Hawkins 2002 {unpublished data only}
    1. Hawkins KS. Adherence to treatment in adolescents with cystic fibrosis. The role of illness perception and treatment beliefs. Doctorate in Clinical Psychology Thesis 2002. [www.elibrary.ru/item.asp?id=8827177] - PubMed
Hebestreit 2010 {published data only}
    1. Hebestreit H, Kieser S, Junge S, Ballmann M, Hebestreit A, Schindler C, et al. Long-term effects of a partially supervised conditioning programme in cystic fibrosis. European Respiratory Journal 2010;35(3):578-83. [CFGD REGISTER: PE145b] - PubMed
    1. Hebestreit H, Lands LC, Alarie N, Schaeff J, Karila C, Orenstein DM, et al. Effects of a partially supervised conditioning programme in cystic fibrosis: an international multi-centre randomised controlled trial (ACTIVATE-CF): study protocol. BMC Pulmonary Medicine 2018;18(1):31. [CENTRAL: CN-01913839] [CFGD REGISTER: PE145a] [EMBASE: 620583585] [PMID: ] - PMC - PubMed
    1. Hebestreit H, Schmid K, Kieser S, Junge S, Ballmann M, Roth K, et al. Quality of life is associated with physical activity and fitness in cystic fibrosis. BMC Pulmonary Medicine 2014;14:26. [CFGD REGISTER: PE145c] - PMC - PubMed
    1. NCT00231686. Effects of a 6-months physical conditioning program in patients with cystic fibrosis. clinicaltrials.gov/show/NCT00231686 (first posted 04 October 2005). [CFGD REGISTER: PE145d]
Hebestreit 2022 {published data only}
    1. Hebestreit H, Kriemler S, Schindler C, Stein L, Karila C, Urquhart DS, et al. Effects of a partially supervised conditioning program in cystic fibrosis: an international multi-centre, randomised controlled trial (ACTIVATE-CF). Journal of Cystic Fibrosis 2021;20 Suppl:S8. [CFGD REGISTER: PE244e]
    1. Hebestreit H, Kriemler S, Schindler C, Stein L, Karila C, Urquhart DS, et al. Effects of a partially supervised conditioning program in cystic fibrosis: an international multicenter, randomized controlled trial (ACTIVATE-CF). American Journal of Respiratory and Critical Care Medicine 2022;205(3):330-9. [CFGD REGISTER: PE244d] - PMC - PubMed
    1. Hebestreit H, Lands LC, Alarie N, Schaeff J, Karila C, Orenstein DM, et al. Effects of a partially supervised conditioning programme in cystic fibrosis: an international multi-centre randomised controlled trial (ACTIVATE-CF): study protocol. BMC Pulmonary Medicine 2018;18(1):31. [CFGD REGISTER: PE244b] - PMC - PubMed
    1. Hebestreit H. How to counsel patients regarding exercise and habitual physical activity? Pediatric Pulmonology 2016;51 Suppl 45:188-9. [ABSTRACT NO.: S20.4] [CENTRAL: CN-01212584] [CFGD REGISTER: PE244a] [EMBASE: 612358802]
    1. NCT01744561. Effects of a partially supervised conditioning program in CF: an international multi-centre, randomized controlled trial. clinicaltrials.gov/show/nct01744561 (first posted 06 December 2012). [CENTRAL: CN-01343886] [CFGD REGISTER: PE244c]
Hlela 2018 {published data only}
    1. Hlela H, Carpenter K, Brodecki D, Chen-Lim M. Creating developmentally appropriate literature for children with cystic fibrosis. Pediatric Pulmonology 2018;53 Suppl 2:435-6. [CENTRAL: CN-01738598] [CFGD REGISTER: PC15] [EMBASE: 624049579]
Huang 2014 {published data only}
    1. Huang JS, Terrones L, Tompane T, Dillon L, Pian M, Gottschalk M, et al. Preparing adolescents with chronic disease for transition to adult care: a technology program. Pediatrics 2014;133(6):e1639-46. [CENTRAL: CN-01053774] [CFGD REGISTER: MH35b] [EMBASE: 2014382824] [PMID: ] - PMC - PubMed
    1. NCT01253733. TAHLC - Texting to promote Adolescent Health Liaisons and Chronic disease management. clinicaltrials.gov/show/nct01253733 (first posted 03 December 2010). [CENTRAL: CN-01412376] [CFGD REGISTER: MH35a]
Jackson 2017 {published data only}
    1. Jackson A, Kirwan L, Jeleniewska P, Fletcher G, McKone E, Doyle G. Examining the effect of CF view: an electronic registry patient portal for people with cystic fibrosis in Europe. Pediatric Pulmonology 2017;52 Suppl 47:424. [CENTRAL: CN-01430893] [CFGD REGISTER: MH58] [EMBASE: 619069167]
Landau 2021 {published data only}
    1. Landau E, Mussaffi H, Kalamaro V, Quittner A, Taizi T, Kadosh D, et al. Efficacy of problem-solving intervention to improve adherence in adolescents and adults with cystic fibrosis. Israel Medical Association Journal 2021;23(9):584-9. - PubMed
Marciel 2010 {published data only}
    1. Blackwell LS, Romero SL, Romero CV, McLean KA, Dawkins K, Hoag J, et al. CFfone: a social networking site for adolescents and young adults with CF. Pediatric Pulmonology 2012;47 Suppl 35:430. [ABSTRACT NO.: 562] [CENTRAL: CN-01004539] [CFGD REGISTER: MH33b] [EMBASE: 70892308]
    1. Marciel KK, Saiman L, Quittell LM, Dawkins K, Quittner AL. Cell phone intervention to improve adherence: cystic fibrosis care team, patient, and parent perspectives. Pediatric Pulmonology 2010;45(2):157-64. [CENTRAL: CN-00867599] [CFGD REGISTER: MH33c] [PMID: ] - PMC - PubMed
    1. McLean KA, Madan A, Monzon A, Quittner AL. Trajectories of depression and anxiety in adolescents with CF. Pediatric Pulmonology 2014;49 Suppl 38:442. [ABSTRACT NO.: 611] [CENTRAL: CN-01008988] [CFGD REGISTER: MH33g] [EMBASE: 71616663]
    1. NCT01183286. CFfone: a cell phone support program for adolescents with cystic fibrosis. clinicaltrials.gov/show/NCT01183286 (first received 17 August 2010).
    1. Quittner AL, Romero SL, Blackwell LS, Marciel KK, Romero CV, Dawkins K, et al. Effect of CFfone on knowledge of disease management, psychological well-being, and health-related quality of life in adolescents and young adults with CF. Journal of Cystic Fibrosis 2012;11 Suppl 1:S137. [ABSTRACT NO.: 313] [CENTRAL: CN-01005105] [CFGD REGISTER: MH33a] [EMBASE: 70793127]
Martinez 2017 {published data only}
    1. Martinez Rodriguez ME, Suarez Cortina L, Maiz Carro L, Ruiz-De-Valbuena M, Jimenez Cosmes L. A pulmonary rehabilitation program to increase adherence to airway clearance techniques for children and adults with cystic fibrosis. Journal of Cystic Fibrosis 2017;16 Suppl 1:S58. [CENTRAL: CN-01461867] [CFGD REGISTER: PE248] [EMBASE: 620749723]
McDonald 2007 {published data only}
    1. McDonald L, Quittner AL, Grimley ME, Botteri M, Barker DH. Effects of a clinic-based intervention on caregiving stress for parents of children with CF. Journal of Cystic Fibrosis 2007;6 Suppl 1:S75. [CENTRAL: CN-00614342] [CFGD REGISTER: MH67]
Meyers 1975 {published data only}
    1. Meyers A, Dolan TF Jr, Mueller D. Compliance and self-medication in cystic fibrosis. American Journal of Diseases of Children 1975;129:1011-3. [DOI: 10.1001/archpedi.1975.02120460005002] - DOI - PubMed
Modi 2010 {published data only}
    1. Accurso FJ, Sontag MK, Koenig JM, Quittner AL. Multi-center airway secretion clearance study in cystic fibrosis. Pediatric Pulmonology 2004;38 Suppl 27:314. [ABSTRACT NO.: 363] [CENTRAL: CN-00507886] [CFGD REGISTER: PE152a]
    1. Modi AC, Cassedy AE, Quittner AL, Accurso F, Sontag M, Koenig JM, et al. Trajectories of adherence to airway clearance therapy for patients with cystic fibrosis. Journal of Pediatric Psychology 2010;35(9):1028-37. [CENTRAL: CN-00770406] [CFGD REGISTER: PE152e] [PMID: ] - PubMed
    1. Modi AC, Sontag MK, Koenig JM, Accurso FJ, Quittner AL, Investigators and Coordinators of the Airway Secretion Clearance Study. Adherence to airway clearance therapies in patients with cystic fibrosis. Journal of Cystic Fibrosis 2006;5 Suppl 1:S97. [ABSTRACT NO.: 436] [CENTRAL: CN-00593164] [CFGD REGISTER: PE152c]
    1. Quittner AL, Modi AC, Accurso FJ, Koenig JM, Sontag MK, Oermann C, et al. Treatment satisfaction, health-related quality of life and airway clearance therapies in patients with cystic fibrosis. Pediatric Pulmonology 2004;38 Suppl 27:314. [ABSTRACT NO.: 364] [CENTRAL: CN-00507916] [CFGD REGISTER: PE152b]
    1. Sontag MK, Quittner AL, Modi AC, Koenig JM, Giles D, Oermann CM, et al. Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis. Pediatric Pulmonology 2010;45(3):291-300. [CFGD REGISTER: PE152d] [PMID: ] - PMC - PubMed
Montero‐Ruiz 2020 {published data only}
    1. Frias J P, Montero-Ruiz A, Galvez LA, Perez-Ruiz E, Huelamo MP, Martin-Montanez E. A music therapy intervention as an adjunct to chest physiotherapy in children with cystic fibrosis. European Respiratory Journal 2018;52(Suppl 62):PA4626.
    1. ISRCTN11161411. Effects of music therapy as complement of chest physiotherapy in patients with cystic fibrosis [Comparing use of specific therapeutic music AS an adjunct to chest physiotherapy versus commercial music or no music in cystic fibrosis children]. www.who.int/trialsearch/Trial2.aspx?TrialID=ISRCTN11161411 (date of registration 15 June 2019). [CENTRAL: CN-01974319] [CFGD REGISTER: PE286]
    1. Montero-Ruiz A, Fuentes LA, Perez Ruiz E, Garcia-Agua Soler N, Rius-Diaz F, Caro Aguilera P, Perez Frias J, Martin-Montanez E. Effects of music therapy as an adjunct to chest physiotherapy in children with cystic fibrosis: a randomized controlled trial. PLOS One 2020;15(10):e0241334. - PMC - PubMed
NCT00688051 {published data only}
    1. NCT00688051. Changes in adherence after playing "My Life With CF" game [Changes in adherence to diet, treatment and medication in the cystic fibrosis patient as a result of participation in a simulation game]. clinicaltrials.gov/ct2/show/NCT00688051 (first posted 02 June 2008).
NCT01025258 {published data only}
    1. NCT01025258. Improving adherence and clinical outcome in cystic fibrosis (CF) patients [Improving adherence and clinical outcomes of cystic fibrosis patients through a collaborative active intervention program of a multidisciplinary team]. clinicaltrials.gov/ct2/show/NCT01025258 (first posted 03 December 2009).
NCT02286050 {published data only}
    1. NCT02286050. Effect of a nursing program for patients with cystic fibrosis on disease management [Auswirkung Eines CF Nursing-Programms Auf Das Krankheitsmanagement Und Die Erfahrungen Der Behandlung Von Patientinnen Und Patienten]. clinicaltrials.com/trialdetails.aspx?p=NCT02286050 (first posted 27 October 2014).
NCT02501369 {published data only}
    1. NCT02501369. Investigating the use of a positive parenting programme to improve treatment adherence in cystic fibrosis. clinicaltrials.gov/ct2/show/NCT02501369 (first posted 17 July 2015).
NCT02906826 {published data only}
    1. NCT02906826. Adherence to airway clearance: novel approaches to improving adherence. clinicaltrials.gov/ct2/show/NCT02906826 (first posted 20 September 2016).
NCT03052231 {published data only}
    1. NCT03052231. Interactive mobile health information to enhance patient care at a cystic fibrosis center (MOMMI3CFCM) [Evaluating the feasibility of interactive mobile health information to enhance patient care at a cystic fibrosis center]. clinicaltrials.gov/ct2/show/NCT03052231 (first posted 14 February 2017).
NCT03226795 {published data only}
    1. NCT03226795. Improving therapeutic adherence with a co-constructed program involving both patients and health care professionals [Improving therapeutic adherence of adult patients with cystic fibrosis: impact of a co-constructed program by patients and health caregivers]. clinicaltrials.gov/ct2/show/NCT03226795 (first posted 24 July 2017).
NCT03304028 {published data only}
    1. NCT03304028. Use of home-based connected devices to early detect and treat pulmonary exacerbations: feasibility and clinical validity in CF patients [MUCOviscidose EXacerbation Outils Connectés Education Thérapeutique (MucoExocet)]. clinicaltrials.gov/ct2/show/NCT03304028 (first posted 06 October 2017).
NCT03518697 {published data only}
    1. NCT03518697. Effects of an individualized exercise program on health-related and skill/performance-related fitness in CF [Effects of an individualized and partially supervised exercise program on parameters of physical fitness, lung function, habitual physical activity in daily life and quality of life in children, adolescents and adults with cystic fibrosis]. clinicaltrials.gov/ct2/show/NCT03518697 (first posted 8 May 2018).
NCT03938324 {published data only}
    1. NCT03938324. Peer i-Coaching for Activated Self-management Optimization in adolescents and young adults with chronic conditions (PiCASO). clinicaltrials.gov/ct2/show/NCT03938324 (first received 6 May 2019).
NCT03992027 {published data only}
    1. NCT03992027. Preventing depression and anxiety: A randomized controlled trial of a CF-specific CBT intervention. clinicaltrials.gov/ct2/show/NCT03992027 (first received 19 June 2019).
NCT04017559 {published data only}
    1. NCT04017559. Clinical impact of MI on inhaled antibiotic adherence in cystic fibrosis [Assessing the clinical impact of Motivational Interviewing on inhaled antibiotic adherence in cystic fibrosis]. clinicaltrials.gov/ct2/show/NCT04017559 (first posted 12 July 2019).
NCT04096664 {published data only}
    1. NCT04096664. Home airway clearance in CF patients (HomeCareSIMEOX) [Efficiency and acceptability of SIMEOX® used autonomously at home for bronchial clearance in patients with cystic fibrosis: prospective, randomized controlled study]. clinicaltrials.gov/ct2/show/study/NCT04096664 (first posted 20 September 2019).
NCT04217889 {published data only}
    1. NCT04217889. Cystic Fibrosis Adherence Study KINOBS [Study of the adherence to chest physiotherapy in patients with cystic fibrosis (CF) at the adult CF center in Lyon]. trialsearch.who.int/Trial2.aspx?TrialID=NCT04217889 (date of registration 2 January 2020).
    1. NCT04217889. Study of the adherence to chest physiotherapy in patients with cystic fibrosis (CF) at the adult CF center in Lyon [Cystic Fibrosis Adherence Study (KINOBS)]. clinicaltrials.gov/ct2/show/NCT04217889 (first posted 6 January 2020).
NCT04453358 {published data only}
    1. NCT04453358. Cystic fibrosis remote monitoring system [Cystic fibrosis remote monitoring system to support resource-limited communities]. clinicaltrials.gov/ct2/show/NCT04453358 (first posted 01 July 2020).
NCT04696484 {published data only}
    1. NCT04696484. Feasibility of a goal-based agenda setting intervention [Feasibility of a goal-based agenda setting intervention for informing conversations in adult cystic fibrosis care]. clinicaltrials.gov/ct2/show/NCT04696484 (first posted 6 January 2021).
    1. NCT04696484. Feasibility of a goal-based agenda setting intervention [Feasibility of a goal-based agenda setting intervention for informing conversations in adult cystic fibrosis care]. trialsearch.who.int/Trial2.aspx?TrialID=NCT04696484 (date of registration 4 January 2021).
O'Hayer 2021 {published data only}
    1. O'Hayer CF, Edouard G, Bennett DS, Taylor DN, Hem Lee MA, Wolfe WM, et al. ACT via telehealth: acceptance-based behavioral therapy for anxiety and depression among individuals with cystic fibrosis. Pediatric Pulmonology 2017;52 Suppl 47:476. [CENTRAL: CN-01449303] [CFGD REGISTER: MH63]
    1. O’Hayer CV, O’Loughlin CM, Nurse CN, Smith PJ, Stephen MJ. ACT with CF: a telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis. Journal of Cystic Fibrosis 2021;20:133-9. [CFGD REGISTER: MH177b] [DOI: 10.1016/j.jcf.2020.11.013] - DOI - PubMed
Ohn 2018 {published data only}
    1. Ohn M, Fitzgerald DA. Question 12: What do you consider when discussing treatment adherence in patients with cystic fibrosis? Paediatric Respiratory Reviews 2018;25:33-6. [DOI: 10.1016/j.prrv.2017.04.002] - DOI - PubMed
Parkins 2008 {published data only}
    1. Parkins VM, Parkins MD, Peets AD, Montgomery M, Brager NP, Rabin HR. Effect of an educational intervention designed to promote cystic fibrosis knowledge in specialty residents. Pediatric Pulmonology 2008;43 Suppl 31:462. [ABSTRACT NO.: 713] [CENTRAL: CN-00867600] [CFGD REGISTER: MH32]
Pendleton 2000 {published data only}
    1. Pendleton DA, David TJ. The compliance conundrum in cystic fibrosis. Journal of the Royal Society of Medicine 2000;93(38):9-13. - PMC - PubMed
Petzel 1991 {published data only}
    1. Petzel SV, Finkelstein S, Budd J, Ellis LB. Adherence in CF. Pediatric Pulmonology 1991;11 Suppl 6:308. [CENTRAL: CN-00291510] [CFGD REGISTER: MH2]
Polineni 2017 {published data only}
    1. Duncan C, Muther E, Lindwall J, Riekert K, Goodman A, Psoter K, Polineni D. Protocol for a feasibility and acceptability pilot trial of a tele-coaching intervention to promote adherence in adolescents and young adults with cystic fibrosis. Journal of Cystic Fibrosis 2021;20 Suppl:S141-2. [CFGD REGISTER: MH57b]
    1. NCT03921229. Tele-coaching intervention to improve treatment adherence in cystic fibrosis [Feasibility, acceptability, and pilot randomized controlled trial of a tele-coaching intervention to improve treatment adherence in cystic fibrosis]. clinicaltrials.gov/ct2/show/NCT03921229 (first posted 19 April 2019).
    1. Polineni D, Chen GJ, Lindwall J, Mitchell M, Muther E, Prickett M, et al. A stakeholder-informed feasibility study of tele-coaching to improve treatment adherence in patients with cystic fibrosis. Pediatric Pulmonology 2017;52 Suppl 47:479. [CENTRAL: CN-01430913] [CFGD REGISTER: MH57a] [EMBASE: 619068564]
    1. Polineni D, Lindwall J, Muther E, Durkin K, Ahrabi-Nejad C, Ruvalcaba E, et al. Development of a pilot trial of a novel tele-coaching intervention to improve treatment adherence in cystic fibrosis. Journal of Cystic Fibrosis 2020;19 Suppl 2:P342.
Quittner 1998 {published data only}
    1. Cruz I, Snell C, Barker DH, Quittner AL, Drotar D. Effects of behavioral-family systems therapy on family conflict and communication in adolescents with CF and their parents. Pediatric Pulmonology 2007;42 Suppl 30:403-4. [CENTRAL: CN-00643113] [CFGD REGISTER: MH22b]
    1. DeLambo KE, Ievers-Landis CE, Drotar D, Quittner AL. Association of observed family relationship quality and problem-solving skills with treatment adherence in older children and adolescents with cystic fibrosis. Journal of Pediatric Psychology 2004;29(5):343-53. [CENTRAL: CN-00875650] [CFGD REGISTER: MH22a] [PMID: ] - PubMed
    1. Quittner AL, Drotar D, Ievers C, Seidner D, Slocum N, Jacobsen J, et al. Interventions to increase adolescent adherence to treatment: preliminary comparisons of family therapy and CF family education. Pediatric Pulmonology 1998;26(17):113-4. [CENTRAL: CN-00291524] [CFGD REGISTER: MH22c]
    1. Romero SL, Blackwell LS, Armenteros E, Quittner AL. Patient and parent-reported conflict: age and gender differences and associations with disease severity. Pediatric Pulmonology 2010;45 Suppl 33:446. [ABSTRACT NO.: 623] [CENTRAL: CN-00867923] [CFGD REGISTER: MH22d]
Rode 2008 {unpublished data only}
    1. Rode C. Adherence and quality of life variations among cystic fibrosis patients [Eléments de variation de l’observance et de la qualité de vie des patients atteints de mucoviscidose]. www.opengrey.eu/item/display/10068/813394 (accessed prior to 29 June 2022).
Ruddy 2015 {published data only}
    1. NCT01325766. Study of yoga as a therapy for cystic fibrosis (CF) patients [Yoga as a therapy for adolescents and young adults with cystic fibrosis: a pilot study]. clinicaltrials.gov/ct2/show/NCT01325766 (first posted 30 March 2011).
    1. Ruddy J, Emerson J, McNamara S, Genatossio A, Breuner C, Weber T, Rosenfeld M. Yoga as a therapy for adolescents and young adults with cystic fibrosis: a pilot study. Global Advances in Health and Medicine 2015;4(6):32-6. [DOI: 10.7453/gahmj.2015.061] - DOI - PMC - PubMed
Sadprasid 2021 {published data only}
    1. Sadprasid B, Eddy E, Tabor A, Scheme E, Bateman S. Percussion Hero: a chest physical therapy game for people with cystic fibrosis and their caregivers. In: Annual Symposium on Computer-Human Interaction in Play (CHI PLAY '21); 2021; Association for Computing Machinery, New York, NY, USA. 2021:361-7. [DOI: 10.1145/3450337.3483508] - DOI
Schandevyl 2021 {published data only}
    1. Van Schandevyl G, Casimir G, Hanssens L. A medication adherence–enhancing simulation intervention in pediatric cystic fibrosis. Journal of Child Health Care 2022;26(2):275-89. [DOI: 10.1177/13674935211013924] - DOI - PubMed
Strawhacker 2004 {published data only}
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Trapp 1998 {published data only}
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Viviani 2006 {published data only}
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Wood 2020 {published data only}
    1. Hoo ZH, Wildman MJ. Regarding the article entitled "A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of exacerbations: results of a randomised controlled trial". Journal of Cystic Fibrosis 2020;19(2):e9. [CFGD REGISTER: MH68f] - PubMed
    1. Wood J, Jenkins S, Putrino D, Mulrennan S, Morey S, Cecins N, et al. A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of exacerbations: results of a randomised controlled trial. Journal of Cystic Fibrosis 2020;19(2):271-6. [CFGD REGISTER: MH68e] - PubMed
    1. Wood J, Jenkins S, Putrino D, Mulrennan S, Morey S, Cecins N, et al. A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of respiratory exacerbations. Respirology 2019;24:23. [CFGD REGISTER: MH68d] - PubMed
    1. Wood J, Jenkins S, Putrino D, Mulrennan S, Morey S, Cecins N, et al. A smartphone application for reporting symptoms in adults with cystic fibrosis: a randomised controlled trial. Journal of Cystic Fibrosis 2018;17 Suppl 3:S22-3. [CENTRAL: CN-01609040] [CFGD REGISTER: MH68b] [EMBASE: 622930894]
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References to ongoing studies

Jirasek 2022 {published data only}
    1. Jirasek M, Homola L, Pleskova J, Gracova Z, Benesova K, Hodkova P, et al. "CF Hero" application as a motivational and therapeutic tool for kids and teenagers with cystic fibrosis. Journal of Cystic Fibrosis 2022;21 Suppl 1:S130. [CFGD REGISTER: MH206]
O'Hayer 2019 {published data only}
    1. NCT04114227. Acceptance and Commitment Therapy vs. Supportive Psychotherapy with cystic fibrosis patients. clinicaltrials.gov/ct2/show/NCT04114227 (first posted 3 October 2019).
    1. O'Hayer CF, Smith PJ, Drescher C, Nurse CN, Stephen MJ. ACT with CF next directions: design of a multisite, randomized controlled trial of telehealth acceptance and commitment therapy vs supportive psychotherapy for anxiety and depression. Pediatric Pulmonology 2019;54 Suppl 2:421.
Phan 2021 {published data only (unpublished sought but not used)}
    1. NCT03637504. Feasibility of a mobile medication plan application in CF patient care (MAP). clinicaltrials.gov/ct2/show/NCT03637504 (first posted 20 August 2018). [CFGD REGISTER: MH186a]
    1. Phan H, Daines C, Green A, Camick N, Goodman A, Woo T, et al. Feasibility and acceptability of a medication schedule mobile application as part of CF care: a pilot, real-world, mobile health study in CF clinics. Journal of Cystic Fibrosis 2021;20 Suppl:S140. [CFGD REGISTER: MH186b]
Thee 2021 {published data only}
    1. DRKS00024642. Coaching and telemonitoring in patients with cystic fibrosis: ConneCT CF. trialsearch.who.int/Trial2.aspx?TrialID=DRKS00024642 (date of registration 1 March 2021).
    1. Thee S, Stahl M, Fischer R, Sutharsan S, Ballman M, Müller A, et al. A multi-centre, randomized, controlled trial on coaching and telemonitoring in patients with cystic fibrosis: conneCT CF. BMC Pulmology Medicine 2021;21(1):131. [DOI: 10.1186/s12890-021-01500-y] - DOI - PMC - PubMed
White 2017 {published data only}
    1. ISRCTN37959826. Randomised trial of a web-based intervention for adherence in cystic fibrosis. trialsearch.who.int/Trial2.aspx?TrialID=ISRCTN37959826 (date of registration 11 November 2015). [CFGD REGISTER: MH59c]
    1. White H, Shaw N, Gillgrass L, Wood A, Chadwick HK, Peckham D. Evaluation of an RCT web based intervention for adherence in cystic fibrosis. Pediatric Pulmonology 2017;52 Suppl 47:503. [CENTRAL: CN-01430890] [CFGD REGISTER: MH59a] [EMBASE: 619069211]
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