Strategies to Optimize Treatment Adherence in Adolescent Patients with Cystic Fibrosis

Overview

Journal Adolesc Health Med Ther

Publisher Dove Medical Press

Date 2016 Nov 2

PMID 27799838

Citations 21

Authors

Lara C Bishay

Gregory S Sawicki

Affiliations

Soon will be listed here.

Abstract

While development of new treatments for cystic fibrosis (CF) has led to a significant improvement in survival age, routine daily treatment for CF is complex, burdensome, and time intensive. Adolescence is a period of decline in pulmonary function in CF, and is also a time when adherence to prescribed treatment plans for CF tends to decrease. Challenges to adherence in adolescents with CF include decreased parental involvement, time management and significant treatment burden, and adolescent perceptions of the necessity and value of the treatments prescribed. Studies of interventions to improve adherence are limited and focus on education, without significant evidence of success. Smaller studies on behavioral techniques do not focus on adolescents. Other challenges for improving adherence in adolescents with CF include infection control practices limiting in-person interactions. This review focuses on the existing evidence base on adherence intervention in adolescents with CF. Future directions for efforts to optimize treatment adherence in adolescents with CF include reducing treatment burden, developing patient-driven technology to improve tracking, communication, and online support, and rethinking the CF health services model to include assessment of individualized adherence barriers.

Citing Articles

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Cross-Cutting mHealth Behavior Change Techniques to Support Treatment Adherence and Self-Management of Complex Medical Conditions: Systematic Review.

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Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor-tezacaftor-ivacaftor combination: a 1-year observational study.

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Telemedicine and Its Application in Cystic Fibrosis.

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References

Majeed-Ariss R, Baildam E, Campbell M, Chieng A, Fallon D, Hall A . Apps and Adolescents: A Systematic Review of Adolescents' Use of Mobile Phone and Tablet Apps That Support Personal Management of Their Chronic or Long-Term Physical Conditions. J Med Internet Res. 2015; 17(12):e287. PMC: 4704897. DOI: 10.2196/jmir.5043. View

Smaldone A, Findley S, Bakken S, Matiz L, Rosenthal S, Jia H . Study protocol for a randomized controlled trial to assess the feasibility of an open label intervention to improve hydroxyurea adherence in youth with sickle cell disease. Contemp Clin Trials. 2016; 49:134-42. PMC: 5024731. DOI: 10.1016/j.cct.2016.06.004. View

Burrows J, Bunting J, Masel P, Bell S . Nebulised dornase alpha: adherence in adults with cystic fibrosis. J Cyst Fibros. 2004; 1(4):255-9. DOI: 10.1016/s1569-1993(02)00095-4. View

Kahana S, Drotar D, Frazier T . Meta-analysis of psychological interventions to promote adherence to treatment in pediatric chronic health conditions. J Pediatr Psychol. 2008; 33(6):590-611. DOI: 10.1093/jpepsy/jsm128. View

Costello R, Foster J, Grigg J, Eakin M, Canonica W, Yunus F . The Seven Stages of Man: The Role of Developmental Stage on Medication Adherence in Respiratory Diseases. J Allergy Clin Immunol Pract. 2016; 4(5):813-20. DOI: 10.1016/j.jaip.2016.04.002. View

Schneiderman-Walker J, Pollock S, Corey M, Wilkes D, Canny G, Pedder L . A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J Pediatr. 2000; 136(3):304-10. DOI: 10.1067/mpd.2000.103408. View

Govan J, Brown P, Maddison J, Doherty C, Nelson J, Dodd M . Evidence for transmission of Pseudomonas cepacia by social contact in cystic fibrosis. Lancet. 1993; 342(8862):15-9. DOI: 10.1016/0140-6736(93)91881-l. View

Butcher J, Nasr S . Direct observation of respiratory treatments in cystic fibrosis: parent-child interactions relate to medical regimen adherence. J Pediatr Psychol. 2014; 40(1):8-17. DOI: 10.1093/jpepsy/jsu074. View

Duff A, Latchford G . Motivational interviewing for adherence problems in cystic fibrosis. Pediatr Pulmonol. 2010; 45(3):211-20. DOI: 10.1002/ppul.21103. View

10.

Kriemler S, Kieser S, Junge S, Ballmann M, Hebestreit A, Schindler C . Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibros. 2013; 12(6):714-20. DOI: 10.1016/j.jcf.2013.03.003. View

11.

McPhail G, Acton J, Fenchel M, Amin R, Seid M . Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use. J Pediatr. 2008; 153(6):752-7. DOI: 10.1016/j.jpeds.2008.07.011. View

12.

Riekert K, Eakin M, Bilderback A, Ridge A, Marshall B . Opportunities for cystic fibrosis care teams to support treatment adherence. J Cyst Fibros. 2014; 14(1):142-8. DOI: 10.1016/j.jcf.2014.10.003. View

13.

Goldbeck L, Fidika A, Herle M, Quittner A . Psychological interventions for individuals with cystic fibrosis and their families. Cochrane Database Syst Rev. 2014; (6):CD003148. PMC: 7388585. DOI: 10.1002/14651858.CD003148.pub3. View

14.

Bucks R, Hawkins K, Skinner T, Horn S, Seddon P, Horne R . Adherence to treatment in adolescents with cystic fibrosis: the role of illness perceptions and treatment beliefs. J Pediatr Psychol. 2009; 34(8):893-902. DOI: 10.1093/jpepsy/jsn135. View

15.

Johnson K, Ravert R, Everton A . Hopkins Teen Central: Assessment of an internet-based support system for children with cystic fibrosis. Pediatrics. 2001; 107(2):E24. DOI: 10.1542/peds.107.2.e24. View

16.

Hilliard M, Hahn A, Ridge A, Eakin M, Riekert K . User Preferences and Design Recommendations for an mHealth App to Promote Cystic Fibrosis Self-Management. JMIR Mhealth Uhealth. 2014; 2(4):e44. PMC: 4259916. DOI: 10.2196/mhealth.3599. View

17.

Eakin M, Bilderback A, Boyle M, Mogayzel P, Riekert K . Longitudinal association between medication adherence and lung health in people with cystic fibrosis. J Cyst Fibros. 2011; 10(4):258-64. PMC: 3114200. DOI: 10.1016/j.jcf.2011.03.005. View

18.

Conway S, Pond M, Hamnett T, Watson A . Compliance with treatment in adult patients with cystic fibrosis. Thorax. 1996; 51(1):29-33. PMC: 472795. DOI: 10.1136/thx.51.1.29. View

19.

Bernard R, Cohen L . Increasing adherence to cystic fibrosis treatment: a systematic review of behavioral techniques. Pediatr Pulmonol. 2003; 37(1):8-16. DOI: 10.1002/ppul.10397. View

20.

Sawicki G, Heller K, Demars N, Robinson W . Motivating adherence among adolescents with cystic fibrosis: youth and parent perspectives. Pediatr Pulmonol. 2014; 50(2):127-36. PMC: 4160425. DOI: 10.1002/ppul.23017. View