First Step in Developing an ICF Core Set for Adults with Cystic Fibrosis: A Systematic Review

Abstract

Objectives: This is the first of four preparatory studies in the process of developing an International Classification of Functioning, Disability, and Health (ICF) Core Set for adults with Cystic Fibrosis (CF). The aim was to identify to what extent outcome measures in studies of adults with CF reported in the scientific literature reflect the ICF. Materials and Methods: A systematic literature review was performed on PUBMED, Scopus, and Embase, including publications from 2014 to 2024. Meaningful concepts were identified from the included studies and linked to the ICF using standardized linking rules. Results: From the 1343 publications found, 802 articles were randomly selected for abstract screening, and 143 studies met the inclusion criteria. The outcome measures in the studies were linked with body functions by focusing on “b440 Respiration functions” (86.01%). It was observed that body structures were addressed by focusing on the categories of “s4301 Lungs” (11.89%) and “s410 structure of cardiovascular system” (5.59%). It was linked with activities and participation by focusing on the categories of “d450 walking” (13.28%), “d4551 climbing”. It was linked with environmental factors by focusing on “e5800 health services” (13.28%), and “Chapter 4-attitudes’ (13.28%). Limitations in activities and participation and environmental factors were less frequently linked. Conclusion: In this systematic review we performed on CF, the focus of scientific research was on limitations in body functions. Body structures, activities and participation limitations, and environmental factors were assessed less frequently. In the next step, we will include the perspectives and experiences of experts and patients.

Keywords

• Rehabilitation
• International Classification of Functioning
• Disability and Health
• Cystic fibrosis

References

1. Aguiar, K. C. A., Marson, F. A. L., Gomez, C. C. S., Pereira, M. C., Paschoal, I. A., Ribeiro, A. F., & Ribeiro, J. D. (2017). Physical performance, quality of life and sexual satisfaction evaluation in adults with cystic fibrosis: An unexplored correlation. Revista Portuguesa de Pneumologia (2006), 23(4), 179-192. https://doi.org/10.1016/j.rppnen.2017.02.009
2. Ahmad, A., Ahmed, A., & Patrizio, P. (2013). Cystic fibrosis and fertility. Current Opinion in Obstetrics and Gynecology, 25(3), 167-172. https://doi.org/10.1097/GCO.0b013e32835f1745
3. Akyazı, N., Kemer, S. N., Dere, T., Demirhan, İ., Önal, B., & Bilgin, S. (2024). Yeniden Düzenlenmiş Kistik Fibrozis Anketinin İşlevsellik Yetiyitimi Ve Sağliğin Uluslararasi Siniflandirmasi Kapsaminda İlişkilendirme Süreci [Linking process of the revised cystic fibrosis questionnaire within the scope of the international classification of functioning, disability and health]. Türk Fizyoterapi ve Rehabilitasyon Dergisi, 35(2), 214-223. https://doi.org/10.21653/tjpr.1367384
4. Aridgides, D., Dessaint, J., Atkins, G., Carroll, J., & Ashare, A. (2021). Safety of research bronchoscopy with BAL in stable adult patients with cystic fibrosis. PLoS One, 16(1), e0245696. https://doi.org/10.1371/journal.pone.0245696
5. Barbosa, R. R. B., Liberato, F. M. G., de Freitas Coelho, P., Vidal, P. D. R., de Carvalho, R., & Donadio, M. V. F. (2020). Sleep-disordered breathing and markers of morbidity in children and adolescents with cystic fibrosis. Pediatric Pulmonology , 55(8), 1974-1983. https://doi.org/10.1002/ppul.24780
6. Blasi, F., Carnovale, V., Cimino, G., Lucidi, V., Salvatore, D., Messore, B., Bartezaghi, M., Muscianisi, E., & Porpiglia, P. A. (2018). Treatment compliance in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection treated with tobramycin inhalation powder: The FREE study. Respiratory Medicine, 138, 88-94. https://doi.org/10.1016/j.rmed.2018.03.034
7. Calella, P., Valerio, G., Thomas, M., McCabe, H., Taylor, J., Brodlie, M., & Siervo, M. (2018). Association between body composition and pulmonary function in children and young people with cystic fibrosis. Nutrition, 48, 73-76. https://doi.org/10.1016/j.nut.2017.10.026
8. Caley, L. R., Jarosz-Griffiths, H. H., Smith, L., Gale, L., Barrett, J., Kinsey, L., Davey, V., Nash, M., Jones, A. M., Whitehouse, J. L., Shimmin, D., Floto, R. A., White, H., & Peckham, D. G. (2023). Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis. Journal of Cystic Fibrosis, 22(6), 1002-1009. https://doi.org/10.1016/j.jcf.2023.06.010

9. Chin, M., De Zoysa, M., Slinger, R., Gaudet, E., Vandemheen, K. L., Chan, F., Hyde, L., Mah, T. F., Ferris, W., Mallick, R., & Aaron, S. D. (2015). Acute effects of viral respiratory tract infections on sputum bacterial density during CF pulmonary exacerbations. Journal of Cystic Fibrosis, 14(4), 482-489. https://doi.org/10.1016/j.jcf.2014.11.009
10. Choyce, J., Shaw, K. L., Sitch, A. J., Mistry, H., Whitehouse, J. L., & Nash, E. F. (2017). A prospective pilot study of home monitoring in adults with cystic fibrosis (HOME-CF): protocol for a randomised controlled trial. BMC Pulmonary Medicine, 17(1), 22. https://doi.org/10.1186/s12890-017-0366-x
11. Cieza, A., Fayed, N., Bickenbach, J., & Prodinger, B. (2019). Refinements of the ICF Linking Rules to strengthen their potential for establishing comparability of health information. Disability and Rehabilitation, 41(5), 574-583. https://doi.org/10.3109/09638288.2016.1145258
12. Collaco, J. M., Raraigh, K. S., Appel, L. J., & Cutting, G. R. (2016). Respiratory pathogens mediate the association between lung function and temperature in cystic fibrosis. Journal of Cystic Fibrosis, 15(6), 794-801. https://doi.org/10.1016/j.jcf.2016.05.012
13. Curran, M., Tierney, A. C., Button, B., Collins, L., Kennedy, L., McDonnell, C., Sheikhi, A., Jurascheck, A., Casserly, B., & Cahalan, R. (2022). Physical Activity and Sedentary Behavior in Adults With Cystic Fibrosis: Association With Aerobic Capacity, Lung Function, Sleep, Well-Being, and Quality of Life. Respiratory Care, 67(3), 339-346. https://doi.org/10.4187/respcare.09270
14. Enright, P. L. (2003). The six-minute walk test. Respiratory Care, 48(8), 783-785.
15. Hente, E., Weiland, J., Mullen, L., Hawke, J., Schuler, C. L., Filigno, S. S., & Siracusa, C. (2021). Assessment of treatment burden and complexity in cystic fibrosis: A quality improvement project. Pediatric Pulmonology , 56(7), 1992-1999. https://doi.org/10.1002/ppul.25361
16. Hommerding, P. X., Baptista, R. R., Makarewicz, G. T., Schindel, C. S., Donadio, M. V., Pinto, L. A., & Marostica, P. J. (2015). Effects of an educational intervention of physical activity for children and adolescents with cystic fibrosis: a randomized controlled trial. Respiratory Care, 60(1), 81-87. https://doi.org/10.4187/respcare.02578
17. Hommerding, P. X., Donadio, M. V., Paim, T. F., & Marostica, P. J. (2010). The Borg scale is accurate in children and adolescents older than 9 years with cystic fibrosis. Respiratory Care, 55(6), 729-733.
18. Nash, E. F., Choyce, J., Carrolan, V., Justice, E., Shaw, K. L., Sitch, A., Mistry, H., & Whitehouse, J. L. (2022). A prospective randomised controlled mixed-methods pilot study of home monitoring in adults with cystic fibrosis. Therapeutic Advances in Respiratory Disease, 16, 17534666211070133. https://doi.org/10.1177/17534666211070133
19. Popli, K., & Stewart, J. (2007). Infertility and its management in men with cystic fibrosis: review of literature and clinical practices in the UK. Human Fertility (Cambridge, England), 10(4), 217-221. https://doi.org/10.1080/14647270701510033
20. Pott, J., Krill, A., Wilkens, H., & Bals, R. (2020). Easy measurement of health related quality of life in patients with cystic fibrosis by the COPD assessment test (CAT) - A pilot study. Respiratory Medicine, 168, 105992. https://doi.org/10.1016/j.rmed.2020.105992
21. Radtke, T., Böni, L., Bohnacker, P., Maggi-Beba, M., Fischer, P., Kriemler, S., Benden, C., & Dressel, H. (2018). Acute effects of combined exercise and oscillatory positive expiratory pressure therapy on sputum properties and lung diffusing capacity in cystic fibrosis: a randomized, controlled, crossover trial. BMC Pulmonary Medicine, 18(1), 99. https://doi.org/10.1186/s12890-018-0661-1
22. Rauch, A., Cieza, A., & Stucki, G. (2008). How to apply the International Classification of Functioning, Disability and Health (ICF) for rehabilitation management in clinical practice. European Journal of Physical and Rehabilitation Medicine, 44(3), 329-342.
23. Reifenberg, J., Gecili, E., Pestian, T., Andrinopoulou, E. R., Ryan, P. H., Brokamp, C., Collaco, J. M., & Szczesniak, R. D. (2023). Lung function and secondhand smoke exposure among children with cystic fibrosis: A Bayesian meta-analysis. Journal of Cystic Fibrosis, 22(4), 694-701. https://doi.org/10.1016/j.jcf.2023.04.020
24. Reznikov, L. R. (2017). Cystic Fibrosis and the Nervous System. Chest, 151(5), 1147-1155. https://doi.org/10.1016/j.chest.2016.11.009
25. Rowe, S. M., McColley, S. A., Rietschel, E., Li, X., Bell, S. C., Konstan, M. W., Marigowda, G., Waltz, D., & Boyle, M. P. (2017). Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR. Annals of the American Thoracic Society, 14(2), 213-219. https://doi.org/10.1513/AnnalsATS.201609-689OC
26. Schmoll, A., Launois, C., Perotin, J. M., Ravoninjatovo, B., Griffon, M., Carré, S., Mulette, P., Ancel, J., Hagenburg, J., Lebargy, F., Deslée, G., Salmon, J. H., & Dury, S. (2021). Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients. Frontiers in Medicine (Lausanne), 8, 804892. https://doi.org/10.3389/fmed.2021.804892
27. Schwarz, C., Grehn, C., Temming, S., Holz, F., & Eschenhagen, P. N. (2021). Clinical impact of levofloxacin inhalation solution in cystic fibrosis patients in a real-world setting. Journal of Cystic Fibrosis, 20(6), 1035-1039. https://doi.org/10.1016/j.jcf.2021.03.002
28. Selb, M., Escorpizo, R., Kostanjsek, N., Stucki, G., Üstün, B., & Cieza, A. (2015). A guide on how to develop an International Classification of Functioning, Disability and Health Core Set. European Journal of Physical and Rehabilitation Medicine, 51(1), 105-117.
29. Shteinberg, M., Haq, I. J., Polineni, D., & Davies, J. C. (2021). Cystic fibrosis. Lancet, 397(10290), 2195-2211. https://doi.org/10.1016/s0140-6736(20)32542-3
30. Stallinga, H. A., Roodbol, P. F., Annema, C., Jansen, G. J., & Wynia, K. (2014). Functioning assessment vs. conventional medical assessment: a comparative study on health professionals' clinical decision-making and the fit with patient's own perspective of health. The Journal of Clinical Nursing, 23(7-8), 1044-1054. https://doi.org/10.1111/jocn.12266
31. Vijayasingam, A., Frost, E., Wilkins, J., Gillen, L., Premachandra, P., McLaren, K., Gilmartin, D., Picinali, L., Vidal-Diez, A., Borsci, S., Ni, M. Z., Tang, W. Y., Morris-Rosendahl, D., Harcourt, J., Elston, C., Simmonds, N. J., & Shah, A. (2020). Tablet and web-based audiometry to screen for hearing loss in adults with cystic fibrosis. Thorax, 75(8), 632-639. https://doi.org/10.1136/thoraxjnl-2019-214177
32. Welsner, M., Dietz-Terjung, S., Stehling, F., Schulte, T., Niehammer, U., Gahbiche, F. E., Taube, C., Strassburg, S., Schoebel, C., Weinreich, G., & Sutharsan, S. (2022). Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis. BMC Pulmonary Medicine, 22(1), 446. https://doi.org/10.1186/s12890-022-02243-0
33. Wildner, M., Quittan, M., Portenier, L., Wilke, S., Boldt, C., Stucki, G., Kostanjsek, N., & Grill, E. (2005). ICF Core Set for patients with cardiopulmonary conditions in early post-acute rehabilitation facilities. Disability and Rehabilitation, 27(7-8), 397-404. https://doi.org/10.1080/09638280400013958
34. Yen, T. H., Liou, T. H., Chang, K. H., Wu, N. N., Chou, L. C., & Chen, H. C. (2014). Systematic review of ICF core set from 2001 to 2012. Disability and Rehabilitation, 36(3), 177-184. https://doi.org/10.3109/09638288.2013.782359
35. Zettner, E. M., & Gleser, M. A. (2018). Progressive Hearing Loss among Patients with Cystic Fibrosis and Parenteral Aminoglycoside Treatment. Otolaryngology–Head and Neck Surgery, 159(5), 887-894. https://doi.org/10.1177/0194599818782444