Quality of life of children with cystic fibrosis with disorders of carbohydrate metabolism

Introduction. Cystic fibrosis (CF) is a hereditary, multisystem disease that is accompanied by numerous complications, which affects the quality of life (QOL) of patients, determining its duration and comfort. With an increase in the survival rate of CF patients, various concomitant diseases were identified, one of which is cystic fibrosis-associated (dependent) diabetes mellitus (CFDM). Traditionally, it is believed that CFDM worsens the patient’s QOL. To establish this, a questionnaire is conducted with validated questionnaires for children — Health Utilities Index (HUI).

Objective: to determine the QOL in CF patients with disorders of carbohydrate metabolism.

Materials and methods. There were surveyed sixty-four CF 5 to 18 year patients (Me = 13.59 years), including 42 girls and 22 boys. To diagnose disorders of carbohydrate metabolism, a standard oral glucose tolerance test (OGTT) was performed in all patients. According to OGTT data CF patients were divided into 3 groups: 1^st — 25 cases without deteriorations of carbohydrate metabolism; 2^nd — 25 children with prediabetes and 3^rd — 14 CFDM patients on insulin therapy and without it. The assessment of the psychological state and development of CF patients with prediabetes and CFDM cases was carried out. The parameters of emotional well-being, attitude to the disease and adherence to treatment were studied.

Results. The authors revealed no significant difference in the QOL in CF patients by all scales of the questionnaire. There was a tendency to decrease in QOL according to the overall score of the questionnaire (HUI3) in CF children with prediabetes (0.77 ± 0.18 units) compared with patients without carbohydrate metabolism disorders and CFDM patients (0.81 ± 0.14 units and 0.80 ± 0.14 units, respectively). In the emotional sphere, there is a tendency to decrease in QOL in patients with prediabetes and CFDM cases. The analysis of indices on separate scales revealed a more pronounced decrease in QOL on the emotion scale in CF patients with disorders of carbohydrate metabolism. CFDM patients are in a more severe psychological state than children with prediabetes. They are significantly more likely to experience psychophysical discomfort and tend to fixate on the symptoms of the disease (Fi­sher’s angular transformation criterion 3.11, p < 0.01). These patterns should be taken into account when analyzing the psycho-emotional state of a CF child for timely prevention and correction.

Acknowledgement. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: February 14, 2022
Accepted: February 17, 2022
Published: March 15, 2022

1. Ode K.L., Moran A. New insights into cystic fibrosis-related diabetes in children. Lancet Diabetes Endocrinol. 2013; 1(1): 52–8. https://doi.org/10.1016/S2213-8587(13)70015-9

2. Smyth A.R., Bell S.C., Bojcin S., Bryon M., Duff A., Flume P., et al. European cystic fibrosis society standards of care: best practice guidelines. J. Cyst. Fibros. 2014; 13(Suppl. 1): S23-42. https://doi.org/10.1016/j.jcf.2014.03.010

3. Kapranov N.I., Kashirskaya N.Yu., Konrdrat’eva E.N., eds. Cystic Fibrosis [Mukovistsidoz]. Moscow: Medpraktika-M; 2021. (in Russian)

4. Southern K.W. Achieving respiratory excellence in pre-school children with cystic fibrosis. J. Cyst. Fibros. 2021; 20(6): 904–5. https://doi.org/10.1016/j.jcf.2021.09.016

5. Moran A., Pillay K., Becker D., Granados A., Hameed S., Acerini C.L. ISPAD clinical practice consensus guidelines 2018: Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr. Diabetes. 2018; 19(Suppl. 27): 64–74. https://doi.org/10.1111/pedi.12732

6. Kayani K., Mohammed R., Mohiaddin H. Cystic fibrosis-related diabetes. Front. Endocrinol. (Lausanne). 2018; 9: 20. https://doi.org/10.3389/fendo.2018.00020

7. Pittman J.E., Khan U., Laguna T.A., Heltshe S., Goss C.H., Sanders D.B. Rates of adverse and serious adverse events in children with cystic fibrosis. J. Cyst. Fibros. 2021; 20(6): 972–7. https://doi.org/10.1016/j.jcf.2021.02.013

8. Ode K.L., Chan C.L., Granados A., Moheet A., Moran A., Brennan A.L. Cystic fibrosis related diabetes: Medical management. J. Cyst. Fibros. 2019; 18(Suppl. 2): 10–8. https://doi.org/10.1016/j.jcf.2019.08.003

9. Iafusco F., Maione G., Rosanio F.M., Mozzillo E., Franzese A., Tinto N. Cystic Fibrosis-Related Diabetes (CFRD): overview of associated genetic factors. Diagnostics (Basel). 2021; 11(3): 572. https://doi.org/10.3390/diagnostics11030572

10. Sperling M.A., ed. Pediatric and Adolescent Diabetes. ISPAD Clinical Practice Consensus Guidelines 2014. September 2014. Wiley & Blackwell; 2014.

11. Prinz N., Wosniok J., Staab D., Ballmann M., Dopfer C., Regenfuß N., et al. Glucose tolerance in patients with cystic fibrosis – results from the German cystic fibrosis registry. Klin. Padiatr. 2020; 232(4): 210–6. https://doi.org/10.1055/a-1117-3771

12. Kasim N., Khare S., Sandouk Z., Chan C. Impaired glucose tolerance and indeterminate glycemia in cystic fibrosis. J. Clin. Transl. Endocrinol. 2021; 26: 100275. https://doi.org/10.1016/j.jcte.2021.100275

13. Granados A., Chan C.L., Ode K.L., Moheet A., Moran A., Holl R. Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis. J. Cyst. Fibros. 2019; 18(Suppl. 2): 3–9. https://doi.org/10.1016/j.jcf.2019.08.016

14. Hunt W.R., Hansen J.M., Stecenko A.A. Glucose ingestion in cystic fibrosis induces severe redox imbalance: A potential role in diabetes. J. Cyst. Fibros. 2020; 19(3): 476–82. https://doi.org/10.1016/j.jcf.2020.02.010

15. Pu M.Z., Christensen-Adad F.C., Gonçalves A.C., Minicucci W.J., Ribeiro J.D., Ribeiro A.F. Insulin therapy in patients with cystic fibrosis in the pre-diabetes stage: a systematic review. Rev. Paul. Pediatr. 2016; 34(3): 367–73. https://doi.org/10.1016/j.rpped.2015.12.010

16. Prentice B.J., Ooi C.Y., Verge C.F., Hameed S., Widger J. Glucose abnormalities detected by continuous glucose monitoring are common in young children with Cystic Fibrosis. J. Cyst. Fibros. 2020; 19(5): 700–3. https://doi.org/10.1016/j.jcf.2020.02.009

17. Sharma P.B., Sathe M., Savant A.P. Year in Review 2020: Multisystemic impact of cystic fibrosis. Pediatr. Pulmonol. 2021; 56(10): 3110–9. https://doi.org/10.1002/ppul.25584

18. da Silva Filho L.V.R.F., Zampoli M., Cohen-Cymberknoh M., Kabra S.K. Cystic fibrosis in low and middle-income countries (LMIC): A view from four different regions of the world. Paediatr. Respir. Rev. 2021; 38: 37–44. https://doi.org/10.1016/j.prrv.2020.07.004

19. Kashirskaya N.Yu., Kondrat’eva E.I., Krasovskiy S.A., Starinova M.A., Voronkova A.Yu., Amelina E.L. Register of Patients with Cystic Fibrosis in the Russian Federation. 2019 [Registr bol’nykh mukovistsidozom v Rossiyskoy Federatsii. 2019 god]. Moscow: Medpraktika-M; 2021. (in Russian)

20. Cheney J., Vidmar S., Gailer N., Wainwright C., Douglas T.A. Health-related quality-of-life in children with cystic fibrosis aged 5-years and associations with health outcomes. J. Cyst. Fibros. 2020; 19(3): 483–91. https://doi.org/10.1016/j.jcf.2020.02.022

21. Smirnov I.E., Tarasova O.V., Lukina O.F., Kustova O.V., Sorokina T.E., Simonova O.I. Structural and functional state of the lungs in cystic fibrosis in children. Rossiyskiy pediatricheskiy zhurnal. 2015; 18(2): 11–7. (in Russian)

22. Thorat T., McGarry L.J., Bonafede M.M., Limone B.L., Rubin J.L., Jariwala-Parikh K. Healthcare resource utilization and costs among children with cystic fibrosis in the United States. Pediatr. Pulmonol. 2021; 56(9): 2833–44. https://doi.org/10.1002/ppul.25535

23. Prieur M.G., Christon L.M., Mueller A., Smith B.A., Georgiopoulos A.M., Boat T.F., et al. Promoting emotional wellness in children with cystic fibrosis, Part I: Child and family resilience. Pediatr. Pulmonol. 2021; 56(Suppl. 1): 97–106. https://doi.org/10.1002/ppul.24958

24. Valero-Moreno S., Lacomba-Trejo L., Montoya-Castilla I., Pérez-Marín M. Is mHealth a useful therapy for improving physical or emotional health in adolescents with cystic fibrosis? A systematic review. Curr. Psychol. 2021; 1-14. https://doi.org/10.1007/s12144-021-02452-6

25. Horsman J., Furlong W., Feeny D., Torrance G. The Health Utilities Index (HUI): concepts, measurement properties and applications. Health Qual. Life Outcomes. 2003; 1: 54. https://doi.org/10.1186/1477-7525-1-54

26. Warren E., Morgan K., Toward T.J., Schwenkglenks M., Leadbetter J. Cost effectiveness of inhaled mannitol (Bronchitol()) in patients with cystic fibrosis. Pharmacoeconomics. 2019; 37(3): 435–46. https://doi.org/10.1007/s40273-019-00767-8

27. Terletskaya R.N., Fisenko A.P., Antonova E.V., Vinyarskaya I.V. Impact of socio-economic factors on the formation of disability in children of Russia. Rossiyskiy pediatricheskiy zhurnal. 2020; 23(6): 365–71. https://doi.org/10.18821/1560-9561-2020-23-6-365-371 (in Russian)

28. Fisenko A.P., Terletskaya R.N., Vinyarskaya I.V., Antonova E.V., Chernikov V.V., Alekseeva E.I., et al. Satisfaction of parents (legal representatives) with the quality of medical care provided to their disabled children. Rossiyskiy pediatricheskiy zhurnal. 2021; 24(2): 106–11. https://doi.org/10.46563/1560-9561-2021-24-2-106-111 (in Russian)

29. Kauser S., Keyte R., Regan A., Nash E.F., Fitch G., Mantzios M., et al. Exploring associations between self-compassion, self-criticism, mental health, and quality of life in adults with cystic fibrosis: informing future interventions. J. Clin. Psychol. Med. Settings. 2021. https://doi.org/10.1007/s10880-021-09831-y

30. McLeod C., Wood J., Tong A., Schultz A., Norman R., Smith S., et al. The measurement properties of tests and tools used in cystic fibrosis studies: a systematic review. Eur. Respir. Rev. 2021; 30(160): 200354. https://doi.org/10.1183/16000617.0354-2020

31. Vinyarskaya I.V., Terletskaya R.N., Chernikov V.V., Soboleva K.A. Development and validation of the Russian version of HUI questionnaire in pediatrics. Sotsial’nye aspekty zdorov’ya naseleniya. 2015; (2): 13. (in Russian)

32. Samsonova M.C., Simonova O.I., Vinyarskaya I.V., Torinova Yu.V., Chernikov V.V. Features of changes in the quality of life in cystic fibrosis patients against the background of its effective treatment. Rossiyskiy pediatricheskiy zhurnal. 2016; 19(2): 86–91. https://doi.org/10.18821/1560-9561-2016-19(2)-86-91 (in Russian)

33. Moran A., Pillay K., Becker D., Acerini C.L. Management of cystic fibrosis related diabetes in children and adolescents. Pediatr. Diabetes. 2014; 15(Suppl. 20): 65–76. https://doi.org/10.1111/pedi.12178

34. Morisky D.E., Green L.W., Levine D.M. Concurrent and predictive validity of a self-reported measure of medication adherence. Med. Care. 1986; 24(1): 67–74. https://doi.org/10.1097/00005650-198601000-00007

35. Mozzillo E., Franceschi R., Piona C., Passanisi S., Casertano A., Pjetraj D., et al. Diabetes and prediabetes in children with cystic fibrosis: a systematic review of the literature and recommendations of the Italian Society for Pediatric Endocrinology and Diabetes (ISPED). Front. Endocrinol. (Lausanne). 2021; 12: 673539. https://doi.org/10.3389/fendo.2021.673539

36. Forte G.C., Barni G.C., Perin C., Casarotto F.C., Fagondes S.C., Dalcin Pde T. Relationship between clinical variables and health-related quality of life in young adult subjects with cystic fibrosis. Respir. Care. 2015; 60(10): 1459–68. https://doi.org/10.4187/respcare.03665

37. Li S., Ning W., Wang W., Ziebolz D., Acharya A., Schmalz G., et al. Oral health-related quality of life in patients with chronic respiratory diseases-results of a systematic review. Front. Med. (Lausanne). 2022; 8: 757739. https://doi.org/10.3389/fmed.2021.757739

38. Ratnayake I., Ahern S., Ruseckaite R. A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis. BMJ Open. 2020; 10(10): e033867. https://doi.org/10.1136/bmjopen-2019-033867

39. Reiter J., Gileles-Hillel A., Cohen-Cymberknoh M., Rosen D., Kerem E., Gozal D., et al. Sleep disorders in cystic fibrosis: A systematic review and meta-analysis. Sleep Med. Rev. 2020; 51: 101279. https://doi.org/10.1016/j.smrv.2020.101279

40. Clemente L.M., Bilbao G.L., Moreno-Galdó A., Campos Martorrell A., Gartner Tizzano S., Yeste Fernández D., et al. Oral glucose tolerance test and continuous glucose monitoring to assess diabetes development in cystic fibrosis patients. Endocrinol. Diabetes Nutr. (Engl. Ed). 2018; 65(1): 45–51. https://doi.org/10.1016/j.endinu.2017.08.008

41. Prentice B.J., Chelliah A., Ooi C.Y., Hameed S., Verge C.F., Plush L., et al. Peak OGTT glucose is associated with lower lung function in young children with cystic fibrosis. J. Cyst. Fibros. 2020; 19(2): 305–9. https://doi.org/10.1016/j.jcf.2019.05.005

42. Chan C.L., Pyle L., Vigers T., Zeitler P.S., Nadeau K.J. The relationship between continuous glucose monitoring and OGTT in youth and young adults with cystic fibrosis. J. Clin. Endocrinol. Metab. 2022; 107(2): e548–60. https://doi.org/10.1210/clinem/dgab692

43. Yi M.S., Britto M.T., Wilmott R.W., Kotagal U.R., Eckman M.H., Nielson D.W., et al. Health values of adolescents with cystic fibrosis. J. Pediatr. 2003; 142(2): 133–40. https://doi.org/10.1067/mpd.2003.51

44. Britto M.T., Kotagal U.R., Chenier T., Tsevat J., Atherton H.D., Wilmott R.W. Differences between adolescents’ and parents’ reports of health-related quality of life in cystic fibrosis. Pediatr. Pulmonol. 2004; 37(2): 165–71. https://doi.org/10.1002/ppul.10436

45. Gojsina B., Minic P., Todorovic S., Soldatovic I., Sovtic A. Continuous glucose monitoring as a valuable tool in the early detection of diabetes related to cystic fibrosis. Front. Pediatr. 2021; 9: 659728. https://doi.org/10.3389/fped.2021.659728

46. Cronly J.A., Duff A.J., Riekert K.A., Fitzgerald A.P., Perry I.J., Lehane E.A., et al. Health-related quality of life in adolescents and adults with cystic fibrosis: physical and mental health predictors. Respir. Care. 2019; 64(4): 406–15. https://doi.org/10.4187/respcare.06356

47. Tomaszek L., Dębska G., Cepuch G., Kulpa M., Pawlik L., Broniatowska E. Evaluation of quality of life predictors in adolescents and young adults with cystic fibrosis. Heart Lung. 2019; 48(2): 159–65. https://doi.org/10.1016/j.hrtlng.2018.08.003

48. Goetz D.M., Frederick C., Savant A., Cogswell A., Fries L., Roach C., et al. Systematic depression and anxiety screening for patients and caregivers: implementation and process improvement in a cystic fibrosis clinic. BMJ Open Qual. 2021; 10(2): e001333. https://doi.org/10.1136/bmjoq-2020-001333

49. Boulay M., Ramelot L., Willems M., Bauwens N., Thimmesch M. Therapeutic education and cystic fibrosis. Rev. Med. Liege. 2021; 76(11): 794–8. (in French)