EVALUATION OF NUTRITION AND TREATMENT APPROACHES IN CYSTIC FIBROSIS DISEASE WITH STATISTICAL ANALYSES

Öz

Cystic fibrosis (CF) is a complex genetic disorder with severe effects on the respiratory system and nutritional status, negatively affecting patients' quality of life and health outcomes. This study aims to evaluate the nutritional status and treatment approaches of children aged 7-17 years diagnosed with CF at Tripoli University Hospital in Libya between March 2024 and May 2024. Within the scope of the study, it was determined that 48.5% of the patients were between the ages of 14-17 years and 56.8% were male. 34.9% of the participants were underweight and their body mass index (BMI) was below 18.5 kg/m². In lung function assessments, 57.4% of the patients had a forced expiratory volume in the first second (FEV1) between 70-90% and mild obstruction. The mean scores of Short Form Sickness Perception Scale (SF-SPS) and Modified Wisconsin Cystic Fibrosis Quality of Life Questionnaire (MWCFC-Q) were 20.94 and 21.20, respectively. The findings revealed that low BMI was significantly associated with decreases in lung function and quality of life.

Anahtar Kelimeler

• Cystic fibrosis
• CFTR gene
• Disease perception scale
• Genetic diseases
• Quality of life questionnaire

Kistik Fibrozis Hastalığında Beslenme ve Tedavi Yaklaşımlarının İstatistiksel Analizlerle Değerlendirilmesi

Öz

Kistik fibrozis (KF), solunum sistemi ve beslenme durumu üzerinde ciddi etkileri olan karmaşık bir genetik bozukluk olup, hastaların yaşam kalitesi ve sağlık sonuçlarını olumsuz yönde etkilemektedir. Bu çalışma, Mart 2024-Mayıs 2024 tarihleri arasında Libya'daki Trablus Üniversitesi Hastanesi'nde KF tanısı alan 7-17 yaş aralığındaki çocukların beslenme durumu ve tedavi yaklaşımlarını değerlendirmeyi amaçlamaktadır. Çalışma kapsamında, hastaların %48,5'inin 14-17 yaş aralığında ve %56,8'inin erkek olduğu belirlenmiştir. Katılımcıların %34,9'u düşük kilolu olup, vücut kitle indeksi (VKİ) 18,5 kg/m²'nin altındadır. Akciğer fonksiyonu değerlendirmelerinde, hastaların %57,4'ünde birinci saniyede zorlu ekspiratuvar volümün (FEV1) %70-90 aralığında olduğu ve hafif obstrüksiyon bulunduğu saptanmıştır. Kısa Form Hastalık Algısı Ölçeği (SF-SPS) ve Modifiye Wisconsin Kistik Fibrozis Yaşam Kalitesi Anketi (MWKFC-Q) ortalama puanları sırasıyla 20,94 ve 21,20 olarak kaydedilmiştir. Bulgular, düşük VKİ'nin akciğer fonksiyonu ve yaşam kalitesindeki azalmalarla anlamlı bir ilişkiye sahip olduğunu ortaya koymaktadır.

Anahtar Kelimeler

• : Genetik hastalıklar
• Hastalık algısı ölçeği
• Kistik fibrozis
• KFTR geni
• Yaşam kalitesi anketi

Kaynakça

1. 1. Alton EWFW, Armstrong DK, Ashby D, Bayfield KJ, Bilton D, Bloomfield EV, vd. Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial. Lancet Respir Med. Eylül 2015;3(9):684-91.
2. 2. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic Fibrosis Adult Care. Chest. Ocak 2004;125(1):1S-39S.
3. 3. Hadj Fredj S, Fattoum S, Chabchoub A, Messaoud T. First report of cystic fibrosis mutations in Libyan cystic fibrosis patients. Ann Hum Biol. Eylül 2011;38(5):561-3.
4. 4. CYSTIC, F. F. 2024 [01 Mart 2024]. Erişim adresi: https://www.KFf.org/intro-KF/newborn-screening-KF.
5. 5. Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, vd. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. Haziran 2016;35(3):557-77.
6. 6. Davies G. Does newborn screening improve early lung function in cystic fibrosis? Paediatr Respir Rev. Haziran 2022; 42:17-22.
7. 7. Ede E, Köseoğlu SZA. Kistik fibrozis hastalığında tıbbi beslenme tedavisi. J Health Sci Med. 19 Mart 2020;3(2):183-6.
8. 8. VanDevanter DR, Kahle JS, O’Sullivan AK, Sikirica S, Hodgkins PS. Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment. J Cyst Fibros. Mart 2016;15(2):147-57.

9. 9. Yürük E. KİSTİK FİBROZİS HASTALIĞI OLAN ÇOCUKLARIN BESLENME ÖZELLİKLERİ VE BÜYÜME PARAMETRELERİNİN İNCELENMESİ. tmj. 2024;9(2):42-51.
10. 10. Mailhot G, Denis M, Beauchamp‐Parent C, Jomphe V. Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges. J Hum Nutr Diet. Ekim 2023;36(5):1675-91.
11. 11. Mariotti Zani E, Grandinetti R, Cunico D, Torelli L, Fainardi V, Pisi G, vd. Nutritional Care in Children with Cystic Fibrosis. Nutrients. 17 Ocak 2023;15(3):479.
12. 12. Bailey J, Baker E, Schechter MS, Robinson KJ, Powers KE, Dasenbrook E, vd. Food insecurity screening and local food access: Contributions to nutritional outcomes among children and adults with cystic fibrosis in the United States. J Cyst Fibros. Mayıs 2024;23(3):524-31.
13. 13. Nayir Buyuksahin H, Dogru D, Gözmen O, Ozon A, Portakal O, Emiralioglu N, vd. Cystic fibrosis related bone disease in children: Can it be predicted? Clin Nutr. Eylül 2023;42(9):1631-6.
14. 14. McDonald CM, Christensen NK, Lingard C, Peet KA, Walker S. Nutrition Knowledge and Confidence Levels of Parents of Children with Cystic Fibrosis. ICAN Infant Child Adolesc Nutr. Aralık 2009;1(6):325-31.
15. 15. Bickel G, Nord M, Price C, Hamilton W, Cook J. Guide to Measuring Household Food Security, Revised 200 [06 Şubat 2025] s. 193.8. Erişim adresi: https://ageconsearch.umn.edu/record/337157
16. 16. Norrish C, Norrish M, Fass U, Al-Salmani M, Lingam GS, Clark F, vd. The Cystic Fibrosis Symptom Progression Survey (CF-SPS) in Arabic: A tool for monitoring patients’ symptoms. Oman Med J. 15 Ocak 2015;30(1):16-25.
17. 17. Tavşancıl E. Tutumların ölçülmesi ve SPSS ile veri analizi. Nobel; 2010.
18. 18. Hair JF. Multivariate data analysis. Pearson Prentice Hall; 2006.
19. 19. Harindhanavudhi T, Wang Q, Dunitz J, Moran A, Moheet A. Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: A single-center analysis. J Cyst Fibros. Ocak 2020;19(1):139-45.
20. 20. CYSTIC, FF 2019 [01 Ağustos 2019]. Erişim adresi: https://www.KFf.org/managing-KF/KFtr-modulator-therapies.
21. 21. Brown PS, Durham D, Tivis RD, Stamper S, Waldren C, Toevs SE, vd. Evaluation of Food Insecurity in Adults and Children with Cystic Fibrosis: Community Case Study. Front Public Health. 26 Kasım 2018; 6:348.
22. 22. Borowitz D, Baker RD, Stallings V. Consensus Report on Nutrition for Pediatric Patients with Cystic Fibrosis: J Pediatr Gastroenterol Nutr. Eylül 2002;35(3):246-59.
23. 23. Steinkamp G. Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax. 01 Temmuz 2002;57(7):596-601.
24. 24. Peterson ML, Jacobs DR, Milla CE. Longitudinal Changes in Growth Parameters Are Correlated with Changes in Pulmonary Function in Children with Cystic Fibrosis. Pediatrics. 01 Eylül 2003;112(3):588-92.
25. 25. Leonard A, Davis E, Rosenstein BJ, Zeitlin PL, Paranjape SM, Peeler D, vd. Description of a Standardized Nutrition Classification Plan and its Relation to Nutritional Outcomes in Children with Cystic Fibrosis. J Pediatr Psychol. 01 Ocak 2010;35(1):6-13.
26. 26. Quittner AL, Schechter MS, Rasouliyan L, Haselkorn T, Pasta DJ, Wagener JS. Impact of Socioeconomic Status, Race, and Ethnicity on Quality of Life in Patients with Cystic Fibrosis in the United States. Chest. Mart 2010;137(3):642-50.
27. 27. Barni GC, Forte GC, Forgiarini LF, Abrahão CLDO, Dalcin PDTR. Factors associated with malnutrition in adolescent and adult patients with cystic fibrosis. J Bras Pneumol. 31 Temmuz 2017;43(5):337-43.
28. 28. Isa HM, Al-Ali LF, Mohamed AM. Growth assessment and risk factors of malnutrition in children with cystic fibrosis. Saudi Med J. Mart 2016;37(3):293-8.
29. 29. GOV UK. 2015 Haz. Erişim adresi: https://www.gov.uk/government/publications/sacn-carbohydrates-and-health-report.
30. 30. Petersen MC, Begnel L, Wallendorf M, Litvin M. Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis. J Cyst Fibros. Mart 2022;21(2):265-71.
31. 31. Castellani C, Massie J, Sontag M, Southern KW. Newborn screening for cystic fibrosis. Lancet Respir Med. Ağustos 2016;4(8):653-61.