KALITSAL METABOLİK HASTALIĞI OLAN ÇOCUĞA SAHİP EBEVEYNLERİN YAŞAM KALİTESİ, BAKIM YÜKÜ VE ETKİLEYEN FAKTÖRLERİN BELİRLENMESİ

Yıl 2024, Cilt: 9 Sayı: 2, 24 - 36, 30.08.2024

Fatma Aslan , Hülya Karataş

https://doi.org/10.52881/gsbdergi.1387492

Öz

Giriş: Kalıtsal metabolik hastalık tanısı olan çocukların ebeveynleri sağlıklı çocukların ebeveynlerine kıyasla birçok yönden güçlükler yaşamaktadır. Amaç: Bu çalışmanın amacı kalıtsal metabolik hastalık tanısı almış çocukların ebeveynlerinin yaşam kalitesini, bakım yükünü ve etkileyen faktörleri belirlemektir. Yöntem: Araştırmanın örneklemini bir hastanenin çocuk metabolizma kliniğine başvuran, kalıtsal metabolik hastalık tanısı olan 121 çocuğun ebeveyni oluşturmuştur. Örneklem sayısı güç analizi ile belirlenmiştir. Veriler Tanımlayıcı Özellikler Soru Formu, Yaşam Kalitesi Ölçeği ve Bakım Verme Yükü Ölçeği kullanılarak elde edilmiş, IBM SPSS V23 ile analiz edilmiştir. Normal dağılıma uygunluk Kolmogorov-Smirnov ve Shapiro-Wilk testleriyle yapılmıştır. Demografik özelliklere göre ölçek puanlarının karşılaştırılmasında Tek yönlü MANOVA kullanılmış ve çoklu karşılaştırmalar Duncan testiyle gerçekleştirilmiştir. Analiz sonuçları nicel veriler için ortalama ± s. sapma, önem düzeyi p<0,05 olarak alınmıştır. Bulgular: Ebeveynlerin bakım yükü puan ortalaması 37.6±13.1 bulunmuştur. Yaşam kalitesi ölçeği alt boyut puanları; genel sağlık durumu ortalama değeri 43,6 ± 18, fiziksel sağlık ortalama değeri 42,7 ± 12,7, psikolojik ortalama değeri 51,3 ± 15, sosyal ilişkiler ortalama değeri 47,5 ± 19,9, çevre ortalama değeri 46,9 ± 16,4 olarak bulunmuştur. Sonuç: Bakım veren ebeveynlerin yaşam kalitesinin, fiziksel sağlık başta olmak üzere birçok alanda etkilendiği ve bakım yükünün ise hafif/orta derecede olduğu tespit edilmiştir.

Anahtar Kelimeler

Kalıtsal metabolik bozukluk, ebeveyn, yaşam kalitesi, bakım yükü

Etik Beyan

Çalışma için etik izin alınmış dosyalara eklenmiştir

Destekleyen Kurum

Herhangi bir kurum tarafından desteklenmemiştir

QUALITY OF LIFE, CAREGIVER BURDEN AND ASSOCIATED FACTORS WITH REGARD TO PARENTS OF CHILDREN WITH INHERITED METABOLIC DISORDERS

Öz

Background: Parents of children with inherited metabolic diseases have many difficulties compared to parents of healthy children. Aim: The aim of this study is to determine quality of life, caregiver burden and associated factors with regard to parents of children with inherited metabolic disorders. Method: The par-ents of 121 children diagnosed with inherited metabolic disorders who were admitted to the pediatric meta-bolic clinics of the university hospital constituted the sample of the study. The sample of the study was determined by Power Analysis. Data were collected using Quality-of-Life Scale and Caregiver Burden Inventory. Data were statistically analyzed using IBM SPSS V23. Kolmogorov-Smirnov and Shapiro-Wilk tests were used to evaluate the suitability of the data for normal distribution. One-way MANOVA was used to compare the scores obtained from the scale in terms of demographic characteristics and significance levels for the differences between any pair of means were evaluated using the Duncan's Multiple Range Test. The significance level of mean ± sd for the quantitative data obtained in the analysis was considered as p<0,05. Results: The mean score of parents in the Caregiver Burden Inventory was found to be 37,6±13,1. Sub-dimension scores of the Quality-of-Life Scale, in this study, were found as follows: mean value of overall health status was 43,6±18, physical health status was 42,7±12,7, psychological health status was 51,3±15, social relations was 47,5±19,9 and environment was 46,9±16,4. Conclusion: It was concluded that quality of life of caring parents is affected in many dimensions, particularly physical health status, and the caregiver burden is mild/moderate.

Anahtar Kelimeler

Inherited metabolic disorders, parent, quality of life, caregiver burden.

Kaynakça

• 1. Carlos F R, et al. An international classification of inherited metabolic disorders (ICIMD). Journal of inherited metabolic disease. 2021;44(1):164-177.
• 2. Ramoser G, et al. 100 years of inherited metabolic disorders in Austria—A national registry of minimal birth prevalence, diagnosis, and clinical outcome of inborn errors of metabolism in Austria between 1921 and 2021. Journal of Inherited Metabolic Disease. 2022;45(2):144-156.
• 3. Hauser S I, et al. Trends and outcomes of children, adolescents, and adults hospitalized with inherited metabolic disorders: A population‐based cohort study. JIMD reports. 2022;63(6):581-592.
• 4. Saudubray J M, Garcia-Cazorla À. Inborn errors of metabolism overview: pathophysiology, manifestations, evaluation, and management. Pediatric Clinics. 2018;65(2):179-208.
• 5. Hoell J I, Warfsmann J, Distelmaier F, Borkhardt A, Janßen G, Kuhlen M. Challenges of palliative care in children with inborn metabolic diseases. Orphanet journal of rare diseases. 2018;13(1):1-7.
• 6. Cederbaum S, LeMons C, Batshaw M L. Alternative pathway or diversion therapy for urea cycle disorders now and in the future. Mol. Genet. Metab. 2010;100(3).
• 7. Yamaguchi K, Wakimizu R, Kubota M. Difficulties in daily life and associated factors, and QoL of children with inherited metabolic disease and their parents in Japan: a literature review. JIMD Reports, 2016;Volume 33: 1-10.
• 8. Hatzmann J, Valstar M J, Bosch A M, Wijburg F A, Heymans, H S, Grootenhuis M A. Predicting health‐related quality of life of parents of children with inherited metabolic disorders. Acta Paediatrica. 2009;98(7):1205-1210.
• 9. Brown A, Crowe L, Boneh A, Anderson V. Parent coping and the behavioural and social outcomes of children diagnosed with inherited metabolic disorders. In JIMD Reports, 2016; Volume 31: 29-36.
• 10. Mlčoch T, Puda R, Ješina P, Lhotáková M, Štěrbová Š, Doležal T. Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic disorders. European journal of clinical nutrition. 2018; 72(1): 87-92.
• 11. Senger B A, Ward L D, Barbosa-Leiker C, Bindler R C. Stress and coping of parents caring for a child with mitochondrial disease. Applied Nursing Research. 2016;29: 195-201.
• 12. Siddiq S, Wilson B J, Graham I D, Lamoureux M, Khangura S D, Tingley K, Potter B K. Experiences of caregivers of children with inherited metabolic disorders: a qualitative study. Orphanet Journal of Rare Diseases. 2016;11(1):1-10.
• 13. Kim K R, Lee E, Namkoong K, Lee Y M, Lee J S, Kim H D. Caregiver's burden and quality of life in mitochondrial disease. Pediatric neurology. 2010; 42(4): 271-276.
• 14. Eser E, Fidaner H, Fidaner C, Eser S Y, Elbi H, Göker E. Psychometric Properties of WHOQOL-100 and WHOQOL-BREF. Journal of Psychiatry Psychology Psychopharmagology. 1999;7(2):23-40.
• 15. İnci F, Erdem M. The Validity and Reliability of the Adaptation of the Caregiver Burden Scale into Turkish. Anatolian Journal of Nurcing and Health Sciences. 2006;11(4):85-95.
• 16. Thomas D S, Shakman L M W, Saraswathy K, Arulappan J. Parenting a child with metabolic diseases: Impact on health related quality of life of parents. Diabetes & Metabolic Syndrome: Clinical Research & Reviews. 2017;11(1):25-29.
• 17. Turan M, Yayan E H. Examining the Effect of Care Burden of Parents whose Child has a Metabolic Problem on the Perceived Social Support and Life Quality. Acıbadem University Journal of Health Sciences. 2021:12(2):445-451.
• 18. Eminoglu T F, Soysal S A, Tumer L, Okur I, Hasanoglu A. Quality of life in children treated with restrictive diet for inherited metabolic disease. Pediatrics International. 2013;55(4):428-433.
• 19. Fabre A, Baumstarck K, Cano A, Loundou A, Berbis J, Chabrol B, Auquier P. Assessment of quality of life of the children and parents affected by inborn errors of metabolism with restricted diet: preliminary results of a cross-sectional study. Health and quality of life outcomes. 2013;11(1):1-8.
• 20. Arpaci T, Altay N, Toruner E K, Gunduz M. Symptoms and Problems in Children with Inherited Metabolic Disorders and Factors Affecting the Caregiver Burden of Mothers. Comprehensive Child and Adolescent Nursing. 2020;43(4):363-377.
• 21. Bozyer H E. The Effect of Neurodegeneretion Precence on Care Burden in Children with Congenital Metabolic Diseases [unpublished dissertation]. Dr. Behçet Uz Pediatric Diseases and Surgery Health Application and Research Center. Health Sciences University; 2019.