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NADİR HASTALIĞI OLAN ÇOCUKLARIN AİLELERİNDE BAKIM YÜKÜNÜN İNCELENMESİ

Year 2021, Volume: 15 Issue: 2, 269 - 277, 20.06.2021
https://doi.org/10.21763/tjfmpc.841608

Abstract

Giriş: Nadir hastalıklar, genel popülasyonda daha az sayıda görülen kronik, ilerleyici ve yaşamı tehdit edici hastalıklardır. Bu hastalıkların karmaşık doğası bakım gereksinimleri doğurmaktadır. Bu çalışma, nadir hastalığı olan çocukların ailelerindeki bakım yükünün incelenmesi amacıyla gerçekleştirilmiştir. Yöntem: Çalışmada Nadir Hastalıklar Ağı aracılığıyla kolayda örnekleme yöntemi ile nadir hastalığı olan çocuğa bakım veren 311 ebeveyne ulaşılmıştır. Veri toplama aracı olarak araştırmacılar tarafından literatür araştırmasına dayanarak hazırlanan sosyodemografik form ile Zarit bakım verme yükü ölçeği kullanılmıştır. Araştırmada elde edilen veriler, SPSS programı aracılığıyla uygun istatistiksel yöntemlerle analiz edilmiştir. Bulgular: Çalışmaya katılanların %70,7’si annelerden oluşmaktadır. Katılımcıların yaş ortalaması 35,94±9,45’tir. Katılımcıların %46,9’u bir işte çalışmaktadır ve %72,7’si bir profesyonel meslek mensubudur. Bakım alan çocukların ise %62’si kızdır ve çocukların yaş ortalaması 7,08±4,56’dır. Bakım verme süresi ortalama 8,48±7 yıldır. Çocuklarda bulunan nadir hastalıklar ise %22,5 fenilketonuri (PKU), %17,6 sistinozis, %10,2 nadir görülen kanserler, %8 Primer İmmün Yetmezlik, %7,7 Duchenne müsküler distrofisi (DMD), %6,4 mukopolisakkaridoz (MPS), %5 mesane ekstrofisi ve Alevi Akdeniz Ateşi Hastalığı (FMF), rett sendromu, kistik fibrozis, %4 spinal müsküler atrofi (SMA) ve glikojen depo hastalığıdır. Katılımcıların bakım verme yükü ölçeğinden aldığı puanların ortalaması 52,82±10,32’dir. Çalışmada bakım yükü ile ebeveynin ve çocuğun yaşı ile cinsiyeti, bakım verme süresi, ebeveynin çalışma durumu ve medeni durumu arasında istatistiksel olarak anlamlı bir fark bulunmamıştır (p>0.05). Sonuç olarak çalışma, nadir hastalığı olan çocuğa bakım veren ebeveynlerde bakım yükünün ileri derecede olduğunu göstermektedir. Sonuç: Bakım yükünün azaltılması için ailelerle tıbbi sosyal hizmet müdahalelerinin gerçekleştirilmesi önerilmektedir.

Supporting Institution

Bu kapsamda çalışma Başkent Üniversitesi Sosyal ve Beşeri Bilimler ve Sanat Etik Kurulu (Proje no: 62310886-604.01.01/) tarafından onaylanmıştır

Project Number

62310886-604.01.01

Thanks

Değerli katkılarından dolayı Prof.Dr. Tarık Tuncay'a teşekkür ederiz.

References

  • 1.Sisk R.J. Caregiver burden and health promotion. International Journal of nursing studies. 2000; 37(1): 37-43.
  • 2.Möller - Leimkühler A. M. & Wiesheu A. Caregiver burden in chronic mental illness: the role of patient and caregiver characteristics. European archives of psychiatry and clinical neuroscience. 2012; 262(2):157-166.
  • 3.Pinquart M. & Sörensen S. Gender differences in caregiver stressors social resources and health: An updated meta-analysis. The Journals of Gerontology Series B: Psychological Sciences and Social Sciences. 2006;61(1):33-45.
  • 4.Chiò A. Gauthier A. Calvo A. Ghiglione P. & Mutani R. Caregiver burden and patients’ perception of being a burden in ALS. Neurology. 2005;64(10):1780-1782.
  • 5. Haendel M. Vasilevsky N. Unni D. Bologa C. Harris N. Rehm H. ... & Dawkins H. How many rare diseases are there?. Nature Reviews Drug Discovery.2019;19(1):77-78.
  • 6. Dawkins H. J. Draghia‐Akli R. Lasko P. Lau L. P. Jonker A. H. Cutillo C. M. & Kaufmann P. Progress in rare diseases research 2010–2016: an IRDiRC perspective. Clinical and translational science.2018;11(1):11-20.
  • 7. Groft S. & Posada dela Paz M. Rare Diseases: Joining mainstream Research and Treatment Based on Reliable Epidemiologic Data. M. Posada de la Paz D. Taruscio S. Groft M. Posada de la Paz D. Taruscio & S. Groft (Dü) içinde Rare Diseases Epidemiology: Update and Overview. Cham: Springer. 2017;3-21
  • 8. Satman İ. Güdük Ö. Yemenici M. & Ertürk N. Nadir Hastalıklar Raporu. Ankara: TÜSEB (Türkiye Halk Sağlığı ve Kronik Hastalıklar Enstitüsü). 2019;1-30.
  • 9.Yavuz Çolak M. Nadir Hastalıklar Epidemiyolojisi. Ö. İnce & M. D. Pak içinde Tüm Yönleriyle Nadir Hastalıklar. Ankara: Nobel Akademik Yayıncılık. 2019;15-25.
  • 10.Vitale S. A. Parent recommendations for family functioning with Prader–Willi syndrome: a rare genetic cause of childhood obesity. Journal of pediatric nursing.2016;31(1):47-54.
  • 11.Péntek M. Gulácsi L. Brodszky V. Baji P. Boncz I. Pogány G.... & Posada-de-la-Paz M. Social/economic costs and health-related quality of life of mucopolysaccharidosis patients and their caregivers in Europe. The European Journal of Health Economics. 2016a; 17(1):89-98.
  • 12.Bruce J. E. Family Quality of Life in Families of Children with Phelan-McDermid Syndrome: A Rare Genetic Disability 2009; (Doctoral dissertation McGill University):42-59.
  • 13.Hwang M. S. Lee M. K. & Song J. R. The factors affecting burdens and quality of life of the family caregivers of patients with rare and incurable diseases using home ventilators. Korean Journal of Adult Nursing. 2014;26(2):191-202.
  • 14.Anderson M Elliott E Zurynski Y. Australian families living with rare disease: experiences of diagnosis health services use and needs for psychosocial support. Orphanet J Rare Dis. 2013.;8(1):22.
  • 15.Zarit S. H. Reever K. E. & Bach-Peterson J. Relatives of the impaired elderly: correlates of feelings of burden. The gerontologist. 1980;20(6):649-655.
  • 16.İnci F.H. & Erdem M. Bakım Verme Yükü Ölçeğinin Türkçeye Uyarlanması Geçerlilik ve Güvenilirliği. Atatürk Üniversitesi Hemşirelik Yüksekokulu Dergisi. 2008;11(4):85-95
  • 17.Cardinali P. Migliorini L. & Rania N. The caregiving experiences of fathers and mothers of children with rare diseases in Italy: challenges and social support perceptions. Frontiers in Psychology. 2019;10:1780.
  • 18.Péntek M. Herczegfalvi Á. Molnár M. J. Szőnyi L. P. Kosztolányi G. Pfliegler G. ... & Szegedi M. Dısease Burden On Duchenne Muscular Dystrophy Patıents And Theır Caregıvers. Ideggyogyaszati szemle. 2016b;69(5-6):183.
  • 19.Oh J. & Kim J. A. Factor analysis of the Zarit Burden Interview in family caregivers of patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.2018;19(1-2):50-56.
  • 20.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.
  • 21.Pangalila R.F. van den Bos G.A. Stam H.J. van Exel N.J. Brouwer W.B. Roebroeck M.E. Subjective caregiver burden of parents of adults with Duchenne muscular dystrophy. Disabil Rehabil 2012;34:988–996.
  • 22.Laurvick C. L. Msall M. E. Silburn S. et al. Physical and mental health of mothers caring for a child with Rett syndrome. Pediatrics.2006;118(4):1152–1164.
  • 23.Kahraman S. Çiftçi E. K. & Timuçin A. Determination of caregiving burden of parents providing care to their children with epidermolysis bullosa. Egyptian Journal of Dermatology and Venerology. 2017;37(1):1.
  • 24.Thomas P. T. Warrier M. G. Sadasivan A. Balasubramanium B. Preethish-Kumar V. Nashi S. ... & Nalini A. Caregiver burden and quality of life of patients with amyotrophic lateral sclerosis in India. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.2018;19(7-8):606-610.
  • 25.Kayadjanian N. Schwartz L. Farrar E. Comtois K. A. & Strong T. V. High levels of caregiver burden in Prader-Willi syndrome. PloS one. 2018;13(3) :e194-655.

EXAMINING THE CAREGIVER BURDEN IN THE FAMILIES OF CHILDREN WITH RARE DISEASES

Year 2021, Volume: 15 Issue: 2, 269 - 277, 20.06.2021
https://doi.org/10.21763/tjfmpc.841608

Abstract

Aim/Background: Rare diseases are chronic, progressive, and life-threatening diseases that are less common in the general population. The complex nature of these diseases creates care requirements. The study aims to examine the caregiver burden in families of children with rare diseases. Method: In the study, 311 parents who cared for a child with a rare disease were reached through the Rare Diseases Network. Sociodemographic form prepared by researchers based on literature research and Zarit caregiving burden scale were used as data collection tools. The data obtained in the study were analyzed with appropriate statistical methods through the SPSS program. Results: 70.7% of the participants in the study are mothers. The average age of the participants is 35.94 ± 9.45. 46.9% of the participants are working in a job and 72.7% is a professional occupation. 62% of the children who receive care are girls and the average age of the children is 7.08 ± 4.56. The average caregiving period is 8.48 ± 7 years. Rare diseases in children are 22.5% phenylketonuria (PKU), 17.6% cystinosis, 10.2% rare cancers, 8% Primary Immunodeficiency, 7.7% Duchenne muscular dystrophy (DMD), 6.4% mucopolysaccharidosis (MPS), 5% bladder exstrophy and Familial Mediterranean Fever Disease (FMF), Rett syndrome, cystic fibrosis, 4% spinal muscular atrophy (SMA) and glycogen storage disease. The average of the scores the participants got from the caregiving burden scale is 52.82 ± 10.32. In the study, no statistically significant difference was found between the care burden and the age and gender of the parents and the child, the duration of care, the working status of the parents and their marital status (p> 0.05). Conclusion: In conclusion, the study shows that the caregiver burden of parents who care for children with rare diseases is high. Medical social work interventions with families are recommended to reduce the burden of care.

Project Number

62310886-604.01.01

References

  • 1.Sisk R.J. Caregiver burden and health promotion. International Journal of nursing studies. 2000; 37(1): 37-43.
  • 2.Möller - Leimkühler A. M. & Wiesheu A. Caregiver burden in chronic mental illness: the role of patient and caregiver characteristics. European archives of psychiatry and clinical neuroscience. 2012; 262(2):157-166.
  • 3.Pinquart M. & Sörensen S. Gender differences in caregiver stressors social resources and health: An updated meta-analysis. The Journals of Gerontology Series B: Psychological Sciences and Social Sciences. 2006;61(1):33-45.
  • 4.Chiò A. Gauthier A. Calvo A. Ghiglione P. & Mutani R. Caregiver burden and patients’ perception of being a burden in ALS. Neurology. 2005;64(10):1780-1782.
  • 5. Haendel M. Vasilevsky N. Unni D. Bologa C. Harris N. Rehm H. ... & Dawkins H. How many rare diseases are there?. Nature Reviews Drug Discovery.2019;19(1):77-78.
  • 6. Dawkins H. J. Draghia‐Akli R. Lasko P. Lau L. P. Jonker A. H. Cutillo C. M. & Kaufmann P. Progress in rare diseases research 2010–2016: an IRDiRC perspective. Clinical and translational science.2018;11(1):11-20.
  • 7. Groft S. & Posada dela Paz M. Rare Diseases: Joining mainstream Research and Treatment Based on Reliable Epidemiologic Data. M. Posada de la Paz D. Taruscio S. Groft M. Posada de la Paz D. Taruscio & S. Groft (Dü) içinde Rare Diseases Epidemiology: Update and Overview. Cham: Springer. 2017;3-21
  • 8. Satman İ. Güdük Ö. Yemenici M. & Ertürk N. Nadir Hastalıklar Raporu. Ankara: TÜSEB (Türkiye Halk Sağlığı ve Kronik Hastalıklar Enstitüsü). 2019;1-30.
  • 9.Yavuz Çolak M. Nadir Hastalıklar Epidemiyolojisi. Ö. İnce & M. D. Pak içinde Tüm Yönleriyle Nadir Hastalıklar. Ankara: Nobel Akademik Yayıncılık. 2019;15-25.
  • 10.Vitale S. A. Parent recommendations for family functioning with Prader–Willi syndrome: a rare genetic cause of childhood obesity. Journal of pediatric nursing.2016;31(1):47-54.
  • 11.Péntek M. Gulácsi L. Brodszky V. Baji P. Boncz I. Pogány G.... & Posada-de-la-Paz M. Social/economic costs and health-related quality of life of mucopolysaccharidosis patients and their caregivers in Europe. The European Journal of Health Economics. 2016a; 17(1):89-98.
  • 12.Bruce J. E. Family Quality of Life in Families of Children with Phelan-McDermid Syndrome: A Rare Genetic Disability 2009; (Doctoral dissertation McGill University):42-59.
  • 13.Hwang M. S. Lee M. K. & Song J. R. The factors affecting burdens and quality of life of the family caregivers of patients with rare and incurable diseases using home ventilators. Korean Journal of Adult Nursing. 2014;26(2):191-202.
  • 14.Anderson M Elliott E Zurynski Y. Australian families living with rare disease: experiences of diagnosis health services use and needs for psychosocial support. Orphanet J Rare Dis. 2013.;8(1):22.
  • 15.Zarit S. H. Reever K. E. & Bach-Peterson J. Relatives of the impaired elderly: correlates of feelings of burden. The gerontologist. 1980;20(6):649-655.
  • 16.İnci F.H. & Erdem M. Bakım Verme Yükü Ölçeğinin Türkçeye Uyarlanması Geçerlilik ve Güvenilirliği. Atatürk Üniversitesi Hemşirelik Yüksekokulu Dergisi. 2008;11(4):85-95
  • 17.Cardinali P. Migliorini L. & Rania N. The caregiving experiences of fathers and mothers of children with rare diseases in Italy: challenges and social support perceptions. Frontiers in Psychology. 2019;10:1780.
  • 18.Péntek M. Herczegfalvi Á. Molnár M. J. Szőnyi L. P. Kosztolányi G. Pfliegler G. ... & Szegedi M. Dısease Burden On Duchenne Muscular Dystrophy Patıents And Theır Caregıvers. Ideggyogyaszati szemle. 2016b;69(5-6):183.
  • 19.Oh J. & Kim J. A. Factor analysis of the Zarit Burden Interview in family caregivers of patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.2018;19(1-2):50-56.
  • 20.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.
  • 21.Pangalila R.F. van den Bos G.A. Stam H.J. van Exel N.J. Brouwer W.B. Roebroeck M.E. Subjective caregiver burden of parents of adults with Duchenne muscular dystrophy. Disabil Rehabil 2012;34:988–996.
  • 22.Laurvick C. L. Msall M. E. Silburn S. et al. Physical and mental health of mothers caring for a child with Rett syndrome. Pediatrics.2006;118(4):1152–1164.
  • 23.Kahraman S. Çiftçi E. K. & Timuçin A. Determination of caregiving burden of parents providing care to their children with epidermolysis bullosa. Egyptian Journal of Dermatology and Venerology. 2017;37(1):1.
  • 24.Thomas P. T. Warrier M. G. Sadasivan A. Balasubramanium B. Preethish-Kumar V. Nashi S. ... & Nalini A. Caregiver burden and quality of life of patients with amyotrophic lateral sclerosis in India. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.2018;19(7-8):606-610.
  • 25.Kayadjanian N. Schwartz L. Farrar E. Comtois K. A. & Strong T. V. High levels of caregiver burden in Prader-Willi syndrome. PloS one. 2018;13(3) :e194-655.
There are 25 citations in total.

Details

Primary Language Turkish
Subjects Health Care Administration
Journal Section Orijinal Articles
Authors

Merve Deniz Pak Güre 0000-0001-7060-3729

Cemre Pak 0000-0002-3867-8082

Project Number 62310886-604.01.01
Publication Date June 20, 2021
Submission Date December 16, 2020
Published in Issue Year 2021 Volume: 15 Issue: 2

Cite

Vancouver Pak Güre MD, Pak C. NADİR HASTALIĞI OLAN ÇOCUKLARIN AİLELERİNDE BAKIM YÜKÜNÜN İNCELENMESİ. TJFMPC. 2021;15(2):269-77.

English or Turkish manuscripts from authors with new knowledge to contribute to understanding and improving health and primary care are welcome.