The Quality of Life in Patients with Familial Mediterranean Fever and Their Parents Perception

Abstract

Background Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and polyserositis. This study aimed to assess children’s quality of life (QoL), as reported by children and their parents, and to compare the results according to clinical variables.

Material and Methods The study examined 107 children with FMF, evaluating their demographic and genetic data, utilizing the Pediatric Quality of Life Inventory (PedsQL) to assess QoL, and comparing scores based on disease severity.

Results The severity of FMF is inversely correlated with QoL scores, with mild cases having the highest scores (97±4), followed by moderate (76±11) and severe cases (52±10.3) (p<0,001). Disease severity, treatment adherence, healthcare utilization, genetic mutations, family income, and maternal age at birth all significantly impact perceived quality of life in FMF patients (p<0,001). Additionally, parents reported lower QoL for children with FMF who experienced various adverse factors such as low family income, household smoking, frequent attacks, hospitalizations, irregular medication use, and low maternal education levels (p<0,001).

Conclusion Children’s daily activities, academic performance, and family functioning are all significantly impacted by FMF. Physicians caring for patients with FMF should be aware of the QoL changes in the management of these patients. As a result, medical therapy, patient education, and indicators of psychological and social support can all be offered more effectively.

Zusammenfassung

Hintergrund Das familiäre Mittelmeerfieber (FMF) ist eine chronische Erkrankung, die sich durch wiederkehrende Fieber- und Polyserositis-Episoden auszeichnet. Diese Studie hatte zum Ziel, die Lebensqualität (LQ) von Kindern zu bewerten, wie von den Kindern selbst und ihren Eltern berichtet, und die Ergebnisse gemäß klinischer Variablen zu vergleichen.

Material und Methoden Die Studie untersuchte 107 Kinder mit FMF, bewertete ihre demografischen und genetischen Daten, nutzte das Pediatric Quality of Life Inventory (PedsQL) zur Bewertung der LQ und verglich die Ergebnisse basierend auf der Krankheitsschwere.

Ergebnisse Die Schwere der FMF korreliert invers mit den LQ Werten, wobei milde Fälle die höchsten Werte aufweisen (97±4), gefolgt von moderaten (76±11) und schweren Fällen (52±10,3) (p<0,001). Krankheitsausprägung, Therapieadhärenz, Inanspruchnahme von Gesundheitsdiensten, genetische Mutationen, Familieneinkommen und das mütterliche Alter bei der Geburt beeinflussen alle signifikant die wahrgenommene LQ bei FMF-Patienten (p<0,001). Darüber hinaus berichteten Eltern über eine niedrigere LQ für Kinder mit FMF, die verschiedenen ungünstigen Faktoren ausgesetzt waren, wie niedriges Familieneinkommen, Rauchen im Haushalt, häufige Anfälle, Krankenhausaufenthalte, unregelmäßige Medikamenteneinnahme und niedriger mütterlicher Bildungsstand (p<0,001).

Schlussfolgerungen Die täglichen Aktivitäten der Kinder, ihre schulische Leistung und das Familienfunktionieren werden alle signifikant von FMF beeinflusst. Ärzte, die Patienten mit FMF betreuen, sollten sich der Veränderungen der LQ bei der Behandlung dieser Patienten bewusst sein. Daher können medizinische Therapien, Patientenaufklärung und Indikatoren für psychologische und soziale Unterstützung effektiver angeboten werden.

Keywords

children - colchicine - familial Mediterranean fever - quality of life

Schlüsselwörter

Kinder - Colchicin - familiäres Mittelmeerfieber - Lebensqualität

Publikationsverlauf

Artikel online veröffentlicht:
06. August 2024

• References

• 1 Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet 1998; 351: 659-664
• 2 Livneh A, Langevitz P, Zemer D. et al. Criteria for the diagnosis of familial mediterranean fever. Arthritis Rheum 1997; 40: 1879-1885
• 3 Bernot A, Clepet C, Dasilva C. et al. A candidate gene for familial Mediterranean fever. Nat Genet 1997; 17: 25-31
• 4 Chae JJ, Wood G, Masters SL. et al. The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1β production. Proc Natl Acad Sci USA 2006; 103: 9982
• 5 Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. Best Pract Res Clin Rheumatol 2000; 14: 477-498
• 6 Giese A, Kurucay M, Kilic L. et al. Quality of life in adult patients with Familial Mediterranean fever living in Germany or Turkey compared to healthy subjects: A study evaluating the effect of disease severity and country of residence. Rheumatol Int 2013; 33: 1713-1719
• 7 McDougall J, Wright V, Schmidt J. et al. Applying the ICF framework to study changes in quality-of-life for youth with chronic conditions. Dev Neurorehabil 2011; 14: 41-53
• 8 Demirag SA. Kronik Hastalıklar ve Yaşam Kalitesi. Turkiye Klin Fam Med – Spec Top 2015; 6: 6-9
• 9 Ozen S, Demirkaya E, Erer B. et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 2016; 75: 644-651
• 10 Makay B, Unsal E, Arslan N. Quality of life of school-age children with Familial Mediterranean fever: a preliminary study. Pediatric Rheumatology 2008; 6: 1-1
• 11 Yalcinkaya F, Ozen S, Ozcakar ZB. et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology 2009; 48: 395-398
• 12 Demirkaya E, Acikel C, Hashkes P. et al. Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF). Ann Rheum Dis 2016; 75: 1051-1056
• 13 Pras E, Livneh A, Balow JE. et al. Clinical differences between North African and Iraqi Jews with familial Mediterranean fever. American journal of medical genetics 1998; 75: 216-219
• 14 Yasar Durmus S, Ozlu SG, Cop E. et al. Health Related Quality of Life of Children Aged 2-18 Years With Familial Mediterranean Fever. Türkiye Çocuk Hast Derg 2022; 16: 469-475
• 15 Varni JW, Seid M, Rode CA. The PedsQL Measurement Model for the Pediatric Quality of Life Inventory. Med Care 1999; 37: 126-139
• 16 Varni JW, Seid M, Knight TS. et al. The PedsQL in pediatric rheumatology: Reliability, validity, and responsiveness of the Pediatric Quality of Life Inventory^TM Generic Core Scales and Rheumatology Module. Arthritis Rheum 2002; 46: 714-725
• 17 Uneri OS, Agaoglu B, Coskun A. et al. Validity and reliability of Pediatric Quality of Life Inventory for 2- to 4-year-old and 5- to 7-year-old Turkish children. Qual Life Res 2008; 17: 307-315
• 18 Memik NC, Agaoglu B, Coskun A. et al. The validity and reliability of the Turkish Pediatric Quality of Life Inventory for children 13-18 years old. Turk Psikiyatri Dergisi 2007; 18: 353 18066726
• 19 Buskila D, Zaks N, Neumann L. et al. Quality of life of patients with familial Mediterranean fever. Clin Exp Rheumatol 1997; 15: 355-360 9272294
• 20 Sahin S, Yalcin I, Senel S. et al. Assesment life quality of familial Mediterranean fever patients by short form-36 and its relationship with disease parameters. Eur Rev Med Pharmacol Sci 2013; 17: 958-963 23640444
• 21 Skevington SM, Lotfy M, O’Connell KA. The World Health Organization’s WHOQOL-BREF quality of life assessment: Psychometric properties and results of the international field trial a Report from the WHOQOL Group. Qual Life Res 2004; 13: 299-310
• 22 Burckhardt CS, Anderson KL. The Quality of Life Scale (QOLS): reliability, validity, and utilization. Health Qual Life Outcomes 2003; 1: 60
• 23 Alayli G, Durmus D, Ozkaya O. et al. Functional capacity, strength, and quality of life in children and youth with familial mediterranean fever. Pediatr Phys Ther 2014; 26: 347-352
• 24 Sousa H, Ribeiro O, Paúl C. et al. Social support and treatment adherence in patients with end-stage renal disease: A systematic review. Semin Dial 2019; 32: 562-574
• 25 Lahai M, James PB, Wannang NNM. et al. A cross-sectional study on caregivers’ perspective of the quality of life and adherence of paediatric HIV patients to highly active antiretroviral therapy. BMC Pediatr 2020; 20
• 26 Al-Ghamdi MS, Taha AZ, Ahmad B. et al. Quality of life in a sample of hypertensive patients attending primary health care facilities in Al-khobar, Saudi Arabia. J Family Community Med 2002; 9: 25-32 23008659 PMCID: PMC3430172
• 27 Adamu K, Feleke A, Muche A. et al. Health related quality of life among adult hypertensive patients on treatment in Dessie City, Northeast Ethiopia. PLoS One 2022; 17: e0268150
• 28 Al Qadire M, ALHosni F, Al-Daken L. et al. Quality of Life and its predictors among patients with selected chronic diseases. In Nursing Forum 2023; 1-9
• 29 Levi RB, Drotar D. Health-related quality of life in childhood cancer: discrepancy in parent-child reports. Int J Cancer 1999; 83: 58-64
• 30 Chang PC, Yeh CH. Agreement between child self-report and parent proxy-report to evaluate Quality of Life in Children with Cancer. Psychooncology 2005; 14: 125-134