Evaluation of quality of life and its associations with clinical parameters in pediatric patients with familial Mediterranean fever

OBJECTIVE: A favorable quality of life (QoL) is important in children with chronic disease, and it reflects successful disease management. The aim of our study was to evaluate QoL and its association with clinical parameters in pediatric patients with familial Mediterranean fever (FMF).
METHODS: The Kinder Lebensqualität Fragebogen (KINDL®) questionnaires (kiddy: 4–7 years; kid: 8–16 years) for children and the proxy version for parents were implemented as a QoL measure. A total of 171 FMF patients, 69 healthy peers and their parents were enrolled in the study.
RESULTS: The KINDL QoL scores of the FMF patients were significantly lower than their healthy peers. The physical and emotional well-being KINDL QoL scores of the FMF children were significantly lower than their healthy peers (p=0.017 and p=0.020, respectively). In the evaluation of the KINDL QoL scores between the kiddy and kid groups, only the self-esteem score was higher in the kiddy group (p=0.004), and the school functioning scores were higher in the kid group (p=0.002). The scores in the physical well-being and disease module had significant differences between patients who were adherent and those who were non-adherent to colchicine therapy (p=0.042 and p=0.047, respectively). The scores in the physical well-being and disease module were significantly higher in patients with fewer attacks than those who had many attacks per year (p=0.004 and p=0.014, respectively).
CONCLUSION: This study suggests that FMF patients have significantly impaired QoL. The irregular use of colchicine and more frequent attacks affect QoL even more. A QoL assessment with multidisciplinary follow-up and control of the disease activity are essential, and if necessary, individualized support should be given to patients.

Evaluation of quality of life and its associations with clinical parameters in pediatric patients with familial Mediterranean fever

Amaç
Kronik hastalýða sahip çocuk hastalarda iyi yaþam kalitesi önemlidir ve hastalýðýn baþarýlý yönetildiðinin göstergesidir. Çalýþmamýzýn amacý çocuk ailevi Akdeniz ateþi (AAA) hastalarýnda yaþam kalitesi düzeyini ölçmek ve klinik parametreler ile iliþkisini deðerlendirmektir.
Yöntem
Yaþam kalitesi (YK), çocuk hasta ve ebeveynine yönelik yapýlan Kinder Lebensqualität Fragebogen (KINDL®) anketi (küçük çocuk: 4-7 yaþ, çocuk: 8-16) ile deðerlendirildi.171 çocuk AAA hastasý ve ebeveynleri; 59 saðlýklý çocuk ve ebeveynleri çalýþmaya dahil edildi.
Bulgular
Çocuk AAA hastalarýnýn KINDL YK skorlarý saðlýklý çocuklara göre anlamlý düzeyde düþüktü. AAA çocuklarýn fiziksel iyilik hali ve duygusal iyilik hali KINDL YK skorlarý saðlýklý yaþýtlarýna göre anlamlý olarak düþüktü (sýrasýyla, p = 0.017 ve p = 0.020). Küçük ve büyük yaþ grubu AAA hastalarý arasýnda KINDL YK skorlarý deðerlendirildiðinde, küçük yaþ grubu AAA hastalarýnda sadece benlik saygýsý skoru daha yüksekti (p = 0.004) ve büyük yaþ grubu AAA hastalarýnda okul fonksiyon skoru daha yüksekti (p = 0.002). Kolþisin tedavisine uyum gösteren ve göstermeyen hastalar arasýnda fiziksel iyilik hali ve hastalýk modülü skorlarýnda anlamlý farklýlýklar vardý (sýrasýyla, p = 0.042 ve p = 0.047). Fiziksel iyilik ve hastalýk modülü skorlarý, bir yýlda seyrek atak geçiren hastalarda sýk atak geçiren hastalara göre daha yüksekti (sýrasýyla, p = 0.004 ve p = 0.014).
Sonuç
Çalýþmamýz çocuk AAA hastalarýnýn yaþam kalitesinin belirgin düzeyde etkilendiðini göstermektedir. Düzensiz kolþisin kullanýmý ve sýk atak geçirme yaþam kalitesini daha da fazla etkilemektedir. Yaþam kalitesi ölçümü eþliðinde multidisipliner bir takip ile AAA hastalarýnýn düzenli deðerlendirilmesi, hastalýk aktivitesinin kontrolü ve gerekli hastalarda bireysel destek önemlidir. (NCI-2020-0222.R1)