Health-related quality of life in pediatric sickle cell disease: insights from a Portuguese hospital-based study.

Sickle cell disease (SCD) significantly affects health-related quality of life (HRQoL) in children and adolescents, with pain being a major contributor. We conducted a cross-sectional study of 27 patients aged 5-17 years attending pediatric outpatient clinics at a secondary-level hospital in Lisbon, Portugal. HRQoL was assessed using the Portuguese version of the Pediatric Quality of Life Inventory™ Sickle Cell Disease Module 3.0 (PedsQL™ SCD), encompassing domains such as pain, pain impact, emotions, treatment, and communication. The total score indicated intermediate HRQoL, with the pain impact domain showing the lowest scores, highlighting the importance of systematic pain management. Associations were observed between age, disease complications, household composition, and specific HRQoL domains, suggesting that both clinical burden and family context influence patient-reported outcomes. These findings underscore the multifactorial nature of HRQoL in pediatric SCD and support the development of standardized outpatient and emergency pain management protocols, alongside family-centered care, to improve quality of life in this population.