Sickle Cell Disease and Psychosocial Well-Being: Comparison of Patients With Preclinical and Clinical Avascular Necrosis of the Femoral Head

Aim: Sickle cell disease (SCD) has significant adverse psychosocial impacts in childhood. Patients with SCD may be affected by avascular necrosis (AVN) and the most commonly involved site is the femoral head. We aimed to conduct a comparative investigation of the psychosocial well-being of pediatric SCD patients with preclinical and clinical femoral head AVN.

Materials And Methods: Patients with homozygous SCD and healthy peers aged 7-17 years were included in this cross-sectional study. Psychosocial well-being was assessed by the Strengths and Difficulties Questionnaire (SDQ), parent version. SDQ scores were compared between the groups.

Results: A total of 74 mother-child couples were enrolled in this study. The SCD with clinical AVN (stages I-IV) group consisted of 17 patients, SCD with preclinical AVN (stage 0) group consisted of 20 patients, and the control group consisted of 37 individuals. The sociodemographic characteristics and medians of total difficulties, emotional problems, conduct problems, hyperactivity, and peer problems scores were not different between the 3 groups ( > .05). There was a significant difference between the 3 groups in the prosocial score that indicates more positive social behaviors. Both groups, SCD with clinical AVN and with preclinical AVN, had lower prosocial scores than the control group ( < .001). The 2 patient groups did not differ in any SDQ scores or disease-related characteristics of vaso-occlusive crises and blood/exchange transfusions in the recent year ( > .05).

Conclusions: Pediatric patients with SCD, whether or not complicated with clinical AVN, had lower prosocial scores than healthy peers. This study has presented the first comparison of the psychosocial well-being of pediatric SCD patients with preclinical and clinical femoral head AVN.