Clinical Study of Mobile Application- (App-) Based Family-Centered Care (FCC) Model Combined with Comprehensive Iron Removal Treatment in Children with Severe Beta Thalassemia

Abstract

Background and Objective. Hemoglobinopathy is one of the most prevalent monogenic disorders in the world. Thalassemia is characterized by autosomal recessive deficiencies in hemoglobin production. The difficulties of iron overload caused by transfusions, which are the foundation of illness management in the majority of patients with severe thalassemia, may further worsen the clinical features. There are numerous obstacles and restrictions to the currently accessible conventional therapy for thalassemia. The purpose of the study is to investigate the clinical impact of the family-centered care (FCC) model based on a mobile application (app) in conjunction with comprehensive iron removal therapy for children with severe beta thalassemia. Methods. A retrospective study was conducted on the clinical records of 148 children diagnosed with severe beta thalassemia who were admitted to our hospital between October 2018 and September 2021. The patients were separated into two groups, a control group and an intervention group, with 74 cases in each group, according to the various care approaches. The basic treatment regimen was given to all of the children: deferoxamine mesylate combined with deferiprone. During treatment, the control group received routine care, and the intervention group adopted the FCC model based on a mobile app. The quality of life scale for children and adolescents (QLSCA) score, the family assessment device (FAD) score, the exercise of self-care agency scale (ESCA) score, and the medication compliance scale score were compared between the two groups. Results. The QLSCA score, ESCA score, and medication compliance scale score of the intervention group were significantly higher than those of the control group and showed a significant difference (intergroup effect: $F=198.400$ , 259.200, and 129.800, all $P<0.001$ ). Scores in both groups increased over time (time effect: $F=19.350$ , 40.830, and 12.130, all $P<0.001$ ), and there was an interaction effect between grouping and time (interaction effect: $F=3.937$ , 12.020, and 5.028). The $P$ values were 0.020, <0.001, and 0.007. The FAD score of the intervention group was significantly lower than that of the control group (intergroup effect: $F=177.200$ , $P<0.001$ ). The FAD scores of both groups decreased over time (time effect: $F=7.921$ , $P=0.005$ ). There was an interaction effect between groups and time (interaction effect: $F=5.206$ , $P=0.006$ ). Conclusion. The application effect of the mobile app-based FCC model combined with the comprehensive iron removal treatment program in children with severe beta thalassemia is significant, which can significantly improve the quality of life, family function, self-care ability, and medication compliance of children, and has high clinical application value.