Young Children, Parents Struggle Socially and Emotionally, Survey Finds
Struggles with “mood and emotions” and feelings of social isolation or inadequate social support are more pronounced in young children with hemophilia and their parents than in age-matched healthy peers, a study from Japan reports.
But such “anxieties,” which affect quality of life, were less a concern in survey responses from teenagers with this disease, “suggesting a progressive understanding of the disorder” as patients age, and supporting the effectiveness of prophylactic treatment, its researchers said.
The study, “Assessment of self‐/parent‐reported quality of life in Japanese children with haemophilia using the Japanese version of KIDSCREEN‐52,” was published in the journal Haemophilia.
Recent therapy advances, like prophylaxis, have made it easier to manage hemophilia. But as research and clinical testing largely focuses on evaluating improvements in annual bleeding rates, the status of patients’ quality of life (QOL) is not fully understood.
However, assessing health‐related quality of life (HRQOL) is critical for providing comprehensive care to people with hemophilia.
Several HRQOL protocols, using disease-specific questionnaires, have been proposed for understanding how hemophilia affects children. Yet, few studies have investigated self-reported assessments using an internationally standardized questionnaire.
KIDSCREEN-52 is a well-established tool for evaluating HRQOL in children and adolescents with hemophilia across 10 dimensions. Its Japanese version (J-KIDSCREEN-52) is also both established and validated.
Researchers used J-KIDSCREEN-52 to evaluate HRQOL in children and adolescents, ages 8 to 18, with hemophilia A or B (a self-assessment), their family members (parent assessment), and age-matched peers with no history of a chronic illness serving as controls.
The questionnaire was completed by 36 parents and children in the hemophilia group, and 160 in the control group.
Results indicated that younger children with hemophilia (ages 8 to 12) had lower scores for “moods and emotions,” defined as a sense of helplessness, loneliness, and depressive feelings compared to controls.
Their parents also reported lower scores for “moods and emotions,” “social support and peers” (relationship with peers and quality of communication with friends), and “school environment” (satisfaction with school life, teachers and academic results) than did adults without disease in their family, the investigators found.
No significant differences in HRQOL between adolescent patients (ages 13‐18) or their parents were seen, with their scores largely matching those of controls. The researchers suggested that prophylactic treatment’s wide use in Japan, and advancing work on preventing inhibitors to treatment, were main reasons for the QOL improvements expressed by older children.
“[I]n the present survey, the self- and parent-assessment HRQOL scores in children aged 13-18 years receiving prophylaxis were not significantly different from those of the control group, strongly confirming the effectiveness of prophylactic therapy,” the researchers wrote.
Researchers also evaluated the impact of three features — weak social support, target joints, and unexpected hospital visits — on HRQOL that had been previously been associated with a poor life quality in patients.
Weaker social support was associated with low physical and psychological states, and negatively influenced family relationships and the school environment. The identification of target joints was linked to a both poorer physical state (defined as physical and motor activity) and psychological state (satisfaction with life and positive thinking).
Unplanned hospital visits were associated with low QOL scores for “self-perception” (body and inner self-image and satisfaction with appearance), “autonomy” (freedom of choice and self-sufficiency), “family relations,” and “school environment.”
“[O]ur data demonstrated that unplanned hospital visits interfered with school events and tended to limit meetings with friends. These social contacts provide the opportunity to enhance HRQOL,” the researchers wrote.
They also evaluated how body pain affects HRQOL. They found that neck and shoulder pain had a negative influence on a patient’s psychological state, including “self‐perception” and “school environment.” But joint pain in the knee, ankle, and elbow was not seen to significantly be linked to any HRQOL dimension.
“The results suggested that children and adolescents with hemophilia and their parents could benefit from psychosocial intervention to reduce the risk of a poor QOL, especially in the lower-aged group,” the investigators wrote.
Specifically, “families would benefit from a careful explanation of the clinical consequences of haemophilia at the time of diagnosis and at important milestones in their lives, for example, kindergarten, primary, junior high and high school,” as well as continuing updates on treatments that might prevent them from forming “negative attitudes towards this inherited haemorrhagic disorder,” the study concluded.