Action Research on Metabolic Control in Children with inborn errors of metabolism

2022 Fiscal Year Final Research Report

• Project/Area Number: 16K21298
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Clinical nursing; Pediatrics
• Research Institution: Kagawa Prefectural College of Health Sciences
• Principal Investigator: MATSUMOTO Yuko 香川県立保健医療大学, 保健医療学部, 助教 (20633639)
• Project Period (FY): 2016-04-01 – 2023-03-31
• Keywords: 看護学 / 慢性看護学 / 小児代謝・栄養学 / 発症予防 / 家族

Outline of Final Research Achievements

We conducted an interview survey of specialists involved in the examination and treatment of inborn errors of metabolism. The specialists believed that in order to prevent the onset and severity of the disease, it was important for the medical staff to (1) provide the affected family with an appropriate diagnosis and a treatment plan that matches the growth and development of the child and the nature of the consultation, and (2) consider the psychosocial aspects of the child's family and build a relationship of trust with the child's family. In addition, from the analysis of the consultations received by the Patient and Family Association, it was found that positive behavior was observed in the consulting families 6 months after the start of the consultation support.
These findings indicate that psychosocial support by medical personnel and other affected families is essential for the families of children with inborn errors of metabolism, a rare disease, to lead happy lives.

Free Research Field

小児看護学

Academic Significance and Societal Importance of the Research Achievements

研究の結果から、患児家族の前向きな力を発揮して児の成長発達を促すためには、児と家族の置かれた状況や心理社会的特徴に寄り添う支援が重要であることが示唆された。
患児家族は、治療に関する教育・指導を受け、児の発達上の特徴や嗜好に合わせて試行錯誤しながら家庭での治療を担ってきた。しかし、親の治療コンプライアンスと児の健康や発達が必ずしも一致するとは限らないことが分かった。児の成長や心理社会的なニーズを捉えた発症リスクの評価と、社会活動のための調整が必要であることが明らかになった。これらの結果は、発症予防のための治療をしながら、児と家族が幸福な社会生活を送るための支援体制を確立することに寄与できる。