Ambler Olivia, Medford Emma, Hare Dougal J
School of Psychology, Cardiff University, Cardiff, UK.
Child and Adolescent Mental Health Services, Doncaster and South Humber NHS Foundation Trust, Doncaster, UK.
JIMD Rep. 2018;41:91-100. doi: 10.1007/8904_2018_105. Epub 2018 Apr 20.
Phenylketonuria (PKU) is an inherited metabolic condition that can lead to the onset of intellectual disabilities if not strictly managed through a low-protein diet. Parents are responsible for supervising their child's treatment for PKU, which may impact on their experience of distress. This cross-sectional study aimed to identify the factors that contribute to distress in parents who care for a child with PKU, distinct from parents in the general population. Thirty-eight parents of children and adolescents with PKU and 32 parents in the general population completed the questionnaires measuring parental psychological resilience, child behaviour problems, perceived social support and distress. Parents of children with PKU also completed measures of their child's care dependency and behaviour related to developmental and intellectual disabilities. The findings revealed no statistically significant differences in distress between the groups, but parents of children with PKU reported more child behaviour problems. Multiple regression analysis identified that parental psychological resilience and child anxious behaviour explained 35% of the variance in distress for parents of children with PKU. By comparison, parental psychological resilience and generic child behaviour only accounted for 19% of the variance in distress for parents in the general population. This has implications for developing interventions in clinical settings that aim to reduce parents' distress by enhancing their psychological resilience and supporting them to manage child behaviour difficulties, particularly anxious behaviour. Future research should include larger, more diverse samples and use longitudinal study designs.
苯丙酮尿症（PKU）是一种遗传性代谢疾病，如果不通过低蛋白饮食严格控制，可能会导致智力残疾。父母负责监督孩子的苯丙酮尿症治疗，这可能会影响他们的痛苦体验。这项横断面研究旨在确定导致照顾苯丙酮尿症患儿的父母痛苦的因素，以区别于普通人群中的父母。38名患有苯丙酮尿症的儿童和青少年的父母以及32名普通人群的父母完成了测量父母心理韧性、儿童行为问题、感知到的社会支持和痛苦的问卷。患有苯丙酮尿症儿童的父母还完成了对其孩子的护理依赖以及与发育和智力残疾相关行为的测量。研究结果显示两组在痛苦方面没有统计学上的显著差异，但患有苯丙酮尿症儿童的父母报告的儿童行为问题更多。多元回归分析表明，父母的心理韧性和儿童的焦虑行为解释了患有苯丙酮尿症儿童的父母痛苦差异的35%。相比之下，父母的心理韧性和一般儿童行为仅占普通人群父母痛苦差异的19%。这对于在临床环境中开展干预措施具有启示意义，这些干预措施旨在通过增强父母的心理韧性并支持他们应对儿童行为困难，特别是焦虑行为，来减轻父母的痛苦。未来的研究应纳入更大、更多样化的样本，并采用纵向研究设计。
