Parenting and Family Support Centre, School of Psychology, The University of Queensland, Brisbane, Australia.
Queensland Lifespan Metabolic Medicine Service, Queensland Children's Hospital, Brisbane, Australia; School of Medicine, The University of Queensland, Brisbane, Australia.
J Pediatr Psychol. 2021 Feb 19;46(2):208-218. doi: 10.1093/jpepsy/jsaa100.
Families of children with phenylketonuria (PKU) report child emotional and behavioral problems, parenting stress, and parenting difficulties, which are associated with worse health-related quality of life. This study aimed to examine acceptability and feasibility of a brief, group-based parenting program (Healthy Living Triple P) for families of children with PKU.
An uncontrolled nonrandomized trial design was used. Families of children aged 2-12 years (N = 17) completed questionnaire measures assessing child behavior and impact of PKU on quality of life (primary outcomes), and parenting behavior, self-efficacy and stress, and children's behavioral and emotional adjustment (secondary outcomes). Routinely collected blood phenylalanine (Phe) levels were obtained from the treating team. Parents selected two child behaviors as targets for change. The intervention comprised two, 2-hr group sessions delivered face-to-face or online. Assessment was repeated at 4-week postintervention (T2) and 4-month follow-up (T3).
Attrition was low and parent satisfaction with the intervention (face-to-face and online) was high. All families achieved success with one or both child behavior goals, and 75% of families achieved 100% success with both behavior goals by T3; however, there was no change in health-related quality of life. There were moderate improvements in parent-reported ineffective parenting (total score, d = 0.87, 95% CI -1.01 to 2.75) and laxness (d = 0.59, 95% CI -1.27 to 2.46), but no effects on parenting stress or children's adjustment. Phe levels improved by 6month post-intervention for children with elevated preintervention levels.
Results support intervention acceptability and feasibility. A randomized controlled trial is warranted to establish intervention efficacy.
苯丙酮尿症（PKU）患儿的家庭报告患儿存在情绪和行为问题、父母压力和育儿困难，这些问题与更差的健康相关生活质量有关。本研究旨在检验一种简短的基于小组的育儿计划（健康生活三重 P）对 PKU 患儿家庭的可接受性和可行性。
采用非随机对照试验设计。2-12 岁儿童的家庭（N=17）完成了评估儿童行为和 PKU 对生活质量影响的问卷（主要结果），以及育儿行为、自我效能感和压力，以及儿童行为和情绪调整（次要结果）。从治疗团队中获得常规收集的血苯丙氨酸（Phe）水平。父母选择两个孩子的行为作为改变的目标。干预措施包括两次 2 小时的面对面或在线小组会议。在干预后 4 周（T2）和 4 个月随访（T3）时重复评估。
失访率低，父母对干预（面对面和在线）的满意度高。所有家庭都成功地实现了一个或两个孩子的行为目标，并且 75%的家庭在 T3 时实现了两个行为目标的 100%成功；然而，健康相关的生活质量没有变化。父母报告的无效育儿（总分，d=0.87，95%置信区间-1.01 至 2.75）和宽松（d=0.59，95%置信区间-1.27 至 2.46）方面有中度改善，但对父母压力或儿童调整没有影响。对于干预前水平升高的儿童，干预后 6 个月 Phe 水平有所改善。
结果支持干预的可接受性和可行性。需要进行随机对照试验来确定干预的疗效。
