Dietary treatment in Dutch children with phenylketonuria:An inventory of associated social restrictions and eating problems

OBJECTIVES: Dietary treatment in phenylketonuria (PKU) is known to cause eating problems, but knowledge of both prevalence and magnitude, especially for social restrictions, is scarce. Our aim was to evaluate the social restrictions and eating problems that children with PKU and their caregivers experience with dietary treatment. METHODS: A web-based questionnaire, based on the Behavioral Pediatrics Feeding Assessment Scale with additional PKU-specific questions, was developed in close collaboration with and distributed by the Dutch PKU Association, which sent an e-mail to its members containing a link to the questionnaire. The questionnaire was completed by caregivers of children with PKU in the Netherlands and caregivers of age-matched children without PKU. Data were analyzed with the Kruskal-Wallis and Mann-Whitney U test using SPSS. RESULTS: Compared with caregivers of children in the control group (ages 1-16 y; n = 50), caregivers of children with PKU (ages 1-16 y; n = 57) reported more difficulty in offering food variety, experienced more stress when eating an evening meal outside the home and during vacation, and were stricter about (accidental) spilling of food during dinner by the child (P < 0.05). They also reported to being angrier, more frustrated, and more anxious when feeding their child, and they more often felt that their child's eating pattern had a negative influence on the child's general health (P < 0.05). CONCLUSION: This pilot study provides further evidence that restriction of social activities and eating problems associated with dietary restrictions is more common in children with PKU, and warrants awareness on this topic among professionals working with these children

A retrospective study of eating and psychosocial problems in patients with hepatic glycogen storage diseases and idiopathic ketotic hypoglycemia: Towards a standard set of patient-reported outcome measures

There is a paucity in literature on eating and psychosocial problems in patients with hepatic glycogen storage disease (GSD) and idiopathic ketotic hypoglycemia (IKH), problems that can greatly affect quality of life. This is a monocentre, retrospective, observational mixed method study of patients with hepatic GSD or IKH treated at the Beatrix Children's Hospital Groningen, who had been referred to SeysCentra, a specialist centre for the treatment of eating problems. Additionally, a systematic literature review has been performed to identify instruments to quantify patient‐reported outcome measures of psychosocial problems in hepatic GSD patients. Sixteen patients from 12 families were included with ages ranging between 3 and 24 years. Five out of sixteen patients were diagnosed with Avoidant/Restrictive Food Intake Disorder and six patients showed characteristics of this disorder. Fourteen patients experienced sleeping problems, and 11 out of 12 parent couples experienced stress about the illness of their child. We subsequently identified 26 instruments to quantify patient‐reported outcome measures for GSD patients. This study demonstrates that GSD patients can develop Avoidant/Restrictive Food Intake Disorder influencing quality of life at multiple domains. The identification of instruments to assess psychosocial wellbeing is an important step towards a standard set of patient‐reported outcome measures