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

Haitjema, Sietske; Lubout, Charlotte M. A.; Abeln, D.; Veen, M. de Bruijn-van der; MacDonald, Anita; Wolffenbuttel, B.H.R.; Spronsen, Francjan J. van

doi:10.1016/j.nut.2021.111576

Cited by 11 publications

(6 citation statements)

References 23 publications

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“…In terms of food choice, the majority of caregivers stated that their children were choosy about food and reluctant to try new food. This behavior could be attributed to the limited exposure to varied foods in the weaning period [55]. Consequently, this hampers the caregiver's attempt at providing a varied dietary plan [56].…”

Section: Discussionmentioning

confidence: 99%

Exploring the Barriers and Motivators to Dietary Adherence among Caregivers of Children with Disorders of Amino Acid Metabolism (AAMDs): A Qualitative Study

et al. 2022

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Dietary intervention is generally accepted as the mainstay of treatment for patients with disorders of amino acid metabolism (AAMDs). However, dietary adherence to a low-protein diet is always reported as a common challenge among these patients. This study explored the perception of barriers and motivators to dietary adherence among caregivers of AAMD patients in Malaysia. Twenty caregivers of children with AAMDs receiving ongoing treatment at the genetic clinic participated in an online focus group discussion from November to December 2021. Findings showed a total of five interrelated main themes identified from focus group discussion (FGD) exploring parents’ experiences related to the management of their child’s daily diet. The barriers to dietary adherence were burden of dietary treatment, diet and dietary behavior, parenting challenges, limited knowledge related to dietary treatment, and challenges in healthcare system delivery. Key factors facilitating good dietary adherence include good knowledge of dietary treatment, parental coping strategies, social coping, and dietary behavior. In conclusion, despite the existence of several barriers to the implementation of dietary treatment, caregivers managed to use a wide range of coping strategies to overcome some, if not all, of the challenges. The important next step is to develop, in conjunction with multidisciplinary healthcare professionals, feasible implementation strategies that could address these barriers and at the same time improve the quality of life of caregivers.

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Section: Discussionmentioning

confidence: 99%

Exploring the Barriers and Motivators to Dietary Adherence among Caregivers of Children with Disorders of Amino Acid Metabolism (AAMDs): A Qualitative Study

et al. 2022

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“…The palatability of low‐protein recipes need to be improved. This is a major problem because dietary diversity is already limited in PKU patients 9 . Some PKU patients do not have access to low‐protein products 10,11 .…”

Section: Discussionmentioning

confidence: 99%

“…This is a major problem because dietary diversity is already limited in PKU patients. 9 Some PKU patients do not have access to low-protein products. 10,11 In addition, some of these low-protein products are not covered by health insurance in some countries, so the financial burden is associated with the diet.…”

Section: Discussionmentioning

confidence: 99%

Actions speak louder than words: Home visits and its effect on dietary adherence in patients with phenylketonuria

Bulut,

Kor,

Kilavuz

et al. 2023

J Paediatrics Child Health

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AimPhenylketonuria (PKU) is an inherited metabolic disorder in which accumulation of phenylalanine (Phe) leads to poor neurological outcomes without treatment. Dietary therapy is the main treatment and nonadherence is associated with elevated blood Phe levels and correspondingly poor neuropsychiatric outcomes. This study aimed to examine the effect of home visits on blood Phe levels in PKU patients.MethodsSixty‐five paediatric PKU patients who were on low‐phenylalanine diet were visited monthly at home for 6 months. At each visit, dietary education was provided, patients' height and weight were measured and blood samples were collected.ResultsTwenty‐eight (43.1%) patients had classic PKU and 37 (56.9%) had moderate PKU. Blood Phe levels decreased statistically significant at first, second, fifth, and sixth months compared with screening visit. Blood Phe levels in moderate PKU patients decreased significantly at the last visit unlike classic PKU patients. A significant decrease in blood Phe levels was observed in patients older than 10 years. Anthropometric parameters improved.ConclusionsDietary nonadherence is the main treatment failure in PKU. Home visits for education are a promising way to improve treatment outcomes by providing quality education, better assessment, and correction of mistakes but they should be ongoing and supported by different interventions that address patients' special needs.

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“…It is established that young children with PKU on dietary treatment only, display abnormal feeding patterns and show less interest in food, accompanied by food neophobia [ 47 , 48 , 49 ]. They may exhibit more food tantrums [ 50 ] and are even fearful of eating high-protein foods. Some children with PKU, usually with co-morbidities such as autism, demonstrate similar feeding behaviours to children with ‘avoidant/restrictive food intake disorder’ and provide additional difficulties [ 51 ].…”

Section: Discussionmentioning

confidence: 99%

Suitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National Consensus

et al. 2022

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Introduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of guidelines for the introduction of higher protein foods into the diets of patients with PKU who tolerate >10 g/day of protein. Methods: In January 2022, a 26-item food group questionnaire, listing a range of foods containing protein from 5 to >20 g/100 g, was sent to all British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 80; 26 Inherited Metabolic Disease [IMD] centres). They were asked to consider within their IMD dietetic team when they would recommend introducing each of the 26 protein-containing food groups into a patient’s diet who tolerated >10 g to 60 g/day of protein. The patient protein tolerance for each food group that received the majority vote from IMD dietetic teams was chosen as its tolerance threshold for introduction. A virtual meeting was held using Delphi methodology in March 2022 to discuss and agree final consensus. Results: Responses were received from dietitians from 22/26 IMD centres (85%) (11 paediatric, 11 adult). For patients tolerating protein ≥15 g/day, the following foods were agreed for inclusion: gluten-free pastas, gluten-free flours, regular bread, cheese spreads, soft cheese, and lentils in brine; for protein tolerance ≥20 g/day: nuts, hard cheeses, regular flours, meat/fish, and plant-based alternative products (containing 5–10 g/100 g protein), regular pasta, seeds, eggs, dried legumes, and yeast extract spreads were added; for protein tolerance ≥30 g/day: meat/fish and plant-based alternative products (containing >10–20 g/100 g protein) were added; and for protein tolerance ≥40 g/day: meat/fish and plant-based alternatives (containing >20 g/100 g protein) were added. Conclusion: This UK consensus by IMD dietitians from 22 UK centres describes for the first time the suitability and allocation of higher protein foods according to individual patient protein tolerance. It provides valuable guidance for health professionals to enable them to standardize practice and give rational advice to patients.

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

Cited by 11 publications

References 23 publications

Exploring the Barriers and Motivators to Dietary Adherence among Caregivers of Children with Disorders of Amino Acid Metabolism (AAMDs): A Qualitative Study

Exploring the Barriers and Motivators to Dietary Adherence among Caregivers of Children with Disorders of Amino Acid Metabolism (AAMDs): A Qualitative Study

Actions speak louder than words: Home visits and its effect on dietary adherence in patients with phenylketonuria

Suitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National Consensus

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