Need analysis of a dietary application among caregivers of patients with disorders of amino acid metabolism (AAMDs): A mixed-method approach.

BACKGROUND: A dietary application can assist the caregivers of AAMDs children in auto-calculating the protein intake, hence improving dietary compliance. However, existing dietary application for patients with AAMDs only focus on delivering the nutritional content of food and monitoring the dietary intake but were lacking in other educational components. OBJECTIVE: To assess the uses, needs and preferences towards a dietary application among the caregivers of AAMDs patients. METHODS: We conducted a mixed-method study comprising focus group discussion (FGD) and a quantitative questionnaire survey among caregivers of patients aged between 6-month-old to 18-year-old with AAMDs who are receiving active treatment, both medical and dietetic treatment in the genetic clinic, Hospital Kuala Lumpur (HKL). RESULTS: A total of 76 and 20 caregivers participated in the survey and FGD respectively. All the caregivers (100%) possessed a smartphone and most of the caregivers (89.5%) had the experience of using smartphone or other technological devices to search for health or medical information. However, majority of the participants were not aware of the existence of any web-based or mobile application related to AAMDs (89.5%). While for the qualitative part, three themes emerged: (1) experience with current source of information; (2) needs for supporting self-management educational contents and needs for technological design application. Most of the caregivers used the nutritional booklet as sources of reference but some of them searched for web-based information. Features perceived by the caregivers included a digital food composition database, sharing diet recall with healthcare providers, self-monitoring diet intake as well as low protein recipes. Besides that, user-friendly and ease to use were also perceived as the important features by the caregivers. CONCLUSION: The identified features and needs by the caregivers should be integrated into the design of the apps to promote acceptance and usage.

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

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.

Effect of nutritional intervention on nutritional status among children with disorders of amino acid and nitrogen metabolism (AANMDs): A scoping review.

Disorders of amino acid and nitrogen metabolism (AANMDs) occur due to an enzyme deficiency in a normal biochemical pathway. Nutritional intervention is recognized as the mainstay of treatment for children diagnosed with AANMD. Hence, this scoping review aimed to identify the nutritional interventions available in managing AANMD disorders and their effects on nutritional status. A systematic search using PRISMA Extension for Scoping Reviews (PRISMA-ScR) method was conducted across 4 databases: PubMed, ScienceDirect (Elsevier), EBSCOhost and Cochrane Central Register of Controlled Trials (CENTRAL). Inclusion criteria for the study to be selected are: subjects aged less than 18-year-old, article published in English, utilized an experimental design and published within the past 20 years. A total of 22 articles were included in this review. The majority of the subjects are boys (55.6%) and employed a randomized controlled trial (RCT) study design (45.4%). Nutritional interventions were categorized into 4 categories which are: "protein substitute" (n = 5), "protein substitute with modified composition" (n = 6), "nutrient supplementation (n=8)", and "distribution and dosage of protein substitute (n = 3)". The most frequently assessed outcomes were biochemical parameters that gauge the effectiveness of metabolic control (68.2%). Overall, "protein substitute enriched with inhibitive amino acids", "long-chain polyunsaturated fatty acids supplementation", and "evenly distributed protein substitute" demonstrated beneficial effects towards the nutritional status, especially in terms of biochemical parameters. In summary, nutritional intervention plays a significant role in improving the nutritional status of AANMD patients. Further investigations of nutritional intervention among AANMD children using a meta-analysis approach are necessary for better comprehension of their impact in management of AANMD disorders.