Phenylketonuria patients’ and their parents’ accep

Authors: Ewa Witalis Bożena Mikoluc Radoslaw Motkowski Justyna Szyszko Agnieszka Chrobot Bozena Didycz Agata Lange Renata Mozrzymas Andrzej Milanowski Maria Nowacka Mariola PiotrowskaDepta Hanna Romanowska Ewa Starostecka Jolanta Wierzba Magdalena Skorniewska Barbara Iwona WojcickaBartlomiejczyk Maria Gizewska Polish Society of Phenylketonuria

Publish Date: 2016/05/31

Volume: 25, Issue: 11, Pages: 2967-2975

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Abstract

Regarding attitude towards the disease our study demonstrated that 63 289  PKU patients did not accept the disease Patients who found accepting the disease difficult more frequently perceived themselves as inferior/different in comparison with their peers In total 36  of patients did not want their friends to be aware of their condition while only 18  of parents believed that their children’s peers should not know about their disease In total 42  of parents wanted to talk to other parents of PKU children and only 13  to a doctor Only 20  of patients saw the need to discuss their condition with a doctor In total 8  of children regardless of age and 14  of parents preferred to talk to a psychologistOur data demonstrated that disease acceptance played an essential role in patients’ social integration The study also indicated the need to overcome communication barriers between patients and their healthy peers and for patients to find the courage to be open about the disease The importance of support groups for PKU families and the significance of strict cooperation between patients and their families with PKU treatment teams were also revealedPhenylketonuria PKU OMIM261600 first recognised by Dr Asbjørn Følling in 1934 and considered a prime example of predictive medicine still poses a therapeutic challenge for both patients and medical professionals Although the dietary management currently recommended enables PKU patients to achieve full personal and social development for parents of an affected child the diagnosis means living in a state of constant anxiety The anxiety which results from the necessity of adhering to the rules imposed by the disease is often present from the first days of the child’s lifeThe disease forces initially parents and subsequently patients to learn the principles of nutritional management which includes a daily meticulous control of food intake and a strict adherence to the prescribed health regimen This means that from early infancy the implications of the condition shape the social and psychological aspects of PKU patients’ family life Therefore a subjective assessment of the quality of life by PKU patients and their families is undoubtedly one of the crucial factors which influences the effectiveness of their therapy In the case of children it is the behaviour of adults and their attitude to the disease that affects the outcome of dietary management and the children’s acceptance of the diseaseIn 1993 The World Health Organization and The International Association for Child Psychology and Psychiatry recommended selfassessment as the most appropriate method to measure children’s quality of life Hence information on the assessment of PKU patients’ and their families’ quality of life should enable the introduction of measures which would facilitate the effective implementation of dietary management standards and improve the families’ quality of life

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