Özet:

Nutritional therapy and adaptation to this treatment are essential in Phenylketonuria (PKU), a congenital metabolic disease. This study was undertaken to determine the contribution of families in coping with problems that make nutritional adaptation difficult. Difficulties faced by families in achieving low-protein, low-phenylalanine-containing products that are inadequate and expensive to achieve proper nutrition in PKU disease,  the ways to cope with these difficulties, the problems that can be or are experienced in harmony with the environment of the patients, and the ways to cope with them consists the subject of the study. As a result of the study it is understood that it is necessary and important to educate the families, to prevent the marriage of the relatives and to raise the awareness of the community in order not to increase the prevalence of recessive diseases in general frame. In the private area, the support of the family, the care to be shown about the nutrition, the minimization of the negative effects of the environment, the self-confidence to be given to the patient can create cognitive health and a life without any problems. 

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