Challenges of Family of Children with Phenylketonuria and its Impact on their Mental Health

Introduction: Phenylketonuria (PKU) is one of the most common metabolic disorders in children. Following the diagnosis of PKU and the child s need for lifelong diet, parents should take immediate responsibility for managing the disease to prevent the adverse consequences of the illness, including mental retardation of the child which is stressful for the family and their mental health and subsequent child health. Therefore, the aim of this article is to identify the challenges of family of child with PKU and its impact on their mental health.Materials & Methods: This article was a review study that done by searching literature published from 2015 to 2019 by using keywords such as: Phenylketonuria, PKU, Family, Mental Health, Challenges and Care Needs, Infant, Children in both languages, Farsi and English, in data bases such as: Web of Science, PubMed, Google Scholar, SID and Magirane. A total of 100 articles were found and 20 articles related to the subject under study were carefully selected and analyzed.Results: The results of most studies indicate the challenges and damages of mental health of family and children with PKU. After reviewing the findings, these challenges were categorized into four general groups based on the age of the child including: 1. Neonatal challenges such as the management of lactation based on blood phenylalanine levels 2. Challenges related to the age of 1 to 8 years, such as imposing diet and parental conflict with the child to avoid eating some of the child s favorite foods and learning difficulties in some school subjects 3. Challenges related to the age of 8 to 21 years such as inability to manage diet and negative emotions related to lack of independence and different diet from family and peers and acceptance of the disease 4. Challenges related to over 21 years of age such as problems with Pregnancy in girls. In addition, the results of studies indicated problems such as anxiety and depression in parents, impaired family cohesion, poor quality of life, lack of access to appropriate health care, and adequate medical counseling. A detailed explanation of these challenges and their impact on family mental health will be presented in the main article in Congress.Conclusion & discussion: Based on the results of studies on the challenges and problems of mental health in these families, the attention of the nursing team especially nurses especially in family-centered and holistic care planning from birth until the child s puberty is emphasized. Nurses, collaboration with other care and treatment team members, are required to prepare the family to face the challenges consciously by receiving adequate support and information and to empower parents, especially mothers. The authors suggest appropriate treatment and care plan for this group based on the challenges identified in this study.

کلیدواژه ها:

Phenylketonuria ، Challenges ، Family ، Mental Health ، Child