The Effect of Nursing Empowerment Program
Condition: Phenylketonurias
MeSH terms: Phenylketonurias
Study type: Interventional

Summary: 
      The study has shown that the strengthening program applied to the families of children
      diagnosed with phenylketonuria (PKU) and the education given; It will be conducted in order
      to examine the effect of the family's coping attitudes, knowledge scores about the disease
      and its management. The research is of the randomized controlled experimental type. Children
      from PKU constitute the universe in the city center of Van. The families of children between
      the ages of 0-3 who were diagnosed with PKU will form the sample of the study. A nursing
      strengthening program will be applied to families in the experimental group for 10 weeks. The
      scope of this program; It consists of face-to-face training on disease management, giving the
      educational guide (booklet) for the families of babies diagnosed with PKU, telephone calls
      during the education process and counseling services. No application will be made to the
      families in the control group. After the application of the last tests to the control group;
      Face-to-face training and a booklet will be given to the experimental group. Data; It will be
      collected with 'Child and Family Introductory Information Form', 'Parents' Information Level
      Assessment Form about Phenylketonuria' and 'Coping Attitudes Assessment Scale'.

      In the literature, there is no study showing the effect of the education given to the
      families of babies diagnosed with PKU on their coping attitudes. Based on the need to fill
      this knowledge gap in the literature, it is thought that the study will contribute to the
      field of child health and disease nursing.
    

Detailed description: 
      Phenylketonuria is a disorder of phenylalanine metabolism and causes intellectual destruction
      if left untreated. It is an autosomal recessive metabolic disease that develops as a result
      of the absence of the enzyme phenylalanine hydroxylase (FAH), which provides the necessary
      amino acid metabolism for protein production in the body, which causes high phenylalanine in
      the blood. In our country, it is one of the places where PKU is commonly encountered
      (Çavuşoğlu, 2015; Hafid et al, 2015; Törner and Büyükgönenç, 2013). While the incidence of
      this disease in European countries is 1/10000-30000, it is 1/3000-4500 in our country
      (Alparslan, 2021). This situation is directly related to consanguineous marriages. It is
      stated that one out of every five marriages in our country is consanguineous marriage. If
      left untreated, it can cause extreme mental retardation, convulsions and death (Alparslan,
      2021; Çavuşoğlu, 2015; Hafid et al, 2015; Törner and Büyükgönenç, 2013).

      The care of the child with phenylketonuria is carried out by the parents. Extra care (diet
      therapy, control of blood phenylalanine level, etc.) for children in this diagnosis group is
      provided by their parents. The treatment and care process of children with PKU affects family
      routines, relationships and parenting styles. Parents of children experience emotional and
      economic burden. Therefore, it is stated that the care burden of parents in this diagnosis
      group is higher than that of healthy parents, and their quality of life is lower (Hatzmann et
      al, 2009). In addition, studies have shown that families of children with PKU have high
      levels of anxiety, depression (Mahmoudi-Gharaei et al. 2011) and stress (Irannejad et al.
      2018). In another study; It is reported that the family's routines have changed (not eating
      in front of the child, not eating in restaurants, etc.) and that they experience anxiety
      about the effect of diet incompatibility on blood phenylalanine levels, feeling overwhelmed
      and guilt in the care process (Carpenter et al 2018). Therefore, having a child with PKU may
      affect coping attitudes.

      Child health and diseases nurse should support children with PKU and their families on issues
      such as informing, training and counseling about the disease and its management.
    

Intervention name(s): Family education
Intervention type(s): Behavioral
Intervention description(s): By calling the families in the experimental group by phone; Nursing interventions for family empowerment will last for 10 weeks, they will be invited to the Family Health Center (FHC) or child metabolism polyclinic twice in this process, they will be called once a week (with 2 phone calls) for two weeks after the face-to-face training initiative in the first stage, In the second stage, at the end of the face-to-face training, they will be informed that they will be called once a week for two weeks (4 phone calls in total). The families in the experimental group will be informed that 4 weeks after the completion of the training and phone calls (at the 10th week), they will call the FHC or the pediatric metabolism polyclinic again for the application of the post-tests and that the nursing training for family empowerment will be completed.
Allocation: Randomized
Intervention model: Parallel Assignment
Intervention model description: The study was completed with the families of 36 children with PKU, 19 in the intervention group and 17 in the control group, who met the inclusion criteria.
Masking: None (Open Label)
Primary purpose: Supportive Care
Keywords: family; Coping; Education; Phenylketonuria(PKU); Empowerment; Nursing.