الفهرس 
An overview about Phenylketonuria (PKU)Only 14 pages are availabe for public view
 |  | from | 219 |  |  |
| | | from | 219 | | |
Abstract
Children with PKU acquire the usual assortment of childhood illnesses, which might be accompanied by decreased intake of food and fluids. While this is often mild and self-limited, more severe or long lasting illnesses might require special management. When children with PKU decrease their energy and protein intake, they will experience some muscle catabolism to meet their immediate needs for glucose and protein synthesis. Because muscle contains substantial amounts of Phe, this will result in elevated Phe levels during illness. Ideally, this is managed by encouraging continued intake of low-Phe, higher energy–containing fluids, generally in the form of PKU formula (Meads, McKenna & Kahler, 2016).
Although many children with PKU have no obvious school or behavioral issues, some experience challenges, and the emergence of a picture resembling attention deficit disorder (ADD) is not uncommon. While children with PKU and ADD are often treated for ADD using the same approaches that are used in other children, it can be extremely valuable to obtained tailed neuropsychological testing to inform school program planning and optimize therapy. Consultation with the metabolic center might be helpful in ensuring access to appropriate testing and follow-up (Ten Hoedt et al., 2018).
So, the current study aimed to assess lifestyle for children with phenylketonuria.
A descriptive research design was used to carry out this study. This study was conducted at genetic counseling clinic in basic health care unit in Fayoum affiliated to of MOH. A purposive sample that included all children have PKU composed of 79 child accompanied by their mothers will attend to the previously mentioned setting according to the following criteria; Children aged from year to 5 years and free from other medical diseases such as (DM, Heart diseases, Hepatitis B). Data collection for the current study was collected using two data collection tools namely; interviewing questionnaire and lifestyle assessment Questionnaire for children with PKU.
An Interviewing Questionnaire sheet: (Appendix I): It designed by researcher after reviewing related literature and translated into Arabic language it consists of two parts
Part I: It concerned with demographic characteristics of child, such as age, sex, educational level, and child ranking. Socio- demographic characteristics of mothers regarding age, sex, relative degree, marital status, educational level, occupation, and family monthly income.
Part II: It related to medical history of children with PKU, including duration, discovery of the disease, follow up frequency per month, the degree to follow up, history of family members. And the relationship of family member suffering from the disease and complications of the disease and measurement (high, weight, body mass and phe serum level).
Lifestyle assessment Questionnaire for children with PKU: (Appendix II): It was adapted from Naturopathic foundation health clinic, (2014) and used to identify current strengths of child’s health, any risk factors that might be present, and highlight recommendations that may want to consider. It consisted of nine categories, general information, mothers’ health during pregnancy, family history and information, first few years of child life, past and present health concerns, general information on diet, atypical day for child, understanding child patterns of behavior, review of physical system.
Data Collection Procedure
Data collection of the study was started at the beginning of October 2018 and completed by the end of April 2019. The researcher attended at genetic counseling clinic in basic health care unit two days per week from 9am to 2pm and two day per week in Fayoum affiliated to MOH for all children have PKU composed of 79 child accompanied by their mothers will attend to the previously mentioned setting according to the following criteria; Children aged from year to 5 years and free from other medical diseases such as (DM, Heart diseases, Hepatitis B). The researcher first explained the aim of the study to the mothers and reassures them that information collected will be treated confidentiality and that it will be used only for the purpose of the research.
The present study revealed the following main results:
• (43%) of children’s age were 2:<3 years.
• (54.4%) of children were females.
• (45.6%) of children were normal in body mass index.
• (44.3%) of mother’s age were 25:<30 years.
• (89.9%) of children were live with extended family.
• (88.6%) of children were from rural.
• (83.5%) of mothers were housewife, and 92.4% of mothers were married.
• (74.7%) of families were discover the disease by MOH screening programs.
• (92.4%) of children were made regular follow up.
• (78.5%) of children had follow up visits once monthly.
• (89.9%) of children were not Taking breast feeding.
• (62%) of mothers had inadequate knowledge about the relation between breast feeding and the disease.
• (74.6%) of children was taking Nutary 2 of artificial milk.
• (95.8%) of families were getting artificial milk from nutrition clinic.
• (63.3%) of children were taking three meals per day and the largest meal was lunch (55.7%).
• (43%) of preferred food for children were Carbohydrates (rice – bead).
• (78.5%) of children were take the last meal from 6pm to 10pm.
• There is a statistically significant relation between giving breast feeding for children and residence of mothers.
• While there is no significant relation with their ages, educational level and type of the family.
• There is a highly statistically significant correlation between compliance with the prescribed diet and effect of the disease on general health.
• There is a statistically significant correlation between compliance with the prescribed diet and growth problems.
• There is no statistically significant correlation between compliance with the prescribed diet and sleep problems.
Conclusion
The current study concluded that:
from the result of present study, it can be concluded that phenylketonuria had a negative effects on the children’s life style especially growth and development, general health while had slight effects on sleep pattern of children.
Recommendation:
In the light of the results of this study, the following recommendations were suggested:
• Emphasis the importance of regular follow up for mothers of children with Phenylketonuria for contentious assessment and meet their actual need to improve their lives.
• Encourage children enrolment to nursery to increase their self-confidence through mass media as television, newspaper, posters, booklets and other communicating channels.
• Encourage behavioral adjustment of children suffering from Phenylketonuria through, decrease stressing events for children to avoid nervous behavior, make children’s diet more attractive to encourage them to eat, improve home environment that support good behavior and increase teacher’s awareness about diet and behavior of children.