The effect of PKU diet on the maternal quality of life and social discrimination in relation to their educational status and place of living
-
Konstantinos Iakovou
,
Anna Madoglou
Abstract
Background
Phenylketonuria (PKU) is an inherited metabolic disorder characterized by high levels of phenylalanine in the blood and brain, resulting in mental retardation, etc. Dietary treatment with low phenylalanine is the common treatment for this disease. Patients with other metabolic disorders, such as diabetes mellitus, were reported to have a higher percentage of quality-of-life damage (QLD) and social discriminations (SDs).
Methods
To evaluate the degree (%) of maternal QLD and SD in relation to their educational status and place of living during the participation of their PKU children in public events, 110 mothers of PKU children with an average age of 25.7 years took part in this study. We evaluated their QLD and SD according to their educational status (primary school, high school and university) and place of living (small town ≤300,000, city >300,000 inhabitants). A control group was not needed. Special questions (checklist) were created to evaluate the QLD and SD of the mothers of children under dietary control. Cronbach’s α test was used for the measurement of the function of the items in the checklists.
Results
The covariance between the item pairs and the variance of the total score were calculated. Mothers who had completed primary school and lived in a city with a population >300,000 experienced the highest degree of QLD. In contrast, mothers with a university degree experienced the lowest SD. Overall, the highest SD was observed in mothers who lived in a small town. The affected group of mothers should be psychologically supported.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission. Konstantinos Iakovou designed the study. Ioannis Monopolis took care of the statistical analysis. Anna Madoglou oversaw the psychological and social part of the study. Kleopatra Schulpis supervised the entire study.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
Key Messages: Maternal psychological problems while following the PKU diet.
References
1. Donlon J, Levy H, Scriver C. Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. In: Scriver C, Beaudet A, Sly W, Valle D, editors. Metabolic and molecular basis of inherited disease. New York: McGraw-Hill, 2007. Chapter 77.Search in Google Scholar
2. NIH Phenylketonuria (PKU): screening and management. NIH Consensus Statement. 2000;17:1–33.Search in Google Scholar
3. Recommendations on the dietary management of phenylketonuria Report of Medical Research Council Working Party on Phenylketonuria. Arch Dis Child 1993;68:426–7.10.1136/adc.68.3.426Search in Google Scholar
4. National Institutes of Health Consensus Development Panel. National institutes of health consensus development conference statement: phenylketonuria: screening and management, October 16–18, 2000. Pediatrics 2001;108:972–82.10.1542/peds.108.4.972Search in Google Scholar
5. Guttler F, Azen C, Guldberg P, Romstad A, Hanley WB, et al. Relationship among genotype, biochemical phenotype, and cognitive performance in females with phenylalanine hydroxylase deficiency: report from the Maternal Phenylketonuria Collaborative Study. Pediatrics 1999;104:258–62.10.1542/peds.104.2.258Search in Google Scholar
6. Goldbeck L. The impact of newly diagnosed chronic paediatric conditions on parental quality of life. Qual Life Res 2006;15:1121–31.10.1007/s11136-006-0068-ySearch in Google Scholar
7. Zwiesele S, Bannick A, Trepanier A. Parental strategies to help children with phenylketonuria (PKU) cope with feeling different. Am J Med Genet A 2015;167A:1787–95.10.1002/ajmg.a.37088Search in Google Scholar
8. Streisand R, Tercyak KP. Parenting chronically ill children – the scope and impact of pediatric parenting stress. In: Handbook of parenting. Hoghughi MS, Long N, editors. London: London, 2004:181–97.Search in Google Scholar
9. Cohen MS. Families coping with childhood chronic illness: a research review. Fam Syst Health 1999;17:149–64.10.1037/h0089879Search in Google Scholar
10. Bilginsoy C, Waitzman N, Leonard C, Ernst S. Living with phenylketonuria: perspectives of patients and their families. J Inherit Metab Dis 2005;28:639–49.10.1007/s10545-005-4478-8Search in Google Scholar
11. Lord B, Wastell C, Ungerer J. Parents reactions to childhood phenylketonuria. Fam Syst Health 2005;23:204–19.10.1037/1091-7527.23.2.204Search in Google Scholar
12. Anderson RM, Funnell MM, Butler PM, Arnold MS, Fitzgerald JT, et al. Patient empowerment: result of a randomized controlled trial. Diabetes Care 1995;18:943–9.10.2337/diacare.18.7.943Search in Google Scholar
13. Pibernik-Okanovic M, Prasek M, Poljicanin-Filipovic T, Pavlic-Renar I, Metelko Z. Effects of an empowerment-based psychosocial intervention on quality of life and metabolic control in type 2 diabetic patients. Patient Educ Couns 2004;52:193–9.10.1016/S0738-3991(03)00038-7Search in Google Scholar
14. Anderson R, Funnell M. The art of empowerment, 2nd ed. New York: American Diabetes Association, 2005.Search in Google Scholar
15. Viklund G, Ortqvist E, Wikblad K. Assessment of an empowerment education programme. A randomized study in teenagers with diabetes. Diabet Med 2007;24:550–6.10.1111/j.1464-5491.2007.02114.xSearch in Google Scholar
16. Group W. The World Health Organization quality of life assessment (WHOQOL): position paper from the World Health Organization. Soc Sci Med 1995;41:1403–9.10.1016/0277-9536(95)00112-KSearch in Google Scholar
17. Costanza R, Fisher B, Ali S, Beer C, Bond L, et al. Quality of life: an approach integrating opportunities, human needs, and subjective well-being. Ecol Econ 2007;61:267–76.10.1016/j.ecolecon.2006.02.023Search in Google Scholar
18. Hernandez CA, Antone I, Cornelius I. A grounded theory study of the experience of type 2 diabetes mellitus in First Nation adults in Canada. J Transcult Nurs 1999;10:220–8.10.1177/104365969901000313Search in Google Scholar PubMed
19. Abdoli S, Ashktorab T, Ahmadi F, Parvizi S, Dunning T. The empowerment process in people with diabetes: an Iranian perspective. Int Nurs Rev 2008;55:447–53.10.1111/j.1466-7657.2008.00664.xSearch in Google Scholar PubMed
20. Abdoli S. Diabetes: “defect point” or “positive opportunity”. Middle East J Nurs 2011;5:22–7.Search in Google Scholar
21. Shestak A. US: the impact of stigma on reactions to an individual with type 1 diabetes, in nursing school, Carnegie Mellon University; Available from: http://shelf1.library.cmu.edu/HSS/a986117/a986117.pdf.Search in Google Scholar
22. Naemiratch B, Manderson L. ‘Normal, but…’: living with type 2 diabetes in Bangkok, Thailand. Chronic Illn 2008;4:188–98.10.1177/1742395308090069Search in Google Scholar PubMed
©2019 Walter de Gruyter GmbH, Berlin/Boston
Articles in the same Issue
- Frontmatter
- Original Articles
- Improved adherence and growth outcomes with jet-delivered growth hormone
- Validation of the Greek version of the Quality of Life in Short Stature Youth (QoLISSY) questionnaire
- Growth hormone deficiency in children with antenatal Bartter syndrome
- Physical growth and development characteristics of children with Williams syndrome aged 0–24 months in Zhejiang Province
- Adiposity and adipocytokines: the moderator role of cardiorespiratory fitness and pubertal stage in girls
- Serum omentin-1 levels in obese children
- A pilot study on newborn screening for congenital adrenal hyperplasia in Beijing
- Typical characteristics of children with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency: a single-centre experience and review of the literature
- Studying the effect of large neutral amino acid supplements on oxidative stress in phenylketonuric patients
- The effect of therapy on plasma ghrelin and leptin levels, and appetite in children with iron deficiency anemia
- The effect of PKU diet on the maternal quality of life and social discrimination in relation to their educational status and place of living
- Evaluating the four most important salivary sex steroids during male puberty: testosterone best characterizes pubertal development
- Case Reports
- Identification of two novel TPK1 gene mutations in a Chinese patient with thiamine pyrophosphokinase deficiency undergoing whole exome sequencing
- A rare case of congenital hyperinsulinism (CHI) due to dual genetic aetiology involving HNF4A and ABCC8
- Cinacalcet therapy in an infant with an R185Q calcium-sensing receptor mutation causing hyperparathyroidism: a case report and review of the literature
Articles in the same Issue
- Frontmatter
- Original Articles
- Improved adherence and growth outcomes with jet-delivered growth hormone
- Validation of the Greek version of the Quality of Life in Short Stature Youth (QoLISSY) questionnaire
- Growth hormone deficiency in children with antenatal Bartter syndrome
- Physical growth and development characteristics of children with Williams syndrome aged 0–24 months in Zhejiang Province
- Adiposity and adipocytokines: the moderator role of cardiorespiratory fitness and pubertal stage in girls
- Serum omentin-1 levels in obese children
- A pilot study on newborn screening for congenital adrenal hyperplasia in Beijing
- Typical characteristics of children with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency: a single-centre experience and review of the literature
- Studying the effect of large neutral amino acid supplements on oxidative stress in phenylketonuric patients
- The effect of therapy on plasma ghrelin and leptin levels, and appetite in children with iron deficiency anemia
- The effect of PKU diet on the maternal quality of life and social discrimination in relation to their educational status and place of living
- Evaluating the four most important salivary sex steroids during male puberty: testosterone best characterizes pubertal development
- Case Reports
- Identification of two novel TPK1 gene mutations in a Chinese patient with thiamine pyrophosphokinase deficiency undergoing whole exome sequencing
- A rare case of congenital hyperinsulinism (CHI) due to dual genetic aetiology involving HNF4A and ABCC8
- Cinacalcet therapy in an infant with an R185Q calcium-sensing receptor mutation causing hyperparathyroidism: a case report and review of the literature
