Self-estimation of phenylketonuria patients on therapeutic diet. Psychological support.

Objectives Self-esteem is the degree to which the qualities and characteristics contained in one's self-concept are perceived to be positive. The aim of this study was to evaluate the self-esteem scores in phenylketonuria (PKU) patients on "strict", "loos" and "off diet". Sixty PKU patients were divided in three equal groups. Methods Group a: "on strict", group b: "on loos" and group c: "off diet". A special questionnaire for self-esteem scores was created for these patients. Results Before psychological support, group a patients demonstrated 6/20 (30%) very high self-esteem, 9/20 (45%) high and 5/25 (25%) moderate. After support 14/20 (70%) were turned to very high, 5/20 (25%) represented high except one whose degrees remained an altered. group b 4/20 (20%) were very high, 7/20 (35%) were high, 3/20 (15%) moderate and the rest of them showed low self-esteem degrees, after support, 10/20 (50%) showed very high, 5/20 (25%) became high, 3/20 (15%) turned to moderate and 2/20 (10%) remained unaltered. Group c, 1/20 (5%) were very high self-esteemed, 7/20 (35%) were high, 6/20 (30%) were moderate and 6/20 (30%) with low self-esteemed, at the end of support, 6/20 (30%) become very high, 8/20 (40%) with high, 4/20 (20.0%), moderate self-esteem whereas the rest were unaltered. Conclusions Very high and high self-esteem degrees were demonstrated in patients who follow their PKU diet. Moderate and low self-esteem degrees were predominantly found in patients on loos and or off diet. Psychological supports commonly result in amelioration of self-esteem degrees.

Self-estimation of phenylketonuria patients on therapeutic diet. Psychological support.

Objectives Self-esteem is the degree to which the qualities and characteristics contained in one's self-concept are perceived to be positive. The aim of this study was to evaluate the self-esteem scores in phenylketonuria (PKU) patients on "strict", "loos" and "off diet". Sixty PKU patients were divided in three equal groups. Methods Group a: "on strict", group b: "on loos" and group c: "off diet". A special questionnaire for self-esteem scores was created for these patients. Results Before psychological support, group a patients demonstrated 6/20 (30%) very high self-esteem, 9/20 (45%) high and 5/25 (25%) moderate. After support 14/20 (70%) were turned to very high, 5/20 (25%) represented high except one whose degrees remained an altered. group b 4/20 (20%) were very high, 7/20 (35%) were high, 3/20 (15%) moderate and the rest of them showed low self-esteem degrees, after support, 10/20 (50%) showed very high, 5/20 (25%) became high, 3/20 (15%) turned to moderate and 2/20 (10%) remained unaltered. Group c, 1/20 (5%) were very high self-esteemed, 7/20 (35%) were high, 6/20 (30%) were moderate and 6/20 (30%) with low self-esteemed, at the end of support, 6/20 (30%) become very high, 8/20 (40%) with high, 4/20 (20.0%), moderate self-esteem whereas the rest were unaltered. Conclusions Very high and high self-esteem degrees were demonstrated in patients who follow their PKU diet. Moderate and low self-esteem degrees were predominantly found in patients on loos and or off diet. Psychological supports commonly result in amelioration of self-esteem degrees.

The beneficial effect of psychological support on mothers with PKU children who suffer from social discrimination and damage of quality of life.

Background Phenylketonuria (PKU) is a metabolic disorder of phenylalanine (phe) metabolism caused by the liver enzyme phe hydroxylase deficiency resulting in mental retardation. The only treatment for this disorder is the lifelong restriction of natural protein supplemented with phe-free amino acid formula. The aim of this study was to demonstrate the beneficial effect of psychological support in relation to the educational status on mothers with PKU children, who suffered from social discrimination (SD) and damage of quality of life (QLD). Methods A total number of 42 mothers with PKU children with a mean age of 26.8 years took part in this study. According to their educational status, they were divided into three groups: group a, primary school (n = 13); group b, high school (n = 15) and group c, university degree (n = 14). Psychological support was given once per week for 1 year. Results University degree mothers achieved the best results in ameliorating their symptoms and signs. Mothers who had completed high school felt a moderate amelioration of their SD and QL, whereas those who had finished primary school only felt a mild reduction of their symptoms. Educational status of the affected mothers should be always taken into account for the classification of the affected mothers into therapeutic groups. Familial and professional environment are very important for the success of psychological support. Conclusions Overall, the psychological support related to symptoms and signs of this metabolic disorder is in comparison with that in diabetic patients.

Replacement of breastfeeding with medical food for the treatment of galactosemia and phenylketonuria: maternal stress.

Breastfeeding replacement is the only treatment for galactosemia (GAL) and phenylketonuria (PKU) during infancy. We aimed to evaluate the stress degree in mothers who were obliged to replace breastfeeding with special formulas as the only treatment for the diseased newborns. Thirty-two mothers with GAL newborns, 19 on breastfeeding only and 13 on breastfeeding plus formula, participated in this study. Additionally, 54 mothers with PKU infants, 32 offered breastfeeding only and 22 breastfeeding plus formula, participated in the study. Stress degree was evaluated in both groups: GAL and PKU. Mothers on breastfeeding only experienced the highest degree of stress than those who were on breastfeeding plus formula. After 1 month of psychological support, most mothers were ameliorated: mothers on breastfeeding only felt better as compared to those on breastfeeding plus formula. Conversely, in mothers on breastfeeding plus formulas, symptoms and signs of stress almost disappeared. In conclusion, GAL or PKU mothers with breastfeeding only experienced the highest degree of stress when asked for breastfeeding replacement. Psychological support made all the studied groups to feel better or free of symptoms and signs of stress.