Nursing Care and Quality of Life Among Children with Hepatolenticular Degeneration

Hepatolenticular degeneration also known as Wilson’s disease (WD), is an autosomal recessive inherited copper metabolic disorder. Objective: The study aimed to determine the relationship between nursing care and the quality of life among children with hepatolenticular degeneration in Anhui Province, China. Methods: This research used the descriptive-correlational design. The researcher investigated 45 regular staff nurses and 96 children with hepatolenticular degeneration ages 12 and 18 years old. Two instruments were used. First, the Nursing Care Score Tool was developed by the researcher based on review of related the literature which is used to measure nursing care. Second, the revised PedsQLTM4.0 Generic Core Scales (Pediatric Quality of Life Inventory™ Version 4.0) is used to measure the quality of life among children with hepatolenticular degeneration. The data were analyzed using the following: Frequency, Percentage distribution, Mean, Median scores, IQR (Inter Quartile Range), One-Way Analysis of Variance (ANOVA), t-Test and Pearson Coefficient. Results: In terms of quality of life, except for age, significant differences were found in terms of children’s sex and educational status. Except for knowledge, there were significant differences in terms of physiological care, medication, psychological care, therapeutic communication and functional training as rated by the children and nurses. There was a significant positive correlation (r = .583, p = .000) between nursing care as rated by the children and quality of life of children with hepatolenticular degeneration. Conclusion: Nursing care is positively correlated with quality of life. A closer look at the results revealed that the quality of life of female children is generally lower than that of male, hence, more attention is recommended to be given to female children in improving their quality of life. Children with hepatolenticular degeneration should be encouraged to continue to receive education for a better quality of life. It is necessary for nurses to continuously explain to the child the disease process and the signs and symptoms of the disease. Careful observation of the physiological status of the child, the provision of timely care, regular functional training, making accurate judgements in the response to medications guidance in the intake of oral injectable medications, regular provision of psychological care and therapeutic communication to the children with this condition important tasks of the nurses. All these can lead to having these children live a better quality of life.