Phenylketonuria (PKU) is a rare inherited metabolic disorder which can cause neurological damage if left untreated. PKU is identified through newborn screening in developed countries, and treatment begins immediately to prevent these severe consequences. When a child is diagnosed, parents must assume immediate responsibility for the management of PKU and prevention of neurological damage. Quantitative studies have identified significant psychosocial stressors for parents, but little is known about how the parents experience this process. This study aimed to explore the experiences of parents of children with PKU under the age of two. It is the first study to examine these experiences in this way. Seven parents were interviewed about their experiences, and interpretative phenomenological analysis was used to analyse the data. Three main themes were identified: control, striving for normality and acceptance of PKU as a continuum. Links between the themes and processes underpinning the results were explored with relation to existing literature and theories from a clinical psychology perspective. The role of acceptance of PKU was central to the parent’s experiences. Clinical implications and suggestions for further research are discussed.Electronic supplementary materialThe online version of this article (10.1007/s10897-018-0227-7) contains supplementary material, which is available to authorized users.
Phenylketonuria (PKU), a rare metabolic disorder, causes cognitive impairment unless treated with a strict, protein-restricted diet, but few studies have examined the relationship between treatment compliance and parental wellbeing. In the present study, 46 primary caregivers of children with PKU completed measures of psychological distress, parenting stress (related to caring for a child with an illness), resilience, perceived social support and child dependency. Treatment adherence was assessed using the proportion of blood phenylalanine concentrations within target range in the preceding year. Results indicated that 59% of caregivers showed clinical levels of psychological distress, which was predicted by their parenting stress and resilience. Whilst the proportion of blood phenylalanine concentrations in range was not associated with parental distress, it was predicted by child age and caregiver's perceived support from family. Despite experiencing high levels of distress, the results indicated that caregivers' ability to adhere to treatment was not affected. Interventions to reduce parenting stress and boost caregiver resilience may have a positive effect on parental wellbeing. Additionally, interventions to promote treatment adherence benefit parents of older children, with a focus on promoting support from family members. Further research with larger sample sizes and longitudinal designs is needed to further establish causal mechanisms.
Purpose -The aim of this study is to test the hypothesis that Clozapine reduces rates of self injury and the use of restraint in a medium secure setting in female patients who have a diagnosis of borderline personality disorder (BPD) and mild learning disability.Design/methodology/approach -A retrospective method of data collection was used with a requirement of six months baseline data on rates of self injury and the use of restraint. Patients were required to remain on Clozapine for a minimum of six months.Findings -The mean number of incidents of self injury in the six months prior to Clozapine was 20.75 (SD 8.8) and 13.5 (SD 4.51) in the six months following Clozapine, a reduction of 35 per cent. The mean number of incidents of restraint in the six months prior to Clozapine was 17.75 (SD 6.86) compared with 9.25 (SD 4.96) in the six months following commencement of Clozapine therapy, a reduction of 48 per cent. The results were analysed using the paired t-test and the difference between the means (self injury and use of restraint) was not statistically significant. This study demonstrates a reduction in the rates of self injury and use of restraint in women with a diagnosis of BPD when treated with Clozapine which was clinically significant although not statistically significant.Originality/value -The authors are not aware of any published articles on the use of Clozapine in women with BPD and co-morbid learning disability.
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