Parenting a Child with Phenylketonuria (PKU): an Interpretative Phenomenological Analysis (IPA) of the Experience of Parents.

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.

Using the Repertory Grid Technique to Examine Trainee Clinical Psychologists' Construal of Their Personal and Professional Development.

The repertory grid technique was used to explore how 26 third-year trainee clinical psychologists construed their personal and professional selves over the course of training and into the future. Each trainee completed a demographic questionnaire and a repertory grid with 10 elements: four 'personal self' elements, four 'professional self' elements and two 'qualified clinical psychologist' elements. They then rated the 10 elements on 10 bipolar constructs of their choosing. Trainees' personal and professional selves were construed to be similar to each other. Trainees had low self-esteem and reported currently feeling anxious, stressed, unsettled and lacking an appropriate work-life balance. These difficulties were attributed to the demands of training and were expected to resolve once training was completed with future selves being construed as similar to ideal selves. Suggestions for future research with improved methodology are made, and the implications of the findings for trainees, training providers and employers of newly qualified clinical psychologists are given. The overall implication being that stress in training is normative and the profession has a duty to normalize this and ensure that self-care and personal development are recognized as core competencies of the clinical psychologist for the benefit of its members and their clients. Copyright © 2015 John Wiley & Sons, Ltd. KEY PRACTITIONER MESSAGE: Clinical psychology trainees experience training as demanding and stressful, which negatively impacts on their personal and professional self-image and self-esteem. However, they are optimistic that they will become more like their ideal self in the future. Stress in clinical training (and beyond) is normative, and thus, personal development and self-care should be recognized as clinical psychologist's core competencies.

Neuropsychological profiles of patients with juvenile myoclonic epilepsy and their siblings: a preliminary controlled experimental video-EEG case series.

OBJECTIVE: The aim of this study was to examine expressive language, memory, and higher executive tasks in patients with juvenile myoclonic epilepsy (JME) as compared with their siblings and a normal control group under video-EEG conditions. METHODS: Eight sibling pairs, one in each pair with JME, were compared with 16 controls matched for age, sex, ethnicity, and educational level. The participants were administered a standard comprehensive set of neuropsychological measures to assess emotional well-being, intelligence, memory, language, visuospatial skills, reaction time, attention, and higher executive function, during and without video-EEG recording. RESULTS: The JME group differed significantly from controls on measures of phonemic and semantic verbal fluency. They also scored significantly higher on the Dysexecutive Questionnaire, being much more likely to report traits associated with executive dysfunction. Qualitative inspection of the data suggested a trend for patients with JME and their siblings to perform worse than controls on some measures, notably those of expressive language and higher executive function, but on other measures the differences were not statistically significant. In contrast, the JME and sibling group performed as well as controls on the Rey Complex Figure and other tests of visuospatial skills. CONCLUSION: The present controlled experimental case series provides support for a neuropsychological profile of patients with JME and their siblings. Significant impairments in verbal and executive functioning may have important ramifications for the social, occupational, and academic performance of people with JME. The apparent corresponding likeness in cognitive performance, exhibited independent of EEG activity, between patients with JME and their siblings warrants further exploration as similar cortical and subcortical structures may be implicated in these two groups.

Using the repertory grid technique to explore the experience of compassion by mothers in a mother and baby unit.

Despite differences between Mother and Baby Units (MBUs) and other inpatient psychiatric settings, research has not yet explored the nature and value of compassionate care offered by MBU staff despite the increasing importance of compassion in healthcare. This novel study investigated the experience of compassionate care by fifteen mothers admitted to a MBU in England using the Repertory Grid Technique. Our findings indicated that these women perceived their MBU care as compassionate. Compassion was central to nursing care and clearly implicated in women's recovery from mental illness. Additionally, other staff characteristics were important to mothers, including how effectively MBU staff coped with stressful situations, staff flexibility in their care approach and how they adhered to professional boundaries. It is important to facilitate the expression of compassion which partially depends on the personalities and training of staff and the cooperation of service managers in fostering compassionate care.

Parenting a Child with Phenylketonuria: An Investigation into the Factors That Contribute to Parental Distress.

Phenylketonuria (PKU) is an inherited metabolic condition that can lead to the onset of intellectual disabilities if not strictly managed through a low-protein diet. Parents are responsible for supervising their child's treatment for PKU, which may impact on their experience of distress. This cross-sectional study aimed to identify the factors that contribute to distress in parents who care for a child with PKU, distinct from parents in the general population. Thirty-eight parents of children and adolescents with PKU and 32 parents in the general population completed the questionnaires measuring parental psychological resilience, child behaviour problems, perceived social support and distress. Parents of children with PKU also completed measures of their child's care dependency and behaviour related to developmental and intellectual disabilities. The findings revealed no statistically significant differences in distress between the groups, but parents of children with PKU reported more child behaviour problems. Multiple regression analysis identified that parental psychological resilience and child anxious behaviour explained 35% of the variance in distress for parents of children with PKU. By comparison, parental psychological resilience and generic child behaviour only accounted for 19% of the variance in distress for parents in the general population. This has implications for developing interventions in clinical settings that aim to reduce parents' distress by enhancing their psychological resilience and supporting them to manage child behaviour difficulties, particularly anxious behaviour. Future research should include larger, more diverse samples and use longitudinal study designs.

The attitudes of pregnant women and midwives towards raised BMI in a maternity setting: A discussion of two repertory grid studies.

OBJECTIVES: Weight-related stereotypes may have a detrimental impact on interactions between midwives and pregnant women with a body mass index (BMI) outside the recommended range of 18-30kg/m2. This paper explores the reciprocal construal of midwives and pregnant women with a raised BMI and considers the clinical implications of these constructs. PARTICIPANTS: Ten pregnant women with a BMI≥30kg/m2 and 11 midwives and from an inner city maternity service were recruited. INTERVENTION: Participants provided information that allowed for the creation of a repertory grid; generating psychological constructs (perceptions or attitudes) identifying similarities and differences between pregnant women and midwives across a BMI range. FINDINGS: Midwives were extremely conscious of being perceived as judgemental. They construed all pregnant women as anxious and vulnerable, but attributed characteristics such as "less health-conscious" and "complacent" to those with a raised BMI. The ideal pregnant woman and ideal midwife were typically construed as more likely to have a BMI of 18-30kg/m2. Pregnant women with a BMI≤18kg/m2 were construed as lacking warmth. While midwives differentiated between the elements based on role, the pregnant women construed the elements according to their BMI. Similarly, they construed those with a BMI≤18kg/m2 as having an undesirable personality, and acknowledged weight-related stereotypes for those with a raised BMI. CLINICAL IMPLICATIONS: It is possible these constructs impact on the way midwives care for and interact with women. Midwives may be supported through reflective clinical supervision and communication skills training to reduce the perceptions of stigma experienced by women with a raised BMI. It may be beneficial to involve pregnant women with a raised BMI in service development to ensure services meet their needs.

Anxiety in Asperger's syndrome: Assessment in real time.

Anxiety is a major problem for many people with Asperger's syndrome who may have qualitatively different fears from a non-Asperger's syndrome population. Research has relied on measures developed for non-Asperger's syndrome populations that require reporting past experiences of anxiety, which may confound assessment in people with Asperger's syndrome due to problems with autobiographical memory as are often reported in this group.Experience sampling methodology was used to record real-time everyday experiences in 20 adults with Asperger's syndrome and 20 neurotypical adults. Within-subject analysis was used to explore the phenomenology of thoughts occurring in people with Asperger's syndrome when they were anxious. Comparisons were made with the group that did not have Asperger's syndrome. The Asperger's syndrome group were significantly more anxious than the comparison group. Factors associated with feelings of anxiety in the Asperger's syndrome group were high levels of self-focus, worries about everyday events and periods of rumination lasting over 10 min. People in the Asperger's syndrome group also had a tendency to think in the image form, but this was not associated with feelings of anxiety. The results are discussed with reference to psychological models of Asperger's syndrome, cognitive models of anxiety and implications for psychological therapy for this group.

A systematic review of interventions used to treat catatonic symptoms in people with autistic spectrum disorders.

A systematic review was conducted to examine the efficacy of a range of treatments for autistic catatonia. The review identified 22 relevant papers, reporting a total of 28 cases including both adult and paediatric patients. Treatment methods included electroconvulsive therapy (ECT), medication, behavioural and sensory interventions. Quality assessment found the standard of the existing literature to be generally poor, with particular limitations in treatment description and outcome measurement. There is some limited evidence to support the use of ECT, high dose lorazepam and behavioural interventions for people with autistic catatonia. However, there is a need for controlled, high-quality trials. Reporting of side effects and adverse events should also be improved, in order to better evaluate the safety of these treatments.

An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III.

BACKGROUND: Mucopolysaccharidosis type-III (MPS III) is an autosomal recessive lysosomal storage disorder. It causes progressive physical and cognitive decline and has been linked to increased incidences of behavioural problems. METHODS: Data on the behaviour and adaptive skills of 20 children with MPS III and 25 children with intellectual disability (ID) (17 included in analysis) were gathered via parental report questionnaire. The frequencies of different types of behaviour displayed by children with MPS III and children with ID were compared across two age categories. RESULTS: The total frequency of challenging behaviours displayed by children aged 2-9 years with MPS III and ID was not significantly different. Behaviours associated with hyperactivity, orality, unusual body movements and inattention were seen significantly more frequently in 2-9 year olds with MPS III than in those with ID. Children aged 10-15 years with MPS III showed significantly fewer problem behaviours than a contrasting group with ID. The frequency of challenging behaviours displayed by children with MPS III and their adaptive skills was found to decrease with age. CONCLUSIONS: Behaviours relating to hyperactivity, orality, unusual body movements and inattention are part of the behavioural phenotype of the middle phase of MPS III. The late phase of MPS III is associated with low rates of problem behaviour and loss of adaptive skills. Therefore, families with a child with MPS III may benefit from a different type of clinical service when the child is aged 2-9 years, than when aged 10-15 years.

Validity of dementia care mapping on a neuro-rehabilitation ward: Q-methodology with staff and patients.

BACKGROUND: Measuring the quality of care for people using neuro-rehabilitation services is a complex area requiring reliable methods that account for variable communication abilities/cognitive functioning. Dementia Care Mapping (DCM) is an observational method widely used in dementia care to improve person-centred care, which may be usefully applied to neuro-rehabilitation settings. Evaluation is vital to determine the tool's acceptability in this setting. PURPOSE: To explore the views of staff/patients regarding whether the use of DCM is acceptable in a neuro-rehabilitation setting. METHOD: DCM was conducted on an acute neuro-rehabilitation ward. Q-methodology, a technique for extracting subjective opinions, was used with 23 staff and 10 patients on the ward to evaluate the acceptability of DCM. RESULTS: Factor analysis was performed separately for staff and patient Q-sorts. Each found a "consensus" factor where all participants indicated positive acceptability for the use of DCM. Further exploratory factors indicated that some staff/patients had additional views/concerns that were not captured by the first consensus factor. CONCLUSIONS: The results from this preliminary study are promising and indicate that DCM is potentially an acceptable tool to use in acute neuro-rehabilitation. Further research is needed to explore the acceptability of this tool more widely across neuro-rehabilitation settings. IMPLICATIONS FOR REHABILITATION: Person-centred care is widely acknowledged as being important in all care settings, including neurorehabilitation. Dementia Care Mapping was deemed to be an acceptable approach for improving the quality of person-centred care on the basis of the views of staff and patients in a neurorehabilitation ward. Dementia Care Mapping, with adaptations for neurorehabilitation settings, successfully provides an acceptable framework for measuring and improving the quality of person-centred care in this setting.

Recognition of emotion with temporal lobe epilepsy and asymmetrical amygdala damage.

PURPOSE: Impairments in emotion recognition occur when there is bilateral damage to the amygdala. In this study, ability to recognize auditory and visual expressions of emotion was investigated in people with asymmetrical amygdala damage (AAD) and temporal lobe epilepsy (TLE). METHODS: Recognition of five emotions was tested across three participant groups: those with right AAD and TLE, those with left AAD and TLE, and a comparison group. Four tasks were administered: recognition of emotion from facial expressions, sentences describing emotion-laden situations, nonverbal sounds, and prosody. RESULTS: Accuracy scores for each task and emotion were analysed, and no consistent overall effect of AAD on emotion recognition was found. However, some individual participants with AAD were significantly impaired at recognizing emotions, in both auditory and visual domains. CONCLUSIONS: The findings indicate that a minority of individuals with AAD have impairments in emotion recognition, but no evidence of specific impairments (e.g., visual or auditory) was found.