Hungry for Change: The Experiences of People with PKU, and Their Caregivers, When Eating Out.

For patients with phenylketonuria (PKU), stringent dietary management is demanding and eating out may pose many challenges. Often, there is little awareness about special dietary requirements within the hospitality sector. This study's aim was to investigate the experiences and behaviours of people with PKU and their caregivers when dining out. We also sought to identify common problems in order to improve their experiences when eating outside the home. Individuals with PKU or their caregivers residing in the UK were invited to complete a cross-sectional online survey that collected both qualitative and quantitative data about their experiences when eating out. Data were available from 254 questionnaire respondents (136 caregivers or patients with PKU < 18 years and 118 patients with PKU ≥ 18 years (n = 100) or their caregivers (n = 18)). Fifty-eight per cent dined out once per month or less (n = 147/254) and the biggest barrier to more frequent dining was 'limited choice of suitable low-protein foods' (90%, n = 184/204), followed by 'no information about the protein content of foods' (67%, n = 137/204). Sixty-nine per cent (n = 176/254) rated their dining experience as less than satisfactory. Respondents ranked restaurant employees' knowledge of the PKU diet as very poor with an overall median rating of 1.6 (on a scale of 1 for extremely poor to 10 for extremely good). Forty-four per cent (n = 110/252) of respondents said that restaurants had refused to prepare alternative suitable foods; 44% (n = 110/252) were not allowed to eat their own prepared food in a restaurant, and 46% (n = 115/252) reported that restaurants had refused to cook special low-protein foods. Forty per cent (n = 101/254) of respondents felt anxious before entering restaurants. People with PKU commonly experienced discrimination in restaurants, with hospitality staff failing to support their dietary needs, frequently using allergy laws and concerns about cross-contamination as a reason not to provide suitable food options. It is important that restaurant staff receive training regarding low-protein diets, offer more low-protein options, provide protein analysis information on all menu items, and be more flexible in their approach to cooking low-protein foods supplied by the person with PKU. This may help people with PKU enjoy safe meals when dining out and socialising with others.

Provision and Supervision of Food and Protein Substitute in School for Children with PKU: Parent Experiences.

Children spend a substantial part of their childhood in school, so provision of dietary care and inclusion of children with phenylketonuria (PKU) in this setting is essential. There are no reports describing the dietary support children with PKU receive whilst at school. The aim of this cross-sectional study was to explore the experiences of the dietary management of children with PKU in schools across the UK. Data was collected using an online survey completed by parents/caregivers of children with PKU. Of 159 questionnaire responses, 92% (n = 146) of children attended state school, 6% (n = 10) private school and 2% (n = 3) other. Fourteen per cent (n = 21/154) were at nursery/preschool, 51% (n = 79/154) primary and 35% (n = 54/154) secondary school. Sixty-one per cent (n = 97/159) said their child did not have school meals, with some catering services refusing to provide suitable food and some parents distrusting the school meals service. Sixty-one per cent of children had an individual health care plan (IHCP) (n = 95/155). Children were commonly unsupervised at lunchtime (40%, n = 63/159), with snacks (46%, n = 71/155) and protein substitute (30%, n = 47/157), with significantly less supervision in secondary than primary school (p < 0.001). An IHCP was significantly associated with improved supervision of food and protein substitute administration (p < 0.01), and better communication between parents/caregivers and the school team (p < 0.05). Children commonly accessed non-permitted foods in school. Therefore, parents/caregivers described important issues concerning the school provision of low phenylalanine food and protein substitute. Every child should have an IHCP which details their dietary needs and how these will be met safely and discreetly. It is imperative that children with PKU are supported in school.

Accidental Consumption of Aspartame in Phenylketonuria: Patient Experiences.

Aspartame is a phenylalanine containing sweetener, added to foods and drinks, which is avoided in phenylketonuria (PKU). However, the amount of phenylalanine provided by aspartame is unidentifiable from food and drinks labels. We performed a cross-sectional online survey aiming to examine the accidental aspartame consumption in PKU. 206 questionnaires (58% female) were completed. 55% of respondents (n = 114) were adults with PKU or their parent/carers and 45% (n = 92) were parents/carers of children with PKU. 74% (n = 152/206) had consumed food/drinks containing aspartame. Repeated accidental aspartame consumption was common and more frequent in children (p < 0.0001). The aspartame containing food/drinks accidentally consumed were fizzy drinks (68%, n = 103/152), fruit squash (40%, n = 61/152), chewing gum (30%, n = 46/152), flavoured water (25%, n = 38/152), ready to drink fruit squash cartons (23%, n = 35/152) and sports drinks (21%, n = 32/152). The main reasons described for accidental consumption, were manufacturers' changing recipes (81%, n = 123/152), inability to check the ingredients in pubs/restaurants/vending machines (59%, n = 89/152) or forgetting to check the label (32%, n = 49/152). 23% (n= 48/206) had been prescribed medicines containing aspartame and 75% (n = 36/48) said that medicines were not checked by medics when prescribed. 85% (n = 164/192) considered the sugar tax made accidental aspartame consumption more likely. Some of the difficulties for patients were aspartame identification in drinks consumed in restaurants, pubs, vending machines (77%, n = 158/206); similarities in appearance of aspartame and non-aspartame products (62%, n = 127/206); time consuming shopping/checking labels (56%, n = 115/206); and unclear labelling (55%, n = 114/206). These issues caused anxiety for the person with PKU (52%, n = 106/206), anxiety for parent/caregivers (46%, n = 95/206), guilt for parent/carers (42%, n = 87/206) and social isolation (42%, n = 87/206). It is important to understand the impact of aspartame and legislation such as the sugar tax on people with PKU. Policy makers and industry should ensure that the quality of life of people with rare conditions such as PKU is not compromised through their action.

Prescribing issues experienced by people living with phenylketonuria in the UK.

BACKGROUND: Foods for Special Medical purposes (Phe-free protein substitutes [PS] and low protein foods [LPFs]) are central to successful dietary management of patients with phenylketonuria (PKU). In the UK, PS and LPFs are prescribed by primary care general practitioners (GPs) and dispensed by high street pharmacists or via home delivery companies. As patients and carers commonly reported access issues with PS and LPFs through the National Society for PKU (NSPKU) helpline, the NSPKU collected more information about the extent of prescriptions issues via a questionnaire. METHODS: A cross sectional online survey was carried out consisting of 7 multiple choice and 3 open questions. The questionnaire was available via the NSPKU website over a 9-month period (15th May 2018-31st January 2019). Carers or patients with PKU were invited to participate if they had any problems accessing PS and LPF. RESULTS: There were 252 responses. 65% for children and 35% for adults, aged ≥18y with PKU. 59% (n = 146/246) of responses described difficulties accessing basic LPF (bread, pasta) and 33% (n = 81/248) PS. 36% (n = 88/248) said problems had re-occurred >1y, and 40% (n = 99/248) for <3 months. 59% (n = 146/246) had supply issues with LPF and 33% (n = 81/246) with PS. 18% (n = 42/234) reported that the local NHS authority had refused, restricted or had a policy to block treatment access; 27% (n = 63/234) cited GPs declining requests or restricting prescription amounts. 21% (n = 49/234) could not access all their products via a pharmacy, 40% (n = 93/234) said their home delivery system failed, and 15% (n = 34/234) of the dispensed prescribed products were out of date, incorrect, damaged or poor quality. For patients and carers these issues caused: stress (87%, n = 210/241); they considered PKU was not taken seriously by professionals (66%, n = 160/241); they reduced patient access to LPF and PS (53%, n = 128/241); and affected the patients' health (53%, n = 128/241). DISCUSSION: Access to PKU dietary treatment is frequently disrupted for reasons relating to GP prescriptions and unreliable dispensing and delivery practices. These issues require immediate attention by UK health professionals in order to create a fairer and more efficient system for everyone.

HOw Patients view Extended half-life products: Impressions from real-world experience (The HOPE study).

INTRODUCTION: Extended half-life (EHL) clotting factors have been shown to offer people with haemophilia (PwH) protection from bleeding with fewer infusions, which might reduce treatment burden. AIM: The HOw Patients view Extended half-life products (HOPE) study aimed to explore, understand and describe patient expectations around the prophylactic use of EHL products and to establish whether these expectations were met through individual follow-up analysis. METHODS: The HOPE study was a prospective, qualitative cohort study conducted among PwH who had switched to Fc fusion protein EHL products in routine clinical care and who had not been recruited to clinical trials of these products. Semi-structured audio-recorded interviews were undertaken over two time points; transcripts were analysed to systematically generate theory from data that contains both inductive and deductive thinking. RESULTS: Forty-three interviews were conducted with 25 participants. Most participants were positive about EHL treatment and intended to continue using them. Reduced frequency of infusions meant lives were less disrupted or dominated by haemophilia, and there was less perceived stress on overused veins. For those PwH who did not reduce infusion frequency, there were other perceived benefits from EHLs with respect to greater protection with higher trough levels and fewer bleeds. CONCLUSION: Patients switching to EHL treatments believe these products will result in fewer infusions and less disruption of everyday life, leaving them feeling more protected with fewer bleeds and increased activity levels, as well as enhanced well-being and mental health. Understanding patient expectation and experience around using products adds real-world data to clinical trial experience.

Reproductive experience of women living with phenylketonuria.

INTRODUCTION: Many women with PKU are well-informed about the risks of maternal PKU but there are several barriers to achieving satisfactory metabolic control before and during pregnancy. Many studies have documented the outcome of maternal PKU infants, but very little has been reported about the experiences of women of reproductive age with PKU, particularly about their psychosexual development, pre-conception, pregnancy and postnatal experience. METHODS: In the UK, in a subsection of an online questionnaire conducted by the National Society for PKU (NSPKU) about living with PKU, women aged 18 years and over completed 9 closed questions about their pre-conception, pregnancy and post-natal experiences and an open-ended question on their reproductive health. RESULTS: 300 women aged 18 years and over with PKU completed this questionnaire with 37% (n = 111/300) of women already having children. 56% (n = 71/128) of pregnancies were planned and a further 18% (n = 23/128) of women had both planned and unplanned pregnancies. Most women (73%, n = 200/273) expressed concerns, fears and distress about pregnancy and two thirds of women who had at least one pregnancy stated that having PKU made pregnancy more stressful and difficult. 60% (n = 164/273) were concerned that they may cause harm to a baby, 58% (n = 159/273) worried about their ability to manage a strict diet during pregnancy, 54% (n = 147/273) had anxiety about their ability to maintain blood Phe within target range, and 48% (n = 132/273) feared having an unplanned pregnancy.Postnatally, 48% (n = 45/93) had low mood or sadness, 41% (n = 38/93) were depressed, 34% (n = 32/93) felt unable to cope, 33% (n = 31/93) said they could not manage their PKU and care for their baby, 14% (n = 13/93) struggled with child care needs and 4% (n = 4/94) worried they might hurt themselves or their baby through being unable to manage their diet. CONCLUSIONS: Many women with PKU have unmet sexual and reproductive health needs. Pregnancy fears are prominent from adolescence onwards and for some women overarching concerns about their ability to cope with pregnancy led to a decision not to have children. Interventions are needed to reduce the psychological impact of the risk of maternal PKU syndrome and assist with safe pregnancies. Post-natal experiences of women with PKU give rise to concern.

Living with Phenylketonuria: Lessons from the PKU community.

INTRODUCTION: We report the practical, social and psychological issues of living with phenylketonuria (PKU) from one of the largest surveys that has been completed by both adults with PKU and parents/caregivers of children. METHODS: In the UK, parents/caregivers of children and adults with PKU were invited to complete an online survey between November 2017 to January 2018 by the NSPKU (National Society for Phenylketonuria). RESULTS: 631 participants (adults, n = 338; parents/caregivers of children, n = 293) with PKU completed the questionnaire. Problems experienced by children with PKU were: difficulty with maintaining focus (48%, n = 114/236), educational difficulties (28%, n = 67/236), anxiety or depression (29%, n = 68/236), and gastrointestinal symptoms (34%, n = 97/282). Fifty one per cent (n = 120/236) described social exclusion; 17% (n = 41/236) had relationship issues with friends or family. Problems experienced by adults were: depression or anxiety (52%, n = 148/286), difficulty maintaining focus (54%, n = 154/286), and low mood (54%, n = 180/334). Difficulties were experienced with relationships (34%, n = 96/286); social exclusion (44%, n = 126/286); and gastrointestinal issues (n = 34%, n = 112/334). Common medications used included antidepressants (40%, n = 131/331) and anxiolytics (18%, n = 60/334). DISCUSSIONS: Adults with PKU or caregivers/parents of children identified significant neurocognitive, mental health and general health issues. Limits on socialisation, perception of social isolation and dietary stigma are major obstacles which are difficult to overcome with conventional dietary management.

Do governing body and CSU nurses on clinical commissioning groups really lead a nursing agenda? Findings from a 2015 Survey of the Commissioning Nurse Leaders' Network Membership.

AIMS: This paper reports the findings from a 2015 survey of the Commissioning Nurse Leaders' Network. Our aim was to understand how governing body nurses perceive their influence and leadership on clinical commissioning groups. METHODS: An online survey method was used with a census sample of 238 governing body nurses and nurses working in Commissioning Support Units, who were members of the Commissioning Nurse Leaders' Network. The response rate was 40.7% (n = 97). RESULTS: While most governing body nurses felt confident in their leadership role, this was less so for non-executive governing body nurses. Nurses in Commissioning Support Units were much less positive than governing body nurses about their influence on clinical commissioning groups. CONCLUSION: Governing body nurses were satisfied with their impact on clinical commissioning groups and so could be said to be leading a nursing agenda but this evidence is limited to their own perceptions and more objective or diverse measures of impact are needed. The purpose of such roles to 'represent nursing, and ensure the patient voice is heard' may be a flawed aspiration, conflating nursing leadership and patient voice. IMPLICATIONS FOR NURSING MANAGEMENT: This is the first study to explore explicitly the differences between executive and non-executive governing body nurses and nurses working in commissioning support units. Achieving clinical commissioning groups' goals, including developing and embedding nursing leadership roles in clinical commissioning groups, may be threatened if the contributions of governing body nurses, and other nurses supporting clinical commissioning groups, go unrecognised within the profession, or if general practitioners or other clinical commissioning group executive members dominate decision-making on clinical commissioning groups.

Compassion in practice-Evaluating the awareness, involvement and perceived impact of a national nursing and midwifery strategy amongst healthcare professionals in NHS Trusts in England.

AIMS AND OBJECTIVES: To report the findings from an evaluation of the impact of the Compassion in Practice Vision and Strategy (National Health Service England (NHSE), 2012) on nursing, midwifery and care staff. BACKGROUND: The Compassion in Practice Vision and Strategy was a programme of work to highlight the importance of compassionate care following the Francis Report in 2013 into the deficits in care in an NHS Hospital Trust. It was launched by NHS England in 2012 at a time when fiscal cuts were introduced by the Department of Health in England. DESIGN AND SETTING: Mixed methods. RESULTS: Inferential statistics were used to test whether there were significant differences between staff at different levels of seniority with regard to awareness and involvement in Compassion in Practice Vision and Strategy and their attitudes to it. Awareness and involvement of staff in Compassion in Practice Vision and Strategy were high amongst middle and senior management but limited at ward level. Staff involvement in Compassion in Practice Vision and Strategy was limited due to a lack of awareness. Ward level staff who were aware and involved, perceived a lack of support and communication from senior leadership to deliver the Compassion in Practice Vision and Strategy. DISCUSSION: Results reveal professional anger, distress and resistance to the Compassion in Practice Vision and Strategy and a view of the programme as a top-down initiative which did not sufficiently recognise structural constraints on nurses' ability to deliver compassionate care. We discuss the implications of our findings for global nursing. CONCLUSION: Participants emphasised that compassion for patients is only sustainable where there is compassion for staff and many participants felt that they were not being treated with compassion. RELEVANCE FOR CLINICAL PRACTICE: National Health Service England should strongly affirm that nurses and midwives in general provide compassionate care. Trust leadership should provide support for ward level staff who deliver compassionate care in difficult circumstances.

The extent, variability, and attitudes towards volunteering among undergraduate nursing students: Implications for pedagogy in nurse education.

In the aftermath of the Francis Report nurses are being called to account for an apparent lack of care and compassion, leading to debate around pedagogy in nurse education. Absent from this debate is a consideration of student volunteering within undergraduate nursing programmes and its potential to promote student nurses self-esteem and to enhance the development of critical thinking skills. The aim of this study was therefore to understand the extent of and attitudes towards volunteering among nursing students. A mixed methods approach using a specifically developed questionnaire, followed by in-depth interviews to ascertain extent, variability, and attitudes towards volunteering revealed low levels of volunteering among nursing students. Limited time, limited access, and lack of academic support were cited as reasons. Nevertheless, students displayed positive attitudes towards volunteering. While volunteering has been shown to impact upon students abilities to think critically, to develop personal values and respond to the needs of others, volunteering within the UK undergraduate nursing programme considered here is neither structured nor formalized. Nurse educators should pay attention to the positive benefits of volunteering for nursing students and consider ways in which volunteering might be incorporated into the curriculum.

Nurses' experiences of clinical commissioning group boards.

Aim To explore the experience of governing body nurses appointed to clinical commissioning group (CCG) boards; how they perform their responsibilities; and their perceived effectiveness in ensuring safe, patient-centred care and the factors that influence their effectiveness. Method This was a small pilot study using a mixed methods approach. There were four phases of the study: literature review, qualitative data collection (interviews), quantitative data collection (survey), and final data analysis. Findings In the early stages of the formation of CCGs, few governing body nurses had relevant experience to meet the needs of a strategic role, and many of these nurses had no proper job description, too little time to carry out their responsibilities, little management support, and unequal access to training, development, formal support or supervision compared to GP colleagues. Two working patterns or models of work of governing body nurses emerged: the full-time integrated executive statutory role and the part-time non-executive statutory role. Quality and quality assurance were the most frequently cited roles or responsibilities of governing body nurses in CCGs, and their highest priority was to improve the population's health. Conclusion The role of governing body nurse has emerged at a time of organisational change, and following extensive criticism of nursing and nurses in the media. Nurses' roles and experiences are affected by these contextual events and by the emerging structures and diversity of CCGs. Further research is required into the leadership role of governing body nurses, succession planning, and the effectiveness of their relationships with other senior nurses.

Fate and transport of phosphate from an onsite wastewater system in Beaufort County, North Carolina.

The objectives for the study described in this article were to evaluate the fate and transport of onsite wastewater system (OWS)-derived phosphate from a residential system in Beaufort County, North Carolina, and to determine if current OWS setback regulations are sufficient to prevent elevated phosphate discharge to surface waters. Piezometers were installed in nests at different depths adjacent to drain-field trenches and up- and down-gradient of a residential OWS. Groundwater and septic effluent phosphate concentrations, temperature, pH, dissolved oxygen, and electrical conductivity were monitored every two months from February 2011 to October 2011 (five times). The mean groundwater phosphate concentration beneath the OWS (3.05 +/- 0.74 mg/L) was not significantly different than septic effluent (2.97 +/- 0.76 mg/L) and was elevated relative to background groundwater (0.14 +/- 0.12 mg/L). Groundwater phosphate concentrations were inversely related (r2 = .83) to distance from the system. Onsite system setback regulations may have to be increased (>30 m) in some areas to ensure groundwater phosphate concentrations are reduced to background concentrations before discharge to surface waters.

Teachers' views of using e-learning for non-traditional students in higher education across three disciplines [nursing, chemistry and management] at a time of massification and increased diversity in higher education.

BACKGROUND: The expansion of the higher educational sector in the United Kingdom over the last two decades to meet political aspirations of the successive governments and popular demand for participation in the sector (the Widening Participation Agenda) has overlapped with the introduction of e-learning. OBJECTIVES: This paper describes teachers' views of using e-learning for non-traditional students in higher education across three disciplines [nursing, chemistry and management] at a time of massification and increased diversity in higher education. DESIGN: A three phase, mixed methods study; this paper reports findings from phase two of the study. SETTINGS: One university in England. PARTICIPANTS: Higher education teachers teaching on the nursing, chemistry and management programmes. METHODS: Focus groups with these teachers. FINDINGS: Findings from these data show that teachers across the programmes have limited knowledge of whether students are non-traditional or what category of non-traditional status they might be in. Such knowledge as they have does not seem to influence the tailoring of teaching and learning for non-traditional students. Teachers in chemistry and nursing want more support from the university to improve their use of e-learning, as did teachers in management but to a lesser extent. CONCLUSIONS: Our conclusions confirm other studies in the field outside nursing which suggest that non-traditional students' learning needs have not been considered meaningfully in the development of e-learning strategies in universities. We suggest that this may be because teachers have been required to develop e-learning at the same time as they cope with the massification of, and widening participation in, higher education. The findings are of particular importance to nurse educators given the high number of non-traditional students on nursing programmes.

Experiences of supernumerary status and the hidden curriculum in nursing: a new twist in the theory-practice gap?

AIMS AND OBJECTIVES: This paper aims to increase our understanding about how student nurses' experiences of supernumerary status are embedded in the hidden curriculum in clinical practice and contribute to the theory-practice gap in nursing. BACKGROUND: Current literature suggests that the hidden curriculum exists in many professional curricula and that it functions to socialise students into professional behaviours and practice. However, in nursing, there is a gap in our understanding of how these socialisation processes have been influenced by supernumerary status and what forms the hidden curriculum might take currently in clinical practice. DESIGN: An ethnographic case study design. METHOD: Data were collected in four sites using fieldwork in clinical practice as well as interviews with students, mentors and key stakeholders, an online survey of student bodies and curriculum analysis in four universities. The findings in this paper are drawn from the qualitative fieldwork and interviews and were analysed thematically. RESULTS: The findings suggest that supernumerary status is an important aspect of the hidden curriculum in clinical learning for nursing students; that students are expected by trained staff to work while they learn and that on registration, they are expected to be competent to work immediately as registered nurses. These expectations are at odds with those of academic nurses and contribute to a theory-practice gap for student nurses. These expectations form part of the hidden curriculum that shapes the clinical context, and students have to learn to negotiate their status as supernumerary students in practice to meet these expectations. CONCLUSION: Consequently, students have to learn in a disintegrated learning context where opposing values of learning exist. RELEVANCE TO CLINICAL PRACTICE: To reintegrate student nurses' learning, educators in universities and clinical practice have to understand how the hidden curriculum and expectations around supernumerary status among trained staff affect learning for students.