Prevalence and perceptions of pain in people with haemophilia: A UK study.

INTRODUCTION: Joint bleeds in haemophilia cause destruction of articular structures, impaired function and pain. Up to 70% of people with haemophilia (PWH) report chronic pain. Little is known about the pain experiences in PWH in the UK. AIM: To identify prevalence and perceptions of pain among PWH living in the UK. METHODS: A cross-sectional, non-interventional survey study conducted among PWH (all severities). The survey incorporated elements from validated tools (EQ-5D; EQ-VAS) and was distributed via participating treatment centres. RESULTS: Five hundred and ninety-nine PWH responded, 91% aged > 18. 81% used factor prophylactically or on demand. More pain was reported by those treated on demand versus prophylaxis particularly in those who reported daily pain. 65% reported 'problem joints' based on individual impact rather than medically defined 'target joints', 2/3 reported multiple joint issues. The ankle was most commonly affected. 59% reported frequent pain, with 56% aware of pain constantly or most of the time and were more likely to report less favourable EQ-5D or EQ-VAS scores (p < .001). Pain frequency/awareness was consistent across all severities. Most discussed pain with care teams, 31% only when asked; 25% did not discuss it. Pain discussions resulted in physiotherapy referral (63%) analgesia prescription (48%), and a minority specialist pain referral (9%). Most felt well supported with regard to their pain, but 70% reported learning to live with it. CONCLUSION: Pain affects PWH of all ages and severities even in a well-resourced country significantly impacting quality of life. Clinicians must be more aware of chronic pain in PWH. Biopsychosocial approaches to pain assessment and management are recommended.

Modelling benzo(a)pyrene concentrations for different meteorological conditions - Analysis of lung cancer cases and associated economic costs.

Air pollution originating from the household presents a significant burden to public health, especially during the wintertime in countries, such as Poland, where coal substantially contributes to the energy market. One of the most hazardous components of particulate matter is benzo(a)pyrene (BaP). This study focusses on the impact of different meteorological conditions on BaP concentrations in Poland and associated impacts on human health and economic burdens. For this study, we used the EMEP MSC-W atmospheric chemistry transport model with meteorological data from the Weather Research and Forecasting model to analyze the spatial and temporal distribution of BaP over Central Europe. The model setup has two nested domains, with the inner domain at 4 km × 4 km over Poland, which is a hotspot for BaP concentrations. The outer domain covers countries surrounding Poland in coarser resolution (12 × 812 km), to ensure that transboundary pollution is properly characterized in the modelling. We investigated the sensitivity to variability in winter meteorological conditions on BaP levels and impacts using data from 3 years: 1) 2018, which represents average meteorological conditions during the winter season (BASE run), 2) 2010 with a cold winter (COLD), and 3) 2020 with a warm winter (WARM). The ALPHA-RiskPoll model was used to analyze the lung cancer cases and associated economic costs. The results show that the majority of Poland exceeds the target level of benzo(a)pyrene (1 ng m-3) mainly due to high concentrations during the cold months. High concentrations of BaP have serious health implications and the number of lung cancers in Poland due to BaP exposure varies from 57 to 77 cases for the WARM and COLD years, respectively. It is reflected in the economic costs, which ranged from 136, through 174 to 185 million euros/year for the WARM, BASE and COLD model runs, respectively.

The lived experience of women with a bleeding disorder: A systematic review.

BACKGROUND: Research studies have described the morbidity associated with inherited bleeding disorders such as hemophilia and von Willebrand disease in women, but their effect on daily living has long been underrecognized. This systematic review sought to document the lived experience of women with a bleeding disorder by assessing research findings on quality of health care, socioeconomic factors, and mental health. METHODS: A systematic search was carried out in Web of Science, the Cumulative Index to Nursing and Allied Health Literature, and PubMed on July 31, 2020. References were hand searched. Abstracts of the 2019 and 2020 congresses on bleeding disorders were hand searched. Key journals were screened for relevant studies published after the search date until the analysis was completed on December 7, 2020. RESULTS: Of 635 potentially eligible publications, 27 published since 1998 were selected for review. Most studies were of moderate to high quality but meta-analysis of quantitative studies was not possible due to difference in outcomes and assessment.Women with a bleeding disorder experience obstacles to accessing care, difficulties living with their disorder, interference with schooling and work, and poor mental health. Diagnostic delay and lack of recognition of symptoms mean treatment and support may not be available. Where comparisons with controls were made, women's negative experiences were greater than those of men. CONCLUSIONS: Women with bleeding disorders experience major negative impacts of their disorders on daily life and mental well-being. Many of the challenges identified in earlier research are evident in more recent studies.

Project GYM: A randomized feasibility study investigating effect on motivation of personal trainer-led exercise in young men with hemophilia.

INTRODUCTION: Many young men with hemophilia engage in physical activity and sport but face challenges to participation because of their hemophilia. Project GYM aimed to investigate the feasibility of a hemophilia-specific fitness program led by a personal trainer (PT) and its impact on gym activity, motivation, and adherence to exercise. METHODS: This was a nonblinded, randomized feasibility study, recruiting participants aged 18 to 25 years with hemophilia A or B (all severities, ± inhibitor) from three London hemophilia centers. All participants were given an activity tracker and free gym membership. Participants were randomized to a "gym only" or "gym and PT" arm. Participants completed questionnaires evaluating motivation to exercise, quality of life, physical activity levels, self-efficacy, and self-esteem at study start and study end. RESULTS: Of 142 eligible individuals, 19 agreed to participate. Participants were healthy, with mean body mass index and adiposity slightly lower than the UK average. They reported low bleed numbers and had good joint health (median Hemophilia Joint Health Score [HJHS], 0; range, 0-13). The gym and PT group had more gym attendance than the gym-only group. Seven participants increased their activity levels and nine stayed the same, with no statistical difference between groups. HJHS scores improved in 3 participants and were unchanged in 12. There was no bleeding associated with gym activity. CONCLUSION: Project GYM has demonstrated the safety and feasibility of a tailored physical training program in young men with hemophilia. Increased gym attendance, with and without support from a PT, is associated with increased physical activity.

Fitness enhances psychosocial well-being and self-confidence in young men with hemophilia: Results from Project GYM.

INTRODUCTION: Contemporary hemophilia care supports physical activity, its benefits being well recognized. Despite recognition of the psychological challenges encountered by people with hemophilia, little is known about the psychological impact of physical fitness in this population. AIM: To identify changes in psychological well-being in young men with hemophilia through participation in a gym program. METHODS: This observational feasibility study of a 6-month gym participation program used validated questionnaires pre- and poststudy to evaluate motivation to exercise, physical activity levels, self-efficacy, self-esteem, and quality of life. Individual audio-recorded interviews about study participation and impact were transcribed verbatim and analyzed for recurring themes using thematic analysis. RESULTS: Nineteen participants aged 18-25 years with hemophilia A or B (all severities ± inhibitor) consented to the study; two were lost to follow-up. There was a shift in motivation to exercise as shown by the Stages of Change grouping moving from contemplation to action and maintenance phases (p = 0.03). Self-efficacy overall scores showed a trend (p < 0.06) towards improvement. Median self-esteem scores improved from 22 (range 12-30, n = 19) to 25 (range 13-30, n = 17), a statistically significant change (p = 0.02). Three participants recorded scores below the accepted normal range before study, of whom two improved at study end. The key themes identified from the interviews were: fear, self-confidence, "being normal," pain, weight loss, ability, getting fitter. CONCLUSIONS: The psychological wellbeing of young men with hemophilia improved during this study. This may have been related to participating in a gym-based, physical exercise program.

Abating ammonia is more cost-effective than nitrogen oxides for mitigating PM2.5 air pollution.

Fine particulate matter (PM2.5, particles with a mass median aerodynamic diameter of less than 2.5 micrometers) in the atmosphere is associated with severe negative impacts on human health, and the gases sulfur dioxide, nitrogen oxides, and ammonia are the main PM2.5 precursors. However, their contribution to global health impacts has not yet been analyzed. Here, we show that nitrogen accounted for 39% of global PM2.5 exposure in 2013, increasing from 30% in 1990 with rising reactive nitrogen emissions and successful controls on sulfur dioxide. Nitrogen emissions to air caused an estimated 23.3 million years of life lost in 2013, corresponding to an annual welfare loss of 420 billion United States dollars for premature death. The marginal abatement cost of ammonia emission is only 10% that of nitrogen oxides emission globally, highlighting the priority for ammonia reduction.

Home-based Extended Rehabilitation for Older people (HERO): study protocol for an individually randomised controlled multi-centre trial to determine the clinical and cost-effectiveness of a home-based exercise intervention for older people with frailty as extended rehabilitation following acute illness or injury, including embedded process evaluation.

BACKGROUND: The majority of older people (> 65 years) in hospital have frailty and are at increased risk of readmission or death following discharge home. In the UK, following acute hospitalisation, around one third of older people with frailty are referred on for rehabilitation, termed 'intermediate care' services. Although this rehabilitation can reduce early readmission to hospital (< 30 days), recipients often do not feel ready to leave the service on discharge, suggesting possible incomplete recovery. Limited evidence suggests extended rehabilitation is of benefit in several conditions and there is preliminary evidence that progressive physical exercise can improve mobility and function for older people with frailty, and slow progression to disability. Our aim is to evaluate the effectiveness of the Home-based Older People's Exercise (HOPE) programme as extended rehabilitation for older people with frailty discharged home from hospital or intermediate care services after acute illness or injury. METHODS: A multi-centre individually randomised controlled trial, to evaluate the clinical and cost-effectiveness of the HOPE programme. This individualised, graded and progressive 24-week exercise programme is delivered by NHS physiotherapy teams to people aged 65 and older with frailty, identified using the Clinical Frailty Scale, following discharge from acute hospitalisation and linked intermediate care rehabilitation pathways. The primary outcome is physical health-related quality of life, measured using the physical component summary score of the modified Short Form 36- item health questionnaire (SF36) at 12 months. Secondary outcomes include self-reported physical and mental health, functional independence, death, hospitalisations, care home admissions. Plans include health economic analyses and an embedded process evaluation. DISCUSSION: This trial seeks to determine if extended rehabilitation, via the HOPE programme, can improve physical health-related quality of life for older people with frailty following acute hospitalisation. Results will improve awareness of the rehabilitation needs of older people with frailty, and provide evidence on the clinical and cost-effectiveness of the targeted exercise intervention. There is potential for considerable benefit for health and social care services through widespread implementation of trial findings if clinical and cost-effectiveness is demonstrated. TRIAL REGISTRATION: ISRCTN 13927531 . Registered on April 19, 2017.

The UK Integrated Assessment Model for source apportionment and air pollution policy applications to PM2.5.

Source apportionment and the effect of reducing individual sources is important input for the development of strategies to address air pollution. The UK Integrated Assessment Model, UKIAM, has been developed for this purpose as a flexible framework, combining information from different atmospheric dispersion models to cover different pollutant contributions, and span the range from European to local scale. In this paper we describe the UKIAM as developed for SO2, NOx, NH3, PM2.5 and VOCs. We illustrate its versatility and application with assessment of current PM2.5 concentrations and exposure of the UK population, as a case-study that has been used as the starting point to investigate potential improvement towards attainment of the WHO guideline of 10 µg/m3.

Potential and limitation of air pollution mitigation by vegetation and uncertainties of deposition-based evaluations.

The potential to capture additional air pollutants by introducing more vegetation or changing existing short vegetation to woodland on first sight provides an attractive route for lowering urban pollution. Here, an atmospheric chemistry and transport model was run with a range of landcover scenarios to quantify pollutant removal by the existing total UK vegetation as well as the UK urban vegetation and to quantify the effect of large-scale urban tree planting on urban air pollution. UK vegetation as a whole reduces area (population)-weighted concentrations significantly, by 10% (9%) for PM2.5, 30% (22%) for SO2, 24% (19%) for NH3 and 15% (13%) for O3, compared with a desert scenario. By contrast, urban vegetation reduces average urban PM2.5 by only approximately 1%. Even large-scale conversion of half of existing open urban greenspace to forest would lower urban PM2.5 by only another 1%, suggesting that the effect on air quality needs to be considered in the context of the wider benefits of urban tree planting, e.g. on physical and mental health. The net benefits of UK vegetation for NO2 are small, and urban tree planting is even forecast to increase urban NO2 and NOx concentrations, due to the chemical interaction with changes in BVOC emissions and O3, but the details depend on tree species selection. By extrapolation, green infrastructure projects focusing on non-greenspace (roadside trees, green walls, roof-top gardens) would have to be implemented at very large scales to match this effect. Downscaling of the results to micro-interventions solely aimed at pollutant removal suggests that their impact is too limited for their cost-benefit analysis to compare favourably with emission abatement measures. Urban vegetation planting is less effective for lowering pollution than measures to reduce emissions at source. The results highlight interactions that cannot be captured if benefits are quantified via deposition models using prescribed concentrations, and emission damage costs. This article is part of a discussion meeting issue 'Air quality, past present and future'.

The UK haemophilia specialist nurse: Competencies fit for practice in the 21st century.

INTRODUCTION: Nurses play a central co-ordinating role in delivering comprehensive care for people with haemophilia and allied bleeding disorders, for which they need a broad range of competencies. The UK Haemophilia Nurses Association (HNA) published a role description in 1994 which was developed into a competency framework in 2014. This has now been updated to reflect current educational and clinical practice. AIM: To summarize the evidence supporting the nurse's advanced role within haemophilia care and develop new competencies to deliver comprehensive care within a multidisciplinary team. METHODS: Systematic reviews were identified by PubMed literature search. The HNA conducted workshops to consult its membership, and the authors incorporated this input to update its competency framework within the structure outlined by Health Education England in multiprofessional framework for advanced clinical practice in England (2017). RESULTS: The proposed framework includes five domains (Clinical knowledge, Clinical/direct care, Communication and support, Collaborative practice and Research) supported by indicators for four levels of practice (beginner, competent, proficient and expert). The framework is a tool which nurses and their managers can use to assess skills and knowledge, and identify learning needs appropriate to personal development and improve patient care and outcomes. CONCLUSION: The HNA has developed a new competency framework to provide a strong foundation for haemophilia specialist nurses to continue improving services for people living with bleeding disorders and their families, as well as supporting personal development alongside new therapeutic options, models of care and follow-up.

The impact of psychosocial determinants on caregivers' burden of children with haemophilia (results of the BBC study).

INTRODUCTION: Caring for a child with haemophilia is burdensome and impacting on caregivers' emotional and financial status. This paper assesses the impact of psychosocial determinants on caregivers' burden across European countries. METHODS: This non-interventional study enrolled caregiver/child dyads at haemophilia treatment centres (HTCs) using the "HEMOphilia associated CAregiver Burden scale" (HEMOCAB). Socio-demographic characteristics and clinical data were collected. RESULTS: A total of 144 dyads from Germany (n = 19), Italy (n = 19), Netherlands (n = 19), Turkey (n = 20), Sweden (n = 21), UK (n = 21) and Poland (n = 25) participated. Caregivers' mean age was 39.84 ± 7 (range 24-57); 81.3% were mothers, married (80.4%), living with a partner (86.6%), had a college/university degree (66.5%) and worked (74.2%). Around two thirds of caregivers (66.2%) reported that haemophilia affected their life; 26.8% reported an economic impact; 57.6% reported their child cannot do certain things because of his condition. Caregivers lost an average of 8.35 ± 14.5 days due to haemophilia. The highest burden was reported in the HEMOCAB domains "Perception of Child" (37.9 ± 24.7), "Emotional Stress" (37.4 ± 22.6) and "Medical Management" (33.1 ± 22.8). Significantly, higher burden was found in caregivers who reported that haemophilia "affects their life" (P < 0.0001), "has an economic impact" (P < 0.0001), "their child cannot do certain things" (P < 0.0001), "they spent ≥5 h/mo infusing" (P < 0.003) and "they needed ≥3 h/mo to reach the HTC" (P < 0.0001). CONCLUSION: This "snapshot" analysis of burden related to caring for a child with haemophilia across Europe revealed the greatest burdens are economic, including days lost from work, and things that a child cannot do, impacting on both child and caregiver.

The burden of bleeds and other clinical determinants on caregivers of children with haemophilia (the BBC Study).

INTRODUCTION: Treatment burden for the people with haemophilia has been documented, as has the burden of caring for a child with a common chronic disease such as asthma or diabetes. However, there remains a paucity of data about caregiver burden in haemophilia. AIMS: The aim of this study was to evaluate the impact of bleeding on caregivers of children with haemophilia. Caregiver burden was stratified by the clinical status of their child. METHODS: A multinational, non-interventional study of caregivers of children with severe or moderate haemophilia, using the HEMOCABquestionnaire to evaluate caregiver burden. RESULTS: A total of 144 caregivers from seven EU countries participated in the study. Differences in caregiver burden were identified based on the clinical situation of the child. Greater burden was seen in caregivers of children who experienced joint bleeding in the preceding 12 months, or had target joints or reduced range of motion in most domains of the HEMOCAB. Caring for a child with a current inhibitor also caused significantly higher burden for caregivers when compared to caring for a child with tolerized inhibitor or without inhibitor. Caregivers of children with chronic pain reported significantly higher burden in all domains of the HEMOCAB except for "interaction with the father." CONCLUSION: Caregiver burden can be affected by the child's haemophilia status, particularly if joint health is impacted (eg bleeds, decreased mobility) or if the child suffers from chronic pain which was moderately correlated with joint bleeds.

Air quality co-benefits for human health and agriculture counterbalance costs to meet Paris Agreement pledges.

Local air quality co-benefits can provide complementary support for ambitious climate action and can enable progress on related Sustainable Development Goals. Here we show that the transformation of the energy system implied by the emission reduction pledges brought forward in the context of the Paris Agreement on climate change (Nationally Determined Contributions or NDCs) substantially reduces local air pollution across the globe. The NDCs could avoid between 71 and 99 thousand premature deaths annually in 2030 compared to a reference case, depending on the stringency of direct air pollution controls. A more ambitious 2 °C-compatible pathway raises the number of avoided premature deaths from air pollution to 178-346 thousand annually in 2030, and up to 0.7-1.5 million in the year 2050. Air quality co-benefits on morbidity, mortality, and agriculture could globally offset the costs of climate policy. An integrated policy perspective is needed to maximise benefits for climate and health.

The Lancet Countdown on health benefits from the UK Climate Change Act: a modelling study for Great Britain.

BACKGROUND: Climate change poses a dangerous and immediate threat to the health of populations in the UK and worldwide. We aimed to model different scenarios to assess the health co-benefits that result from mitigation actions. METHODS: In this modelling study, we combined a detailed techno-economic energy systems model (UK TIMES), air pollutant emission inventories, a sophisticated air pollution model (Community Multi-scale Air Quality), and previously published associations between concentrations and health outcomes. We used four scenarios and focused on the air pollution implications from fine particulate matter (PM2·5), nitrogen dioxide (NO2) and ozone. The four scenarios were baseline, which assumed no further climate actions beyond those already achieved and did not meet the UK's Climate Change Act (at least an 80% reduction in carbon dioxide equivalent emissions by 2050 compared with 1990) target; nuclear power, which met the Climate Change Act target with a limited increase in nuclear power; low-greenhouse gas, which met the Climate Change Act target without any policy constraint on nuclear build; and a constant scenario that held 2011 air pollutant concentrations constant until 2050. We predicted the health and economic impacts from air pollution for the scenarios until 2050, and the inequalities in exposure across different socioeconomic groups. FINDINGS: NO2 concentrations declined leading to 4 892 000 life-years saved for the nuclear power scenario and 7 178 000 life-years saved for the low-greenhouse gas scenario from 2011 to 2154. However, the associations that we used might overestimate the effects of NO2 itself. PM2·5 concentrations in Great Britain are predicted to decrease between 42% and 44% by 2050 compared with 2011 in the scenarios that met the Climate Change Act targets, especially those from road traffic and off-road machinery. These reductions in PM2·5 are tempered by a 2035 peak (and subsequent decline) in biomass (wood burning), and by a large, projected increase in future demand for transport leading to potential increases in non-exhaust particulate matter emissions. The potential use of biomass in poorly controlled technologies to meet the Climate Change Act commitments would represent an important missed opportunity (resulting in 472 000 more life-years lost from PM2·5 in the low-greenhouse gas scenario and 1 122 000 more life-years lost in the nuclear power scenario from PM2·5 than the baseline scenario). Although substantial overall improvements in absolute amounts of exposure are seen compared with 2011, these outcomes mask the fact that health inequalities seen (in which socioeconomically disadvantaged populations are among the most exposed) are projected to be maintained up to 2050. INTERPRETATION: The modelling infrastructure created will help future researchers explore a wider range of climate policy scenarios, including local, European, and global scenarios. The need to strengthen the links between climate change policy objectives and public health imperatives, and the benefits to societal wellbeing that might result is urgent. FUNDING: National Institute for Health Research.

A pharmacoeconomic approach to assessing the costs and benefits of air quality interventions that improve health: a case study.

OBJECTIVE: This paper explores the use of pharmacoeconomic methods of valuation to health impacts resulting from exposure to poor air quality. In using such methods, interventions that reduce exposure to poor air quality can be directly compared, in terms of value for money (or cost-effectiveness), with competing demands for finite resources, including other public health interventions. DESIGN: Using results estimated as part of a health impact assessment regarding a West Yorkshire Low Emission Zone strategy, this paper quantifies cost-saving and health-improving implications of transport policy through its impact on air quality. DATA SOURCE: Estimates of health-related quality of life and the National Health Service (NHS)/Personal Social Services (PSS) costs for identified health events were based on data from Leeds and Bradford using peer-reviewed publications or Office for National Statistics releases. POPULATION: Inhabitants of the area within the outer ring roads of Leeds and Bradford. MAIN OUTCOMES MEASURES: NHS and PSS costs and quality-adjusted life years (QALYs). RESULTS: Averting an all-cause mortality death generates 8.4 QALYs. Each coronary event avoided saves £28 000 in NHS/PSS costs and generates 1.1 QALYs. For every fewer case of childhood asthma, there will be NHS/PSS cost saving of £3000 and a health benefit of 0.9 QALYs. A single term, low birthweight birth avoided saves £2000 in NHS/PSS costs. Preventing a preterm birth saves £24 000 in NHS/PSS costs and generates 1.3 QALYs. A scenario modelled in the West Yorkshire Low Emission Zone Feasibility Study, where pre-EURO 4 buses and HGVs are upgraded to EURO 6 by 2016 generates an annual benefit of £2.08 million and a one-off benefit of £3.3 million compared with a net present value cost of implementation of £6.3 million. CONCLUSIONS: Interventions to improve air quality and health should be evaluated and where improvement of population health is the primary objective, cost-effectiveness analysis using a NHS/PSS costs and QALYs framework is an appropriate methodology.

Enhancing patient safety in enteral feeding.

Misconnection of enteral feeding tubes to intravenous devices has occurred a number of times and could be life-threatening, or even fatal. A new connector has been designed to make misconnection impossible. This article outlines the development of ENFit, how its use will be introduced into the UK and how this will affect nurses' clinical practice.

Costs and benefits of nitrogen for Europe and implications for mitigation.

Cost-benefit analysis can be used to provide guidance for emerging policy priorities in reducing nitrogen (N) pollution. This paper provides a critical and comprehensive assessment of costs and benefits of the various flows of N on human health, ecosystems and climate stability in order to identify major options for mitigation. The social cost of impacts of N in the EU27 in 2008 was estimated between €75-485 billion per year. A cost share of around 60% is related to emissions to air. The share of total impacts on human health is about 45% and may reflect the higher willingness to pay for human health than for ecosystems or climate stability. Air pollution by nitrogen also generates social benefits for climate by present cooling effects of N containing aerosol and C-sequestration driven by N deposition, amounting to an estimated net benefit of about €5 billion/yr. The economic benefit of N in primary agricultural production ranges between €20-80 billion/yr and is lower than the annual cost of pollution by agricultural N which is in the range of €35-230 billion/yr. Internalizing these environmental costs would lower the optimum annual N-fertilization rate in Northwestern Europe by about 50 kg/ha. Acknowledging the large uncertainties and conceptual issues of our cost-benefit estimates, the results support the priority for further reduction of NH3 and NOx emissions from transport and agriculture beyond commitments recently agreed in revision of the Gothenburg Protocol.