Screening for sickle cell disease and thalassaemia: a systematic review with supplementary research. Health Technol Assess 2000;4(3). Health Technology Assessment is indexed in Index Medicus/MEDLINE and Excerpta Medica/ EMBASE. Copies of the Executive Summaries are available from the NCCHTA web site (see overleaf). NHS R&D HTA Programme T he overall aim of the NHS R&D Health Technology Assessment (HTA) programme is to ensure that high-quality research information on the costs, effectiveness and broader impact of health technologies is produced in the most efficient way for those who use, manage and work in the NHS. Research is undertaken in those areas where the evidence will lead to the greatest benefits to patients, either through improved patient outcomes or the most efficient use of NHS resources. The Standing Group on Health Technology advises on national priorities for health technology assessment. Six advisory panels assist the Standing Group in identifying and prioritising projects. These priorities are then considered by the HTA Commissioning Board supported by the National Coordinating Centre for HTA (NCCHTA). This report is one of a series covering acute care, diagnostics and imaging, methodology, pharmaceuticals, population screening, and primary and community care. It was identified as a priority by the Population Screening Panel and funded as project number 93/33/03. The views expressed in this publication are those of the authors and not necessarily those of the Standing Group, the Commissioning Board, the Panel members or the Department of Health. The editors wish to emphasise that funding and publication of this research by the NHS should not be taken as implicit support for the recommendations for policy contained herein. In particular, policy options in the area of screening will be considered by the National Screening Committee. This Committee, chaired by the Chief Medical Officer, will take into account the views expressed here, further available evidence and other relevant considerations. Reviews in Health Technology Assessment are termed 'systematic' when the account of the search, appraisal and synthesis methods (to minimise biases and random errors) would, in theory, permit the replication of the review by others. Criteria for inclusion in the HTA monograph series Reports are published in the HTA monograph series if (1) they have resulted from work either prioritised by the Standing Group on Health Technology, or otherwise commissioned for the HTA Programme, and (2) they are of a sufficiently high scientific quality as assessed by the referees and editors.

BackgroundAvoidable hospital mortality is often attributable to inadequate patient vital signs monitoring, and failure to recognise or respond to clinical deterioration. The processes involved with vital sign collection and charting; their integration, interpretation and analysis; and the delivery of decision support regarding subsequent clinical care are subject to potential error and/or failure.ObjectiveTo determine whether introducing an electronic physiological surveillance system (EPSS), specifically designed to improve the collection and clinical use of vital signs data, reduced hospital mortality.MethodsA pragmatic, retrospective, observational study of seasonally adjusted in-hospital mortality rates in three main hospital specialties was undertaken before, during and after the sequential deployment and ongoing use of a hospital-wide EPSS in two large unconnected acute general hospitals in England. The EPSS, which uses wireless handheld computing devices, replaced a paper-based vital sign charting and clinical escalation system.ResultsDuring EPSS implementation, crude mortality fell from a baseline of 7.75% (2168/27 959) to 6.42% (1904/29 676) in one hospital (estimated 397 fewer deaths), and from 7.57% (1648/21 771) to 6.15% (1614/26 241) at the second (estimated 372 fewer deaths). At both hospitals, multiyear statistical process control analyses revealed abrupt and sustained mortality reductions, coincident with the deployment and increasing use of the system. The cumulative total of excess deaths reduced in all specialties with increasing use of the system across the hospital.ConclusionsThe use of technology specifically designed to improve the accuracy, reliability and availability of patients’ vital signs and early warning scores, and thereby the recognition of and response to patient deterioration, is associated with reduced mortality in this study.

Summary.A range of estimates for sickle cell and b thalassaemia have been derived for the different ethnic groups living in the U.K., reflecting uncertainty over the true population value in certain countries and the heterogeneity within and between countries of origin comprising the same ethnic group. These were validated against six community screening programmes, with the estimated range correctly predicting the number of affected births observed by the programmes.In England approximately 3000 affected babies (0·47%) carry sickle cell trait and 2800 (0·44%) carry b thalassaemia trait annually; with approximately 178 (0·28 per 1000 conceptions) affected by sickle cell disease (SCD) and 43 (0·07 per 1000) by b thalassaemia major/intermedia. Allowing for termination, about 140-175 (0·22-0·28 per 1000) affected infants are born annually with SCD and from 10 to 25 (0·02-0·04 per 1000) with b thalassaemia major/ intermedia.These are the first evidence-based rates for sickle cell and b thalassaemia for use in the U.K., and should underpin the future planning of services. The long-term solution to monitoring changes in the rates of trait and disease in the population is to introduce a standardized instrument for collecting ethnicity for all community screening programmes.

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Objective-To evaluate universal antenatal screening for haemoglobinopathies. Setting-District general hospital serving a London borough with 45% ethnic minorities. Methods-Retrospective cohort study of 1444 women referred in 1688 pregnancies and 95 tertiary referrals during 101 pregnancies. Results-Unselected women at risk for sickle cell disease booked 2.7 weeks (95% confidence interval (CI) 0.14 to 5.1) later in gestation than those at risk for thalassaemia were less likely to attend counselling (83% v 93%, relative risk (RR) 0.89; 95% CI 0.85 to 0.94), their partners were less likely to be tested (77% v 95%, RR 0.81; 0.77 to 0.83), and they were less likely to accept prenatal diagnosis (22% v 90%, RR 0.37; 0.24 to 0.57).Over 99% of tertiary referrals attended counselling and had their partners tested. There were no significant diVerences in acceptance of prenatal diagnosis between those at risk of sickle cell disease and thalassaemia (55% v 67%).Unselected women at risk of sickle cell disease were significantly less likely to have their partner tested or to accept prenatal diagnosis than tertiary referrals, but not those at risk of thalassaemia. 80% of thalassaemia and 16% of SS births were prevented. Conclusions-Uptake of prenatal diagnosis among unselected women at risk of thalassaemia is similar to that reported by tertiary centres. It is considerably lower for sickle cell disease but could increase considerably if screening occurred earlier in gestation.Acceptance of counselling is universally high, suggesting that informed choices are made, and indicating a need to measure these outcomes for cost eVectiveness studies. (J Med Screen 1999;6:3-10)

Background: Nosocomial norovirus infections and their control measures disrupt patient care, increase

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