Neonatal Screening for Haemoglobinopathies in South East London: findings and comparisons with Jamaica

INTRODUCTION: Neonatal screening for sickle cell disorders has been shown to reduce mortality and morbidity. Methods of screening vary but in 1994, the local Health Authority funded universal neonatal screening across the whole of Lambeth, Southwark and Lewisham. This paper will report the findings of the first three years of operation of the programme and compare findings with the screening programme currently operating in Jamaica. METHOD: since May 1994 dried bloo[d] spots of all infants have been screened at King's College Hospital and screen positive cases followed up by counsellors. Infants are followed up at four sites (Guy's, King's, Lewisham and St.Thomas') according to parental preference. Minimum standards for follow-ups have been agreed by clinicians across all four sites and information of the success in achieving these standards is now being collected. RESULTS: Overall there have been 122 affected infants detected in the first two years 10 months of the programmes operation. This is made up of 83 HbSS, 35 HbSC, 4HbSBThal. This gives a birth preference of 23.4 per 1000 total population (2.3HbSS, 1.0 HbSC). Allowing for a termination rate of 20 percent this indicates that the expected birth prevalence in the district would be 4.2 per 1000 births. This compares with birth prevalence of of 0.3 per 1000 for congenital hypothyroidism, 06. per 1000 for cystic fibrosis and 0.1 for phenylketonuria. The distribution of the births is unevem with 57 in Southwark, 40 in Lambeth and 25 in Lewisham. The paper will report on the follow-up and outcome of care provided for this population to date. Discussion: South East London has the highest prevalence of sickle cell disorders of any district in the UK. Sickle cell disorder is now as common in South East London as it is in Jamaica. The follow-up arrangements established in South East London provide an opportunity for colloboration with the West Indies which may help to determine some of the reasons for differences in the natural course of the disease in these populations. The population based approach established should allow monitoring of the impact of community education and antenatal screening programmes on the birth prevalence over time. (AU)

Improving the effectiveness of screening for haemoglobinopathies in primary and community care in Lambeth: antenatal findings

An antenatal screening programme for haemoglobinopathies aims to allow couples to make informed choices about reproductive options. Screening must be carried out early in pregnancy and must be supported with accurate information and a genetic counselling service. An audit in 1990 at St. Thomas' found that couples were referred for genetic counselling late in pregnancy or not at all. The aim of the study was to icrease the uptake of screening and counselling in the antenatal clinic by improving midwives knowledge of the condition and available counselling services. A research worker was employed to evaluate the intervention. METHODS: Knowledge of haemoglobionpathies, genetic risk and available services was assessed by means of a questionnaire. An audit of ante-natal screening was undertaken before and after the introduction of specific guidelines and a porforma by reviewing over 1400 antenatal notes of those recieving community or shared care. Laboratory data on the number of tests requested for both the women and their partners was obtained for the year of the first audit. RESULTS: 23/44 midwives completed the questionnaire (65 percent). Analysis of the responses indicated that hospital midwives were better informed on every question. There was a significant difference (p<<0.001 in the proportion of questions answered correctly by hospital midwives compared to community midwives (77 percent of 45 percent, 95 percent CI 17-45 percent). (AU)

The findings of a London wide needs assessment for sickle cell dis orders

The size of the population in London with Sickle cell disorders has increased markedly in the past 20 years and it is now one of the commonest reasons for admission of children to hospital in inner London. This paper presents the findings of a London Wide Needs assessment for sickle cell disorders.It reviews Health and Local Authorities response to the needs of their populations in terms of the development of strategy and services. It also considers reasons for the failures to develop appropriate services[AU]

A Comprehensive approach to needs assessment of sickle cell services: The value of seeking service users' views

The recent `Fair Shares for London' study used multiple methods to carry out a needs assessment of services for sickle cell disorders in Greater London. This paper reported on one of these, a series of focus group discussions with users of services for sickle cell disorders. The aims of the focus groups were as follows: 1. To investigate and record service users' experiences, perceptions and opinions about current service provision for people with sickle cell disorders. 2. To structure the discussions so as to cover the same range of services investigated by the questionnaire surveys. Three focus groups, each consisting of 6-8 people, were conducted at different locations in London. The results of the focus groups reflect those of the questionnaire survey, indicating that service users recognise some recent improvements in service delivery, but that many aspects of services for sickle cell disorders in Greater London remain inadequate. Improvements most often mentioned included ambulance services and some aspects of acute care. Significant problems persist in the delivery of community services and education, housing and social services. Some examples of good practice in primary care were highlighted. However the majority of participants made little or no use of GP services due to previous negative experiences. The Fair Shares for London study shows how the use of qualitative methods in needs assessment can enhance and validate results. The focus group discussions made an important contribution to the overall findings, complementing and elucidating the results of the questionnaire survey of purchasers and providers, as well as identifying areas for further research.(AU)