Health maintenance in adults with sickle cell disease: a UK perspective – SCiF 2013

Dr Jo Howard, Consulant Haematologist, Guy’s & St. Thomas’ NHS Foundation Trust, UK

Adult sickle services in England underwent a peer review process for the first time in 2012/3. This process was supported by the West Midlands Quality Review Service and the UK Haemoglobinopathy Forum and services were based on agreed Quality Standards (1). The adult peer review followed on from a successful paediatric peer review (2) and both were based on the previously published standards of care (3,4). As part of a separate process a specialised services definition set for haemoglobinopathies has been developed which specifies that care for sickle patients should take place within a clinical network, divided into three levels of care. Specialist teams should provide tertiary level care, specialist input and annual reviews on all patients, local teams should provide routine acute care and outpatient follow up and accredited local centres provide an intermediate level of care.

27 centres, which were either specialist or accredited local centres, were reviewed across the country as part of peer review, accounting for approximately 8200 adults, of whom 80% were cared for in London. Whilst there were six centres caring for over 500 adults, five had fewer than 100, and there were concerns whether centres caring for this few adults have adequate exposure to build up and maintain staff expertise.  Furthermore there were unknown numbers of adults that are cared for by haematology services in hospitals which did not link to a specialist team or who were not part of an established network of care. Networks were generally not well developed and had poor infrastructure; no networks could identify all patients in their area.

Many of the specialist centres provided excellent care and most services had thorough guidelines for care. Chronic disease screening was taking place but not always in a routine manner. Some centres had excellent specialist clinics for patients with renal, cardiac, respiratory, neurology and orthopaedic problems, but these were not accessible to all patients across a network and referral pathways were not always clear. The majority of centres had identified high risk obstetricians, joint sickle obstetric (or haematology-obstetric in smaller centres) and obstetric pathways. In every unit visited there were key members of medical and nursing staff who were dedicated to providing high quality care, but they were often poorly resourced with inadequate cover arrangements in place. With a few notable exceptions there was poor access to psychology services and welfare and benefits support and transition services were of variable quality.

Most centres were using the National Haemoglobinopathy Registry and had registered the majority of their patients, but it was not being used routinely for adverse event reporting or annual reviews often due to a lack of data support. Many centres did have robust annual review proformas and were performing these regularly.

The peer review process has given us a very clear insight into the inequalities in care for adult sickle patients across the country and an overview report with recommendations will be published shortly. It has also highlighted many examples of good practice, which will be shared as part of a good practice event later this year.



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