Prime Minister Gordon Brown And Rt Hon Dr John Sentamu, Archbishop Of York, Welcome New Standards Of Care For Sickle Cell Disease

Main Category: Blood / Hematology
Article Date: 09 Jul 2008 - 1:00 PDT

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The lives of thousands of sickle cell patients in the UK will hopefully be improved following today's launch of the first 'Standards for the Clinical Care of Adults with Sickle Cell Disease', by the Rt Hon Dr John Sentamu, the Archbishop of York, at the House of Commons.

For the first time, the standards provide guidance to health professionals on the minimum levels of care expected for every adult with sickle cell disease (SCD) in the UK. This ranges from the role of GPs in disease management, to the need for all A&E staff to recognise and manage patients effectively when they present with a painful crisis.

A statement from the Prime Minister, Gordon Brown said: "I am delighted to welcome the publication of the first 'Standards for the Clinical Care of adults with Sickle Cell Disease'. I know how important these standards will be in ensuring the highest levels of medical care for patients and families who learn to live with sickle cell. I know it is an extraordinarily difficult disease to manage. These standards will help achieve one of our foremost goals: the lessening of health inequalities in the UK."

Until now, people with SCD have suffered from inequalities in the delivery and quality of care. A survey of 110 A&E workers revealed that 65 per cent felt they did not have enough information to provide the right care to people with SCD, which is alarming when the average A&E department sees someone with SCD six times a year.1

A national survey by the charity 'National Confidential Enquiry into Patient Outcome and Death' (NCEPOD) also recently revealed an unexpectedly high number of unknown causes of deaths of SCD patients. The NCEPOD survey showed that of 19 patients in the study who had complained of pain and who subsequently died in hospital, nine had been given excessive doses of painkillers and five of those patients died because of complications due to overdose.2

Fortune Esimaje, 42, a chartered accountant from London who has SCD said: "When I had a sickle cell crisis during a business meeting in Birmingham, the local hospital was unable to help me. It was a struggle for them to find my veins to treat me and so I had to take an expensive taxi to my usual hospital in London. I welcome these standards in the hope they deliver the same standards of care across the country, to give people with sickle cell the best treatment possible."

SCD is an inherited blood disorder that predominantly affects people whose families come from the Caribbean, Africa, the Eastern Mediterranean, the Middle East and Asia. In SCD, red blood cells become deformed ('sickle' shaped) and tend to get stuck in small blood vessels, restricting the blood flow to tissues and organs. This blockage can cause severe pain and lead to major complications such as stroke, damage to major organs in the body and even death.3

The Archbishop of York, Dr Sentamu said: "Although sickle cell disease is the most common genetic disorder in England, there is still a vast difference in care between major cities and other areas of the country. These standards are another step in providing consistent care across the country for people with sickle cell disease and bringing this under recognised condition from the margins to the mainstream."
Dr Lorna Bennett, Chairperson, Sickle Cell Society said. "The Sickle Cell Society believes that every person with sickle cell disease has the right to quality care without discrimination between ethnic groups, age, gender or area of residence of those affected. The reality is that provision of care for adults with sickle cell disease can vary significantly between individual professionals as well as health care provision organisations. These standards are the tool needed to address the inequalities in provision and access to good quality care."

SCD affects approximately 12,500 adults and children in the UK, with 240,000 people carrying the gene trait. If a mother and father both have the sickle cell gene, their child has a 25% chance of being born with sickle cell disease.4 The demographics of where patients live have changed. At one time SCD was mostly found in London and the West Midlands, but it is now found across the UK, meaning that the standardisation of care across the country and a consistent level of healthcare professional knowledge about SCD is vital to ensure adequate patient care in the UK.

About the 'Standards of Clinical Care of Adults with Sickle Cell Disease'

The Sickle Cell Society invited a multidisciplinary group of professionals and service users (including expert patient programme, psychology, social work, counselling, haematology specialist care and primary care) working within healthcare and charitable organisations to form a working group to assess the current standards of care and work together to develop an organisational model of service delivery for holistic care for people with SCD. The standards are a tool with which to address inequalities in provision and access to good quality care and represent the very best vision of good practice available at the time of development.

Key points within the standards include:

Primary Care:

- GPs to provide written information regarding approaches to preventing and managing symptoms at home
- Support to be given to community practitioners from specialist centres

Hospital Services:

- Specialist hospital clinics available for all people with sickle cell disease
- Specialist networks to provide guidance and support for local hospitals including management of common sickle cell presentations at A&E

Managing Acute Complications:

- Patients suffering from a pain crisis to be treated within 30 minutes
- Hospitals should have a protocol in place for managing acute chest syndrome

Managing Chronic Complications:

- The cause of chronic pain to be established as far as possible
- Patients with stroke should undergo urgent neuroimaging

Pregnancy, Contraception and Fertility

- Genetic screening and partner testing to be offered to all men and women with SCD

Blood Transfusion

- Patients undergoing transfusions to be regularly monitored for iron overload
- Iron chelation should be started in all regularly transfused patients when repeated serum ferritin levels are >1000mg/l

Surgery and Specific Therapies

- New therapies to be discussed with patients and if the local hospital is unable to provide them, a clear pathway should be made for provision of care

The Standards of Clinical Care of Adults with Sickle Cell Disease were developed through an unrestricted educational grant from Novartis Oncology. A full copy of the Standards of Clinical Care of Adults with Sickle Cell Disease is available from the Sickle Cell Society at http://www.sicklecellsociety.org

Standards of Clinical Care of Adults with Sickle Cell Disease