Sickle Cell Awareness Month 2007

Sickle Cell Awareness Month 2007 will run from Monday 2nd July.

Encouraging people to improve their understanding of this genetic blood disease, the Sickle Cell Society and the NHS Screening Programme will be supporting activities across England to improve knowledge and challenge common mis-conceptions about this disease.

Throughout July, local centres and support groups across England will be running events and open days to raise awareness of the challenges faced by those living with sickle cell, while highlighting the benefits of early screening for the condition. It is planned that all pregnant women in England will be offered screening for sickle cell and thalassaemia by the end of summer 2007.

Background information

There is much to celebrate. Since last year, screening for all newborns has been rolled out across the country, ensuring that all babies with sickle cell are identified early.

This year campaigners will be working to highlight the availability and advantages of screening for parents either before conception or in the very early stages of pregnancy. Early screening ensures that prospective parents can have all the information required to make the most informed choices. In England sickle cell is as common as cystic fibrosis – around 12,500 people live with the disorder and there are an estimated 240,000 carriers.

Sickle Cell consists of a range of conditions – some more serious than others.

The most serious form is sickle cell anaemia but there are other forms of the condition such as sickle haemoglobin C disease and sickle beta thalassaemia.

The conditions affect the normal oxygen carrying capacity of red blood cells. The symptoms can include severe anaemia, intense pain, damage to major organs and infections. Although there is no routine cure for sickle cell, patients can be supported to manage their pain, and regular monitoring can help to avoid life threatening complications such as stroke.

In England, sickle cell affects an estimated 12,500 people with an estimated 240,000 carriers.

In beta thalassaemia major, the most severe form of thalassaemia, the body is unable to produce haemoglobin – the element in blood that transports oxygen. Patients therefore need regular blood transfusions throughout their lives. They also need daily treatment to deal with the iron overload that builds up in their body. Complications include diabetes, growth problems, and problems with puberty or early menopause.

In England, around 700 people are affected by thalassaemia major with an estimated 214,000 carriers.

Bone marrow transplantation may be a treatment option for some people affected by sickle cell and thalassaemia.

The Sickle Cell & Thalassaemia (SC&T) Screening Programme was set up in England in 2001 following Government commitment in the NHS Plan (2000). It is the world’s first linked antenatal and newborn screening programme.

The aims of the programme are to:

Save lives through prompt identification of affected babies Offer informed choice to couples expecting a baby Work towards offering testing to young people before they start a family Support the development of a managed clinical care network such that people have fair access to quality services throughout England Raise public awareness of the disorders and challenge stigma

Newborn bloodspot screening for sickle cell disorders is offered to all babies and is integrated into the standard testing. Screening identifies approximately 300 babies a year who would be at risk of death unless penicillin is administered promptly.

At the antenatal stage, a screening blood test identifies pregnant women who carry a gene for sickle cell, or other haemoglobin disorders. Where a woman is a genetic carrier, the baby’s father is also offered testing. If both parents are carriers, there is a 1 in 4 chance with each pregnancy that the baby will have the disorder. At risk couples will be offered a range of counselling and diagnostic tests for the baby.

Antenatal screening has been rolled out in most high prevalence areas and the target is to achieve coverage throughout England by the end of summer 2007. In low prevalence areas, a questionnaire looking at family origin will be used as an initial screen to assess risk for sickle cell and other haemoglobin variants.

Sickle cell and thalassaemia are among the world’s most commonly inherited genetic diseases. In England sickle cell is as common as cystic fibrosis.

The Programme is also lobbying for funding for the provision of managed clinical care networks and support of those affected by sickle cell and thalassaemia. Managed care is not only important in relieving suffering, but also cost effective in reducing the cost of specialist interventions. Many of the strategies planned will help families to spot warning signs and take early action. This, in turn, will reduce complications that are more expensive for the NHS in the longer term.

The NHS Sickle Cell and Thalassaemia Screening Programme is based at the Division of Health and Social Care Research, King’s College London School of Medicine. For further information please visit the website: