Welcome to the Haemoglobinopathy Registry website.

Sickle Cell Disease (SCD) and Thalassaemia are a group of inherited blood disorders known collectively as 'the haemoglobinopathies'. Both conditions affect selected populations originating from Africa, the Mediterranean, Middle East and Asia. They are one of the most common genetic diseases and until the last decade management was purely preventative and supportive aimed at symptom control. Now stem cell transplant offers a chance of cure and hydroxyurea, an oral chemotherapeutic drug, has established a role in ameliorating the disease and improving life expectancy. However these treatments are not without risk and are currently only available to the most severely affected patients and, in the case of transplant, patients that have best chance of cure and survival.

More then 10,000 British people suffer from Sickle Cell Disease (SCD) and approximately 600 have Beta Thalassaemia Major, the majority of which live in and around London. The number of SCD sufferers continues to rise annually and current estimates suggest that 160 infants are born each year in England with SCD. Approximately 17 infants are born each year in England with Beta Thalassaemia Major or Beta Thalassaemia Intermedia.

The case has long been made for a clinical network and registry to follow haemoglobinopathy patients so that reliable evidence is available about the natural history of the diseases and long-term effectiveness of the current treatment options. Only then will patients and parents/carers be able to make informed choices about their health care. Here at Central Middlesex Hospital we have already started this work. Our hydroxyurea database is part of a larger haemoglobinopathy registry, known at the European Haemoglobinopathy Registry (EHR), currently under development to provide information support for the newly formed North West London Sector Managed Clinical Network for Haemoglobinopathies. So far the data collection has provided reliable information on clinical and laboratory outcomes in hydroxyurea treatment for both individual patient reporting and group evaluation. This registry is composed of a number of sub-registries and doctors can opt to join the registries that suit their client group and/or requirements. For example you can decide to only send data on patients to populate the hydroxyurea registry.

If this registry interests you please read the 'The Registry' homepage to know more about us and further details on how to join.

You can also link to pages explaining more about the haemoglobinopathies, the Managed Clinical Network, patient services available in the North West London Sector and other useful links.

Director:	Professor Sally C Davies
EHR Co-ordinator:	Annette Gilmore
IT Consultant:	Sameer J Patankar