Welcome to the Haemoglobinopathy Registry homepage

What is the EHR?
This registry, known as the European Haemoglobinopathy Registry (EHR) is a multicentre project, co-ordinated by the Haematology Department at Central Middlesex Hospital, London, UK, which aims to systematically collect clinical data on patients with haemoglobinopathies. Teleform software is used to develop patient data forms and the Patient Analysis & Tracking System (PATS) for data storage and analysis. The completed forms are scanned into PATS and each participating centre’s data is held in a separate sub-registry. Participating centres will be provided with copies of their own individual patient data on excel spreadsheets and reports at mutually agreed time intervals. Anonymised EHR group reports will also be provided.

How did it start?
The pilot phase, co-ordinated by Dr Marilyn Roberts-Harewood, consisted of a registry to address the effectiveness and toxicity of hydroxyurea therapy in sickle cell disease. This started with an EU Concerted Action No. [BMH4-LT 96-1659 (GDIZ-SSMA)], whose steering group involves key clinical experts in Europe. The hydroxyurea sub-registry continues to flourish and the registry has expanded to collect data on all patients with sickle cell disease. Currently Ealing, Hammersmith, West Middlesex and Central Middlesex Hospital patients populate the SCD registry.

What does the EHR hope to achieve?
The EHR collects longitudinal clinical data to assist with clinical management and research. This information is analysed for a number of purposes including:
• Study of the natural history of the diseases locally and in the European environment.
• Evaluation of interventions (e.g. hydroxyurea in SCD patients)
• Accurate information to assist with the routine clinical management of patients.
• Collaboration and information sharing

How the data is collected?
This is a collaborative multi-centre project. Any hospital consultant caring for patients with a haemoglobinopathy can join in. Patients have to give their written consent to be on the registry. Participating centres make their own arrangements for data collection. At Central Middlesex the registry co-ordinator, the doctors and nurse specialist help with prospective data collection during the outpatient visit. Retrospective data is collected from the medical notes by the registry co-ordinator.

What data is collected?
Routine data normally recorded in hospital medical records, including the past medical history, demographics, clinical, treatment and laboratory data. The project requires initial registration questionnaires to be completed for all patients and at least an annual follow-up. In addition if patients are treated with hydroxyurea a one page clinical monitoring form is completed during each clinical visit.

How can you join?
The current active registries are the Haemoglobinopathy Registry (Adult Sickle Cell Disease (SCD) and Paediatric SCD) and Hydroxyurea in SCD patients. If you would like to participate in any or all of these please read the instructions on how to participate. You must also read the confidentiality and ethics section for instructions and regulations regarding patient consent and data security.

In this section you can also view samples of the hydroxyurea and paediatric data collection forms, the patient information sheet and consent form.