Haemoglobinopathy Registry

What are haemoglobinopathies?

  • Haemoglobinopathies are inherited conditions, caused by changes (mutations) in the genes that code for parts of the haemoglobin molecule of red blood cells, which carries oxygen in the blood
  • Haemoglobinopathy genes are common in people of South East Asia, China, the Pacific, Africa, the Middle East and Southern Europe
  • People with a single altered gene usually have no symptoms and might not be aware that they carry the altered gene - problems generally only occur when a person inherits two altered copies of a gene

People with haemoglobinopathies make insufficient and/or abnormal haemoglobin (e.g. thalassaemia and sickle cell disease), which can cause a wide variety of problems. They may have very high health care requirements throughout their lives, including needing many blood transfusions, and experience serious complications of the disease and of its treatment.

The Australian Haemoglobinopathy Registry is a database for collecting medical information from patients with thalassaemia and sickle cell disease, over many years.

 

Why do we need an Australian Haemoglobinopathy Registry?

  • Australia has a significant number of affected people and a unique mix of cases, which is changing as migration patterns change, but we don’t know exactly how many people are affected, how severely they are affected, or how their health is changing over time.
  • People with haemoglobinopathies are living much longer than in the past, but little is known about their long-term complications and outcomes.
  • Different centres currently manage the same disease in slightly different ways, and we don’t know which strategies work best for patients.

The registry will tell us all of these things. It will also:

  • Provide an important framework for future research to improve patient care
  • Be used to help to plan health care services for the future
  • Bring together a network of Australian health care professionals with a special interest in haemoglobinopathies, for sharing ideas.
The Registry is the first step in a major project to ensure that all Australians with a haemoglobinopathy continue to receive the best possible care.

 

Why is Monash University’s Transfusion Research Unit involved?

  • The Transfusion Research Unit (TRU) is part of Monash University’s Department of Epidemiology and Preventive Medicine, Australia’s leading institution for clinical registries, and has the required infrastructure and expertise in public health, epidemiology, biostatistics and management of large databases of confidential information.
  • Patient privacy is given the highest priority and identifiable information is not shared with third parties.  Monash University provides high security data management.
  • TRU is an independent academic unit, made up of specialist haematologists and medical researchers.
  • The Transfusion Research Unit has existing relationships with key players, such as the National Blood Authority, the Australian Red Cross Blood Service, Monash Medical Centre’s Thalassaemia Service (the largest thalassaemia service in Australia) and patient advocacy and support groups, including Thalassaemia Australia, Thalassaemia Centre of NSW and Thalassaemia International Federation in Cyprus.

How will patients participate?

Patients with a clinically significant haemoglobinopathy* will be identified by their treating clinician. The clinician will ask the patient to participate before entering their details into the secure website. This will usually occur in the setting of an outpatient appointment, or during a hospital admission. Patients can decline to participate at any time, even after their details have been entered, without any consequences for their treatment.

Why should patients participate?

This project will provide important information that will help improve the way haemoglobinopathy patients are cared for in the future. Each patient is like a piece of a large jigsaw puzzle - each one gives us only a tiny amount of information, but together, they build a picture. The more pieces of the puzzle we can collect, the clearer the picture will be, and the greater the benefit to all patients.

Every Australian with a haemoglobinopathy is important to this Registry.

 

Project update

The Haemoglobinopathy Registry (HbR) has begun collecting core medical information from patients receiving treatment for thalassaemia or sickle cell disease at 10 Australian hospitals.  These hospitals are:

  • Monash Medical Centre* (Vic)
  • Royal Children's Hospital (Vic)
  • Royal Prince Alfred Hospital* (NSW)
  • The Children's Hospital at Westmead (NSW)
  • Prince of Wales Hospital (NSW)
  • Mater Adult Hospital (Qld)
  • Royal Adelaide Hospital (SA)
  • Women's & Children's Hospital (SA)
  • Princess Margaret Hospital for Children (WA)
  • Sir Charles Gairdner Hospital (WA

*The project has collected initial data from consented patients at Monash Medical Centre and Royal Prince Alfred Hospital. The remaining Stage 1 hospitals will come online in the coming months. In Stage 2, we will collect additional patient data, and will include more hospitals, and this will be undertaken over a period of years, so that we can monitor changes in people's health and health care over time.  

HbR Privacy Policy

We thank all the staff at participating hospitals, and Thalassaemia Australia, Thalassaemia Centre of NSW, and Thalassaemia International Federation for their support so far.

This project is overseen by a national, multidisciplinary Steering Committee, chaired by Professor Joy Ho of Royal Prince Alfred Hospital, Sydney. Please click here for full Steering Committee Details.

If you have questions about the project, please contact the project officer, Ri Scarborough:

hbr@monash.edu or 1800 811 326