UKHCDO – Haemophilia Deaths, Causes & Record Keeping

2nd May 2016 Mark Ward

Haemophilia deaths, causes & record keeping

The UKHCDO (United Kingdom Haemophilia Centre Directors Organisation) is required by the Department Of Health to collect data on diagnosis, management and complications of bleeding disorders. This information is kept in the National Haemophilia Database, which is located in Manchester.

The United Kingdom Haemophilia Centre Doctors’ Organisation is an association of medical practitioners who work within the Haemophilia Centre’s of England, Scotland, Northern Ireland or Wales and have an interest in the care of people with Haemophilia or other inherited bleeding disorders.

The aims of the organisation are:

To preserve, protect and relieve persons suffering from Haemophilia and other inherited bleeding disorders.

To advance the education of the medical profession, the nursing profession, professions allied to medicine and the general public in the knowledge of Haemophilia and other inherited bleeding disorders and their treatment.

To promote or assist in the promotion of audit and research into the causes, prevention, alleviation and management of Haemophilia and other inherited bleeding disorders and to disseminate the useful results of such research.

The UKHCDO is an organisation of doctors who manage patients with bleeding disorders. UKHCDO was established in 1968 to improve haemophilia care, research into bleeding disorders, their treatment epidemiology and complications and to facilitate healthcare planning.

UKHCDO established a secretariat and a national data-base (NHD) as early as 1978* and has produced annual reports and many publications from the data generated. Although the data-base and secretariat remained in Oxford since that time, the Executive Committee, advised by the Advisory Committee decided in May of 2002 that the data-base and secretariat should be moved to Manchester Royal Infirmary.

The data for the NHD[1] was collected annually using a paper system with information regarding new registrations and death being reported as they occur. For this reason, the Data Management Working Party directed that the data-base be upgraded and national networked with the introduction of individually networked Haemophilia Centre management systems for real-time data downloading. Dr Rob Hollingsworth, Chartered Software Engineer designed this system with the assistance of the previous Data Manager. Dr Hollingsworth is based in the University Department of Haematology at CM&MC. This software development is now complete and in the process of being rolled out nationally.

It is anticipated that this software, when fully implemented, will permit the generation of more detailed reports.

The UKHCDO has been a registered charity since 1991.

A leaflet entitled “Your Questions Answered[2]” is available online. Patients wishing to request a copy of the data held by the UKHCDO must now complete an application form.[3] This includes a question on vCJD and if a person wishes to know about their possible exposure through treatment.

However we must highlight there are a number of haemophiliacs who, for previous litigation purposes requested this data. The received information was not only entitled as an “Abstract” but it also contained many irregularities and discrepancies.

For example, a severe haemophiliac received his “abstract” from the UKHCDO which stated in a number of years he had not received any treatment. (This is not something a severe haemophiliac would be able to do, as they require higher levels of treatment for a number of reasons, such as trauma, spontaneous bleeds or prophylactic purposes). However in reality the patient had undergone a series of operations including a total knee replacement. When questioned about these discrepancies, no explanation could be given and the patient was advised the data collected by the UKHCDO was submitted voluntarily from participating hospitals. This raises suspicion on where the full data has been sent along with how this information is being used.

When recording deaths and the known causes we run into huge problems. In the UKHCDO Annual Report 2010 – 2011[4], a list of deaths by year can be seen, unfortunately this is also incomplete as some years are absent of information. The process in which information is recorded has been designed in such a way that one is unable to truly join any of the dots up, to give a complete picture.

For many years Hepatitis was the highest recorded cause of death in haemophiliacs but nothing more specific, such as Hepatitis A, B, etc was given. Viral exposure and abnormal liver function in haemophiliacs is a subject which has been greatly studied for decades. Mass infection with multiple strains of Hepatitis viruses can lead to “super hybridization” causing liver failure. Most commonly seen to be a major factor in liver disease is contamination of Hepatitis viruses and CMV (Cytomegalovirus).

This can also widely be observed during the crisis years of the AIDS epidemic. Before 1985 haemophiliacs were still being infused with non-heat treated factor 8 products. This meant those with already compromised immune systems were infected and re-infected with multiple contaminants, which would have lead to death. Once the heat treatment process was bought in, further research showed the process did not kill certain Hepatitis viruses, for instance HCV, along with other contaminants, Parvovirus B19 and various strains of Human Herpes Viruses (HHV) are just two.

The cause of infection and subsequent death would have been known in some cases, such as CMV but were recorded as an AIDS Related Condition (ARC). With multiply contaminated patients such as the haemophilia community, identifying the true nature of the death would have been something doctors at the time would not be keen to disclose. Haemophiliacs were being told their infection to HIV and HCV was a huge accident. Therefore when haemophiliacs were diagnosed with other infections alternative reasons for the infection would have been given, if prompted.

We feel we will never truly be able to say how many haemophiliacs died from contaminants other than HCV and HIV/AIDS received through their treatment or the patterns that have emerged since. The opportunity to record the worst treatment disaster in the history of the NHS has either been lost or is recorded in a location unknown to the general public. Today we are still not fully informed of the dangers we face nor the possible risks to our health and those of our loved ones.

Those responsible for the care of infected haemophiliacs appear to be distancing themselves from the past and refuse to communicate about the harm these other contaminants are causing. We know there are ongoing studies into cardiovascular, renal, lung / repertory, neurological as well as psychological problems across the community. These conditions are not discussed and whenever the possibility could lead to an admission of viral contamination as the cause of a particular problem faced by haemophiliacs, they are often ignored or pushed around the care system. Examples of patients being denied tests for bowl cancer, HTLV 1&2 (Human T Lymphotropic Virus), denied access to renal specialists can be given. Also the attitude of those caring for haemophiliacs refuse to assist in identifying what contaminants patients have been exposed to, this we believe is a breach in their duty of care.