18 May 2022: Presentation (CJD Timeline) - A

Once upon a time, in the 1920s, a new neurological health problem was identified in Germany. It was designated as a prion disease and named after the two researchers who made the discovery; Creutzfeldt–Jakob disease (CJD).

The 1960s were the next significant staging post, with numerous papers being published about a group of prion-type diseases including scrapie in sheep.

That period also saw the establishment of the Oxford surveillance study centre. There were retrospective studies through the 1970s.

In the early 1995 there was a death put down to CJD. It was probably vCJD, but that had not been identified then. The types of CJD were becoming more understood.

In 1996, Prof Ironside and his colleague Prof Will presented work to SEAC which identified variant CJD. Shortly after that (12 days later), Government Minister Stephen Dorrell announced in Parliament the probable link between BSE in cows and CJD in humans. The vulnerability of younger people was highlighted. The mobilisation of blood services on this issue began, including the need to consider blood donors who might be implicated due to several factors. There were also moves to ensure European directives were applied. Other initiatives were kicked off, such as the Surveillance Unit, the review of blood transfusion databases, and specific recording and coordination of CJD cases to protect public health, and banning the use of bovine offal products.

Considerations began to include the potential impact of blood donor activity arising from the CJD turn of events. CMO letters started to be distributed to inform various part of the NHS about CJD. Though there was no initial evidence, the possibility of blood being a transmission route needed to be kept under review. A study in 1997 in Lothian looked specifically at the possible blood link, with any people identified not being told, which was part of the ethical approval. Initially, the use of brain biopsy in a deceased person was recognised as the only diagnostic option. This is also where the tonsil study fitted in, and it would later indicate a possible diagnostic option, too.

Other cases were emerging, and the symptoms were being recognised. There were further guidelines issued on blood donations and early screening attempts (e.g. medical and family histories, etc.) SEAC led on efforts to protect the public. The cow to human crossover, and the plasma transmission links were strengthened. Implicated blood and blood product batches began to be recalled. The justification was precautionary due to a lack of hard evidence.

Studies were getting closer to identifying the unique features of vCJD compared to classical CJD. The realisation of the potential contamination of large amounts of the UK blood supply was clearly a concern, but the policy continued of not telling people that there was a (probably) small risk due to the mental health and external impacts that such news might have. The experience of AIDS and Hepatitis were to a smaller scale, but the learning proved to be useful. The lack of a test or of a treatment meant that the progression towards death was fraught since it was already known among the population how horrific that end-of-life condition was. Professionals were discussing the possible development in terms of an epidemic level issue. More knowledge of the parts of the body where vCJD was most likely to be concentrated included the lymphatic organs like the spleen, and especially the brain and spinal cord.

In 1997, as heard in oral evidence, Frank Dobson the Health Minister accepted the SEAC recommendations to extend leuko-depletion to further protect the UK population. There were also information documents published and disseminated specifically on the relation between vCJD and Haemophilia. It gave impetus to the development and general use of Recombinant Factor products as the treatment of choice.

Sir Brian picked out the anomaly of the comparable conditions which resulted in some people being told or being considered implicated, while other were not.

The difficult ethical issues being raised by vCJD were having an increasing impact on policy making and published advice. For the Haemophilia community, discussions between clinicians were towards not informing patients due to the likely impacts on them of a situation that is mostly very unlikely to affect them. Recombinant concentrates were designated to be made available to children and new patients not previously treated.

Across the country there was a shift to leuko-depletion and restricting the use of UK plasma for fractionation. Directives become specific on issues such as the risk of contaminating surgical instruments and the requirement to report suspected cases.

In 1999 came the shift to informing patients of any risk involving them having been exposed to contaminated blood. It was deemed unethical to not tell a donor when they came forward, and to carry on the deception by apparently accepting their blood donation and then dispose of it without their knowledge. Greater coordination and uniformity was established between the four nations. An incident group was established to look at each reported case individually. In 2000, a fund was set up to support the victims of vCJD.