5 After the storm?|
88. Variant Creutzfeldt-Jakob Disease (vCJD) is not
like other infectious diseases. Caused by a mysterious pathogen
which we are still only just beginning to understand, vCJD is
an invariably fatal disease of sudden onset, which has historically
inflicted on its young victims a progressive dementia more often
seen in the oldest and sickest members of society. When the first
cases began to emerge in the mid-1990s, the tragic images of young
vCJD victims worked alongside the existing narrative of 'mad cow
disease' to create an unprecedented level of public anxiety, maintained
over subsequent years as the number of cases gradually rose.
89. Underlying this anxiety was the suggestion that
these deaths were an avoidable and man-made tragedy: that the
Government had mishandled the BSE crisis and was therefore to
blame for vCJD. Between 1998 and 2000, the Government's role in
the crisis came under increasing scrutiny as a result of the BSE
inquiry, and it was during this period that the Government took
its first major steps to protect the UK blood supply from vCJD.
These steps were largely precautionary: in the late 1990s there
were no confirmed cases of vCJD having been transmitted via blood
transfusion and many scientists thought this unlikely to occur.
Nevertheless, costly risk mitigation measuresleucodepletion
and the importing of fractionated plasma productswere implemented
as part of a "precautionary policy" which sought to
"minimise" any potential risk.
In 2004, following the report of the first presumed case of transfusion-transmitted
vCJD, a second wave of precautionary measures was introduced:
the deferral of donors who had themselves previously received
a blood transfusion and an extension of the existing imported
In the words of one witness:
The climate that existed round about 2000 to
2005 was one of real concern. The UK blood agencies and the Department
of Health were very concerned that there was going to be [
a growth of cases of vCJD by virtue of blood transfusion. There
was, I think, a genuine desire to do something about that.
90. Several witnesses told us, however, that this
climate of concern, in which the precautionary principle had been
at the forefront of Government policy, dissipated in the late
2000s. The initial wave of vCJD appeared to have peaked and cases
were down to a handful a year, leading to a gradual diminishing
of the sense of panic that had existed a decade earlier. According
to Dr Steven Burton, Chief Executive of ProMetic Biosciences,
at this time the "spirit of collaboration" which had
previously existed between the Government, UK Blood Services and
research companies such as his "disappeared", making
it more difficult for new risk mitigation technologies to reach
the market. Dr Burton
stated that his company was now:
witnessing an environment where, from our perception,
road blocks were being placed in the way and things were being
stretched and taking longer. As soon as we achieved one hurdle,
another one was, all of a sudden, in the way.
Other witnesses argued that the Government's approach
to blood safety was, and remained, "a political issue"
and that for many years the Government's uptake of risk mitigation
technologies had been based not just on their effectiveness, but
on "public sentiment and the perceived risk and need to do
ProMetic went further, stating its belief that the decision made
by the Advisory Committee on the Safety of Blood, Tissues and
Organs (SaBTO) not to recommend adoption of its prion filtration
technology was based not on the scientific evidence, but on "other
considerations" such as cost (at a time of economic austerity)
and "a widely held belief within parts of the Department
of Health that the vCJD emergency has passed and there [was] no
need for the implementation of additional blood safety measures".
91. The Minister told us that "successive governments"
had applied a precautionary approach to vCJD and that this had
been maintained by the current administration.
However, now that the initial storm of cases has passed, we too
have perceived a change in the Government's attitude to vCJD.
During this inquiry, we have amassed considerable evidence to
challenge the Government's claim that it maintains the precautionary
approach that it has always taken. For example:
Government accepts that some of those who have potentially been
exposed to vCJD and are therefore at increased risk of transmitting
it may not have been notified of this risk. These people are therefore
not in a position to take the precautions recommended to prevent
further transmission. To our knowledge, the Government has taken
no steps to rectify this situation and has delegated significant
responsibility for ongoing surveillance to the UK Haemophilia
Centre Doctors' Organisationa body which has, for many
years now, evidently failed to maintain an accurate record of
this 'at risk' population. (Paragraphs 75-78).
· The Government
appears unconcerned by the extremely low rate of research participation
from this 'at risk' population, citing this as "a cultural
issue" and failing to assure us that it is taking any steps
to increase consent rates in order to preserve potentially invaluable
scientific information. (Paragraphs 79-81).
· The Government
tells us that it is concerned about the risk of prion transmission
via surgical instruments, but is "not aware" of evidence
suggesting that national guidance intended to reduce this risk
is not being followed. (Paragraphs 27-29).
· The Government
has failed to ensure that a technology with the potential to render
this guidance redundantwhich was itself based on publicly-funded
researchis adopted by the NHS. Seven years after DuPont's
Rely+On product received its CE mark, neither it, nor any alternative
product capable of inactivating prions present on surgical instruments,
has yet been introduced. (Paragraphs 31-38).
· Despite witnesses
overwhelmingly considering a vCJD blood test to be the most important
prospective vCJD risk reduction measureand despite the
considerable progress made in the development of such a testthe
Government has failed to declare its explicit support for this
technology. (Paragraphs 51-52). Moreover, it has taken no steps
to ensure that the prototype test developed by the MRC Prion Unit
receives the support necessary for the next stage of its development:
a blood prevalence study which could also provide valuable data
on the rate of subclinical vCJD infection in the UK donor pool.
· Current assumptions
about blood infectivity and susceptibility to infection appear
to be largely based on an analysis conducted by the Department
of Health in 2011, in which it attempted to solve the 'calibration
problem' by matching these assumptions to the observed number
of vCJD cases. This is despite fears, acknowledged by the national
surveillance unit, that there might be under-reporting of the
disease, particularly in the elderly, in whom both classical and
variant forms of CJD could feasibly be misdiagnosed as others
forms of dementia. (Paragraphs 83-87).
· After a lengthy
evaluation, SaBTO has decided not to recommend the adoption of
prion filtration: a technology with the potential to significantly
reduce the risk of prion transmission. This decision was made
following several years of evidence gathering and a detailed cost-effectiveness
analysis, neither of which were carried out in advance of the
introduction of another prion reduction measureleucodepletionin
1999. (Paragraphs 41-46).
92. We would draw particular attention to this final
point. The decision to introduce leucodepletion in the 1990s was
a genuinely precautionary step much praised by witnesses to this
had leucodepletion been subject to the same requirements in the
late 1990s that prion filtration was in the late 2000s, it would
not have been recommended. In 1999, there was little evidence
that prions could be transmitted via transfusion and none to conclusively
demonstrate that leucodepletion would mitigate this risk. Under
today's approach, it is therefore likely that leucodepletion would
not have been adopted for several years, if at all.
93. We may never know what the impact of such a delay
in the adoption of leucodepletion would have been; whether the
measure has saved hundreds of lives or wasted millions of pounds.
Because now, as in 1999, there remains "a good deal of uncertainty
about the risk" of transfusion-transmitted vCJD.
However, while the Government was previously prepared to assume
the worst and take every precaution to prevent it from happening,
its attitude now appears to be one of measured optimism, in which
the apparently low incidence of cases is repeatedly used as a
"key piece of evidence" to justify an approach which
can no longer be described as genuinely precautionary.
We consider this change to be deeply regrettable and unjustified
by the available evidence.
decision not to recommend the adoption of prion filtration, taken
alongside the other evidence that we have gathered during this
inquiry, in our view signals a change from what was a genuinely
precautionary approach to vCJD risk reduction in the late 1990s
to a far more relaxed approach today. Much of the uncertainty
surrounding prions, their potential modes of transmission and
the possible rate of undetected infection and disease remains:
recent evidence that subclinical prevalence could be as high as
one in 2,000 people would suggest that a precautionary approach
is now more warranted than ever.
fear is that the Government's current attitude is driven less
by the available scientific evidence than it is by optimism: a
hope that the storm has now passed and that vCJD is no longer
the threat to public health that it once was. In the current economic
environment, this attitude is not surprising. However, it is not
justified. For all we know, the storm may well be ongoing. We
conclude this report by recommending that the Government take
a more precautionary approach to both vCJD risk mitigation and
blood safety more generally, in order to safeguard against future
infections. We suggest that it begin by assessing the key risks,
known and unknown, that the UK blood supply currently faces and
might face in the future, so that it can identify and fill relevant
knowledge gaps and support the development of appropriate risk
reduction measures and technologies. The Government should initiate
this work immediately and we ask that it provide us with an update
on its progress well before the dissolution of Parliament.
288 See Washer, P., 'Representations of mad cow disease',
Social Science and Medicine, Volume 62, Issue 2, January
2006, pp.457-466. DOI: 10.1016/j.socscimed.2005.06.001. Washer
refers specifically to the "descriptions of the physical
and mental decline of the young people who succumbed to the disease,
juxtaposed mentally as they are with images [
] of uncoordinated
and frightened cows", which contributed to the public fear
of dehumanisation: "of becoming like a maddened (rabid) animal".
Spongiform Encephalopathy Advisory Committee (SEAC), SEAC annual report 1997-98,
p.10; p.33 Back
Advisory Committee on the Safety of Blood, Tissues and Organs
(SaBTO), Measures currently in place in the UK to reduce the potential risk of transmitted variant Creutzfeldt-Jakob Disease via blood,
December 2013 Back
Q124 [Dr Steven Burton] Back
Q125 [Mr Nigel Talboys]; Q79 [Dr Steven Burton]. See also Q74
[Dr Alex Raeber and Mr Nigel Talboys] Back
BTO53 [ProMetic supplementary] Back
It has been argued that other aspects of the Government's response
to the BSE crisis were less in line with the precautionary principle.
See, for example: European Environment Agency, Late lessons
from early warnings: the precautionary principle 1896-2000,
Chapter 15, '"Mad cow disease" 1980s-2000: how reassurances
undermined precaution, 2001. Back
Department of Health, Blood-Borne Transmission of vCJD Re-Examination of Scenarios,
September 2011, p.11. See also SaBTO, Prion reduction filters
for red cell concentrates, Agenda item 4, 10 December 2012
and Q287 [Jane Ellison MP] Back