13 May 2022: Collinge - A

(Unfortunately, due to late arrival, the introduction involving the witnesses’ CV review was missed.)

Prof Collinge explained that a prion is a protein not a virus. It is an anomaly of normal forms of protein and has the capacity to link into chains, split, self-propagate (most often in the brain and spinal cord), and do various forms of damage, including fatalities. Variant CJD is a product of exposure to encephalopathic catalysts (?) It is acquired, not naturally or randomly occurring as some forms of CJD are. It is often caused by consumption of contaminated food, but it can also be an infection from injecting with blood or blood products.

The BSE era was the mid-1990s; remember the horrific burning pyres of cattle carcases. It was discovered that the vCJD prions had the unusual capacity to exist not only in the brain or spinal cord, but also in the blood and elsewhere, which when added to the rising number of cases, increasingly led to the realisation of the potential of passing the prion through blood products. The studies had to involve observing the passage and progress in animals since it was not possible to do studies on humans. There was no immediate impact on the infected person – they were asymptomatic – so people would not know they were infected and could go about their normal life, including donating blood. Incubation periods are typically long, and start from six or seven years, often more than ten years.

Symptomatically, the onset of vCJD is usually manifest psychologically, including depression, and other socially impacting issues. Latterly, these progress to physical impacts more typical in CJD including pain, dementia, complete incapacity, and death. There are indicators to give a “probable” diagnosis, and that can be confirmed by autopsy. There are no cures for CJD despite various trials. Currently, there are promising results after the use of monoclonal antibodies, but all that can be done at the moment is treating the effects of the illness, but not curing it.

Early diagnosis is always beneficial, not least because it allows the patient to have the capacity to make their own decisions about their care for as long as possible, and to le them orientate the remainder of their lives to maximise the available time with loved ones.

Back in the day, the relatively small number of blood-infected people were advised of a low risk assessment, yet out of an initial study group of about 23, three had already died. The dissemination of a low-risk situation negated an option to offer psychological counselling and specialist medical care, and in the witnesses’ opinion it should have been. The letter sent out to people, via Dame Sally Davies, said the risk appeared to be “very small”, but Prof Collinge was concerned about this minimisation. He was also concerned about who was taking clinical responsibility for those who received warning letters. His specialist unit was available to help anyone who became anxious or ill, but he had no way of accessing patients to offer support. This is despite writing to the most senior health policy-makers.

Prof Collinge wanted people to be offered indicative tests, the option of being involved in a trial, and long-term monitoring. He was “deeply concerned” about how these patients were being treated, (or not treated, it seems). After a year of writing letters, a patient came to the witness via a GP referral which sadly confirmed the reality of the risks he had been raising. After writing again to the CMO, Prof Collinge received a reply from a senior civil servant on behalf of the CMO which was typically full of civil servant-speak. The case of a man who had CJD came to his attention after that man had been diagnosed with and treated for clinical depression only. In reality, the poor man’s situation was much more serious. Sir Brian summed up the situation and frustration. He suggested it was a case of Prof Collinge’s specialist service having been set up, but the communication of its existence to those who needed to know about it was just not there. The “low risk” message just made it worse.