What is CJD?
Creutzfeldt-Jakob Disease (CJD) is a rare neurological disease that causes brain damage. It belongs to a group of diseases called transmissible spongiform encephalopathies (TSEs) or prion disease. It is fatal and there is no known cure. There are a number of types of CJD. Sporadic CJD , which is the case identified at Beaumont Hospital, is the most common form. Its cause is not clear, but is believed to be due to prion protein changes in the brain. The less common variant CJD (vCJD) is caused by eating meat infected with BSE (sometimes called 'mad cow' disease). Older people, usually over 40-50, tend to get sporadic CJD, whereas vCJD tends to develop in people in their 20s.
What happens to people who get CJD?
The disease causes a person's neurological functioning to deteriorate rapidly to the point where they can no longer care for themselves and they become immobile and unable to speak. With sporadic CJD, dementia develops early and the length of survival after developing the disease can be as little as four months. There is a 14 months survival window with vCJD.
How common is CJD?
Estimates vary, but it is reckoned to affect one in a million people. Seven cases of sporadic CJD were notified in Ireland in 2011 and three in 2010. Four cases of vCJD have been reported since December 1996.
How concerned should we be about the Beaumont case?
We should obviously be concerned about anyone unfortunate enough to develop CJD or that up to 20 patients must endure anxiety over whether they may have been infected through surgical instruments used on the patient concerned, however small the risk. This type of case very rare and health authorities have moved to reassure people that there have been no recorded cases worldwide for 30 years of a person contracting CJD via surgical instruments. Varying levels of possible risk for the affected patients have been quoted - however, overall the level of risk to these patients is believed to be very low, it a little bit higher than in the general population
Did the hospital do all it could do to try to prevent other patients being affected?
Unlike with other patient treatment controversies, there appears to be no evidence as yet of serious sins of omission. The hospital says it uses current guidelines on the prevention of transmission of CJD and says necessary pre-surgery checks would have been carried out in this case. Health Minister James Reilly has stressed that there had been no clinical suspicion prior to the discovery of CJD in a routine pathology test on the patient post-surgery that they might have the condition. Beaumont says as soon as the diagnosis of CJD was made (by which time, unfortunately, other people had been been operated on with the same instruments as were used on the infected patent) the instruments were quarantined and a review began on the possible risk to other patients.
Experts say that due to the nature of CJD, it can be difficult to detect in its earlier stages, and the patient's symptoms did not signify CJD, although the disease develops rapidly. On the basis of information provided to date, while the information provided has been very minimal, there appears to have been little more the hospital could have done in the absence of any clinical suspicion of the disease before the patient was operated on.
Would the instruments/equipment used not normally have been sterilised before it was used again, and would this prevent infection?
The instruments would presumably have been sterilised as normal, but with suspected CJD contamination, special sterilisation of instruments with sodium hydroxide or sodium hypochlorite is needed. Unfortunately, as there was no suspicion of contamination at the time, the instruments were presumably not sterilised in this way.
Why is there relatively little information emanating from the hospital/HSE about the case?
So far, this is the main area where the health authorities can be criticised for their handling of the case, although they will claim they made the right judgment call by releasing minimal information. In any case such as this, the authorities have to balance the need for the public to know about a major health issue versus the rights of affected people and their families to privacy and confidentiality. Notwithstanding the HSE/Beaumont's understandable concerns in a media-intrusive age, most reasonable members of the public would expect to be told exactly how many patients at Beaumont may be affected by potential contamination, and whether any children are affected (Beaumont is the main centre for carrying out neurosurgery procedures on children). So far we have not been told this. It has so far not even been officially confirmed that the incident happened in the hospital's neurosurgery deparment, although it is widely known by now that this was where it occurred.
The HSE has also been criticised for making public the CJD issue before it told affected patients and their families, but it may have been anxious to reveal the incident prior to it being potentially 'leaked' to the media. This, however obviously caused additional concern among the public. Beaumont has been contacting the affected patients, who will have to be monitored in future to check for signs of the disease.
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