Despite the rareness of a CJD diagnosis in Ireland, there have been a number of high-profile incidents involving the lethal condition in recent years.
From one of the longest survivors of the disease in history to court battles over the first human use of a highly controversial drug treatment, calls for a compensation system for those needlessly infected. to an infection person donating blood, CJD has grabbed the headlines whenever it has appeared.
Jonathan Simms confounded medical experts by living for 10 years with CJD, one of the longest known survival rates.
The Belfast man, who died in Mar 2011 aged 26, contracted variant CJD in May 2001, after initially being diagnosed with multiple sclerosis.
He and his family were initially told he could die within months. However, Mr Simms survived until Jan 2003, when he won a high-profile court battle for the right to use the experimental drug pentosan polysulphate.
It was the first time the medication had been used on a person.
In 2005, then health minister Mary Harney was embroiled in a major controversy after it emerged a man suspected to be infected with a form of CJD was allowed to donate blood.
Two people received blood from the man, who was being treated in a Dublin hospital at the time, one of whom subsequently died from an unrelated condition.
Ms Harney insisted the blood supply system was secure and that all safety checks had been followed.
In 2006, concerns were also raised by a county coroner over a potential “cluster” of CJD infections in the South Dublin/Wicklow area.
During the inquest into the death of Jason Moran, 24, from Shankill in Co Dublin, coroner Dr Kieran Geraghty said he was concerned about three incidents occurring in a five-mile radius of the Bray area.
At the time, then opposition TD Eamon Gilmore called for an inquiry into what happened, and for a compensation system to be potentially set up if there was proof the infections were preventable.
A number of other cases have also hit the headlines in recent years, including the death of a Cork-based father of two just months after Mr Simms passed away.
The 48-year-old long-haul truck driver from the north side of Cork City, whose identity has never been revealed, was originally from England but had been living in Ireland for more than a decade.
HSE figures show there have been 30 cases of CJD in Ireland in the past six years — an average of one every 10 weeks.
In addition, there have been four known cases of vCJD — a condition linked to BSE in cattle — since the disease was identified in 1996.
- Anyone worried about whether they may have contracted CJD at Beaumont is urged to contact the HSE’s dedicated helpline number on: 1800-302-602.
Condition triggers cause for concern
Q: Why is CJD back in the news?
A: On Thursday the HSE confirmed up to 20 people may have been infected with Creutzfeldt-Jakob Disease, more commonly known as CJD, due to potential surgical cross-infection. All of the patients were treated at Beaumont Hospital between Jun 1 and Jul 15.
They were contacted by the HSE yesterday and are currently being tested for the lethal disease.
The risk of infection is because a patient who attended Beaumont at the start of June was subsequently diagnosed with CJD. This patient may have been treated with the same medical equipment the 20 other patients where in contact with, meaning they could have accidentally been infected by the extremely dangerous illness.
Q: How many people are affected?
A: The HSE has yet to clarify the exact number. However, an initial statement from officials said the figure is between 10 and 20.
Q: If doctors cleaned the equipment, why is there a problem?
A: Normal equipment cleaning is insufficient to remove any CJD danger.
Under national and international rules, any equipment used on the brain of someone with the condition must be thoroughly sterilised as part of a “deep clean” or be destroyed.
The difficulty for Beaumont and the HSE is that they appear not to have been aware the initial patient was suffering from CJD for up to two months after the individual was treated — meaning other patients received care before this “deep clean” process took place.
Q: What is likely to happen to those affected?
A: It is still unclear how many, if any, of the 20 potential victims have in fact contracted CJD. However, CJD is a lethal condition for which there is no cure.
The lethal degenerative neurological disorder acts by effectively causing holes in brain tissue. This occurs when the protein at the centre of the condition, prion, develops in a way that causes it to fold in on itself. The main symptoms of the condition are dementia, memory loss and hallucinations.
This is generally followed by speech problems, co-ordination and seizures. Early dementia symptoms, coma, and ultimately death can quickly follow.
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