Family Support Meeting

The CJD Support Network's annual family support meeting will be held on Saturday November 20 2010 and is open to all members of the network.

For further information and to book please contact us at info@cjdsupport.net or 01630673993.

Latest newsletter

Read a good mixture of news, research, conference summaries and carers' stories in our latest March 2010 newsletter - March 2010

Clicking on newsletters on the left hand side of the home page will also take you to the March 2010 newsletter and previous newsletter editions.

vCJD and patients with bleeding disorders

The Health Protection Agency informs us that a person with haemophilia has been found to have evidence of infection with the agent (abnormal prion protein) that causes variant Creutzfeldt-Jakob Disease (vCJD) only in his spleen at post mortem. All Haemophilia Centres are contacting their patients throughout the UK to give them this information.

Tests carried out on a haemophilia patient who died last year have shown that he was infected with variant Creutzfeldt-Jakob Disease (vCJD). The patient did not die of vCJD, and never had any symptoms of this disease when he was alive. The patient was in his 70s when he died of a completely unrelated cause. The tests were carried out as part of an ethically approved research study jointly co-ordinated by the UK Haemophilia Centre Doctors Organisation and the National CJD Surveillance Unit.

This haemophilia patient had been treated with several batches of UK-sourced clotting factors, including one batch of factor VIII that was manufactured using plasma from a donor who went on to develop vCJD. 

A final view as to how this haemophilia patient became infected with the vCJD abnormal prion protein has yet to be reached and investigations are therefore continuing to establish this. 

This is the first time that the vCJD abnormal prion protein has been found in a patient with haemophilia, or any patient treated with plasma products. This patient did not die of vCJD, and the only reason we know he was infected with the vCJD abnormal prion protein is because of the research tests carried out after he had died.

This new information does not change the way any patients with bleeding disorders will be treated.

Patients who have a bleeding disorder or congenital antithrombin III deficiency [1] and who received clotting factors or antithrombin derived from UK-sourced plasma [2] between 1980 and 2001, should have already been told that they have an increased risk of vCJD, and should be following public health advice (see box below).

If patients are unsure about this and would like more information, they should contact their haemophilia centre.

Other patients with bleeding disorders who do not have an increased risk of vCJD, do not need to take any action. Patients should contact their haemophilia centre if they are unsure about their vCJD ‘at risk’ status.

This case does not change the public health ‘at risk’ status of any patients with bleeding disorders.

Patient information leaflets and other information are available on the Health Protection Agency website http://www.hpa.org.uk/vcjdplasmaproducts

Advice on how to reduce the risk of spreading vCJD to other people If you have been identified as being at increased risk of vCJD, you can reduce the risk of spreading vCJD to other people by following this advice.

• Don’t donate blood. No-one who is at increased risk of vCJD or who has received blood donated in the United Kingdom since 1980 should donate blood
• Don’t donate organs or tissues, including bone marrow, sperm, eggs or breast milk
• If you are going to have any medical or surgical procedures, you should tell whoever is treating you beforehand so that they can make special arrangements for the instruments used to treat you
• You are advised to tell your family about your increased risk. Your family can tell the people who are treating you about your risk of vCJD if you need medical or surgical procedures in the future and are unable to tell them yourself.

Background information on the 2004 notification

In 2004 the HPA, the UKHCDO and colleagues notified patients who had received plasma products manufactured using plasma from donors who had developed vCJD. That notification dealt with plasma donations which had been used to manufacture factor VIII, factor IX, antithrombin, intravenous immunoglobulin G, albumin, intramuscular human normal immunoglobulin and anti-D.

Patients treated with these plasma products were managed according to an assessment of potential vCJD infectivity carried out by the Health Protection Agency with the CJDIP.

The UK Haemophilia Doctors and patient representatives, the CJDIP and UK Health Departments agreed that it was likely that many patients with bleeding disorders would have had a sufficient exposure to these implicated plasma products to put them ‘at risk of vCJD for public health purposes’. It was also thought likely that further batches of UK-sourced plasma products would be implicated in the future as more cases of vCJD arose.

Therefore all patients with bleeding disorders who were treated with UK-sourced pooled factor concentrates or antithrombin between 1980 and 2001 are ‘at risk of vCJD for public health purposes’ and public health actions should be taken. This risk is in addition to the general risk of vCJD that many people in Britain have through eating beef or beef products.

[1]

Congenital and acquired haemophilia (Haemophilia A and Haemophilia B), Von Willebrand Disease, other congenital bleeding disorders and congenital antithrombin III deficiency.

[2]

Factor VIII, factor IX, factor VII, factor XI, factor XIII and prothrombin complexes, as well as antithrombin.

vcjd and blood transfusions

A new factsheet, vCJD and blood transfusions, has been added to the factsheets published by the CJD Support Network.

 This leaflet  answers some commonly asked questions by people who have been told that they are at a higher risk of CJD through a blood transfusion.  A copy of this leaflet can be viewed or downloaded by following the link, or can be obtained by telephoning the CJD Support Network helpline on 01630673973.

click here to download vcjd and blood transfusions

2 cases of vCJD in one family

Jesus de Pedro Cuesta of the Spanish CJD Registry today confirmed that there have been two cases of vCJD in one family in Spain.  This is the first time in any country that two cases of vCJD have been identified in one family.

Professor Bob Will of the National CJD Surveillance Unit in Edinburgh today issued the following statement

 STATEMENT ON A VARIANT CJD FAMILY CLUSTER

Three cases of pathologically confirmed variant CJD have been identified in Spain in recent years, including a man in his early 40s who died earlier this year. The clinical illness in this individual was typical of variant CJD, including the appearances on the MRI brain scan.

A few months ago his mother, who was in her 60s, developed a rapidly progressive neurological illness and died about 5 months from the onset of this illness. An MRI brain scan showed appearances suggestive of variant CJD and preliminary results from post-mortem examination suggest that the suspected diagnosis of variant CJD is correct. Further results, which may confirm this diagnosis, should be available within a few days.

Since 1994 there have been 167 cases of variant CJD in the UK, 23 cases in France and 15 cases in other countries, excluding Spain . The occurrence of variant CJD in more than one member of the same family has not been seen before and it has been the general view that family members of variant CJD cases are not themselves at greater risk of developing this condition. This raises the question as to why two cases of variant CJD have now been found in a family in Spain .

 There is no evidence of a genetic form of CJD in these Spanish cases and preliminary investigation has not shown any risk of CJD through medical or surgical treatment. There is no evidence of any risk of transmission of CJD through direct personal contact. The mother and son lived in an area of Spain in which BSE has been found and it is possible that direct consumption of material with high levels of BSE infection may have been the source of the infection. In the UK and other countries it is believed that processed bovine tissues were the most likely source of BSE infection and it is possible that different forms of exposure to BSE infection may explain the occurrence of variant CJD in two family members in Spain and not elsewhere.

This, however, is uncertain and public health policies in relation to variant CJD may have to be reviewed in the light of these two cases in Spain .

For more information on this news, please telephone our helpline on 01630673973.

 

 

 

 

 

 

Another "Mad Cow" reference by the media

A member of the CJD Support Network has contacted us about a reference made "do you have mad cow or something?" by Mike Parry in TalkSport on the 10 August at 16.00.

This was a friendly debate and banter to where Everton would finish in the league, Mike Perry questioned Andy Townsend's judgement for him having the view that Everton would not finish fourth stating "do you have mad cow disease or something?".

A letter sent to Martin Campbell, Head of Content and Standars Radio Team" martin.campbell@ofcom.org.uk, brought the following reply

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Ofcom's response

Mike Parry, talkSPORT, 10/08/2007, 16:00

Thanks for contacting us.  We have noted your concerns about the reference to Mad Cow Disease in this programme.  The broadcasters are responsible for what content they choose to have in their programmes. 

We consider complaints about such matters in relation to Section Two of the Ofcom Broadcasting Code, which requires that generally accepted standards must be applied to protect viewers from the inclusion of material which may cause harm or offence.

In applying generally accepted standards, the Code directs broadcasters to ensure that material which may cause offence is justified by the contexts, and also not to include material which, taking into account the context, condones or glamorises violent, dangerous or seriously antisocial behaviour and is unlikely to encourage others to copy such behaviour.

Context includes but is not limited to:

*    the editorial content of the programme;

*    the service on which the material is broadcast;

*    the degree of harm or offence likely to be caused

*    the  likely expectation of the audience.

The reference occurred after Andy Townsend said that Everton would be 12th in the premiership table.  This of course had Parry flummoxed and subsequently strongly disagreed by saying that such a comment wasn't intelligent.  He then asked whether he had Mad Cow disease for even suggesting it.  While we appreciate your distaste and offence at this, we do not believe it was made in a deliberate or serious intent to denigrate any sufferer of CJD or their families or make light of the illness in general.

Given the above, we do not feel this has been exceeded audience expectations or is at odds with the generally accepted standards.

Thank you for taking the time to give us your views.  Even when we don't uphold complaints, it is always useful for us to receive feedback on the output of our licensees.

Yours sincerely

Robert Ince

If you would like to support our member in this complaint please either contact us at the CJD Support Network or write direct to Oftcom.  References like this made by media personalities are offensive to all those families who have been affected by this devastating disease.

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Research shows that blood is a very efficient carrier of CJD

Research published in the Lancet  (www.thelancet.com Vol 368 December 9 2006) strengths the knowledge that blood is a very efficient carrier of CJD.

Professor John Collinge, Director of the National CJD Prion Unit, and others publish findings of a case study on the Clinical presentation and pre-mortem diagnosis of vCJD associated with blood transfusion. The case study gives details of a third case of transfusion-associated vCJD infection, identified before death.  This was the third case  of a group of 23 known recipients who survived at least 5 years after receiving a transfusion from donors who subsequently developed vCJD. 

 This study concludes that the risk to the remaining recipients of such transufsion is probably high, and these patients should be offered specialist follow-up and investigation. 

Professor John Collinge in an interview on Radio 4 (9 December 2006) said that it was important that we get early results from an anonymised study on 100,000 tonsils being undertaken at the Health Protection Agency to indicate how many people in the UK are incubating CJD,  He also said that there was an urgent need for a test to be developed to screen blood as soon as possible.

If you are concerned about the significance of this new research please telephone our helpline on  01630 673973. 

CJD Fears prompt blood donor ban

The Health Minister, Rt Hon John Reid announced in the House of Commons that people who had received a blood transfusion since 1980 would be banned from donating blood.

This is a further precautionary measure against the possible risk of variant Creutzfeldt-Jakob Disease (vCJD) being transmitted by blood and blood products.

Related Link: http://www.doh.gov.uk http://www.nhsdirect.nhs.uk/misc/ht.asp?ID=278

Pentosan

Dr Richard Knight, Consultant Neurologist at the National CJD Surveillance Unit has written a "position statement" on treatment for CJD with Pentosan.

Dr Knight's article on treatment with Pentosan can be read, together with other articles on topical issues surrounding CJD.  This article can be read and downloaded from the website in Issue 13, March 2004 of the CJD Support Network newsletter.

Related Link: mailto:info@cjdsupport.net