Thursday, November 12, 2009

Pewsey, died on October 22, more than six years after being diagnosed with Variant CJD

Family say goodbye at Calne funeral of CJD victim


2:36pm Thursday 12th November 2009



By Lewis Cowen »

Tribute was paid yesterday to the bravery of Edward Peduzie, from Calne who died aged 25 after a long battle with Creutzfeldt-Jakob Disease, CJD.

The former painter and decorator, who shared his time between the home of his father in Stokescroft and his mother's house in Pewsey, died on October 22, more than six years after being diagnosed with Variant CJD, the human equivalent of BSE, or mad cow disease, Friends believe the care given by Mr and Mrs Peduzie to their son was the reason he was one of the longest survivors of the incurable degenerative brain disease.

The funeral service took place at St Mary’s Church in Calne yesterday and Mr Peduzie’s coffin was carried in to the sounds of his favourite rap music.

Mr Peduzie’s mother, his father Paulsister Vicky and brother Benjamin led the mourners at the service attended by family and friends.

In his address, the Rev Bob Kenway told the congregation that Mr Peduzie was educated at Pewsey Vale School and qualified as a painter and decorator at Swindon College.

He worked for Calne building firm Wilkins before the disease made it impossible for him to continue with them.

Paul Peduzie told the congregation: “When Ed first became ill, if you asked him if he was all right, he would say, I’m all right.

“Then when he lost the use of his voice and you asked him the same question, he would give you a thumbs-up.

“When he lost the use of his hands, he just smiled. He was the bravest person I ever knew and I am proud to be his dad.”

After the congregation sang Jerusalem, Mr Peduzie’s coffin was carried out to the strains of Swing Low, Sweet Chariot. He had been a keen supporter of Rugby Union.

Although Mr Peduzie had been diagnosed with Variant CJD, doctors do not believe he contracted the disease from contaminated meat.

The family were supported through Mr Peduzie’s illness by the CJD Support Network and mourners were invited to make contributions to the charity.

Maggie Rae, the director of public health in Wiltshire, said CJD was an extremely rare disease.

She said: “I want to reassure people that this rare case poses no infection risk to the public. There have been fewer than 170 cases of Variant CJD in the UK since 1995.”

A new form of CJD, called Variant CJD, was identified in 1996. In May 1995, air cadet Stephen Churchill of Devizes was the first person to die from it. He was 19.

The Health Protection Agency said Variant CJD is strongly linked to people eating meat from cattle infected with BSE. However the possibility of contracting Variant CJD from surgical procedures cannot be ruled out.

The Department of Health says there have been four possible cases of Variant CJD transmission through blood transfusion.

As a result of this, the Government announced that people who had received a blood transfusion since January 1980 would be excluded from donating blood.


http://www.thisiswiltshire.co.uk/news/4736033.Family_say_goodbye_at_Calne_funeral_of_CJD_victim/




Greetings,


Interesting, sad, but interesting.


"Although Mr Peduzie had been diagnosed with Variant CJD, doctors do not believe he contracted the disease from contaminated meat."


Seems they may assume Mr Peduzie did not eat meat. That would be hard to assess fully over a lifetime i.e. cross contamination via processing of foods etc. however, nvCJD is as likely to transmit via Iatrogenic routes as the oral route, in my _opinion_. SO, we are left to ponder once again. That's the saddest part.


Interesting also, is the length of illness to death, from diagnostic date ;


"more than six years after being diagnosed with Variant CJD".


I wonder how long Mr Peduzie was subclinical ???



kind regards,
terry






Tuesday, November 03, 2009

SEVENTEENTH ANNUAL REPORT 2008 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK


snip...


2.3 Variant Creutzfeldt-Jakob Disease Up to 31st December 2008, 167 cases of definite or probable vCJD had been identified in the UK (115 definite, 49 probable who did not undergo post mortem and 3 probable cases still alive). Seventy-three (44%) of the 167 cases were women. The median age at onset of disease was 26 years and the median age at death 28 years (compared with 67 years for the median age at onset and 67 years for the median age at death for sporadic CJD). The youngest case was aged 12 years at onset while the oldest case was aged 74 years. To date, no case of vCJD has been identified in the UK in individuals born after 1989. The age- and sex-specific mortality rates for vCJD over the time period 1 May 1995 to 31 December 2008 are shown in Figure 6. The median duration of illness from the onset of first symptoms to death was 14 months (range 6-40) compared with a median duration of illness for cases of sporadic CJD of 4 months (range 1 to 74) during the period 1990-2008.


In 2008 the NCJDSU was referred for the first time an individual who met the clinical criteria in life for possible vCJD and who was heterozygous (methionine/valine) at codon 129 of the PRNP gene. This individual died in 2009 after a disease of 22 months duration. Consent for a post-mortem was not given. The clinical picture was typical of vCJD seen to date, which is reassuring for surveillance purposes since the clinicopathological phenotype of vCJD in this genotype is unknown. To put this possible vCJD case in perspective, it is useful to examine the final diagnostic outcome of the 116 suspect vCJD cases that were classified as possible vCJD at some point during their diagnostic pathway (that is, they met the criteria for possible vCJD at some point between referral to NCJDSU and death or other final diagnostic outcome). Of the 116 possible vCJD cases, 94 (81%) had a final classification of definite or probable vCJD, 10 (9%) had a final diagnosis of definite sCJD, 5 (4%) had alternative diagnoses to CJD (Alzheimer’s disease, Wilson’s disease, viral encephalitis, syphilis, SSPE), one was diagnosed with genetic CJD, one improved clinically and for one individual the diagnosis remains unclear, but clinically was suggestive of vCJD. Four cases (3%) have resulted in a final classification of possible vCJD, 3 were methionine homozygotes at codon 129 and the recent case heterozygous (methionine/valine) at PRNP codon 129. On the basis of our knowledge of the natural history of other human prion diseases, clinical cases of vCJD in PRNP codon 129 genotypes other than methionine homozygotes could be anticipated.



snip...see full text ;


http://www.cjd.ed.ac.uk/report17.pdf




http://creutzfeldt-jakob-disease.blogspot.com/2009/11/seventeenth-annual-report-2008.html





Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification



Abstract

Six subtypes of sporadic Creutzfeldt–Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenotypic features and concurrent PrPSc types has been reported but with inconsistencies among studies in both results and their interpretation. The issue currently complicates diagnosis and classification of cases and also has implications for disease pathogenesis. To explore the issue in depth, we carried out a systematic regional study in a large series of 225 cases. PrPSc types 1 and 2 concurrence was detected in 35% of cases and was higher in MM than in MV or VV subjects. The deposition of either type 1 or 2, when concurrent, was not random and always characterized by the coexistence of phenotypic features previously described in the pure subtypes. PrPSc type 1 accumulation and related pathology predominated in MM and MV cases, while the type 2 phenotype prevailed in VVs. Neuropathological examination best identified the mixed types 1 and 2 features in MMs and most MVs, and also uniquely revealed the cooccurrence of pathological variants sharing PrPSc type 2. In contrast, molecular typing best detected the concurrent PrPSc types in VV subjects and MV cases with kuru plaques. The present data provide an updated disease classification and are of importance for future epidemiologic and transmission studies aimed to identify etiology and extent of strain variation in sporadic Creutzfeldt–Jakob disease.

snip...






http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html






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