CJD dad’s Disney dream comes true
Source: Lynn News Friday
Published Date: 14 February 2010
http://www.lynnnews.co.uk/news/CJD-dad39s-Disney-dream-comes.6065859.jp
By Sarah Lucas
A 25-YEAR-OLD West Norfolk dad who has the human form of so-called Mad Cow Disease has had his dream holiday with his family – thanks to local support.
Dad-of-two Dean Burrell is among the youngest in Britain to have contracted vCJD or “variant” Creutzfeldt-Jakob disease, a fast-acting debilitating disease which affects movement, actions and memory.
It was when Dean was still 24 – just two days before his 25th birthday – when doctors first suspected he had the disease, which has only affected around 200 people in Britain in the last 30 years, his wife, Sarah (23), previously told the Lynn News.
Fundraising then started to enable Dean, dad to Chloe (4) and Charlie, nearly two, to go on a trip of a lifetime to see Manchester United.
Over £4,000 was raised for the family, of Northgate, Hunstanton, though tickets for a Man Utd game against Aston Villa in December were sent to Dean, meaning the family could then concentrate on their other wish – taking him to Euro Disney.
And, recently, the trip has become reality. Dean, together with Sarah and the children, spent four days at the French resort and had the time of their lives, Dean’s mother, Marie Dugard, said. The holiday was thoroughly enjoyed, said Mrs Dugard, adding that the family could not thank local people enough.
Unable to fly because of Dean’s on-going condition, the family travelled by Euro Star to the resort. Other family members also went, though paid for themselves.
It is thought as little as one meal of contaminated meat could have given Dean the condition, which he must have contracted between birth and the age of 12.
It was 1996 when it was discovered the disease had jumped to humans and the decision was made to remove tissue from beef, such as the spinal chord, which may have carried the agent that causes CJD. No case of vCJD has been identified in the UK in individuals born after 1989.
Dean, a scaffolder who used to work at Narford in Swaffham, is unable to work and Sarah gave up her job at Old Hunstanton’s Le Strange Arms Hotel to look after him. The couple only married last July. Sadly, Dean’s prognosis is poor, his condition constantly deteriorating.
Mrs Dugard is desperately trying to get hold of some tickets for Dean to attend the Six Nations rugby championship. Anyone who can help is asked to ring her on 01553 676030
변종 크로이츠펠트-야콥병 진단 및 분류를 위한 기준
출처 : 질병관리본부, 크로이츠펠트-야콥병 및 변종 크로이츠펠트-야콥병 관리지침, 2009
○ 환자 : (표 4)의 IA(진행성 신경-정신 질환)이면서 변종 크로이츠펠트-야콥병과 부합하는 합당한 신경병리학적 소견을 보이는 자
○ 의사환자
① 추정환자(probable)
– (표 4)의 I, II, IIIA, IIIB를 모두 만족하는 자, 또는
– (표 4)의 I과 IVA에 해당하는 자
② 의심환자(possible)
– (표 4)의 I, II, IIIA를 만족하는 자
표 4 변종 크로이츠펠트-야콥병 진단기준 I A 진행성 신경-정신 질환 B 이환기간이 6개월 이상 C 일반검사에서 다른 진단을 의심할 만한 소견이 없음 D 전파 가능한 의인성 위험요인에 폭로된 과거력이 없음 E 가족성 크로이츠펠트-야콥병의 증거가 없음
II 다음 중 4가지 이상의 소견 A 초기에 나타나는 정신과적 증상2) B 지속적인 동통성 이상감각증3) C 운동실조 D 근경련증이나 무도증 또는 근긴장이상증 E 치매
III A 뇌파검사상 산발성 크로이츠펠트-야콥병의 전형적 소견4)을 보이지 않는 경우 (또는 뇌파검사를 시행하지 않은 경우) B 뇌 자기공명영상에서 양측 시상베게에 고신호강도가 있는 경우
IV A 편도 조직검사상 변종 크로이츠펠트-야콥병에 합당한 소견5)
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