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About CJD and CJD Support Network in Japan February of 2006

CJD Support Network in Japan


Creutzfeldt-Jakob Disease (CJD) is one of intractable and fatal diseases caused by an infectious agent called prion. There are four types of CJD: Sporadic, Familial, Variant and Iatrogenic. Sporadic CJD is said to occur in one out of one million people. Variant CJD is thought to occur in relation to Bovine Spongiform Encephalopathy(BSE). Outbreak of BSE and Variant CJD is reported from many countries, especially the UK.


Japanese government researched whether there was Variant CJD in Japan in 1996. Consequently, in Japan, however, none of variant CJD has yet been reported from 1996 until 2004. Instead, until February 2006, 117 iatrogenic CJD patients due to human dura mater grafts have been reported. This figure of dura mater victims is abnormally high in comparison with other countries.


The main reason for this is thought to be the results of using many prion contaminated human dura mater named Lyodura produced by B.Braun in Germany between 1973 and 1996. B.Braun had been adopting gamma radiation for the sterilisation of Lyodura since 1973. This method, however, was found to be ineffective for the sterilisation of CJD agent by Gibbs, Gajdusek and others in 1978. In spite of this fact, this company continued to produce Lyodura. In addition to adopting ineffective sterilisation method and not identifying CJD donor, B.Braun produced Lyodura by "pooling". This was primarily due to the packaging technique by B. Braun. In this process, 600 sheets of duras were packaged together in one plastic bag regardless of the duras condition. As the results, healthy duras were thought to be contaminated with prion-infected duras.


In 1987, the FDA in the US prohibited the use of Lyodura because the first CJD patient was reported after using Lyodura. However the Japanese government did not follow this measure and did not take any step to prohibit selling Lyodura.


In Japan, CJD victims filed suits against the Japanese government, B.Braun and BSS, the importer in Ohtsu and Tokyo since 1996. In March 2002, after 6 years of litigations, an historical out- of-court settlement was reached. The defendants apologised to CJD victims. By 2006, 71% of plaintiffs have received compensation from the settlement. The litigation is not yet finished as of February 2006.


The CJD Support Network(CS Net) of Japan was established on June 2002. Volunteers and victims themselves, medical workers, researchers, lawyers support this Network. CS Net provides information, support and assistance for any forms of CJD to patients and their families in Japan. CS Net also promotes research, education and awareness concerning CJD problems. CS Net has been getting financial support by the Japanese Government from the fiscal year of 2003 as one of the results of the settlement. In Japan, there has been 22 cases of BSE reported as of January 2006, and regrettably, one Variant CJD case has been reported in February 2005.