Harmonizing to the World Health Organization ( W.H.O ) fact sheet, no. 180, the Variant Creutzfeldt-Jakob disease ( vCJD ) is a rare but fatal happening in human existences. The organisation labels the disease as a neurodegenerative status, classified as a “ Catching Spongiform Encephalopathy TSE ” ( WHO, 2011 ) . The fatal encephalon upset affects one individual in a population of a million people in the universe annually ; hence, is a really rare disease. Harmonizing to the Centers for Disease Control and Prevention, “ the average age at decease for vCJD patients is 28 old ages, compared with 68 old ages for patients with authoritative CJD ” ( CDC, 2010 ) . These statistics show that vCJD can remain in a individual for rather a long clip without doing the bearer ill or demoing profound symptoms. Some of the profound symptoms associated with the disease as it progresses include psychiatric symptoms such as depression, backdown and anxiousness, sensory symptoms neurological abnormalcies such as ataxy. The patterned advance of the disease finally leads to decease ( Bradley, p. 1759 ) .
Although the Creutzfeldt-Jakob disease ( CJD ) foremost appeared in 1920, a new discrepancy of CJD appeared in 1996 in the UK. It is possible to pull analogues and contrasts between these two diseases. They both lead to fatal progressive devolution of encephalon cells taking to decease. A contrast between the two diseases is that vCJD has the possibility of infecting much younger people than the other signifiers of CJD. In add-on, transmittal of the variant type of this disease can be by eating contaminated beef merchandises and blood transfusion. Unlike CJD, vCJD has wholly different symptoms and a longer survival period after the oncoming of symptoms, which is above one twelvemonth, ( an norm of about 14 months ) while CJD has a survival period of one twelvemonth after the oncoming of its profound symptoms. vCJD is besides characteristic of bring forthing a rare abnormalcy in encephalon tissue referred as “ aureate plaques ” , which are seldom or non found in the other signifiers of CJD ( CDC, 2010 ) .
Evidence collected since its description, seems to indicate that a nexus can be established with the eruptions of Bovine Spongiform Encephalopathy ( BSE ) , otherwise known as “ huffy cow ” disease with vCJD. The discrepancy is extremely linked to exposure, particularly through nutrient to a catching spongiform brain disorder of cowss referred to as Bovine Spongiform Encephalopathy ( BSE ) . Scientific cogent evidence point out that the agent that causes eruption of the prion disease in cowss is the same agent that causes vCJD in worlds. Both conditions in worlds and cowss lead to fatal encephalon diseases caused by catching agents referred to as prions ( proteinaceous infective atom ) , which are composed of “ misfolded prion proteins ” . Prions are the lone known catching agents other than bacteriums, parasites or Fungis, considered as “ pure proteins ” ( Bishop, Will & A ; Manson, 2010 ) . Research has shown that the incubation period for vCJD is around 10 old ages. Research workers and scientists have nevertheless non established a vaccinum to cover with the disease. The prions are responsible for doing progressive encephalon harm and the profound symptoms characteristic of the disease. They attack the encephalon and kill tissues, which creates spreads in the tissues. The European Centre for Disease Prevention and Control, points out that “ prions are stable and comparatively immune to proteaseas, high temperatures, UV radiation, and normally used germicides ” ( ECDC, 2011 ) .
Harmonizing to the Food and Drug Administration, “ Persons in the UK are at a greater hazard for this rare disease than are persons elsewhere because of the old higher hazard of possible exposure to contaminated beef in the UK diet ” ( FDA, 2009 ) . To back up this claims, the FDA points out that statistics carried out between1995 and 2007, indicate that 165 out of the 202 persons diagnosed worldwide are from the UK, with the US holding merely three instances reported. Two of the victims in the US instances had lived in the UK between these periods. The disease peaked in 1999 and started worsening afterwards. However, there is a really low hazard of geting vCJD even after devouring the septic meat, with the FDA saying that the “ current hazard of geting vCJD from eating beef and beef merchandises appears to be negligible ” ( FDA, 2009 ) .
Although the transmittal of vCJD is chiefly through ingestion of septic cowss merchandises, there are instances in the UK where the agent blood transfusions have been the cause of transmittal of the agent. Four people infected after having ruddy blood cells, had gone through the transfusion procedure from three givers who were vCJD bearers. However, there has been no grounds linked with the spread of the disease through personal contact such as caressing, caressing or even holding sexual brushs ( FDA, 2009 ) .
Since the chief cause of the happening of vCJD is through the ingestion of beef merchandises, accent has chiefly been on cut downing the spread of BSE. This is through curtailing importing of animate beings from states that have high prevalence rates of BSE. Most Europeans states have in the past few old ages increased the surveillance of BSE and placed prohibitions of bad merchandises. The other type of transmittal of the disease, blood transfusion has seen enhanced actions by medical governments to cut down the spread of prions. Restrictions on donating blood are tightly in topographic point for persons who lived in the UK between 1980 and 1996, when the prevalence of the disease was high. In the UK, “ cosmopolitan leukocyte decrease was introduced ” , as a preventive step to cut down transmittal of the disease through blood transfusion ( Pall, 2011 ) . The medical fraternity in the UK ensures that all babies born after 1996 receive blood plasma from outside the UK to cut down the hazard of vCJd. However, research seems to propose that the effectivity of leukocyte decrease stands at merely 42 % proposing that the hazard of transmittal is still well high, particularly sing the fact that “ non all prions found in the blood are cell associated ” ( Pall, 2011 ) . Persons who have received “ pituitary derived endocrines, dura affair, sclerotic coat or corneal transplants ” from the UK are differed from donating blood in most European states ( Moiz, 2006 p. 78 ) .
The human death of vCJD makes it imperative to device schemes to antagonize its fatal nature. It is of import to observe that other signifiers of CJD have a negligible hazard rate in transmittal through blood transfusion ; the same is non applicable to vCJD. The fact that current information shows that four instances of transmittal of the disease has taken topographic point through blood transfusion, makes it an issue of concern ( CJD, 2011 ) . Since there is no known vaccinum or intervention, the lone manner to cut down its infection through blood transfusion is a device that cleans the blood of the giver from the proteins responsible for doing the disease. The effect behind filtering is capturing the misfolded proteins ( prions ) , an action that eliminates the possibility of prions aggregating in the encephalon. The fact that no trials exist to find if an person has the vCJD proteins, leads to limitations on finding who has the prions and this increases the hazard of the disease distributing. There is a high possibility that some people may be asympotomatic bearers, which means they have the prion proteins but have non developed the clinical unwellness. The promise that the prions filter device offers could change this state of affairs and cut down the likely goon of conveying the vCJD disease through blood transfusions. The device merely used one time, plants by capturing and taking vCJD proteins from blood by a “ extremely specific affinity absorptive stuff ” that is passed through the device under the force of gravitation ( MacoPharma, 2009 ) .
In decision, it is apparent that Variant Creutzfeldt-Jakob disease is rare yet fatal disease that leads to decease. Unlike other signifiers of CJD, it attacks much younger people, has a longer incubation period, and transmitted through ingestion of beef merchandises and blood transfusion. Taking steps against this fatal disease is emphasized and particularly testing of blood from givers to cut down efficaciously the hazard of transmittal.