Creutzfeldt Jakob Disease
Creutzfeldt-Jakob Disease- Recognition of iCJD
Creutzfeldt-Jakob disease (CJD) is a disease that falls into the category of untreatable and ultimately fatal prion diseases, known as transmissible spongiform encephalopathies (TSE). There is a primary cause for concern for contracting vCJD, in which the disease is spread through tainted meat directed for human consumption. When this meat is ingested, the human has unknowingly contracted the disease and has the ability to spread it on to others by the sharing of blood or tissues.
The first issue with CDJ arose in 1996, when vCJD was acknowledged as a transmittable disease amongst humans. Experiments with sheep have shown that this particular disease has the ability to spread while it is still in the incubation period; it then goes on to infect others who weren't exposed to the original contaminant. The first actual case of iatrogenic CJD (iCJD) was confirmed in December of 2003, and since then, up to 250 more unconfirmed cases have come to light.
The incubation period for vCJD can range from 1.5-18 years, giving individuals the false reassurance of being healthy. During these years, it is possible to donate blood, undergo surgery on major organs or bone tissues, or even become an organ or tissue donor in the event of a non-recoverable injury to the brain. Undoubtedly, it will be several more years before we can even identify the total number affected by the primary epidemic; we cannot tell the total number infected.
Diseases that are easily transmittable through blood and blood products are often carefully regulated by government appointed agencies. Guidelines are then put in place to make sure that the tissues used for donation to others are not contaminated with this new disease. The United States currently follows the recommendations and regulations of The Food and Drug Administration for all blood and tissue donations. At this time, there is no definitive non-invasive diagnostic testing that can be done on blood or urine samples to prove the samples to be CDJ free, so we must rely on extensive questioning. There are currently eight main questions to determine the likelihood of CDJ infection, mostly focusing on time and travel in Europe, and there are also three additional questions inquiring about individual and family health. The questionnaire went on to more in depth inquiries toward whether you've had blood transfusions in the UK or if you've had spinal or brain surgery. Most recently, all persons that have had grafts from the dura matter of the brain or cornea were refused from donation.
Because of the world's previous experience with a similarly spread disease, HIV/AIDS, we knew the possible devastating effects this could bring if this disease was not carefully contained and proper preventative measures taken. Before diagnostic tests were created for the screening for HIV/AIDS, questioning was the only preventative measure available. The urgency quickly increased for diagnostic testing, and soon an antibody testing protocol was created for not only HIV/AIDS, but also other transmissible diseases.
Currently there is no way to definitively diagnose a person with vCJD or iCJD without doing an autopsy on adenoid and brain tissues after death. However, there is extensive research currently in progress for simple diagnostic testing of blood and urine.
For now, some theories in the research field are focusing on the leukoreduction of all blood donated. The white blood cells offer no benefit to those receiving the transfusion and the probability of the b-leukocytes, which originate in the bone marrow, to carry the disease is high. So by eliminating the white blood cells all together, we are able to reduce but not entirely eliminate the possibility of transmission.
Another prospect of transmission is through the plasma in the blood. Some research is pending on the possibility of removing certain proteins by fractionation of the blood product. By having these more suspect carrier-proteins removed, there is a reduced chance of passing on the disease.
The main focus in the research field involves the development of a direct prion filter. This filter would ideally be used after leukoreduction to remove all harmful prions from the blood product. This process would prove most effective overall; but the ability to completely remove all prions without causing removal of other important blood components is difficult.
Since December 11, 1996, measures have been taken to insure that all existing blood and plasma products associated with donors who have contracted CDJ or at risk for CDJ are removed from circulation, quarantined, and then destroyed. This ensures that all possible products deemed suspect of contamination are not allowed to be administered to others.
The age group most affected by the variant form of this disease is in relatively young individuals aged 10-30. The reasons are not yet known for why this disease is far more common in the younger individuals, but there are several possible explanations including the increased intake of hamburgers and beef products versus older individuals.
For children born after 1995, the chances of having been exposed to this disease through infected meat are rare if any. To keep these children free of CDJ, the United Kingdom and several other European countries began to import fresh frozen plasma from the United States as early as 1998. Although the US has had one confirmed case of vCJD; the United States plasma is still considered extremely safe versus the plasma currently available in the UK.
Preventative measures are important in controlling further outbreaks of infection, but concern has recently to those that have been already iatrogenicly infected. Since 2004, there has been an active effort on the part of governments, blood distribution agencies, and medical practitioners to track down persons deemed “at risk” for contraction of CDJ. Individuals or guardians of children that have received blood products or plasma knowingly contributed by a positive donor, are then often notified via mail. In these letters they are explained the degree of possible infection they might have encountered and are given a phone number to contact for further assistance. They are then banned from further blood or tissue donation, and in the event of a major surgery, they must inform the surgeon of their possible condition. By informing the surgeon, the post-operative measures for sterilization of the instruments and materials are heightened, or in some cases they may even be discarded altogether.
About 6,000 notification letters have been sent out in the UK alone. Most of the people receiving these letters are hemophiliacs that have had transfusions in the UK from the years 1980-2001. Other letters were also sent out to the “at risk” person's primary physician to guarantee the strictest measures are taken when handling any medical procedures.
This is not the only occurrence where the government has needed to individually notify the public of possible infection through diseased blood. In the past decades, hemophiliacs have also received similar letters in regard to blood products tainted with possible HIV/AIDS and Hepatitis C. After receiving these letters, individuals were often traumatized and in need of professional counseling due to the seriousness of the error. In the case of CDJ, persons who have received infected blood have no way of confirming the fatal disease and must learn to live with the possibility of unforeseen early death. Several persons that were diagnosed with iCDJ, had elected to bring lawsuits against responsible parties. However, by the time the lawsuits were debated in court, some of the infected had passed on.
As the years go by with still no clear indication of who exactly is infected, we must consider what could be done in the future to prevent this from happening again. The policy holders must think of new ideas and systems to be put in place immediately when another possible disease arises that can be transmitted in a similar manner. Time after time we have seen different countries take dissimilar courses of action and with everyone choosing their own ways of containing the outbreak.
Yes, we need simple blood or urine tests to definitively detect CJD in people around the world, but we also need standard practices and policies around the globe to prevent the contamination to others until the tests are ultimately developed.
Since world travel is more common than ever, we should start thinking globally rather within our own countries. The first step to world coordination would be to make sure all blood regulation organizations from different countries (ie. The Red Cross, The National Blood Service) all came together to address the risks associated with the disease and format an official questionnaire to be used worldwide. This would allow all countries the equal opportunity to help protect the world's population.
The previous epidemics of disease led some countries to be cautious when informing the public of possible infection through blood products. The world is in need of a strict policy, instated by governments, to individually inform those who have received a product potentially hazardous to their health. Legal and moral policies have a slight conflict in this area when the disease has not yet been proven to infect others through this manner. This may cause unnecessary worry and concern if proved to not be transmissible. However, the prospect for infection has not yet been proven to be impossible. So we must still take action to help those “at risk” and protect others from even further transmission by notification as soon as possible.
In the next few years, we can hope for significant advancements in technology to determine who in fact is positive for CJD. At that time we will be able to assess our past strategy for possible blood transmitted disease by calculating the number of people iatrogenicly infected before and then after policies were put in place. If the number significantly dropped after policies were instated, we have proven that our system is effective and can make small adjustments as needed. However, if the number of infected remained the same or increased after current policies were instated, we would then need to impose even more invasive measures and notification policies as stated previously in this report.
References:
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