Hi, My name's Debbie. I'm new to the writers list and since I became interested in Creutzfeldt-Jakob Disease after finding out about Creutzfeldt-Jakob Disease that is mainly what I've been writing about and would like to suggest ways in which you might also like to. How about doing a story on Creutzfeldt-Jakob Disease? Maybe a true story on a person affected by the disease. There are many people directly affected by CJD, the horrendous infectious fatal brain-deteriorating disease: the victims, their families, people at high risk for the disease, and people who have received notices saying that the blood products, they, or worst yet, their children received have been withdrawn due to a member of the plasma pool dying from CJD or being at high risk of getting it. There is a great deal of debate going on among government officials as to whether recipients of withdrawn blood products should be notified. For the notification is the right of the person to know this information. Against notification is that the jury is still out as to whether CJD is even spread by blood and receiving such a notice is devastating. The risk of getting CJD from blood products is described as hypothetical and theoretical. The infectious agent has been found in blood yet there are no documented cases of CJD having been spread this way. However, with the incubation period often being decades and most cases being classified as sporadic, who is to say how CJD was contracted. Congress has mandated the Centers for Disease Control to determine if the CJD infectious agent is spread through blood (I have attached the mandate to the bottom of this e-mail). What makes the whole situation even more complicated is that recipients of these withdawal notifications are allowed to give blood themselves. It seems strange to many people that it is felt by some decision makers to be an important enough issue to tell someone of the withdrawal and thereby create havoc in their lives but not considered serious enough to stop them from donating blood. If they are really infected by their blood product receipt why have them donate blood and spread the infection to more people. I have included a speech given by one such person at the end of this e-mail. Some information on Creutzfeldt-Jakob Disease. Creutzfeldt-Jakob Disease (CJD) has greater public health consequences than the mere number of reported case might lead one to believe because it is not a reportable disease in most places, is often misdiagnosed, is infectious and is not killed by normal sterilization. CJD is a horrendious infectious fatal brain-deteriorating disease for which there is no treatment or cure. It is caused by a prion. One strain of CJD is linked to bovine spongiform encephalopathy (i.e. Mad Cow Disease) in England. In the United States and throughout the world people get CJD through 3 means: familial (genetic), sporadic (don't know how) and iatrogenic (through a medical procedure such as human pitutitary growth hormones, contaminated surgical equipment and dura mater and cornea transplants.) CJD can take decades after exposure for the patient to show symptoms. However, once a person shows symptoms their decline is rapid and they die within a year of first showing symptoms. CJD should be considered whenever a patient develops a rapid dementia and myoclonus. The initial symptoms are subtle and ambiguous and include insomnia, depression, confusion, personality and behavioral changes, strange physical sensations, and memory, coordination and visual problems. Rapidly progressive dementia and usually myoclonus (involuntary, irregular jerking movements) develop as CJD progresses. Also, language, sight, muscular weakness, and coordination problems worsen. The patient may appear startled and become rigid. In the final stage the patient loses all mental and physical functions. The patient may lapse into a coma and usually dies from an infection like pneumonia precipitated by the bedridden, unconscious state. The duration of CJD from the onset of symptoms to death is usually one year of less. A 14-3-3 spinal fluid test is over 95% effective in diagnosing CJD when symptoms are present. (For information on the test contact Dr Clarence Joseph Gibbs, Jr.; National Institutes of Health, (30l) 496-4821 or 6321.) CJD patients often die at home and therefore have home health service and have family members as caregivers. Since early signs of CJD are often psychological, victims will often receive psychological treatment. In addition, people, including school-age children of Creutzfeldt-Jakob Disease victims who die in their 40s, often require counseling to deal with the death. And, then there's the recipients of medical treatments such as human pituitary growth hormones and dura mater transplants who are at high risk of CJD and the people with a genetic mutation for familial CJD who must live with CJD hangiing over their heads. Another group includes people who receive withdrawal notices that the blood they or, worst yet, their children, received came from a pool which included a donor that died of CJD. While the question of whether CJD is passed through blood products is yet to be resolved, receiving this type of notice causes great anxiety in people. CJD is more common than reported. In one study of Alzheimer patients 13% when autopsied were found to really have CJD. Also, since normal sterilization methods do no kill the CJD infectious agent and , it can therefore be spread by surgical instruments, it is more of a danger to public health than mere number of cases would suggest.. Whether CJD is spread by human blood is controversial,. Pooled blood products are withdrawn as a precaution if it is found after the product has been released that a person in the donor pool has died of CJD of is at higher risk of contracting CJD. However, by this point it has often already been used in humans. While the controversy remains as to whether CJD can be passed through blood products, blood products continue to be used in vaccines such as rabies and allergy shots; in InVitro Fertilization (IVF) cultures; and in medical test fluids. Toward the end of this e-mail I have included the U. S. Congressional Mandate requesting the Centers for Disease Control to conduct a study to assess whether the CJD infectious agent is spread through blood products. CJD Voice is an e-mail discussion group. Most members have lost a loved one to CJD or currently have a loved one with CJD. Other members include people who took human pituitary growth hormones as children and therefore are at higher risk of getting CJD and people who received notices that the blood products their children received were from a pool that included a person who died from CJD. It provides support to these people as well as tries to increase funding for CJD research so treatments and a cure can be found. The CJD webpage has a message board, chat room, links to other websites with CJD information and a list of CJD researchers accepting financial contributions. The address for the CJD Voice Webpage is http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm Please feel free to visit the website and to refer other people to it. Feel free to distribute this information to whomever you choose. DebbieOney@aol.com The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob Disease Program A Congressional Mandate In response to concerns that CJD may be transmitted through blood or blood products, the U.S. Congress requested that the CDC conduct a study to assess whether CJD is a threat to the safety of the nation's blood supply. Researchers believe that the risk of transmission to humans through blood products is very small since there have been no known cases of CJD contracted by humans in this manner. However, precautions are being taken to assure that the blood supply is safe from this infectious agent. Current blood safety policy requires that any blood products made from blood donated by a person who later develops CJD, or is found to have risk factors for CJD, must be withdrawn. Until the question of transmissibility is resolved, the availability and the price of blood products will continue to be adversely impacted by shortages caused by recalls and the destruction of blood products which, to date, has cost over $100 million. The Program: How You Can Help Because the signs and symptoms of CJD may not develop for up to 30 years, a person could be infected and not show any symptoms during his or her lifetime. Furthermore, there is no screening test available for CJD and the only sure way to test for CJD is by analyzing brain tissue after death. The CJD program asks families of individuals who have received blood products to donate brain tissue after their death. The brain tissue will be shipped to Stephen J. Armond, M.D., Ph.D., Professor of Neuropathology, University of California, San Francisco to test for evidence of CJD. Other brain tissue will be stored at CDC in Atlanta for analysis in the future when more is known about the cause of CJD. Participation in the CJD program is voluntary. The CDC is aware that the death of a family member is a difficult time to make important decisions. Therefore, the CDC is working through your regional coordinator, physician, and treatment center staff to provide support to you and your family as you discuss brain tissue donation. To help with making an informed and rational decision, the CDC has developed information packets which answer commonly asked questions about the program including: how to authorize the donation; how confidentiality will be assured; the ability to retrieve the brain tissue without disfigurement and without affecting funeral arrangements; and the time frame for receiving test results. The following is a speech given at a Canadian Goverment Conference a few weeks ago by a mother of an 8 year old boy against notification of recipients of blood products withdrawn due to a member of the donor pool dying of Creutzfeldt-Jakob Disease. As she indicates in her speech she was initially pro-notification until finding out that a person getting notified of a withdrawn blood product can still give blood. "Good Afternoon Ladies & Gentlemen. First of all I am honoured at being invited to attend and speak at this conference. I will tell you about my sons situation and then about how I came to the decision of not Notifying people who have received tainted blood. My son received Albumin. in 1990. I was notified of the recall in 1996, It went as follows: to date there has been virtually no evidence that your child will develop CJD. Therefore, there is little reason for concern. But we also know that the more informed you are about this situation, the more assured you will feel. How can anyone get any reassurance from that letter...it didn't even state it was a theoretical problem and with stating that there is virtually no evidence does not state that there are no confirmed cases. I attended their conference they held in May 1996. I left that conference in dismay, overwhelmed by the information and gravely worried about my son. I was left on my own to deal with this anxiety, with no other support given, it was horrendous. It was a time of such limited information and CJD being the disease of many unknowns. In the two years that followed I did research at whatever means were available, the library, I wrote Health Canada and the Canadian Red Cross. Until I went on the Internet I found information to be limited. Any mailings were just of statistical information, and some even said to discuss with your family doctor....many of them don't even know anything about it and you have to educate them. My initial reaction was that I was glad I was told, I felt it was my right to now......now I don't think I should of been told. It causes me much pain and worry. Especially the unknowns of the disease. Upon asking questions as to whether my son would be allowed to donate blood the response was one I had not expected it was then that I changed my opinion and thought. Why bother informing us at all then? To cause myself as a mother much pain and worry for 2 years it will be something I will carry with me to my grave. You can't even imagine what I and others have lived thru, we are told of this horrific disease and our relation to it, and of the recall which seems important enough. But in the same breath I am told it is no big deal, why are you so worried and I am treated as such. It is my son we are talking about here. I was and am very frustrated with all of this. I assumed the main reason I was told was so that my son wouldn't be able to give donations of any sort blood or organs until we get answers, until I was told it was a step no-one was prepared to make. So if years down the road when we get some answers other people do not have to go thru the pain I have endured. With United States recently suggesting to use recalled Albumin in emergency situations when it is considered to be so risky it makes you wonder if the crisis is not so great that I had to of been told and others alike. Unless you research this on the Web and know who to ask you are left with only assumptions. When speaking with a representative from RED CROSS she said not to worry because by the time your son has become of age to donate blood we will have some answers. My response to her was what about the teenagers that got these letters and would be eligible to donate this year. are we reinfecting with infectious blood by doing nothing preventative with recipients of tainted blood. I was really emotional with her I really think she thought I was over-reacting to it all, and maybe I was but it was important enough to tell me so why shouldn't I react this way. With the decision to inform being based on ethical requirements of what to do and not whether the risk is real seems ironical that we are told of this one and not of other possibilities. It seems it is alright with some hospitals not to notify and with others to notify, those notified I feel have been given an injustice, you took away my piece of mind and sometimes I think my sanity. With researchers suggesting that if the incubation period for CJD is 30 years and over 1/2 of all donor pools would be contaminated would that now mean that in time at least 1/2 the population would need notification letters eventually and with all the products that contain blood and blood products would it now make it impossible to trace all the uses of such products, for example shots which contain Albumin. I do not rationalize now why we are told. I feel it would be more beneficial to keep a registry of the donor recipients to be used when the time of proof and answers are available. If the risk from acquiring CJD from blood is theoretical, not small or really small but theoretical why are we the only ones being informed of such a possibility. There are many other situations which are theoretical, such as the risk that cancer could be transmitted by blood transfusion or that allergies could be transmitted. Since the risk is only hypothesized and not demonstrated in these cases as well why are they not notified if you set a precedent with one then you must follow thru in all areas. Not just pick one. We do know that this disease has been around ever since transfusions were begun I believe in the past a connection would of already been made if it was possible for CJD to be transmitted this way when I think of the constant transfusions some people need to be able to live. I have spoken to a woman who has received some 30 recalls there are many of these people and they would of succumbed to CJD if it was possible. I do believe that recalls of tainted blood should continue but as we know the blood could of already been used these Recipients of this blood should be kept in a registry . I can not believe that anyone can be held liable for doing such a practice the unknowns of so much with any disease is cause for research to get the answers we all need, not dropping it in the laps of recipients and expecting us to continue on as normal I wonder how you expected us to react to such information. I am fortified in knowing that Health Canada and the CDC are presently doing studies on risk factors of blood. It is unfortunate that these studies were not completed before sending out notification letters. I have since spoken to other parents thru CJD VOICE, which is a support network for the people involved with CJD either thru losing someone to this horrendous disease or receiving notification, it also tries to raise awareness among the general public and increase funding. These parents who have been notified are terrified and frightened and want to know what their chances are of their child getting CJD really are and like one father asked how long do they have to live. They feel like they have no where to turn for answers, it is all right to logically expect us to understand that the risk is minimal if at all. But emotions usually rule out logic. I am fortunate on one aspect because my child is not inflicted with any other medical problems I can only imagine what they are going thru. In other countries they are not telling the recipients....such as in the Netherlands they did not want to alarm the recipients which I feel is a good thing as once you are told it is hard to listen to the logic without concrete proof. This is our loved ones, our children, someone we are supposed to protect, but in life as we know it we cannot protect them from everything. Other thoughts I have gone thru are how do I tell my son about this, if something was to happen to me, would he really need to know just because someone felt we needed to know. I have also felt do I need to stop him from donating blood or organs until research proves to me that it is alright for him to do so. It was important enough for me to be notified but not enough for him to be considered a risk factor. As long as I am alive he will NOT be told it would be too much for him as I am sure it is too much for alot of people. At some point I have also believed the only reason we were told was because of the legal aspect when I rationalize the history it is the only reason I can come up with. Noone can be held responsible for the unknowns of any disease, it would be a greater benefit to have more knowledge, not have hundreds of people feel like they are lepars in society. I am also worried that if others do find out about my son's situation in our community he would become an outcast. We have seen that in the past with other diseases. There is much confusion when you receive this letter of notification and when you are done researching all that is available you become frustrated, angry and many tears are shed. I believe some of the examples I have stated illustrate the reasons for such emotions I have personally thought of it as a pendulum swinging and each side has it's own emotions, and each side its own rights and wrongs. Never-the less I am still left with my sons notification letter nothing will change what has already been carried out but what I am doing is hoping for a turnaround in not telling more people and having them deal with such an unjust and inhumane thing such as notifications for theoretical risk. I would also add where would some of us be without these products today. THANK-YOU"