Creutzfeldt-Jakob Disease: Appears More Common Than Thought

  : CJD may have 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 and can therefore be spread by surgical equipment.  CJD is a horrendous infectious fatal brain-deteriorating disease for which there is no treatment or cure.  It is caused by a prion, which is a protein.  One strain nvCJD is linked to bovine spongiform encephalopathy (i.e. Mad Cow Disease) in England.  CJD is more common than reported.   In one study conducted by Yale University researchers 13% of clinically-diagnosed Alzheimer patients were found upon autopsy to have CJD.  (Manuelidis, Elias E. and Laura Manuelidis Alzheimer Disease and Associated Disorders_ 3 (1989): 100-109 Suggested Links between Different Types of Dementias: Creutzfeldt-Jakob Disease, Alzheimer Disease, and Retroviral CNS Infections.)  
  : 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 cadaver-derived growth hormone (GH) treatments, contaminated surgical equipment and dura mater and cornea transplants.)   Also, athletes who have used cadaver-derived Growth Hormones (GH) are at risk of developing CJD. CJD can take decades after exposure for the patient to show symptoms.  However, once a person shows symptoms their decline is rapid and they usually 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 as either an in-patient or an outpatient prior to CJD diagnosis.  In addition, people, including school-age children of Creutzfeldt-Jakob Disease victims who die in their 40s or younger, 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 the constant fear of developing CJD.  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.  
  : Whether CJD is spread by human blood is controversial,.  The infectious agent has been found in blood products but there are no documented cases of human transmission through blood products. However, since the incubation period can be decades and most cases are classified as sporadic (cause unknown) it is possible some of these cases are caused by blood products.   Until September, 1998, pooled blood products were withdrawn as a precaution if it was found after the product had been released that a person in the donor pool has died of CJD of was at risk of contracting the disease.  However, by this point it had often already been used in humans.  Many people such as hemophiliacs and Alpha 1-Antitrypsin Deficiency sufferers used blood products constantly to maintain their health and therefore, received many withdrawal notices.   Also, while the controversy remains as to whether CJD can be passed through blood products, blood products continue to be used as an ingredient in vaccines such as the measles-mumps-rubella, rabies and allergy shots; in InVitro Fertilization (IVF) cultures; and in medical test fluids.  In addition, many people get gammaglobulin shots.
  : The United Kingdom recently decided to no longer use its own citizens' blood for plasma products and to purchase blood plasma from other countries due to fears that nvCJD (the CJD related to Mad Cow Disease) may be transmitted through blood.  Yet people from the UK can donate and sell blood in the United States as can American servicemen and women who were stationed in the UK during the 1980s and 1990s.  At 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 support/discussion group. Most members have lost loved ones to the disease. The CJD Voice website has a great deal of information on the disease, including a CJD webring, a library of links, a chat room, bulletin board, and support group registration.  The CJD Voice  website is located at http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm
  : Blood Recall/Withdrawal - CJD is an e-mail discussion/support group for people who have received blood recall/withdrawal notifications due to possible CJD risk. People can register for the group at the Blood Recall/Withdrawal - CJD website which is located at http://members.aol.com/debbieoney/blood.htm  
  : The Many Faces of Creutzfeldt-Jakob Disease website is a collection of personal stories that put a face on CJD.  People tell how it affects their loved ones and how it affects them. They describe early symptoms and progression of the disease as well as the problems in getting it correctly diagnosed.  The Many Faces of Creutzfeldt-Jakob Disease website is at http://members.aol.com/stacy91434/cjd/cjd.htm
  :  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.
  : Deborah Oney, MSW, MHA
  : ***@****
======================================================================================
Was there also a question?....