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. 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 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.

Visit the CJD Voice Web page.