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In 18th century Britain, the most important commercial product was wool, involving in one way or another nearly one fourth of the population, and as the century wore on, the industrial revolution and a growing dominance of mercantilism put fine wool at a premium. In this setting, a discussion took place in the British Parliament in 1755 about the economic effects of a fatal and spreading disease in sheep, and the need for government to do something about it. Thus begins the recorded history of scrapie.
Scholars of the disease describe its unpredictable waxing and waning in different countries...
Sporadic CJD occurs in a random distribution all over the world, and has no known genetic or environmental cause. It apparently arises from a one-in-a-million spontaneous conversion of a normal cellular protein (encoded by a gene on chromosome 20) into an...
I would like to share with you who my mom was as a person, our family history, and my experience with my mom’s battle with familial CJD, and her subsequent death. This is truly ‘Our Story’ for I know you will find similarities to your own experience. Remember that we are not alone but … IN THIS TOGETHER.
My mom was a vibrant, intelligent businesswoman, mother to nine children, and wife of 44 years. In her retirement, she loved working part time as a school crossing guard. She was strong willed, had a great deal of energy and, prior to the onset of symptoms, she was walking six miles a day. My mom was very caring and helpful to everyone in the community. Her faith and her family were her life.
My mom and dad were just beginning their retirement travels. I was enjoying mom not just as ‘mother to daughter’ but as ‘mother to mother’ as well as ‘friend to friend’. Then in one moment it all changed, and in a matter of months, it was all taken away.
Deana Simpson is a registered nurse and has had first hand experience with Familial Creutzfeldt-Jakob Disease (CJD). Deana's mother died of Familial CJD at the age of 64 and her family has lost 15 people spanning 5 generations. See 'Our Story' for more details surrounding her mother's illness and subsequent death.
Deana's purpose in establishing this web site is to provide support to other families afflicted with Familial CJD. Whether you have had a loved one die of Familial CJD or are currently watching a loved one die with this illness, Deana wants to provide you with the information and resources you may need in order to deal with the realities of this horrible disease. This disease can leave you feeling helpless and alone -- be assured that you are not alone.
Dr. Paul Brown is a graduate of Harvard College and the Johns Hopkins School of Medicine, and trained in internal medicine on the Osler Service of the Johns Hopkins Hospital. He is a Board Certified Internist, and for over 40 years worked in the Nobel Laureate Laboratory of Central Nervous System Studies at the National Institutes of Health. He retired from the NIH in 2004, but continues to serve as chairman of the International Scientific Advisory Committee for the French Laboratory of Biotechnology that monitors prion research, especially with respect to iatrogenic risk from blood and blood products. His role in this web site will be to provide accurate and current information regarding familial and other forms of TSE, including availability by e-mail (email@example.com) to anyone with questions about CJD.
Approximately 5-10% of all cases of TSE have now been found to result from one or another of more than two dozen different mutations in the gene that encodes the ‘prion’ protein. The translated portion of the gene has 253 codons, and mutations occur in many...