Understanding Creutzfeldt-Jakob Disease (CJD) and Prions

Prion diseases are a group of rare and sadly fatal brain disorders, which can occur in people and animals. Many people first learned of prions in the 1980s in the form of Bovine Spongiform Encephalopathy (BSE) which is a prion disease in cattle.

In humans the best known of the prion diseases is Creutzfeldt-Jakob Disease (CJD), which reportedly affects around one person per million per year. In the United States this translates to 250-300 new cases per year. My father died of CJD in February 2008 after a 40 day stay in an ICU unit.

Some of the first symptoms of CJD include dementia, personality changes and blurred vision. In my father's case, his vision began to change several months before the other symptoms set in. Although each case is different, other possible symptoms include seizures, hallucinations, gait changes and memory loss. These symptoms are caused by death of the brain's nerve cells from abnormal proteins (prions) in the brain. CJD is very difficult to diagnose leading to speculation that the one case per million reported may be incorrect. In most cases, death occurs within one year with the median life expectancy of four months.

CJD is usually considered from a spinal tap test although most confirmations occur through autopsy. There is no known cure for CJD at this time. The disease is found most frequently in people 55-65 years of age.

CJD can mimics Alzheimer's or Dementia and misdiagnoses are common. The three types of CJD, are sporadic, hereditary and acquired. The majority of cases are Sporadic and account for 85 percent of reported cases. Around 10 percent of cases are hereditary, and less than 1 percent is acquired.

While CJD can have a devastating impact on your loved one's health, it can never take away the years of love and memories you've shared. I believe it's important to continue to visit with loved ones even if they are unable to respond. My mother played my father's favorite music, she read him get well cards and communicated with him until the day he died. She told him to fight until the doctors could figure out a diagnosis and he did just that.

We were given the CJD diagnosis on 2-13-08, and when my mother communicated the grim news to my father, he passed on that evening. While it is sad we were not able to help him heal, I do believe that he was aware of what was being said to him and could feel and process information. We were very careful to provide him with only love and kindness during the last stages of his life. A memorial service in his honor brought more than 700 people. While my father may have died from CJD, his memory and the memory of every CJD victim will live on too.

For more information visit
www.cjdfoundation.org