Friday, August 19, 2005
Creutzfeldt-Jakob Disease (CJD)---Human Form of Mad Cow Disease
So many would wonder why I am writing about this disease. Well my cousin Canon John Erb just passed away a few weeks ago from this disease. For a true diagnosis of this disease, it can only be made by an autopsy and thus we are waiting for those results which can take up to 8 weeks. So right now we can not say for sure if he had the human form of mad cow or not but what we do know is he had CJD.
To help you understand the disease more I have included some information for you to read and a link or two to read more about the disease and about my cousin.
Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. Patients are usually aged between 50 and 75 and typical clinical features include a rapidly progressive dementia associated, myoclonus and a characteristic electroencephalographic pattern. Neuropathological examination reveals cortical spongiform change, hence the term `spongiform encephalopathy'.
What is Creutzfeldt-Jakob disease (CJD) ? Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. Typically, onset of symptoms occurs at about age 60... There are three major categories of CJD: sporadic CJD, hereditary CJD, and acquired CJD. There is currently no single diagnostic test for CJD. The first concern is to rule out treatable forms of dementia such as encephalitis or chronic meningitis. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that is can be examined by a neurologist. Because a correct diagnosis of CJD does not help the patient, a brain biopsy is discouraged unless it is need to rule out a treatable disorder. While CJD can be transmitted to other people, the risk of this happening is extremely small.
Is there any treatment?There is no treatment that can cure or control CJD.
What is the prognosis?About 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
This is such an awful disease for anyone to get but especially for my Cousin John. John was the most vibrant man I have ever known. John had away about him that you always knew his presence when you where at a gathering that he was at. He had away about him that each and every time he met you he made you feel like you were the most important person on the face of this earth. And after he built you up he would move on to the next person.
In the history of the Erb family John known as The Erb is one of the youngest to pass away most of them live well into their 90's. He was also known as The Big Guy, and that place where The Big Guy touched us all has left a huge void in many peoples lives and hearts. We will all miss him dearly. I will miss his big hugs, laughter, smile and kind words. John has had a very special place in my life and heart. John was not only our cousin, he was our friend, and he was one of my mentors. John may not be here physically with us but he will live on in our hearts for ever
As you read the links I provided which talks about my cousin you can see how devastating this disease is and was on our family. For such a vibrant, lively, enthusiastic person to be robed of his body movement, to go blind, to loose his mind and then lastly to his death. The only thing about this devastating disease that we are all so grateful for is that it did not linger on in each stage that it took John quickly.
So John I miss you, I loved you and I plan on seeing you some day so till then....
To help you understand the disease more I have included some information for you to read and a link or two to read more about the disease and about my cousin.
Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. Patients are usually aged between 50 and 75 and typical clinical features include a rapidly progressive dementia associated, myoclonus and a characteristic electroencephalographic pattern. Neuropathological examination reveals cortical spongiform change, hence the term `spongiform encephalopathy'.
What is Creutzfeldt-Jakob disease (CJD) ? Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. Typically, onset of symptoms occurs at about age 60... There are three major categories of CJD: sporadic CJD, hereditary CJD, and acquired CJD. There is currently no single diagnostic test for CJD. The first concern is to rule out treatable forms of dementia such as encephalitis or chronic meningitis. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that is can be examined by a neurologist. Because a correct diagnosis of CJD does not help the patient, a brain biopsy is discouraged unless it is need to rule out a treatable disorder. While CJD can be transmitted to other people, the risk of this happening is extremely small.
Is there any treatment?There is no treatment that can cure or control CJD.
What is the prognosis?About 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
This is such an awful disease for anyone to get but especially for my Cousin John. John was the most vibrant man I have ever known. John had away about him that you always knew his presence when you where at a gathering that he was at. He had away about him that each and every time he met you he made you feel like you were the most important person on the face of this earth. And after he built you up he would move on to the next person.
In the history of the Erb family John known as The Erb is one of the youngest to pass away most of them live well into their 90's. He was also known as The Big Guy, and that place where The Big Guy touched us all has left a huge void in many peoples lives and hearts. We will all miss him dearly. I will miss his big hugs, laughter, smile and kind words. John has had a very special place in my life and heart. John was not only our cousin, he was our friend, and he was one of my mentors. John may not be here physically with us but he will live on in our hearts for ever
As you read the links I provided which talks about my cousin you can see how devastating this disease is and was on our family. For such a vibrant, lively, enthusiastic person to be robed of his body movement, to go blind, to loose his mind and then lastly to his death. The only thing about this devastating disease that we are all so grateful for is that it did not linger on in each stage that it took John quickly.
So John I miss you, I loved you and I plan on seeing you some day so till then....
