Lessons of Life

Some folks lead a simple uncomplicated life never having to face unpredictable challenges. We are born completely innocent of the experiences this life can bring. More often than not our experiences are a product of our choices. These experiences color our thinking, our personalities, our mood, and even further choices that we make. I think my childhood was one of the most protective a child could have. I was the only child in my family for six years. My parents provided the age appropriate developmental experiences I needed but my world was certainly far from what one would call worldly. I never could have fathomed what the future held for me as an adult. My experience(s) with Creutzfeldt-Jakob Disease ranks right up at the top of the cruel. Of course, I know not what the future holds as none of us do. But when a loved one dies of this disease you never ever really get far beyond the experiences you shared. They may dull with time but they never really go away.

What is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year. In the early stages of disease, people 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.

What are the Symptoms of the Disease?

CJD is characterized by rapidly progressive dementia. Initially, individuals experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. People with the disease also may experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms. As the illness progresses, mental impairment becomes severe. Individuals often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these individuals and can lead to death.

Scientists are trying to learn what causes variations in the symptoms and course of the disease.

Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer’s disease or most other types of dementia.

What Causes Creutzfeldt-Jakob Disease?

Some researchers believe an unusual "slow virus" or another organism causes CJD. However, they have never been able to isolate a virus or other organism in people with the disease. Furthermore, the agent that causes CJD has several characteristics that are unusual for known organisms such as viruses and bacteria. It is difficult to kill, it does not appear to contain any genetic information in the form of nucleic acids (DNA or RNA), and it usually has a long incubation period before symptoms appear. In some cases, the incubation period may be as long as 50 years. The leading scientific theory at this time maintains that CJD and the other TSEs are caused by a type of protein called a prion.

My husband was a microbiologist by profession. His specialty for the last twenty-five years of his career was mycology. His attending doctors believe he probably contracted this disease through his work in some way -- most likely in what was air-borne. His variation of this disease presented with many of the classic symptoms. So extremely frustrating as I and the doctors tried to help him without success. So tough when nothing works. Eventually he did develop pneumonia and went into a coma. An otherwise healthy strong body taken down by a rare, rare disease.