Mary's Story

My name is Mary. I'm 38, married and live and work in Connecticut. My father died at the age of 68 at 10:55 pm on June 8, 1998. CJD was confirmed after his death by cerebral spinal fluid. My father lived and died in PA. He was lucky enough that the physicians who treated him had seen CJD before. I believe he was the 3rd or 4th case they've had!

My father's illness was brief. Looking back on it, I think he knew 6-8 months before he exhibited symptoms that something was going to happen. He made sure his estate planning was up to date and set up so that my mom could avoid probate. He bought a head stone for the cemetary plot, told my brother and I where the safe deposit keys and estate papers were, and he decided not to book the usual summer vacation.

His symptoms really started to show 5/1/98. My husband and I had gone to PA for my mom's birthday. That day, he complained that his right hand hurt and he had difficulty writing. He thought it was because he had been cutting up boxes all week long for the local community library book sale. That night at dinner, he had a difficult time expressing himself and stumbled over words. My father was always articulate and extremely well-educated. The following day, my mother pointed out that my dad seemed to be holding his arms slightly away from his torso when he walked, as if to balance himself better. His affect was also a little flat. That day my husband and I drove back to CT, with my mom promising to take him to the doctor's on Monday.

My mother ended up taking him to the ER that night. She later told me that when I called my folks to let them know we had returned to CT in one piece, my father had written his comments down on a notecard. He was afraid that he wouldn't be able to converse with me on the phone, and my mother was out of the house. That night he was admitted for series of tests and a provisional diagnosis of cerebellar stroke. He had had a carotid endarterectomy about 5 years ago, and the neurologists thought he might have thrown plaque from the side that wasn't done.

The tests showed nothing. He had the other carotid done May 6th, and his mental status was much worse following surgery. I came home May 7th to find him with significant expressive aphasia and right sided weakness. He was discharged home on Mother's Day. He started physical therapy May 12th and seemed to be doing a little better. I went back home to CT.

By May 20th he was back in the hospital. He was showing signs of dementia and myoclonus. He went thru all the same tests again, and the doctors concluded it was CJD when they couldn't confirm anything else. They all knew my dad professionally and personally and felt terribly guilty that they didn't diagnose it earlier. But it wouldn't have made a difference. And I think they were slow to diagnose it because they didn't want this to be the case for their peer and friend.

We brought my dad home on June 3rd with round the clock nurses. His whole family was with him. He was never alone. And he died in the spot where his favorite chair had always been, looking out into the back yard. For me, those 5 days at home where an eternity. My dad had a PICC line for drugs (we kept him sedated because of the seizures) and his private duty nurses were not trained to use it. I ended up being his nurse and pushing morphine, until I was able to get an oncologist to take over his care. We finally got him on a morphine drip.

Intellectually, I know I did the right thing in easing his journey home. However, emotionally I still have a hard time with it.

P.S. His doctors believe he got CJD from a blood transfusion he received during open heart surgery 2 1/2 years before his diagnosis. We (his family) have all been told that we can never donate blood, bone marrow or organs. I believe that most doctors who have treated CJD believe in the ban on blood donation.

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