PVCC Lynx: May 2005

I stood alone in the room at the funeral home, looking at my mother’s body in its body bag marked with biohazard stickers and the word “biohazard” in thick black magic marker. A towel covered her head so I couldn’t see how her brain had been harvested in New Mexico a day before. If anyone were to mishandle her body, they could die of the brain wasting disease that took her life; Creutzfeldt-Jakob disease, or CJD for short. It’s the human form of mad cow disease.

Creutzfeldt-Jakob disease is a prion disease that attacks the brain and there is currently no way of treating it. A prion is an infectious protein; it’s not a bacteria or a virus and, therefore, can’t be killed. Creutzfeldt-Jakob disease is considered a degenerative brain disease like Alzheimer’s or Parkinson’s. Prions literally eat the brain, leaving microscopic holes that look like Swiss cheese under a microscope. Some people with CJD die quickly like my mother. Other people can suffer for a year or longer. It is transmissible via the blood supply, medical instruments and the food chain. If one were to eat meat containing bovine spongiform encephalopathy, they could fall ill from a prion disease anytime during the disease’s incubation period of up to 40 years.

Creutzfeldt-Jakob disease killed my mother on Nov. 10, 2004. My father had only taken her to the emergency room on Oct. 8. In August, my mother acted depressed. She slept much of the time, often in the middle of the day. She kept asking me if clearly rotten food should be thrown away. She became very upset over little things, like a broken shower head that was easy to fix.

A Litany of Symptoms

September brought a litany of physical symptoms. My mother complained of an inner ear problem. She had vertigo and didn’t feel well. She became “unglued” at the end of September when I put a fan in the hallway to cool down the back of the house. The noise from the fan had upset her that much. She screamed at me to turn it off. She was angry and acted erratically. My dad walked behind her in the kitchen and she punched him, saying he had knocked her off balance.

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My mother was gone, but I didn’t even know it yet
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By the first week of October, my mother could hardly walk. She was shaking like a Parkinson’s patient. She reminded me of Muhammad Ali carrying the Olympic torch. She kept my dad awake at night because the shaking was violent. She didn’t want her parents to see her as she was.

I came home one day to find her in her office clutching a white plastic bag. She said she was nauseous and could hardly walk, so she carried it with her because she didn’t think she’d be able to walk to the bathroom. Her left leg looked stiff and she had no control of which direction it went when she walked. She told me I had to look at her handwriting because it didn’t look right.

Her handwriting looked like the writing of a child who was learning to write. It looked like she had tried to spell her name, Phyllis, but I couldn’t tell if she had spelled it with two 'l's." She didn’t seem to know when I asked her about it and looked very confused. My mother was gone, but I didn’t even know it yet.

She had tried weeks earlier to get help by visiting a neurologist, but her disease progressed too fast. When my father drove her to the emergency room, we were concerned that something might be causing permanent damage. I was worried we hadn’t acted in time.

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'I watched my mother go from a healthy, 56-year-old woman, to a zombie'
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Later, I would find out I was wrong and nothing anyone could have done would have helped my mother. At that time, I was scared. But I was not yet scared I would lose her. I honestly thought she had multiple sclerosis.

A Brain in Disintegration

She was tested for every ailment imaginable at Paradise Valley Hospital. There wasn’t one doctor who wasn’t mystified by her symptoms. She came up negative for everything she was tested for. She went through MRI’s, EEGs, PET scans, spinal taps, blood tests and urine tests. She was finally moved to Barrow Neurological Institute.

The doctor called my father and I in for a family conference. My mother tested positive for Valley Fever, which could have been latent. The doctor thought that was brought on by heavy steroids she’d been given at Paradise Valley Hospital. Valley fever, in rare cases, can affect the brain. Doctors performed a brain biopsy on my mother. The doctor told us my mother might have something called Creutzfeldt-Jakob disease, or CJD. The doctor also told us that CJD is the human form of mad cow disease and is fatal.

Since my mother’s brain biopsy, she had lost the ability to speak. At first, doctors and nurses said it was just the affects of anesthesia. But after a couple days, I knew something was wrong. My mother had aphasia even before the biopsy, which is when the patient can understand people, but can’t talk back, or vice versa. She was slurring her words and we couldn’t understand her. She was incontinent before she arrived at BNI, and was unable to swallow. She had a feeding tube inserted through her nose. She had no motor skills.

Cognitive abilities were rapidly going away, as were any physical abilities. I am not even sure when she stopped walking. I do remember the last time she sat up. It was Nov. 2, the day she was diagnosed with CJD. We moved her to a hospice two days later.

My mother hallucinated for a few days before becoming completely unresponsive. She told one of the BNI nurses she saw snakes on her legs and that was one of the last times she spoke. I don’t know what my mother saw as she hallucinated on her first night at hospice because she didn’t speak. She screamed and for just a second her eyes locked with mine in a look of terror I’ll never forget.

Before my mother lost the ability to speak, one of the last things she told my father was, “I don’t want to be retarded.” I watched my mother go from a healthy, 56-year-old woman, to a zombie in a matter of weeks. I knew I would do whatever I could to prevent this from happening to other people.

Untouchable

I was working on getting her an autopsy but no one in Arizona would perform one. Doctors at BNI said they didn’t want to risk contamination by doing the procedure, and that the National Prion Disease Pathology Surveillance Center hadn’t paid for previous procedures. Every time doctors operate on a CJD patient, the instruments must be destroyed. The operating room has to be washed down. I worked with the NPDPSC to arrange the autopsy as my mother died. By this time I had found out there was another relative on her paternal side who had died of CJD. This would mean my mother had the familial form of CJD.

It took several days of phone calls, but the NPDPSC called me and said they had finally found someone who would do an autopsy on my mother—in New Mexico. So after my mother died, that’s where her body was taken. I wanted to identify her body once it was back in Phoenix and before it was cremated. It was my final goodbye to her. My father didn’t want to come, which I understood. I stood alone in that room and looked at her body. The biohazard stickers surprised me; I didn’t expect to see them.

It was the first time I realized the magnitude of what killed my mother. She not only would have wanted to know what killed her, she would have wanted to understand it. So I fight every day to understand it myself. I read the research on the disease and fight every day for something, whether that’s writing a letter or helping someone else who is going through the pain of losing someone to CJD. I can’t bring my mother back. But I can and will do whatever I can to make sure this stops happening someday. I have a box of ashes in my living room that reminds me that I must.