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The stillness of the morning was interrupted only by the sound of uneven, labored breathing, and Tom Baker climbed into his wife’s bed and cradled her in his arms, knowing these would be the final moments of their love story.
As she drifted away, memories came rushing to his mind — their 1995 wedding on the rocks at La Jolla Cove, long walks in their El Cajon neighborhood, a cruise through the Panama Canal, another in the French Polynesian Islands. And then the terrible diagnosis just days before: An obscure and fatal brain disorder, sporadic Creutzfeldt-Jakob Disease, so rare there’s literally a one-in-a-million chance of getting it.
Doctors have no way to test for it, usually aren’t able to diagnose it until days before death, and nobody knows how it’s contracted.
Like Carol Baker, most patients suffer for months with unanswered questions, uneducated doctors, fears that the condition is contagious or genetic and frightening symptoms that quickly progress — memory loss, dementia, an unsteady gait, hallucinations and sudden jerking movements.
Creutzfeldt-Jakob Disease, or CJD, has some high-profile relatives: The human form of Mad Cow Disease and Alzheimer’s. In all cases, normal proteins known as prions mutate into shapes that then attack cells in the cerebellum, the part of the brain that controls movement.
Despite strides in research, CJD is still an enigma.
CJD Researchers, who are greatly outnumbered by those for better-known diseases, are eager to quash fears by noting that CJD is not communicable. But they also point out that the disease does pose a public health problem because there is so little awareness, few research dollars and so little knowledge about how it is contracted.
“It’s under investigation and we don’t really understand the primary cause of the disease,” said Dr. Jiri Safar, internationally renowned neuroscientist and biochemist and co-director of the National Prion Disease Pathology Surveillance Center in Cleveland. “We know how it evolves and how it destroys the brain but we don’t know the triggering elements.”
Doctors in California are required to report all cases of CJD to the local public health department, not because it is communicable but because it is unusual and of unknown cause. The office looks at the victim’s age, gender, race, ethnicity and zip code to detect trends that could help identify causes. And, they interview the victim’s family to find out if genetics played a role.
In San Diego County, with a population of more than 3 million, there have been between two and five CJD deaths reported annually since 2000, said Dr. Michele Ginsberg, clinical director of community epidemiology for Public Health Services, in the Health and Human Services Agency for the County of San Diego.
It is so rare, and so misunderstood, that various doctors initially told Carol — whose first symptom was feeling off balance — that she had an inner ear problem, or that she just needed to relax, that stress was causing her symptoms.
When one of numerous doctors, neurologist Charles Smith of Scripps Clinic La Jolla, finally diagnosed CJD in June following three agonizing months of rapid deterioration, Tom actually felt relieved to know the reason for his wife’s suffering. But there were, and still are, too many questions remaining.
“I asked him, where did it come from? I want to know more,” Tom recalled recently. “He said there is no answer.”
So Carol Baker, born in Rochester, N.Y. on July 7, 1945, a San Diego County public health nurse to foster kids, with a life enriched by a happy marriage, children and grandchildren, many friends and a deep devotion to God, died July 9 of what was — and still is — a mystery illness. She was 64.
‘There Is So Little Known’
Carol’s family members and friends were confused by the diagnosis, said her youngest daughter, 35-year-old Rebecca Nordquist. The doctor had mentioned Mad Cow disease. Was it Mad Cow? Was it contagious? Was it inherited?
“Through this experience we found that there is so little known about the disease, and all of my friends and family had so many unanswered questions,” Nordquist said. “To me it was torture for three or four months not knowing what was happening.”
CJD has three forms: The kind Carol had, sporadic CJD; the kind that is genetic; and the kind people have come to know as Mad Cow, technically called “variant” CJD, which is contracted through consumption of tainted beef or through transplant of tainted organs. Dr. Smith assured Carol’s family members they were not in danger of contracting Carol’s disease.
Eighty-five percent of reported CJD cases are sporadic like Baker; 14 percent are genetic, and less than 1 percent are the Mad Cow form. There have been no known cases of the Mad Cow type in the United States since August 2005.
Diagnosis is usually accomplished through the process of eliminating other diseases such as cancer.
Carol had symptoms for three months before diagnosis, and she died 13 days after that. Researchers are trying to develop a test that would help with diagnosis long before symptoms show up, in hopes that early diagnosis could slow the progression or eventually lead to a cure.
‘I Feel Like I’m Going In All Different Directions’
In early April, Carol just felt awkward, unbalanced. On walks, which she did often with coworkers at lunchtime or with her husband after work, she would veer to one side, occasionally leaning into her exercise partner.
Doctors told Carol it was migraines or she was working too hard and needed to eliminate stress. Symptoms worsened rapidly, and Carol stopped working in mid-May.
By the time she went to Dr. Smith, who had just moved to San Diego from the Bronx in New York, she was desperate for answers, and desperate for someone to recognize something serious was happening. He assured the couple he would figure out the mystery.
At home, each day it was getting worse. “I feel like I’m going in all different directions,” she told Tom. They got a wheelchair. Tom carried her up the stairs for showers. She lost her short-term memory. Not long after that she was bedridden. Tests continued. Still no answers.
Her memory was spotty, which was hard on everyone, Carol’s daughter said. “There’s no pattern to it. She would forget something that would happen 30 minutes ago, but she would remember details from three years ago, four years ago, 30 years ago. It was just bizarre.”
Mystery Solved, Sort Of
Dr. Smith said he began to suspect CJD about a month after first seeing Carol, after ruling out drug abuse and cancer, but he didn’t tell her. It was the progressive problem with her gait and the way her eyes darted in all directions that tipped him. He quietly sent spinal fluid to the National Prion Surveillance Center for assessment.
The family knew only that Smith had ordered rare blood tests and the samples had to be sent to out-of-town labs. The weeks-long waiting was excruciating and symptoms worsened each day.
Smith called a meeting with the Bakers June 26 at Scripps Green Hospital.
The doctor had diagnosed just one other case in 30 years. Delivering the news to the Bakers was wrenching. “It’s like one of those things where you feel like you should have a shot first. It’s terrible.”
Looking back on that day, Tom said, as odd as it sounds, it was a relief to finally know what they were dealing with, and that someone finally recognized and validated what they were going through. The Bakers were grateful to Smith and embraced him.
The next day, Carol received close friends at her home and laughed and cried with them. Tom went to church Sunday the 28th and shared the diagnosis with the congregation.
“I got up in church and told the congregation, ‘It is under the heading of Mad Cow disease, and most people die within 7 months, and we are already at 3 months, and all of them pass away within a year. Pray for us.”
That night, Carol could not close her eyes. Could not stop talking. And at 3 a.m. Monday, she did not recognize her husband and started screaming. “I mean, screaming,” Tom recalled. Carol got on the phone with Nordquist, her daughter in Texas, and claimed Tom was attacking her. He kept his distance, worried that the daughter would think it was true.
It was a wrenching ordeal. Paramedics arrived and put Carol in restraints. Nordquist came home from Texas to help care for her mother. Her siblings, Laurie Nordquist Scribellito and Matthew Nordquist, shared grief and offered support, too.
Carol was in the hospital a few days, and by the following weekend, Tom said, his wife “had lost all contact with people, very rarely responding to anything or anyone.” The week after that, her legs started jumping, flinching uncontrollably.
In the end they could do nothing but give her morphine.
Those final weeks were agonizing for the family. The screaming incident had been almost too much to bear for Tom.
When she was all but gone, he was beside her hospital bed, provided by hospice, in their El Cajon condominium.
“I climbed up in bed with her. I saw that on a movie. There’s an old movie — you can see through the window of the medical ward, he just crawled up in bed with his wife. I decided that’s where I was going to be. I climbed up and held her, I didn’t let anyone else in.”
That’s when the memories flooded his mind.
“Her breathing was real slow. I knew it was over and I just held her as she passed away. I knew the Lord had come and taken her.”
(Correction: The original version of this story stated that Carol Baker received her diagnosis 10 days before she died in one part of the story. It later stated the correct number, which was 13 days. We regret the error.)
Please contact Kelly Thornton directly at firstname.lastname@example.org.