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Nowhere to Turn

Published on 18 May 2015

By Sheila Haar Siegel

What do you do when you have no idea why your spouse is acting so strangely? Doug Joseph ’ 85/ ’ 90 knew something wasn’t quite right with his wife, Kris, yet no one could give him any answers—until one Sunday morning when she couldn’t get up to walk.

Doug Joseph's memory of Monday, April 28, 2008, is amazingly clear. That night, his wife, Kris, asked him to watch her walk. She asked if anything seemed strange about her gait. Doug noticed her right arm—it wasn’t swinging like the left one. Later, when he was getting their four young daughters ready for bed, Doug heard a crash from the kitchen. Kris began to scream, “Something is wrong with me!” She had shattered a ceramic mug on the floor and was crying uncontrollably. Back upstairs, they both could see a muscle twitching on the top of her right hand. A few hours later, she started to complain of numbness in her arm. Doug called an ambulance to their home at 2 a.m., fearing the possibility of a stroke. The paramedics refused to take her to the hospital because the numbness had subsided by the time they arrived.

The next day, she was sent to a neurologist who ordered a nerve conduction study and, what would turn out to be the first of many, MRIs of her brain. Both came back negative. A few days later, Kris couldn’t touch her nose with her right index finger—completely missing the target. Doug drove his wife to the emergency room himself this time, only to be sent home a few hours later. A doctor told her that there were no signs of a stroke and he could find nothing wrong. He said the hospital was full and there were no beds available to evaluate her further. He suggested she follow up with her primary care physician for neurological testing. So they went back home. It never even entered Doug’s mind that he and the girls would see Kris die in less than 90 days.

The changes in Kris had started two and a half years earlier, but this was the first physical sign they had both noticed. Before that night in April, there had been only subtle changes in her personality and behavior.

At first, shortly after their fourth daughter was born, Doug ’85/’90 began to pick up on some strange behaviors. Kris started having severe anxiety attacks, which, at times, made her intensely irritable—so different than the spirited, funny, and compassionate person that Doug and others had always been easily drawn to. She had been a critical care nurse for organ transplant and cardio-thoracic surgery patients for 15 years before starting a family. Even after her days as a nurse were well behind her, she kept a well-stocked first-aid kit in the family’s van, in case she came across an accident and needed to pull over to help. She had always wanted to stay home with their children, and was thrilled when it actually happened after the birth of their first child. She loved being a mom. Still, Doug knew Kris wasn’t happy in Illinois.

During their nine-year marriage, Doug and Kris traveled to the Caribbean and Mexico, willing the days to last just a little longer in the warm humidity of the islands. They dreamed of packing it in and leaving the cold winters in Joliet, Ill., for a warmer climate. Before they were married, Kris had even lived in Hawaii for a while, and the couple often talked about moving there. So in August 2005, Doug, a senior manager in the pharma- coepidemiology department at Takeda Global Research and Development at the time, put their house up for sale in Illinois and flew to Honolulu to interview for a management position there. The company wanted him to move to the island as soon as possible, so he suggested the family (which now included all four girls) stay in Illinois until the house sold and he got settled in. Suddenly, Kris was dead set against the idea and said, “If we can’t go with you, then nobody is going.” Doug turned down the job offer, yet Kris kept telling all of their family, friends, and neighbors that that they were moving to Hawaii. She even started giving away some of their belongings—patio furniture, a stereo system, clothes, and other valuable things they had around the house. Doug thought it was odd—and a little embarrassing—but he didn’t know what to make of it at the time.

Six months later, the family of six moved to Tucson. Doug wasn’t sure if they should go through with the move because, by then, arguments between he and Kris were becoming common and their relationship was strained. And Kris was starting to struggle with depression. But she was convinced that moving to Tucson, where she and Doug had gotten engaged, was the place for them to be. Doug stuck with their decision, thinking the change might do Kris some good. At least, he hoped so. He wanted to help bring back the person he married and was so desperately seeking again. Yet, gradually, things only got worse. The “d-word” was mentioned several times by Kris who, as a devout Catholic, had been fervently opposed to divorce up until then. Kris was becoming full of anger, turning into someone he no longer knew. Their marriage was becoming more and more fragile. Kris blamed Doug for her unhappiness and, soon, he started to blame himself, too. “I thought, maybe it’s me,” says Doug, “because she turned from such a loving, gentle, and kind person to somebody who hated my guts.” They even saw a therapist for a while. Turns out, the problem wasn’t marital. Doug would eventually discover the shocking diagnosis: Kris had Creutzfeldt-Jakob disease.

Sometimes misunderstood and very often misdiagnosed, Creutzfeldt-Jakob disease (CJD) is most commonly known as “mad cow disease.” But there are three different kinds of the disease: sporadic, acquired, and familial, which is the type Kris had. It is an elusive and always fatal brain disorder that causes memory loss, psychosis, impaired coordination, visual disturbances, mental deterioration, and Parkinson’s disease-like involuntary movements, inflexibility of limbs, and gait disturbances. Its victims eventually succumb to blindness, coma, and death, when the disease is often finally diagnosed by autopsy. CJD is extremely rare. Only one case per million is reported each year. Kris was that one in a million.

By the time the first physical symptoms appeared, Doug knew something was seriously wrong. And at that point, Kris knew it too. She had said, several times, that she thought she was going to die soon. One night, while lying in bed together, she asked Doug if he was happy that she was going to die. Doug said, “Of course not, and you’re not going to die! Why would you think I’d be happy?”  She seemed oblivious to his question, instead saying “If I do die, I want my friend in Illinois to raise the girls.” While Doug was still trying to make sense of what his wife had just said to him, she jumped in again, apologizing for all the hurtful things she had ever said, asking him to forgive her. Her thought processes were already becoming jumbled.

“When she made that comment about the kids,” says Doug, “I knew there was something wrong. I had known for a little bit prior to that, but now, I knew she was really sick.” Something was tearing them apart. And neither she nor Doug had any idea what they were going to have to go through to find out what it was.

In 2007, a few months after moving into their new home in Oro Valley, Ariz., Kris gave in to nagging tingling and numbness in her right hand, which she then attributed to carpal tunnel syndrome, and scheduled surgery. Everything went smoothly. But soon her unusual personality changes escalated dramatically, sometimes into explosive, violent outbursts. Doug remembers one morning before work when Kris shoved him into a corner and started hitting him with her fists. She threatened to kill him if he called the police. He doesn’t remember why she was mad at him. Another time, in a fit of rage, Kris wrapped a belt around his neck, attempting to choke him. She accused Doug of having an affair, which was only fodder for the divorce argument. “It is a disease that causes paranoia and psychosis,” Doug says. “I never had an affair with anybody.” And all the while, Kris was feeding false information to her parents, telling them that Doug was the reason for her increasingly bizarre behavior. They thought Kris was suffering from anxiety or stress and that Doug was the real problem. In fact, Kris’s family accused him of poisoning her during her last days in the hospital. “It was hard for people to really comprehend what was going on. That’s what happens when you don’t have any answers. People start grabbing at straws to try to make sense of the situation.”

After visiting nearly every neurologist and emergency room in Tucson and coming up empty for answers, Kris kept getting worse every week, day, and minute of her life. By June 2008, things had gotten so bad, so quickly, that her speech began to slur and she felt numb on the right side of her face. She had terrible muscle spasms that would force her arm behind her head. And she was having trouble walking. But no one could figure out why. She was admitted to hospitals for full workups of MRIs, CAT scans, and EEGs—all showed nothing. Doug and Kris didn’t know what was going on either—and they were health care professionals. Because the symptoms don’t manifest until the end stages of CJD, Kris was continually given a diagnosis of “psychiatric illness” or “conversion disorder.” No one seemed to be able to explain her disturbing behavior or had a clue as to what was really happening inside her central nervous system. Yet there was one key piece of information that modern medicine kept overlooking. Kris’s 83-year-old uncle had recently died of CJD, and familial CJD is passed from generation to generation. At one point, while admitted to the University of Arizona Medical Center, Kris flat out asked one of the neurologists if she might have CJD since her uncle died of the disease just nine months earlier. He confidently—and somewhat arrogantly—replied, “No, that’s not what you have.” They were being written off by the medical establishment, and their frustration was mounting.

Kris spiraled downward so rapidly that she couldn’t bathe, dress, or wipe herself after a bowel movement. Doug did all of that for her. He was working full time as a clinical pharmacist at Tucson Heart and Cardiovascular Institute, tending to Kris’s needs, and taking care of his little girls, ages 3, 5, 7, and 8. On Sunday, June 29, Kris could no longer get up to walk. She could barely talk. Doug took her to the only hospital left in Tucson they hadn’t yet tried because he just couldn’t care for her by himself anymore. The next day, he was told once again that Kris was going to be discharged—this time to a psychiatric facility. He put his foot down and demanded one more evaluation by another neurologist. Finally, on July 1, the last of Kris’s MRIs came back positive for some kind of brain abnormality. She had had three negative MRIs in the past month. Now Doug was finally learning that there was something neurologically wrong with his wife. Three weeks before Kris died, a neurologist told Doug, “Some neurological diseases can progress very fast and not show up until later in the final disease process. It’s definitely not psychiatric. It could be brain cancer or it could be Creutzfeldt-Jakob disease.”

Doug knew exactly what that meant. There’s no cure for CJD. It meant his four little girls were going to have to spend the rest of their lives without their mom. He was helpless. He and Kris both knew she didn’t have much time left, and they cried about it together in her hospital room. Doug spent their few remaining weeks together at Kris’s bedside, trying to make her as comfortable as possible.

On July 16, a biopsy of Kris’s brain tissue came back positive for CJD. By July 18, she was in a coma. The last time the girls had seen their mom, she was able to move and still talk a little. Now she was unable to move or speak, though she still knew what was going on around her. Talia, the youngest at 3, climbed up on the bed to snuggle with her mom for the last time. A tear rolled down Kris’s cheek. She passed away a few days later, on July 25, 2008, at 9:21 a.m. She was 43.

After Kris died, Doug started writing a letter to his daughters. He wanted them to know what their mom was really like before her illness. He kept writing and writing until he realized that he could use the information to educate other people, especially medical professionals, about CJD. The result is his book, Kris’s Story: Battling Creutzfeldt-Jakob Disease, published in 2010. In July, he will attend a meeting of the Creutzfeldt-Jakob Disease Foundation in Washington, D.C., for researchers around the world who are presenting the latest information on the disease. He now works to educate others. He works to find a cure for his daughters.

Because Kris’s disease was genetic, there’s a 50 percent chance that each of his daughters may carry the same genetic mutation that caused the disease in their mom. And a carrier has a 90 percent chance of developing the disease at some point in their lifetime. Though they may not yet fully understand the ramifications, Doug simply tells the girls the truth. “They know their mom died of a disease that was genetically transmitted and there’s a possibility they can die from it, too. But it’s not something we worry about or think about a lot. We just take one day at a time.”

Right now, his job is just to be here.

This story was originally featured in the spring 2011 issue of Script Magazine at http://dev.stlcop.edu/news/publications/script.html.

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