Dean Cree at work in October 1998 (family photo)

“After April 1999, when Dean could no longer work, I put breakfast at his bedside before leaving for work. I would come home at lunch and get him out of bed and help with the teeth-brushing and have him shower, if he would agree to that. I made him lunch and returned to work. I did this all in a 50-minute period. He would go right back to bed. After work I came right home and got dinner going and got him back out of bed. After dinner he went back to bed. For a year and a half that is what he did – stayed in bed.” Sheila constantly juggled her schedule and budget to provide in-home caregivers to keep Dean safe and occupied.

“Saturday mornings were always a nightmare, as that was the only day he permitted me to groom him – shave, cut nails, etcetera,” she continues. “He did let me brush his teeth every morning, though. He would only shower every other day – no exceptions. If I needed to do something out of the ordinary, I would have to give him options to gain his cooperation.” Her freedom gone and tensions building, Sheila’s life was changed forever.

“Many people told me, ‘Sheila, get an annulment and run like hell!’ I said, ‘For you to say that, you don’t know me.’ I’m a compassionate person by nature. I just felt that it was something I needed to do. I feel that we were put together for me to take care of him. I married him and he needs me. I love him.”

Dean's faith keeps him going

Like all stories about Huntington’s disease, Sheila and Dean’s is about facing risk, falling ill, providing care, and confronting the horrible repercussions for the family. It is also a story about religious faith: both Dean and Sheila are devout Catholics. Faith is often the only hope for people who are waiting for a cure or treatment. But Sheila and Dean’s story is unusual for several reasons. Remarkably, Sheila decided to marry Dean even after learning fully about his at-risk status. His mother, now deceased, had the disease, and his sister was already ill. His grandfather also fell victim to HD. “When his grandfather had HD, they didn’t know what it was,” Sheila says. “They just thought he went crazy. And they institutionalized him.” Dean had not been tested, but two weeks before the wedding Sheila thought she saw some small symptoms. “I thought we’d have five good years together,” she says. But then HD struck. While many patients decline gradually over years, Dean’s disease progressed into full-blown symptoms in just months. And yet, although losing many of his cognitive abilities, Dean still displays the inner self that drew him and Sheila together.

It is a warm Sunday afternoon in February at Edgemoor Hospital, a public nursing home in Santee. The wards are old but clean, and the semi-rural setting would conjure up cheerful feelings if it were not for the sad lives contained within the cell-like rooms. A visit to Edgemoor stirs powerful emotions – fear, relief at having one’s health, and a deeply uneasy sensation about the fragility of life. Patients with various conditions reside at Edgemoor, but it is the Huntington’s people that lead one to imagine utter hopelessness. One HD person thinks she is the hospital supervisor. Another writhes uncontrollably in a special recliner secured with a seat-belt type strap to keep her from falling out. One rolls about her bed like a toddler trying to find a comfortable position to sleep. Yet another, who sometimes wears just a diaper and a t-shirt, lies on a large mattress on the floor because she falls out of bed. These are standard, professional ways for dealing with the constant bodily movements and dementia of HD. It is no wonder that so many people at risk for Huntington’s prefer to live in complete denial, hoping that they are negative for the genetic defect that they have a 50-50 chance of inheriting from an affected parent or that their positive test result will not change their life for years. Sadly, such denial helps keep attitudes about HD in a kind of shameful Dark Ages and frustrates HD activists trying to build a sense of community and project their voice to the media and the government.

Edgemoor has been Dean’s home since May 2002. Private nursing facilities refused to take Dean because they didn’t want to deal with the difficult behavioral symptoms in the later stages of HD – a barrier faced by many families. His passage from his own understandable denial to helplessness was so devastating that Sheila could no longer be his full-time caregiver. It was a wrenching decision. “I know I promised you I’d never do this,” she told Dean. “It’s affecting me. I’m not doing you any good by being upset. I can’t provide the care you need. You can’t be home alone. Too many things can happen.” After discussing it one night in a Huntington’s disease support group meeting, Dean accepted Sheila’s suggestion to go to Edgemoor. Sheila felt enormously guilty. “I felt as if I were abandoning my duty,” she says. “I felt as if I were copping out on the whole thing. I had a harder time than he did. I cried for a week. I felt guilty. I felt ashamed.”

Sheila had to get a court judgment against the MediCal system, which had tried to force her to forfeit Dean’s entire $1,400 Social Security monthly disability payment to help cover the cost of Edgemoor. “I said, ‘You want me to live on the streets so that I can take care of my husband,’” Sheila recalls. She still has to pay the mortgage on their home, in addition to adjusting to Dean’s lost income, making up for lost hours devoted to caregiving, and working off the $50,000 in debt the couple sunk into for expenses related to the disease. She currently works six days a week at four different jobs.

What he most needs: a hug

On this day Dean is quickly moved to tears, which begin to flow when he receives what he most wants from Sheila: a hug. Because of the accompanying depression and mood swings, many HD patients cry easily. Dean takes an anti-depressant, an anti-psychotic, and a mood-control pill. These medications affect only the symptoms, and not the cause, of HD.

The walls of Dean’s room jump out with pictures of his family and signs of his faith, including a Catholic calendar Sheila has just hung. She has made the room as cheery as possible. She even bought Dean a pair of slippers stamped with the insignia of UCLA, where Dean took his degree in music theory and composition.

Dean on his daily walk at Edgemoor (family photo)

Dean knows he is the subject of an article, and he strives to express himself clearly. “This is a very debilitating disease,” he whispers. “It takes an awful lot of my faith to get by. But I’ve pretty well accepted that this is God’s plan for me. I basically do that every day.” Dean believes that perhaps God is trying to show something through disease. “Maybe that He is in control. Sometimes, no matter what you do, it’s going to happen differently,” he says. When he can, Dean prays. “I carry a rosary with me,” he says. He also regularly attends a Sunday afternoon Bible study held at Edgemoor, and on Wednesdays he receives Holy Communion. For those who are at risk for HD, Dean has important advice: “Physically and spiritually you want to be as healthy as possible. The doctor told me that because I was healthy I’ve been able to get by longer than if I had just let myself go.”

Dean’s main challenge is just coping with the basics of life. He remembers how in his first days at Edgemoor he would wake up at night scared and uncomfortable. By choice he bathes only three times a week. He must make his way down the hall with his walker to the shower room, where a helper assists in removing his clothing. Dean sits in a shower chair, and the helper remains to make sure he does not fall. He brushes his teeth and shaves before ambling back to his room. Dean takes his meals in the day room just off his corridor. There he sees some of the other twenty HD patients at Edgemoor. Meals are a noisy affair, with the patients yelling out their needs to the staff. Once a week Dean receives physical therapy, and every day he uses his walker for a slow, mile-long stroll around the hospital grounds. Sometimes he visits a small park next to the hospital.

Keeping connected to music

Dean’s pleasures are simple. The serious condition of the other HD patients makes communication almost impossible, but Dean has made friends with a neighbor disabled by polio. Sheila might bring their dog Jackie, a boxer mix she is having trained to be certified as a therapeutic dog to boost the morale of the patients. “The animal shelter every so often brings a dog or a cat or a rabbit,” Dean says. “The patients can pay attention to it and not be harmed.” On some days Dean decorates ceramics; in his room he has a dolphin, an angel, and a Madonna. “I’m still able to keep my arm steady enough to paint,” he says. On other occasions someone will read the newspaper aloud to the patients. “I can’t concentrate enough to read,” Dean explains. “I can see, but I can’t put two or three lines together. I get lost. I can hear better than I can read. I’ve got some books on tape that I can check out.” Weekends are good because of the many sports events on television. “I watch TV a lot, and I’ve got some video tapes,” he says. Sunday’s moments with Sheila are precious. “I try to catch up with what’s going on with my family,” Dean says. His 21-year-old son David, who came from a previous marriage, is also a frequent visitor. “We usually go out and have something to eat. We take a walk around Santee Lakes, which is a regional park.” While many HD people nap during the day, Dean is able to stay awake. He usually retires at 8 p.m. and sleeps until 6:30 a.m.

Losing the ability to play the trumpet deeply distressed Dean. “When he realized he could no longer play the trumpet – it was at mass one day when he was having a hard time – he came home and said he wasn’t going to play anymore,” Sheila says. “He stopped playing around six months after the diagnosis. He sold all of his trumpets. He didn’t want to keep one for himself.”

But Dean still remains connected to music. He especially likes jazz. And he has the memories of his many accomplishments, such as the day in 1995 at a Carl’s Junior restaurant, where, while contemplating at a holy card of Jesus embracing a child, he wrote the lyrics to a song on a napkin. It took him just ten minutes. At home he added the music. “I had a feeling from God that there was a song there,” Dean recalls. “There was a melody that just came out of the sky or wherever those things come from.” Dean titled it “Only Let Go, and Let God.” It is also the name of an album he recorded on cassette. The worshippers at St. Patrick’s Church in Carlsbad still sing it. It is also played at Edgemoor’s monthly memorials. The final verse reads: “Many the fears that I once knew. I did not know how to get through them. But now I can go boldly through them all, putting my trust in Him. If you are burdened only let go and let God take charge of your life for you. Only let go, and let God!”

Dean touched many others with his music. He directed the choir at St. Patrick’s and played in different groups, including the Crystal Cathedral Choir in Garden Grove and the La Jolla Symphony and Chorus whenever it needed a third chair at trumpet. He played at many weddings and funerals and taught private lessons.

Son also faced risk of HD

HD shadowed Dean in another way: his son David might have the defect. When a mother passes on HD, it generally retains the same level of severity. But for reasons not yet understood, a father can give his children a far worse case of Huntington’s than he experiences.

Growing up, David didn’t feel especially close to his father, but Dean used music to bring them together. “He would help me not just listen to music,” David says. “He would play some classical music or symphonies for me and help me to visualize different things: ‘What does this song remind you of? How can you make this song important to you? How can you allow yourself to really feel the music, to live the music?’ I loved music. I loved going to concerts and hearing him play. As a young kid sometimes I wouldn’t want to go to those concerts. I would rather be with friends. But he wouldn’t give up.” Dean paid for David to take a few guitar lessons, and David later learned to play songs by ear in a matter of minutes. For a while he played in a band with some friends and even made a video with a vocalist trying to angle a professional contract.

Dean’s passion for music turned into a desperate drive for success that signaled to David and his mother that something was awry. In a classic early warning that HD was at work, Dean made a series of irrational decisions in the years leading up to the full-blown illness. He also started lashing out at others. Dean quit his job to concentrate fully on music. Investing in studio time, he made a tape with about a dozen Gospel songs and sold copies at the church gift shop. Broke and struggling, Dean saw his marriage head for divorce. “I knew that he was dealing with something,” David remembers. “I knew that this was not the same dad that I knew as I was growing up.”

One day David suddenly learned from Dean that the grandmother he had always thought “just wasn’t feeling good” had suffered from HD. Shortly thereafter David was stunned with the news that his father also had HD. “I was crushed when I first found out,” David says. “My dad and I weren’t super close like my mom and I are, but I still love him. It hurt real bad when I found out. For about a week or two I kind of distanced myself from him. I felt, ‘My God, this can’t be happening. He’s such a strong man with a good mind.’”

David was just sixteen. The normal testing protocol for HD prohibits testing before the age of eighteen, because of the emotional impact the results can have on a child or adolescent and because there is no treatment. But when he was seventeen, David insisted on being tested. The Huntington’s disease clinic at the University of California, San Diego agreed to make an exception. “They told me there was a fifty percent chance that I might have it. I waited about 3 or 4 months before I went to get tested myself. I didn’t really want to know at first,” David says. He faced the prospect of having to think about HD the rest of his life. “But it’s better that I know and be able to tell my [future] wife everything.”

Before receiving his test results, David received counseling from a psychologist. “She kind of helped me learn that if I do have it, it could happen when I’m 20, or it could happen later. The disease varies. She helped me realize that dying is a part of life. If it comes about that I have it, it will just be another obstacle that I’ll have to deal with, it’ll be just part of life. It was good to talk with somebody, even before.”

The protocol also requires a support person to be present when results are revealed. But David refused offers from Dean and a friend to go along. “If I had it, I wanted to deal with that. I wanted to have it sink in before I told anybody,” David says. “I didn’t want someone feeling sorry for me. I didn’t want anybody to feel bad.”

David was convinced he had the genetic defect. “That was really weighing on me. My dad’s sister had it. My grandma had it. There was no way I was going to escape from that,” he says.

David tested negative for HD. For Dean the outcome was “a big load off my shoulders.” But difficult, contradictory feelings gnawed at David. “When I found out the news, I was happy. I won’t have this horrible disease when I turn 35 or 40,” he recalls. “But I was also sad in a way. I felt that I was getting away, and my dad wasn’t. I just felt bad that I couldn’t relate to him in a couple years and know how he’s feeling.”