A haunting inheritance

Second of two partsIn his later years, Frank Jackowski didn't leave his wheelchair except to return to his bed in the nursing home. He couldn't speak. His family had little idea what he was thinking or feeling. Most of the day, he sat in front of the TV.Huntington's disease, a rare, incurable neurological disorder, had already stolen his mind, and his body followed, wasting away to 65 pounds. He died of heart failure a few months before his 45th birthday."He kind of shriveled up," said his son, Dale Jackowski. "He wasn't able to keep any muscle on, and he was just deteriorating. It really was a blessing when he died because he couldn't do much at that point."After Frank Jackowski's death, his family entered a kind of reprieve. For about 12 years, Huntington's disease faded into the background of their lives.Danny Jackowski, the baby of the family, grew up, got a job and used his inheritance to buy a condo in Rosemont. Dale, the eldest, married and settled in Schaumburg. Cheryl, the only daughter, bought a house in Carol Stream with her husband.Both couples decided against genetic testing that could have revealed whether they risked passing the Huntington's gene to their children. Dale and his wife Cyndee had three kids; Cheryl and Bill Hinde had two.And then their little brother, Danny, got a wrenching diagnosis. Suddenly, Huntington's disease was back in their lives.A first glimpseHuntington's disease is genetic. A parent with the abnormal gene has a 50 percent chance of passing it to his offspring. A child who inherits the gene will eventually develop Huntington's disease, unless he or she dies of something else first.Danny Jackowski started showing symptoms at age 24, earlier than his father had. When he could no longer work, drive or manage his personal life, Danny moved in with his mother, Rose. In January, he moved into the DuPage Convalescent Center in Wheaton.Until Danny got sick, his nieces and nephews had no experience with the devastating reality of the disease. Their grandfather -- Frank Jackowski -- died before they were born."I don't think they understand totally what is going to happen," Dale Jackowski said. "My father wasn't around for them. His sister was in a nursing home, so they didn't really see her a lot."Really the first glimpse they're getting is with Dan."The questions started a few years ago, when Danny's decline became obvious."Why is Uncle Danny sick?" Amanda Hinde, then 11, asked her mother Cheryl."He has Huntington's," Cheryl said. "That's what my father had.""Does that mean you'll have it?""I hope not."Family fearsIn fact, Cheryl is terrified of Huntington's."I think about it every day," says Cheryl, who is 45. "When I lose my keys or misplace my glasses, I think, 'Oh, my God.' It's just there."Cheryl's fears are not just for herself, but for her son, 20-year-old Matthew, and Amanda, now 14. And for her husband, Bill, who watched his father-in-law's final years and now struggles emotionally with Danny's illness.Bill Hinde worries the same disease could afflict his wife and children."I freak out all the time," he said. "The hardest part is the burden on the family and the caretakers. The emotional drain, the financial drain, is tough on a family."If they had to do it over, Bill Hinde says, he would still marry Cheryl, his high school sweetheart. But maybe he would consider genetic testing before having children. Bill was 21 and Cheryl was 19 when they got married in 1981."It was different then," he said. "You were young, you got married, you had kids. We never gave it a second thought. Now being older, would I give it a second thought? Absolutely."Until scientists isolated the Huntington's gene in 1993, the only test available required multiple family members -- including one with the disease -- to submit blood samples to look for genetic markers, or similarities that help determine a person's risk.Dale said he would have been tested if other family members agreed. Dale had seen the worst of the disease in his father and knew what a positive test would mean for his future. So he was ambivalent about risking passing the gene to another generation. But his wife, Cyndee, had always wanted a large family."She understood what the risks were," Dale said. "I always knew family was important to her, and she wanted children."Dale and Cyndee had three kids: Kristy, 22; Bradley, 21, and Kelly, 18.At 50, Dale doesn't see a need to be tested now. He thinks he's probably safe from the disease, which tends to hit people in their 30s or 40s, though some do develop symptoms at younger or older ages. He stays in shape and feels healthy.Cheryl didn't want to be tested when she married, particularly if the only option after a positive result was to forgo having children. She doesn't regret that decision."I chose to have children. That was our choice," Cheryl said. "I still don't want to know. Would you want to know if you had the gene?"Tough decisionsHer son gives a different answer to that question.As Danny became sicker, Matthew Hinde told his parents he'd like to be tested for the gene. The change in his uncle was dramatic."He always used to be funny, always joking around," Matthew said. "So, now, it's just different. I just want to know."Most people don't. Of all the people at risk for Huntington's disease -- by some estimates 200,000 people -- only 3 percent to 5 percent will seek testing to learn whether they carry the gene before they develop symptoms.Today's test is more sensitive and can detect the gene in an individual's blood sample, even without other family members.But there's no medical advantage to knowing early, said Dr. Kathleen Shannon, a movement disorder specialist at Rush University Medical Center in Chicago. No medications or therapies can prevent or delay the disease.The decision to be tested is so momentous that people are required to undergo psychological counseling first.According to Shannon, people who seek testing fall into two groups:bull; Some people can't stand not knowing. They'd rather know they have the gene than wonder whether they'll get sick.bull; The other group includes those who are facing a decision: Should I go to grad school? Should I have a child? In some cases, middle-aged adults will be tested as their children marry and contemplate starting families. If the parent doesn't carry the gene, the children won't, either.Stephen Clingerman, a neuropsychologist who evaluates Huntington's patients at John H. Stroger, Jr. Hospital in Chicago, said many people see no reason to be tested."Until there's a treatment or a cure, they're waiting," he said.Younger people like Matthew might be more likely to take the test, a sign of changing attitudes about disease, he said. Most people who are tested, even if they turn out to have the faulty gene, feel less anxiety and stress after they know one way or the other, Clingerman said. The most difficult periods are usually the year after they receive the test result and the year they begin to show symptoms."Obviously, there can be a long time in between," he said.Dale's kids haven't raised the issue of genetic testing."As long as I'm OK, they're going to be OK," Dale said.Dale's son, Brad Jackowski, a 21-year-old college student, says he does worry about his dad getting Huntington's."I am worried, a little bit," Brad said. "I don't know 100 percent what the disease is about, if it's in your genes. I am a little concerned about it."Until Danny was diagnosed, Brad says, he had never heard of Huntington's disease. Now he struggles to understand his uncle when he speaks. Danny mumbles half his words. This summer, Brad, Dale and Danny went to a White Sox game together."He's dramatically changed in the last couple of years," Brad said. "It's frustrating to watch him go through all that and see how my dad and my aunt are dealing with this."He said he'd be curious to learn about a test that could reveal his risk of developing the same disease, but he hasn't given it much consideration.Matthew Hinde's parents have advised him against genetic testing. Besides the devastating ways a positive result could color his future, they worry about the very real implications regarding health insurance. No federal law protects people against discrimination based on the results of genetic tests.Testing a single member of a family also raises thorny issues for those who don't want to know their own results. If Matthew tested positive, that would mean his mother carries the gene, too, and that his sister is at risk.Medical science offers little in the way of effective treatments for Huntington's disease, but it does offer an option unavailable when Matthew's mother faced the question of having children. Today, advancements in reproductive technology can eliminate the risk of passing the gene to the next generation.As part of in vitro fertilization, a doctor can screen embryos for the Huntington's gene and implant the embryos that do not carry it. This can be done without disclosing whether the at-risk parent carries the gene.Would Matthew live differently if he knew he carried the faulty gene?"I'd probably just have to live with it and do what I can," said Matthew, a student at College of DuPage. "There are things out there that might slow it down."Hopes and worriesBill Hinde finds comfort in that idea, too. He is hopeful for his kids' futures, whether or not they have the gene. At a Huntington's fundraising walk last year, he met a researcher who offered encouraging words about the chances for good treatments."If you already have the symptoms, it's too late," he said. "But kids in the future have a much better shot than someone Danny's age or my wife's age. Odds are, they're going to be all right, with or without it. Does that mean they should go out and have kids? Well, no."Danny is providing a heartbreaking glimpse at what could be the next generation's future if the hoped-for treatments don't materialize quickly enough.A couple of years ago, while Danny Jackowski was still well enough to travel, the family took a cruise to Costa Rica, Mexico and Belize. They built some good memories, and some bad ones.They rode dune buggies, visited Mayan ruins, drank beer after beer in dockside bars. "We tried to do a lot of things with him because he probably wouldn't get another shot," Bill Hinde said.But Bill chafed at the negative attention directed toward Danny when he showed symptoms, such as shaking or coughing up his food. Now it's hard for Bill to visit Danny at the convalescent home."I have a seriously hard time with it now, just watching it," Bill said. "It's even hard for me to be in the same room. I become unglued."Conversations are difficult. "He'd love to have a conversation with you, but he can't," Bill said. "He'll come over and we'll be watching the Super Bowl, and he wants to say something and it will just blurt out."Cheryl says, "My dad was the same way.""You might understand one or two words," Bill said. "I always wondered what does he really understand? I understand the disease affects your motor skills. Does he have a clue his life sucks?"A place for DannyCaring for Danny has been hard on Rose. After he was diagnosed, she took two months of disability leave to deal with her grief. At 68, she has her own health issues, and the stress of Danny's situation has only exacerbated them.Stress makes it impossible to clear up her rosacea, which leaves her face permanently flushed. She has diabetes, high blood pressure and extra weight. She went to a doctor to be treated for depression.When it became too difficult to care for Danny at home in Schaumburg, Rose decided to place him in a nursing home. He couldn't shower on his own. She worried about him choking while he ate, and she wasn't strong enough to physically support him if he fell.Finding the right place took months. Rose and Cheryl called dozens of facilities; many were unfamiliar with Huntington's and unwilling to care for someone with the disease. Huntington's afflicts a younger population than some centers are designed to care for, and its victims progressively worsen. That requires specialized care.It's such a common problem for families that the Huntington's Disease Society of American produced an instructional video about caring for patients with the disease; the society delivers it to nursing homes who get a new patient.Danny is the only resident with Huntington's at the DuPage Convalescent Center. He lives in a wing populated mostly by other young people; some have diseases like cerebral palsy or cystic fibrosis, others suffered brain or spinal cord injuries.Above his bed, his mother has pinned photos of his dog, a yellow Lab named Luke. In another picture, a brown-haired Danny -- in healthier days -- smiles next to one of his nieces. A rosary hangs from a pushpin.Two weeks before Danny was due to move in, Rose had a birthday. But she didn't feel like celebrating, and the family postponed her dinner two months. It has grown a bit easier since then, knowing he seems happy there."There are days I still stay home in bed," she said. "It took a lot of crying and a lot of thinking to put him here, and I still cry on the way over. But this is the best place for him."Now she visits him a few times a week. Even if they don't carry on a conversation, Rose finds comfort sitting next to Danny for a couple of hours while his laundry dries. For special occasions -- such as a birthday party or a Sox game -- his family will take him out on a field trip.Danny seems to have adjusted well to his new life.He wakes up around 7:30 and an aide helps him dress. He walks down the hall for breakfast -- usually pancakes, cereal or another soft food. He can feed himself."If I eat slow, I'm fine," he says.After breakfast, he'll often sit in his room, sometimes listening to his collection of heavy-metal CDs, which he finds soothing. He has regular occupational and physical therapy sessions to help him practice walking and picking up silverware. He participates in trivia quizzes and card games organized by the staff.When his brother Dale calls, they talk about Chicago sports. Danny can be slow to respond as his brain struggles to find the words. Often, those words come out muddled.In the afternoons, Danny likes to watch TV, especially if there's a game on. Or he'll watch whatever program his roommate has chosen. Occasionally, he gets a visitor."A friend of mine, Don, he came to see me with his daughter," Danny said. "He was a nice guy."Danny regrets that he'll never marry and have a family of his own. It's been a long time since he dated a girl, he says. And he misses his dog, who lives with Rose. When asked what he'd like people to know about Huntington's disease, he answers, "how it screws up your life."Danny says he knows it's easier on his mom for him to be at the center. He likes the staff -- "they're like your family," he says.He's not in pain, and he's not sad or angry like he was in the early days after his diagnosis."I'm a mellow guy," he says. "I've accepted it."Yet Danny is more optimistic than his mother, who believes any cure will come too late to help her family. Danny hasn't given up on himself -- not yet."I'm hoping for a cure."What is Huntington's disease? bull; Huntington's disease arises from a genetic abnormality that causes brain cells to degenerate, leading to uncontrolled movements, loss of intellectual abilities and emotional disturbance. bull; Early symptoms include mood swings, depression and irritability, as well as trouble driving, learning new things and making decisions. bull; A doctor can diagnose Huntington's with a genetic test, medical history, neurological and laboratory tests. Pre-symptomatic testing is available for those at risk of carrying the Huntington's gene. In 1 percent to 3 percent of people with the disease, no family history of Huntington's can be found.Source: National Institutes of Health For more information on Huntington's diseasebull; National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htmbull; Huntington's Disease Lighthouse http://www.hdlighthouse.org/bull; Huntington's Disease Society of America http://www.hdsa.orgbull; Huntington's Outreach Project for Education at Stanford University http://www.stanford.edu/group/hopes/Discovery of Huntington's gene launches search for a curePart 1 -- A family fate 512312From left to right, cousins Bradley Jackowski, Matthew Hinde, Kelly Jackowski, Kristy Jackowski and Amanda Hinde pose at a family party this summer.Courtesy of Cheryl Hinde 512335Dale Jackowski, 50, of Schaumburg believes he's probably safe from Huntington's disease, which usually strikes younger people. His father died of the disease.Daniel White | Staff Photographer