Georgia mercy killing shines light on plight of Huntington’s sufferers
Many don’t understand families’ anguish as loved ones decline
Before that one fateful summer morning, Hampton, GA, homemaker Carol Carr’s life wasn’t the stuff of front-page news. The 63-year-old former telephone company employee was known as a friendly, hardworking, and religious woman who devoted her life to caring for her terminally ill husband and three sons, friends and family members say.
All that changed June 9, 2002. That day, Carr went to the nursing home where her two sons, Randy Scott, 42, and Andy Scott, 41, were living. In the advanced stages of Huntington’s disease (HD), both were unable to walk, talk, feed, or clothe themselves and required round-the-clock care.
Shortly after she arrived, police reports state that Carr took a small-caliber handgun and fatally shot each man behind the ear, then went to the lobby to wait for police. She was arrested and charged with two counts of malice murder.
In the weeks that followed, news accounts of the case divulged the private tragedy Carol Carr had been living with — a husband who spent a decade slowly dying from a disease that also would claim the lives of all of her children.
Her arraignment earned front-page coverage in the Atlanta Journal-Constitution, with a picture of her sobbing, sitting in the courtroom in handcuffs. Newspapers across the country are following the case closely, and her arrest has sparked a renewed debate over mercy killing and the availability of quality health care at the end of life.
But for many who live with HD and the physicians who care for them, the case is only a sad reminder of how the condition devastates those it strikes. "When I talk to patients and family members about the Carr case, almost everyone says that they understand what happened and sympathize, although there is a sense that they wish it had not come to that — that she felt that this was her only alternative," says Andrew Feigin, MD, assistant professor of neurology at the North Shore-LLJ Research Institute in Manhasset, NY.
Feigin treats patients with HD and works with them at the institute.
"There are few people around the country who are really educated about Huntington’s disease and what it does to families," he says. "Even well-trained neurologists and internists may not be fully aware of what the implications are."
Patients have called him from California because they felt they could not find a health care provider who understood HD and the problems they were facing, he adds.
"I know that there are people with expertise in HD in their area, but they don’t always know how to go about finding them," he says.
Disease decimates whole families
HD is a hereditary brain disorder that slowly robs a person of his or her ability to walk, talk, think, and reason. Symptoms usually appear in midlife, and the disease then progresses over the next 10-25 years. Currently, there is no effective treatment to slow the progression of HD, and there is no cure. Medical management focuses on alleviating symptoms and maximizing the quality of life.
Families affected by HD must cope with physical and emotional stresses, Feigin says.
"For the patient, there is the fact that HD primarily affects people in the prime of life," he explains. "It can happen at any time, from childhood on through later in life. But the average person with HD starts to show signs in their 20s, 30s, and 40s. It is a time when people are starting out in their jobs, having families, that sort of thing."
HD is not just physically debilitating, but also causes significant behavioral changes in many patients, he adds.
"Some people’s personalities completely change. Some people become severely depressed. Some people start thinking about suicide or commit suicide," Feigin explains. "People become paranoid about other family members or friends and can become combative or even violent."
Because the condition is a dominant genetic condition, a child of a HD patient has a 50% chance of also having the condition.
"You end up with families in which the parent has the disease, multiple children have the disease, possibly in-laws and cousins have the disease," he continues.
In Carr’s case, her mother-in-law died of HD, her husband died in 1995 after living with the illness for 20 years, and her living son, James Scott, is in the early stages of the disease.
Family members of patients with HD can feel overwhelming isolation, depression, and guilt as they watch their loved ones suffer, Feigin says.
Carr is not the only person in Georgia to have killed a loved one who was affected by HD. In 1985, Glenda Caldwell shot and killed her son and also wounded her daughter. Caldwell, in the early stages of HD, wanted to spare her children the suffering she knew awaited her.
It is important that health care providers make sure families know there are resources available to help them cope.
"Support groups for patients with HD, with caregivers, and for people at risk are important so that they don’t feel isolated, that they are the only people in the community dealing with these problems," Feigin says. "There are a number of groups out there, but people don’t always know about them."
Physicians should also know that there are medications available to help alleviate some of the behavioral symptoms of HD and treat depression.
But even when support groups, counseling, and knowledgeable providers are available to HD patients and families, tragedies still occur — not all are as well-publicized as the Carr case, but devastating all the same, Feigin adds.
"Terrible things happen, and it is not necessarily because we didn’t do everything we could," he says. "I have been involved in situations also where bad things have happened to my patients after they were given the diagnosis of HD. You don’t want to see it happen, but it does."
More HD research needed
To avoid the sense of hopelessness and despair that many patients and families feel, more needs to be done to help HD patients live meaningful lives for as long as possible, and then to have control over the time and manner of their death, adds Jerry Lampson, whose wife had HD and died two years ago. Lampson is the founder and administrator of an Internet support network for HD patients and families, HDlighthouse.org. He started the site to collect information that would be helpful to caregivers in the nursing home where his wife was cared for in the final months of her life.
"HD may begin by taking away a woman’s ability to wear high heels and end by taking all that a person is," he says. "Most HD patients know who they are and what is going on around them. They are frustrated by treatments that isolate them."
Whenever possible, clinicians who work with HD patients should strive to find a balance between aggressively treating a patient’s physical symptoms and selecting treatments that allow them to retain some control of their bodies and environment.
"For instance, chorea [involuntary movements of body and face] is only a small part of HD," he says. "In comparison to all that is happening to a patient, it is insignificant and may not even be noticed by the patient. But, just seeing a patient with chorea upsets some caregivers. When a patient is treated to decrease involuntary movements, voluntary movements are also taken away. Sometimes, it seems the patient is treated more to relieve the distress of the caregivers."
Historical treatments such as high-fat diets and use of haloperidol (Haldol) has been contraindicated by modern research, Lampson claims. "The overuse of narcoleptics as a patient restraint just tends to produce incoherent, suffering zombies."
Little research is currently being done related to newer possible treatments of HD and its symptoms, he adds.
"Most health care workers are ignorant of treatments that are low-risk and are likely to be of great benefit," Lampson says. "There have still been no phase III trials of any effective intervention even though, [for example], small trials of fish oil have reversed HD symptoms in humans and prevented their onset in mice."
There are some proactive HD patients who have reported doing well with a healthy diet, use of vitamin supplements, and exercising as much as possible, he adds.
The other key problem is that once a patient reaches the end stage of the disease and needs advanced skilled nursing care, payment and reimbursement policies do not meet their needs, Lampson says.
Unlike Parkinson’s, HD is not a condition eligible for fast-track Social Security benefit approval. And most hospice programs require that a patient have a diagnosis of only six months to a year to live in order to receive care, Lampson says.
"The time limit for hospice care does not fit HD," he says. "Here, it takes a committee of people with medical backgrounds to find that the patient will die within a year. That may fit most things, but not HD. There is an average of 17 years between onset of HD and death, and the need for hospice care is greater because the patient usually knows what is going on."
Lampson’s wife, Peggy, decided to end her life through starvation — refusing to eat and refusing artificial nutrition — in order to maintain control over the quality of her life, he says. "She picked her day to die."
Society fears and ostracizes patients
It is important for physicians to remember the stress and pain that families face and act to ensure they are aware of resources available to them, says Joanne Lynn, MD, MA, MS, director of the Washington Home Center for Palliative Care Studies and president of Americans for Better Care of the Dying in Arlington, VA. But that’s not enough and, in reality, the resources that patients and families truly need simply aren’t there, she adds.
"In the long run, we, as a society, will have to confront our inadequate responses to the situation that this mother was left with," she says, speaking of the Carr case. "If we were a caring community, as we could be, people in her situation would be seen as tragic and noble and worthy of community support."
Families devastated by HD and other terminal and chronic illnesses not only face the physical and emotional toll of dealing with the illness itself, but also face severe financial challenges in health care, have difficulty finding adequate skilled nursing care, and often feel that their lives are no longer seen as worth anything to the community around them, she says.
"If we were a caring society, she would have had confidence that she and they would live out lives of reasonable comfort and support, networked with people who care for them," she says. "Most people in long-term care do not feel this way. They are made to feel as if they are burdens on society, worthless, and irrelevant. We could do better."
Sources
- Andrew Feigin, MD, North Shore-LLJ Research Institute, North Shore University Hospital, 300 Community Drive, Manhasset, NY 11030.
- Jerry Lampson, 10965 Old Pond Lane, Grass Valley, CA 95949. E-mail: [email protected]. Web site: www.HDLighthouse.org.
- Joanne Lynn, MD, MA, MS, The Center to Improve Care of the Dying, RAND Corp., 1200 S. Hayes St., Arlington, VA 22202-5050.
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