The Huntington's Disease Society of America: Dedicated to Education and Care

November 1, 2006
Gerald W. Keister

Huntington disease (HD) is a devastating hereditary, degenerative brain disorder for which there is, at present, no effective treatment or cure. More than a quarter of a million Americans have HD or are at risk for the disease because of potential genetic transmission. The disease slowly diminishes the affected person's ability to walk, think, talk, and reason. As it progresses, concentration and short-term memory diminish and involuntary movements of the head, trunk, and limbs increase.

Huntington disease (HD) is a devastating hereditary, degenerative brain disorder for which there is, at present, no effective treatment or cure. More than a quarter of a million Americans have HD or are at risk for the disease because of potential genetic transmission. The disease slowly diminishes the affected person's ability to walk, think, talk, and reason. As it progresses, concentration and short-term memory diminish and involuntary movements of the head, trunk, and limbs increase. Walking, speaking, and swallowing abilities deteriorate. Eventually the person is unable to care for himself or herself, and becomes totally dependent on others for care. Death follows from complications such as choking, infection, or heart failure.

The average life span after onset of HD is 10 to 20 years. The disease profoundly affects the lives of entire families--emotionally, socially, and economically. To assist and support patients and caregivers, the Huntington's Disease Society of America (HDSA) provides a national network of services and educational programs through its many volunteer-based chapters and affiliates, support groups, and HDSA Centers of Excellence for Family Services.

HDSA CENTERS OF EXCELLENCE

The HDSA has formed a nationwide network of resources and referrals for families and the allied health care professionals who serve them, according to Debora Lovecky, the HDSA's director of educational programs and services. "A cornerstone of that care program is our 21 HDSA centers of excellence," she said. "These are medical facilities recognized by the HDSA as having expertise in movement disorders and/or HD." These centers provide a multidisciplinary approach to the treatment of HD. Services include--but are not limited to--neurology; speech, physical, and occupational therapies; genetic counseling; and genetic testing. The centers also have resources for providing regional referrals.

In the clinics at the centers of excellence, patients are evaluated about where they are in the progression of their disease. Their treatment plan is periodically reevaluated, and any adjustments that need to be made in either medication or in ancillary treatment, such as physical, occupational, or speech therapy, can be made as well.

Lovecky explained that although centers of excellence are used for annual or semi-annual evaluations, they are not the primary care providers for HD services. The patient takes the treatment plan that is developed by the center of excellence back to his primary care provider in his local city, and that provider uses the plan to treat the patient until his next regularly scheduled evaluation or until a crisis occurs.

Assisting at these centers of excellence are neurologists, psychologists, psychiatrists, neuropsychologists, social workers, speech language therapists, physical therapists (PTs), occupational therapists (OTs), nutritionists or dietitians, genetic counselors who do genetic testing, and clergypersons for spiritual counseling.

Funding for each center of excellence begins with the HDSA Celebration of Hope events, and the centers also receive an annual grant from the HDSA.

LOCAL CHAPTERS AND AFFILIATES ASSIST

Also assisting the centers of excellence in delivering services are the society's 36 volunteer-based local chapters and affiliates, which provide referrals to community-based services that are available for persons living with HD--whether they be patients, caregivers, or family members. The chapters and affiliates also help identify primary care providers and allied health care professionals, such as PTs, OTs, and speech therapists, to serve patients with HD. The HDSA provides a toll-free hotline with information about referral sources and local resources available to families and patients. The number is 800-345-HDSA.

Local chapters and centers of excellence offer several types of support groups. Lovecky pointed out that there are about 150 different support groups all over the country. Some are targeted to specific audiences, such as caregivers or persons at risk, while others are general support groups where anyone in the HD community can receive emotional support. Moderators and attendees work together in problem solving and assist in identifying resources within the community. Locations of support groups are listed on the HDSA's national Web site at www.hdsa.org.

"In addition to emotional support, family members can get good references about health care professionals from support group members," said Lovecky. "They're assured that the referral they get is someone who is familiar with the disease. Many health care professionals simply never see a patient with HD."

SOCIAL WORKERS COORDINATE, EDUCATE

Social workers, employed by the HDSA at many local chapters, play a pivotal role in orchestrating and delivering services to patients and families. They work with the families to locate a PT, OT, or nutritionist; or to identify an adult day-care center, a long-term-care facility, or a respite facility. They help families obtain wheelchairs, walkers, or assistive devices. Medicare or health insurance policies cover most devices, Lovecky pointed out.

Social workers frequently make home visits. Indeed, they work closely with PTs and OTs when they visit patients' homes to evaluate how fixtures might be rearranged or modified to make daily living easier for the patient.

Lovecky explained that social workers also conduct in-service programs in long-term-care facilities where patients with HD are residing. They train the staff about the special needs of the patient with HD. "One of the biggest problems for a person with HD is taking in enough calories to maintain good health," she said. "The patient may have involuntary movements called chorea, which burn up a lot of calories. They may also have trouble swallowing, chewing, or drinking liquids, and require a very long time to eat a meal. Because they can't feed themselves, they must rely on an aide to feed them. Frequently, the in-service program will stress the need to provide more and smaller meals consisting of foods that are easy for the person with HD to swallow and digest. These are things that staff members in long-term facilities often do not understand or appreciate, and they must be educated."

Joanne M. Luz, social worker at the HDSA's Delaware Valley Chapter in Plymouth Meeting, Pennsylvania, does in-service training for the staff at assisted-living facilities, nursing homes, and long-term- care facilities. These institutions arrange to have a variety of people--including nurses aides and nurses and sometimes OTs, PTs, speech therapists, and dietitians--attend training. Luz explained that among other things, the nursing home staff learns how to better communicate with patients. Because a patient with HD often has difficulty in communicating his needs to nurses, the nurses often ignore the patient's agitation and anxiety and instead medicate him and put him to bed.

Luz said a majority of neurologists and psychiatrists have heard about HD, but they are not that familiar with it. "I would be happy to help them become more familiar with HD if they asked for it," she said, "but doctors tend to want to talk to another doctor rather than to a social worker. Most neurologists and psychiatrists are only going to see a few patients with HD in their whole career. Unless they decide they really want to help a particular patient, they don't go looking for these resources." She said there are a few neurologists in her area who specialize in treating patients with HD, and they have a huge caseload.

CAMP GETAWAYS

Certain chapters operate camps where the person with HD can go away for a week or so to enjoy a change of scenery and provide respite for the family. "We've been doing a weekend retreat for a number of years," Luz explained. "It consists of a small group of persons who are in the early stages of HD without their families. It's an opportunity to get away with other people. Caregivers get a break, and persons with HD enjoy a new environment in which to socialize."

Many camps also have family days, and some chapters offer retreats for caregivers, which allow them to get away for a weekend.

EDUCATIONAL PROGRAMS

The HDSA provides educational programs for both health care professionals and laypersons through its local chapters and affiliates. Professional education is provided through centers of excellence, either in direct consultations, through miniresidencies, or through grand rounds programs. The centers bring in and train medical students, residents, and interns to work in the HD clinics to familiarize them with the disease. In addition, the centers provide one-on-one consultation with primary care providers and other medical professionals who may be treating a patient with HD but who are not very familiar with the disease. Many physicians are convinced, for example, that haldoperidol is the best medication for patients with HD, when it is the worst.

The society also conducts professional training for social workers. The course is designed to provide them with new ideas about how to work within the community and to update them about clinical trials so they know how to refer a patient to a clinical or observational trial.

Local chapters and centers of excellence provide lay education through seminars, workshops, symposia, statewide conventions, and national conferences for families. The HDSA conducts a national convention annually, with attendance usually averaging about 800 persons. National conventions offer opportunities for families to learn more about HD, what's going on in research, the newest medications, clinical trials, new approaches to how to live with the disease, living positively through exercise and nutrition, and spirituality.

NATIONAL YOUTH ALLIANCE

The HDSA's National Youth Alliance (NYA) provides a support network for adolescents and young adults who are living with HD; it is open to anyone aged 9 through 29 years and can include persons with HD, persons who are at risk, a caregiver, or a friend.

"The NYA has a chat room that allows teens who have HD in the family to share their common experiences," noted Luz. "It can be accessed by clicking on the 'Community' link on the society's national Web site."

PRINT RESOURCES

The HDSA offers a vast array of publications:

  • A pamphlet titled Fast Facts About HD presents the basics of the disease.
  • The Family Guide series consists of 7 titles that target specific aspects of HD. They are short pamphlets, meant to give an overview of a specific part of HD. The series includes pamphlets on PT and OT, speech, genetic testing, nutrition, long-term care, and juvenile HD.
  • The HDSA sends out family information packets to those who inquire by phone, mail, or e-mail. These packets are an important first step in good care for families who are just starting to face the challenges of HD.
  • The Professional Guide series consists of 4 handbooks: The Physicians Guide discusses HD from a medical professional point of view, Understanding Behavior in HD discusses the psychological aspects of the disease, The Caregivers Handbook for Advanced-Stage HD is a resource for long-term-care facilities and explains why HD is unique from other movement disorders or chronic progressive illnesses, and The Juvenile HD Handbook is intended for families who have a child in whom juvenile HD is diagnosed.

Magazines, newsletters, and research updates also offer HD education and information to more than 65,000 readers:

  • Marker Magazine, published twice a year, covers research, care, and HDSA activities at large. It is geared toward caregivers, those at risk for the development of HD, advocacy groups, family services, and those involved in juvenile HD.
  • Toward a Cure, the society's semiannual research newsletter, provides insight about research in drug discovery and discusses clinical trials.

GRANTS and fellowships

The HDSA Research Grants program serves as important seed funding for new or innovative research projects with the expectation that these projects will be developed sufficiently to attract funding from other sources. Without the HDSA Research Grants program, many new initiatives in HD research might never be undertaken. These research grant awards are available to principal investigators in the amount of up to $50,000 for 1 year. They are not renewable, but investigators can apply for another grant.

HDSA Research Fellowship awards are designed to help promising postdoctoral investigators in the early stages of their career. Fellowship awards are available in the amount of up to $40,000 for 1 year. Fellows who have demonstrated sufficient progress are eligible to apply for a second year of funding.