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Managing Patients With HD

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        Managing Patients With Huntington's Disease
By: Steven M. Hersch, MD, PhD
Department of Neurology,
Massachusetts General Hospital, Boston, Ma.

Anthony Lechrish, MD
Medical Director
Terence Cardinal Cooke Health Care Center, NY, NY
When a distraught Georgia mother killed her two sons afflicted with late-stage Huntington's disease in a nursing home last June, the tragedy triggered full tabloid treatment of "justifiable homicide."  Huntington's disease, and the capacity of a skilled nursing home to provide humane,
dignified care.

This desperate act defined the lifelong role of Carol Carr as caregiver to her husband, who died of the disease, and her three boys.  After witnessing the tortured course and demise of her husband and the rapid progression of the disease in two of her sons, Mrs. Carr apparently hit her emotional bottom.  She shot her sons, ages 41 and 42 (her youngest son is now in early stages of the disease), as they slept in their room.

Could there have been a different out-come?  What as missing that convinced
Mrs. Carr not to choose life for her sons?

What follows are some thoughts on the nature of Huntington's disease (HD), the care challenges it presents to long-term care practitioners and positive examples of the nursing home experience with late-stage HD patients.


Huntington's Disease, an autosomal-dominate neurodegenerative disorder, is
characterized by progressive emotional, cognitive, and motor dysfunction. There is a 50% chance that an affected parent's offspring will inherit the defective gene.  Everyone who has the mutant gene will develop the disease if they live long enough.  Symptom onset is typically between 35 and 50 years of age, but may occur much earlier or later, making the prediction of onset and course of disease difficult in any given case.

Because of its genetic nature, members
of HD families often have previous
experience of the disease and chronic concerns about it how it may affect
them or other family members.  As was the case for Mrs. Carr, caregiving can
extend across multiple generations and decades of effort and losses.

A staging system has been suggested, and is reprised here to help frame the course
of HD, thought it should be noted that there is tremendous variability among individuals.

  • Emotional difficulties, including irritability, depression, anxiety
  • Cognitive problems, including forgetfulness, errors in judgment
  • Infrequent involuntary movements
  • Diminished adaptability and ability to learn
  • Frequent and severe involuntary movements
  • Difficulties with job performance and personal financial management
  • Frustration and depression in response to increasing dependency
  • Difficulties with activities of daily living, particularly eating
  • Social isolation due to speech deterioration
  • Increasing mental deterioration
  • Falls, increasing compromises of all voluntary motor function
  • Severe chorea, dystonia, and rigidity; loss of ambulation and ability to assist transfer
  • Tube feeling to maintain nutrition when desired
  • Speech totally impaired, communication extremely difficult
  • Severe mental disability
Information is rapidly accumulating that will facilitate the prediction and prog-nostication of HD, and ultimately chart the best treatment in an individual case. 
Recent advances in our understanding of the pathophysiology of HD, and the therapeutic experiments using transgenic
animal models, are leading to new treat-ments with great promise for treating
the progression and expression of the disease.

However, although scientific and therapeutic advances are providing
unprecedented hope for the future, individuals already affected by the
disease will continue to require care for
the foreseeable future.

What, then, of nursing home care for Huntington's disease?

With the exception of a handful of centers that have established effective programs for a critical mass of residents - such as Sunbridge Rehabilitation  in Lowell, MA; Laurel Lake Center in Lee, MA; Runnymeade Chronic Care Hospital in Toronto, Canada, Tewksbury Hospital  in Tewksbury, MA; and Terence Cardinal Cooke Health Care Center in NY, NY - most long term care providers see fee HD patients.  The demands on staff, the cost and the heartache that late-stage HD patients present to uninitiated providers are major deterrent for establishing cohort of patients.

The HD experience in New York began at the insistence of the late John Cardinal O'Connor.  His order was a response to a desperate call from a family, the Whites, who were struggling with HD.  The Whites found no welcome or understanding at the nursing facilities they explored.  And so
the Terence Cardinal Cooke Center esta-blished a care module for the patriarch of the family, James F. White, after whom the unit is now named.

Mr. White entered the facility in 1988 with a six-month prognosis, yet he lived for more then 10 years.  Through his wife Mary, we learned of the sanctity and value of a life, though damaged.  Now, 14 years later, many of the original caregivers are still working on the unit.  Despite their long service, however, they continue to require ongoing support to help with the
stress of daily encounters with functional and social loss in their beloved

The mission of these special units may be summarized as follows:  to provide a safe, comfortable, home-like environment to clients and to ensure the best quality of life possible.  Among the many common themes encountered in successful long-term care programs, perhaps the most important is family inclusion. 

What, then, of nursing home care for Huntington's disease continued?
The more input families have, the better the result in creating uniquely appropriate care plans for family members with the disease.  The degree to which an institution becomes a home is directly proportional to the presence of the family.  Mrs. Carr's comments after the tragedy seemed to reflect her profound alienation from the care environment.
Since the anticipated stay in a nursing home is measured in years, the operative philosophy must be that, despite an inevitable decline, the individual should always be considered optimistically.   Despite the cognitive decline that marks the illness, we have found that even at
thelatest stages, patients have much comprehension and retained ability for participation, resolution and joy.
The following strategies characterize an optimal care approach for patients with HD in LTC:
  • Identify and maximize preserved abilities
  • Strategize to retard deterioration of ability, self-esteem and
  • Create opportunities of personal decision making
  • Help patients understand that a certain level of dependence is necessary and acceptable
  • Discourage regressive behavior in order to maintain and strengthen self esteem and relationships
  • Seek to understand causes of behavior before implementing pharmacological constraints
  • Recognize needs for periods of withdrawal
  • Understand that anger is most often a response to dependency
When the press release of the two murders was read at a quarterly meeting of our HD Family Council for discussion and infor-mation, many of the 40 to 50 participants had not yet heard of the incident.  What was interesting and gratifying was the consistent outpouring of sympathy and acceptance of the mother's anguish (not the act), and a general nodding in agreement that they, too, had been close to that point at some time in their own journey.

The clearly stated and resonant difference for the council members was the trust and consolation that they derived from the knowledge that their loved ones are cared for with dignity and as much freedom as safely allowed.  Also expressed was the great appreciation and strength derived from the tacit support and understanding that they received from each other.

This tragedy has spurred us to better organize and disseminate information
on best-practice techniques in the care of late-stage HD patient.  Some 30,000 individuals in the United States have HD.  We feel certain that many patients and caregivers in nursing homes would benefit from these care advances.

It is also essential that all payors under-stand that the special requirements for good care in late-stage HD are more expensive than care for residents with comparable resource-utilization scores.  The investment in better nursing home care will prevent unnecessary complications and effectively avoid costly hospital stays.


Even with sound advice and experience, additional expertise is frequently
required to manage patients with HD.  An important resource may be secured
by establishing a liaison with neurologists experienced in HD.  The Huntington's Disease Society of American (HDSA) Center of Excellence (COE) Program is a vital source of regional specialists that can be consulted for help.  The HDSA chapters and COE's can also provide in-services and other valuable assistances.  Websites are now available that include discussion
boards, and many other new communications modalities.  For information on the HDSA Chapter or peer-led group nearest your facility, contact the HDSA National Office at 1-800-345-HDSA or by email:


Physical Therapy/Maintenance of Functioning
Much can be done in preserving function with specialized occupational, physical, and speech therapy.  Speech requires special attention because of the particular assault on phonation and swallowing that always complicates the course of the illness.  Some of the declines are temporized or even reversed for a time by interventions.  For example, the introduction of special walkers to the unit at Terence Cardinal Cooke Health Care Center has returned several patients to their feet for independent ambulation.

Adaptive Environment
Falls and injuries may be prevented through padding, care planning,
gastrostomy-tube shielding and judicious use of medication.

Special Recreation Approaches
With proper personal history information about the patient preferences, small pleasures can be strung together to brighten a moment, or even a day.

Hospital Education
Education is needed at the receiving end to assist busy emergency ward or unit staff in dealing with not only an HD patient but a sick, injured or emotionally uncontrolled HD patient.  Chances are high that the patient will not receive the needed understanding and treatment from an unenlightened staff.  Not long ago, many HD patients spent their last years in chronic psychiatric facilities under heavy sedation.

Adequate Staffing
The universal experience of cohorted HD units is that significantly more staff is needed than the standard formulas permit.  Where the averaged skilled nursing home resident requires one nursing assistant to bathe a patient, for example, assistant to bathe a patient, for example, a flailing HD resident might require three nursing assistance for that task.  Eating even the minimal amounts of calories requires extra time and caution.
Cleanup is also heavy, with spills and extra soiling.  Continuous contact with families by social services is required to screen and minimize transfer trauma.  Unfortunately, payors have largely ignored these added costs, which has negative impact on the ability of providers to adequately meet the clinical challenges of HD patients.
October 2002 "Caring For The Ages" Vol. 3, Number 10 "Unique Challenges for Long Term Care"
Response To Article
     Managing Patients with Huntington's Disease      
                                 Huntington's Disease
By Jim Pollard
Huntington's Disease Program Director
                       Laurel Lake Center for Health and Rehabilitation
December 07, 2002
Dear Editor,

I read the article on "Managing Patients with Huntington's Disease (HD)" in
the October issue with great interest. As a former administrator of Sunbridge of Lowell (Massachusetts) and a member of the team that founded their HD program in 1986; and as the current program director of the HD Treatment Prrogram at The Laurel Lake Center in Lee (MA), I have spent the last 16 years managing special care programs for residents with HD in
nursing homes. After reflecting on the article I offer these observations.

First, relatively few nursing home residents in the United States receive
care in specialty programs. The authors identify four homes (My own among
them, thank you!) in the US that specialize in caring for residents with HD. Their collective census of affected residents in those homes is less than 200.
The fact is that most American nursing home residents with HD receive their care in typical skilled nursing facilities with no specialized training or additional staff. Although a number of residents with HD have needs so complex that they test the resources of the finest long-term care
teams, the overwhelming majority of families appear to be pleased with the
care these typical homes deliver. Congratulations to those medical
directors, nurses, aides, therapists and social workers for their care to
these residents!

Second, I am concerned that readers may draw a misinference that caring for
a resident with HD requires "significantly more staff." The acuity scales used across the country are designed to identify the varying amounts of care that residents of a home require for reimbursement purposes. They also show that, in every home, some residents require more care than others.
Similarly, some residents with HD require more care than their fellow residents and others require less. 
Whatever the resident's need for care may
be, most often it's adequately met within the existing staffing patterns of homes across the US. To the benefit of their residents, nurses and aides adjust very effectively to these different and dynamic care needs day by day and shift by shift.
Third, the single most important strategy for care planning for residents with HD is to provide a daily schedule that offers the comfort of consistency and protects them from disruptions or surprises that may serve to annoy them. There is a great reassuring power in a predictable daily
routine for residents with all neurologic disorders.

Fourth, the residents' rights delineated under OBRA and the MDS Careplanning
process required of all skilled facilities by HCFA offers careplanning formats that, if followed in letter and spirit, assure all nursing home residents, those with HD among them, adequate care based on their individual needs. It is very rare that the unique needs of a resident with HD cannot be accommodated within their purview.

Fifth, the indictment of Carol Carr and the death of her two affected sons was a profound tragedy for all those interested in long-term care. The legal process needs to further unfold before presuming that the care in that nursing home was inadequate. I am certain that so profound a tragedy has
multiple contributing factors. The fact remains that most families touched by HD are satisfied (at least) with the care that nursing homes provide their loved ones.

It would be yet another tragedy presented by this horrible disease to mystify its care by unwittingly suggesting that those affected require specialized settings with additional staff.

Jim Pollard
Huntington's Disease Program Director
Laurel Lake Center for Health and Rehabilitation
620 Laurel Street
Lee, Ma 01238