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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.
DISEASE STAGES
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.
STAGE I
- Emotional difficulties, including irritability, depression, anxiety
- Cognitive problems, including forgetfulness, errors in judgment
- Infrequent involuntary movements
STAGE II
- Diminished adaptability and ability to learn
- Frequent and severe involuntary movements
- Difficulties with job performance and personal financial management
STAGE III
- 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
STAGE IV
- 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
clients.
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.
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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
relationships
- 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.
RESOURCES
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: hdsainfo@orgNOTEWORTHY FEATURES OF HD CENTERS OF CARE
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.
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