From 1996 to 2005, the Robert Wood Johnson Foundation (RWJF) funded over 22 demonstration projects, 337 grants, and 15 training conferences for nursing faculty through the Promoting Excellence in End-of-Life Care Initiative. As part of the initiative, workgroups of experts were convened to target specific clinical subgroups impacted by end-of-life care. Individuals with Huntington disease (HD) were targeted because, as the disease advances, they typically receive end-of-life care in institutional settings including nursing homes (NHs) or hospices (Dubinsky, 2010). Members of the HD workgroup identified 10 clinical priority areas for excellent end-of-life care: promotion of autonomy, dignity, meaningful social interaction, communication, comfort, safety and order, spirituality, enjoyment or entertainment and well-being, nutrition, and functional competence (Dubinsky, 2010).
The purpose of this article is twofold: to present demographic information of a cohort of 53 individuals with middle- to end-stage HD who received healthcare during the years 2000-2008 at a distinct part in the skilled nursing facility operated with San Diego County's Psychiatric Hospital and to describe the nursing and interdisciplinary lessons we learned in caring for them over this 8-year period. The 10 clinical priority areas identified by the HD workgroup will be used as the organizing framework for the article. We used these priority areas to focus our efforts in providing cohort members with meaningful lives and dignified deaths in an institutional setting.
HD is a progressive genetic neurodegenerative disorder that results in institutionalization of individuals in the final stages of the disease. In North America, five to eight people per 100,000 manifest the disease. An individual typically manifests symptoms of HD in his or her late 30s, although it may occur in children or the geriatric population. The typical disease trajectory is 10-25 years of progressive dementia and physical deterioration before death (Walker, 2007).
The natural history of HD is typically characterized as evolving in three stages:
Early or stage 1 HD includes subtle neurological symptoms, such as increased irritability and disorganization with early cognitive loss, personality change, subtle choretic movements, and behavioral problems among people living independently. Chorea is an abnormal involuntary movement (a type of dyskinesias) caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement. It is characterized by brief, irregular contractions that are not repetitive or rhythmic but appear to flow from one muscle to the next and may occur in the trunk, limbs, or face (Hofmann, 1999; Kilgore, 2008; Nance, 2007). Individuals in stage 1 may face employment, familial-marital, substance abuse, and legal problems.
In middle or stage 2 HD, individuals develop more choretic movements involving gait and balance, becoming increasingly at risk of falling. Even more troubling than the physical symptoms is the progressive neurocognitive decline. This is manifested by impulsive and often aberrant behaviors, personality changes, psychotic or depressive symptoms, perseverative activities or thinking patterns, restlessness and anxiety, and poor judgment. The combination of the neuropsychiatric symptoms and maintenance of a high degree of physical mobility can result in a plethora of unsafe behaviors. Individuals may be involved in alcoholic episodes, unsafe driving, drug use, unsafe smoking or cooking, suicidality or self-harm, aggression, refusal to wear clothing, disinhibited sexual behaviors, and constant pacing. These behaviors may prompt involvement of law or mental health professionals, establishment of conservatorship, or institutionalization. Individuals with HD may die of suicide, trauma, or aspiration (Hofmann, 1999; Kilgore, 2008; Nance, 2007).
End-of-life care is primarily associated with late or stage 3 HD, which, in its most advanced form, resembles Alzheimer and other late-stage dementias. An individual at this stage may be nonverbal, require assistance with all activities of daily living, and has reduced interaction with the environment. The individual may be extremely rigid or may continue with writhing, choretic movements. Although over time cognitive functioning progressively declines, comprehension of language and awareness of people in their environment are thought to be relatively well maintained. The most common cause of death in late-stage HD is likely aspiration. Table 1 displays common physical and psychiatric or behavioral symptoms and comorbidities associated with each stage of HD (Hofmann, 1999; Kilgore, 2008; Nance, 2007).
Promoting Excellence in End-of-Life Care at Edgemoor for Residents With HD
Edgemoor is a distinct part of the skilled nursing facility operated by the county of San Diego under the license of the San Diego County Psychiatric Hospital. Located in a suburb of San Diego (Santee), Edgemoor is a referral site for individuals with middle- and end-stage HD who are unable to be managed at home or in other nursing facilities. From 2000 to 2008, Edgemoor staff cared for 53 middle- to-late-stage HD patients. They ranged in age from 29 to 78 years at the time of death. Patients who died or were discharged averaged 48 years of age at admission and stayed an average of 11 years. Tables 2 and 3 display the cohort's demographic and clinical characteristics. The lessons that we learned in caring for these individuals are summarized under each of the 10 clinical priority areas identified by the RWJF HD workgroup.
The most successful and essential approach to caring for individuals with HD in the NH involved promotion of autonomy and choice. Residents tended to have strong wills and opinions about what they wanted from their care. For example, they wore clothing that was comfortable for them, although it might not conform to what others expected. For some residents, bathing consisted of a quick bed bath only when certain staff members could assist or when a special soap was available.
Nursing staff did their best to honor residents' wishes and preferences while balancing individual accommodation with our ongoing responsibility to maintain safety. Edgemoor was and continues to be a restraint-free NH. Although we did provide close observation by staff to try to prevent falls, families were told that, even with one-to-one staffing, falls could occur. In some cases, family members had guilt and anguish about the disease and high expectations about what the NH staff could do to keep their loved ones happy. We educated residents and family members about reducing the negative impact of falls. It was essential that they worked to share common expectations with the staff about the nature of the trade-offs between independence and choice versus safety that were made. For example, among residents who refused to use to padded chairs or helmets, we provided first aid for minor bruises and abrasions. We picked up residents from the floor who refused to let staff help transfer them and spent hours convincing someone to shower or turn or take medications.
Implementing consistent nursing assignment and an extensive education on HD and specific needs of residents enabled us to individualize nursing care. Staff was encouraged to familiarize themselves with residents' routines, habits, preferences, and propensities to implement plans that were tolerable and effective. We were careful to minimize assigning staff with a high need for control to HD residents; generally, these clinicians were not effective.
Members of the interdisciplinary team included specialists in recreational, occupational, and physical therapies, social work, psychology, and psychiatry, enabling us to develop individualized solutions to challenging clinical situations. The team identified individuals who continued to have strong needs for independence, and we evaluated resident and family tolerance for risk. Our approach involved building relationships with residents and family members, enabling us to positively influence behaviors and strike the best balance between safety and independence. At times, autonomy was restricted in situations where an individual's behavior posed as a significant and urgent risk of danger to self or others. For example, a resident who refused to bathe and was so dirty as to create an infection control hazard might be offered a much more limited choice (i.e., "Which one will you be having this morning, a bath or a shower?") and other forceful encouragement.
By definition all treatment for HD is palliative; no disease-altering therapy exists (Dawson, Kristijanson, Toye, & Flett, 2004; Moskowitz & Marder, 2001). The therapeutic goal was and continues to be mitigating symptoms, promoting dignity, and enhancing quality of life of individuals afflicted with HD and their family and friends. Maintaining dignity was challenging at times. Residents with HD in the cohort were relatively young and often appeared more intact cognitively than they actually were. At the same time, they required a high degree of supervision, sometimes refused care, and had behavioral problems that placed them and the staff at risk.
We suggested end-of-life choices that, on the basis of our clinical experiences, were in the best interest of the resident and family. For example, facility physicians recommended hand-feeding rather than enteral feeding; care be given at Edgemoor rather than a hospital; and not resuscitating individuals at their end of life. Most individuals (75%) and their families or legal representatives selected such supportive care.
Among the 24 residents still living, 38% want hospitalization, resuscitative efforts, and intensive-care-level interventions. Some of these individuals are mute, with almost no interaction with the environment. We continue to discuss end-of-life choices with residents and/or family members in some instances, emphasizing the benefits of "dying in place" in familiar surroundings at Edgemoor.
Meaningful Social Interactions
One of the tragic aspects of HD was that many individuals lacked friends or support from family members. Perhaps they were estranged from relatives, had small families because of the disease, or had mental illness and substance abuse problems as youngsters. Most of the HD residents living at Edgemoor appeared to be "loners." Some seemed to appreciate social interaction with interdisciplinary team members and nursing staff, fostered by consistent assignments. Some types of social interaction were restricted. For example, some more social women were vulnerable to exploitation and inappropriate social behaviors with unwanted touching or aggression from other residents.
Individuals with HD experienced significant difficulties in expressing themselves verbally. Their ability to express choices was lost, leading to frustration, aggression, depression, or acting out. Careful observation of resident behaviors and preferences and maintenance of consistent care assignments helped to mitigate the negative consequences of these communication deficits. Teaching staff, residents, and family members about HD provided an opportunity for demonstrating effective communication skills. We determined what the residents and their family knew and wanted to know, providing honest information about prognosis and options.
There were many types of suffering experienced by residents with HD and their family members. Some individuals had physical pain-either from the constant movements or secondarily to acute and chronic trauma. Some experienced delusions or hallucinations, anxiety, poor sleep or depression, or perseverative thoughts. Others anticipated what was to come, fearing that they might not only be a burden to their children now but unwittingly passed the disease onto their loved ones. They had relatively few other physical health problems to accelerate their deaths aside from having HD. Our goals included caring for their physical and spiritual needs and suffering. For physical comfort, many preferred to wear few clothes, not use linens on the bed, and had few belongings. Most preferred that their rooms were kept at a cool temperature.
Eighty-two percent of the cohort took scheduled and/or PRN (as needed) medications for pain, often Tylenol, but sometimes opioids. For relief from chorea, we used benzodiazepines (often at high doses), antipsychotic medications, and tetrabenazine. For relief from psychiatric symptoms, 79% took psychotropic medications, with 63% of these being antidepressants. For some, we provided private duty nursing and constant companionship. For others, we "left them alone" as much as possible, providing television, a low bed, and staff providing supervision that was not invasive.
Safety and Order
Maintaining safety and order while respecting the individual's autonomy was an ever-present concern, especially in individuals with middle-stage HD. Mitigation of symptoms was emphasized, with an emphasis on improving quality, not quantity, of life. The Edgemoor staff shared with families and patients our "restraint-free" philosophy and individualized approach to reducing falls while acknowledging that the risk of falls could not be eliminated.
In an attempt to maximize staffing resources and promote safety, at one point, we attempted to cohort HD residents together, both in single-bed and multi-bed rooms. It was not workable for residents or staff, except when single rooms were used. In 2009, we relocated to a newly constructed facility. We now have a few rooms configured so that visual privacy between residents is maintained while one staff can observe and assist several residents simultaneously. This arrangement resembles a family unit with a 1:3 ratio on the day shift; it worked well for a small cadre of women with HD.
Interventions focused on fall prevention included environmental modifications, mechanical alterations, therapeutic seating, and skilled therapies. Environmental interventions included padding head and footboards, walls, or corners in the rooms, altering the type or length of the call cord, removing curtains, which pose a risk of entanglement. Mechanical alternatives were used, such as antitippers or anti-roll-backs for wheelchairs and use of pommel cushions, sticky mats, Y-leg straps or seat belts, helmets, padding of knees or elbows, and heels to prevent trauma. Therapeutic seating was used, including the use of couches, Broda®-type wheelchairs or chairs with Y-leg straps (Broda® Seating, 2010), lounge, or rocking chairs. Some individuals with HD preferred to sit or sleep on the floor. An important component to fall prevention was educating other patients about HD and the need to "clear the way" when a patient was passing by.
Traditional interventions for fall prevention were also used, including maintaining a clean, clutter-free environment with adequate lighting; marking floors when they were wet or otherwise unsafe; providing call lights in bedrooms and bathrooms where residents could obtain help; placing beds in the low position, which was optimum for the patient's mobility; providing grab bars in bathrooms; using shortened rails on beds for grabbing; and low-slip sock or slippers and routine use of footwear (American Medical Directors Association, 2008).
Creating a safe and secure person-centered environment where residents, family, and staff were protected from violence and aggression was essential. To achieve and maintain this, we initiated several proactive interventions. Much violent behavior arises from emotions such as fear, confusion, anger, power struggles, or frustration. Many HD residents were young, and their lives were significantly and irreversibly changed, causing anger, frustration, and sometimes self-destructive behavior or depression. Addressing the underlying emotion and making a connection with the individual was the best way to prevent violence in the first place or to reduce escalation and injury related to violence.
We avoided admitting individuals with HD who had a history of intentional violence that was not related to a medical or psychiatric illness. Individuals who were known to irritate others (identified by peers or through frequent victimization) were monitored in social situations. Potentially violent individuals were only cared for by staff members who did not aggravate them and who were knowledgeable of the resident's propensities, triggers, and effective interventions. These included pharmacological interventions, separation from potential victims, closer monitoring, restriction of rights, or transfer to an acute psychiatric hospital.
An individualized risk assessment that focused on identifying factors that triggered aggressive behaviors amenable to modification was conducted. The assessment was done at admission, after an aggressive incident, quarterly, annually, and with any change in condition. A staff psychologist assisted in evaluating and managing high-risk individuals. A consultant psychiatrist worked with the primary physician in devising a plan of care that included medication management for potentially violent individuals.
Reactive interventions designed to minimize episodes of violence and aggression were employed. Edgemoor staff was trained to respond to violent or aggressive incidents with a first prompt response of de-escalation, protecting individuals from harm, and reporting the incident. Although we were and continue to be committed to maintaining a restraint-free care environment, there were situations in which restraint use was unavoidable. For example, in rare acute emergencies with violent patients posing a risk to themselves or others, short-term physical or chemical restraints were used with appropriate safeguards and physician orders.
High-risk residents were not taken outside the facility on outings in the community. Violent acts committed by patients were reported to law enforcement, who responded by maintaining the patient in a community placement rather than filing criminal charges.
We cared for the whole person, addressing needs of the body, mind, and spirit. Some family members exhibited spiritual suffering of guilt, regret, and sadness at passing the gene to family members. Although spiritual needs were occasionally appropriately addressed using religious providers, spiritual needs were more frequently met through constant relationships with residents, family, or staff.
Enjoyment, Entertainment, and Well-Being
Individuals with middle-stage HD often had very short attention spans and experienced difficulty in working with others. They required customized activities that were brief and that frequently changed. In general, larger group activities were not possible. Particularly effective activities included ceramics, computer use (e-mail and YouTube), and a fine arts program with painting on canvas with oils, individually or in small groups. Some residents enjoyed massages; most enjoyed being outdoors, walking or being assisted in a wheelchair. Many enjoyed television, DVD or movies, and music, which provided diversion in a more solitary setting. Obtaining outside food, the swimming program, walks, massages, and music were particularly popular. Those at later stages of HD benefited from sensory-type programs. For those who received 1:1 nursing care, therapists devised a therapeutic recreation plan that could be implemented throughout the day by the nurse if the resident so chose.
Another key component in promoting well-being was the establishment of routines. Individuals with HD tended to tolerate changes poorly, for example, changes in plans, rooms, staff, furniture, and activities. It was important to maintain familiar structures and routines.
Individuals with middle-stage HD had difficulty with changing their minds. They could become upset with a staff member or develop a perseverative idea; it was extremely difficult to change their minds. For example, one gentleman felt slighted by a nurse. He was so focused on her wrong-doing and the punishment she deserved that it went on for months and began to generalize to others who had any association with her. The only thing that ended up working was appealing to his Catholic faith to allow him to "forgive" her the transgression. Another threw himself out of bed and injured himself because he did not like the certified nursing assistant assigned to him. Another had a single bad bathing experience and, afterwards, refused baths for more than 7 years.
In the middle stages of the illness, some HD residents in the cohort were overnourished and overweight, perhaps because of medication side effects or high consumption of calories. They commonly had nutritional needs such as high caloric requirements, preference for junk-type foods, swallowing difficulties, and disinterest in eating modified diets. Many patients did need to eat in the middle of the night; PRN food worked far better than PRN psychotropics at putting a resident back to sleep. At the end stage of HD, most began to lose weight. Care goals were to offer food around the clock to meet preferences as much as possible and to feed them until they were satisfied. Experts do not recommend tube feeding for all individuals with HD, although 23% of our cohort preferred and used tube feedings (Dubinsky, 2010). Most residents preferred to eat textures of liquid and solid foods.
Individuals in the middle stages of HD were often admitted to Edgemoor because of their unsafe behaviors and the risk of harming themselves if they continued to live in the community. They still were able to do many things by themselves-such as dressing, transferring, and ambulating. Promotion of independence often came informally with the primary caregivers rather than through participation in skilled therapies. Rooms were often organized so that items were in reach and pathways were clear. Often pads were placed beside the bed. As the disease progressed, the movements became disabling and the residents lost their ability to ambulate. They used wheelchairs, Broda® (Broda® Seating, 2010) or Carefoam™ chairs (Carefoam™Chairs, 2010) for comfort and positioning.
In general, individuals with HD had difficulty benefiting from skilled occupational or physical therapy because of the progressive nature of the disease and the gait disturbances, their short attention spans, and their inability or dislike of "following directions" or "accepting influence." Very few used walkers. They did benefit from speech therapy interventions for dysarthria, dysphagia, and communication strategies and benefited from skilled assessments of seating, environmental modifications, and adaptive equipment.
The 10 clinical priority areas identified in the RWJF Promoting Excellence in End-of-Life Care Initiative provided us with an effective framework to use in caring for institutionalized individuals in the middle and end stages of HD. These individuals presented challenges to nursing staff and interdisciplinary team members because of their safety risks, high supervision requirements, and behavioral difficulties. Focusing on the 10 clinical areas identified by members of the HD workgroup helped us provide high-quality and compassionate care over an extended period in an institutional setting.
This material is based in part on work supported by the Department of Veterans Affairs, Veterans Health Administration, Office of Research and Development, Health Services Research and Development (CD2 06-015).