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Pallimed Case Conferences (cases.pallimed.org) is closed to comments and new posts as of April 25, 2013.

This site will stay online as an archived source, but will no longer be updated.

Read here about the switch-over.

For active posts on these cases and new cases go to www.pallimed.com. 

This site will stay online as an archived source, but will no longer be updated.

Read here about the switch-over.

For active posts on these cases and new cases go to www.pallimed.com. 

Pallimed Case Conferences (cases.pallimed.org) is closed to comments and new posts as of April 25, 2013.

This site will stay online as an archived source, but will no longer be updated.

Read here about the switch-over.

For active posts on these cases and new cases go to www.pallimed.com. 

Case

A 43-year-old woman, WM was diagnosed with Huntington’s Disease (HD) in September 1996. In 1997 WM stopped working due to disability related to her disease progression. Since 2004 she was living at a skilled nursing facility where her care had been stable until 4 weeks prior when her choreiform movements intensified, her oral intake declined, and she was unable to rest or sleep due to incessant body movements. Several medication changes at the nursing facility were unsuccessful and she was transferred to an inpatient hospice unit for end-of-life care. On exam the patient appeared younger than her stated age and cachectic. She was non-ambulatory. She had flailing involuntary movements of her arms and legs, which made a comprehensive physical examination difficult. Her speech was unintelligible and skin on her arms and legs showed bruises and open wounds that she sustained when she hit any furniture around her. The patient’s husband and power of attorney’s primary goal was to control her movements and allow her to rest.

Dr. Huntington via Wikipedia

Brief overview of Huntington’s disease

HD is a neurodegenerative disorder that is inherited in an autosomal dominant fashion so that each child of an affected patient has a 50% chance of developing the disease. Most patients develop symptoms in their forties and fifties. Symptoms are usually a triad of motor, cognitive, and psychiatric issues. The motor symptoms involve impairment in voluntary movements and appearance of involuntary movements called chorea (hence the name Huntington’s chorea). Impairment in voluntary movements results in loss of manual dexterity, slurred speech, swallowing difficulties, problems with balance, and falls. The motor symptoms worsen in the middle stages followed by rigidity in the late stages of the illness. Cognitive symptoms manifest in the patient’s inability to perform complex tasks with loss of speed and flexibility, progressing to global impairment in the later stages of the disease. Psychiatric symptoms most commonly include depression. Patients may also manifest irritability, anxiety, agitation, impulsivity, apathy, social withdrawal, and obsessiveness.

Treatment

Medications do not alter the progression of HD. Patients are vulnerable to cognitive side effects of pharmacologic therapy. Motor symptoms are usually managed using either neuroleptic agents such as haloperidol and/or benzodiazepines. There are no medications to treat swallowing difficulties or rigidity that occurs later in the disease. Depression is treated with serotonin specific reuptake inhibitors such as sertraline and paroxetine. The mainstay of therapy for HD remains non-pharmacologic management. Padding of furniture and weights on wrists and ankles will help reduce injuries and the amplitude of involuntary movements respectively. As swallowing difficulties progress, the food consistency can be softened and pureed. Eating is usually supervised, and the patient is given time to eat with no distractions. Caregivers should be trained in Heimlich maneuver. Cognitive impairment and difficulties in communication can be eased by giving the patient time to answer and asking closed-ended questions. Establishing routines around the patient and activities that adjust to his or her cognitive level help to decrease anxiety and allow cognitive stimulation. Identifying and avoiding triggers for emotional outbursts and providing family and caregiver supports are measures to help manage behavioral manifestations of the disease.

Survival

Average survival time after diagnosis is 15 to 20 years. Disease course is variable, and every patient is unique, which makes prognostication difficult.

Back to the Case

Initial attempts to sit the patient in a padded wheelchair and feed her proved unsuccessful. A trial of phenobarbital was initiated to allow her to rest and monitor response. All furniture was removed from her room. Several mattresses were placed on the floor and padding was applied to the walls. Over the course of 3-4 days the patient’s movements subsided, and she was able to stay awake. Her appetite improved, and she drank shakes. Feeding times were unique; one staff member would hold WM in her lap while the other would feed her. Over a period of 2 weeks, WM thrived. Although she was incontinent for bowel and bladder, she would call out to the nursing staff when she needed care. Her room was decorated with her favorite sport team jerseys and family photos. Her husband visited daily and was able to participate in feeding times, and together they watched sports events on TV. WM’s only medications were oral fluoxetine, laxatives, antacids and phenobarbital. While we initiated our treatment plan to relieve distress and attempted palliative sedation therapy as a means to relieve what appeared to be refractory symptoms at the end of life, the patient’s response was so dramatic that we now planned for her care for the next several weeks at a nursing facility. Our objective was to achieve a smooth transition for WM, and we encouraged nursing facility staff to visit the hospice unit and witness her personal care and feeding. The patient was eventually transferred and has continued to be stable at the nursing facility. She has not required any medication adjustments.

References:
1. Aubeeluck A, Wilson E. Huntington's disease. Part 1: essential background and management. Br J Nurs. 2008;17(3):146-51.
2. Rosenblatt A, Ranen NG, Nance MA, Paulsen JS. A Physician's Guide to the Management of Huntington's Disease. Second Edition. Huntington's Disease Society of America 1999.

Pallimed Case Conferences (cases.pallimed.org) is closed to comments and new posts as of April 25, 2013.

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Case:

Miss C. is a 28-year-old female with a history of a spinal cord tumor with metastasis to the brain. She was admitted to the hospital for further management of related complications. She had suffered with multiple neurological complications, including paraplegia since 1997. She had endured many procedures, surgeries, treatments, and hospitalizations prior to the Palliative Care Team’s meeting with her on this admission. Her disease had become more extensive. She had recently received chemotherapy, and developed complications likely related to this. When she arrived at the hospital, it was determined that she had suffered an intraventricular hemorrhage. For this reason, she was intubated and placed on the ventilator.

During her hospital stay, she had multiple complicated medical issues: the intraventricular hemorrhage, quadriplegia, infection, hypotension, and delirium. Communication with this patient was difficult and painstaking, but not impossible. Behavioral medicine was recruited to assist with communication efforts and help the family with coping techniques. The Palliative Care Team met with the patient and her family daily to assist them in setting the goals of care. The primary service was an integral part of the communication process. During the previous years of cancer treatments, Miss C. had developed very close bonds with this staff.

Miss C. began to express concerns about the therapies she was receiving. After long, detailed conversations with her, we recommended that some limits be placed on medical interventions such as resuscitation, and blood pressure support. The patient and family agreed. She was successfully discharged to a ventilator facility closer to her family.

Discussion


Dealing with the impending death of a patient is perhaps one of the most difficult tasks that a nurse or physician can encounter. For staff who deal with these types of issues on a day-to-day basis, it can become even more distressing. Traditionally, the intensive care units care for the most critically ill patients. Unfortunately, death becomes an inevitable part of the experience. How each staff member deals with that grief and sorrow varies greatly. It is well acknowledged that the death of a loved one under any circumstance is a tremendous loss for that family. Little is written or spoken about the grief and coping of staff who deal with dying patients on a daily basis. This case illustrates that not all patients who die in the hospital are elderly, frail patients who have lived a full life. Understandably, nurses, doctors, and staff would view this case in a different light. For many of us, we see a young, 28-year-old girl who reminds us of our wives, our sisters, our daughters, or ourselves. 28-year-old women are not supposed to die.

This case also reminds us that we cannot endure the stress and grief alone. While it is important to maintain consistent contact with our patients, we also need to know our limits. Traditionally, nurses are caretakers and nurturers. They typically “vent” about these types of cases with their colleagues and managers. It is also nice to know that there are services within our system for patients, families, and staff. Staff should know that they need to take care of themselves, too. Services like social work, behavioral medicine, palliative care, clergy, and family support services are readily available to consult with staff members about their concerns and grief.

References:
1. Reimer, J.C., Davies, B., & Martens, N. (1991) Palliative Care: The nurse’s role in helping families through the transition of “fading away.” Cancer Nursing, 14(6), 321-327. Vachon, M.L.S.,

2. The stress of professional caregivers in Doyle, D. et al eds. Oxford Textbook of Palliative Medicine, New York, Oxford University Press 1998, 919-929.

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Case:

A 67-year-old man with end-stage congestive heart failure presented to the hospital with worsening respiratory distress and was intubated in the emergency room. Despite a week of intensive efforts, he could not be weaned from the ventilator and remained dependent on intravenous dobutamine for blood pressure support. The ICU staff met with the patient and family to discuss the medical situation and options for care including withdrawal of life support. The patient did not want to continue on long-term ventilatory support nor did he want to die in the hospital. The patient expressed to the care team a strong desire to leave the hospital and to die at home.

A palliative care consultation was initiated to support the patient and family and to assist with a discharge to home. The patient, family, ICU team and palliative care team discussed potential options for discharge and decided on a plan to transport the patient home with ongoing mechanical ventilation and inotropic support. This option allowed the patient to be comfortable at home with his family before removing the ventilator and providing medications for comfort that would likely be sedating. Efforts to carry out this plan were hindered by the reluctance of home care and hospice agencies to participate in this “unusual” and expensive plan that provided little or no reimbursement (i.e. withdrawal of life-support in the home setting while the patient continued on Dobutamine).

Following hours of coordination and planning, the physician from the Palliative Care service and a nurse from the ICU rode home in the ambulance with the patient where they were met by many of the patient’s family and friends and a private duty nurse to support the patient and family. After a few hours of visiting with his loved ones, the patient indicated that he was ready for the ventilator to be withdrawn. Intravenous morphine and ativan were administered until the patient was comfortable, then the endotracheal tube was removed. Additional medication was administered intermittently to maintain adequate comfort and the patient died comfortably at home a few days later. The family expressed their gratitude that the patient’s wish to die at home was achieved.

Discussion

For many patients facing a life-threatening illness, death at home is viewed as a desirable and even essential element of a “good death.” Patients in the hospital expected to die imminently after the withdrawal of life-support do not typically have this option because of the obstacles and lack of payment incentives for coordinating this type of care. Withdrawal of life support in the home can provide patients and families with the opportunity to achieve their goal of dying in the familiar setting of their own home at a lower cost than the average cost of care in an ICU.

However, in our current care system it requires extenuating circumstances and significant coordination and planning to assure that patients and families are well supported and the patient’s comfort is maintained. When the goals of care shift for seriously ill patients from a disease-specific and curative approach to an approach that focuses on comfort, symptom management and other personal preferences for care (such as wanting to die at home), the health care system is often inadequate and discontinuous. One reason, as in our highlighted case, is that “ownership” for this type of coordination does not exist and few providers (home care and others) are willing to assume this role because most insurance programs (such as Medicare) will not pay. Since most care is reimbursed according to location, it is difficult to provide comprehensive services that meet the changing goals of patients as their disease progresses.

Public policies and financial incentives that encourage and reward hospitals, home care agencies, nursing homes and hospices for seamless care need to be established. Surely to compliment our modern care system and life prolonging technologies we should be able to develop a reliable and sustainable system that also relieves symptoms, honors preferences and supports patients and their families at the end of life.

References:
1. Lynn, J. Serving patients who may die soon and their families. JAMA 2002;285: pp. 925-932.

Pallimed Case Conferences (cases.pallimed.org) is closed to comments and new posts as of April 25, 2013.

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Case:

Ms. Z was a middle-aged Colombian woman with pulmonary hypertension and was awaiting a double lung transplant. Her presenting symptoms included extreme shortness of breath with minimal exertion, tiredness, and chest pain. A palliative care consult was requested for support and symptom management for anxiety and depression. She was also very concerned about her bills. She had trouble concentrating, was unable to watch TV or use her laptop, and was constantly tearful. Through palliative care, a consult was obtained for music therapy. In the context of music therapy, her anxious and depressive symptoms were significantly diminished by using music that was specific to her native culture.

Discussion
Music therapy has consistently been demonstrated to help alleviate symptoms of anxiety and depression, especially when the implemented music is a type of music that is preferred by the patient. In fact, a study researching the effects of subject-preferred music found that subjects who listened to their preferred music exhibited an increase in positive mood if their pre-music mood was negative. It also found that subjects listening to preferred music maintained a positive mood if the subjects’ pre-music mood was positive (Wheeler 1985).

Since Ms. Z was a Colombian immigrant, there were many times that she had family members and friends with her to both support her and translate some of the more difficult conversations that were in English. Though this author did know rudimentary Spanish, and did successfully implement short phrases, it was the music that became the common language throughout each session.

The first music therapy session was met with much enthusiasm and laughter from Ms. Z’s family and friends. Ms. Z was visibly anxious and nervous as she sat in the corner, but as she began to watch those around her enjoying themselves, her face softened and she smiled from behind her oxygen mask. The two attending interns closed the session having played some of the requested music, but with the momentous assignment to learn many more songs. Countless hours were spent learning songs by one of her favorite artists, Juanes, who is famous in Colombia and other South American and Spanish-speaking countries.

The second session was filled with a myriad of their favorite Spanish songs. They sang every song along with the attending interns, laughing and crying at the same time. Ms. Z was visibly relaxed and at peace. She continued to enjoy music therapy after she got her double lung transplant. Through the implementation of music therapy, her anxious and depressive symptoms were significantly diminished through singing, the use of percussion instruments, and by using her preference of cultural-specific music.

Through singing, she used her lungs—both before and after her double lung transplant. Even when Ms. Z was only able to whisper, she was still stretching her new lungs while “mouthing” the words she knew from memory. It distracted her from the monotony of being hospitalized, from the bills she couldn’t pay, and from her constant medical problems. It fostered a deeper and more meaningful socialization with her loved ones, and helped her to find cultural familiarity within someone else’s culture. Singing also gave her choices: she could participate or watch; she could choose which song she wanted to hear; she could choose which artist she wanted to hear; and she could choose the language with which she wanted to surround herself. It also gave her a sense of ownership and pride that the attending interns learned these songs specifically for her.

Using percussion instruments had its own benefits: it was a less threatening way to participate in the music-making experiences; it gave another series of choices for her to make; and because there was a wide array of South American instruments, it was a physical and tangible connection to her cultural heritage.

Music can help patients to appreciate the beauty and wisdom of their own cultural backgrounds and promote positive feelings within them that can be tapped into when celebrating and coping with life’s highs and lows (Chase 2003). By validating Ms. Z’s culture and bringing it into her hospitalization, music therapy fostered a sense of normalization and familiarity within the hospital walls which to her had previously represented feelings of anxiety and depression.

References:
1. Chase, Kristen M. "Multicultural Music Therapy: A Review of Literature." American Music Therapy Association Music Therapy Perspectives 21 (2003): 84-88.

2. Wheeler, Barbara L. "Relationship of Personal Characteristics to Mood and Enjoyment After Hearing Live and Recorded Music and to Musical Taste." Psychology of Music 13 (1985): 81-92.