Abstract
Abstract
For most families, the preferred location of death for their child is home, yet most children still die in the hospital. Many children with life-threatening and life-limiting illness are medically dependent on technology, and palliative transport can serve as a bridge from the intensive care unit to the family's home to achieve family-centered goals of care. Palliative transport may also present an opportunity to prioritize cultural care and rituals at end of life which cannot be provided in the hospital. We describe a case series of pediatric patients from communities espousing markedly diverse cross-cultural values and limited financial resources. Specific cultural considerations at end of life for these children included optimizing the presence of the shared community or tribe, the centrality of healing rituals, and varied attitudes toward withdrawal of life-sustaining medical treatment. By addressing each of these components, we were able to coordinate palliative transport to enhance cross-cultural care and meaning at end of life for children with life-limiting illness.
Introduction
T
Pediatric palliative transport, using ground or air ambulances, serves as a bridge for these children to die outside of a hospital setting. According to Nelson, pediatric palliative transport is “the medical transport to home or inpatient hospice of children with levels of life-sustaining support demanding a critical care transport team, with the expectation of death within minutes to days after the cessation of that support.” 6
As patients approach end of life in the hospital, medical providers have a number of priorities to address, including code status, symptom management, organ donation, and autopsy preference. With these demanding considerations, providers often overlook culturally specific care at end of life. By not addressing culturally specific care, we miss opportunities of engagement with families to truly uphold their value-driven goals.
Pediatric palliative transport has been rarely described in children and families with diverse socioeconomic and cultural backgrounds. We describe a case series of patients from communities espousing markedly diverse cross-cultural values and limited financial resources. Each case required extensive PPC coordination with multidisciplinary teams, transport services, and home hospice to provide family-centered, community-centered, and culturally centered care at the end of life.
Case Descriptions
Case 1
An Amish newborn girl presented to a quarternary pediatric center with seizures and multiple organ dysfunction. Echocardiogram confirmed hypoplastic left heart syndrome (HLHS). Over the next week, the patient stabilized on prostaglandins and antiepileptics. Liver and kidney function improved. The family learned about proposed open-heart surgical staged repair for HLHS. Given the history of multiple organ dysfunction and seizures, this patient presented increased risk of postsurgical life-threatening complications and death. The PPC service consulted to establish goals of care. The family expressed grave concern regarding their child's potential neurologic impairment, multiple proposed surgeries, anticipated suffering, and chronic hospitalizations in the setting of critical congenital heart disease.
For this child, the family's goals of care considered quality of life weighed against prognostic uncertainty, while also deliberating the anticipated long-term hospitalization given the community's limited resources to care for a child with complex illness. While they accept modern medicine, guided by their religious beliefs of separation from the modern world, the Amish choose to live in settings without technology. Furthermore, many Amish are self-pay clients, relying on pooled community resources instead of state-based insurance. 7 In consulting with community elders, this family expressed their faith-informed, community-oriented values consistent with optimizing quality of life over longevity, understanding that their baby would die without ongoing intensive interventions.
As a family-oriented society, the Amish “depend on community integrity for survival.” 8 Embracing this priority, the parents hoped to bring their daughter home to be with her 11 siblings and surrounded by their Amish community. PPC identified resources supporting medical transport home, including referral to a local hospice agency, identifying medical staff necessary for the transport, securing equipment for transport and home, and preparing medications to ensure comfort during transport. The family opted to travel the 400 miles by ground transportation, as air travel was not consistent with their Amish values. The Amish typically travel by horse and buggy, although are open to hiring “English” drivers for longer trips or buses for out-of-state travel. 9 The baby was transported by the neonatal transport team. She was compassionately extubated and the prostaglandins and central line were discontinued. Care was seamlessly transitioned to the local home hospice that provided comfort measures at end of life. She died in her parents' arms with her siblings at her side.
Case 2
A three-month-old Hutterite boy presented with hypotonia, severe respiratory insufficiency, and poor feeding. His magnetic resonance imaging showed widespread diffusion restriction abnormalities involving the corticospinal tracts and brainstem, a pattern suggestive of a metabolic cytopathy. He did not tolerate several trials of extubation, culminating each time with acute metabolic crisis. Given his dependence on endotracheal intubation and mechanical ventilation, the ICU service presented the indication for tracheostomy to the family.
PPC consulted to support the family's goals of care and explored the family's concern around diagnostic and prognostic uncertainty. The family lived in a Hutterite community of ∼100 people and shared communal dining, working, and religious spaces. The patient's parents described the interconnectedness of their community and the importance that their son returns to this setting. Hutterites are Anabaptists similar in ethnoreligious beliefs to Amish and Mennonites. They embrace a community-centered self-sustaining social and spiritual culture. 10
The parents outlined their goals for their son to live as long as possible, without dependence on a mechanical ventilator or chronic inpatient medical care. With whole exome sequencing and mitochondrial testing pending, the family expressed discomfort with making end-of-life decisions without a definitive diagnosis. Following multidisciplinary consultation, the patient's parents expressed understanding that regardless of a specific diagnosis, imaging, laboratory, and clinical evidence, all presented the overwhelming likelihood of a severely debilitating and life-limiting illness. They shared their hope to transition their son home for comfort measures at end of life, with the support of their community.
A symptom management plan was created for the interstate transport covering 300 miles by ground ambulance, physician orders for life-sustaining treatment forms were completed for each state traveled, and contingency plans were outlined consistent with each state's laws in the event of death in transport given acute threat of metabolic deterioration.
Upon arrival home, he was compassionately extubated, and local home hospice assumed care. He died in the presence of his family and community later that day. Genetic testing later confirmed Leigh's Syndrome, an autosomal recessive mitochondrial disorder characterized by failure to thrive, respiratory failure, hypotonia, and psychomotor disability.
Case 3
An eight-month-old Native American girl from a rural tribal reservation presented with acute decompensated heart failure secondary to congenital cardiac defects, including atrioventricular canal with right ventricle dominance, double outlet right ventricle, and pulmonary stenosis status-post Blalock-Taussig shunt. Given her complex single ventricle physiology with chronic cyanosis and a shunt-dependent circulation with a dilated and poorly functional systemic ventricle, she was not a candidate for cardiac surgery on her native heart. She was listed for heart transplant, but continued to clinically worsen requiring mechanical ventilation and inotropic support. Given the acute deterioration, with PPC consultation, the girl's family shared understanding of her life-limiting clinical trajectory and defined goals to prioritize her transition home with comfort measures at end of life.
Finding comfort in their Native American heritage, the parents stressed the importance of specific tribal rituals at end of life that could not be performed in the hospital. Before palliative transport, the family requested sage, an herb for healing and cleansing, to be placed by the patient. They also requested a peace pipe for a ritual to connect the spiritual and physical worlds. Following these rituals, the patient was transported over 600 miles by air ambulance and ground transportation across state lines. As hospice was not available in the patient's home area, a physician accompanied her on the transport, performed the extubation in the patient's home, and provided comfort medications. Shortly after extubation, she died within the presence of her family and members of the tribe.
Discussion
Each of the families presented in this case series expressed goals of care to prioritize their child's end-of-life care at home in the presence of extended family and supports, while providing microculture-specific customs and rituals representative of their community's values. The families' worldviews and faith claims presented unique value systems and resource considerations. The interdisciplinary team considered socioeconomic and cultural characteristics that informed shared decision making with the families and shaped palliative transport and end-of-life care to meet those needs. The following four cultural considerations emerged from the cases highlighted above.
Community
In the Anabaptist tradition, shared by both the Amish and the Hutterites, the concept of family is broadened, including not only parents and children but also more distant relatives, elders, and community members. A person exists primarily as part of a moral community, and the welfare of the community ranks above individual rights and choices. 10 In this worldview, health care decisions not only consider what is best for the individual but also what is best for the community. The Amish are concerned with how disease impacts the entire family or group, not just individuals. In our first two cases, the parents discussed medical decisions with the elders of their respective communities, acknowledging the relationship between their child's welfare and the community's welfare. A core belief of the Native American culture is that all things live in relationship to one another. 11 In the third case, this respect for the importance of community drove the family's decision to be home in the midst of that community at a time of vulnerability and sacredness. With this emphasis on community, it is not surprising that the families preferred transport home to be immediately supported by their entire community at the time of death. 7 The medical teams recognized this priority and supported the family at this critical time by offering and arranging palliative transport home.
Resources
As there is great variation among Native American tribes, individual assessments with each family should be done to determine potential significance of resources in the face of serious illness. The Amish and Hutterites, both from the Anabaptist tradition, pool resources in an effort to support one another. With their commitments to personal responsibility and distance from the world, the Amish practice mutual aid in lieu of commercial and government insurance. In this system, the community holds responsibility for medical expenses, spreading the costs among church members through “Church Aid” or “Amish Aid.” 12 The burden of medical costs is both shared and felt by the entire community, thereby factoring into all medical decisions. It should be noted that such decisions are not plain economics, but part and parcel of what drives the community and their decision making. Similarly, the Hutterites embrace communal living—each colony sharing joint ownership of their collective assets. 10 In our conversations with the Amish and Hutterite families, high medical burdens were balanced with quality of life. With poor neurodevelopmental prognoses and the cost of ongoing intensive care, each family transitioned to comfort-directed care, and pediatric palliative transport allowed them to do that in the midst of their community.
Ritual
For Native Americans, traditional healing practices strengthen their relationships with four realms: spirituality, community, environment, and self. 13 These traditional healing practices, including ceremonies, use of herbal remedies, and contact with specialized traditional healers, are an important aspect of American Indian culture. “Today Native Americans frequently combine traditional healing practices with allopathic medicine to promote health and well-being.” 11 So, healing cannot come from Western medicine alone and must include spiritual healing practices. For the Amish, after the death of a loved one, the body of the deceased is washed and dressed in white, and a viewing happens shortly after death in the family's home. 14 In an effort to build a trusted relationship with our patients, our team asked specifically about any spiritual or religious considerations. Palliative transport facilitated the family's participation in important rituals in the presence of their communities.
Technology
Both the Amish and Hutterites accept modern medical practices and the medical technology that comes with it. Despite their common roots, the Amish and Hutterites view technology in their daily lives differently. Most Amish live without technology. If they do incorporate aspects of modern technology, they must first perceive a greater benefit than potential threat to their community practice and ideals. 12 For example, the family in our first case declined air transport and opted for ground transport instead. The Hutterites, on the other hand, have a more progressive orientation toward technology. They accept most up-to-date forms of technology, although emphasizing the importance of controlling use so as not to disrupt their cultural patterns. 10 For Native American communities, although a deviation from ancestral ways, technology is accepted as consistent with a commitment to adaptation and change in many Indian belief systems. 15 In our three cases, the families would have been open to mechanical ventilation from a technology standpoint, but ultimately prioritized quality of life over quantity of life. Palliative transport allowed a peaceful death at home, away from the advanced technology of an ICU setting.
This case series considers interdisciplinary care coordination and effective exploration of goals of care for three different families from three different cultures. By asking if there is anything we should know about the patient's cultural or religious practices, we learned important values and priorities that allowed us to provide enhanced cross-cultural care. Furthermore, this invitation appreciates that religious and community belief systems may influence any value-driven goal. With this opening, we were able to explore the importance of community, community resources, healing rituals, and perspectives on technology to provide culturally sensitive care for these families. All of these components led to well-coordinated palliative transport to allow for enhanced cross-cultural care and meaning at end of life for children with severe life-limiting illness. Pediatric palliative transport should be considered in the end-of-life paradigm for children in the ICU, especially those from microcultures and remote communities.
Footnotes
Author Disclosure Statement
No competing financial interests exist.