(Data for 1980-1999 ages 1-14 from National Center for Health Statistics (2002). Health United States, 2002 with Chartbook on Trends in the Health of Americans. Table 36; Data for 1990-1999 ages 15-19 from Federal Interagency Forum on Child and Family Statistics. America’s Children Key National Indicators of Well-Being, 2002. Washington, DC; Data for ages 15-19 for 1999 from Hogert DL, Arias E, Smith BL, Murphy SL, Kochanek KD. “Deaths: Final Data for 1999.” National Vital Statistics Reports 49(8), Hyattsville, Md: National Center for Health Statistics, 2002; Data for ages 15-19 for 2000 from Minino AM, Arias E, Kochanek KD, Murphy SL, Smith BL. “Deaths: Final Data from 2000.” National Vital Statistics Reports 50(15), Hyattsville, Md: National Center for Health Statistics, 2002; Data for 2000 from Arias E, Smith BL. “Deaths: Preliminary Data for 2001.” National Vital Statistics Reports 51(5), Hyattsville, Md: National Center for Health Statistics, 2003, Table 1; Data for 2001 from Arias E, Anderson R, Kung H, Murphy SL, and Kochanek KD. (2003). “Deaths: Final Data from 2001.” National Vital Statistics Reports 52(3), Hyattsville, Md: National Center for Health Statistics, Tables 3 and 4. Estimates for ages 5-14 total and gender are based on Childs Trends calculations using July 1, 2001, population estimates based on the 2000 census. Race population estimates for ages 5-14 are based on population estimates from National Center for Health Statistics; Data for 2002 from Kochanek KD, Murphy SL, Anderson RN, Scott C. “Deaths: Final Data for 2002.” National Vital Statistics Reports 53(5), Hyattsville, Md: National Center for Health Statistics, Table 4. Estimates for ages 5-14 are based on Childs Trends calculations using July 1, 2002, population estimates based on the 2000 census, as presented in Table 1 in the same report. Data for 2003 from Hogert DL, Heron MP, Murphy SL, Kung H. (2006). “Deaths: Final Data for 2003.” National Vital Statistics Reports 54(13), Hyattsville, Md: National Center for Health Statistics.)

Fig. 7-2 A, Deaths of children, ages 1-4, by state. B, Under age 5 deaths, (by country, per 100,000).

(Data adapted from Field MJ and Behrman RE, eds. “Patterns of Childhood Deaths in America,” Institute of Medicine of the National Academy of Sciences, the National Academy of Press:2003, pp 41-71.)

A 2003 Institute of Medicine report ³ estimates that there are approximately 55,000 deaths of children younger than 18 years per year, compared to more than 2 million adult deaths per year in the United States. Approximately, 51 percent of those children die in the first year of life (34 percent in the neonatal period and 17 percent from one month to 12 months of age), 10 percent from 1 year to 4 years of age, 14 percent from 5 years of age to 14 years, and 25 percent from 15 to 19 years of age. In addition there is a large group of young adults (ages 20 to 24), who succumb to chronic debilitating pediatric diseases.

The causes of death vary and are age-dependent: birth defects, low birth weight, maternal complications, respiratory distress, and sepsis account for the majority of deaths in the first year of life. Unintentional injuries, homicide, suicide, cancer, heart disease, and sudden infant death syndrome account for more than 51 percent of pediatric deaths. There still remains a large category of other causes, accounting for 18 percent of pediatric deaths.³

Conditions appropriate for pediatric palliative care fall into one of the following groups:

1. Children with diseases, such as cancer or complex congenital heart disease, in which attempts at cure may fail.

2. Children who have life-threatening diseases, such as cystic fibrosis and HIV, for whom treatment may significantly extend life.

3. Children with progressive diseases, such as spinal muscular atrophies, metabolic and genetic disorders, for whom palliative care may be the only treatment.

4. Children who have severe non-progressive neurologic disabilities, such as cerebral palsy, that may result in premature death from recurrent respiratory difficulties or sepsis.

5. Previously healthy children who have a sudden and totally unanticipated death, such as trauma, severe infections, or an adverse event in the hospital.⁴^–⁶

The Hospital

As children live for longer periods, the need for complex services also escalates.^3,⁶^–⁹ In addition to multiple visits to sub-specialty clinics, many of these children have prolonged and repeated hospital admissions. Whatever the nature and duration of the admission, hospitalization exerts extraordinary psychological demands. The child’s physical distress, and the parents’ witnessing of that distress, is compounded by the implications of the illness that necessitate hospitalization, and by the separation from normal life and from other family members, especially siblings or other children.¹⁰ Counterbalancing these stressors, particularly for children with illnesses who have required frequent and prolonged admissions, is the sense that the hospital clinicians become a second family who understand and share in the family’s experiences. Hospitals are a place where many children with life-threatening illnesses and families live, often for prolonged periods.

The Intensive Care Unit

The majority of children (more than 56 percent) die in hospitals and more than 85 percent of these deaths occur in the ICU.^3,⁷ These ICU settings include neonatal (NICU), pediatric (PICU) and more recently created cardiovascular (CVICU) units. Exceptions include tertiary children’s hospitals with end-of-life programs that work collaboratively with community resources.¹¹ A retrospective analysis of deaths in a Canadian tertiary care children’s hospital over a 21/2 year period studied children who were hospitalized for at least 24 hours prior to their deaths and those who were hospitalized for at least 7 days prior to their deaths.¹² Acutely ill children who were previously healthy were excluded. Demographic data included age, gender, primary diagnosis, location of death, pain and symptom management, communication at end of life including CODE status, family preference for location of death, and the child’s involvement. Of the 236 deaths, only 86 met study criteria. Neonates accounted for 56 percent of the deaths; in 8, death was unexpected. The ICU saw 83 percent of the deaths and 78 percent of those children were intubated at the time of death. More than half, 57 percent, were medically paralyzed and 3 were on extra-corporeal membrane oxygenation (ECMO). Opioids were administered, mostly by continuous infusion, in 84 percent of the children for pain, dyspnea, discomfort related to mechanical ventilation, or post-operative pain. Acetaminophen, non-steroidal anti-inflammatory drugs and complementary medications were also used, as well as non-pharmacologic therapy, including relaxation and imagery.

A retrospective analysis reviewed the hospital care required for 9000 children and young adults with complex chronic conditions (CCCs) during their last year.⁸ Children with these conditions accounted for one-fourth of all pediatric deaths. More than 84 percent were hospitalized at death and 50 percent received mechanical ventilation during their terminal admission. Neonates who were less than 7 days of age spent 92 percent of their lives in the hospital; those aged 7 to 28 days spent 85 percent; and infants between one month and 1 year of age spent 41 percent of their lives in the hospital. In the non-neonates, 55 percent were hospitalized at death, with 19 percent mechanically ventilated. The rate of hospital use increased overall as death approached.

This study ⁸ also found variability as to whether children’s code and resuscitation status had been addressed. Discussion about code status was documented only for 79 percent of patients. Results demonstrated that multiple discussions with families were required regarding resuscitation and an actual Do Not Resuscitate (DNR) order. Once a DNR decision was obtained, the majority of children died within one day, although some children lived as long as 30 days. In charts without documentation of DNR status, cardiopulmonary resuscitation was initiated but was unsuccessful.

The mode of death in a number of PICUs has been well described.¹³^–¹⁸ A decision to forgo life-sustaining treatments was made in only 20 to 55 percent of critically ill children who eventually died. Diverse cultural, religious, philosophical, legal, and professional attitudes often affect these decisions. Despite the children’s grave prognoses, CPR was initiated and had a failure rate that varied from 16 percent to as high as 73 percent. Full life support at the time of death varied from 18 to 55 percent of the patients. A DNR order was found in less than 25 percent of the patient’s charts. Withdrawal or limitation of life-sustaining therapy, including extubation, occurred in 43 percent of the patients. More than 90 percent of these children died within 24 hours; the rest died on the second day.

The Intensivist’s Role

The intensivist must engage the family in ongoing discussions about end-of-life issues and repeatedly address and evaluate the goals of care.⁵ This role continues long after decisions to forgo life-sustaining treatments have occurred, as families look to the attending intensivist throughout the dying process. The ICU team assures the family that the child’s care is undertaken with a focus on quality of life, dignity, and comfort.^19,²⁰ The ICU nurses play a central role; at the bedside, families often call upon them for explanations of the child’s care and the existing options as well for emotional sharing.²¹ This is also where the social workers, psychologists, child life specialists, and chaplains of the interdisciplinary team can play pivotal roles. Studies have shown that when the ICU staff spends time conversing and providing bereavement resources, the families experience less stress, anxiety, and depression after the child’s death.²²

Decision-Making in the ICU Setting

The decision-making process is often extremely difficult and may require many meetings and interdisciplinary care conferences with the family. The process may be divided into three steps: deliberation, eventually leading to the decision and goal setting; implementation; and evaluation of the decision and its application.²³^–²⁵ During the deliberation, the family’s decision makers and the clinical teams must weigh the benefits and burdens of various options in terms of survival, long-term outcomes, and quality of life for the child. The concept of shared decision making allows for a consensus to be reached by all active participants once the pertinent information has been shared. It should enable the family to make a truly informed consent.

Cardio-pulmonary resuscitation

The use of cardio-pulmonary resuscitation (CPR) has had enormous impact on children’s care. Unfortunately, providing CPR for both acutely and terminally ill patients is not as beneficial as had been hoped, because less than 27 percent of children who arrest in the hospital survive to discharge. Some advocate CPR should be reserved only for those children with truly reversible medical conditions.²⁶ Nonetheless, with the use of more invasive procedures in critical care, more children may be resuscitated onto an ECMO circuit following a cardio-pulmonary arrest. This intervention may indeed improve survival, but is associated with the high risk of neurologic injury.^27,²⁸ Clinicians should inform families about the risks and benefits of CPR. It is also crucial that the family be reassured that a Do Not Resuscitate order does not mean the cessation of care for the child. Rather, it indicates a shift in priorities, toward the implementation of more comfort interventions for the child.²⁹^–³¹

Withholding or withdrawing life-sustaining support

The decision to withhold or withdraw life-sustaining treatment is always difficult. While they are considered to be ethically equivalent, physicians seem to be more comfortable with withholding; withdrawing may increase a sense of responsibility for a patient’s death. The minimizing and eventual withdrawal of technology increases opportunities for the family to hold the child. Parents may hold and cuddle their child, or lie in bed and comfort the child during this transitional period. The process of withdrawal of technological intervention requires a team effort and an environment that is supportive for the family. A room that provides privacy for the family, while also shielding other patients and families in the intensive care unit, is beneficial.^5,^13,^32,³³

Compassionate extubation

Families’ questions about what to expect when a terminal, or compassionate, extubation is planned revolve around whether the child will feel pain or suffer, be conscious, or breathe. Liberal use of sedatives, analgesics, and anxiolytics should prevent most negative symptoms. Families must be prepared for different scenarios that could unfold; however, no sure predictions can nor should be made regarding when their child will die. Certain children will die shortly after extubation, including those who are brain dead, in severe refractory shock, or in severe respiratory failure with refractory hypercarbia and hypoxia. Children who are not brain dead may linger for hours, days or longer, with variable respiratory patterns. If the parents or nurses perceive the child to be uncomfortable, then opioids and/or benzodiazepines should be given; for children who are difficult to sedate, propofol or pentobarbital may be used. Pain and suffering should be treated aggressively, even if this results in a foreseen unintended hastening of death. This principle is referred to as the “doctrine of double effect.”³³^–³⁵

Withholding or withdrawing medically provided nutrition and hydration

Although not a frequent issue in the ICU, pediatricians often have a difficult time with this aspect of terminal care and benefit from team discussion. Arguments that have been made for continued hydration and nutritional support are that children have a remarkable ability to recover, infants need assistance with feedings, and very little is known about whether or not a child at the end of life willfully refuses food and water. The American Academy of Pediatrics concluded in 2009 that the withdrawal of medically administered fluids and nutrition for pediatric patients is ethically acceptable in certain circumstances.¹⁹

Autopsy

Although it is often difficult to request an autopsy, it may provide the parents and clinicians with more information about the child’s illness and death. An autopsy may be all-inclusive or limited to those organs most involved in the disease process. Many clinicians have found the request for autopsy to be more successful if the subject is broached during end-of-life discussions with the family before the child has died. Parents may be too distraught to consent if they are approached for the first time in the immediate aftermath of the child’s death. When an autopsy is performed, it is imperative that one of the physicians who cared for the child review the results with the parents when they feel emotionally ready.³⁶ In addition to providing information, this meeting provides a valuable opportunity for follow-up with the family. If the death is a coroner’s case, then the coroner will make the determination about the post-mortem exam and the potential for organ and tissue donation.

Organ and tissue donation

Most ICUs work closely with an Organ Procurement Organization (OPO), whose professionals are trained to counsel families. In the past, brain death criteria had to be met in order to be a donor. Now, all hospitals must have a Donation after Cardiac Death (DCD) policy. This allows organs to be donated after life-sustaining interventions are discontinued, even when criteria for brain death have not been met. In these instances, it is anticipated that the patient will have a cardiac death within 2 hours in the operating room, and then have the appropriate organs retrieved for transplantation. Although the procedure is designed to increase the number of organs available, it is a cumbersome process and involves many additional steps and significant waiting times. If the patient does not sustain a cardiac death in the requisite time for donation, the child must be returned to the ICU or another in-patient area for ongoing care.³²^–³⁴

Emergency Medical Services

Unintentional injury remains the leading cause of mortality among children age 1 to 19 years, accounting for 44 percent of deaths.^40,⁴¹ Thus, from an epidemiologic perspective, the dominant trajectory of childhood death is sudden and unexpected.⁴² An estimated 20 percent of childhood deaths actually occur in the emergency medical services (EMS) environment.^43,⁴⁴ Myriad pediatric palliative care issues face clinicians in the EMS, a setting where all trajectories of pediatric life-threatening conditions may intersect. As a result of biomedical, procedural and technologic advances, many children with complications of prematurity, congenital malformations, complex chronic, and life-threatening diseases are surviving longer and living in community-based settings such as sub-acute care facilities or at home. Physiologic exacerbation of an underlying disease state, intermittent infection risk and/or mechanical complications of technology dependence all require immediate attention, and present a spectrum of palliative care challenges to staff. This section will focus on sudden and unexpected death in childhood, and elucidate:

• The organization of emergency medical services for children (EMSC) in the United States as it relates to palliative care,

• The incorporation of a family-centered approach with the family present during end-of-life care for the child in the emergency department.

Organization of emergency medical services for children

EMSC are organized around the premise that childhood is a relatively healthy time of life and that the death of a child is a relatively rare event. The overwhelming majority of children who present for emergency care are initially seen close to home at community hospital emergency departments (EDs). Only 3 percent of seriously ill or injured children initially present to pediatric tertiary, critical care, or trauma centers.^45,⁴⁶ More so than for adults, EMSC are highly regionalized, with scarce subspecialty and multidisciplinary services strategically concentrated in a hub-and-spoke wheel model in population centers. The more extensive the anatomic or physiologic derangement and the higher the clinical acuity, the greater is the need for care of the child to flow centrally to the hub. Especially for life- or limb-threatening conditions of sudden and unexpected onset, the goal is to get the right patient to the right place in the right time. This often necessitates bypassing local community resources to access more sophisticated levels of care. The EMSC system is an integrated continuum of care model, such that patient care flows seamlessly from the local community response through pre-hospital transport and on to hospital-based definitive care (Fig. 7-3). Implementation of this model necessitates a minimum level of expertise and readiness by multiple professionals, including basic emergency medical technicians, paramedics, nurses, and physicians at every step to ensure that timely and appropriate care is rendered.

Fig. 7-3 The emergency medical services for children (EMSC) continuum of care. Seriously ill and injured children interface with many health care personnel as they move through the EMSC system.

Among the unique challenges of such an approach are:

• Separation of a child from the family in order to expeditiously accomplish transport, such as air medevac from the scene or inter-facility transfer to definitive care at a distant referral center,

• Families are displaced from existing support systems and clinicians with whom they are familiar. A high likelihood of the absence of pre-existing clinical relationships with patients and their families is germane to most scenarios in the EMSC environment,

• Extraordinary, non-systematic behaviors and interventions on the part of clinicians, including the provision of futile care, as part of a family-centered approach to the dying child in the ED.

The following hypothetical example illustrates a typical system response pattern, as well as the complex responsibility for managing sudden and unexpected death that often occurs in EDs of tertiary care centers. These challenging circumstances notwithstanding, end-of-life care principles must be systematically incorporated into the EMSC model.

ND is a 6-year-old girl with a neurodegenerative illness. Her disease is characterized by a slow deterioration punctuated by acute complications such as aspiration pneumonia. ND lives at home with her family in a midsized community where her pediatrician, the rescue squad and emergency department personnel are all familiar with her clinical baseline and the nuances of her condition, including her inability to mount a fever in response to a brewing inflammatory process. Of late, ND has become less mobile and disruption of skin integrity secondary to pressure lesions increases her risk of infection. One evening, ND’s parents note decreased alertness and an unusual flushed appearance. They call 911 and are transported, as they have numerous times before, to the local community hospital ED. This time, however, the staff immediately recognizes that ND’s bounding pulses and worsening skin perfusion signal a problem beyond their capabilities and will require critical care intervention to manage her evolving septic shock. As the local team struggles to stabilize ND, a helicopter is dispatched to transport her to the tertiary care pediatric facility 50 miles away in another state. The family is instructed to begin driving to the definitive care facility, as they will be unable to accompany their daughter in the helicopter. ND is placed aboard the helicopter in clinical extremis. Although experienced, the flight paramedics have never cared for a patient with her underlying condition, complicated by uncompensated shock and impending vascular collapse. Upon arrival at the tertiary care center, ND is immediately taken to the resuscitation bay. After several minutes it is evident that despite extraordinary measures there will be no return of spontaneous circulation; she has succumbed to overwhelming sepsis. However, the code team leader, aware that the family is still en route, orders now-futile resuscitative efforts to continue. The team also begins to plan how best to receive and support the inbound family.

Family-centered care

A growing body of evidence attests to the benefits of family-centered care in the ED. Such an approach focuses on effective, culturally sensitive communication and close interaction with parents.⁴⁷ A number of institutions have formally incorporated practices for parental presence during invasive procedures, even when a child is in full arrest and requires resuscitation in a code room or trauma bay. Family presence involves their attendance in a location that affords visual or physical contact with the patient. This topic has generated strong debate. The most compelling and frequently cited argument against family presence is based on surveys that presented a hypothetical situation. Respondents thought that the event might be so traumatic that families could lose emotional control, and as a result compromise the functioning of the medical team and the care of the child. Another issue that emerged is providers’ fears that family presence could intensify the risk of litigation, unsettle clinicians such that their technical skills would decline, and infringe upon the patient’s confidentiality and privacy rights. However, no evidence exists to support any of those arguments.⁴⁸ Thus the time-honored practice of banning families from the bedside during resuscitation and other emergency procedures appears to be grounded in tradition, rather than in evidence of beneficial or adverse outcomes.

For the purpose of illustration, the hypothetical case presented in the preceding section is repeated here with an alternate ending:

ND is a 6-year-old girl with a neurodegenerative illness. Her disease is characterized by a slow deterioration punctuated by acute complications such as aspiration pneumonia. Each successive bout has been more difficult to clear, requiring multiple courses of antibiotics and aggressive pulmonary toilet. Despite the occasional need for supplemental oxygen at night, ND has never required mechanical ventilation. The family understands that were she ever to be intubated, she would never be able to be extubated. However, advance directives are not in place. After an unusually stubborn bout of pneumonia, ND appeared to be on the road back to her clinical baseline, when she acutely developed severe respiratory distress. Her family rushed her to the emergency department where, based on her clinical appearance, she was triaged directly to a resuscitation bay. By all objective measures, including oxygen desaturation via pulse oximetry and rising carbon dioxide on arterial blood gas, she was in impending respiratory failure and in need of endotrachael intubation and mechanical ventilation. ND’s parents were initially directed to a designated private waiting area near the resuscitation area. A social worker from the ED accompanied them as their liaison and clinical interpreter. As the personnel, equipment and medications were gathered to perform the intubation procedure, the social worker brought ND’s parents into the resuscitation bay. They were positioned just outside the perimeter of the activity surrounding their daughter. They were able to touch her and she acknowledged their presence through eye contact. The lead physician briefly explained what they would see as their daughter underwent rapid sequence administration of sedative and paralytic agents prior to the intubation. As the procedure progressed, including two unsuccessful attempts interspersed by bag-valve-mask ventilation, the social worker provided ongoing commentary about what was being done. The interaction among the medical team members was uncensored and seemingly unaffected by the parental presence. Despite full knowledge that this was likely the last time they would see their daughter without life support dependence, the parents expressed gratitude at being present during the procedure. ND’s deterioration had advanced to the point where she could no longer support her own ventilation and after a short period of being intubated in the intensive care unit, she died comfortably and peacefully with family present.

Invasive, technical procedures typically accompany resuscitation and stabilization of critically ill children in the emergency care setting. It is crucial that the team be clinically transparent, especially in circumstances where the child’s death may be imminent. While family presence certainly should not be undertaken lightly, responsible, evidence-based approaches and protocols will offer confidence for clinicians and parents alike in the future.

The Community

While sitting at a particularly lengthy and detailed family meeting, a nurse was struck by the family’s lack of focus, as if they had checked out of the discussion. When she asked them, “What do you wish for your child?” their response was straightforward: to take her home and make her comfortable.

Transitioning the child from the acute care setting to the community involves challenges not only for the child and family, but also for the clinical team and the institution. The decision to leave the hospital and receive care at home usually reflects a shift in treatment from life-prolonging therapy to comfort measures and quality of life.¹ This change requires advanced planning and coordination of care amongst many stakeholders: the family, the hospital team, the hospice home care team, and the third-party payer. It is essential that one member of the team be identified as a coordinator, that a social worker or case manager participate in the discussion, and that a well-organized plan for care and communication be in place.⁴⁹ Additionally, in finalizing the discharge plans, it is extremely beneficial to have representation from the community-based agency and, when possible, the third-party payer. The effort to include these stakeholders upfront is well worth the end result of smooth coordination. The team must advocate that:

• The family have the appropriate services to support the child’s needs,

• The goals and wishes of the end-of-life care plan are clearly communicated,

• The healthcare benefits can be maximized.

The team must facilitate conversation with the family to dispel their fears and misconceptions about hospice, particularly their sense of taking their child home to die and giving up hope.¹ While their child may ultimately die at home, families need reassurance that the focus is to maximize the quality of their child’s life and provide all possible comfort measures. They also need to know that returning to the hospital is always an option. This entails having a plan for a seamless transition back to the hospital should that become preferable or advisable.

The fact that most children still die in the hospital is related to many factors. On an affirmative note, the comfort that many families feel with the hospital staff is often a reason for choosing the hospital as the setting for a child’s end-of-life care and death. Other reasons include:

• A completely unanticipated death such that the child is admitted to the hospital and the family has not had the opportunity to plan for end-of-life care until very late in the disease,

• The family was never referred to hospice because the hospital staff was not knowledgeable about the community’s options,

• The parents have the perception that the hospice agency has inadequate pediatric expertise,

• Socioeconomic factors can play a role in the decision to have the child die in the hospital, including that a family living in poverty may not have a house that is comfortable or equipped for adequate care for the child,

• Psychological and cultural beliefs also contribute to the decision.

Home-Based Hospice Services

In the United States, the most common setting for providing palliative care to children in the community occurs through hospice-based home-care programs. The majority of children die in hospitals, except where tertiary children’s hospitals have palliative care programs that work collaboratively with community resources.¹¹ Although some adult hospices and programs have an interest and willingness to care for pediatric patients, adult-focused programs and staff are typically unprepared to respond to infrequent pediatric referrals and also lack connections to pediatric providers to assist them in providing safe, appropriate hospice care.⁵⁰

Adult hospices vary in the amount of experience staff has had with children.¹ One study indicated that of the palliative and hospice programs in the United States that admit children, 40 percent care for an average of 3 or fewer children per year.⁵¹ The Children’s Project on Palliative/Hospice Services (ChiPPS) conducted a survey of the more than 3,000 hospice programs in the United States in 2001. These results showed that only 450, or 15 percent, of those hospices surveyed indicated that they were prepared to offer hospice services to children.⁵² In a 2005 report from The National Hospice and Palliative Care Organization (NHPCO), it is noted that of the 4,100 hospice and palliative care programs in the United States that year, only about 738, or 18 percent, provided any pediatric services.⁵³

Many physicians are unaware of or uncomfortable with the specialized services that pediatric hospice and palliative care programs can provide and thus are reluctant to make referrals.^54,⁵⁵ Inadequate training in pediatric palliative care⁵⁶^–⁵⁹ can in turn have clinical ramifications in the children and may not receive services that would enhance their quality of life. Parents whose child had died at home with palliative or hospice care reported a calm and peaceful last month of life.⁵⁸

There are practical steps to facilitate the process of transitioning the child to the home. Acquiring information about the hospice home care agencies in the family’s area is essential prior to the transition (Box 7-1, 7-2).

BOX 7-1 Evaluating Home Hospice Agencies

Carroll JM, Torkildson C, Winsness JS. Issues related to providing quality pediatric palliative care in the community. Pediatr Clin North Am. 2007 Oct;54(5):813-827, xiii.

Information to obtain from home hospice agencies when considering referral of a child

• What experience has the agency had in pediatric palliative care?

• If the agency has had experience with children and their families, what did they learn about working with this patient population?

• Does the agency have personnel that are trained in pediatric end-of-life care?

• Will attendants from the agency be available to meet with the child, family and staff prior to discharge from the hospital?

• What is the agency’s capacity to provide continuing bereavement support for the family?

• Does the agency have a provision for respite care for the caregivers?

BOX 7-2 Guidelines for Coordinating Community-Based Palliative and Hospice Services

The following are guidelines for coordinating community-based palliative and hospice services for the child and family:

• Meet with the family to identify their goals and wishes for their child’s care, including provision of antibiotics, oxygen, nutrition and hydration, resuscitation, and blood transfusions as well as other treatment modalities. A form such as the Physician Orders for Life-Sustaining Treatment (POLST), at www.caPOLST.org, can serve as a valuable basis for discussion and explanation of options. Furthermore, the family can bring the form with them across settings so that their wishes are communicated with clarity and consistency.

• Verify insurance plans and benefits. Identify the available provider network for home hospice agencies and respite facilities.

• Clarify the treatment plan with the third-party payer in order to assure both authorization and reimbursement of services.

• Make a referral to the home hospice agency best able to meet the child’s and family’s needs.

• Provide a detailed treatment plan, including medications for comfort in emergency situations at the end of life.

Free-Standing Palliative and Respite Care Facilities

There are only two facilities that offer inpatient palliative and end-of-life care services for children and their families: the George Mark Children’s House in San Leandro, California and Exceptional Care for Children in Newark, Delaware. Both of these programs also offer respite support.

The concept of respite care ⁵⁹ is to provide temporary relief or rest to the caregivers of the seriously ill child, either in or outside the home, whether for hours or weeks at a time. Respite care is designed to give parents and other caregivers the opportunity to recharge their batteries so that they can better manage the challenges of caring for the child. Respite care may be offered voluntarily or on a fee-for-service basis, as a benefit with some insurance plans. A case manager or care coordinator can assist the family in accessing this essential service.

School

Appreciating the context of the child’s life, understanding what makes it meaningful, and respecting the relationships that are central to it are essential.⁶⁰ For the child who wishes to attend school, many crucial issues must be considered in preparation. Does the school have a nurse or other designated personnel available to accommodate the child’s medical needs? Is there a need for an out-of-hospital DNR order? What is the plan for managing issues in the child’s care that might arise during the school day? What is the responsibility of the administration to the other students regarding the possibility of a child dying at school? Frequently parents decide that it is easier to have their child home-schooled rather than deal with these many and often controversial issues. However, under the Education for All Handicapped Children Act (Public Law 94-142), all children are guaranteed the right to be in the classroom. The question of “What is in the best interest of the individual child?” must be balanced against the interests of others in the situation, such as the school community.

In 2000, the American Academy of Pediatrics (AAP) Committees on Bioethics and School Health issued the policy statement that “parents have a legal right to forego cardiopulmonary resuscitation and to ask the school to respect that decision.” Rushton ⁶⁰ says DNR orders are simply an extension of the right of families to make decisions about medical treatment or non-treatment. However, school officials argue that medically untrained staff may misinterpret a DNR order, thereby doing more harm than good for the child. Administrators are concerned that failing to act could make the school and/or school district liable.

The DNR order is usually honored without reservation in the hospital or hospice setting, but is rarely supported for children in school or other community settings. Although 43 out of 50 states and the District of Columbia have enacted out-of-hospital DNR orders, only five would extend legal protection to school personnel for honoring such decisions.⁶¹ Only 17 of these states and D.C. permit advance healthcare decisions for minors. These ill-defined laws obviously create problems for parents and legal guardians of children who choose to attend school.

Hospital teams and community agencies can collaborate with the school for the child’s care. Establishing a relationship with the school team early and sorting out dilemmas prior to the child’s return to class will assure a better outcome. The school team should be included in discharge planning discussions, particularly about an out-of-hospital DNR. In anticipation of a life-threatening event occurring at school, the parents, school administrators, and clinicians, in conjunction with the local EMS system, need to delineate a plan that would invoke comfort care for the child, rather than life-sustaining measures. The school nurse is a crucial part of this collaborative effort to assure that the best interests of the child are upheld. A proactive, coordinated plan that is communicated to the appropriate school personnel can go a long way in relieving their anxiety.

Reimbursement Issues

The reasons for woeful treatment of dying children and grieving families is due in large part to the healthcare system’s inability to recognize the need for palliative care even when children are receiving curative care.⁶²

Reimbursement issues are a major obstacle to children and families receiving palliative care services for the following reasons:

• Criteria used to determine reimbursement for pediatric palliative care are only partly applicable to children,

• Eligibility restrictions related to life expectancy frequently make it difficult for families to choose palliative care for their children,

• Different systems of payments between the government and private insurers affect both reimbursement and the types of services provided.

A significant barrier is that state Medicaid hospice benefits are based on the federal Medicare model. Hospice eligibility criteria restrict care to individuals who will die within six months. Furthermore, concurrent treatment to prolong life cannot be pursued while receiving palliative care. Additionally, within the 2010 regulations and reimbursement structure, families risk the loss of benefits such as dietary supplements or skilled home nursing care if they accept hospice. These criteria prevent families and clinicians from integrating both aspects of care.⁶³ It is also important to note that private insurers model their hospice benefits along Medicare guidelines. There are multiple payers and financial sources for pediatric palliative care, versus one payer source, Medicare, for adult palliative care.

The Institute of Medicine report ¹ states: “Approximately two-thirds of children are covered by employment-based or other private health insurance; about one-fifth are covered by state Medicaid or other public programs, but some 14 to 15 percent of children under age 19 have no health insurance.” Some of these uninsured children receive services based on safety-net providers, grants, or private donations. However, there remain children who do not receive necessary services. The IOM report further states that “for insured children and families, coverage limitations, provider payment methods and rules, and administrative practices can discourage timely and full communication between clinicians and families and restrict access to effective palliative and end-of-life care.”

The IOM report’s recommendations are based on the premise that the hospice benefit be restructured to better meet the needs of children. These recommendations include:

• Elimination of eligibility restrictions related to life expectancy,

• Elimination of the requirement to discontinue all curative treatment,

• Addition of an outlier payment category for children whose care is unusually costly.

The IOM also warns that the hospice healthcare delivery model for adults and children in 2010 can create “incentives for under treatment, overtreatment, inappropriate transitions between settings of care, inadequate coordination of care, and poor overall quality of care.”³

In an effort to counterbalance the inequities created by the Medicare hospice model, Children’s Hospice International (CHI) developed the Program for All-Inclusive Care for Children (PACC) and their Families. This program provides an alternative to the existing barriers regarding referral and reimbursement to obtaining palliative care for children. The PACC model is an innovative program, which provides access to care for all children diagnosed with life-threatening conditions. It allows for reimbursement by all payers including private insurance, workplace coverage, managed care, and Medicaid. CHI PACC permits states to receive federal reimbursement for more coordinated services than are usually provided under Medicaid.⁶⁴

Congressional appropriations through the Centers for Medicare and Medicaid Services (CMS) initially funded pilot programs in Colorado, Florida, Kentucky, New York, New England, Utah, and Virginia.^64,⁶⁵ Florida and Colorado have been fully approved for CMS waivers.⁶⁵^–⁶⁷ Florida received its waiver approval in July 2005 for a 5-year, statewide demonstration project of the PACC program.⁶⁶ In January 2007, Colorado received approval of their request for a CMS waiver. They will implement a 3-year renewable demonstration project for pediatric palliative care.⁶⁷ Based on legislation mandated in the fall of 2006, California began a 3-year pilot program in 2009 under the Children’s Hospice and Palliative Care waiver.⁶⁸ Other states interested in implementing CHI PACC and are submitting requests for waivers include Arkansas, Illinois, Louisiana, Maryland, New Mexico, Pennsylvania, Ohio, Tennessee, Texas, Utah, West Virginia, and the District of Columbia.⁶⁴ The myriad issues that surround reimbursement for palliative and end-of-life care, both for adults and children, are trigger topics for challenge and reform.