At 7 months of age, Blake (Figure 5-1) was diagnosed with a rare, progressive malignancy. His initial admission was prompted by swelling of his left lower extremity, and imaging studies revealed an extra-renal rhabdoid tumor. His family experienced a rapid indoctrination into the world of childhood cancer. Blake had a central line placed and chemotherapy begun; his parents faced the accompanying challenges of work absence, needs of their other child, the responsibilities of communicating complicated information to supportive communities, and the fear of losing their infant son. Six weeks later, Blake was admitted for emesis and the detection of brain metastases lent new gravity to his prognosis. A ventriculoperitoneal shunt was placed and bone marrow studies performed. The Palliative Care service met Blake, his parents, and 4 year-old sister shortly thereafter. Blake’s family sought support in managing anticipatory grief, supporting and preparing his sister, decision-making discussions, end-of-life planning, as well as in planning for life after his death. The Palliative Care service also provided symptom control consultation for Blake’s disease- related pain and agitation. Music and art therapies were helpful for diversion and expression of difficult emotions, particularly for Blake’s sister. Blake received palliative chemotherapy which was temporarily quite efficacious and resulted in marked clinical improvement. He would play, smile, engage those around him, and grant his family an unexpected gift of treasured time. Between admissions for chemotherapy, his family was supported by local hospice, as well as the on-call oncology and palliative care staff. Upon admission for a fourth round of high-dose chemotherapy it became evident that the hydration therapy necessary for chemotherapy administration would worsen Blake’s already tenuous respiratory status. His parents, who had clearly expressed that they desired only therapies that would be beneficial for Blake, wished to discontinue chemotherapy. Supportive measures, including opioid treatment for pain and dyspnea, and benzodiazepine infusion for anxiety and dyspnea, were titrated to address clinical symptoms. Team members supported the family through this decision-making process, and offered discussions regarding common occurrences at the end of life. His parents elected to have Blake’s end of life in the hospital setting as they felt most comfortable with the resources there. He died peacefully in the company of his family. The Palliative Care team continues to provide bereavement support.
FIGURE 5-1
Blake was diagnosed with an extra-renal rhabdoid tumor. Blake appears healthy in his home at an early stage of his disease. His parents elected to have Blake’s end of life in the hospital setting as they felt most comfortable with the resources there. He died peacefully in the company of his family. The Palliative Care team remains involved. (Used with permission from Blake’s family.)
Pediatric palliative care (PPC) is comprehensive, interdisciplinary, compassionate, inclusive, adaptive and effective. Ideally, it begins at the time a child or adolescent is diagnosed with a life-threatening illness (including prenatally) whether or not the etiology is completely understood. It comprises a community of professionals trained in medical, spiritual and psychosocial arenas. PPC provides support during the unpredictable course of an illness, attends to needs at the time of death, and supports grieving persons for the years leading up to and following the death of a child.
According to National Summary of Vital Statistics data, there were 2,437,163 deaths in the US during the year 2009.1 Of these deaths, 48,033 were among persons under the age of 19 years.
It is difficult to compare statistics involving pediatric deaths, as the ages of inclusion often vary. Some studies include children and adolescents up to 19 years of age, while others include participants up to 24 years of age.2
Overall, deaths among children are decreasing in number in the US and worldwide.3,4
Over half of all pediatric deaths occur in the first year of life.3
Antepartum losses (not included in the above statistics) occur at a rate of more than one million per year. In 2008, the most recent year for which data is available, 1,118,000 pregnancies ended in pregnancy loss. Over one million pregnancies in the same year were electively terminated.5
The US infant mortality rate (IMR), or total number of deaths of all children under the age of one year divided by the number of live births, was 6.39 in 2009.1 Preliminary 2010 data predict a decrease of IMR to 6.14.6
IMR, while decreasing in the US, continues to be markedly higher among black and Hispanic populations. IMR among whites was 5.30 for the same year, while non-whites had a rate of 10.02. The highest IMR was among black families, at a rate of 12.64.6
The US has higher IMRs than Hong Kong (1.7), Ireland (3.3), Netherlands (3.8), the United Kingdom (4.7), or Croatia (5.3).3
Causes of death in the US pediatric population vary with age group.
Congenital malformations and chromosomal abnormalities are the most frequent cause of death among infants.
Among children greater than one year of age, the most common cause of death is unintentional injury.
Malignant neoplasm is a common cause of pediatric death, particularly for children under 14 years of age, and represents the most common cause of disease-related death.
Homicide and suicide are the second and third leading causes of death in adolescents aged 15 to 19 years, respectively.
Internationally, the WHO reports that:
Twelve million children under the age of 5 years died in 1990. In 2011, deaths in this age group declined to fewer than 7 million, a decrease of 41 percent.7
Half of these deaths occurred in India, Nigeria, Pakistan, China, and the Democratic Republic of the Congo.
Rates of death of children under 5 years of age have steadily decreased since 1990, by about 2.5 percent per year. The decline is associated with increasing disparity, as American and Western Pacific countries have much greater decline than African and Southeast Asian countries.
Worldwide, leading causes of death decreasing order are undernutrition, pneumonia, preterm birth complications, diarrhea, and malaria.7
Most recent WHO information regarding adolescent mortality is from 2004. In that year, 2.6 million persons aged 10 to 24 died worldwide, with common causes including maternal conditions (15%), traffic accidents (14% in males and 5% in females), violence (12% males), and suicide (6%).4
A 2012 report on international adolescent health observed maternal mortality as a significant contributor to adolescent mortality. Remarkable variation in mortality rates from causes such as suicide, traffic injury and violence—even among high-income nations—was also noted.8
Location of death has been shown to be important to parents of children dying with cancer Parents who have the opportunity to choose are more prepared for and comfortable with their child’s end of life.9
Of children who die in the US, most die in the hospital. While the precise number of children who die at home in the US is not known, studies of children with complex chronic diseases (CCCs) have shown the number of home deaths to be increasing in that population.10 A Canadian study of children receiving PPC, in an area offering pediatric hospice facility services, showed an even distribution of number of deaths among hospital, pediatric hospice facility and home.11
In a study of Florida children who died between 2003 and 2006, 65 percent died at home. Among hospice enrollees included in the study, 55 percent died at home; 15 percent of children not enrolled in hospice died at home.12
The same study showed minority populations enrolling in hospice at reduced rates when compared to whites. Authors proposed distrust, cultural insensitivity, or lack of staff diversity as causes.12 In addition, home deaths among black and Hispanic children with chronic illness were found to be significantly fewer than those among white children.10
Persons under the age of 24 accounted for 0.4 percent of the 963,000 hospice admissions in 2009.2 A Michigan survey of pediatric providers with experience in end-of-life care cited medical and non-medical support, preparation for death, care coordination and dignified death as benefits of hospice care. Detractors were a sense of intrusion, loss of hope, and distrust.13
A 2007 survey of a small number of hospice agencies showed that 78 percent of hospice agencies care for children. Over 30 percent have pediatric programs and more than 20 percent have specialized staff members who care exclusively for children.2 An upcoming survey conducted by the Center to Advance Palliative Care (CAPC) will detail more specifically PPC services that are available in hospitals.
It is estimated that 100,000 parents suffer the death of a child each year. Such grief has been shown to be more intense and of longer duration than that experienced when mourning the loss of a parent or spouse. Approximately 19 percent of people in the US have been affected by the loss of a child in their nuclear or extended family.14
As mentioned previously, etiologies of pediatric death vary with age group.
Prematurity and low birth weight (LBW).
Following a 16-year trend of increasing frequency, the rate of preterm birth (<37 weeks gestation) in the US declined from 2007 to 2009.1
LBW incidence has not declined, likely as a result of an increase in multiple compared to singleton births, early obstetric intervention, advanced maternal age and increased utilization of treatments for infertility.1
Chromosomal abnormalities and congenital malformations.
According to the March of Dimes, the most common serious birth defects are congenital heart defects, neural tube defects such as anencephaly and encephalocele, and hemoglobin disorders such as sickle cell disease and thalassemia. The US experienced a 46 percent decline in birth defect-related mortality between the years of 1980 and 2001. Such declines have been observed in other nations with similar income levels; 95 percent of the 3.3 million annual deaths due to birth defects occur in middle or low-income nations.15
Most deaths due to congenital heart disease occur in the first year of life or after the age of 18 years.16 Congenital heart malformations resulted in a death rate among infants of 38.8 annually.1 Between the years 1999 and 2006 there was a decrease in infant mortality due to congenital heart disease attributed to improved surgical and catheter-based interventions.16
Mortality due to sickle cell disease has decreased a result of identification with newborn screening and the routine administration of pneumococcal vaccine and penicillin prophylaxis.17
Neural tube defects resulted in a death rate of 5.9/100,000 live births in 2009. The rate decreased after 1998, when guidelines mandating fortification of foods with folic acid were instituted.18
Unintentional injury.
Among persons between the ages of 1 and 19 years, the death rate due to unintentional injury declined 29 percent between the years 2000 and 2009.19
Infant deaths from unintentional injury increased, primarily as a result of the increase in incidence of deaths due to suffocation. In 2009, 907 infants died as a result of suffocation.19
SIDS, or Sudden Infant Death Syndrome, is the most common cause of sudden unexpected infant death (SUID). SIDS occurs in a child under one year of age and investigation including autopsy does not reveal an etiology.20 Over 2000 children died from SIDS in 2009, a decrease of more than 50 percent since 1990.20 It is believed that safe sleeping recommendations such as supine positioning have resulted in this decrease, as well as identification of tobacco smoke exposure, bed sharing, overheating, prematurity and LBW, soft sleeping surfaces, and loose bedding as risk factors.21 In addition, accurate diagnosis of other causes such as suffocation, have caused a decline in the incidence of SIDS.22
Poisonings among 15- to 19-year-olds nearly doubled between 2000 and 2009, associated with an increase in prescription drug misuse and deaths associated with prescription drugs.19
Increased seat belt and child safety seat usage, decreases in alcohol impaired driving, improved vehicle and road design and licensing requirements are credited with a 41 percent decline in traffic-related deaths since 2000. Despite this, traffic-related deaths remain the most frequent cause of death due to unintentional injury for persons aged 5 to 19 years.19
Males and American Indian/Alaskan Natives are more likely to die from unintentional injuries and great variation in death rates exists between states.19
Deaths due to unintentional injuries occur more often in the US than in countries of similar economic status.19
Assault
In 2010, the Department of Health and Human Services reported 1,537 childhood deaths as a result of maltreatment such as neglect, physical, or sexual abuse. The majority of children who died as a result of maltreatment suffered multiple types of abuse. Almost 80 percent of deaths occurred in children under the age of 4 years, with 40 percent of these in children under one year of age. Over 12 percent of families had been previously referred to Child Protective Services. Parents are most commonly the perpetrators.23
Deaths from homicide in 10 to 24 year-olds occurred at a rate of 14.1 per 100,000 in 1990. In 2009 the rate for 15-24 year-olds was 11.3 per 100,000. Males are more likely than females to commit or become a victim of homicide. Over 80 percent of homicides involve a firearm and deaths due to homicide are most common among non-Hispanic black adolescents.24
Suicide
Suicide occurred at a rate of 10.1 per 100,000 15 to 24 year-olds in 2009.1 More suicides are attempted by women than men, and 7 percent of high school students had attempted suicide in 2007. On the other hand, more suicide completions occur in men (24). Approximately 4,600 adolescents and young adults die of self-inflicted harm each year.25
Risk factors for suicide include a history of substance abuse, suicide of a family member, child maltreatment, physical or mental illness, isolation, and access to means of committing an act of self-harm.
Protective factors include support from family and care providers, problem-solving skills, and cultural or religious beliefs that preclude suicide.26
Malignant neoplasms
Surveillance, Epidemiology, and End Results (SEER) data from 1975 to 2006 show the incidence of childhood cancer to be increasing, most prominently in acute lymphoblastic leukemia (ALL). Brain tumor incidence has stabilized since increasing in the 1980s.27
Prognosis is more favorable with malignancies such as ALL, NHL, Wilm’s tumor, non-CNS germ cell tumors, and Hodgkin’s lymphoma than for solid tumors such as Ewing sarcoma, rhabdomyosarcoma, or osteosarcoma.27
Overall, the survival rates for childhood cancers have been increasing for the last forty years. Of children diagnosed with a pediatric malignancy, 80 percent will survive for at least five years from the time of diagnosis.28 This represents a 50 percent decline in death rates from childhood malignancy between 1975 and 2006.27 These advances are attributed to the increased use of combination and multimodal therapies, enhanced knowledge regarding molecular underpinnings of malignancy, and the customization of treatment regimens including advances in imaging and neurosurgical techniques.29
With increased survival comes the reality of a new population of persons living with long-term effects of cancer treatment. Cancer survivors have an increased risk of death from cancer-related illness for as long as 30 years after diagnosis and an increased risk of death from illnesses not related to their primary oncologic diagnosis.28
Infants typically have poorer outcomes due to inadequate therapy response, increased treatment-related morbidity, and therapy limitations due to known adverse effects.29
Despite advances, most cancer deaths are due to leukemias, followed by malignancies of the central nervous system. Total deaths from childhood cancers in 2006 numbered 2,035.27
Decreased survival rates can result from unfavorable genetic features and cancer subtypes, delayed detection and decreased likelihood of receiving therapy in accordance with a pediatric treatment regimen.29
Multiple studies have described ethnic disparity in cancer survival. Proposed explanations include differences in access, genetic predisposition to unfavorable subtypes, tendency toward poor therapy response, co-morbidities, and inadequate monitoring.29,30
Complex Chronic Conditions (CCCs)
Some studies have distinguished characteristics of deaths among children with chronic illness from those of children without health issues. The death rate among children with CCCs, such as cancer, metabolic disorders and neuromuscular disorders, is double that of age-matched peers without disease. At the same time, the number of children with CCCs appears to be increasing, largely as a result of improved technology and supportive care.10
Factors predictive of pediatric death have not yet been determined. Scoring tools such as PRISM, Pediatric Risk of Mortality Score, have accurately predicted death in some populations.31 The tool accounts for factors such as mechanical ventilation, parenteral nutrition, vasoactive medication dependence, presence of hospital-acquired infection, and duration of hospitalization.
A recent study of new admissions to six US palliative care programs included children with genetic/congenital (40.8%), neuromuscular (39.2%), oncologic (19.8%), respiratory 12.8%), and gastrointestinal (10.7) diagnoses32 (Figure 5-2).
Criteria developed in the United Kingdom by the Association for Children with Life-threatening or Terminal Conditions and their Families characterize children with life-limiting conditions into four groups by prognosis and treatment availability. See Table 5-1 for descriptions of the categories created and clinical examples of each. Such categorization illustrates that patients and families will likely utilize palliative care services in different ways over the trajectory of their diseases and grieving processes and that each family has needs unique to the experience.33
CAPC has developed assessment tools to identify adult or pediatric patients and families who would benefit from palliative care services.34,35
As illness trajectory is uncertain, particularly in pediatrics, it is essential that families are introduced to PPC at the time of diagnosis. Furthermore, early establishment of a relationship gives parents and patients access to resources for symptom management, financial and practical concerns, bereavement, decision-making and spiritual support. These services should be offered alongside curative efforts.
Unfortunately, many children do not receive PPC until curative efforts are ceased, or until they are very near death.36 Barriers such as uncertain prognosis, language barriers and time constraints, as well as inability of a family to accept illness as incurable have been identified. Limited staff resources, insufficient knowledge of palliative care and absence of staff members trained in palliative care have also been identified as barriers.37 However, it has been proposed that situations of diagnostic and prognostic uncertainty benefit greatly from early palliative care support, precisely because the disease trajectory is unknown. In addition, many illnesses are of unknown etiology and additional knowledge does not change treatment course.38
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