Key points

Introduction

Palliative care is patient and family-centered care that optimizes quality of life by anticipating, preventing, and treating suffering. The initiation of palliative care increasingly begins at diagnosis and continues throughout the illness trajectory. Pediatric palliative care (PPC) is provided to children ranging in age from prenatal to young adult or older when receiving treatment of a pediatric diagnosis. Most PPC is provided in a hospital setting; however, community-based PPC (CBPPC) programs are an integral component of assuring access to PPC for children with life-threatening illnesses when provided along with curative or life-prolonging treatment.

Children are dynamic beings who are learning, growing, and developing along a continuum that leads to self-awareness, self-advocacy, and competencies that enable the youngster to become a functional adult. Seriously ill children arrive to PPC at a point along that continuum of development and are very different from adults with serious illnesses. Children have not yet learned to cope with adversity, identified sources of support during a crisis, nor can they answer the question “Who am I?” Children may have encountered serious illness since birth, gradually entered into serious illness, or been thrust suddenly into the chaos. In any case, the focus quickly becomes the child’s diagnosis and related challenges; however, that does not halt the child’s movement along the developmental continuum as she or he continues to grow, change, and learn about herself or himself and the world. Throughout even the most serious of illnesses, the child continues to be a child and possesses all of the developmental, social, emotional, and spiritual needs of a child. To be effective, PPC must address the child’s needs on a developmentally appropriate level for that individual child and provide services in the places where the child lives, learns, grows, and develops. Typically, that place is not the hospital. Rather, it is the child’s natural environments of home, school, worship, play, friends, and other interactive activities.

Currently, there is a zeitgeist in our culture that presents an opportunity to change the way PPC is provided to children. Two generations ago, children with serious diagnoses lived in institutions, received minimal medical care, and did not attend school. For example, children with muscular dystrophy, cystic fibrosis, or spina bifida lived only into adolescence, whereas those with cerebral palsy or other neurologic or developmental disabilities were often institutionalized. Today, many of those same youngsters are living into their 20s, 30s, and beyond. Medications, ventriculoperitoneal shunts, central venous access lines, gastrostomy tubes, and ventilators have increased the life expectancy of children with neuromuscular disorders. Care in neonatal intensive care units has improved survival of premature infants and infants with chronic heart or lung conditions, genetic disorders, or birth defects. As children with serious illnesses are often living longer, schools, athletic teams, places of worship, and other venues are developing an understanding of the needs of these children. Thus, participating in school, athletics, and worship are viable options.

With increased survival come unavoidable consequences of significant disability and medical, educational, psychological, social, and spiritual challenges. More children are surviving, resulting in more children who need PPC. These PPC programs should provide coordinated, efficient, and cost-effective care. Health care has opened the doors for seriously ill children to gain entry into activities. Therefore, the interdisciplinary PPC team is called on, in collaboration with adults in the child’s natural environments, to engage a broad team of providers to provide a new definition of PPC: comprehensive PPC (CPPC).

This article considers the possibility of developing strong alliances and partnerships between hospitals, CBPPC, schools, athletic teams, places of worship, and other community-based organizations. Partnerships will result in increased knowledge for all caregivers, thus enabling children to have the opportunity to address physical, psychological, social or emotional, and spiritual needs in all of the natural environments where they live, learn, grow, and develop.

Introduction

Palliative care is patient and family-centered care that optimizes quality of life by anticipating, preventing, and treating suffering. The initiation of palliative care increasingly begins at diagnosis and continues throughout the illness trajectory. Pediatric palliative care (PPC) is provided to children ranging in age from prenatal to young adult or older when receiving treatment of a pediatric diagnosis. Most PPC is provided in a hospital setting; however, community-based PPC (CBPPC) programs are an integral component of assuring access to PPC for children with life-threatening illnesses when provided along with curative or life-prolonging treatment.

Children are dynamic beings who are learning, growing, and developing along a continuum that leads to self-awareness, self-advocacy, and competencies that enable the youngster to become a functional adult. Seriously ill children arrive to PPC at a point along that continuum of development and are very different from adults with serious illnesses. Children have not yet learned to cope with adversity, identified sources of support during a crisis, nor can they answer the question “Who am I?” Children may have encountered serious illness since birth, gradually entered into serious illness, or been thrust suddenly into the chaos. In any case, the focus quickly becomes the child’s diagnosis and related challenges; however, that does not halt the child’s movement along the developmental continuum as she or he continues to grow, change, and learn about herself or himself and the world. Throughout even the most serious of illnesses, the child continues to be a child and possesses all of the developmental, social, emotional, and spiritual needs of a child. To be effective, PPC must address the child’s needs on a developmentally appropriate level for that individual child and provide services in the places where the child lives, learns, grows, and develops. Typically, that place is not the hospital. Rather, it is the child’s natural environments of home, school, worship, play, friends, and other interactive activities.

Currently, there is a zeitgeist in our culture that presents an opportunity to change the way PPC is provided to children. Two generations ago, children with serious diagnoses lived in institutions, received minimal medical care, and did not attend school. For example, children with muscular dystrophy, cystic fibrosis, or spina bifida lived only into adolescence, whereas those with cerebral palsy or other neurologic or developmental disabilities were often institutionalized. Today, many of those same youngsters are living into their 20s, 30s, and beyond. Medications, ventriculoperitoneal shunts, central venous access lines, gastrostomy tubes, and ventilators have increased the life expectancy of children with neuromuscular disorders. Care in neonatal intensive care units has improved survival of premature infants and infants with chronic heart or lung conditions, genetic disorders, or birth defects. As children with serious illnesses are often living longer, schools, athletic teams, places of worship, and other venues are developing an understanding of the needs of these children. Thus, participating in school, athletics, and worship are viable options.

With increased survival come unavoidable consequences of significant disability and medical, educational, psychological, social, and spiritual challenges. More children are surviving, resulting in more children who need PPC. These PPC programs should provide coordinated, efficient, and cost-effective care. Health care has opened the doors for seriously ill children to gain entry into activities. Therefore, the interdisciplinary PPC team is called on, in collaboration with adults in the child’s natural environments, to engage a broad team of providers to provide a new definition of PPC: comprehensive PPC (CPPC).

This article considers the possibility of developing strong alliances and partnerships between hospitals, CBPPC, schools, athletic teams, places of worship, and other community-based organizations. Partnerships will result in increased knowledge for all caregivers, thus enabling children to have the opportunity to address physical, psychological, social or emotional, and spiritual needs in all of the natural environments where they live, learn, grow, and develop.

Speaking the same language

In the interest of developing CPPC services provided across a child’s natural environments, it is imperative that all stakeholders use consistent terminology to define the children and the services they will receive. Currently, heath care providers (HCPs) use terminology that is often unfamiliar to other professions. Other stakeholders may not yet have an awareness of PPC or their role in providing it. As groups work to develop a foundation for CPPC, an initial time investment will be required to identify and teach shared terminology, as well as the essential components of CPPC as it pertains to the psychological, social or emotional, and spiritual needs of the child and family. Uniformity in key terms will ensure that individuals from each profession are operating from the same conceptual base and can work collaboratively to provide comprehensive care.

Pediatric palliative care and pediatric hospice care

A greater number of infants, children, and adolescents (collectively referred to as children for the remainder of this article) receive both PPC and pediatric hospice care (PHC) than ever before. Although the terms palliative and hospice are often used synonymously, the meanings of these 2 models of care are actually quite different. Palliative care suggests a strong focus on the child’s quality of life and may be provided to the child for months or years either as the main focus of care or, when appropriate, along with concurrent disease-modifying therapy. The focus of PPC is on quality of life and enabling the young person to live as productive a life as possible despite the existence of a serious illness.

Conversely, PHC is a program and philosophy of care for children facing a life-limiting illness or condition who have a life expectancy of months, not years, and includes expert medical care, pain management, and psychological, social, and spiritual support expressly tailored to the patient’s and family’s needs and wishes. Hospice focuses on caring, not curing.

Most definitions of PPC emphasize referral early in a child’s disease trajectory and a focus on quality of life. The World Health Organization defines palliative care as

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  The active total care of patients whose disease is not responsive to curative treatment. Control of pain, of other symptoms, and of psychological, social, and spiritual problems is paramount. The goal of palliative care is achievement of the best quality of life for patients and their families.

The American Academy of Pediatrics (AAP) definition includes the following key points:

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  PPC-PHC should be provided as collaborative integrated multimodal care, including cure-seeking, life-prolonging (when in the child’s best interest), comfort-enhancing, and quality-of-life enriching modes of care.

  
  
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  Psychological, spiritual, and social support for the family.

  
  
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  Collaboration is essential. Patient, parents, other involved extended family members and friends, schools, parental employers, and all involved members of the primary and specialty health care team must collaborate to effectively meet the needs of patients.

  
  
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  For all patients, high-quality PPC-PHC should routinely prevent and treat distressing symptoms, such as pain, nausea, or anxiety, and seek to maximize quality of life, which may entail various interventions, depending on the patient’s specific goals.

  
  
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  PPC consultations can occur throughout the child’s illness experience, including at initial diagnosis, when the goals of care are focused on cure.

  
  
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  PPC-PHC should be integrated throughout the illness course, providing interventions to support the goals of care, which often shift over time.

Attempts to describe the specific population of children who receive PPC services in the United States have been inconsistent. Ages that are included in PPC are reported with different parameters. For example, most community and hospital PPC programs serve children up to age 19 years but some hospitals continue care to age 21 years or beyond. National Hospice and Palliative Care Organization’s Standards of Practice for Pediatric Palliative/Hospice Care defines pediatrics as “for infants through young adults.” The adolescent and young adult population is defined by the National Cancer Institute as patients from 15 to 39 years of age.

Other attempts to identify eligible children focus on prognoses referring to those who will die, those who may die, those with life-limiting or life-threatening diagnoses, and children who are terminally ill. The literature reports difficulty with prognostication identifying “unrealistic physician and/or parental prognostic expectations may be leading to inappropriate treatment goals” and “We often do not know how long the patient may live or even whether he or she will survive the illness.” Thus, prognoses may not be reliable criteria for children in need of PPC.

Specific diagnoses that suggest children’s eligibility for PPC may be the method with the greatest efficacy. The Center to Advance Palliative Care offers the Pediatric Palliative Care Referral Criteria that identify diagnoses that should receive an automatic consult, as well as those diagnoses that suggest a palliative care referral. Chronic complex condition (CCC) is a broad descriptive category of specific diagnoses that identify many, if not most, children who receive PPC and sometimes PHC. The health care literature first described CCC as

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  Any medical condition that can be reasonably expected to last at least 12 months (unless death intervenes) and to involve either several different organ systems or a single system severely enough to require specialty pediatric care and probably some period of hospitalization in a tertiary care center.

The original definition of CCC identified 9 diagnostic categories, including cardiovascular, respiratory, neuromuscular, renal, gastrointestinal, hematologic or immunologic, metabolic, other congenital or genetic, and malignancy. The 2014 update added the category of premature and neonatal conditions. The update, which also addresses changes in the International Classification of Diseases , 10th revision, includes a domain of complexity arising from dependence on medical technology (devices), and a domain indicating the child has been the recipient of a transplant.

Regardless of what method of eligibility is used, some children do not ever receive a timely referral to PPC. Data from a 2001 study by the Children’s International Project on Palliative/Hospice Services estimated that on any given day 5000 children with CCCs were in the last 6 months of life and another 8600 children would be eligible for palliative care services. In addition, only 5000 of the 53,000 children who died that year received hospice services and usually only for a brief period of time. A uniform definition would likely ensure that more children in need of PPC would be identified and, thus, receive referrals for care.

Education definitions

It is anticipated that schools would be primary partners to HCPs in providing CPPC because children typically spend 7 to 8 hours a day in school. HCPs may benefit from understanding the terminology and reference point from which educators join the discussion.

In 1995, the Department of Health and Human Services Maternal and Child Health Bureau defined children with special health care needs as those

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  Who have or are at increased risk of developing a chronic physical, developmental, behavioral, or emotional condition and who also require health and related services of a type or amount beyond that usually required by childhood conditions.

This definition is commonly used in schools to describe students with serious chronic health conditions, 27% of whom have a condition or conditions that affect their activities usually, always, or a great deal.

The 2009 to 2010 National Survey of Children with Special Health Care Needs (CSHCN; Chartbook 2013) indicates that

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  Fifteen percent of children younger than 18 years of age in the United States, or more than 11 million children, have special health care needs.

  
  
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  One in 4 households with children has at least 1 child with a special health care need.

  
  
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  These health care needs include cystic fibrosis, asthma, diabetes, food allergy, sickle cell disease, juvenile idiopathic arthritis, genetic disorders, spina bifida, neuromuscular diseases, epilepsy, congenital heart diseases, trauma-related injury, and human immunodeficiency syndrome, as well as disorders of speech, language, learning, and behavior.

  
  
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  Literature regarding CSHCN frequently refers to the existence of “limitation of function or activity.”

CSHCN is consistent with criteria used by schools to identify students who have a physical or health disability significant enough to result in special education services or accommodations to enable them to access educational and extracurricular activities. There are significant similarities between the categories of CCC and CSHCN. Both definitions

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  Identify the existence of a serious chronic health condition

  
  
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  Acknowledge the child’s increased need for hospitalization or increased frequency of health care services

  
  
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  Acknowledge that the number of children in the category is increasing

  
  
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  Suggest an impact on the child’s functional ability

  
  
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  Includes children who may not have survived into childhood or adolescence if born a generation sooner.

There is a natural juncture for collaboration between health care and education in service delivery, research, resources to support the child and family, and broader advocacy and policy issues. Children with CCCs are already commonly identified as those in need of PPC and the terminology often appears in the health care literature. The similarities between CCC and CSHCN suggest the transition to using CCC may be easy for educators, whereas other collaborative partners do not routinely use this term to refer to children with serious health conditions. Therefore, CCC is used in this discussion and suggested for use in referring to the target population of children who could benefit from CPPC in all natural environments.