212
Seminars in Oncology Nursing, Vol 30, No 4 (November), 2014: pp 212-226
CONTRIBUTIONS OF PALLIATIVE CARE TO PEDIATRIC PATIENT CARE CARMEN MANDAC AND VANESSA BATTISTA OBJECTIVES: To provide an overview of pediatric palliative care (PPC) as it relates to children and families living with oncologic disease.
DATA SOURCES: Journal articles, clinical research reports, clinical guidelines, and national statistics.
CONCLUSION: As new treatment protocols become available, the need for simultaneous supportive PPC, including adequate pain and symptom management, is evident. Further research and PPC program development is necessary for adherence to the current recommendation that PPC should be initiated at the time of diagnosis and continue throughout the course of a child’s disease.
IMPLICATIONS FOR NURSING PRACTICE: Palliative care nursing holds a specific role in the pediatric oncology setting. Registered nurses and advanced practice nurses should be adequately trained in PPC because they are in an optimal role to contribute to interdisciplinary PPC for pediatric oncology patients and their families.
KEY WORDS: Pediatric oncology, Pediatric palliative care, Nursing F the approximately 53,000 children that die each year, approximately 2,200 are from pediatric cancers.1 Cancer is the leading disease-related cause of death in children and adolescents ages 1 to 19,1 (referred to collectively as children for
O
the purposes of this article). In 2014 it is predicted that of the 10,450 diagnosed with cancer, 1,350 children are expected to die from their disease.2 This presents a crucial need for pediatric palliative care (PPC) within pediatric oncology, yet PPC programs are not as common as adult palliative care
Carmen Mandac, RN, MS, CPNP: Pediatric Nurse Practitioner Student, Columbia University, School of Nursing, New York, NY. Vanessa Battista, RN, MS, CPNP, CCRC: Pediatric Nurse Practitioner, The Children’s Hospital of Philadelphia, Pediatric Palliative Advanced Care Team (PACT), Philadelphia, PA. Address correspondence to Vanessa Battista, RN, MS, CPNP, CCRC, Pediatric Nurse Practitioner, The
Children’s Hospital of Philadelphia, Pediatric Palliative Advanced Care Team (PACT), 34th and Civic Center Boulevard, 11NW90, Philadelphia, PA 19104. e-mail: [email protected] Ó 2014 Elsevier Inc. All rights reserved. 0749-2081/3004-$36.00/0. http://dx.doi.org/10.1016/j.soncn.2014.08.003
CONTRIBUTIONS OF PALLIATIVE CARE TO PEDIATRIC PATIENT CARE
programs. In fact, although 58% of Children’s Oncology Group institutions (an institution that is supported by the National Cancer Institute and is devoted to pediatric cancer research that conducts clinical trials) have palliative care programs, only 6% discuss palliative care at diagnosis.3 There are several barriers that challenge palliative care delivery in oncology which are both specific and non-specific to pediatrics. Several barriers exist that prevent sufficient palliative care for children with cancer, including: relative rarity of cancer and death in children, the developmental stage of the child, a lack of evidence-based medicine to define symptoms and causes of death, difficulty in symptom management, lack of education for healthcare providers about palliative care, and reimbursement issues. All of these barriers contribute to the lack of integration of PPC in pediatric oncology. Utilization of registered nurses (RNs) and advanced practice nurses (APNs), referred to collectively as nurses for the purposes of this article, can optimize delivery of PPC within the oncology setting as they are in a unique position to bring together the interdisciplinary team caring for children and families. Semantics and assumptions have led palliative care to become synonymous with end of life (EOL), death, and hospice, and a lack of a clear definition of PPC often impedes its delivery. For this reason, it is important to define clearly PPC. Several groups, such as the World Health Organization, the American Academy of Pediatrics (AAP), and the Institute of Medicine, have each developed definitions that specifically address PPC. In summary, PPC can be defined as a philosophy of care and an organized program that focuses on total care and enhancement of quality of life of a child and family in the face of life-threatening illness.4-6 Different developmental tasks influence how children perceive and cope with illness and possible death, and a crucial aspect of PPC is the consideration of the developmental stage of the child (see Table 1).7,8 Children whose disease trajectory spans more than one developmental stage call for constant evaluation and adaptation of the delivery of support and care.1 Interventions and approaches should be informed by both the child’s chronological age and developmental stage,9 because they do not
213
always coincide. It is not unusual for children with advanced illness to either progress or regress in developmental stage. Therefore, when delivering PPC, the child should be considered as a product of their chronological age, developmental stage, medical condition, size, handicap, and cognition.9 Another barrier is the lack of distinction between PPC and hospice. The use of the terms palliative care and hospice care interchangeably in the common vernacular has led to confusion in the healthcare field. Although hospice and palliative care share a similar philosophy, they are different entities. Hospice is a part of PPC used for children at later disease stages, whereas the larger palliative care model represents an integrative approach in which care is initiated at diagnosis and delivered through the EOL. Integrative models combining curative care and palliative care enable continuity of care and facilitate a seamless transition to EOL care. It is suggested that palliative care be delivered within disease-focused medical treatment, regardless of curative goals.10
PEDIATRIC PALLIATIVE CARE IN ONCOLOGY Medical and pharmacologic advances within the last 40 years have drastically improved the survival rate in childhood cancers. Hodgkins and non-Hodgkins lymphoma, Wilms tumors, and acute lymphoblastic leukemia are now considered very curable diseases.11 Also, survival rates have increased for bone cancer, gonadal cancer, and neuroblastoma.11 Yet, despite these advances, cancer remains the overall leading cause of pediatric death second to motor vehicle accidents. Metastatic solid tumors and brain tumors continue to be difficult to treat.11 In short, it is evident that palliative interventions are necessary as suffering is universal for children with cancer and their families.10 A further examination of definitional clarity, disease trajectory, and symptom management point out the unique obstacles facing PPC delivery in pediatric oncology.
DEFINING PALLIATIVE CARE Definitional clarity affects the delivery of PPC in all fields. Although it is recommended that palliative care be offered early in the diagnosis, the various ways in which PPC is defined contributes to incomplete or inadequate interventions. For
214
C. MANDAC AND V. BATTISTA
TABLE 1. Developmental Stages and Perceptions of Death Age
Basic Conflict
Birth to 18 months
Trust vs mistrust
Early childhood (2-3 years)
Autonomy vs shame and doubt
Preschool (3-5 years)
Initiative vs guilt
School age (6-11 years)
Industry vs inferiority
Adolescence (12-18 years)
Identity vs role confusion
View of Death
Suggestions
No sense of finality and is viewed as continuous with life Reactive to stress Death is seen as reversible and not final May feel that death is a punishment May feel responsible for death
Use simple physical communication and provide comforting and nurturing care Expect regression, clinging, or aggressive behavior Encourage expression because the child may be concerned about family function after they die Use honest and clear language to explain death and dying Continue to use open communication with clear language Encourage questions about death and dying
Death continues to be understood as temporary May have a literal understanding of death and will respond with curiosity and questioning Death is understood as permanent Understands that the body does not function (heart stops beating and breathing stops) May feel responsible and guilty for the illness May have spiritual ideas about afterlife May not want to discuss feelings Understands the finality of death and may develop a mature understanding of death May try to take responsibility for adult concerns within the family (such as finances and care-taking) Feelings of anger may be present
Reassure the child that death is not their fault Aim to maintain as much structure and normal routine as possible Include the child in after life plans (funeral planning, last wishes)
Allow time for reflection Listen to concerns and questions Support efforts for autonomy and control
Data from Vern-Gross7 and Foster et al.8
example, 303 pediatricians in various areas of practice were asked to define palliative care.12 Of the pediatricians surveyed, 41.9% defined palliative care as hospice care, 31.9% gave an alternative definition, and 26.2% did not know what it was; with 49.3% making referrals to palliative care, and 29.4% unaware of community or local services,12 thereby exemplifying how lack of a universal definition impedes access to PPC. The study also reported that palliative care services are most often accessed in a manner similar to hospice
care.12 Within oncology specifically, a lack of ‘‘definitional clarity’’ relevant to palliative and supportive care exists within the literature, indicating a breakdown in knowledge dissemination regarding palliative care and thus affecting delivery and development of services and frequency of referrals.13 Because PPC has traditionally been introduced at EOL, the term has become synonymous with ‘‘giving up’’ by both medical teams and families.7 While this seems to be a ubiquitous theme
CONTRIBUTIONS OF PALLIATIVE CARE TO PEDIATRIC PATIENT CARE
throughout PPC, the focus of oncology is driven by a curative ideology. A study in which oncologists were asked to describe their experience with their most recent patient death revealed that oncologists who described their role as biomedical relayed a sense of failure when they did not have the ability to change the course of the disease.14 It is apparent that the curative biomedical perspective clashes with PPC when it is associated with termination of treatment and the death of a patient. Furthermore, death of a child has been described as a ‘‘triple failure’’ for healthcare providers because they feel that: 1) they did not have the ability to save a life, 2) they failed in their social role as adults by not being able to protect a child, and 3) they have betrayed the parents who trusted them with their child.1 Thus, the perception that palliative care and curative treatment should be delivered separately negatively affects healthcare providers and families within pediatric oncology.
DISEASE TRAJECTORY Advances in medical technology have drastically modified disease trajectories within pediatric oncology as healthcare providers are challenged with predicting outcomes for pediatric cancer patients.15 In one of the earliest studies of its kind, a retrospective analysis evaluated variables influencing EOL care in pediatric cancer. The reported median age of death was 9.3 years, with a range of 0.3 to 32 years; the median length of survival from diagnosis was 13 months, with a range of 0.5 to 100 months.16 The wide ranges indicate the marked variability in disease trajectory for pediatric cancers. Physical, mental, and spiritual/ existential changes will occur throughout the oncologic disease process and contribute to a high level of prognostic uncertainty as the rate and predictability of changes is never certain. Therefore, it is not surprising that referral to PPC services is highly influenced by uncertain disease trajectory. Referral to PPC services also varies according to disease process, as revealed by a retrospective chart review that showed the average referral time to PPC after diagnosis was 461 days, with 16% referred within 30 days of diagnosis, 26% before a relapse occurred, 40% after the first relapse, and 18% after more than two relapses.17 Although a majority of the children who died received PPC services, their referral was dictated
215
by their disease trajectory, concluding that referral to palliative care services should occur earlier in the disease.17 In a review of barriers to PPC, uncertainty about treatment response and overall chances for survival were shown to complicate a child’s plan of care.18 Early and influential research found that the level of certainty that the child would get better was the most prominent factor in the healthcare provider’s decisions about plans of care. Further research has continued to support this concept, showing that providers do not believe that referral to PPC will benefit the patient if they are not going to die of their disease.17 Furthermore, because pediatric cancer disease course has a high degree of uncertainty, goals of care can become unclear and result in dichotomous care, in which curative and palliative care are separate entities.18 This has also been termed a cure–care dichotomy that is associated with poorly controlled symptoms, chronic pain, patient and family distress, and decreased survival within pediatric oncology.19 Yet, if palliative services are reserved for only those who are dying, the opportunity to ease a substantial amount of children’s suffering will be missed. Higher parental ratings of quality of care were reported when both the physician and parent recognized there was likely no cure 50 days before a child’s death.20 Earlier discussion about EOL and integration of PPC can help alleviate suffering throughout the course of the illness.
PAIN AND SYMPTOM MANAGEMENT Children with cancer often present with complex symptom management needs and quality symptom management is a vital part of PPC, yet few reliable and valid instruments for symptom assessment in children exist.10 Some known cancer- and treatment-related symptoms experienced in children with cancer include emesis, anemia, fatigue, distress, and pain.10 These symptoms should be managed from diagnosis, supporting an integrative palliative and disease-directed approach to care; yet symptom management in pediatric oncology is not always successful. One hundred and three parents of children with cancer who died of advanced disease or treatment-related complications were interviewed after their child’s death; 89% reported that their child had experienced substantial suffering from at least one symptom and only 27% of parents felt that treatment of
216
TABLE 2. Oncologic Process Acute leukemias
CNS tumors
Description
Statistics/Survival
Complications
Relapse
ALL: 20% will relapse, 1/3 of those Treatment-related nausea, patients will have long-term vomiting, infection, pain, remission with intensified myelosuppression, therapy cardiotoxicity, peripheral AML: after relapse 80% 5-year event-free survival High-risk medulloblastoma and supratentorial PNET 5-year event-free survival is 50%-60% Pontine glioma survival is 8-9 months, with 90% dying within 2 years of diagnosis High-grade glioma: survival rate is 10%-30% Low-grade gliomas: survival rate is 90% HL and NHL account for 10%-12% Skeletal metastases HL and NHL B or T lymphocytes increase Chemotherapy-related side of pediatric malignancies rapidly or survive longer than effects, in particular nausea, NHL 5-year event-free survival in normal, eventually forming a peripheral neuropathy, children 15 years is 77% and Reed Sternberg cells. NHL cardiac toxicity (when combined HL of all stages, 5-year event-free is divided into diffuse large with radiation) survival is >80%, long-term B-cell lymphoma, lymphoblastic Varicella zoster survival is 90% lymphoma, Burkitt’s lymphoma, and anaplastic large cell lymphoma Wilms tumor Tumor of the developing kidney 6% of pediatric malignancies Abdominal pain 85% are cured of their disease Hypertension occurring between ages 1-5, most cases are sporadic with Malaise, fever Polycythemia few being familial, sometimes associated with congenital Bleeding within the tumor can lead syndromes to anemia, pallor, and fatigue Renal insufficiency Low-risk subgroups have a >90% Horner’s syndrome Neuroblastoma Malignancy of the sympathetic Spinal cord compression survival nervous system, or a disease of Anemia a neural crest tissue. Prognosis High-risk subgroups have Ataxia 30%-40% survival is varied according to age, Hypertension differentiation/biology of the Treatment-related side effects tumor, and extent of disease spread Sarcomas account for about 6% of Airway obstruction Sarcomas (soft tissue and bone) Heterogeneous group of tumors Cranial nerve complications pediatric cancers that arise in soft tissue or bone. Urinary obstruction Soft-tissue sarcoma survival Most common sarcomas Radiation-related side effects without relapse is >70% include osteosarcoma and Chemotherapy-related side Ewing sarcoma. Osteosarcoma effects arises from osteoblasts and
217
218
TABLE 2. (Continued) Description Ewing sarcoma is derived from the neural crest
Germ cell tumors
Retinoblastoma
Liver tumors
Arise from primordial cells involved in gametogenesis and occur in testes and ovaries. Originate in yolk sac endoderm and migrate along the genital ridge. Frequently occur in the sacrococcygeal region, midline of brain, mediastinum, and retroperitoneum Malignant ocular tumor, rarely diagnosed after 5 years of age, arises from the photoreceptor cells of the innermost layer of the retina
Malignancy of the liver, hepatoblastoma is the most common hepatic tumor occurring in infancy and young children. HCC and other rare tumors account for the other cases. Tumors are associated with genetic syndromes and environmental risk factors
Statistics/Survival Nonrhabdomyotamous soft tissue sarcoma with completely excised tumor survival exceeds 85%, unresectable tumors >5 cm have 50% survival Localized osteosarcoma survival is >70%, metastatic disease 2-year survival is 10%-30% 4% of pediatric cancers 5-year survival is >80%
Affects 200-300 children per year
1% of pediatric cancers metastatic disease, HCC have poor prognosis
Complications
Relapse
Limb amputation Cardiomyopathy Gonadal toxicity Secondary malignancy Renal dysfunction
Risk for secondary malignancy if Pain treated with alkylator therapy Constipation 20% will have metastatic disease Urinary obstruction Respiratory distress Gynecomastia Uterine bleeding Treatment-related proximal tubule renal dysfunction Cosmetic and functional Children with bilateral impairment retinoblastoma and unilateral Decreased visual acuity, eye retinoblastoma
Seminars in Oncology Nursing, Vol 30, No 4 (November), 2014: pp 212-226
CONTRIBUTIONS OF PALLIATIVE CARE TO PEDIATRIC PATIENT CARE CARMEN MANDAC AND VANESSA BATTISTA OBJECTIVES: To provide an overview of pediatric palliative care (PPC) as it relates to children and families living with oncologic disease.
DATA SOURCES: Journal articles, clinical research reports, clinical guidelines, and national statistics.
CONCLUSION: As new treatment protocols become available, the need for simultaneous supportive PPC, including adequate pain and symptom management, is evident. Further research and PPC program development is necessary for adherence to the current recommendation that PPC should be initiated at the time of diagnosis and continue throughout the course of a child’s disease.
IMPLICATIONS FOR NURSING PRACTICE: Palliative care nursing holds a specific role in the pediatric oncology setting. Registered nurses and advanced practice nurses should be adequately trained in PPC because they are in an optimal role to contribute to interdisciplinary PPC for pediatric oncology patients and their families.
KEY WORDS: Pediatric oncology, Pediatric palliative care, Nursing F the approximately 53,000 children that die each year, approximately 2,200 are from pediatric cancers.1 Cancer is the leading disease-related cause of death in children and adolescents ages 1 to 19,1 (referred to collectively as children for
O
the purposes of this article). In 2014 it is predicted that of the 10,450 diagnosed with cancer, 1,350 children are expected to die from their disease.2 This presents a crucial need for pediatric palliative care (PPC) within pediatric oncology, yet PPC programs are not as common as adult palliative care
Carmen Mandac, RN, MS, CPNP: Pediatric Nurse Practitioner Student, Columbia University, School of Nursing, New York, NY. Vanessa Battista, RN, MS, CPNP, CCRC: Pediatric Nurse Practitioner, The Children’s Hospital of Philadelphia, Pediatric Palliative Advanced Care Team (PACT), Philadelphia, PA. Address correspondence to Vanessa Battista, RN, MS, CPNP, CCRC, Pediatric Nurse Practitioner, The
Children’s Hospital of Philadelphia, Pediatric Palliative Advanced Care Team (PACT), 34th and Civic Center Boulevard, 11NW90, Philadelphia, PA 19104. e-mail: [email protected] Ó 2014 Elsevier Inc. All rights reserved. 0749-2081/3004-$36.00/0. http://dx.doi.org/10.1016/j.soncn.2014.08.003
CONTRIBUTIONS OF PALLIATIVE CARE TO PEDIATRIC PATIENT CARE
programs. In fact, although 58% of Children’s Oncology Group institutions (an institution that is supported by the National Cancer Institute and is devoted to pediatric cancer research that conducts clinical trials) have palliative care programs, only 6% discuss palliative care at diagnosis.3 There are several barriers that challenge palliative care delivery in oncology which are both specific and non-specific to pediatrics. Several barriers exist that prevent sufficient palliative care for children with cancer, including: relative rarity of cancer and death in children, the developmental stage of the child, a lack of evidence-based medicine to define symptoms and causes of death, difficulty in symptom management, lack of education for healthcare providers about palliative care, and reimbursement issues. All of these barriers contribute to the lack of integration of PPC in pediatric oncology. Utilization of registered nurses (RNs) and advanced practice nurses (APNs), referred to collectively as nurses for the purposes of this article, can optimize delivery of PPC within the oncology setting as they are in a unique position to bring together the interdisciplinary team caring for children and families. Semantics and assumptions have led palliative care to become synonymous with end of life (EOL), death, and hospice, and a lack of a clear definition of PPC often impedes its delivery. For this reason, it is important to define clearly PPC. Several groups, such as the World Health Organization, the American Academy of Pediatrics (AAP), and the Institute of Medicine, have each developed definitions that specifically address PPC. In summary, PPC can be defined as a philosophy of care and an organized program that focuses on total care and enhancement of quality of life of a child and family in the face of life-threatening illness.4-6 Different developmental tasks influence how children perceive and cope with illness and possible death, and a crucial aspect of PPC is the consideration of the developmental stage of the child (see Table 1).7,8 Children whose disease trajectory spans more than one developmental stage call for constant evaluation and adaptation of the delivery of support and care.1 Interventions and approaches should be informed by both the child’s chronological age and developmental stage,9 because they do not
213
always coincide. It is not unusual for children with advanced illness to either progress or regress in developmental stage. Therefore, when delivering PPC, the child should be considered as a product of their chronological age, developmental stage, medical condition, size, handicap, and cognition.9 Another barrier is the lack of distinction between PPC and hospice. The use of the terms palliative care and hospice care interchangeably in the common vernacular has led to confusion in the healthcare field. Although hospice and palliative care share a similar philosophy, they are different entities. Hospice is a part of PPC used for children at later disease stages, whereas the larger palliative care model represents an integrative approach in which care is initiated at diagnosis and delivered through the EOL. Integrative models combining curative care and palliative care enable continuity of care and facilitate a seamless transition to EOL care. It is suggested that palliative care be delivered within disease-focused medical treatment, regardless of curative goals.10
PEDIATRIC PALLIATIVE CARE IN ONCOLOGY Medical and pharmacologic advances within the last 40 years have drastically improved the survival rate in childhood cancers. Hodgkins and non-Hodgkins lymphoma, Wilms tumors, and acute lymphoblastic leukemia are now considered very curable diseases.11 Also, survival rates have increased for bone cancer, gonadal cancer, and neuroblastoma.11 Yet, despite these advances, cancer remains the overall leading cause of pediatric death second to motor vehicle accidents. Metastatic solid tumors and brain tumors continue to be difficult to treat.11 In short, it is evident that palliative interventions are necessary as suffering is universal for children with cancer and their families.10 A further examination of definitional clarity, disease trajectory, and symptom management point out the unique obstacles facing PPC delivery in pediatric oncology.
DEFINING PALLIATIVE CARE Definitional clarity affects the delivery of PPC in all fields. Although it is recommended that palliative care be offered early in the diagnosis, the various ways in which PPC is defined contributes to incomplete or inadequate interventions. For
214
C. MANDAC AND V. BATTISTA
TABLE 1. Developmental Stages and Perceptions of Death Age
Basic Conflict
Birth to 18 months
Trust vs mistrust
Early childhood (2-3 years)
Autonomy vs shame and doubt
Preschool (3-5 years)
Initiative vs guilt
School age (6-11 years)
Industry vs inferiority
Adolescence (12-18 years)
Identity vs role confusion
View of Death
Suggestions
No sense of finality and is viewed as continuous with life Reactive to stress Death is seen as reversible and not final May feel that death is a punishment May feel responsible for death
Use simple physical communication and provide comforting and nurturing care Expect regression, clinging, or aggressive behavior Encourage expression because the child may be concerned about family function after they die Use honest and clear language to explain death and dying Continue to use open communication with clear language Encourage questions about death and dying
Death continues to be understood as temporary May have a literal understanding of death and will respond with curiosity and questioning Death is understood as permanent Understands that the body does not function (heart stops beating and breathing stops) May feel responsible and guilty for the illness May have spiritual ideas about afterlife May not want to discuss feelings Understands the finality of death and may develop a mature understanding of death May try to take responsibility for adult concerns within the family (such as finances and care-taking) Feelings of anger may be present
Reassure the child that death is not their fault Aim to maintain as much structure and normal routine as possible Include the child in after life plans (funeral planning, last wishes)
Allow time for reflection Listen to concerns and questions Support efforts for autonomy and control
Data from Vern-Gross7 and Foster et al.8
example, 303 pediatricians in various areas of practice were asked to define palliative care.12 Of the pediatricians surveyed, 41.9% defined palliative care as hospice care, 31.9% gave an alternative definition, and 26.2% did not know what it was; with 49.3% making referrals to palliative care, and 29.4% unaware of community or local services,12 thereby exemplifying how lack of a universal definition impedes access to PPC. The study also reported that palliative care services are most often accessed in a manner similar to hospice
care.12 Within oncology specifically, a lack of ‘‘definitional clarity’’ relevant to palliative and supportive care exists within the literature, indicating a breakdown in knowledge dissemination regarding palliative care and thus affecting delivery and development of services and frequency of referrals.13 Because PPC has traditionally been introduced at EOL, the term has become synonymous with ‘‘giving up’’ by both medical teams and families.7 While this seems to be a ubiquitous theme
CONTRIBUTIONS OF PALLIATIVE CARE TO PEDIATRIC PATIENT CARE
throughout PPC, the focus of oncology is driven by a curative ideology. A study in which oncologists were asked to describe their experience with their most recent patient death revealed that oncologists who described their role as biomedical relayed a sense of failure when they did not have the ability to change the course of the disease.14 It is apparent that the curative biomedical perspective clashes with PPC when it is associated with termination of treatment and the death of a patient. Furthermore, death of a child has been described as a ‘‘triple failure’’ for healthcare providers because they feel that: 1) they did not have the ability to save a life, 2) they failed in their social role as adults by not being able to protect a child, and 3) they have betrayed the parents who trusted them with their child.1 Thus, the perception that palliative care and curative treatment should be delivered separately negatively affects healthcare providers and families within pediatric oncology.
DISEASE TRAJECTORY Advances in medical technology have drastically modified disease trajectories within pediatric oncology as healthcare providers are challenged with predicting outcomes for pediatric cancer patients.15 In one of the earliest studies of its kind, a retrospective analysis evaluated variables influencing EOL care in pediatric cancer. The reported median age of death was 9.3 years, with a range of 0.3 to 32 years; the median length of survival from diagnosis was 13 months, with a range of 0.5 to 100 months.16 The wide ranges indicate the marked variability in disease trajectory for pediatric cancers. Physical, mental, and spiritual/ existential changes will occur throughout the oncologic disease process and contribute to a high level of prognostic uncertainty as the rate and predictability of changes is never certain. Therefore, it is not surprising that referral to PPC services is highly influenced by uncertain disease trajectory. Referral to PPC services also varies according to disease process, as revealed by a retrospective chart review that showed the average referral time to PPC after diagnosis was 461 days, with 16% referred within 30 days of diagnosis, 26% before a relapse occurred, 40% after the first relapse, and 18% after more than two relapses.17 Although a majority of the children who died received PPC services, their referral was dictated
215
by their disease trajectory, concluding that referral to palliative care services should occur earlier in the disease.17 In a review of barriers to PPC, uncertainty about treatment response and overall chances for survival were shown to complicate a child’s plan of care.18 Early and influential research found that the level of certainty that the child would get better was the most prominent factor in the healthcare provider’s decisions about plans of care. Further research has continued to support this concept, showing that providers do not believe that referral to PPC will benefit the patient if they are not going to die of their disease.17 Furthermore, because pediatric cancer disease course has a high degree of uncertainty, goals of care can become unclear and result in dichotomous care, in which curative and palliative care are separate entities.18 This has also been termed a cure–care dichotomy that is associated with poorly controlled symptoms, chronic pain, patient and family distress, and decreased survival within pediatric oncology.19 Yet, if palliative services are reserved for only those who are dying, the opportunity to ease a substantial amount of children’s suffering will be missed. Higher parental ratings of quality of care were reported when both the physician and parent recognized there was likely no cure 50 days before a child’s death.20 Earlier discussion about EOL and integration of PPC can help alleviate suffering throughout the course of the illness.
PAIN AND SYMPTOM MANAGEMENT Children with cancer often present with complex symptom management needs and quality symptom management is a vital part of PPC, yet few reliable and valid instruments for symptom assessment in children exist.10 Some known cancer- and treatment-related symptoms experienced in children with cancer include emesis, anemia, fatigue, distress, and pain.10 These symptoms should be managed from diagnosis, supporting an integrative palliative and disease-directed approach to care; yet symptom management in pediatric oncology is not always successful. One hundred and three parents of children with cancer who died of advanced disease or treatment-related complications were interviewed after their child’s death; 89% reported that their child had experienced substantial suffering from at least one symptom and only 27% of parents felt that treatment of
216
TABLE 2. Oncologic Process Acute leukemias
CNS tumors
Description
Statistics/Survival
Complications
Relapse
ALL: 20% will relapse, 1/3 of those Treatment-related nausea, patients will have long-term vomiting, infection, pain, remission with intensified myelosuppression, therapy cardiotoxicity, peripheral AML: after relapse 80% 5-year event-free survival High-risk medulloblastoma and supratentorial PNET 5-year event-free survival is 50%-60% Pontine glioma survival is 8-9 months, with 90% dying within 2 years of diagnosis High-grade glioma: survival rate is 10%-30% Low-grade gliomas: survival rate is 90% HL and NHL account for 10%-12% Skeletal metastases HL and NHL B or T lymphocytes increase Chemotherapy-related side of pediatric malignancies rapidly or survive longer than effects, in particular nausea, NHL 5-year event-free survival in normal, eventually forming a peripheral neuropathy, children 15 years is 77% and Reed Sternberg cells. NHL cardiac toxicity (when combined HL of all stages, 5-year event-free is divided into diffuse large with radiation) survival is >80%, long-term B-cell lymphoma, lymphoblastic Varicella zoster survival is 90% lymphoma, Burkitt’s lymphoma, and anaplastic large cell lymphoma Wilms tumor Tumor of the developing kidney 6% of pediatric malignancies Abdominal pain 85% are cured of their disease Hypertension occurring between ages 1-5, most cases are sporadic with Malaise, fever Polycythemia few being familial, sometimes associated with congenital Bleeding within the tumor can lead syndromes to anemia, pallor, and fatigue Renal insufficiency Low-risk subgroups have a >90% Horner’s syndrome Neuroblastoma Malignancy of the sympathetic Spinal cord compression survival nervous system, or a disease of Anemia a neural crest tissue. Prognosis High-risk subgroups have Ataxia 30%-40% survival is varied according to age, Hypertension differentiation/biology of the Treatment-related side effects tumor, and extent of disease spread Sarcomas account for about 6% of Airway obstruction Sarcomas (soft tissue and bone) Heterogeneous group of tumors Cranial nerve complications pediatric cancers that arise in soft tissue or bone. Urinary obstruction Soft-tissue sarcoma survival Most common sarcomas Radiation-related side effects without relapse is >70% include osteosarcoma and Chemotherapy-related side Ewing sarcoma. Osteosarcoma effects arises from osteoblasts and
217
218
TABLE 2. (Continued) Description Ewing sarcoma is derived from the neural crest
Germ cell tumors
Retinoblastoma
Liver tumors
Arise from primordial cells involved in gametogenesis and occur in testes and ovaries. Originate in yolk sac endoderm and migrate along the genital ridge. Frequently occur in the sacrococcygeal region, midline of brain, mediastinum, and retroperitoneum Malignant ocular tumor, rarely diagnosed after 5 years of age, arises from the photoreceptor cells of the innermost layer of the retina
Malignancy of the liver, hepatoblastoma is the most common hepatic tumor occurring in infancy and young children. HCC and other rare tumors account for the other cases. Tumors are associated with genetic syndromes and environmental risk factors
Statistics/Survival Nonrhabdomyotamous soft tissue sarcoma with completely excised tumor survival exceeds 85%, unresectable tumors >5 cm have 50% survival Localized osteosarcoma survival is >70%, metastatic disease 2-year survival is 10%-30% 4% of pediatric cancers 5-year survival is >80%
Affects 200-300 children per year
1% of pediatric cancers metastatic disease, HCC have poor prognosis
Complications
Relapse
Limb amputation Cardiomyopathy Gonadal toxicity Secondary malignancy Renal dysfunction
Risk for secondary malignancy if Pain treated with alkylator therapy Constipation 20% will have metastatic disease Urinary obstruction Respiratory distress Gynecomastia Uterine bleeding Treatment-related proximal tubule renal dysfunction Cosmetic and functional Children with bilateral impairment retinoblastoma and unilateral Decreased visual acuity, eye retinoblastoma
