PEDIATRIC

PALLIATIVE CARE
Glen Medellin, M.D., FAAP
Greehey Distinguished Chair in Palliative Care
for Children
Session Objectives
• Compare adult and pediatric palliative care
• Identify barriers to provide inter-professional comprehensive
pediatric palliative care for pediatric patients with advanced
illness
• Discuss assessment and management of pain and non-pain
symptoms in pediatric patients
• Review processes to be followed when a child dies including
bereavement
Definitions
• Neonate: Birth to 28 days old
• Infant: Birth to 1 year old
• Child: 1-18 years old
Statistics
• 81 million children in the United States
• United States 2.5 million deaths annually
• About 50,000 deaths are pediatric 0-19 (2.2%)
• Children represent 25% US population

• Half of childhood deaths are in first year of life

• Half of infant deaths are in the first month of life
Symptoms in Dying Children
• 89% suffered “a lot” or “a great deal” from at least one
symptom in their last month of life
• Most common reported symptoms:
• Pain
• Fatigue
• Dyspnea

Wolfe, NEJM, 342:5; 2000

 Causes of Deaths
All Infants
1. Congenital malformations
2. Short gestation / LBW
3. Sudden Infant Death Syndrome
4. Maternal complications
5. Complications of placenta, cord, or membranes
6. Accidents/unintentional injury

www.nhpco.org, Facts & Figures on Pediatric Palliative Care and

Hospice
 Causes of Deaths
Infants with Complex Chronic C0nditions
1. Cardiovascular (32%)
2. Congenital / genetic (26%)
3. Respiratory (17%)
4. Neuromuscular (14%)

www.nhpco.org, Facts & Figures on Pediatric Palliative Care and

Hospice
 Causes of Death Children 1-19
1. Accidents
2. Assault
3. Malignancy
4. Suicide
5. Congenital malformations, deformations
6. Chromosomal anomalies
7. Heart disease
8. Cerebrovascular diseases

www.nhpco.org, Facts & Figures on Pediatric Palliative Care and

Hospice
 Causes of Death
Children 1-19 with Complex Chronic Condition
1. Malignancy (43%)
2. Neuromuscular (23%)
3. Cardiovascular (17%)

www.nhpco.org, Facts & Figures on Pediatric Palliative Care and

Hospice
 Pediatric Palliative Care Diagnosis
• Conditions for which curative treatment is possible
but may fail
• Advanced or progressive cancer or cancer with a poor
prognosis
• Complex and severe congenital or acquired heart disease

N Engl J Med 2004; 350:1752-1762

• Conditions requiring intensive long-term treatment
aimed at maintaining the quality of life
• Human immunodeficiency virus infection
• Cystic fibrosis
• Severe gastrointestinal disorders or malformations such as
gastroschisis
• Severe epidermolysis bullosa
• Severe immunodeficiencies
• Renal failure in cases in which dialysis, transplantation, or
both are not available or indicated
• Chronic or severe respiratory failure
• Muscular dystrophy
 • Progressive conditions in which treatment is
exclusively palliative after diagnosis
• Progressive metabolic disorders
• Certain chromosomal abnormalities such as trisomy 13 or
trisomy 18
• Severe forms of osteogenesis imperfecta

N Engl J Med 2004; 350:1752-1762

• Conditions involving severe, nonprogressive
disability, causing extreme vulnerability to health
complications
• Severe cerebral palsy with recurrent infection or difficult-
to-control symptoms
• Extreme prematurity
• Severe neurologic sequelae of infectious disease
• Hypoxic or anoxic brain injury
• Holoprosencephaly or other severe brain malformations
Diagnoses in Pediatric Palliative Care
• Genetic/Congenital (40%)
• Neuromuscular (40%)
• Oncologic (20%)
• Respiratory (12%)
• Gastrointestinal (10%)
• Cardiovascular (8%)

Some children with multiple diagnoses

Pediatrics Vol. 127 No. 6 June 1, 2011

 Survival function in the cohort of 515 patients who received pediatric palliative care
consultation services and among patients with the 3 most prevalent conditions.

Feudtner C et al. Pediatrics 2011;127:1094-1101

©2011 by American Academy of Pediatrics

Dichotomous Care Process
Mutually Exclusive

•Family
•Forced Choices
•No best option
Curative •Abandonment
•Hope
‘The Dying Point’
Docherty et al Ped Nurs 2007 End of Life
•Health Care Providers Care
•Prognostic Uncertainty
•Family resistance
•Challenge of changing course of care
•Conflict around goals of care
•Avoidance of emotionally charged situations
•Relinquishing care to another team
•Hope
Illness Trajectories
Role of Palliative Care

Primary Care Palliative

Care
Diagnosis

Primary Care

Death
Palliative Care

Primary Care
Comprehensive Care
Palliative Care
Models of care
• Inpatient consultation palliative care teams
• Inpatient palliative care
• Home hospice
• Perinatal and neonatal hospice
• Concurrent care
• Respite
Palliative Care Team
• Nurses
• Dietary
• Respiratory care
• chaplains
• bereavement counselors
• social workers
• child life therapists
• primary care physicians
• subspecialty physicians
• consultants.
 PALLIATIVE CARE TEAM FOR CHILDREN AND FAMILIES
Home

Inpatient Services Respite

Child and Family

Inpatient Palliative Care Unit School

Interdisciplinary Team of Health Care

Professionals
ICU Community

Care coordination
Clinic across settings
Home Care

Primary Care Physician

Consultation
Therapy programs Service

Specialty clinics
Concurrent Care for Children Requirement
• The CCCR provision (Section 2302 of the ACA) states that
• children under the age of 21
• diagnosed with a life-limiting illness
• eligible for Medicaid or the Children’s Health Insurance Program
• May receive all services that are related to the treatment of a
child’s life-limiting illness.
• This allows these young people to have palliative and
hospice care services while they are receiving other disease-
modifying treatments.
DEVELOPMENTAL STAGES
Infants and toddlers (age 0-3)
• Interactions initially limited to sensory and motor actions.
Needs a sense of trust and hope in others. Developing
feelings of self-worth and love.
• No concept of death.
• Limited concept of reality and may simply sense something is
wrong.
Infants and toddlers (age 0-3)
• Care focused on providing maximal physical relief of
suffering.
• Benefits from simple physical communication.
• Benefits from physical touch, maintaining routines, and safe
environment.
Preschool age (age 3-6)
• Egocentric. Uses magical and animistic thinking.
Understanding centers on interweaving fact and fantasy.
• Spirituality is magical and imaginative. Participation in rituals
becomes important.
• Death is a temporary separation and reversible. Illness
perceived as the result of a “contagion” or from contact
with an object or person and benefit from reassurance.
Preschool age (age 3-6)
• May feel responsible for illness and misinterpret emotions
(eg, think sadness is disappointment). Benefits from
clarification of misconceptions and reassurance.
• Use precise language (avoid euphemisms). Evaluate for
feelings of guilt and anger at self or others.
• Benefits from minimizing separation from family and
maintaining consistency in daily routine. Utilizes play,
puppets, dolls, expressive therapies and story telling in
coping and learning.
School-age (age 7-12)
• Begin to differentiate between self and others. Concrete
thoughts with beginning transition to more logical thinking.
• Start connecting ritual with personal identity and accepting
external interpretation as truth.
• Understand that death is irreversible and personal.
Interested in physiology and details of death.
• May feel responsible and need reassurance. Begin to
demonstrate mature understanding of illness.
School-age (age 7-12)
• Should be invited to share their emotions when they are
ready. May exhibit stoic responses in an attempt to protect
their parents and caregivers.
• Should have some participation in decision making. Benefits
from open, honest communication and concrete details of
treatments.
• Benefits from maintaining access to peers and fostering a
sense of control/mastery over the illness. Benefits from
maintaining usual activities as much as possible.
Adolescents (age 13-17)
• Developing abstract thoughts. Body image and self-esteem
paramount. Begins to challenge parental views. Separate by
developing peer group identity.
• Searches for meaning, purpose, hope, and value of life.
Evolution of relationship with higher power.
• Explores nonphysical explanations of death.
• Very self-conscious of physical change. Struggle with need
for independence and physical dependence due to illness.
Adolescents (age 13-17)
• Should be helped to communicate feelings and invited to
share their thoughts and sadness when they are ready. May
turn to a non-parental adult to share sadness.
• Key participant in decision making. Benefits from clear,
direct, honest communication.
• Benefits from reinforcement of body image and fostering
self-esteem by providing privacy and promoting
independence. Benefits from access to peers and support
groups and utilizing creative outlets. Is at risk for developing
depression and risk-taking behaviors.
Advanced Care Planning
• The child should participate to the fullest extent possible,
given his or her illness experience, developmental capacities,
and level of consciousness.
• Regardless of the prognosis, respect for the child requires
that he or she be given a developmentally appropriate
description of the condition along with the expected
burdens and benefits of available management options,
while soliciting and listening to the child’s preferences.

PEDIATRICS Vol. 106 No. 2 August 2000

ASSESSMENT AND
MANAGEMENT
A. Wong’s essentials of pediatric nursing. 7th edition. St. Louis:
Mosby; 2005. p. 125
B. Pain 2001;93:176
Pediatr Nurse 1997;23(3):293–7
Neurological Impairment
• Developmental
• Cerebral palsy
• CNS insult
• hypoxic ischemic encephalopathy (HIE)
• anoxic encephalopathy
• traumatic brain injury
• Specific diagnosis
• genetic disorder (muscular dystrophy, spinal muscular atrophy)
• congenital anomaly (holoprosencephaly)
• structural brain malformation (lissencephaly)
• metabolic disorder (mitochondrial disease, Tay-Sachs)
Identifying presence of pain
• Behaviors seen in validated pain assessment tools for
nonverbal children with neurologic impairment
• Vocalizations (crying, moaning)
• Facial expression (grimacing, fussy)
• Consolability
• Interactivity (withdrawn, less active)
• Movement (pulls legs up)
• Tone and posture (arching, stiffening)
• Physiological responses (sweating)

Hunt 2004, Breau 2002, Malviya 2006

 Nocioceptive
Pain

Neuropathic
Spasticity
pain

Suffering
Visceral
Dysautonomia
Hyperalgia

Drug Effects
Opioid dosing basics
• Dosage initially based on weight
• Same escalation principles as in adults
• No upper dose limits
• Taste can be a limiting factor
Morphine
• Gold standard for moderate or severe pain
• Increased half-life and diminished clearance in neonates.
• Starting doses for infants about ½ of older children.
• Infants more sensitive to respiratory depression.
Codeine
• Use in mild pain only, limited use in severe pain
• Maximum recommended dose (60mg) produces analgesia
equal to 600mg aspirin
• Combination product with acetaminophen
• Highly variable metabolism makes it unreliable
• Not a preferred agent
Hydrocodone
• Only available in combination with acetaminophen, aspirin or
ibuprophen
• Schedule 3 controlled substance makes it easier to prescribe
• Preferred over codeine
Fentanyl
• Used in anesthesia, procedural sedation
• Acute moderate to severe pain
• Patch has found use in some cancer and chronic non-
malignant pain
• Patches are high enough dose that can’t be used in opioid
non-havbituated children based on weight
Methadone
• Used in chronic pain
• Long half-life therefore longer time to steady state
• Should not be used for breakthrough pain
• Only liquid long-acting formulation
Sustained-release Opioids
• Children often cannot swallow pills
• Even lowest dosages of sustained release products may be
too high for children
• High rates of gastrostomy tubes in pediatric palliative care
population necessitate liquid formulations
Co-Analgesics
• Antidepressants - amitriptyline, nortriptyline
• Anticonvulsants - valproic acid, phenytoin
• Anxiolytics - lorazapam, diazapam, midazolam
• Corticosteroids – dexamethasone
• Anesthetics - lidocaine, ketamine, propofol
• Barbiturates - phenobarbitol, pentobarbitol
SUMMING IT UP
 Pediatric Palliative Care vs. Adult
• Smaller numbers of dying children than adults mean that
there is less professional expertise and underrepresentation
of children in palliative care protocols.
• The heterogeneity of illnesses, many rare, requires the
involvement of many disciplines and specialists.
• Many children have genetic diseases so that there may be
more than one affected child in a family.

Curr Probl Pediatr Adolesc Health Care, October 2005

• The time course of some illnesses is extremely variable;
pediatric palliative care may extend over years, even
decades. Prognosis is very difficult.
• A broad developmental spectrum is represented, including
changes in the individual child through time.
• Pediatric hospice care tends to be more expensive, and
palliative and/ or curative oriented therapies may happen in
concert with active end of life care.
• Kids have specific developmental needs which are
dependent upon age, but also impact of disease,
developmental capacity, etc.
• Family centered care: family as the unit of care
• Emotional intensity: tends to be a concern for adult specific
providers, and is realistic.
Not talked about, but can discuss
• Legal and ethical issues of non-autonomous individuals
• Artificial nutrition and hydration
• Informed consent vs. assent
• Declaration of brain death difficult
• Baby Doe law
http://www.chionline.org/