Comprehensive care
Neonatal palliative care is a modality of medical treatment that focuses not only on the physical aspects of disease but also on alleviating suffering and promoting the developmental, emotional, spiritual, and sociocultural well-being of the infant and his/her family.
It is appropriate for infants at all stages of the disease process, from birth to death or discharge, and it can be continued at home.
Palliative care is different than end-of-life care and is not hospice care.
Promotes comfort
It promotes the provision of comfort care with meticulous treatment of pain and other symptoms that interfere with the infant’s quality of life.
It promotes infant activities directed to comfort, such as increasing the time spent with parents, kangaroo care, infant massage, and music therapy, while supporting the developmental needs of the infant.
Promotes communication
It furthers good communication with families, helping with decision making and defining goals of care.
New concept of palliative care in neonates
As a new area of neonatal medicine, most palliative care guidelines and protocols that are currently being developed apply to the newborn with life-threatening conditions.
The majority of deaths in the neonatal intensive care unit occur soon after birth; among very low birthweight infants (≤1500 g), 51% of the deaths occur in the first 3 days of life.
Palliative care for the convalescent newborn infant is a novel concept.
Integrative model
Neonatal palliative care is provided alongside routine neonatal intensive care (Figure 51-1).
Before birth
The diagnosis and management of a suspected sick infant frequently starts before birth, when a fetal life-threatening condition is diagnosed during the pregnancy.
Prenatal conferences to discuss fetal and neonatal medical care often include obstetricians, neonatologists, geneticists, and other subspecialists including palliative care team members.
These conferences may reach high levels of complexity, involving not only the physical aspects of the fetal condition, but also the psychosocial, emotional, cultural, and religious challenges for the parents and their families.
Some infants may have undergone surgical procedures during fetal life.
Decisions about limitations of resuscitation and maximization of comfort care may have occurred prenatally.
In the delivery room
A detailed evaluation by an experienced clinician shortly after birth is necessary to confirm the prenatal diagnosis, assess the infant’s clinical condition, and make sure plan of treatment is followed.
In the NICU
Integration of palliative care to curative treatments may occur from birth or later in the course of a worsening medical condition despite intensive care efforts.
Palliative care protocols, guidelines, and electronic order sets are useful tools to guide providers in this process.
The role of palliative medicine specialists may be crucial in blending palliative care into daily neonatal intensive care, and also care once the infant is discharged home.
End of life
Death may occur in the hospital, at home with hospice care, or in an inpatient hospice facility, in concert with the infant’s needs, parental wishes, and community resources.
Care of the family continues following the death of an infant.
Bereavement support can be provided by the hospital or by hospices in the community.
Consultative model
A pediatric/neonatal palliative care specialist or team is consulted as needed, providing guidance through the entire process.
Any infant who has a clinical condition so severe that it seems incompatible with long-term survival and/or carries the risk of severe impairment of quality of life should be considered a candidate for palliative care.
Clinical conditions that may lead to the consideration of palliative care
Pulmonary disease
Severe chronic lung disease, lung hypoplasia, refractory pulmonary hypertension.
Deteriorating chronic lung disease in spite of treatments, where further intensive care may merely prolong death rather than offer any hope of survival.
Complicated, severe congenital diaphragmatic hernias, and thoracic dystrophies.
Cardiovascular disease
Complex cardiac anomalies, sometimes in patients who have exhausted surgical options.
Mitochondrial myopathies and other conditions affecting the myocardium.
Refractory congestive heart failure and cor pulmonale.
Neurologic disease
Severe hypoxic-ischemic encephalopathy (HIE). Although treatment with hypothermia has shown to improve survival in infants with HIE, about 25% of infants who undergo cooling still die.
Severe intraventricular hemorrhage, marked hydrocephalus, significant cerebral atrophy, severe strokes, and large arteriovenous malformations.
Brain malformations including holoproscencephaly, hydranencephaly, lissencephaly, severe neural tube defects, and large encephaloceles. Intractable seizures and brain tumors.
Brain infections such as devastating herpetic encephalitis, refractory meningitis, and disseminated brain abscesses.
Degenerative disorders of the central nervous system such as leukodystrophies.
Renal disease
Renal dysplasia, polycystic kidney disease. Renal failure, sometimes in infants not candidates for renal transplantation, other times following discontinuation of dialysis.
Gastrointestinal disease
Congenital anomalies such as gastroschisis and giant omphaloceles.
Intestinal failure secondary to intestinal atresias or other bowel complications, including those resulting in ischemic bowel injury and severe short bowel syndrome.
Musculoskeletal disease
Some skeletal dysplasias or severe muscular dystrophies.
Conditions related to prematurity
Although mortality and major morbidity continue to decrease for premature infants, a large number of ELBW infants still die or survive with a major complication.
Some of these morbidities may be so severe that the infant may not be expected to survive long term, such as severe chronic lung disease, severe pulmonary hypertension, heart failure, complicated intraventricular hemorrhage, encephalomalacia, intestinal failure, and failure to thrive.
Genetic disorders
Chromosomal anomalies, single gene defects, metabolic disorders, and clinical syndromes incompatible with long-term survival.
Neonatal malignant neoplasms
Neuroblastomas, leukemia, renal and liver tumors, sarcomas, teratomas, and brain tumors not responsive to treatment.
Adequate and meticulous symptom management is essential to ensure infant’s comfort and to reduce high levels of parental stress.
Management of neonatal pain
Classification of pain
It is important to differentiate between nociceptive and neuropathic pain.
Nociceptive pain: Tissue injury activates pain receptors called nociceptors, which can respond to heat, cold vibration, and chemical substances released in tissue injury. Nociceptive pain is divided into the following:
Somatic pain: Due to activation of nociceptors in superficial tissues (eg, skin, mucosas) or deep tissues (bone, tendons, joints, muscles)
Visceral pain: Due to activation of nociceptors located in viscera (such as stomach, intestines, bladder). This can be caused by inflammation, distention with fluid or gas, stretching, or compression.
Neuropathic pain: It is due to structural damage and nerve cell dysfunction in the peripheral and central nervous system.
Although neuropathic pain has not been well described in infants, any process that causes damage to nerves, such as metabolic, traumatic, infectious, ischemic, toxic, or immune mediated, can result in neuropathic pain.
It can also be caused by nerve compression.
Evaluation of neonatal pain
N-PASS (Neonatal Pain, Agitation, and Sedation Scale)
A valid and reliable assessment tool used to monitor acute neonatal pain.
Scores ≥4 indicate the need for nonpharmacological and/or pharmacological measures.
Scores should be assessed at least every 4 to 6 hours or with every infant assessment.
Parental involvement in infant pain recognition and management is feasible and enhances parental confidence. This is crucial in the management of sick infants who will be discharged home, with or without hospice services.
Treatment of neonatal pain
Nonpharmacological interventions (Table 51-1)
The infant should be kept swaddled and warm as much as possible.
Effective comforting techniques include skin-to-skin holding, nonnutritive sucking, swaddling, and rocking/holding.
Other comfort measures include breast-feeding, nesting, minimal stimulation, uninterrupted sleep, and cluster care based on the infant’s cues.
Other therapies to consider are music therapy, including listening to recordings of the mother’s voice, and massage therapy.
An important goal is to avoid or minimize painful procedures, such as blood draws, and provide adequate comfort when procedures are needed. Noise and light levels should be minimized as much as possible during painful procedures.
Excessive and unpredictable sound levels have been associated with increased physiologic and behavioral stress in preterm infants in the NICU.
Pharmacological interventions
The goal for pain relief in infants is to maximize comfort, while maintaining function to the greatest extent possible.
A two-step approach is recommended.
For mild pain, initiate pain management with nonopioids such as acetaminophen or ibuprofen.
For moderate or severe pain, add opioids (Table 51-2).
Administration of pain medication in neonatal palliative care
By the clock: At regular intervals
By the infant: Based on the infant’s pain level and response to treatment
By the appropriate route: Use the least invasive route possible.
The oral/enteral route is preferred in palliative care, and some convalescent infants may have nasogastric or gastrostomy tubes in place for their nutritional needs.
The sublingual route is an option for some opioids, such as morphine, and anxiolytics, such as lorazepam.
The intranasal route is very effective for midazolam and fentanyl.
The parenteral route should be used when rapid titration is required or when the infant does not tolerate the oral route.
Intramuscular injections for analgesia have no justification in palliative care.
Use of nonopioids
Acetaminophen administered intravenously is a good supplemental option to opioids for postoperative management, as it reduces the cumulative dose of morphine needed following major noncardiac surgery.
Both oral and rectal administration of acetaminophen are acceptable routes for treatment of fever or as an analgesic for mild pain.
Ibuprofen may be used in older infants greater than 6 months of age.
Oral sucrose is safe and effective in reducing procedural pain in infants, and should be considered for intravenous line placements, blood draws, and wound care.
Use of opioids
Opioids block the afferent input to the central nervous system (CNS) from the activation of peripheral nociceptors. They exert their effects by binding to mu, delta, and kappa opioid receptors in the CNS.
In newborn infants, opioids have a longer half-life due to immature hepatic biotransformation pathways at birth.
Morphine
Metabolized in the liver by the uridine-5′-diphosphate (UDP) glucuronosyltransferase to morphine-6-glucuronide (M6G) and morphine-3-glucuronide (M3G), metabolites that are cleared renally.
The immaturity of the blood brain barrier, particularly in preterm infants, facilitates the access of opioids to the CNS, increasing their potency.
All these reasons lead to marked variability in the pharmacokinetics and pharmacodynamics of opioids according to chronological age, gestational age, prematurity, underlying pathology such as hepatic dysfunction and treatment circumstances, such as reduced morphine clearance during hypothermia treatment.
Fentanyl
100 times more potent than morphine, has rapid onset of action and a short half-life, making it useful for brief painful procedures such as central line placements and removals, manipulations of drains, and dressing changes.
It is metabolized to inactive metabolites in the liver, and it is generally well tolerated by infants with hepatic and renal dysfunction.
In infants, rapid administration of IV fentanyl should be avoided as it can cause chest rigidity, with inability to ventilate the patient.
Fentanyl intranasal doses of 1 to 2 mg/kg may be a safe and effective option for brief painful procedures and for palliating distress in dying infants.
TABLE 51-1.
Nonpharmacological comfort care interventions
Nonpharmacological Comfort Care Interventions
Skin-to-skin holding
Nonnutritive sucking
Swaddling
Rocking-holding
Breast-feeding
Nesting
Minimal stimulation
Limiting environmental noise
Listening to mother’s voice (or recordings)
Cue-based care
Cluster care
Providing containment during painful procedures
Limitation of painful procedures
Music therapy
Massage therapy
TABLE 51-2.
Palliative care medications for infants
Medication
Starting Dose
Frequency
Infusion
Comments
For Pain
Morphine
0.02 to 0.1 mg/kg IV
0.2 to 0.4 mg/kg PO
q2-4h
0.02 to 0.1 mg/kg/h
Opioid
Medication of choice for pain management in palliative and end-of-life care.
For end-of-life care, may use in combination with a benzodiazepine.More frequent doses may be needed to ensure patient’s comfort
Fentanyl
1 to 3 μg/kg
IV/intranasal
q1-2h
1 to 3 μg/kg/h
Opioid
Short acting
Preferred in patients with renal failure
Methadone
0.05 to 0.2 mg/kg IV/PO
q4-24h
Long-acting opioid
Usual starting frequency is every 8 to 12 hours
Useful in refractory, chronic or neuropathic pain
Oxycodone
0.1 mg/kg PO
q4-6h
Opioid
Acetaminophen
10 to 15 mg/kg PO/IV or 20 mg/kg PR
q6h
Analgesic
Oral sucrose 24%
< 1 kg: 0.1 mL
1 to 2 kg: 0.5 mL
> 2 kg: 1 to 2 mL
PRN
Analgesic
For Pain/Sedation
Clonidine
1 to 3 μg/kg PO
q6-8h
α-Agonist. Watch for hypotension and bradycardia
Has mild analgesic and sedating properties
Dexmedetomidine
0.5 to 1 μg/kg IV
2 to 4 μg/kg buccal/intranasal
q1-4h
0.2 to 1 μg/kg/h
α-Agonist. Watch for hypotension and bradycardia
Has analgesic and sedating properties. Causes less respiratory depression than opioids and benzodiazepines
Gabapentin
5 to 10 mg/kg PO
q8-12h
Adjunctive therapy for neuropathic or visceral pain. Start with lowest dose
For Sedation
Midazolam
0.05 to 0.1 mg/kg IV
0.2 mg/kg SL/intranasal
q1h
1 to 2 μg/kg/min
Very short-acting benzodiazepine
Anticonvulsant. Rapidly penetrates the CNS
Produces amnesia
During end-of-life care, more frequent
Doses may be needed to ensure patient’s comfort
Lorazepam
0.05 to 0.1 mg/kg IV/PO
q2-4h
Benzodiazepine
Consider adding to opioids for sedation
Reduces anxiety and agitation Anticonvulsant
Diazepam Chloral hydrate
0.05 to 0.25 mg/kg PO/PR
25 to 75 mg/kg PO/PR
q4-12h
q4-12h
Long-acting benzodiazepine
Use for sedation and somnolence
Other
Glycopyrrolate
2 to 10 μg/kg IV/SC
20 to 100 μg/kg PO
q6h
Decreases oral secretions
Glycerin suppository
1/8 to 1/4 suppository PR
q12-24h
To treat constipation
Polyethylene glycol 3350
0.5 to 1.5 g/kg PO
q24h
To treat constipation 
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