What Are Special Considerations for Perinatal Palliative Care?

Perinatal palliative care and perinatal hospice work is a unique facet of care of the maternal–fetal dyad and of brand-new families. These parents have rarely had relevant life experiences to prepare them for the possibility that their infant may die before or after birth. Providing seamless care to families during these transitions requires intentional collaboration with clinicians who typically do not work in the pediatric space—obstetricians, adult emergency department clinicians, and adult anesthesiologists. This requires perinatal palliative care providers to participate in creating systems of care that can overcome these barriers and allow people who are pregnant and their partners to rely on longitudinal palliative supports across multiple environments—outpatient prenatal care, inpatient labor and delivery, neonatal intensive care unit, and home hospice. Perinatal providers also need to be aware of local hospice resources specific to infant care, since these can be limited. While advances in fetal and neonatal care are improving outcomes from conditions such as complex congenital heart disease and prematurity, advances are also shifting a group of previously lethal neonatal diseases into chronic conditions. The demand for robust perinatal palliative care supports is only going to grow. As such, research, education, and advocacy in this area must continue.

Introduction and Scope of the Problem

In 2000, the American Academy of Pediatrics recommended “clinical policies and minimum standards that promote the welfare of infants and children living with life-threatening or terminal conditions, with the goal of providing equitable and effective support for curative, life-prolonging, and palliative care.” Calling out care for infants as separate from children is significant for several reasons. First, the number of infants (defined as all children under 1 year of age), and specifically newborns (defined as all infants 30 days of age or younger) who die annually is higher than the number of all other pediatric deaths combined. A second reason is that they and their families have several unique palliative needs requiring dedicated approaches. This chapter will highlight those needs.

Palliative care specific to newborns is alternatively called perinatal palliative care, which incorporates prenatal and postnatal palliative care; perinatal hospice, which designates prenatal and postnatal hospice services; neonatal palliative care; and pediatric palliative care. This chapter will use the phrases “perinatal palliative care” and “perinatal hospice” as they are inclusive of prenatal and postnatal services. Likewise, family and parent structures can vary in myriad and blended ways; for both simplicity and space constraints, this chapter will simply refer to the pregnant person or to parents when in many cases the unit of care is a family unit which can comprise multiple forms.

Primary perinatal palliative care is often delivered by interdisciplinary professionals who typically manage adult, not pediatric, patients. This includes anesthesiologists providing maternal grief counseling in the delivery room, obstetrical social workers facilitating perinatal hospice referrals, and labor and delivery nurses helping mothers through the physical and emotional pain of lactation initiation in the midst of neonatal death. This collaboration from nonpediatric team members requires extra planning and supports from the pediatric team.

Subspecialty perinatal palliative care services are growing nationwide and include both outpatient and inpatient supports. Well-resourced programs may enroll people following a wide range of prenatal “trigger diagnoses,” such as miscarriage or fetal complex congenital heart disease. Because the annual number of pregnancy losses in the United States may exceed one million, perinatal palliative care programs commonly leverage collaborations with obstetrical providers to support all people with pregnancy loss. Smaller perinatal palliative care programs may focus on newborns with life-threatening conditions.

Pediatric hospices are less available than adult hospices, and not all pediatric hospices have the expertise to care for infants. As the pregnant person is an adult, pediatric hospices may not be able to bill for prenatal services, further constraining hospice availability to programs able to provide free prenatal care. Where they exist, perinatal hospice services can include birth plans, counseling, sibling supports, and preparation for end-of-life care at home. Perinatal hospices also provide bereavement supports which may extend through future pregnancies.

This chapter will review unique features of providing palliative care to the maternal–fetal dyad, to newborns, and to extended families including young siblings. It highlights the prognostic uncertainty which can be substantial before birth. It also describes key elements of care plans for newborns, relevant to the intensive care unit and hospice. The chapter also reviews several previously “lethal” conditions that are evolving into survivable conditions with the application of clinical advances and chronic medical technology.

Relevant Pathophysiology

Prenatal

Perinatal palliative care offers an extra layer of support for parents and families navigating pregnancy and birth planning. Common prenatal diagnoses include genetic conditions, major congenital anomalies, and in-utero neurological insults. Key testing modalities include ultrasound, genetic screening, and advanced fetal imaging (e.g., echocardiogram or fetal MRI). Parents are routinely offered cell-free DNA testing, a noninvasive screen for trisomy 13, 18, and 21. A comprehensive anatomy screen typically occurs around 20 weeks of gestation. In many states, this time frame coincides with the window in which termination remains legal. (At the time this textbook is going to press, there are significant changes and legislative challenges occurring with regards to termination of pregnancy.) Several maternal conditions can impact fetal health and prompt additional monitoring, including diabetes, hypertension, and substance use. Maternal toxic stress can also contribute to negative birth outcomes, including prematurity and low birth weight.

Around Birth

Some life-threatening diagnoses do not present until birth, including extreme prematurity and severe perinatal asphyxia. For some parents, severe congenital anomalies present in this period due to lack of prenatal care or testing limitations. Several aspects of transitional physiology—the natural process that occurs as infants transition out of the intrauterine environment—can complicate early assessments of neonatal well-being. For example, transient tachypnea of the newborn can cloud assessments of pulmonary function. Many newborns experience hypoglycemia, and it can be challenging to know whether this will be transient or is evidence of an underlying condition. For patients without a prenatally diagnosed condition, the time-sensitive demands of resuscitation rarely allow for diagnostic testing to aid decision making; for example, a newborn with congenital heart disease may require urgent intubation prior to clarification of whether or not their cardiac lesion is operable.

Neonatal/Infant

For families who have received a prenatal diagnosis, the early neonatal period is often when diagnoses are confirmed or refined. For families just learning of a condition at birth, the initial days are a critical time of evaluating the infant’s need for cardiorespiratory support and determining the testing needed for diagnosis. For some families, their infant’s condition evolves more slowly over weeks to months, as with neuromuscular conditions, often with less urgent but more complicated testing.

It is important to note that many aspects of infant development evolve rapidly throughout the first weeks and months of life. Vital signs change significantly in a child’s first year. Most features of an infant’s exam vary by gestational age; for example, infants under 30 weeks of age normally have fixed and dilated pupils, but the same finding in a term infant would be highly abnormal. Many primitive reflexes are present for several months, even with severe neurological injury; parents may misinterpret these reflexes as volitional movements. Metabolism matures rapidly in the months after birth, such that medications used to manage symptoms may have altered dosing ranges and frequencies.

Summary of Evidence Regarding Treatment Recommendations

Prenatal

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