KEY POINTS
- 1.
Neonatal intensive care unit (NICU) admission rates have increased over time, with a consequent increase in the number of extremely premature and critically ill infants who are at risk of chronic illness and mortality.
- 2.
The death of an infant is one of the most devastating and difficult experiences in life. Parents of seriously ill infants bear many roles such as caregiver, advocate, and decision-maker while maintaining hope and managing uncertainty related to their infant’s prognosis.
- 3.
Palliative care requires interdisciplinary specialty groups who seek to work with seriously ill infants and their families to provide comprehensive treatment of suffering.
- 4.
Families’ spiritual needs to be considered. End-of-life care is extremely important to bereaved NICU parents as they cling to the limited memories of their deceased infant.
- 5.
Family bereavement supports may include individual/family counseling, spiritual support, efforts to encourage verbal and/or nonverbal expression in parent groups, family camps, longitudinal staff remembrances, and follow-up meetings.
In this chapter we will describe the evidence for palliative care family support beginning in the prenatal period and extending to acute and chronic neonatal intensive care unit (NICU) care and finally to bereavement. Neonatal providers have the unique opportunity to improve family-centered care for patients and families experiencing serious illness in the NICU and to make a long-term impact on NICU families’ lives ( Fig. 91.1 ). For infants who do not survive, neonatal providers may improve the care leading up to death and affect parent grief experiences by facilitating an interdisciplinary palliative approach and supporting families to create meaningful and positive memories despite the limitations of an intensive care unit (ICU) environment. However, there are challenges to implementing this palliative approach and providing care that is concordant with family wishes ( Fig. 91.2 ). Perinatal palliative care (PPC) as an additional layer of family support is often delayed until death is imminent, preventing families from experiencing the full range of this layer of support. Beginning with a fetal diagnosis, families require intensive support given the extremely difficult decisions they must make in the event of a life-limiting or life-threatening diagnosis. During a NICU hospitalization, the infant’s state of clinical uncertainty and unknown survival makes decision-making very difficult for families, and they require intensive support to balance their new identity as a “NICU parent” with life and other demands outside of the hospital. Medical advancements have decreased the number of perinatal and infant deaths over time; however, there are now more infants surviving with chronic critical illness, and families continue to require support as their goals of care shift.
Prenatal Phase
Clinical Case Report
Cynthia is a 26-year-old woman from Mississippi who is pregnant with her third child. Cynthia and the child’s father are no longer together; the father of the baby is involved but lives in another city 45 minutes away. Cynthia currently lives in a small town and travels nearly 2 hours to the nearest tertiary care hospital for the majority of her medical care.
Cynthia arrives at her 20-week ultrasound appointment. During the ultrasound, Cynthia can tell something is wrong. Her worries are confirmed when she and her mother are unexpectedly asked to meet with a maternal-fetal medicine specialist after the ultrasound. Cynthia is heartbroken as she learns her baby, a boy, has multiple congenital malformations. Although the maternal-fetal medicine specialist states that additional testing is needed, she explains to Cynthia that she will likely have difficult decisions to make soon. The specialist predicts the fetus may survive until delivery, but it is unclear how long he may survive after birth. If he survives, the specialist worries he will never breathe on his own and will have severe delays in development. The specialist shares with Cynthia that there are multiple care pathways to consider at this time, but given the severity of the anomalies, she encourages Cynthia to think about termination of the pregnancy.
Cynthia is overcome with sadness and grief. Cynthia has a strong faith tradition and fears judgment for even considering termination of the pregnancy. She is torn because she also worries her child will not have the quality of life she would want for him. Cynthia is left feeling completely overwhelmed and is unsure how to proceed.
Epidemiology of Perinatal Death and Parent Outcomes
With advancements in perinatal medicine, perinatal mortality has steadily declined during the past several decades, although it has begun to plateau in more recent years. , In 2016, the perinatal mortality rate in the United States was 6.0 deaths per 1000 live births. Although there have been overall declines in perinatal mortality rates, a large disparity remains in outcomes related to race and geography. The mortality rate of infants born to non-Hispanic Black women is more than twice that of non-Hispanic White women, and the southern states of Mississippi and Alabama have consistently had worse outcomes than the rest of the country.
It is important to recognize that perinatal loss is a traumatic event with long-lasting parental effects. Gold et al. found that after a perinatal loss, bereaved mothers had four times higher odds of depressive symptoms and seven times higher odds of posttraumatic stress disorder symptoms. Although no differences in symptom levels were noted among races, disparities in treatment were noted. African American mothers who screened positive for posttraumatic stress disorder or depression were much less likely to receive treatment. Perinatal loss also affects the marital dyad. Couples who had stillbirths were at higher risk of dissolution of their relationship in the decade following the death compared with those who did not have a fetal loss.
In 2017, congenital malformations were the most common cause of infant mortality in the United States. Many advancements in perinatal medicine have led to earlier and more accurate fetal diagnoses. Although these early forms of screening can provide families and clinicians with beneficial information regarding the health of the mother and fetus, parents are often not prepared for a potentially life-threatening or life-limiting diagnosis for their child.
Role of Perinatal Palliative Care
Parents who have received news of a life-limiting or life-threatening fetal diagnosis describe “grieving multiple losses,” including the loss of their healthy baby, the loss of a normal pregnancy, and the loss of future parenting. After the diagnosis of a life-limiting or life-threatening fetal diagnosis, the American College of Obstetricians and Gynecologists committee on ethics and the American Academy of Pediatrics committee on bioethics recommend that the “full range of options, including fetal intervention, postnatal therapy, palliative care or pregnancy termination” be discussed with families. Early PPC, introduced at the time of diagnosis, may help develop a trusting relationship and can guide decision-making and support parental needs during this complex time. PPC brings the interdisciplinary approach into the prenatal period and the immediate postnatal period (see Fig. 91.1 ). An interdisciplinary approach is vital to palliative care principles and should be integrated throughout the care of the patient and family. , For example, social workers often work with the PPC team to identify financial or counseling resources, child life specialists can assist with preparing siblings for the birth and possibly death of a newborn, and lactation specialists may assist the mothers with either weaning off of breast milk or donating their breast milk.
Spiritual and Cultural Considerations
A family’s spiritual needs and wishes should be considered at all points in care. Parents may struggle with existential questions after the diagnosis of a life-limiting condition. In a study evaluating values applied to parental decision-making in delivery room resuscitations, family decision-making was largely steered by religion, spirituality, and hope as opposed to the medical information presented. Wishes for spiritual or cultural rituals should be coordinated whenever possible. The care team should be aware of any culture-specific customs or practices and honor them whenever able. ,
Communication
After news of a life-limiting or life-threatening diagnosis, parents are often forced to make difficult and often time-sensitive decisions regarding multiple aspects of care for their child. Communication and information delivery during this prenatal time is crucial. In a study of 19 families given the prenatal diagnosis of trisomy 18, the majority of parents felt they were not appropriately informed about the potential diagnosis during the screening process and believed ultrasound findings were poorly communicated. From the same study, empathic communication was found to be essential in parents’ overall satisfaction with care. It is important to recognize that a family’s communication needs may vary. Whereas some families may choose to seek out additional information regarding the diagnosis, others may avoid it as a method of coping. Dialogue with parents about their preferred communication styles and approach to receiving information is important in making sure parental needs are being met.
Prenatal consultation with neonatologists provides parents with the opportunity to discuss care and talk through decisions specific to their child and family. Miquel-Verges et al. conducted interviews with 22 women after a diagnosis of congenital anomalies and discovered parents valued five major themes in prenatal consultation. Parents valued the opportunity to feel prepared, have a knowledgeable physician, have a caring physician, have the opportunity to allow hope, and spend time with the physician. Parents also felt touring the NICU beforehand equipped them with additional knowledge and helped them feel more prepared for a transition to NICU care.
Prenatal Decisions: Termination, Imaging, Monitoring
After a discussion of possible options, some parents face the difficult decision of continuation or termination of the pregnancy. In a study by Breeze et al., the median time for parents to decide on termination or continuation of pregnancy was 1.5 days (range, 0–8 days), illustrating the urgent nature in which this decision must sometimes be made. Parents’ decision to continue or terminate a pregnancy may be multifactorial. In a study by Guon et al. of 332 parents who chose continuation of pregnancy in the setting of a trisomy 13 or trisomy 18 diagnosis, themes of moral beliefs and child-centered reasons such as love for their child, the value of their child, and uncertain outcomes affected their decision to continue the pregnancy. Families who choose continuation of pregnancy often hear messaging to terminate the pregnancy or face feeling unsupported by their medical team.
Ongoing maternal and fetal care, including potential interventions and monitoring, should be discussed and individualized to facilitate plans matching parental goals. Offering families routine fetal surveillance may provide reassurance and add a sense of normalcy to the pregnancy. Routine surveillance and standardized testing such as ultrasounds can also promote memory-making and bonding. In a qualitative study, seven parents were interviewed who received life-threatening fetal diagnoses, and all parents expressed their desire to hear the fetal heartbeat and see ultrasound images as a chance to “get to know their baby.”
Delivery Decisions: Location, Mode, and Infant Care
Parental preferences surrounding intrapartum care should be discussed in conjunction with the obstetrician and neonatologist or pediatrician (see Fig. 91.2 ). Parents who reported struggling for control over prenatal and delivery options were reported to have lower levels of satisfaction with care. The location of delivery is one component that should be reviewed. Based on parental goals, some families may choose to deliver within their community to be closer to home and familiar supports. Some families may plan for delivery in a larger, tertiary care center with additional access to neonatology and potential life-sustaining measures. The mode of delivery is another key component to be evaluated. Although vaginal delivery has been previously considered the recommended mode of delivery in the setting of a lethal diagnosis, a cesarean section may be considered in some situations. Women may request a cesarean section with the goal of delivering a live infant. , Parents were noted to be dissatisfied with care in situations where physicians declined to perform a cesarean section when requested at the time of delivery.
Reviewing steps of resuscitation with the family and determining how these potential actions and interventions fit with their goals should be completed prenatally whenever possible. For families planning on a focus on comfort after delivery, it is important to discuss anticipated symptom management needs. , Plans for memory-making activities such as photographs, hand and foot prints, and molds may also be coordinated prior to delivery when possible. Communication of intrapartum plans should be shared with all members of the care team who may participate in the delivery and provide ongoing care to ensure parental wishes are met.
Ongoing Decisions: The Unexpected and the Uncertain
Recognizing the limitations of prognostication that remain despite technological advancements, preparing families for unexpected outcomes is an important step ( Table 91.1 ). Introducing families to potential decisional points they may face in the future allows parents the opportunity to consider these complex choices in a more controlled setting. This may include reviewing potential ongoing interventions or familial wishes for discharge with hospice support if feasible. , Diagnosis of a life-limiting or life-threatening prenatal diagnosis forces parents to make complex and often unexpected choices regarding the care of their child. Palliative care provides longitudinal and interdisciplinary support to families and individualized guidance centered on goals of care throughout the decision-making process. , , , ,
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Acute Neonatal Intensive Care Phase
Continuation of Clinical Case Report
Cynthia decides to maintain her pregnancy and delivers her baby boy, named Jacob, at 28 weeks’ gestation. Upon birth, Jacob’s multiple congenital anomalies are confirmed, and he is intubated, stabilized, and transferred 2 hours away to the only specialized children’s hospital in the state. During Jacob’s first week of life, he is extubated and placed on nasal continuous positive airway pressure , and feedings are started via nasogastric tube. Cynthia is still recovering from childbirth and is relieved that Jacob is stable. However, she is struggling to care for herself, Jacob, and her other two children, who are temporarily staying with her mother 2 hours away. She is on the waiting list for a room at the Ronald McDonald house and is currently residing in the NICU at the bedside. The palliative care team is now involved and is starting to talk to her about her goals of care given Jacob’s life-limiting prognosis and the likely need for chronic feeding and respiratory support. On day of life 8, Jacob develops a distended abdomen, and a necrotizing enterocolitis diagnosis is confirmed. His congenital anomalies are now complicated by surgical resection of the bowel and short-gut syndrome. Cynthia was just beginning to accept the reality of a complex, chronic illness and was hopeful to have time at home with Jacob and her family. Now, she is struggling to make decisions given the sudden change in Jacob’s clinical course.
NICU Hospitalizations and Infant Death in the United States
NICU admission rates have steadily increased over time. In 2012, there were 77.9 NICU admissions per 1000 live births and a substantial increase in admissions for infants born weighing at least 2500 g. Therefore more and more parents are experiencing the stress of a NICU hospitalization. In 2017 more than 22,000 infants died in the United States, with the majority of infant deaths occurring in an intensive care setting. As with perinatal mortality, there are known racial disparities within infant mortality. Parents of non-Hispanic Black infants were twice as likely to suffer from infant mortality (11.4 per 1000 births) than were parents of non-Hispanic White infants (4.9 per 1000 births). These significant disparities must be addressed in the provision of culturally appropriate palliative and end-of-life care for families in the NICU. Clearly, NICU parents are a growing group within NICU family-centered care who require intensive support throughout a NICU admission.
Parent Experiences and Needs in an Uncertain NICU Environment
NICU patients commonly oscillate between periods of stability and then intense periods of uncertain survival. During the ups and downs of NICU illness trajectories, parents of seriously ill infants bear many roles such as caregiver, advocate, and decision-maker, while maintaining hope and managing uncertainty related to their infant’s prognosis. , Parents of infants in the NICU are heavily burdened psychologically, psychosocially, financially, spiritually, and physically because of the heavy demands associated with caring for a seriously ill infant and maintaining life outside of the NICU. These demands place parents of infants in the NICU at a higher risk for and prevalence of anxiety, depression, stress, acute stress disorder, and posttraumatic stress disorder. Therefore, it is critical to understand how neonatal healthcare providers may better support parents of seriously ill infants, and in particular, those infants and parents who receive end-of-life care.
Parent Support Needs
Parents of seriously ill NICU patients experience unique needs in order to develop their identity as a parent and meaningful memories as a family. Because of the inherent uncertainty in NICU patient survival and discharge to a home setting, NICU providers should optimize the quality of time and provide opportunities for memory making regardless of prognosis. For infants who die in the NICU, these patients and families experience life together within the intensive or acute care setting. , This presents limitations for parents who wish to involve other children and extended family members in their infant’s care, because there are often NICU policies preventing young children or numerous family members from visiting the NICU. Privacy is a priority for parents as they anticipate the death of their infant and wish to make the most of the limited time they have together. If the NICU is an open unit with multiple bed spaces in one room, transferring the patient to a private room where the restrictions on visitors are not as rigid may be an appropriate alternative for inpatient end-of-life care.
Developing and maintaining the parent role in an unnatural setting such as the NICU requires careful consideration and communication from the neonatal medical team. Providing “normal” parenting opportunities through end-of-life care, such as holding the infant and providing hands-on care,is extremely important to bereaved NICU parents as they cling to the limited memories of their infant after death. , Parents of seriously ill infants prefer communication from healthcare providers that is compassionate, sensitive, kind, sincere, nonjudgmental, and sympathetic to the stresses parents must manage while their infant is hospitalized in the ICU. Parents of infants in the NICU also reported the need for healthcare providers to take care of parents during the NICU admission by giving “permission” to leave the bedside and engage in self-care without feeling like a bad parent. , Outside of the neonatal medical team, bereaved parents have reported friends, family, and religious or community groups as supportive. However, other NICU parents are seen as especially supportive because of the ability to express empathy and seek support from those who “know their road.”
Cultural Differences in Parent End-of-Life Experiences
Because of the known racial disparities in perinatal and infant mortality, it is critical to understand cultural preferences in the NICU. Brooten et al. found racial differences in what parents did not find helpful from healthcare providers near their child’s death. White and Hispanic parents reported insensitive and nonsupportive staff as the most unhelpful characteristics, whereas Black parents reported conflict between providers and parents as the least helpful. Davies et al. explored the palliative care experiences of Mexican and Chinese American parents and found less optimal patterns of communication, including no information or basic information regarding prognosis of the child’s health status. This resulted in parent frustration, anger, and sadness. If interpretation is required, the interpreter must be trained to provide accurate information to the families, even if it is “bad news.” Providing honest, compassionate communication to families is essential to build trust between the medical team and the family. Lack of accurate interpretation could result in the perpetuation of cultural barriers and make the already tragic experience worse for these vulnerable parents.
Decision-Making: Central Role for Parents in the NICU
Decision-making is often shared between the parents and neonatal medical team. High-quality communication from the medical team is critical for parents to join in informed, goal-concordant decisions. Parents of infants hospitalized in a NICU preferred straightforward information that was presented in a positive way due to parental beliefs of medical miracles and the importance of maintaining hope in the decision-making process. , However, the fast-paced clinical changes and urgent nature of decisions make this a difficult process for clinicians and parents. Parents often receive updates and prognostic information in clinician-parent conferences. During these conferences, parents are presented with complex clinical information and sometimes uncertain prognoses.
Conflicts Between the Healthcare Team and Parents
Parents often have different opinions and beliefs about survival of preterm infants than their neonatal providers do. Boulais et al. conducted a study comparing concern for infant mortality among perinatal and neonatal physicians and parents of infants who were discharged alive from the NICU. Physicians believed that parental concern for mortality increased with decreased gestational age. However, parents were just as worried for their infants no matter the gestational age of their infant. Parental concern for mortality was, however, associated with infant length of stay and the documentation of at least one discussion regarding infant mortality with physicians. It is clear that conversations with clinicians have an impact on family understanding; thus the first step to getting families and clinicians on the same page about mortality is to talk about it.
Determining prognostic information in the neonatal population is difficult for providers because they must forecast survival and quality of life without reliable data. However, neonatal providers are tasked with delivering prognostic information to families and predicting the infant’s prognosis, short- and long-term outcomes, and quality-of-life issues. Boss et al. recorded and analyzed 16 parent-clinician conferences that discussed “difficult news” (e.g., severe intracranial hemorrhage, cardiopulmonary resuscitation decisions, or genetic diagnoses). Prognostic information was shared in most care conferences, and prognosis discussions were initiated by the provider. However, this prognostic information was delivered broadly rather than as detailed information related to the prognosis. Without detailed information, broad statements may be subject to different interpretations. Also, clinicians in this study explained that less than 25% of the cases discussed had a chance of surviving without serious complications; however, clinicians were twice as likely to be optimistic versus pessimistic when explaining the prognosis to parents. Parents and clinicians often walked away from these care conferences with different interpretations of the infant’s prognosis and quality of life, with parents having more of an optimistic view of their infant’s survival and quality of life.
Decision-Making at the End-of-Life
Parents describe an infant’s death as the most devastating and difficult experience they have faced. Parents may see their infant suffering from invasive procedures that are deemed futile and decide to limit medical treatment, withdraw life-sustaining technology (e.g., compassionate extubation), or use home hospice services. After infant death, parents must decide to see or hold their infant, donate organs, agree to an autopsy, and make funeral arrangements. These end-of-life decisions are particularly stressful, partly because they are often made in a NICU environment with unfamiliar people and noises, little privacy, and impending loss of their identity as a parent and the parent caretaking role. Neonatologists may support parents by offering to discuss the autopsy and organ donation processes with them before a precipitous decision must be made.
Parents often struggle with the “what-ifs” after infant death. That is, parents may report feeling at peace with the death but continue to wonder what the outcome would have been if different interventions were attempted at different times. Brooten et al. explored parents’ retrospective reflections on what they had wished was done or not done during their child’s death in the NICU or pediatric intensive care unit. Mothers wished to have spent more time with the child, held the child more, and selected a different treatment course. Fathers wished to have spent more time with their child and monitored their child more closely. These regrets emphasize the importance of providing high-quality, supportive communication surrounding decision-making, recognizing the importance of the parent role, and creating meaningful opportunities for parents to develop memories with their infant in the NICU. Neonatologists may also support parents by providing a postdeath conference and discussing autopsy reports and the events leading up to their infant’s death to clarify any questions and provide an element of clarity to the parents’ grief process. , , ,
Sources of Support for Parents During End-of-Life Decision Making
Use of a Question Prompt List to Empower Parents
The use of a question prompt list (QPL) in the processes of information gathering and informed decision-making is one strategy to engage parents in informed decision-making and provide goal-concordant care. A QPL is a suggested list of questions for the patient or caregiver that guides communication with the healthcare team. Lemmon et al. developed a QPL for NICU families to use in preparation for clinician-parent care conferences. This QPL was developed using audio-recorded NICU care conferences with parents of infants treated for therapeutic hypothermia and was universally accepted by NICU clinicians and parents. An example QPL item on being a NICU parent is the following: “What is the best way for me to participate when the team makes the plan for the day (rounds)?” Parents who used a QPL found that it was useful and facilitated more prepared answers from their neonatology team. Implementing decision-making supports such as a QPL may empower parents to ask questions that are most important to them during a time when they may be too overwhelmed to develop a list of questions on their own.
Perinatal Palliative Care as an Added Layer of Support in the NICU
Palliative care is an interdisciplinary specialty that aims to provide the best possible quality of life for seriously ill infants and their families and involves comprehensive treatment of suffering. By definition, serious illness is “a health condition that carries a high risk of mortality and either negatively impacts a person’s daily function or quality of life, or excessively strains their caregivers.” There are three general categories of patients who receive PPC in the NICU: (1) newborns born at the threshold of viability, (2) newborns or infants born with birth anomalies that may threaten vital functions, and (3) newborns or infants who are receiving intensive care but become burdened with interventions that no longer seem beneficial and are instead only prolonging the infant’s dying or causing suffering. For infants and their parents to receive the maximum benefit, PPC should be initiated at the time of diagnosis and provided concurrently with curative efforts. , Early integration of PPC is a great opportunity to build trust with the medical team, and PPC support may assist parents with eliciting goals of care and planning questions and discussions with the neonatal medical team during care conferences. Palliative providers specialize in eliciting care preferences by using excellent communication skills, supporting the decision-making process, managing distressing symptoms, helping to increase the continuity of care, providing family support, and enhancing quality of life in all realms of suffering including physical, spiritual, psychological, and psychosocial suffering. PPC may facilitate the opportunities for parents to make memories with their infant by coordinating resources, eliciting parent preferences for care, aligning care with their preferences, and facilitating cultural rituals that are important to families.
However, PPC is commonly avoided in the NICU or integrated only near the end of life when death is imminent. One common explanation for the avoidance of PPC in the NICU is the misconception that palliative care means giving up on aggressive treatment and transitioning to exclusive end-of-life or comfort care. However, bereaved parents have reported positive experiences with PPC teams before infant death in the NICU. For example, parents wished they had involved the PPC team earlier and more often because the palliative care team acted as a sounding board for their questions and concerns and orchestrated meaningful opportunities for them to create memories with their infant when survival was uncertain. For NICU patients who did receive PPC, PPC teams were most often consulted for communication needs or aligning care with the goals of care and wishes of the family. Neonatologists have reported the value of communication expertise that palliative care providers brought to their complex clinical cases.
Chronic Critical Illness Phase
Continuation of Clinical Case Report
Cynthia has returned to work part-time. She and Baby Jacob’s siblings faithfully visit the NICU every weekend because of work schedules and financial hardships associated with the 2-hour commute to the hospital. Baby Jacob is recovering from acute necrotizing enterocolitis. His feeds are through a gastrostomy tube. Due to having undergone multiple bowel surgeries and a subsequent short gut, he receives calories through total parental nutrition in a central line despite multiple attempts to advance to full feeds. Cynthia expresses disappointment that he isn’t able to “enjoy eating” or “receive comfort from either breastfeeding or even a bottle.” He depends on a tracheostomy with a back-up ventilator rate for respiratory support. Due to the home health nursing shortage in his community, Baby Jacob remains in the hospital setting for his first 11 months of life. Cynthia is struggling with the reality that he may reach his first birthday never having been home. His siblings continue to ask, “When will Baby Jacob come home?”
Epidemiology and Definition of Chronic Critical Illness
Advances in medicine and development of biomedical technology have dramatically shifted the epidemiology of pediatric outcomes in the past 2 to 3 decades. Life-prolonging interventions have resulted in the survival of infants with previously terminal medical conditions. Infants are now surviving with chronic critical illness, often through the support of multiple advanced medical technologies and extended hospitalizations to include intensive-care stays ( Table 91.2 ). Although less than 1% of the United States pediatric population consists of children with medical complexity, this population accounts for as much as one-third of total pediatric healthcare spending and almost half of pediatric hospital charges. ,
