Abstract
Background
Pediatric Palliative Care (PPC) has been shown to offer a wide berth of supportive services for families as they navigate life with pediatric heart disease. PPC assists families in medical decision making, symptom management, emotional and spiritual resources, clarifying goals, educating families on medical and community resources, and advanced care planning.
Aim of review
The aim of this review is to examine the data that shows early PPC consultation at diagnosis, often even prior to birth, gives the best opportunity to access the multitude of services. Building early relationships during times of wellness allows the PPC team to be a strong and crucial support for families when critical illness or surgical needs arise. Many barriers prevent pediatric cardiologists and pediatric cardiovascular surgeons from incorporating these benefits for their patients. There is a national lack of access to PPC programs. Therefore, it is important to educate providers on the services PPC can provide so children with pediatric heart conditions will be able to live longer and better lives.
Key scientific concepts of review
We reviewed the current literature to summarize benefits of early introduction of PPC in complex cardiac conditions.
1
Introduction
Historically, generic terms such as “palliative” or “palliation” have been associated with only end of life care or non-intervention [ ], yet with the development of a multidisciplinary team dedicated to supporting patients, families, and staff, Pediatric Palliative Care (PPC) has now proven to offer much more [ ]. PPC is now recognized as a unique medical subspeciality which uses a multidisciplinary approach to improve quality of life (QOL), minimize pain and suffering, establish goals and preferences, assist in medical decision making advanced care planning, and improve interdisciplinary and family communication [ , , , ]. The role of early PPC in patients with complex heart disease has been described in the literature as both beneficial and underutilized [ , , , ]. This parallels the broad recognition that early involvement of PPC has increasingly become a standard component of most complex pediatric care management in children’s hospitals across the United States [ ]. The American Academy of Pediatrics (AAP) recommends concurrent use of PPC services along with curative care at the time of complex medical diagnosis with continued involvement through the progression of the disease [ , , , ]. Approximately 1 million children in the US are living with complex congenital heart disease (CCHD) [ ]. Cardiac disease remains a leading cause of death in pediatrics, with the 1-year mortality rate estimated between 10 % to 24.8 % [ , ]. Yet only an estimated 16 % of pediatric patients with CCHD had a PPC consult [ ], with a total of 8 % of all PPC consultations being for cardiovascular disease [ ]. Another study found the number of children eligible for PPC services with cardiovascular disease was significantly higher than those who received consultation [ ]. PPC benefits for patients with CCHD are improving over time, yet still fall behind other service lines, such as pediatric oncology [ ].
2
Methods
Review of the literature was performed in the PubMed database using the Boolean search term phrase: ((cardiology OR congenital heart disease OR heart diseases) AND (palliative care OR palliative medicine)) AND (child). Articles were filtered to include only those with ages less than 18 years old and studies which were in the English language.
2.1
Review
There have been many documented benefits to early implementation of PPC. Studies have shown PPC can result in fewer in-hospital days [ , , ], fewer invasive interventions that may not benefit patients [ , , ], decreased maternal anxiety [ , ], and improved communication among family members and the healthcare team [ , , ]. [ , , , ] PPC teams are trained to help families anticipate and process stress, anxiety, grief, and post-traumatic stress disorder which are common among pediatric intensive care unit (PICU) patients [ ]. Goals of care discussions with the family regarding pathophysiology as it relates to medical decision making and psychosocial support are the services PPC teams frequently provide [ , ]. Preparing a family for what a life with increased reliance on medical technology may look like, including hospitalizations and complications, is also an important component of PPC [ ]. A prospective randomized trial showed that mothers of infants with single-ventricle heart disease had significantly higher levels of prenatal anxiety and depression at baseline and that mothers randomized to include early palliative care reported less prenatal anxiety, more positive thinking, and more effective communication [ ].
The cost of medical care can burden a family whose child is experiencing CCHD. Patient care without PPC involvement was associated with significantly higher costs on the day of death and 7 days prior [ ]. PPC consults showed a significantly lower median length of stay and average daily charges compared to patients without PPC consults [ ]. Children with PPC spent fewer days in the hospital, received fewer invasive interventions, and fewer patients died in the PICU [ , , ]. PPC can provide insight into options for a patient that are less invasive, and that enhance quality of life [ ]. Most deaths (estimated 70 %) related to heart disease happen in the hospital, with 80 % of those occurring in an intensive care unit setting [ ]. In the past, the quality metric most valued was inpatient mortality. PPC has been shown to increase not only the quantity of life but also the quality [ , ]. More studies are exploring tools that can objectively measure QOL outcomes [ ]. Another less emphasized benefit of PPC consultation is to educate and ease moral distress of staff [ , ]. PPC teams are trained in facilitating ethical concerns and promoting self-care of families and staff [ ].
There are several gaps in the current healthcare system related to PPC. There are inconsistent methods for identifying and referring children to PPC services [ ], false assumptions that palliative care is only for end of life care [ ], and the lack of existing frameworks for hospitals to create a comprehensive, longitudinal palliative care program that supports patients and their families [ ]. Also, there is a lack of guidelines on how and when to involve PPC [ ]. Triggers for standard PPC consults specifically related to CCHD may include single ventricle physiology, any CCHD with genetic syndrome, heart failure, transplantation consideration, future or past need for extracorporeal membrane oxygenation/ventricular assist device, need for defibrillation, and controversial or unclear cases [ , ]. Screening trigger tools, such as champion based models or nurse-driven screening, have been shown to decrease the time to consult [ ]. There is a lack of PPC accessibility in the US. Only 2/3 of cardiologists reported having access to PPC services [ ]. Palliative care training is not routinely offered to pediatric cardiology fellows, with an estimated 30 % of pediatric cardiologists reporting formal training [ , ]. A dedicated PPC team is well-trained in leading a family through difficult and lengthy conversations.
Families are encouraged to continue to hope for and pursue curative therapies [ , ]. Yet, surveyed cardiologists reported that one of the barriers to seeking PPC consults is that they will be perceived as undermining the hope of families or “giving up” [ , ]. While it is easy to see the value in PPC if a patient is dying, other supportive services that PPC offers are valuable for teams [ ]. These include providing emotional, psychosocial, and disease-specific education to support caregivers and staff [ , , , , ]. PPC patients at end of life will receive symptom management and bereavement services, while patients pursuing interventions will work with a PPC team to provide advocacy and assist in education and medical decision making [ ].
Many pediatric cardiologists are uncertain about the optimum time to introduce PPC. A survey study of pediatric cardiologists and cardiac surgeons across 19 centers showed that over half felt PPC was offered “too late,” and no respondents thought that PPC was offered “too early” [ ]. The PPC community suggests the following times to include PPC consults ( Fig. 1 ): 1) when being hospitalized for any reason, 2) when in recovery from an acute decompensation, 3) prior to surgical interventions, or 4) if there is an increase in symptom burden indicating chronic decompensation [ ]. There is evidence that length of PPC involvement improves QOL outcomes, also supporting the benefit of early consultation [ ]. Advanced planning discussions with families develop over time as a supportive relationship evolves [ ]. This discussion may take place over a long period of time, which is why there is an emphasis on early PPC consultation, ideally at diagnosis [ ]. The purpose of this longitudinal relationship is to prepare families for possible future decision making, while keeping interventions consistent with family goals, possibly avoiding burdensome procedures [ , ]. Yet, one study showed in pediatric patients who died of advanced heart disease, the median time of PPC involvement prior to death was only one month [ ].
To increase access to PPC for everyone, one needs to start with a needs assessment. This should include physicians, nurses, therapists, social workers, chaplains, administration, child life specialists, as well as input from bereaved families [ ]. The Bridge Program at USA Health Children’s and Women’s Hospital in Mobile, AL, recognizes these gaps and utilizes a multidisciplinary team to support women and families expecting infants with critical diagnoses [ ]. The Bridge Program is a team that specializes in PPC and recognizes the emotional impact of high-risk pregnancies. They provide extensive, collaborative care from diagnosis through medical decision-making points in a patient’s life and, ultimately if needed, potential bereavement. The Program is grounded in Family-Centered Care (FCC) principles, which emphasize respect for cultural diversity, informed decision-making, and collaboration. The Program is composed of nurses, physicians, and nurse practitioners specially trained in PPC, maternal-fetal medicine (MFM) physicians, and neonatologists available for consultation. Child life specialists, behavior health professionals, chaplains, and case managers complete this multidisciplinary support service line. The goal of this paper is to share the Bridge Program’s framework so other children’s hospitals can adapt this PPC approach to better support complex cases ( Table 1 ). This paper details a CCHD case and demonstrates when and how the Bridge Program assists in providing families with quality evidence-based PPC. The use of the term “Bridge Program,” as opposed to Pediatric Palliative Care Service, is helpful to describe the purpose (to help transition or bridge the patient from life stage to stage) and also gently avoid stigma from use of terms such as “palliative” or “palliation”, which can be associated with “giving up,” and this may damage an early trust bond. The feedback from the community has encouraged this early Bridge Team consult. We value building strong bonds and are grateful to have the honor of walking alongside families facing serious illness.
