Category
Recommendation
Patient – provider communication
Offer conversations about EOL earlier in the disease course and to patients/parents irrespective of disease complexity
Consider scheduling a unique visit to discuss EOL and advance care planning issues
Normalize advance care planning discussions
Acknowledge a patient-reported history of previously having “beaten the odds”
Repeat discussions as necessary (e.g., after a significant change in clinical status)
Provide information in the simplest and clearest language possible
Ensure sensitivity to a patient’s cultural background
Be aware of the cognitive and developmental abilities of an individual patient and tailor EOL discussions accordingly
Provide information in verbal and written format (consider developing education materials specific to advance care planning for patients with congenital or pediatric heart disease)
Offer a range of time regarding prognosis and acknowledge uncertainty
Ensure a clear discussion of the expected outcomes of cardiopulmonary resuscitation
For patients with ICDs, discuss the implications of device deactivation
Facilitate the completion of advance care planning documents (e.g., naming of substitute decision-maker)
Emphasize that the team will continue to provide care in the event of a decision to cease life-sustaining medical treatment
Provide ongoing support and communication following a referral to palliative care
After a patient’s death, follow up with a telephone call and/or condolence card
Interdisciplinary care
Consider identifying a primary attending physician for an inpatient facing death who can provide continuity of care (and communication) in settings in which attending staff typically rotates
Ensure that EOL discussions are well-documented so that all members of the outpatient and inpatient care team are informed
Include health providers on the care team who can attend to the physical, emotional, and spiritual needs of patients facing death
Consider referrals to palliative care
Consider bioethics consultations in challenging situations
As necessary, use interpreters to ensure that information is provided in the patient’s language
When an adult with CHD dies, consider informing that patient’s pediatric providers
Acknowledge emotional reactions within members of the team when a (young) patient dies and encourage self-care practices
Table 9.2 offers recommendations unique to the pediatric cardiology setting; strategies focus on patient-provider and parent-provider communication. Below, we highlight the importance of effective communication and suggest caution when considering the role of technology at the end of a patient’s life.
Table 9.2
Recommendations for advance care planning and end-of-life care specific to the pediatric cardiology setting
Category | Recommendation |
|---|---|
Patient – provider communication | Do not exclude children from discussions of health and illness |
Do not assume that children are unaware of their poor health | |
Provide the pediatric patient with information regarding their medical condition, treatment options, and prognosis | |
Adapt communication to the chronological age and developmental level of the patient | |
Give children the opportunity to ask questions (with and without their parents present) | |
Encourage children and adolescent to participate in medical decision-making to the extent they are able and also desire to be involved | |
Be mindful of the age of consent, which varies across states and provinces | |
Parent – provider communication | Unless parents suspect their children do not want to talk about death, they can be encouraged to maintain an open dialogue about death and dying |
Develop a system by which interested parents can connect with other parents who are going through (or have gone through) a similar situation | |
Address parental anticipatory bereavement and grief | |
Consider offering parents the option to be present during any resuscitation efforts |
Focus on Communication
Greutmann et al. offer a conceptualization of “comprehensive care” in CHD [58], which is also applicable to patients with other forms of pediatric-onset heart disease. In their model, comprehensive care begins at birth, or even earlier in the case of prenatal diagnoses. Discussions regarding prognosis occur with parents of affected children, and adolescents are gradually included into discussions and the decision-making process. Advance care planning and EOL discussions may occur more frequently and formally in the adult care setting when cardiac complications (e.g., heart failure, arrhythmias) and hospitalizations become more common. A transition from active treatment to palliative care becomes appropriate for many patients.
Effective patient-provider communication is critical when facing death, regardless of patient age. Hsiao et al. interviewed 20 child and parent pairs in which the child (mean age = 14 years) had cardiac disease or cancer with a poor prognosis [62]. The following five domains of effective physician communication were identified by both patients and parents:
1.
strong relationship building skills that facilitated rapport and trust,
2.
demonstration of knowledge, competence, and effort,
3.
effective information exchange,
4.
availability of physician to child and parent, and
5.
appropriate level of child and parent involvement in discussions and decision-making.
Adults with CHD have similarly identified honest communication as a priority for effective EOL care [54].
Advance care planning and EOL discussions should always be tailored to the cognitive and developmental abilities of an individual patient. Further, it is wise to ensure comprehension of medical decisions and EOL discussions by asking patients to repeat a summary of what was said or decided. Such practices are likely even more important when working with patients with CHD in the pediatric or adult setting, because individuals born with CHD are at increased risk of cognitive and developmental disorders [63]. The term “life-shortening medical condition” can be a helpful term for many patients [59]. It is also important to attend to the potential religious and spiritual facets of death, dying, burial or cremation, and mourning for many individuals. Guidance from spiritual care or religious leaders can be extremely helpful to guide the timing and context of many EOL interventions.
The specific components of EOL discussions will vary depending upon a patient’s unique cardiac condition. For example, for patients at risk of arrhythmic death (e.g., patients with inherited arrhythmias, hypertrophic cardiomyopathy, certain forms of CHD), a discussion of the anticipated outcomes of cardiopulmonary resuscitation is critical. For pediatric patients listed for heart transplantation, the decision not to proceed with resuscitation is not uncommon [23]. A discussion of the medical, ethical, and legal implications of device deactivation is also appropriate for patients with implantable cardioverter defibrillators (ICDs) [20, 64, 65].
Given obvious room for improvement in the EOL care of adults with CHD, it is a positive finding that the majority of surveyed adult CHD providers want more information and resources about advance care planning and communication strategies for EOL or advance care planning [58]. The “Ask-Tell-Ask” framework [66] has been recommended for use with adults with CHD [59]. It is also applicable to adolescents with heart disease or family members of patients of any age.
The first step is to ASK what the patient (or parent) understands about an issue (e.g., heart disease diagnosis, treatment plans, prognosis, cardiopulmonary resuscitation) and what they wish to learn.
Second, a provider should TELL information that is requested by the patient/parent or that the provider knows should be communicated to them.
Third, the provider can again ASK for a confirmation of understanding and whether there are additional questions.
Consider the Role of Technology
In-hospital deaths among children with advanced heart disease and adults with CHD are frequently accompanied by highly technical care, including cardiopulmonary resuscitation, mechanical circulatory support and ventilation [57, 67]. Further, most in-hospital deaths occur in intensive care units [57, 67]. Guidelines for palliative care in pediatric oncology emphasize the avoidance of curative treatment after the point at which cure is no longer possible [39]. A similar approach is reasonable in the cardiac setting. The Academy of Pediatrics emphasizes that technology should only be used when the benefits outweigh the harms [25, 26], and this principle certainly holds equally true for adults. Further, at the time of evaluation for high-risk interventions, such as ventricular assist devices and/or transplantation, a concurrent palliative care approach should be undertaken [24].
A study of the in-hospital deaths of 111 children with heart disease (mean age = 4.8 months; age range = 1 day to 20.5 years; median hospital stay = 22 days) revealed that “highly technical care” was common [67]. Specifically, within 24 h of death, 92 % received ventilation, 46 % had extracorporeal membrane oxygenation (ECMO) or a ventricular assist device (VAD), 23 % had peritoneal drains, and 19 % had gastrostomy tubes. Approximately two-thirds of patients died after disease-directed interventions were withdrawn, and one-quarter died during resuscitation. Medical records documented parental presence at bedside for 83 % of deaths. A documented EOL discussion occurred with three-quarters of patients and 16 % had received palliative care consultation. These results mirror the retrospective study of 48 adults with CHD in which documented EOL discussions with patients were rare but mechanical ventilation and resuscitation efforts were common [57]. Thus, clear discussions about the use of technology should become part of routine advance care planning discussions, particularly because the general public tends to over-estimate survival following out-of-hospital cardiac arrest and inpatient resuscitation [68, 69].
Formal palliative care consultations have been associated with fewer medical procedures (including cardiopulmonary resuscitation attempts) in neonates, children, and adults [38, 57, 70]. Unfortunately, palliative care referrals remain uncommon, especially for neonates and children dying from circulatory disease [28].
Conclusions
Though the basic principles of advance care planning and EOL care apply to patients with both acquired and congenital/pediatric heart disease, there are several unique considerations for the latter group. Within the pediatric cardiology setting, a discussion of palliative care may begin as early as the prenatal period. For children and adolescents with complex heart disease, an approach to advance care planning and EOL discussions must take into account the cognitive and developmental abilities of each individual patient, while at the same time recognizing the prominent role of the parent in the decision-making process. Though the majority of infants born with CHD now reach adulthood, CHD represents a chronic life-shortening medical condition such that many patients remain at risk of morbidity and premature mortality in adulthood. Unfortunately, advance care planning and EOL discussions have not been commonly documented among adults with CHD. For clinicians in both pediatric and adult cardiology settings, an emphasis on effective communication and interdisciplinary care is recommended. Clinicians should also take care to minimize the use of unwarranted highly technical care. Further, decisions not to proceed with high-risk interventions should be supported.
There is also ample room for empirical investigation in this area. Researchers are encouraged to develop and evaluate strategies to overcome barriers and improve advance care planning and EOL discussions with children, adolescents, and adults with congenital or pediatric-onset heart disease. Such strategies may include the development of patient and family educational materials, provider communication skills training, prognostication tools, and the identification of the optimal timing of referral to and role of interdisciplinary team members including palliative care, bioethics, spiritual care, and mental health providers. The ultimate goal of combined clinical and research efforts is to improve advance care planning and EOL care for this deserving group of patients.
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