Children on HMV via tracheostomy are a heterogeneous child population with complex chronic conditions and reliance on invasive ventilation. Although this technology aids in sustaining the child's respiratory function, it does not eliminate the need for continued ACP for those with severe comorbidities or life-limiting conditions. ACP is the process that guides patients to an understanding of their diagnosis — and, if possible, their prognosis — and then to consider the decisions and treatment options they will face in the future. In the case of someone whose personal goals and values cannot be known, a surrogate decision-maker should make choices in accord with that person's best interests (13
). While ACP should be goals- and values-oriented, discussions of possible choices at the end of life are also necessary. Previous studies have demonstrated that parents of children with chronic conditions feel that ACP is or would be beneficial and wish to know of available options at the end of their child's life, including those that forgo interventions (14
Our review demonstrated that unscheduled hospitalizations, ICU stays, and cardiopulmonary arrests were relatively common among our cohort of children on HMV. In addition, an earlier review showed that many of their deaths were unexpected and not the result of the progression of their underlying diseases (3
). Thus, ACP is especially relevant to this population. End-of-life discussions were documented in about three-fourths of our cohort. Many of these discussions were first documented after the child experienced an acute deterioration and during a hospitalization. Some of the discussions took place after the child suffered an arrest or just prior to the child's death. This may suggest that earlier opportunities were missed, although it is also likely that more discussions occurred than were documented. This timing also reflects our experience that many families were not open to candidly discussing end-of-life issues during times of relative stability. However, some families were willing to limit future care that was unlikely to alter their child's course. These decisions were most commonly made after the children had already experienced a decline in clinical condition. Our review also demonstrated that close to one quarter of those families who chose to limit treatment efforts later elected to rescind the limitation. These two factors — resistance to discussing future end-of-life decisions before deterioration and the reversal of earlier limitations after the child survives a critical illness — may indicate the existence of a survival plateau. Such a plateau was first described by Simonds and colleagues, who observed it in patients with Duchenne muscular dystrophy after initiation of non-invasive ventilation (17
). Birnkrant and Noritz later referenced this phenomenon as an obstacle to gaining families’ assent to palliative care for their children with progressive neuromuscular disease on non-invasive ventilation (18
). It is important to note, as well, that ventilatory support was not withdrawn from any child; this may suggest that the option is not being offered, or it may highlight the difficulty of reversing prior fundamental directions of care.
In part due to this review and other reflections on our practices, the CHLA HMV program has refocused its efforts on engaging families in ACP at the time of and after initiation and before critical illness. Ideally, ACP should begin prior to CRF, initiation of chronic ventilation, and critical illness (19
). However, CRF often results after an acute illness forces an at-risk child into non-compensable respiratory insufficiency and it is no longer possible to wean the child from mechanical ventilation (6
). In either situation, we consider conversations about HMV to be part of ACP for children with CRF. ACP is no less important after HMV has been initiated than it is when HMV is addressed well in advance.
In all cases, we now provide patients’ families with anticipatory guidance related to the limitations and risks of HMV (see Box 1) beginning with initial conversations about the nature of HMV and whether it is a good option for their child. To help ensure that expectations are realistic, we attempt to ensure that caregivers receive and understand this guidance before HMV initiation. We highlight these points again during the final discharge-planning meeting and, as part of completing the written discharge checklist, caregivers must sign a document acknowledging that they have heard the points discussed.
While much of ACP revolves around an exploration of families’ goals for their children and the values that shape those goals, candid discussions of likely clinical scenarios should also be undertaken. Admittedly, it is difficult to address all such scenarios; the choice of which ones to cover depends largely on the individual child. For instance, if the child has chronic renal insufficiency, chronic renal replacement therapies should be discussed before the onset of end-stage renal failure. At minimum, for children at risk of acute deterioration, CPR should be discussed. In order for families to assent to or forgo these measures, they must understand that chest compressions with possible defibrillation and cardiac medications are the default in the face of cardiac arrest, that CPR is often successful only when the arrest is triggered by an acute reversible cause, and that non-fatal arrests can lead to new neurologic morbidity (22
). After initiation, we assume that a family's choice of HMV is an implicit decision for future interventions and resuscitation in the face of acute, unexpected deterioration. Teaching CPR and management of airway emergencies is an explicit recognition of this choice. Since the causes of acute deterioration are often initially unclear and may be reversible, we believe that this conclusion is pragmatically and morally defensible, even for children who are medically fragile or who have life-limiting illnesses.
Reconciling the preference for resuscitation and extraordinary care can become a challenge when the child's underlying condition declines more significantly or the care team concludes that the child's critical illness is likely not reversible. Thus, this preference, while initially appropriate, often needs to be gently recalibrated as circumstances and family readiness change. This approach is reflected in our study finding that clinical instability often prompts endof-life discussions. However, our review has also compelled us to proactively, rather than reactively, strive to gauge our families’ readiness for ACP and to document all discussions, even those that do not result in a decision. When we engage in reactive discussions, we risk limiting ourselves to addressing narrow, immediate medical concerns during crises rather than exploring larger goals of care in anticipation of likely or inevitable outcomes (23
). Other potential prompts for readdressing end-of-life goals include more frequent or more severe acute illnesses, development of a new comorbidity, and the child approaching the upper range of the anticipated lifespan. During periods of instability, the situation's gravity trumps family readiness, and the physician must alert the family of significant concerns related to morbidity or death.
If the child is clinically stable, it is not necessary for families to pre-emptively articulate their preferences for future scenarios. Rather, it is sufficient for them to gain a realistic understanding of their child's condition and risks and think through the values and goals that will likely shape their decisions. To help with the latter, one approach is to regularly recommend to families that they contemplate what they want for their child when he or she is in a period of relative health and, in the opposite scenario, what they want when the child is threatened with serious acute illness or worsening of an underlying condition. This approach permits the physician to gauge a family's readiness for further ACP — if, of course, the family is willing to share their thoughts with the physician. It also encourages families to reflect on the inevitability or the serious risks of their child's condition while also contemplating positive and hopeful goals or scenarios. The amount of time and effort medical caregivers should expend discussing scenarios of relative health versus illness depends on the child's condition and trajectory. As a child's condition worsens, ensuring that families are contemplating end-of-life goals and eliciting more specific decisions become more crucial. Regular encouragement to contemplate and possibly discuss these goals or decisions would ideally be offered during regular out-patient visits, but it could also be provided during hospitalizations.
Although end-of-life issues and options will have been raised earlier (for example, during discussions about initiating HMV, or during previous critical illness), ACP after HMV initiation is no less challenging. In the case of children on HMV who have life-limiting conditions or severe comorbidities, balancing the risks or inevitability of their condition with their family's wish to continue to support them through subsequent critical illnesses is a delicate matter. Constantly reminding families of their child's serious diagnosis or overwhelming them with negative anticipatory guidance risks destroying their hope and sabotaging the family-physician relationship. Yet, by communicating belatedly or in a manner that is too tempered, physicians risk under-informing families. Box 2 lists potential challenges to ACP that we and other medical caregivers of children with complex chronic conditions have encountered (17
). Challenges can stem from both families and health care providers. Becoming aware of these challenges is the first and most important step in overcoming them, and eliciting families’ hopes, goals, and fears is always beneficial. Some families refuse limitation of extraordinary measures at the end of their child's life. While some health care professionals may believe that this refusal prolongs the child's suffering, such decisions should be viewed respectfully if they are consistent with the family's ACP goals (28
). The emphasis of ACP should be placed not on eliciting a particular decision but on informing the family about events that could occur and on reassuring them that caring for their child will be the primary goal under all circumstances.
Our review of ACP for children on long-term assisted ventilation raises a number of questions that deserve further investigation. Future studies are needed to: survey HMV families and other health care providers to obtain their views on how and when ACP and anticipatory guidance are best undertaken; survey HMV families for information on any written advance directives they might have prepared before initiation of HMV, and on how HMV altered these directives; determine whether family readiness for ACP is complicated by a period of relative stability for the child after HMV initiation and/or by an expectation that HMV will be life-sustaining or prolonging; determine whether and how chronic ventilation alters perceptions of, and decisions for, subsequent invasive or extraordinary interventions during critical illnesses; ascertain whether the perceptions and decisions of these families are different from those of families whose children have complex chronic conditions but are not on long-term ventilation; and establish whether families of children on HMV because an acute illness has rendered them incapable of being weaned from invasive ventilation have different readiness for ACP or perceptions of subsequent interventions than families whose children have been proactively started on HMV due to their known chronic respiratory insufficiency. All of these questions would be best addressed prospectively and with a multi-institutional and diverse cohort.
While our review helps both to frame the importance of ACP and the difficulties it poses for children on HMV and to highlight several additional questions that deserve further study, it does have some notable limitations. First, we only reviewed HMV patients who died. We felt that these patients represented a reasonable convenience sample of patients who ought to have had ACP. However, there were certainly more non-deceased HMV patients with life-limiting conditions for whom ACP was necessary. Our convenience sample may have introduced selection bias to our review; and because 23 deceased patients were excluded, we may have introduced additional selection bias. Second, our review of ACP largely focused on end-of-life issues, though there can certainly be more to ACP. This is the consequence of end-of-life discussions being more consistently documented in the medical records than other ACP topics. Nevertheless, it skews our presentation of ACP and diverts the accompanying discussion away from other important ACP topics. Third, our lengthy study period covers a time during which the relative complexity of complex chronic conditions in children increased, home ventilators with continuous flow were introduced, and patient autonomy in decision making gained even greater prominence. However, we surmise that these developments did not significantly change the underlying situation or challenges for HMV patients with life-limiting illness. Throughout the period, a subset of HMV patients lived and died at the limits of medicine at that time. Fourth, as this was a retrospective study, included discussions were limited to those documented in our institution's records. It is likely that more discussions and, possibly, more arrests occurred than we could identify. Finally, it is difficult to know whether all of our recorded arrests were in fact events that warranted cardiopulmonary resuscitation, or if some were confounded by caregiver overreaction.