Anticipatory guidance on the risks of unfavorable outcomes among children with medical complexity

Recent decades have seen profound improvements in the care and survival of children with medical complexity—that is, children with complex chronic conditions (CCC) and functional limitations, who require substantial and on-going medical and community-based care.¹ Nevertheless, many of these children remain at disproportionate risk for unfavorable health-related outcomes due to their medical fragility and prodigious care needs. These outcomes include, but are not limited to, acute-on-chronic illness, hospitalization, and unexpected death. Understanding these risks as a provider is essential to avert preventable ones and to anticipate those that may not be preventable. Likewise, families of children with medical complexity need to be informed beforehand about these potential risks. Such preparation can be considered a form of anticipatory guidance for these families and should be a basic part of optimal care for this vulnerable population.

For those caring for the general pediatric population, anticipatory guidance is a familiar educational practice that seeks to proactively prepare families for what they should expect in the future and how to meet the child’s needs at her current/next developmental stage.^2,3,4 Common topics focus on well-child and preventive health, including feeding, sleeping, growth, immunizations, safety and accident prevention, behavior, puberty, and sex education. Parents have reported increased healthcare satisfaction when anticipatory guidance is provided.⁵ Understandably, some topics need to be modified for children in different circumstances or settings. Similarly, for the families of children with medical complexity, anticipatory guidance is equally important. However, the topics require adaptations and additions to be germane because the complex child’s developmental and medical trajectories may be significantly different than those of the typical child. Among other topics, anticipatory guidance for families with children with medical complexity should include information on their risks of unfavorable health-related outcomes.

Characterizing the risks of this heterogeneous population was perhaps first attempted in earnest at the turn of this century when several insightful investigators started publishing groundbreaking research on these children and the impact of their chronic conditions. The first was Chris Feudtner and colleagues’ seminal study of pediatric deaths in Washington State between 1980 and 1997 attributable to underlying CCCs.⁶ Their paper highlighted that while pediatric mortality rates were declining, the proportion of deaths due to CCCs were substantial and growing. Importantly, Feudtner et al’s work also gave us a terminology to collectively conceptualize these patients’ severe chronic conditions and a method to identify them. While Feudtner’s 2000 paper and subsequent works^7,8 highlighted the increased risk of mortality among children with CCCs, Dosa and colleagues focused on their high risk of severe acute illness.⁹ Prospectively studying a 17-county region in upstate New York, they determined that children with chronic conditions had a more than 3 times greater risk of unplanned PICU admission compared to children without a chronic condition. Furthermore, children dependent upon various medical technologies, such as mechanical ventilation, tracheostomy, intravenous medications or nutrition, tube feeding, and implanted cardiac pacemakers, had a more than 300 times greater risk. Almost half of these admissions were estimated to have been related the children’s chronic conditions. While some of these admissions were considered preventable, most were characterized as unavoidable. These early studies conveyed what providers were undoubtedly observing in their hospitals and clinics—children with medical complexity were a growing group of vulnerable children with risks that far exceeded those of children who were not medically fragile.

Since then, numerous investigators have studied children with medical complexity and their increased risks for severe illness, hospitalization, and death. One such study by Havers and colleagues¹⁰ recently was published in the March issue of The Journal. This study focused on children with neuromuscular and neurodevelopmental disorders, a relatively large group of patients that often meet criteria for being medical complex, and their risks for severe respiratory infections, which are a frequent cause of acute illness among children with medical complexity. Using a sizeable cohort drawn from multiple states, Havers et al compared the rates of respiratory infection-related hospitalization among children with neurologic disorders to those of the general pediatric population. They found that 12% of hospitalizations of children with neurologic disorders were for respiratory infections; a quarter of these hospitalizations were repeat respiratory hospitalizations among less than 4% of these children. Children with neurologic disorders had over a 5 times greater relative rate of respiratory infection-related hospitalization than the general pediatric population. Children with specific neurologic disorders, such as cerebral palsy, muscular dystrophy, motor neuron diseases, degenerative diseases, metabolic diseases, and chromosomal disorders, had even higher relative rates of hospitalization. Relative rates of death during respiratory infection-related hospitalizations were likewise substantially greater among children with neurologic disorders. While this study did not delineate the various infectious etiologies of these severe respiratory illnesses, they undoubtedly included an array of common organisms that lead to upper respiratory tract infection, tracheobronchitis, and other non-severe respiratory illnesses in most children. Havers et al’s study supplements a growing body of literature that has found increased infection-related risks among children with severe chronic conditions^11,12,13 and technology dependencies.^14,15,16 These studies also underpin the importance of routine and annual immunizations for children with medical complexity to decrease infection-related risks.^17,18

While infection is a major reason for children with medical complexity to become acutely ill and need hospitalization, it is certainly not the only one. They are likewise susceptible to non-infectious respiratory insults, hemodynamic instability, metabolic derangements, gastrointestinal dysfunction, seizures, autonomic imbalances, and so on. Many of these children have multiple bouts of acute illness or exacerbations that require numerous hospitalizations.¹⁹

Once hospitalized, their recovery from acute illness is often complicated and protracted. Children with medical complexity have longer lengths of stay^20,21, greater use of intensive care^22,23, and more adverse events^24,25,26,27 than most children. Beyond the risk for symptomatic disease, children with medical complexity are at disproportionate risk for in-hospital, sudden, or unexpected death.^{28,29,30,31,32,33,34,35}

As outpatients, children with medical complexity are at higher risk adverse drug reactions and drug errors because of the high number of prescription medications they require. While not directly related to their underlying conditions, their high-level of dependence on the care of others also put these children at risk for abuse and neglect.^36,37,38

As part of good care, providers are obliged to understand the risks their patients carry and to help their families anticipate and prepare for the range of possible events that they may encounter. For children with medical complexity, proactively providing anticipatory guidance means offering tailored, culturally-sensitive information about what unfavorable health-related outcomes could happen to the child. Doing so in a measured manner helps ensure they are well-informed and can supplement psychoeducation of the family. Psychoeducation is a related concept to anticipatory guidance; it seeks to provide information in order to empower, relieve uncertainty, and enhance psychosocial adaptation to the illness at hand.^39,40 For example, a family of a child with profound cerebral palsy and static encephalopathy should know that she is still at risk for hospitalization due to a common respiratory viral infection and that such misfortunes do not belittle their meticulous home care.

The responsibility of providing this anticipatory guidance falls upon those providers directing the child’s overall care and have a longitudinal relationship with the child and family, as well as any provider that has significant interactions with them and insights into their pertinent risks. Such providers often include primary care physicians, complex care providers, and specialists.

Anticipatory guidance on risks for unfavorable outcomes is best introduced early and during periods of stability--that is, soon after diagnosis and before a crisis, such as acute-on-chronic critical illness. When families are completely unprepared for even the possibility of a crisis, the crisis may be even more traumatic^41,42 and may make necessary decision-making all the more difficult. When the trajectory of a child’s underlying condition and/or treatment is known, anticipatory guidance should be provided at appropriate antecedent points. Providers should periodically reinforce and expand on pertinent points during longitudinal care. Considering the trajectory of the child’s underlying disease is not always known and crises often cannot be predicted, other potential opportunities include when the child starts to be left in the care of others, the development of a new comorbidity, prior to surgical procedures, the death or critical illness of another child the family knows, and other transition points. Anticipatory guidance and psychoeducation should also be given or reinforced after a crisis (ie, before hospital discharge and during post-hospital visits), given that such crises may increase the family’s readiness to hear this guidance. Thus, the responsibility of providing anticipatory guidance also falls upon acute care providers, such hospitalists and intensivists.

Notably, anticipatory guidance on disproportionate risks of unfavorable outcomes is not prognostication for the individual child, and conveying it as such may frustrate the family-provider relationship if erroneous predictions are made. In addition, families should not be overwhelmed with information on all the possible “bad things” that might happen to their child. If their child has never been severely ill, asking a family to absorb a long list of possible unfavorable outcomes that might happen can be disorienting and without context. Overemphasizing the child’s risks also can make families believe that they are “on a different page” from providers and obstruct the family-provider relationship. However, by addressing these topics belatedly or in a manner that is not forthright, providers risk under-informing families.

While providers may view these risks as simmering threats to the child’s well-being and want families to acknowledge the possible negative outcomes, families may view them as mere possibilities that need not be dwelled on until they happen. Thus, providers must balance the need to be sensitive with the need to be forthright. They should explain to families that it would be inappropriate for them to keep information and concerns from them, that they are obliged to sometimes discuss difficult topics, and that these certain topics will increasingly need to be addressed as the child’s medical trajectory changes or becomes more clear. Then, providers must be able to gauge the family’s readiness to receive information on these topics. A family’s readiness to accept information on their child’s risks for unfavorable outcomes may be enhanced by integrating anticipatory guidance and psychoeducation on other topics pertinent to their child. These include medical (eg, progressive mobility and feeding difficulties, spasticity, and dysautonomia), developmental (eg, stigmatization among peers, growth to adult size, survival into adulthood and transition to adult healthcare, and surviving parents), and psychosocial topics (eg, the physical, emotional, and financial impact of caring for such a child). Beyond just informing families of these future issues and milestones, discussion of these topics permits the provider to better understand the family’s goals, values, and preferences and the opportunity to show that she will work to help achieve them. Patience, sincerity, compassion, candor, active listening, avoidance of any suggestion of abandonment, and propitious timing are other useful approaches to help broach these and other difficult topics. Generalists and other providers also can learn from specialists such as palliative care professionals and from educational resources for high-quality communication that are published and online.^43,44

Together, measured and tailored anticipatory guidance and psychoeducation on unfavorable health-related outcomes may help ensure that expectations are realistic. This, in turn, may inform future decision-making and perhaps even facilitate advance care planning, which like anticipatory guidance is ideally addressed early, before a crisis, and revisited after significant events. In addition, anticipatory guidance and psychoeducation may help to curtail the fear and anxiety associated with having a medically fragile child and to mitigate any shock or self-blame experienced when an unavoidable unfavorable outcome does occur.

It is important to bear in mind that even for children with medical complexity, the probability of many of these unfavorable outcomes is still relatively low (despite being much higher compared to other children) and their severity and impact vary considerably. Similarly, while it is useful to think of children with medical complexity as a group and try to span their heterogeneity, it is critical to acknowledge it as well. There are obvious differences in these children’s underlying chronic conditions, as well as a spectrum of severity and variation of associated morbidities within each condition. Such heterogeneity limits our ability to provide more tailored anticipatory guidance to children with particular conditions. However, because they can occur unpredictably and their impact and sequelae can be significant, they deserve to be addressed in a measured manner with families.

Longitudinal and acute care providers who care for children with medical complexity must help inform and prepare their families for the possible unfavorable events that can occur, including severe illness, protracted hospitalization, and unexpected death. In order to help providers, especially generalists, better be able to provide this sort of anticipatory guidance to their families, information on these risks need to be easily accessible and relevant to them and their families. Appreciating the risks of these outcomes among this heterogeneous and vulnerable population also should spur more nuanced research into their likelihood, as well as appropriate resource planning, quality improvement initiatives, advancements in risk stratification and case-mix adjustment, and, most importantly, better care. We must also admit to ourselves and our patients/families that these risks will likely always remain to some extent. As we become increasingly successful at avoiding or alleviating some of these preventable outcomes, some of the children that benefit will survive with even greater medical complexity and thus even greater risks for other unfavorable outcomes.

Abbreviations

CCC

complex chronic conditions

PICU

pediatric intensive care units