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Anticipatory Guidance on the Risks for Unfavorable

Outcomes among Children with Medical Complexity
Jeffrey D. Edwards, MD, MA, MAS

R ecent 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
gave us terminology to collectively conceptualize these
patients’ severe chronic conditions and a method to identify
them. Although the study by Feudtner et al and subsequent
reports7,8 highlighted the increased risk of mortality among
require substantial and ongoing medical and community- children with CCCs, Dosa et al9 focused on their high risk of
based care.1 Nevertheless, many of these children remain at severe acute illness. Prospectively studying a 17-county
disproportionate risk for unfavorable health-related outcomes region in upstate New York, they determined that children
as the result of their medical fragility and prodigious care with chronic conditions had a more than 3 times greater risk
needs. These outcomes include, but are not limited to, acute- of unplanned admission to the pediatric intensive care unit
on-chronic illness, hospitalization, and unexpected death. compared with children without a chronic condition.
Understanding these risks as a provider is essential to avert Furthermore, children dependent on various medical
those risks that are preventable and to anticipate those that technologies, such as mechanical ventilation, tracheostomy,
may not be preventable. Likewise, families of children with intravenous medications or nutrition, tube feeding, and
medical complexity need to be informed beforehand about implanted cardiac pacemakers, had a more than 300 times
these potential risks. Such preparation can be considered a greater risk. Almost one-half of these admissions were
form of anticipatory guidance for these families and should estimated to have been related to the children’s chronic
be a basic part of optimal care for this vulnerable population. conditions. Although some of these admissions were
For those caring for the general pediatric population, considered preventable, most were characterized as
anticipatory guidance is a familiar educational practice that unavoidable. These early studies conveyed what providers
seeks to proactively prepare families for what they should undoubtedly were observing in their hospitals and clinics—
expect in the future and how to meet the child’s needs at her children with medical complexity were a growing group of
current/ next developmental stage.2-4 Common topics focus vulnerable children with risks that far exceeded those of
on wellchild and preventive health,including children who were not medically fragile.
feeding,sleeping,growth, immunizations, safety and accident Since then, numerous investigators have studied children
prevention, behavior, puberty, and sex education. Parents with medical complexity and their increased risks for severe
have reported increased healthcare satisfaction when illness, hospitalization, and death. One such study by Havers
anticipatory guidance is provided.5 Understandably, some et al10 focused on children with neuromuscular and
topics need to be modified for children in different neurodevelopmental disorders, a relatively large group of
circumstances or settings. Similarly, for the families of patients that often meet criteria for being medical complex,
children with medical complexity,anticipatory guidance is and their risks for severe respiratory infections, which are a
equally important; however, the topics require adaptations frequent cause of acute illness among children with medical
and additions to be germane because the complex child’s complexity. Using a sizeable cohort drawn from multiple
developmental and medical trajectories may be significantly states, Havers et al compared the rates of respiratory
different than those of the typical child. Among other topics, infectionrelated hospitalization among children with
anticipatory guidance for families with children with medical neurologic disorders with those of the general pediatric
complexity should include information on their risks for population. They found that 12% of hospitalizations of
unfavorable health-related outcomes. children with neurologic disorders were for respiratory
Characterizing the risks of this heterogeneous population infections; one-quarter of these hospitalizations were repeat
was perhaps first attempted in earnest at the turn of this respiratory hospitalizations among less than 4% of these
century, when several insightful investigators started children. Children with neurologic disorders had a more than
publishing groundbreaking research on these children and the 5 times greater relative rate of respiratory infection-related
impact of their chronic conditions. The first was the seminal hospitalization than the general pediatric population.
study by Feudtner et al6 of pediatric deaths in Washington Children with specific neurologic disorders,such as cerebral
State between 1980 and 1997 attributable to underlying palsy,muscular dystrophy,motor neuron
CCCs. Their report highlighted that although rates of
pediatric mortality were declining, the proportion of deaths
due to CCCs were substantial and growing. Importantly, the
report by Feudtner et al also From the Division of Pediatric Critical Care Medicine, Columbia University College of
Physician and Surgeons, New York, NY
The author is supported by the National Institutes of Health (K23 HD 082361). The
author declares no conflicts of interest.
CCC Complex chronic conditions
0022-3476/$ - see front matter. © 2016 Elsevier Inc. All rights reserved.
247 The responsibility of providing this anticipatory guidance
diseases, degenerative diseases, metabolic diseases, and falls on those providers who direct the child’s overall care
chromosomal disorders, had even greater relative rates of and have a longitudinal relationship with the child and
hospitalization. Relative rates of death during respiratory family, as well as any provider who has significant
infection-related hospitalizations were likewise substantially interactions with them and insights into their pertinent risks.
greater among children with neurologic disorders. Such providers often include primary care physicians,
Although this study did not delineate the various infectious complex care providers, and specialists.
etiologies of these severe respiratory illnesses, they Anticipatory guidance on risks for unfavorable outcomes
undoubtedly included an array of common organisms that is best introduced early and during periods of stability—that
lead to upper respiratory tract infection,tracheobronchitis,and is, soon after diagnosis and before a crisis, such as acute-
other nonsevere respiratory illnesses in most children. The onchronic critical illness. When families are completely
study by Havers et al supplements a growing body of unprepared for even the possibility of a crisis, the crisis may
literature that has found increased infection-related risks be even more traumatic40,41 and may make necessary
among children with severe chronic conditions11-13 and decision-making all the more difficult. When the trajectory
dependency on medical technology.14-16 These studies also of a child’s underlying condition and/or treatment is known,
underpin the importance of routine and annual anticipatory guidance should be provided at appropriate
immunizations for children with medical complexity to antecedent points. Providers periodically should reinforce
decrease infection-related risks.17,18 and expand on pertinent points during longitudinal care.
Although infection is a major reason why children with Considering that the trajectory of the child’s underlying
medical complexity become acutely ill and need disease is not always known and crises often cannot be
hospitalization, it is certainly not the only one. They are predicted, other potential opportunities include when the
likewise susceptible to noninfectious respiratory insults, child starts to be left in the care of others, the development of
hemodynamic instability, metabolic derangements, a new comorbidity, before surgical procedures, the death or
gastrointestinal dysfunction, seizures, autonomic imbalances, critical illness of another child the family knows, and other
and so on. Many of these children have multiple bouts of transition points. Anticipatory guidance and psychoeducation
acute illness or exacerbations that require numerous also should be given or reinforced after a crisis (ie, before
hospitalizations.19 hospital discharge and during posthospital visits), given that
Once hospitalized, their recovery from acute illness often such crises may increase the family’s readiness to hear this
is complicated and protracted. Children with medical guidance. Thus, the responsibility of providing anticipatory
complexity have longer lengths of stay,20,21 greater use of guidance also falls upon acute care providers, such as
intensive care,22,23 and more adverse events24-27 than most hospitalists and intensivists.
children. Beyond the risk for symptomatic disease, children Notably, anticipatory guidance on disproportionate risks of
with medical complexity are at disproportionate risk for in- unfavorable outcomes is not prognostication for the
hospital, sudden, or unexpected death.28-35 individual child, and conveying it as such may frustrate the
As outpatients, children with medical complexity are at familyprovider relationship if erroneous predictions are
greater risk for adverse drug reactions and drug errors made. In addition, families should not be overwhelmed with
because of the high number of prescription medications they information on all the possible “bad things” that might
require. Although not directly related to their underlying happen to their child. If their child has never been severely
conditions, their high level of dependence on the care of ill, asking a family to absorb a long list of possible
others also put these children at risk for abuse and neglect. 36- unfavorable outcomes that might happen can be disorienting
38 and without context. Overemphasizing the child’s risks also
As part of good care, providers are obliged to understand can make families believe that they are “on a different page”
the risks their patients carry and to help their families from providers and obstruct the family-provider relationship.
anticipate and prepare for the range of possible events that By addressing these topics belatedly or in a manner that is not
they may encounter. For children with medical complexity, forthright, however, providers risk underinforming families.
proactively providing anticipatory guidance means offering Although providers may view these risks as simmering
tailored, culturally sensitive information about what threats to the child’s well-being and want families to
unfavorable healthrelated outcomes could happen to the acknowledge the possible negative outcomes, families may
child. Doing so in a measured manner helps ensure they are view them as mere possibilities that need not be dwelled on
well-informed and can supplement psychoeducation of the until they happen. Thus, providers must balance the need to
family. Psychoeducation is a related concept to anticipatory be sensitive with the need to be forthright. They should
guidance; it seeks to provide information to empower, relieve explain to families that it would be inappropriate for them to
uncertainty, and enhance psychosocial adaptation to the keep information and concerns from them, that they are
illness at hand.2,39 For example, a family of a child with obliged to sometimes discuss difficult topics, and that these
profound cerebral palsy and static encephalopathy should certain topics increasingly will need to be addressed as the
know that the child is still at risk for hospitalization due to a child’s medical trajectory changes or becomes more clear.
common respiratory viral infection and that such misfortunes Then, providers must be able to gauge the family’s readiness
do not belittle their meticulous home care. to receive information on these topics. A family’s readiness
to accept information on their child’s risks for unfavorable

248 Edwards
outcomes may be enhanced by integrating anticipatory their families. Appreciating the risks of these outcomes
guidance and psychoeducation on other topics pertinent to among this heterogeneous and vulnerable population also
their child. These include medical (eg, progressive January should spur more nuanced research into their likelihood, as
2017 well as appropriate resource planning, quality improvement
initiatives, advancements in risk stratification and case-mix
adjustment, and, most importantly, better care. We also must
mobility and feeding difficulties, spasticity, and admit to ourselves and our patients/families that these risks
dysautonomia), developmental (eg, stigmatization among will likely always remain to
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
some extent. As we become increasingly successful at
informing families of these future issues and milestones,
avoiding or alleviating some of these preventable outcomes,
discussion of these topics permits the provider to better
some of the children that benefit will survive with even
understand the family’s goals, values, and preferences and
greater medical complexity and thus even greater risks for
the opportunity to show that she will work to help achieve
other unfavorable outcomes. ■
them. Patience, sincerity, compassion, candor, active
listening, avoidance of any suggestion of abandonment, and
propitious timing are other useful approaches to help broach Thanks to Dr Gloria Chiang for her reading of the manuscript and
her suggestions.
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-
Submitted for publication May 4, 2016; last revision received Sep 6, 2016 ;
quality communication that are published and available accepted Oct 5, 2016
online.42 , 43 Reprint requests: Jeffrey Edwards, MD, MA, MAS, Division of Pediatric Critical
Together, measured and tailored anticipatory guidance and Care, 3959 Broadway, CHN 10-24, New York, NY 10032. E-mail: jde2134@
psychoeducation on unfavorable health-related outcomes

may help ensure that expectations are realistic. This, in turn,

may inform future decision-making and perhaps even References
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