Supporting Vulnerable Children after Life-Threatening Neonatal Illness: Opportunities for Improving Outcomes

Major advances in the management of neonates with prematurity, especially those born very preterm (<32 weeks gestational age [GA]), and congenital heartdisease have resulted in greater than 90% survival rates for these infants.^1,2 This translates into over 1 000 000 very preterm survivors and over 500 000 survivors of complex cardiac mal-formations ages birth to 20 years. With this good news, there has been increasing recognition among these children of a spectrum of long-term neuromotor (coordination), communicative, cognitive, perceptual, executive function, social, regulatory, and specific learning disorders.^3–5 Two studies reported in this volume of The Journal bring attention to these issues.

Although it has long been recognized that children with critical illness in the neonatal period are at risk for major neurodevelopmental disabilities (eg, cerebral palsy, blindness, deafness), only in the past 2 decades has there been a greater appreciation of a spectrum of neurodevelopmental disorders affecting integration of cognitive, attention, and social skills later in school.⁵ Kindergarten readiness assessments, use of special education services, and test proficiencies are imperfect measures of neurodevelopmental disability influencing school performance, but can give some insight into unmet needs in these vulnerable populations. Importantly these metrics help inform both health professionals and parents about the need to be proactive in addressing developmental and learning needs so that the isolation of school and social failure are avoided.

In this volume of The Journal, Shah et al⁶ examined kindergarten readiness after prematurity in a large, nationally representative sample of US children in the Early Child Longitudinal Study Birth cohort.⁶ Their cohort included 5250 infants of whom 550 were very preterm (<32 weeks GA), 950 moderate/late preterm (32–36 weeks GA), 1400 early term (37–38 weeks GA), and 2350 term (39–41 weeks GA) gestation.The investigators were able to obtain birth certificate data (weight, gestation, multiple births, Apgar scores, and duration of hospitalization) and also obtained maternal information, such as tobacco use, breastfeeding duration, maternal education, poverty status, and access to early intervention and Head Start. All of these are important factors when examining early trajectories of kindergarten readiness.

The study by Shah et al⁶ is important for several reasons. First, it starts to address systematically what was called for over one-quarter of a century ago, that all children enter kindergarten ready to learn.⁷ The Early Child Longitudinal Study Birth cohort is able to provide this outcome because the developmental follow-up evaluations occurred during a home visit by certified assessors and used a structured battery of language assessments, including the Peabody Picture Vocabulary Test, the Preschool Comprehensive Test of Phonological and Print Processing, and indicators of early literacy. The later included letter and word recognition, understanding letter-sound relationships, phonological awareness, sight word recognition, and understanding word meaning in sentences. In addition, a standardized battery of mathematical concepts, including number quantity and operations were assessed.

What did these authors find? Importantly, Shah et al⁶ found that survivors of very preterm birth had a 250% increase of scoring below the 10th percentile in reading and math at kindergarten entry. This cut point is used by school districts as a criterion for ongoing need of special education supports. The second important finding was that children receiving subsidized lunches, living in a household with low maternal education levels, and not accessing preschool services had higher rates of not being ready for kindergarten across all preterm as well as term groups.

What does this mean? First, in the current era of neonatal follow-up, increased efforts must go beyond tracking short-term outcomes to only 21–24 months corrected age. There must be increased efforts to implement systematic collaborative systems of surveillance, engagement, and assessment for children of all GA.At the very least this should include all children less than 32 weeks. It does not make sense, recognizing the current morbidity of very preterm infants, to focus on screening for blindness, deafness, and severe cerebral palsy alone. One must bolster supportive resources for a spectrum of communicative, learning, intellectual, behavior regulation, and social problems. These changes cannot occur in single hospital follow-up programs alone, but must cross institutions and systems. This will require leadership not only from academic health centers, but also must from primary care settings with linkage to quality family supports, early child education, and Head Start. Most importantly, there must be continuous efforts to increase the access of all preterm survivors to comprehensive quality early childhood education services.

Why does this matter? There is increased evidence that kindergarten readiness is the nucleus for positive educational trajectories. Additional evidence also links kindergarten readiness to children meeting national standards in third and fourth grade. These educational milestones are not only key predictors of succeeding in middle elementary school years, but also prognosticators of completing high school education and aspiring to adult college education. Currently, we are beyond the era of multisystem, physiological crisis instability, and we are ready to focus on the impact of cumulative neurologic perturbations from prenatal, perinatal, and postnatal stressors. Our efforts must include strategies to document best practices, address proactive interventions that can help optimize children’s trajectories, and create a collaborative model so that there is continual improvement of outcome. This will result in improved neuroprotection and resiliency as well as help us address the challenges of managing life-threatening illness and its impact across middle childhood and adulthood.

Though the authors noted a threshold effect in children <32 weeks gestation, we caution that our full understanding of long-term trajectories of late prematurity is not fully known. We recommend that greater attention be given to these children between the ages of 2 and 8 years, with an emphasis on accessing quality early childhood education experiences and interventions that promote mastery of literacy and numeracy and the social skills of middle childhood.

Also in this volume of The Journal, Mulkey et al⁸ examined medical and surgical factors impacting children with congenital heart disease (CHD) and their later school age achievement test performance. Children who had survived CHD surgery were more than twice as likely to receive special education services and less likely to have achieved math and reading proficiency. This study excluded all children with conditions known to influence academic performance (ie, genetic or brain abnormalities), as well as those who were unable to complete achievement testing because of severe intellectual disabilities and learning challenges. Thus, to what extent their findings underestimate the total population of school age children with CHD who require special education support is unknown. Remarkable in this study is the ability to connect, in great detail, medical records with school records. We commend the rigor required for this investigation as well as the cooperation of the Arkansas Department of Education and the Arkansas Children’s Hospital to share data and enable longitudinal follow-up of educational outcomes. We hope that future investigators can continue investing in these population-based cross-institutional collaborations to improve our understanding of educational and learning outcomes after critical illness.

Interestingly, the authors note that shorter hospitalizations were associated with increased likelihood of achievement test proficiency, which suggests that hospital length of stay may either be a marker of critical illness severity, which impacts upon neurodevelopmental status and/or, perhaps, there is an independent contribution of longer hospitalization to worse neurodevelopment.

In a 2012 statement of the American Heart Association, periodic developmental surveillance, screening, and evaluation was recommended for all children with CHD.⁹ However, it is not known how frequently survivors of CHD do have access to such services and what the impact of early developmental diagnoses and habilitative interventions are on long-term outcomes. Mulkey et al⁸ report that more than 1 in 4 (26.9%) of children with CHD received special education services. This may reflect successful advocacy for obtaining educational support services, or may yet underestimate the overall special education needs of this population because some of the more subtle delays that have an effect on long-term learning are not yet apparent by middle childhood.¹⁰

These 2 articles provide evidence for longitudinal support of infants who survive critical illness. Current systems, pressured by economic constraints and a lack of neurodevelopmental specialists, may be inadequately prepared to perform sufficient neurodevelopmental surveillance throughout early childhood, yet our failure to address fully the neurodevelopmental needs of these high-risk population may have enormous life course implications.

Recognizing these unmet needs include the appreciation that early childhood academic achievement is only part of the neurodevelopmental puzzle. Survivors of prematurity and low birth rate are found to have higher rates of autism spectrum disorder⁴ and attention deficit hyperactivity disorder⁵ both of which influence later academic achievement but may not impede simpler testing tasks for kindergarten readiness. Children who survive neonatal critical illness are at risk for socioemotional disorders, which have treatment options, and certainly affect long-term academic, vocational, and independent living trajectories.

In the current era of neonatology and critical care cardiology, we must shift our neurodevelopmental detection paradigm from a reactive late detection of significant cognitive and neurodevelopmental disabilities to a framework of proactive neurodevelopmental surveillance, comprehensive educational management, and family supports. In this manner, we can continuously improve our medical, developmental, and behavioral interventions so that critically ill children are not left behind and we promote resilience.

Glossary

CHD

Congenital heart disease

GA

Gestational age