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With increasing rates of premature births, primary care providers face questions about how to follow the neurodevelopment of these at-risk infants and children. Here's what to expect and how to optimize outcomes.
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The rates of premature births (<37 weeks gestation) continue to increase. In the US, over 500,000 infants are born preterm each year, accounting for over 12.7% of all births.1 The cost of caring for these children is estimated to be $26 billion a year, in part because infants born with very low birth weight (VLBW; <1,500 grams) are surviving more than ever before.1 For this reason you are seeing, and are likely to see, more premature infants in your practice, whose care presents you with a variety of clinical challenges.
For example, lower gestational age and low birth weight lead to higher rates of challenging neurodevelopmental outcomes, such as cerebral palsy, learning disabilities, and attention-deficit hyperactivity disorder (ADHD). However, up to 50% of extremely low birth weight (ELBW; <1,000 grams) infants have no measurable disabilities within their first three years of life,2 but then may go on to demonstrate serious learning and behavioral problems. Additionally, while biological risks heavily influence a preemie's outcome, environmental risks become even more influential as the child ages.3 Finally, the effects of prematurity on the families (eg, parental grief and/or traumatic stress) are increasingly being recognized as drivers in the long-term outcomes of preterm births.
These factors bolster the importance of an appropriate follow-up on a premature infant's neurodevelopmental status by their primary care provider. In an effort to help, this article examines the risk factors and potential neurodevelopmental and behavioral outcomes associated with preterm births. We will then highlight the availability of assessment tools, and offer management recommendations.
The most important indicators for the development of neurological/behavioral problems among preterm births are gestational age and birth weight. These are followed by the presence of intrauterine growth restriction (IUGR) and a positive history of drug use by the mother. Figure 1 lists additional risk factors to preterm neurodevelopmental outcome, and can be used as a convenient checklist when assessing your preemie patients.
Although many of the factors denoted in Figure 1 are biological in nature, you should pay equal attention to the environmental considerations, such as maternal mental health and socio-economic status. These play an increasingly important role in the child's development into their second year and beyond.4
Most primary care providers are familiar with the potential medical complications of prematurity. They include: intraventricular hemorrhage (IVH), periventricular leukomalacia (PVL), cardiac lesions, bronchopulmonary dysplasia (BPD), growth and feeding problems, and retinopathy of prematurity (ROP). These problems invariably affect the neurodevelopmental status of preterm infants and children in an ongoing way. For example, Grade 3 and 4 IVH increases the risk of a neurodevelopmental disability among preterm infants from 35 to 90%.5Table 1 outlines the most common adverse neurodevelopmental outcomes of premature births and their prevalence in primary care environments.
These outcomes involve neurosensory functioning, cognitive and motor skills, language and social development, learning ability, as well as behavioral problems. For example, parents often acknowledge their preemie's "self-regulation" challenges, such as difficulties in regulating their transitions from sleep to waking states, and in integrating the sensory stimuli of sound, touch, and light. Other behavioral problems such as anxiety and depression are found more frequently in low birth weight (LBW; <2500 grams) infants as they grow.6,7 ELBW infants have also been shown to develop poorer social skills and adaptive behaviors.8 This is coupled with exceedingly high rates of ADHD-up to a two- to six-fold increase among preterm births.2
Adding to the number of outcomes are studies that have found that nearly half of VLBW children will require special education services.9 Lastly, as many as 18% of VLBW infants go on to have severe disability, which is defined as spastic quadriplegia, blindness, deafness, uncontrolled epilepsy, or severe learning disability.10
Having a premature infant is often quite stressful for families-regardless of their socioeconomic status. Those who come from disadvantaged backgrounds are at a higher risk for a preterm birth, and this resource-poor environment continues to exert an effect on the child as he or she ages. Families that undergo the rigors and expense of artificial reproductive technology are also at increased risk for a preterm birth. In either case, there can be an initial grief reaction at the loss of a "perfect" imagined child.
Additionally, a seriously ill child who has been cared for in the alien technological environs of the neonatal intensive care unit (NICU) can stir parental traumatic stress reactions, such as anxiety nightmares, sleep problems, and avoidance of medical visits. NICU parents may also suffer a loss of control in not being able to protect their infant. This reaction, combined with the natural hormonal changes after childbirth, make mothers especially vulnerable to postpartum depression (PPD). PPD symptoms can resolve or persist and disrupt the attachment process between mother and child, resulting in higher rates of cognitive and behavioral problems.
If mom or dad continues to see their child as overly sensitive or fragile, a pattern of "vulnerable child syndrome" may also develop.17 In this maladaptive interaction pattern, the parents prevent their child from exploring and developing at their level due to their overprotectiveness.
Given the varying emotional and physical outcomes associated with preterm births, how can we as providers truly make the most of visits with these children and their families? A recent expert panel examining this issue looked at the quality of care indicators for the primary care of preterm infants born with VLBW.16 These indicators offer guidance for the follow-up care of the preemie population. Figure 2 summarizes the most salient of these recommendations and can be used in conjunction with Figure 1 while assessing your preterm patients.
A more detailed discussion on the value of these measures, along with what tools to use, follows in the paragraphs below:
Attention to the general care and physical health of the infant is, of course, the foundation of all comprehensive primary care. For premature infants, this begins with a detailed discharge summary that documents the pregnancy, neonatal, and NICU history (Figure 1). General care measures such as monitoring infant growth parameters such as head circumference, weight, and length must be closely watched and corrected for prematurity up until the child's third birthday.
Nutrition can be yet another area of concern. Some preterm infants will go on to demonstrate failure to thrive, a condition that will need aggressive management or referral to a nutrition specialist for management with special formulas or feeding milieu changes. Soy-based formulas are not recommended for preterm infants due to the possibility of aluminum toxicity, poorer growth rates, as well as a risk of osteopenia.18
In general, minimizing a premature infant or child's exposure to potential health risks can reduce their chances of developing neurological and behavioral problems. Simple measures, such as treating anemia and chronic lung disease (CLD), preventing respiratory syncytial virus (RSV) with palivizumab (Synagis), avoiding sudden infant death syndrome by supine positioning for sleep, and counseling against smoking at home, can all contribute to the creation of a positive outcome.
Formal vision and hearing assessments are needed for all preterm infants at baseline, with ongoing assessments for those at higher risk. Moreover, providers should feel comfortable performing an infant and child neuromotor examination, assessing tone, strength, symmetry, primitive and deep tendon reflexes, and, later, gait.19 Atypical development or neurological findings are red flags for later problems, but could also be transient. Therefore clinical judgment is required as to whether to follow these symptoms or refer the child for evaluation by a subspecialist. In either event, close follow-up is essential.
The usual developmental screening tools appropriate for term infants and children often do not reliably pick up delays in a preterm infant. This is due in large part to the lack of standardized developmental screening instruments for premature infants/children. Popular screening tests such as the Denver and PEDS have not been validated specifically on preterm populations; PEDS has shown to miss problems in the 0- to 3-years age group in the VLBW group.20 New AAP recommendations suggest developmental screening at the nine-, 18-, and 30-month visits for all infants.21 The preterm group should receive especially close adherence to these recommendations.
Although developmental screening tools may be problematic, if the chronological age cut-offs are used, they can be helpful in assessing the various streams of development. We recommend correction for gestational age up to at least 30 to 36 months for those born under 32 weeks to give a more accurate picture of the child's developmental status.
Table 2 lists the commonly used developmental screening tools. If these instruments or your own surveillance raise concerns, formal evaluation by a subspecialist is warranted. In the VLBW group, a referral for a formal developmental assessment through an early intervention (EI) program, a developmental and behavioral pediatrician, or a NICU follow-up program is recommended at 9 to 15 months and again between 21 to 30 months.16 For high-risk children, the AAP recommends an 18- to 24-month NICU follow-up clinic assessment for the best yield of adverse findings.22 Any of these assessment options can formally look at the streams of development including speech and language, gross and fine motor, cognition, and social-emotional as well as later academic, behavioral, and adaptive functioning.
Behavioral and psychosocial surveillance/screening is another area of ongoing assessment. Early infancy is dominated by acquiring self-regulation skills, such as feeding and sleeping. Crying, for example, may be hard to regulate for preterm infants with poor self-soothing skills. Questioning the parent about these behaviors and observing parent-child interactions in the office visit is an important way to assess these challenges. At 4 years of age and above, tools such as the Pediatric Symptom Checklist can help to identify children with impairment in their psychosocial functioning in primary care settings.25
As the child ages, understanding the child through the perspective of temperament can be helpful in managing challenging behaviors. Temperament is often referred to as the "how of behavior" and has several dimensions (eg, regularity, activity level, and mood). Knowing a child's temperamental profile can direct families to successful interventions such as providing flexible schedules for a child with low regularity or self time-outs for children with high activity levels.26
An often overlooked stream of development is the child's social skills. The Modified Checklist for Autism in Toddlers(Table 2) is especially helpful for assessing developmentally appropriate social skills starting at 18 to 24 months.27
Higher rates of ADHD and symptoms of inattention/ impulsivity among preemies also warrant a primary care-based screening assessment based on new AAP guidelines28 using a DSM–4 based tool such as the Vanderbilt Assessment Scale. With the high prevalence of learning and neuropsychological problems, consideration of a school-based assessment or Developmental-Behavioral Pediatrics referral may be helpful to rule out these problems. Table 2 lists several more formal ways of assessing temperament, social behavior, and attention.
Due to the importance of a child's environment during its development, a survey of family function is highly recommended. A thorough family and social history are important to assess for genetic contributions, social risk factors, and potential social supports. Care should be made in looking for signs of "vulnerable child syndrome" and parental mental health such as grief, depression, and PPD. These symptoms may arise later in the child's life at times of transition such as going to school.
The AAP recommends that all children with medical and developmental challenges have a medical home in which pediatric care is delivered. This involves creating a system of care that is: accessible, continuous, comprehensive, family-centered, coordinated, compassionate, and culturally effective.29
Creating a medical home is especially relevant for premature infants who are technology dependent. These infants need primary care for their multiple subspecialty appointments, durable medical equipment, prescription medications, and complex care regimens. Similarly, the families of these infants require referrals to medical and allied health providers, connections to Supplemental Security Income, and other forms of government support (eg, Medicaid or Katie Beckett programs). Most of all, however, these families will need your emotional support.
Primary care providers should also serve to integrate and coordinate other screening and referral needs of the child and family. For example, families of preemies under the age of 3 often need a prompt referral to an early intervention (EI) program. After the age of 3, a referral to a school's special education program is required (IDEA law). Admission into these programs is based on eligibility criteria. These criteria are unique from state-to-state and are summarized in Table 3. In some (but not all) states, a diagnosis of ELBW automatically qualifies an infant as eligible for services.
Beyond the screening referrals indicated above, other specialists such as subspecialty pediatricians; social workers; speech and language; occupational and physical therapists; lawyers; and others could all be part of a dynamic team based on the child and family needs.
While many premature infants and children do quite well, the primary care provider has the unique opportunity to find those who are at high risk of neurodevelopmental and behavioral problems. Using Figure 1 to document the potential risks, and Figure 2 as an intervention guide, can help organize your approach. Your influence may also be felt through an ongoing assessment of the child's relationships (eg, among family, in school, etc.) as they continue to grow.
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