31. Discharge Planning and Follow-Up of the Neonatal Intensive Care Unit Infant*
Donna K. Daily, Angel Carter and Brian S. Carter
Parents with infants in the neonatal intensive care unit (NICU) have immediate worries about whether their newborn infant will survive but soon thereafter start having concerns about how their child will do through infancy and into adulthood. As the infant’s convalescence begins, so does discharge planning; this brings to the forefront questions about outcomes. Unfortunately, it is almost impossible to know the outcome of any individual infant at the time of discharge from the NICU. Caregivers within the NICU must be knowledgeable of the latest outcomes literature to respond to these questions and to guide parents in the importance of follow-up care.
Of the many reasons that newborns require neonatal intensive care, the most common one is preterm birth. Numerous publications report the outcomes of very-low-birth-weight (VLBW, birth weights <1500 g) and extremely-low-birth-weight (ELBW, birth weights <1000 g). 3,23,28,33,65 Recent studies have focused on the survival and outcome of even more immature infants with birth weights below 750 g, or on infants born at the limits of viability, 22 to 25 weeks’ gestation. 45,64,68,84 Late preterm infants have been a topic of renewed interest because they are at risk for a unique set of problems with adverse outcomes. 2,52 Infants with intrauterine growth restriction (IUGR) are also vulnerable to a wide range of complications requiring neonatal intensive care, especially if they are also preterm and experience extrauterine growth failure. 32,80 Full-term infants with meconium aspiration syndrome (MAS), persistent pulmonary hypertension of the newborn (PPHN), infection, congenital diaphragmatic hernia (CDH), or neonatal encephalopathy also require intensive care and are at risk for health and developmental sequelae. 121 Finally, a number of infants with multiple congenital anomalies require surgery or neonatal intensive care.
PLANNING FOR DISCHARGE
An organized, well-implemented discharge plan is the beginning of successful follow-up of a NICU graduate. A family-centered multidisciplinary team approach uses the expertise of many disciplines, along with the family, to formulate and implement the discharge and follow-up plan. The team can comprise parents, grandparents, other caregivers, physicians, nurses, case managers, dietitians, therapists, developmental specialists, and social workers.
Integrating family-centered principles into the discharge process as a continuation of family-centered care practiced throughout the NICU stay facilitates better parental adaptation to the transition to home.62 For many infants, the NICU stay has been lengthy and complex, and families may experience varying degrees of anxiety and stress as they prepare for the infant to come home. In some cases, attachment and bonding may have been affected by a long, complicated medical course. 41 A survey of preterm mothers found that symptoms of psychologic distress (fatigue, depressive mood, anxiety, physical symptoms) persisted up to a year after the birth of their premature baby. 57 Families may need extra attention paid to these issues before they can successfully attend to the discharge process. A thorough assessment of caregiver needs, environmental issues, and knowledge of their infant’s care before discharge is an important part of the planning process. Implementation of a parent educational-behavioral intervention program during the NICU stay may be one mechanism to reduce stress, depression, and anxiety and effect more positive interactions of parents with their infant, a shorter NICU stay, and shorter total hospital stay. 86 In addition, an assessment tool may be considered in efforts to quantify the discharge readiness of the family. 111
Considerations Before Discharge
INSURANCE
Many parents may need assistance to enroll their infant in their existing insurance policy or to identify the procedures necessary to apply for medical assistance. This process can take many weeks and must be accomplished before discharge in order to select a pediatrician for follow-up care. Social workers, case managers, and financial counselors are valuable resources to assist families in this process.
IDENTIFICATION OF A FOLLOW-UP PROVIDER
A pediatric health care professional to follow the infant after discharge, trained in the care of NICU graduates, should be identified before discharge. Ideally, this provider has been identified early in the admission to facilitate regular communications regarding the infant’s medical course. Immediately before discharge, a written summary should be provided, with follow-up recommendations regarding nutrition and growth, developmental surveillance, and subspecialty referrals, along with verbal notification of the discharge date. Parents should be advised to keep this summary with the infant at all times because this written documentation of the NICU stay is invaluable if the infant needs to be seen on an emergency basis shortly after hospital discharge. Providing families with a “care notebook” containing specialized forms and organizing tools can be a valuable addition to the discharge process, particularly for those with anticipated complex follow-up needs. The American Academy of Pediatrics (AAP) also provides a form for summarizing the history and current needs of children with special health care needs.
CAREGIVER EDUCATION
In a family-centered environment, families have been partners in caring for their infant throughout the hospital stay. Discharge teaching then becomes a process of reinforcing and attending to final details. In some instances, however, this teaching may be limited by the inability of the family to be present because of transportation and family or job constraints. In these cases, readiness of the caregivers and home environment should be thoroughly evaluated (Box 31-1).
BOX 31-1
• Every encounter with the parents is a teaching opportunity. Assess each individual family’s readiness for discharge.
• Inform parents verbally and in writing about the tests included in the newborn genetic screening and how they will receive the results.
• Teach parents the special nutritional needs of preterm infants after discharge, including nutritional supplementation, lactation support and intervention to promote breast feeding, and use of alternative feeding methods, if necessary.
• Teach parents the importance of maintaining their infant on home oxygen therapy (e.g., for growth and development, sleep, and feeding) at designated pulse oximetry targets until pulse oximetry studies (e.g., awake, feeding, asleep) document that the infant can tolerate weaning and discontinuing the supplemental oxygen.
• Teach parents to dress their infant appropriately to maintain adequate axillary temperature.
• Teach parents appropriate safety precautions:
• Proper positioning (supine) for sleep: “Back to Sleep”
• Proper use of car seats
• Importance of a smoke-free environment
• Never shake the baby! Dangers of shaking infants include blindness, brain damage, developmental delays, seizures, paralysis, and death
• Information, in writing, about all medications for their infant including name, action, dose, route, side effects, schedule
• Provide parents opportunity to participate in infant CPR class
• Teach parents the importance of follow-up care and appointments:
• Timely follow-up for infants with retinopathy of prematurity (ROP) provided verbally and in writing
• Timely follow-up for hearing screen and referral for re-screening
• Need for monthly RSV immunizations throughout the RSV season
• Give parents the newborn immunization record.
• Teach parents the importance of their own self-care, and assist in identifying resources for support.
HOME EQUIPMENT
When a home apnea monitor is used, a clear plan outlining the reasons for initiating home monitoring and the indications for discontinuing it should be discussed with the family and the primary care provider before discharge.5 Any necessary durable medical equipment or supplies, such as an apnea monitor, feeding pump, ventilator, suction equipment, or oxygen for home use, should be delivered to the hospital before discharge to give parents practice using the equipment. The company supplying the equipment should provide training in its use. NICU nurses or respiratory therapists should verify the parents’ understanding of the purpose of the equipment and its operation and also ensure that home caregivers for the baby have been trained in cardiopulmonary resuscitation (CPR).
ROOMING IN
Whether or not an infant is going home with equipment, giving family caregivers the opportunity to provide “independent” care of their infant with professional caregivers nearby for assistance has been shown to increase parental competence and provide confirmation of readiness for independent care at home.39 One intensive care nursery’s experience in establishing a step-down unit where mothers provided all basic care for their infant under supervision resulted in earlier discharge to home with no increases in short-term complications or readmissions. 29
DISCHARGE CRITERIA
Clearly defined discharge criteria provide both the family and the staff a point of reference from which to judge the infant’s progress. Discharge criteria should be reviewed in a multidisciplinary team meeting with the family. Setting goals that the infant, parents, and staff must accomplish before discharge helps keep everyone focused and prevents important components of the discharge process from being overlooked.
For preterm infants, the attainment of a weight of 5 pounds is no longer the criterion for discharge. Rather, the ability of a preterm or recovering neonate to maintain physiologic stability and the ability of the family to care for the infant’s physiologic and developmental needs are the criteria for discharge (Box 31-2). There are significant variations across NICUs for specific discharge criteria, with assessment of apnea and feeding behavior significantly influencing the duration of hospitalization in a healthy preterm infant. 49 The recently published AAP policy statement “Hospital Discharge of the High-Risk Neonate” provides guidance that should minimize such variations. 6
BOX 31-2
Infant
• Sustained weight gain of sufficient duration
• Maintain normal body temperature, clothed in an open bed, at normal room temperature (20 ° to 25 ° C)
• Establish and maintain competent breast or bottle feeding without cardiopulmonary problems
• Nutrition assessment and dietary management provided as indicated
• Hematologic assessment and management provided as indicated
• Documented physiologically mature and stable cardiopulmonary function of sufficient duration
• Parents have been given a report of neurodevelopmental and neurobehavioral status
• Completed metabolic, hearing, and indicated funduscopic screenings
• Appropriately immunized, including (respiratory syncytial virus [RSV] prophylaxis) and plan for subsequent injections
• A completed car seat evaluation
• A completed review of the hospital course, pending medical problems noted, and follow-up plans identified
• A home-care plan, individualized to the patient’s needs, has been provided by all disciplines
Parents, Family, and Home Environment
• Identify and assess at least two caregivers for home.
• Assess psychosocial and parenting strengths and risks.
• Consider the home environment and on-site visit as indicated.
• Review resource availability (including financial, utilities, and transportation).
• Determine caregiver availability, ability, and commitment to the following:
• Provide basic infant care: diapering, bathing, dressing, cord and circumcision care.
• Maintain infant’s thermal state: able to take temperature and dress appropriately.
• Feed infant (breast, bottle, or alternative method—nasogastric tube, gastrostomy, parenteral nutrition), and demonstrate formula preparation if required.
• Manage home feeding tube, infusion pump, intestinal stoma care, and other devices as indicated.
• Manage home monitoring, oxygen, and other equipment as indicated; address initial problem solving; demonstrate CPR and initial emergency interventions.
• Maintain safe environment, car seat, heat, electricity, telephone, transportation, smoke-free, emergency resuscitation.
• Recognize signs of illness, and identify when to call primary care provider or emergency services.
• Have support system identified to assist in infant’s care.
• Demonstrate medication administration and recognize signs of medication adverse effects (e.g., toxicity); understand importance of follow-up care, and know whom to call for questions or concerns.
Modified from American Academy of Pediatrics, Committee on Fetus and Newborn: Hospital discharge of the high-risk neonate, Pediatrics 122:1119, 2008.
TRANSFER
Some infants are not discharged home from the regional or tertiary NICU but, instead, are transferred from a regional referral center to a community unit or facility for the duration of their hospital stay. Transfer to a community hospital may be beneficial to families because they are often closer to the parents’ home (especially if the NICU is part of a regional referral center). Possible locations for transfer, as well as the criteria for transfer, should be discussed with the parents early in the hospitalization if this is an expected possibility. Communication of a comprehensive discharge plan should take place with the receiving hospital before transfer.66,79 As the capacity for back-transporting convalescing neonates to community hospitals has increased in the United States over the past 20 years, persistent issues of communication, trust, and psychosocial support remain for parents. 44
EARLY DISCHARGE
Preterm infants are often discharged between 35 and 37 weeks chronologic age.89Parental concerns at the time of discharge may include their ability to have adequate rest, their readiness to learn and assume self-care and newborn care, their readiness to parent, and availability of support systems. Concerns about the newborn may include transition from the intensive care nursery to the home care environment, ability to feed and hydrate adequately, and the early development and recognition of complications. 117 In addition, late-preterm infants (34 to 36 weeks gestational age) are sometimes discharged early (<48 hours) using criteria developed for term infants. Tomashek et al demonstrated that late-preterm, early discharged, breast-fed infants were 1.5 times more likely to require hospital-related care and 2.2 times more likely to be readmitted than term infants who were breast fed, with jaundice and infection accounting for the majority of readmissions. 132 Early discharge requires appropriate post-discharge follow-up and monitoring to prevent morbidity. Numerous studies document the positive effects of home visitation programs to assist with post-discharge education and support. 95,102,146
Screening
GENETIC SCREENING
Initial screening of sick or premature infants is performed as soon as possible after birth, before the administration of blood or antibiotics. Although it is common for these infants to have some relatively abnormal results—especially for thyroid function or amino acid profiles while on parenteral nutrition—early screening is recommended to identify in a timely manner those infants who may have an inborn metabolic disorder, congenital endocrinopathies, hemoglobinopathies, or infectious processes so that early treatment can be initiated. 12,109Subsequent screenings should take place according to an established routine, depending on state requirements. Recommendations for continued screenings after discharge should be clearly outlined in the discharge summary.
HEARING
All infants, especially those who need NICU admission for more than 5 days, should be screened for hearing loss using otoacoustic emissions or auditory brainstem response testing. This initial screening should be performed once the infant is medically stable, and if there are any concerns that warrant a secondary screen, re-screening should occur before 1 month of age. Infants who do not pass (are “referred” after secondary screening) should have a full-scale auditory diagnostic evaluation by 3 months of age. Infants with confirmed hearing loss should receive intervention by 6 months of age from an infant hearing specialist. 11,134 Those infants with an increased risk for hearing impairment should be assessed by a pediatric audiologist with a follow-up schedule outlined for the parents (Box 31-3). The goal of early detection and intervention is to maximize language, cognitive, literacy, and social development of the hearing impaired.126
BOX 31-3
• Neonatal intensive care unit (NICU) admission for more than 5 days or any of the following regardless of length of stay: extracorporeal membrane oxygenation (ECMO), assisted ventilation, exposure to ototoxic medications (gentamicin and tobramycin) or loop diuretics (furosemide/Lasix), hyperbilirubinemia requiring exchange transfusion
• Syndromes associated with hearing loss such as neurofibromatosis, osteopetrosis, and Usher syndrome
• Family history of hereditary childhood hearing loss
• Craniofacial abnormalities
• Congenital infections such as cytomegalovirus, toxoplasmosis, bacterial meningitis, syphilis, herpes, and rubella
• Physical findings (white forelock) associated with syndromes known to include hearing loss
• Neurodegenerative disorders (e.g., Hunter syndrome) or sensory motor neuropathies (e.g., Friedreich ataxia and Charcot-Marie-Tooth syndrome)
• Culture-positive postnatal infections such as bacterial and viral (especially herpes and varicella) meningitis
• Chemotherapy
• Caregiver concerns regarding hearing, speech, language, or developmental delay
Modified from American Academy of Pediatrics and Joint Committee on Infant Hearing, Year 2007 Position Statement: Principles and guidelines for early hearing detection and intervention programs, Pediatrics 120(4):898, 2007.
VISION
Development of severe retinopathy of prematurity (ROP) may still be a concern at the time of NICU discharge for infants born prematurely. Infants born at less than 32 weeks’ gestation or less than 1500 g birth weight, as well as infants with a birth weight between 1500 and 2000 g or born at more than 32 weeks with an unstable clinical course, should have a retinal screening examination with pupillary dilation.13 Follow-up of infants should be according to the ophthalmologist’s recommendations based on retinal findings. Arrangements for follow-up examinations should be made before discharge. Current research indicates that the risk to visual development in preterm infants does not end when the risk for ROP has passed. Any infant born prematurely, whether or not they develop ROP, are at increased risk for amblyopia/strabismus and refractive errors.97
IMAGING
Premature infants are at increased risk for injuries to the brain, potentially causing permanent damage. The most common form of damage and the leading cause of chronic neurologic morbidity is periventricular white matter injury. 25Identification of infants at high risk for poorer outcomes related to brain injury allows for timely referrals to early intervention therapies. A recent Cochrane review indicates that early intervention can improve cognitive outcomes up to preschool age. 127 Imaging techniques for routine screening for white matter injury have traditionally been the cranial ultrasound, although the ability of ultrasound to predict developmental outcome is inferior to magnetic resonance imaging (MRI). 90 A recent review of MRI screening to identify risks of suboptimal neurologic outcomes suggests the following be considered as indications for screening by MRI at approximately 36 weeks postmenstrual age67:
• Grade III to IV intraventricular hemorrhage
• Periventricular hemorrhagic infarction
• Cystic periventricular white matter damage
• Cerebellar hemorrhage or other abnormalities on ultrasound
• Suspected white matter abnormalities on ultrasound (echodensities/echolucencies)
• Post–hemorrhagic hydrocephalus
• Abnormal neurologic examination
• Other conditions warranting detailed neuroimaging (metabolic disorders or suspected congenital structural abnormality)
Preventive Care
IMMUNIZATIONS
Infant immunizations are recommended for all NICU infants, according to the guidelines issued by the Centers for Disease Control and Prevention (CDC) and approved by the AAP.37 Immunizations administered in the NICU should appear in the discharge summary. When immunizations have been declined by parents, this should be clearly indicated in the discharge summary, along with follow-up recommendations.
RESPIRATORY SYNCYTIAL VIRUS INFECTION PROPHYLAXIS
Respiratory syncytial virus (RSV) infection poses a risk for serious morbidity or even death for infants who were born prematurely, especially those with chronic heart or lung disease. For qualifying infants, RSV prophylaxis should be initiated with intramuscular palivizumab before discharge into the community setting during RSV season. RSV infection prophylaxis should be coordinated with the follow-up pediatrician for subsequent monthly injections.
Assessments
CAR SAFETY
All 50 states require infants to be restrained in a safety seat while riding in a motor vehicle, although laws vary from state to state. The AAP’s recent clinical report on transporting infants home states that “Infants with documented oxygen desaturation, apnea, or bradycardia in a semiupright position should travel in a supine or prone position in an Federal Motor Vehicle Safety Standard (FMVSS) 213–approved car bed after an observation period that is free of such events as described in point 1 above [e.g., ‘increased frequency of oxygen desaturation and episodes of apnea or bradycardia while sitting in car safety seats…preferably their own…for a minimum of 90 to 120 minutes or the duration of travel, whichever is longer…’].” This may need to be revised as new evidence becomes available from future research. 4,7,9
Discharge of smaller infants results in the use of car seat restraint devices that were designed for 7- to 8-pound term infants. In these devices, preterm infants may experience oxygen desaturations and apnea and bradycardia caused by head slouching and airway obstruction.105 Use of rolled diapers/blankets may be necessary to support upright posture, prevent slouching, and enable the preterm to maintain stability while in the car seat. A recent study of a foam insert that enabled the preterm’s head to be maintained in a neutral position showed a significant reduction in the rate of apneas. 133 In addition, infants with certain conditions (e.g., Down syndrome, osteogenesis imperfecta, myelomeningocele, Pierre Robin syndrome, cerebral palsy) may benefit from special-needs car restraints. 82
A car seat challenge before discharge is recommended for all infants born less than 37 weeks’ gestation; this includes “late preterm” infants (i.e., 34 to
weeks) who are cared for and discharged from level I/normal newborn nurseries.7,9 Although the car seat challenge has not been standardized, certain components are common: (1) using the car seat purchased by the parents, (2) positioning the infant in the car seat immediately before discharge while on cardiorespiratory and pulse oximetry monitoring, (3) for a prescribed period of time (e.g., 30 to 90 minutes), and (4) recording respiratory/heart rates, oxygen saturations, apnea/bradycardia events. Although this is a recommended practice, limitations have been identified. First, little objective evidence supports the ability of this challenge to absolutely confirm safe travel for an infant. 60 A recent Cochrane review of the literature found no randomized controlled trials that fulfilled eligibility criteria for their review and were unable to recommend or refute a car seat challenge before discharge. 106 Further, common clinical practice has been to recommend the use of a car bed as a safe alternative to infants failing the car seat challenge, although recent studies have failed to prove any difference in apneic events. 77,119 A recommendation has been put forth regarding changing the notion of a “test” or “challenge” to a car seat “orientation” in which the emphasis would be on education on proper positioning, limiting duration of automobile travel, and close observation during travel. 60
weeks) who are cared for and discharged from level I/normal newborn nurseries.7,9 Although the car seat challenge has not been standardized, certain components are common: (1) using the car seat purchased by the parents, (2) positioning the infant in the car seat immediately before discharge while on cardiorespiratory and pulse oximetry monitoring, (3) for a prescribed period of time (e.g., 30 to 90 minutes), and (4) recording respiratory/heart rates, oxygen saturations, apnea/bradycardia events. Although this is a recommended practice, limitations have been identified. First, little objective evidence supports the ability of this challenge to absolutely confirm safe travel for an infant. 60 A recent Cochrane review of the literature found no randomized controlled trials that fulfilled eligibility criteria for their review and were unable to recommend or refute a car seat challenge before discharge. 106 Further, common clinical practice has been to recommend the use of a car bed as a safe alternative to infants failing the car seat challenge, although recent studies have failed to prove any difference in apneic events. 77,119 A recommendation has been put forth regarding changing the notion of a “test” or “challenge” to a car seat “orientation” in which the emphasis would be on education on proper positioning, limiting duration of automobile travel, and close observation during travel. 60NUTRITION AND GROWTH
Before discharge, (1) current growth trends should be reviewed; (2) either breast feeding, if desired, should be established or guidelines given for increasing and monitoring; and (3) special feeding considerations such as the use of increased calorie formula, vitamin and mineral supplementation, and use of “special” formulas, tube feedings, or home total parenteral nutrition (TPN) should be outlined.144 In studies of infants who experienced extrauterine growth restriction (EUGR), commonly defined as weight less than the 10th percentile for corrected gestational age (CGA) at discharge, the period from discharge to 30 months is shown to be a critical period for growth. 116 Nutritional intake at this time sets the trajectory for growth and neurodevelopment in childhood and adolescence. VLBW children who have low weight gain in early years of life have a higher probability of cognitive deficits; conversely, those with excessive weight gain had a higher likelihood of obesity, cardiovascular disease, and diabetes. 36
NEURODEVELOPMENT
Some premature infants may have recognized risks to their later development evident at discharge. For these infants, early evaluation of their functional neurologic status may facilitate referrals to early intervention services soon after discharge. 104 Early intervention has been shown to improve neurobehavioral development with improved cognitive outcomes and parent-child interactions. 31,98
Technology-Dependent Infants
Infants who rely on long-term technologic support are being discharged home in increasing numbers. In the past, children who were ventilator dependent, who had tracheostomies, gastrostomies, or jejunostomies, and even those who required long-term intravenous (IV) access for medications or parenteral nutrition would remain hospitalized, separated from their families and susceptible to other morbidities associated with long hospital stays (e.g., infection, delayed development, impaired mental health). With this increase of technology-dependent children discharged into the community comes a greater need for support services for the parents, providers, and the infants themselves. 142 Numerous investigators have undertaken projects to understand the impact that caring for these children has on the individual child and the family as a whole. Carnevale et al described the experiences of 12 families with technology-dependent children at home. 35 They identified key themes in this population to include (1) parental responsibility being stressful and, at times, overwhelming; (2) the devotion of significant energy in trying to “normalize” their home life; (3) living in isolation and feeling like strangers in their own communities; and (4) an overall theme of “daily living with distress and enrichment.”35
In-home nursing care, extensive parent training, and an identified primary care provider comfortable with all aspects of the child’s care are essential for successful discharge of technology-dependent children to home.70 Developing home care plans that contain emergency and resuscitation procedures, as well as parental support and respite services, is vital. 91 Parents need training around all the details of their child’s care, including early signs of illness and emergency procedures, whether or not home shift nursing will be provided. Rooming in with their infant for one or more nights is an important safety net to evaluate their abilities and confidence in caring for their child. Communication and care coordination among the subspecialties following these infants are crucial.
NEURODEVELOPMENTAL FOLLOW-UP OF HIGH-RISK INFANTS
Ideally, parents of all NICU infants would be offered comprehensive, coordinated, developmentally based, family-centered follow-up for their child through infancy and childhood (Figure 31-1). Each infant is unique, as is each infant’s family. The following are the primary objectives of follow-up care:
 |
| FIGURE 31-1
(Courtesy Angel Carter, Brian S. Carter, and Donna K. Daily, Vanderbilt University Medical Center.)
|
• To counsel the family about their child’s development so that they are empowered to optimize the child’s health, growth, and development
• To recognize and diagnose (early) significant health conditions and neurodevelopmental disabilities to facilitate appropriate referrals for community services
• To anticipate future difficulties and needs so that optimal development is promoted and secondary complications are avoided or minimized
The ultimate goal is to promote the child’s integration into the family, school, and community.
Follow-up resources are generally limited, however, and today health insurance plans (both public and private) determine how children will access care. Consequently, criteria for developmental follow-up of NICU infants vary widely. Some high-risk infants are routinely referred to early intervention programs for developmental care, but implementation of these programs varies among states. The dynamics of development are such that periodic assessments of the child’s health and developmental progress are needed to determine whether current interventions are effective and sufficient. Parents often need guidance to better understand what to expect from their child, how to interpret their own observations of their child, and how health care and community services can support their child’s development. The AAP has emphasized that each child have a “medical home,” and especially the child with complex health and developmental needs. Unfortunately, the care of these children often is fragmented among numerous subspecialists and therapists. 10 Well-organized NICU follow-up clinics can facilitate developmental and health care of the NICU infant in coordination with the primary care provider and the family.
Developmental Milestone Attainment
In developmentally based follow-up, much of the information about the child’s development comes from a careful interview of the parent about the child’s health status and developmental milestone attainment. Noting the age of acquisition of the gross-motor, fine-motor, language, and adaptive-behavioral milestones helps determine a possible developmental delay. Parents are very good historians of their child’s current functioning and recent accomplishments, which is why eliciting a history of milestone attainment during serial clinic visits is so useful in assessing a child’s rate of development. Sometimes additional explanation may be needed to clearly determine the age of acquisition, especially language milestones.
A number of accurate screening tools are available to monitor general developmental progress or domain-specific evaluation. 58 Early delay in language and self-help milestones raises concerns about cognitive development, language disorder, or hearing impairment. 1 Many NICU developmental follow-up clinics rely on pediatric clinical psychologists to formally evaluate the cognition of high-risk infants, preferably with sequential assessments but sometimes with one assessment at a specific age (e.g., at 18 to 24 months corrected age). For infants, the Bayley Scales of Infant and Toddler Development, 3rd edition, is the most commonly used assessment tool in NICU follow-up programs in the United States. 27 For preschool-age and school-age children, several cognitive tests are available, including the Wechsler Preschool and Primary Scale of Intelligence (WPPSI), the Stanford-Binet Intelligence Scales, and the Kaufman Assessment Battery for Children (K-ABC II). 75,112,145
Correction for Degree of Prematurity
One controversy that arises in monitoring developmental scores of preterm infants is whether to correct for degree of prematurity (i.e., whether to use the child’s chronologic age, calculated from birth, or to use corrected age for degree of prematurity). The best evidence supports correcting for degree of prematurity, but whether it is best to correct throughout infancy is controversial, and there is no agreement as to when one should stop correcting for degree of prematurity. 138By convention, most practitioners correct through 2 years of age. It is necessary to be very cautious when interpreting corrected age scores at 12 months or less for ELBW infants. Parental understanding may lead to an overly optimistic outlook that will not be supported by testing at a later date.
Neurodevelopmental Examination
For high-risk infants, the standard pediatric neurologic examination is expanded to include a detailed assessment of posture, muscle tone, reflexes, postural reactions, and functional abilities. Interpretation of the examination requires a thorough understanding of the normal pattern of development over time, the examiner’s skill at assessing the infant’s performance, recognizing deviations from the norm, and determining the significance of these findings. 1,15,18,51
Abnormalities of posture, muscle tone, and reflexes are common in preterm and other high-risk NICU infants during the first year. These abnormalities include asymmetries of movement, marked extensor tone through the neck and trunk with significant shoulder retraction or elevation, hypotonia, and lower extremity hypertonia and hyperreflexia. Cerebral palsy (CP) should be considered in infants with persistent abnormalities in tone, posture, movement, and motor delay. Mild delay and neuromotor tone variation suggest transient neuromotor abnormalities. For many preterm infants, these abnormalities can no longer be elicited at 1 year but they remain at risk for later school and behavior problems. 76
NICU Follow-up Guidelines
The issue of how to do NICU developmental follow-up and how to conduct follow-up studies for high-risk infants has been complex. The methodology can lead to inadequate interpretation of published studies. The National Institute of Child Health and Human Development (NICHD), the National Institute of Neurologic Disorders and Stroke, and the CDC convened a workshop in 2002 to address these issues. Their purpose was to provide standardized guidelines for follow-up care, especially for tertiary care centers with neonatal fellowship training programs in the United States. The results of that workshop have been published and address topics such as risk factors that affect outcome, appropriate assessments, correction for prematurity, assessment tools, and research-related subjects. 138
Again, these criteria may not fit the need or focus of every program. The resources available across states, within communities, and in individual hospitals and the commitment within the NICU strongly drive follow-up programs in different communities. Programs that wish to have developmental follow-up for quality care surveillance and to provide families with information about their high-risk infant can be resourceful in developing partnerships with local early intervention programs and community physicians. Programs that address scientific questions generally need a focused approach or a research network to approach the study. 135
It is now recognized that survival to the end of the NICU stay is a very short-term outcome. It is generally recommended that NICU graduates be followed until at least 8 years of age, but most programs do not have the resources to do this. Furthermore, it has become apparent that the effects of prematurity may extend throughout the life span.64 This is important to know but clearly goes beyond the capability of most NICU follow-up programs.
NEURODEVELOPMENTAL DISABILITIES
Neurodevelopmental disabilities are a group of chronic, nonprogressive disorders of central nervous system (CNS) function that occur as result of malformation of or insult to the developing brain. 1There is a spectrum of neurodevelopmental disabilities, from the major disabilities (CP and mental retardation [MR]), to sensory impairments, and to the more subtle disorders of higher cortical functioning (Box 31-4). Preterm infants are at an increased risk for major disabilities and also for more subtle disabilities. Both may have a long-term impact on their life. 151
BOX 31-4
1. Major disability
Cerebral palsy
Global developmental delay or mental retardation
2. Sensory impairment
Hearing impairment
Visual impairment
3. Subtle disorders of higher cortical function
Language delay or disorder
Expressive language delay
Receptive and expressive language delay
Developmental coordination disorder
Fine-motor incoordination
Sensorimotor integration problems
Learning disability
Variable cognitive abilities
Visual-perceptual problems
Behavior problems
Attention deficit hyperactivity disorder
Social emotional adaptation
Modified from Accardo PJ, editor: Capute and Accardo’s neurodevelopmental disabilities in infancy and childhood, ed 3, Baltimore, Md, 2008, Paul H Brookes; Wolraich ML, editor: Disorders of development and learning, ed 3, Hamilton, Ontario, Canada, 2003, BC Decker.
The World Health Organization (WHO) has revised its definition of disability, impairment, and handicap to an International Classification of Functioning, Disability and Health (ICF) and places emphasis on the interaction of functioning and disability, health condition of the individual, and factors of the environment. The ICF is structured around the following components: (1) body functions and structure, (2) activities and participation, and (3) additional information on severity and environmental factors. 150 This view has aided our thinking not only of the specific disability but also how it affects a child’s ability to function physically and socially within his or her home and community.
Cerebral Palsy
Cerebral palsy “describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.”114Important to this current definition is the recognition of the accompanying disturbances of sensation, perception, cognition, communication, and behavior and by epilepsy and secondary musculoskeletal problems. CP is the most disabling motor impairment found in preterm infants and is difficult to diagnose with any degree of certainty before 6 to 12 months of age. It sometimes takes until the child is 2 years or older before the diagnosis becomes clear. Cerebral palsy occurs in 7% to 18% of preterm infants and is more common in the most immature infants. 33,92
CP has traditionally been classified by physiologic type (tone abnormality), topography (i.e., muscle groups involved), and severity. 1 Again, the most recent recommendation for definition and classification has been expanded and relates to the WHO ICF. 114,150 Components of the currently recommended classification stress motor abnormalities, accompanying impairments, anatomic and neuroimaging findings, and causation and timing. Because consensus has not been clear on the definition of level of severity, the Gross Motor Classification Scale is now commonly used to address motor function, and scales are being developed to more reliably measure other important areas of functioning, such as hand control, speech, and swallowing. 101,114 Persistently increased muscle tone and increased deep tendon reflexes with persistence of pathologic reflexes (e.g., Babinski) are early signs of spasticity. Variable tone with persistent primitive reflexes, often with involuntary movements, is a sign of extrapyramidal CP. The child may be 2 to 3 years old before involuntary movements are seen. Children who manifest signs of both spasticity and extrapyramidal CP have mixed CP. Extrapyramidal CP is generalized, but spasticity should be further typed according to which limbs are most significantly involved.
Spastic diplegia, the most common form of CP in preterm infants, is characterized by spasticity in both lower extremities, with mild or minimal involvement of the upper extremities.1,92Spastic hemiplegia is characterized by involvement of one side of the body, with the upper extremity more involved than the lower. Because intrauterine and perinatal strokes are usually unilateral, children who had strokes often demonstrate spastic hemiplegia. Quadriplegia is the most severe form of spastic CP, with involvement of both upper and lower extremities and the lower more severely affected than the upper. Children with neonatal encephalopathy (whether caused by hypoxia/ischemia, metabolic disorders, or other causes) who develop CP are most likely to have spastic quadriplegia or severe mixed CP.
Global Developmental Delay and Mental Retardation
Developmental delay is used to describe a deficit in any of the five developmental domains (cognition, motor, language, adaptive, social-emotional skills). Global developmental delay is used to define deficits in two or more areas of development with scores more than two deviations below norm referenced standards.123Currently in the United States, a child may receive services through his or her local school district special education program with a diagnosis of developmental delays until almost 8 years of age before the definition may be switched to mental retardation, again based on standardized testing. Standardized tests, such as the Bayley, 3rd edition, have score groupings and definitions to match, such as low average and borderline, for each domain. 27 It is during this early period of detection that early intervention services become important for the high-risk infant. 31,98,104
Mental retardation is a global impairment of cognitive functioning resulting from injury to or malformation of the developing brain that impairs the child’s ability to adapt and function in society.1,150
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