Follow-up of the NICU Patient

Updated: Jan 05, 2016
  • Author: Michael P Sherman, MD, FAAP; Chief Editor: Ted Rosenkrantz, MD  more...
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Overview

Overview

In developed countries, follow-up for neonatal intensive care unit (NICU) patients is commonly performed at designated clinics. [1, 2] From their inception, NICU-related follow-up clinics have focused on outcomes of premature infants. Some clinics perform follow-up for medical conditions (eg, bronchopulmonary dysplasia, posthemorrhagic hydrocephalus); however, the intent in all NICU-related clinics is to determine neurodevelopmental outcomes. Many clinics do both, especially if the institution is part of a research network. [3, 4]

The intent of the NICU-related follow-up should be several-fold. Less-than-acceptable outcomes may result in practice changes within individual NICUs. Most importantly, professionals in the clinic should direct "NICU graduates" to appropriate rehabilitative or social services if they are not aligned with optimal care in their community. [5]

The growth of NICU-related follow-up clinics reflects the increase in a population of infants with complex needs. Currently, many neonates born prematurely or with major malformations survive, whereas just a few decades ago, neonates born with these birth defects died.

At the same time, pediatricians and family practitioners have less experience with the advances in NICU care than they did 2-3 decades ago. In addition, current clinical training schedules give pediatric and family practice house staff only limited time to spend in the follow-up care of NICU graduates. The evaluation of preterm and term infants with complex conditions requires the involvement of professionals from multiple medical, rehabilitative, psychological, and social-service subspecialties. [6]

Follow-up of extremely low-birth-weight infants (ELBW), who have a birth weight of less than 1000 g, from infancy to adulthood has revealed subtle neurodevelopmental problems that require evaluations and interventions that are more complex than previously appreciated. [7, 8, 9, 10] A retrospective analysis of information on ELBW infants from the National Institutes of Child Health and Human Development revealed that these patients have high use of special outpatient services, and efforts to improve these services are needed. [11]

This article is intended to inform pediatricians, family practitioners, other health professionals, and families about the follow-up care of NICU graduates. It includes information about the following:

  • The 2008 update of the American Academy of Pediatrics (AAP) Statement on Hospital Discharge of the High-Risk Neonate [12] and other citations adding supplemental information to this document
  • Apprehension related to follow-up of the late preterm infant (ie, 32-36 weeks' gestation)
  • Concerns that grade I and II intracranial hemorrhages (ICH) have less favorable outcomes than previously appreciated
  • Results of studies of ventricular dilatation after ICH
  • Use of brain-related imaging studies before hospital discharge as a predictor of outcome and early interventions
  • Follow-up of infants with hypoxic-ischemic encephalopathy
  • The relationship of preterm birth to autism and schizophrenia in childhood and later life
  • Resources for assisting families of NICU patients and graduates
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Benefits of Birth at Regional Centers

Published reports for many years have emphasized the benefits of preterm birth at regional centers with advanced perinatal and neonatal services. Regional centers for perinatal care are often an accepted referral institution that is periodically reviewed by state health care agencies.

Regional centers include level III hospitals that must meet high standards of care, and these institutions have many obstetric and pediatric subspecialists. Care at these advanced perinatal facilities is associated with lower mortality and morbidity for very-low-birth-weight (VLBW) infants (< 1500 g body weight).

A recent meta-analysis documented the mortality risk for VLBW and very-preterm (VPT) infants who are born outside of a level III hospital (level III NICUs are defined by the Perinatal Section of the American Academy of Pediatrics). For VLBW infants, mortality for those born in level III hospitals was 21%, compared with 36% in lower-level hospitals; for infants weighing less than 1000 g, mortality was 32% versus 59%. For VPT infants, mortality was 7% versus 12%, respectively. [13]

At level I and level II hospitals, all perinatal caregivers are encouraged to refer pregnant women to level III hospitals for delivery if the labor occurs very prematurely, particularly if the infant is likely to have very low birth weight. This referral assumes safe transport of the mother and fetus so that delivery does not occur during transit. The mother and fetus must also be in a reasonable state of health so that complications do not occur during transport.

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Patient Education

A structured teaching plan must be individualized for the primary caregivers to educate them in the infant’s care. Each infant has unique needs, and the education program should be directed at those needs. A checklist is essential to accomplish all of the required teaching needs (e.g., cardiopulmonary resuscitation, gastrostomy feedings, total parenteral nutrition administration, monitor use). The appropriate educators must be identified to provide the training. Research shows that training educators for this activity results in better training of the primary caregivers at home. [14]

The goal of this education is to ensure that the parents are capable and confident in caring for their infant at home. It may be appropriate for caregivers to stay overnight and provide care with minimal staff interventions. The staff providing care should not appear overprotective.

Two or more caregivers in the home must receive this training. Having an additional caregiver allows respite for the primary caregiver. A young mother with other small children and no grandmother or husband can be particularly stressed. The role of confounding factors associated with siblings in the home must be considered in the teaching.

For extremely complicated care situations (eg, infants with a tracheostomy), parents should have a rooming-in experience before discharge occurs. This is probably a good practice for all families before they go home with their infant but is especially important for infants who have ongoing, complex problems. Home visits by experienced home health care professionals and/or follow-up telephone calls are essential for the success of the transition process.

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Resources for Parents and Health Care Professionals

To meet the complex needs of the neonatal intensive care unit (NICU) graduate, parents and other caregivers may require educational material that covers conditions that require special evaluation or care after the infant is discharged from the hospital. Resources available to parents and caregivers today are diverse and include medical and lay journals, books and other print materials, videotapes, and Web sites.

The information below is provided without any endorsement of the resources. Articles in medical journals are usually peer reviewed (ie, evaluated and accepted by experts in the field) before publication. The accuracy of information provided in lay journals and on Web sites can be suspect. Consultant experts may not have reviewed information from these sources. Physicians should always investigate the particular sites that caregivers are using to obtain information.

Because of the emotional turmoil that having an infant in an NICU can cause, patients and caregivers often find benefit in joining a support group. Information about such groups is available, especially on the Internet. Support by hospital personnel and other parents may be the best therapy. In addition, specialized units or hospitals (e.g., Shriners Hospitals for Children, Variety Children's Hospitals) are available to manage some conditions. Caregivers should be aware that children’s hospitals, especially those supported by the Children’s Miracle Network, may have additional resources available that are not available at large general or university hospitals.

Books and pamphlets for parents

The following books for parents are available online and from other booksellers.

  • Preemies: The Essential Guide for Parents of Premature Babies, Second Edition, 2010, Authors: Dana Wechsler Linden, Emma TrentiParoli, Mia Wechsler Doron, MD; Pocket Books, New York, NY.
  • Intensive Parenting: Surviving the Emotional Journey through the NICU, 2013, Authors: Deborah L. Davis, PhD, Mara Tesler Stein, PsyD; Fulcrum Publishers, Golden, CO.
  • The Preemie Primer, 2010, Author: Jennifer Gunter, MD; Da Capo Press, Perseus Book Group, Philadelphia, PA.
  • For the Love of Babies: One Doctor’s Stories about Life in the Neonatal ICU, 2011, Authror: Sue L. Hall, MD; WorldMaker Media, Newton, MA.

The following books, pamphlets, and videotapes may assist parents and health care professionals in the care of high-risk infants during and after hospital discharge. The list is not exhaustive and newer resources become available frequently. State governments may have pamphlets useful for local needs, so visit the appropriate Web site.

  • Brown L, Irwin L. Parent to Parent: Encouraging Connections between Parents of Children with Disabilities. Madison, WI: Wisconsin Department of Health and Social Services Birth to Three Program; 1992
  • Brown PH, Thurman K, Pearl LF. Family Centered Early Intervention with Infants and Toddlers: Innovative Cross Disciplinary Approaches. Baltimore, MD: Brookes; 1993
  • Bryant DM, Graham MA. Implementing Early Intervention: From Research to Effective Practice. New York, NY: Guilford; 1993
  • Catlett C, Winton PJ. Resource Guide: Selected Early Childhood/Early Intervention Training Materials. 11th ed. Chapel Hill, NC: FPG Child Development Institute, University of North Carolina at Chapel Hill; 2002
  • Ensher GL, Clark DA. Newborns at Risk: Medical Care and Psycho-educational Intervention. Gaithersburg, MD: Aspen; 1986
  • Geenstein D, Miner N, Kudela E. Backyards and Butterflies: Ways to Include Children with Disabilities in Outdoor Activities. Cambridge, MA: Brookline Books; 1997
  • Geralis E, ed. Children with Cerebral Palsy: A Parents Guide. Bethesda, MD: Woodbine House; 1998
  • Green M, ed. Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents. Arlington, VA: National Maternal and Child Health Clearinghouse, 1998
  • Flehmig I. Normal Infant Development and Borderline Deviations. New York, NY: Thieme Medical; 1992
  • Hagan Arnold J, Buschman Gemma P. A Child Dies: A Portrait of Family Grief. Philadelphia, PA: Charles; 1994
  • Hanson MJ. Atypical Infant Development. Austin, TX: Pro-Ed; 1996
  • Hanson MJ, Lynch EW. Early Intervention: Implementing Child and Family Services for Infants and Toddlers Who Are at Risk or Disabled. Austin, TX: Pro-Ed; 1995
  • Hanson MJ, Vandenberg KA. Homecoming for Babies after the Intensive Care Nursery: A Guide for Parents in Supporting Their Baby’s Early Development. Austin, TX: Pro-Ed; 1993
  • Johnson BH, Jeppson ES, Redburn L. Caring for Children and Families: Guidelines for Hospitals. Bethesda, MD: Association for the Care of Children’s Health; 1992
  • Judge SL, Parette HP. Assistive Technology for Children with Disabilities: A Guide to Family-Centered Services. Cambridge, MA: Brookline Books; 1998
  • Kurtz L, Batshaw M, Dowrick P, Levy SE, eds. Handbook of Developmental Disabilities: Resources for Interdisciplinary Care. Austin, TX: Pro-Ed; 2004
  • Meisells SJ, Provence S. Screening and Assessment: Guidelines for Identifying Young Disabled and Developmentally Vulnerable Children and Their Families. Washington, DC: National Center for Clinical Infant Programs; 1989
  • Meyer D, Vadasy P. Living with a Brother or Sister with Special Needs: A Book for Sibs. Seattle, WA: University of Washington Press; 1996
  • National Early Childhood Technical Assistance System. Part H Updates (The program for children from birth to age 2). Chapel Hill, NC: National Early Childhood Technical Assistance System; 1998
  • Pediatric Projects. Support Groups for Parents of Children in Healthcare: A Bibliography of Professional and Popular Literature. Tarzana, CA: Pediatric Projects
  • Porkorni JL. Developmental Intervention for Hospitalized Infants: The Neonatal Intensive Care Unit (NICU) Series (7 videotapes or DVDs). Van Nuys, CA: Child Development Media, Inc.
  • Schleichkorn J. Coping with Cerebral Palsy: Answers to Questions Parents Often Ask. Austin, TX: Pro-Ed; 1993
  • Sell E, Hill-Mangan S, Ratzan P. Your Baby and You: Understanding Your Baby’s Behavior (video and manuals). San Antonio, TX: Communication Skill Builders; 1992
  • Stengle LJ. Laying Community Foundations for Your Child with a Disability: How to Establish Relationships that Will Support Your Child after You’re Gone. Bethesda, MD: Woodbine House; 1996
  • Swan WW, Morgan JL. Collaborating For Comprehensive Services for Young Children and Their Families: The Local Interagency Coordinating Council. Baltimore, MD: Brookes; 1992
  • Thom V, Krahn G, Hale BJ, et al. Supporting Families and Their Prematurely Born Babies: A Guide for Training Care Providers. Portland, OR: Child Development and Rehabilitation Center, Oregon Health Sciences University; 1990
  • Well SA. A Multicultural Education and Resource Guide for Occupational Therapy Educators and Practitioners. Bethesda, MD: American Occupational Therapy Association; 1994
  • Woodrich DL. Children’s Psychological Testing: A Guide for Nonpsychologists. 3rd ed. Baltimore, MD: Brookes; 1997
  • Zipper IN, Weil M, Rounds K. Service Coordination for Early Intervention: Professionals and Parents. Cambridge, MA: Brookline; 1993

Internet resources

The American Academy of Pediatrics (AAP) is a national organization of pediatricians that is devoted to the health and well-being of infants and children. The American Academy of Pediatrics Web site has many policy statements regarding the proper care of infants and children and offers information about the care of premature infants and childhood immunizations.The items to be found include “A Parent’s Guide to Safe Sleep,” which explains the prevention of sudden infant death syndrome (SIDS). The AAP bookstore sells an extensive collection of pamphlets and books that cover the medical and psychosocial care of infants and children. Among the pamphlets is Early Arrival: Information for Parents of Premature Infants.

The Premature Birth Website of the March of Dimes Foundation is an outstanding site for parents and professionals that discusses preventing prematurity and diseases of pregnancy that cause preterm birth. The site offers information about the diseases infants might have, and it provides information about neonatal nutrition, including the importance of breastfeeding. The site addresses disabilities in prematurely born infants. Resources are available in Spanish.

Shriners Hospitals for Children offer care to infants and children who have qualifying medical conditions, such as spina bifida, cleft lip and/or palate, orthopedic anomalies, and especially cerebral palsy. The Shriners Hospitals Web site provides information on the location of Shriners Hospitals and on patient referrals. In addition, clinicians or parents may call a toll-free patient-referral line to determine if a particular child qualifies; the referral numbers are 1-800-237-5055 in the United States and 1-800-361-7256 in Canada.

A Primer on Preemies from the KidsHealth for Parents Website sponsored by medical experts at the Nemours Foundation [http://kidshealth.org/parent/]. It summarizes the NICU and conditions common to premature infants. Resources are available in Spanish.

Parents of Premature Babies Inc (Preemie-L) offers a good compendium of on-line and off-line resources.. Preemie-L is a non-profit foundation supporting families of children who arrive in this world more than 6 weeks prior to the due date. This site has a discussion forum for parents.

Child Care Resources has a list of day care requirements needed to establish programs for special-needs children. There are recommendations for child care. It provides insight into early childhood learning resources. The site also discusses how the Affordable Care Act may influence services.

Additional resources

To obtain additional information or help with finding support groups, parents can also ask the neonatologist, the nursing supervisor, and/or the social worker for resources in or near the hospital. Some facilities provide parents with a pamphlet that contains this information when their child is admitted.

Community agencies, such as the March of Dimes, and religious organizations may offer support services. Local and national support groups have been organized for infants and children with certain conditions. Examples include the Down Syndrome Foundation, the Cystic Fibrosis Foundation, and the Little People of America (addressing dwarfism). Many rare disorders present in newborn infants have parent support groups and can be found via an Internet search.

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Criteria for Hospital Discharge

In 1998, the American Academy of Pediatrics (AAP) published guidelines related to "Hospital Discharge of the High-Risk Neonate." In November 2008, the AAP released an update of these guidelines. [12] This information has been supplemented by discharge planning for late preterm infants, [15] the evaluation of extreme premature infants for discharge, [16] the special needs for discharging infants born at the limits of viability, [17] and the reasons for rehospitalization after discharge of very preterm infants. [18]

The 2008 AAP monograph places high-risk neonates into the following 4 categories:

  • Preterm infants
  • Infants with special health care needs or dependence on technology
  • Infants at risk because of family issues
  • Infants with anticipated early death

Preterm infant

No set weight criteria for discharge have been established, and studies reveal that preterm infants can be discharged safely and well while weighing less than 2 kg. [19] Discharge should be based on physiologic maturity and stability of the infant. A principal criterion for discharge is the resolution of medical or surgical problems that require continued hospitalization. Additional criteria generally involve the following:

  • Weight gain and feeding
  • Temperature stability in open crib
  • Training and comfort of parents and caregivers
  • Pre-discharge health care maintenance
  • Post-discharge environment
  • Support team and follow-up

These criteria for discharge are applicable to all ill neonates and not just those born prematurely.

Weight gain and feeding

Weight gain of 15-30 g/day must continue over a reasonable time (several days to 1 week). This weight gain should approximate fetal or early postnatal growth. In addition, weight gain must be associated with the patient's ability to feed by mouth or by other methods (eg, gavage tube, gastrostomy, intravenous nutrition). Feedings must be accomplished without any distress or problems. The parents should demonstrate competence in using the method or technique selected for the infant’s nutrition.

Weight gain should occur while the infant is in an open environment (eg, crib). The infant must have been clothed appropriately, and the body temperature should be maintained in the normal range for at least 48 hours in this environment (ie, thermal stability achieved).

Training and comfort of parents and caregivers

Parents or other assigned caregivers must be trained adequately and must be comfortable in all aspects of the preterm infant’s care, including the administration of medications and the use of technical devices (eg, monitors, aerosol delivery equipment). Parents may pressure physicians to order a home monitor, but monitors are infrequently needed (see Apnea of Prematurity). In the hospital, the parents' performance with their infant must be adequate before discharge.

Predischarge health care maintenance

Certain aspects of health care maintenance must be performed before discharge. Appropriate metabolic screening must be completed. Anemia should be assessed, and its follow-up care must be established.

Patients who had prolonged stays may need to begin age-appropriate immunizations before discharge. Consult the AAP Red Book, [20] Immunization Information for Parents and Important Information for Clinicians from the AAP, or Immunization Schedules from the Centers for Disease Control and Prevention (CDC). [21]

All neonatal intensive care unit (NICU) patients should receive a hearing test before they are discharged from the hospital. The infants should have an appointment with an audiologist soon after discharge if testing in the hospital is abnormal.

For preterm infants, revised criteria have been published in 2013 regarding the assessment of retinal vascularization by a trained ophthalmologist. [22, 23] For transferred infants, a qualified pediatric eye specialist must be available at the accepting facility. For outpatients, the infants must be seen by a qualified ophthalmologist according to the designed schedule.

Postdischarge environment

An assessment of the postdischarge environment, possibly including an on-site evaluation of the home, is emphasized as part of discharge planning. This very effective evaluation is rarely performed.

The parents and the home environment must be suitable so that neither neglect nor physical abuse is likely to occur. Indicators of concern can include the frequency of parental visiting and the physical involvement of parents in care during the infant's hospital stay. Knowledge of poor parenting skills with other children, a history of marital discord, past or present substance abuse, or criminal activity requires that hospital and outside social services be involved.

Reports may be required for submission to local or state authorities. A contract may need to be signed by parents so benchmarks of care are set.

All medical equipment, termed "special technologies," required at home should be in place and in working order. Training of the caregivers should be such that they are experts. The caregivers must have emergency numbers to call when equipment malfunctions or supplies become meager.

Support team and follow-up

A program of parental support, including in-home visits by health care professionals and assistance by family and friends, should be in place before discharge occurs. Community services should be informed and willing to help. [11]

Prior to discharge, the NICU program must be responsible for coordinating visits among different consultants that will occur after discharge. A primary care physician (PCP) should have been identified during the mother's pregnancy; if not, one should be chosen early in the patient's hospital stay. The PCP must be familiar with the care of high-risk neonates and should help coordinate subsequent visits to subspecialists. The PCP also provides ongoing health care maintenance.

The most important tasks of the PCP are ensuring adequate nutrition and proper growth and development of the patient. These duties may be performed in conjunction with follow-up programs of the NICU from which the patient graduates or with follow-up programs of private or governmental agencies that specialize in infant development.

Obstetric and neonatal attending physicians should begin verbal and written communication with the PCP, and vice versa, shortly after the baby's birth. Team members should periodically provide the PCP with verbal and written reports about the progress of the future NICU graduate so that information is available in the outpatient record. An organized system must exist to assess the physical and psychological outcome of the NICU graduate.

Infant with special health care needs or dependence on technology

With the emergence of managed care, early discharge of infants with active medical and/or surgical conditions is common. [24, 25] Infants may leave the hospital with many unresolved issues, such as a need for nutritional assistance (eg, feeding by means of gavage or intravenous alimentation), for respiratory support ranging from supplemental oxygenation to assisted ventilation through a tracheostomy, and for maintenance of indwelling or external medical devices (eg, ventriculoperitoneal shunt, cardiorespiratory monitor, urinary catheter).

In patients requiring nutritional assistance, the ability of parents to provide adequate nutrition by the appropriate method is vital to success, as is the ability to prevent or recognize complications of gavage or gastrostomy feedings. Caregivers who must give home parenteral nutrition to their infant require special training to avoid infection and other complications (eg, hepatocellular liver disease).

Some infants, such as those with myelodysplasia, may require repeated urinary catheterizations. Caregivers must be trained in catheterization techniques and complications.

In patients with tracheostomies, the PCP must know the risks of clinically significant neurologic morbidity or mortality associated with the home care of tracheostomies during infancy. [26, 27, 28] Tracheostomy care may also involve assisted ventilation at home. In this circumstance, care by the PCP should involve consultation with a pediatric pulmonologist.

Home oxygen therapy may allow earlier discharge and avoid growth failure and chronic pulmonary hypertension. The infant must receive sufficient oxygen to accomplish these goals. These infants require a functional home oxygen saturation monitor. Care by a physician experienced in weaning oxygen in an appropriate manner is essential.

Parents usually do not anticipate complex home medical care for their infant at the beginning of a pregnancy. When the medical condition requires extensive care in the home, the parents may be in need of a respite to perform other family duties or to have some leisure time. Planning for respite care for the parents should be part of the discharge process.

Infant at risk because of family issues

Problems with bonding between parents and their infant are reported with prolonged hospitalizations. [29, 30] The NICU is a stressful environment for parents, and the environment itself may hinder bonding. Infants who are born prematurely, have congenital defects, or have a chronic disease are all at increased risk for experiencing physical harm or neglect.

Other risk factors for an adverse outcome in the home environment are as follows [31] :

  • Low educational level
  • Lack of family help
  • Unstable marriage or relationship
  • Sporadic or no medical care during pregnancy
  • Use of illicit substances or alcohol abuse during pregnancy
  • Infrequent family visits during the infant’s hospitalization

The problem of adverse outcomes in the home environment has led to the widespread use of foster homes for the care of NICU graduates. If the foster home environment is a transitional approach, continuing medical care should be available after discharge.

Child protective services should monitor visitation by the natural parents. The parents' participation in a rehabilitation program is essential in planning to reunite infants with their parents. The likelihood of success in reuniting infants with their parents increases if the parents comply with a structured rehabilitation program.

If an infant is to be discharged to a home where illicit substances were used, a home visit before discharge and many home visits after discharge are necessary to protect the infant's health and well-being. Child protective services must be involved continuously in the care and treatment of infants in this situation.

Infants with anticipated early death

Home hospice care for neonates with lethal conditions (eg, trisomy 13, trisomy 18) has increased. [32, 33] This type of care is increasingly available, but several factors are needed for its success. [34] With the advent of prenatal ultrasound examinations and genetic studies of the fetus, families may discuss neonatal hospice before birth. [35] Initiating a hospice plan following a conference that includes the family, invited visitors (eg, clergy, friends), caregivers, and social workers is appropriate. Neonatal ethics has offered an opinion about more aggressive treatment of these infants, and it is recommended that parents’ rights be respected in the decision-making process. [36, 37]

If the parents request hospice care, death of the baby at home is often the best option. A "do not resuscitate" (DNR) order should be in place before discharge. [38] Parents must have multiple copies of a letter that explains that the infant has a fatal condition for which resuscitation would be futile and a DNR order is in place. One or more contact physicians for the infant should be identified.

A multidisciplinary approach must be established before discharge. If available, skilled professionals experienced in the hospice care of infants should be present in the home. However, few professionals are trained to provide hospice care to graduates of NICUs. Daily home visits may be needed just after discharge and near the terminal event. Distress or discomfort must be promptly alleviated, if possible. Arrangements must be made for the family’s needs, including the process of bereavement. Social services and/or clergy must be involved.

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Discharge Planning

From the initiation of intensive care, high-risk neonates should have an identified primary care physician (PCP). This person should become part of the discharge planning team. Unfortunately, finding a PCP and the degree of the PCP's involvement are problems in NICUs throughout the United States.

Often, medical funding for the infant (eg, Medicaid) makes identifying a PCP difficult for parents or caregivers early in the hospital stay. Moreover, the education of the parents may result in a lack of aggressiveness in securing the services of a PCP or the involvement of other community resources.

Most importantly, the PCP must have training and experience in caring for various problems associated with high-risk neonates. A significant number of NICU graduates require the services of a PCP, a neurodevelopmental follow-up clinic, and pediatric medical or surgical subspecialists after discharge. A common example is the medical follow-up of the extremely low-birth-weight (ELBW) infant who has ongoing bronchopulmonary dysplasia and requires a pediatric pulmonologist to work with the PCP.

The ideal follow-up clinic has physicians that can perform assessments of ongoing medical problems and a neurodevelopmental team that undertakes psychological evaluations and appropriate interventions (eg, team consisting of a neonatologist or pediatrician, psychologist, dietitian, physical therapist, occupational therapist, speech therapist, social worker).

Each NICU should have a discharge-planning group consisting of designated professionals. The members should include at least the following:

  • A discharge planner or case manager
  • One or more social workers
  • Nursing representatives (eg, nurse manager, clinical nurse educator, neonatal nurse practitioners)
  • Physicians (always the attending neonatologist plus others)
  • Community service representatives (eg, home health nurses, Medicaid case workers, protective services case workers, personnel from private or government-funded neurodevelopmental follow-up clinics)

Discharge planning should involve the above professionals. Because of rules related to the protection of patients' information, certain participants (eg, Medicaid and protective services case workers) may need to discuss their cases first and then leave when other patients are being discussed.

NICUs that maintain a high census and that care for high-acuity patients may have a discharge planner, a neonatologist, one or more nursing representatives, dietitians, physical therapists, occupational therapists, speech therapists, respiratory care practitioners, and representatives from the NICU follow-up clinic. On occasion, surgical subspecialists, pediatric subspecialists, or the PCP may be invited to the meetings to offer advice regarding infants with complex problems.

A busy office practice often precludes the PCP from attending these meetings. Nevertheless, PCPs should be given periodic reports on the progress of their future patients after every meeting and must be willing to assume care upon discharge.

The time of day, the designated day of the week, and the frequency of the meetings associated with discharge planning depend on the needs of a given NICU. For NICUs with large populations, the frequency, day, and time depend on having an optimal number of team members present. For small NICUs, formal discharge-planning rounds may be inefficient.

Few published reports make recommendations about the discharge-planning process. One summary is the 2008 policy statement from the American Academy of Pediatrics (AAP) Committee on Fetus and Newborn. [12]

Hospital discharge guidelines

The 2008 AAP Guidelines on Hospital Discharge has indicated 6 critical components of the discharge-planning process, as follows:

  1. Parental education
  2. Completion of appropriate elements of primary care in the hospital
  3. Development of management plan for unresolved medical problems
  4. Development of a comprehensive home care plan
  5. Identification and involvement of support services
  6. Determination and designation of follow-up care

Completion of appropriate elements of primary care

The implementation of primary care includes more than just identifying a PCP within the first week of the patient's hospitalization. In and out of the hospital, primary care involves a range of responsibilities, including the following:

  • Assessment of the infant's nutrition and growth
  • Immunizations before and after discharge
  • Indicated respiratory syncytial virus (RSV) prophylaxis
  • Car and home safety
  • Neurodevelopmental outcomes (including hearing and vision screening)

Among the most important follow-up items are monitoring for serious anemia and continuing assessment of retinopathy until its resolution. In-depth nutritional evaluation is mandatory for infants who received prolonged parenteral nutrition, who had GI anomalies, and who may have either an inborn error of metabolism or other metabolic/biochemical disorders.

Development of management plan for unresolved medical problems

A list of unresolved problems must be developed during the patient's hospital stay, and this list must be made available to the PCP. Ongoing conditions should have been completely diagnosed, and a management plan for diseases that persist must be established at the time of discharge.

The plans for ongoing care by the PCP must be transmitted in detail. The current treatment plan and all medications the infant is receiving should be conveyed to the home health professionals and the PCP before discharge. Resolved problems should also be identified at the time of discharge because some unanticipated complication may arise. Individual infants have received the best in-home and follow-up care with this method.

Development of a comprehensive home care plan

The AAP guidelines for technology-dependent infants are helpful in the discharge-planning process. [12] Publications about the care and outcomes of NICU graduates requiring complex technology are limited. [39, 40, 11] The plan of care in the home should have the following elements:

  • Identification and training of in-home caregiver
  • Planning for optimal nutrition and follow-up (eg, total parenteral nutrition, gastrostomy feeds)
  • A list of equipment, supplies, and resources that are needed at home
  • Referrals to home health care professionals and community resources that can provide ongoing assessments and care after discharge and the full knowledge that the PCP understands what resources have been initiated
  • An assessment of the home environment to determine that it is suitable for care and to determine what improvements must be made before discharge
  • A plan for emergency care and transport should the need arise
  • Assessment of financial resources identified by using either indemnified insurance or governmental programs to finance future hospital, office, and home health care needs

When appropriate, Social Security Income (SSI) must be applied for during the hospital stay; applications for other free services (eg, care at a Shriners hospital) must be under way at discharge. PCPs must be aware of the elements in the plan of home care. This is especially true when the PCP does not participate in their implementation.

Identification and involvement of support services

The ability of the primary caregiver and other family support members to deliver care must be assessed before discharge. Ongoing assessment of the caregiver's physical and emotional abilities to continue providing care at home must be established.

In-home evaluations regarding the availability of supplies, medications, complicated technologies, and nutritional support must be started before discharge. Healthcare workers who provide ongoing home evaluations and who can identify new problems are essential for a favorable long-term outcome for the NICU graduate.

Determination and designation of follow-up care

The discharging neonatologist has overall responsibility for follow-up care at discharge. Ongoing communication between the neonatologist and the PCP during the hospital stay and at discharge improves outcomes for the infant.

Early identification of a PCP is important. In the ideal circumstance, the PCP reviews the records, examines the infant, and meets with the family before discharge. In the era of managed care, the busy office practice of a pediatrician or family practitioner may not allow for such a review before discharge.

In some cases, the skills of the PCP are limited relative to the complex problems of the infant. Therefore, appropriate follow-up care with surgical and pediatric subspecialists is necessary. Teamwork is the key to success regarding discharge planning and follow-up.

Most important among the subspecialist visits is a follow-up appointment with a neurodevelopmental specialist. Although such an appointment may be in the distant future, a list of scheduled and unscheduled (but anticipated) appointments should be made and given to the parents and PCP before discharge.

The discharge-planning process is usually well established at tertiary NICUs; however, level II nurseries should also conduct such activities. Infants may be transported close to their home for convalescent care, and the staff at the level II nursery is responsible for determining the infant's health status (eg, the absence of potentially blinding retinopathy of prematurity) before discharge. If retinal pathology is still active, an appropriate discharge plan must be formulated or preventable visual disability may be the outcome. [41]

Arrangements for follow-up appointments may be made locally; however, this is not always possible. Some level II nurseries may be in relatively rural areas, and the infant still needs to return to the tertiary care center for certain services (eg, ophthalmologic examinations, hearing assessments, developmental follow-up). In rural areas, home services may also be limited, which means that the PCP has additional responsibility in evaluating the transition to home care.

Finally, the NICU staff is responsible for assessing the performance of a home health care agency and of its workers' abilities to provide quality care to the infant who is technology dependent. If an agency's staff does not perform properly, they should be so informed. If the quality of care does not improve, alternative arrangements for care must be sought.

In addition to the guideline components discussed above, the 2008 AAP guidelines also contain recommendations for infant readiness for hospital discharge, family and home environmental readiness, and community and health care system readiness. Caregivers, whether they are hospital-based or home-based, should be familiar with these summary guidelines.

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Long-Term Monitoring

The major goals of the pediatrician or family practitioner who monitors a neonatal intensive care unit (NICU) graduate include the following:

  • Provide an ongoing assessment of growth
  • Evaluate the adequacy of nutrition
  • Deliver preventive care
  • Periodically examine the infant's, child's, or adolescent's motor, intellectual, and behavioral development

This section mainly covers the assessment of growth and nutrition and the delivery of preventive care. Periodic examination of development is discussed in the Developmental Follow-up sections below. Another important aspect of health care maintenance of the NICU graduate is health supervision and anticipatory guidance.

Assessment of growth

The first duty of the primary care physician (PCP) is to accurately monitor the growth of the NICU graduate. [42, 43, 44] Therefore, the patient's weight, length, and head circumference must be plotted on appropriate growth charts and evaluated over time.

Any infant with a growth rate in the lower percentiles of the curve or whose growth curve flattens or decelerates should have the causes assessed. If improved nutritional support does not reverse the growth pattern or if diagnostic studies do not reveal an obvious cause, referral to an endocrinologist, gastroenterologist, and/or dietitian is indicated.

Growth rates

For infants who were born prematurely, growth should be plotted after it is corrected for the patient's gestational age at birth. Special growth charts are available for this purpose. [45] Growth charts for term infants hospitalized in the NICU are also available. [46] Graduates of the NICU often have accelerating growth patterns after discharge. This is particularly true for preterm infants. [47]

In the first 3-4 months after birth, normal weight gain averages 15-40 g/day and then declines, reaching about 5-15 g/day by the age of 12-18 months. From a postmenstrual age of approximately 40 weeks until month 4 of postnatal life, weight proportionally increases more in infants who were born prematurely than in those who were born at term.

The rate of accelerated growth in preterm infants is still larger than that in term infants, but the magnitude is far less. In the months after preterm infants are born, the crown-heel length may incrementally increase by 0.8-1.1 cm/wk, whereas term infants gain a mean of 0.7-0.75 cm/wk. By the age of 12-18 months, the gain in length declines to 0.75-1.5 cm/mo.

During the first 3-6 months after discharge, the velocity of growth related to weight, length, and head circumference has been inadequately studied in NICU graduates. The reason may lie with difficulties in establishing the values for the healthy preterm or term infant. Investigators would need to address birth-weight categories, gestational ages, sex, race, nutritional methods, countries where the studies are performed, and ongoing diseases that affect growth.

A study of infants with birth weights of 750-2500 g in Brazil showed substantial growth deceleration by age 12 weeks. [48] Ehrenkranz et al reported on the longitudinal growth of hospitalized VLBW infants in the United States; however, the duration of study was limited to near-term gestation. [45] Therefore, the study lacked information about growth after hospitalization, which is crucial information needed by the PCP.

Head growth

Measurement and documentation of head growth, a predictor of future outcome, [49, 50] is especially important in this high-risk population. In preterm infants, head growth correlates with magnetic resonance imaging findings and neurodevelopmental outcome. [51] An assessment of head growth is especially important in patients with a history of a chromosomal disorder, brain insult secondary to hypoxic-ischemic encephalopathy, or a metabolic disease.

The largest frontal-to-occipital plane is used to determine the patient's head circumference. For preterm infants, the increase in head circumference is in the range of 0.7-1 cm/wk, whereas the increase in term infants averages about 0.5 cm/wk during the immediate postnatal period.

Increases in head circumference of more than 1.25 cm/wk suggests ventricular dilatation (ie, hydrocephalus or other causes of increased intracranial pressure [eg, subdural hematoma]). If ultrasonography reveals that ventricular size is stable or declining after intraventricular hemorrhage, the risk of post-hemorrhagic hydrocephalus is small. This issue is generally resolved before the patient is discharged from the NICU. Other causes of accelerated head growth include autism or genetic or metabolic disorders. A rapid deceleration in head growth occurs by age 12-18 months. Beyond that age, the increase is only 0.1-0.4 cm/mo.

Growth failure

Growth failure is a concern among preterm babies who weigh less than 1500 g at birth (very low-birth-weight [VLBW] infants), and particularly those who weigh less than 1000 g at birth (extremely low-birth-weight [ELBW] infants). The National Institute of Child and Human Development (NICHD) Neonatal Research Network has reported that 97% of VLBW infants and 99% of ELBW infants had weights less than the 10th percentile at the postmenstrual age of 36 weeks. [52] Extremely low birth weight infants may never reach the height of their full-term peers. [53]

Growth failure begins during the NICU hospitalization in this group. It is now the focus of many providers who are concerned about the late consequences of this poor growth that occurs during a critical period of brain development. Growth failure may have many origins in NICU graduates. [54, 55, 56]

As noted above, infants who weigh less than 1500 g at birth must undergo frequent assessments of growth. The PCP should know whether preterm infants or those small for gestational age (SGA) are gaining at least 20 g/day before discharge. If growth is less than 20 g/day, a plan should be in place before the patient is discharged from the NICU and communicated to the PCP, who should carefully follow up the patient's growth.

A prospective study by the NICHD of infants who weighed 500-1000 g at birth showed that their growth in the NICU influenced subsequent neurodevelopmental and growth outcomes in positive or negative ways. [57] The PCP should be aware of their patient’s growth characteristics in the NICU.

The type of nutritional support may also help in identifying infants at risk for growth failure. These infants include those who are breastfed, infants given special formulas, and infants who are receiving total parenteral nutrition for longer than 4 weeks or who are still receiving total parenteral nutrition at discharge. [58]

Despite some increased risks associated with breast milk–related nutrition in ELBW infants (eg, osteopenia of prematurity), neurodevelopmental outcomes at age 18 months are more favorable in breastfed infants than in others. [59] Infants requiring nasogastric tube or gastrostomy feedings after discharge are at significant risk for impaired growth.

Infants may also be at risk for nutritional deprivation, depending on their disease states. Common neonatal conditions for which an evaluation for growth failure is required include the following:

  • Chronic lung disease of prematurity (ie, bronchopulmonary dysplasia)
  • Severe central nervous system injuries or birth defects
  • Congenital heart disease
  • Esophageal and intestinal anomalies
  • Chronic renal disease
  • Inborn errors of metabolism
  • Chromosomal and/or major malformation syndromes

To rectify growth failure, the PCP must understand its origins, especially in the very preterm infant. [60] For example, an infant with congenital heart disease may have growth failure because of feeding difficulties associated with congestive heart failure and an increased work of breathing. Corrective surgery may be the only solution for this condition.

An infant with severe perinatal asphyxia may be unable to suck and swallow because of brain injury. Such an infant may require a permanent gastrostomy (and gastric fundoplication) to ensure adequate nutrition. Even when this is accomplished, the brain insult may still result in poor growth secondary to hypothalamic and pituitary effects or other yet-undefined consequences of severe cerebral damage.

A premature infant recovering from severe bronchopulmonary dysplasia may have reduced growth because of pulmonary disease, which increases the work of breathing, and inadequate protein intake. Severe chronic lung disease in the NICU graduate is commonly associated with gastroesophageal reflux (GER). [61] In infants with GER, alleviating esophageal pain with H2 receptor antagonists or proton-pump inhibitors in conjunction with prokinetic agents may mitigate the problem and promote increased feeding volumes and weight gain. However, use of these agents in infants has been associated with necrotizing enterocolitis. [62, 63]

Convalescent infants who had severe necrotizing enterocolitis may have an insufficient epithelial surface or a damaged mucosa that does not allow adequate absorption of nutrients from the gut. Persistent poor growth in these infants may require a return to partial parenteral nutrition.

Completion of catch-up growth

Controversy surrounds the age at which catch-up growth is complete for infants who were born prematurely; many clinicians now believe that catch-up growth is not complete until age 2.5-3 years. In some small-for-gestational-age (SGA) infants, body mass may rapidly increase, but a substantial number have little catch-up growth. [64] These SGA infants should be referred to a pediatric endocrinologist, because therapy with recombinant human growth hormone may be useful in some cases.

Early growth and adult disease

A current concern involves the rate of catch-up growth and its association with an increased risk of obesity and heart disease in later life. [65] In part, the pathophysiology of low birth weight and later adult disease is derived from the Barker hypothesis. [66] Emerging data are causing a reexamination of the best rate for catch-up growth and of the strategy to avoid the consequences low birth weight. [65, 67]

Greer presents a critical review of this topic and recommends postdischarge nutrition. [68] The benefits of breastfeeding appear to substantially affect adult diseases associated with the Barker hypothesis. [69] Continued and prolonged use of breast milk for nutrition after discharge is associated with higher Bayley Mental Development Index scores in ELBW infants assessed at 30 months. [70]

Evaluation of nutrition

Nutritional assessment begins with a complete history and physical examination. [71] Evaluation of the patient's general health status, heart rate, breathing rate, temperature control, and fluid balance are parts of this examination. Also included are anthropometric measurements, including weight, length, head circumference, and sometimes skin-fold thickness, which are plotted over time. The PCP should also study the patient's fluid and mineral intake and appraise caloric and substrate consumption.

Increases in weight versus length may differ in NICU graduates who are having problems with adequate nutritional intake. A comparison of the 2 measurements (eg, length increasing faster than weight) can provide evidence of nutritional sufficiency.

The PCP must also monitor the route by which nutrition is provided. Parenteral nutrition, enteral nutrition, or both may be used to attain adequate nutrition. Inadequacies in delivering nutrients by either the parenteral or enteral route must be recognized.

The PCP may be untrained or inexperienced in this type of nutritional evaluation; consequently, the role of follow-up care of the NICU graduate must often include a pediatric dietitian, and a specialized follow-up clinic may be needed for certain infants. When access to a follow-up clinic and a pediatric dietitian is limited, the PCP may need to use a home feeding diary and/or a nutritional assessment sheet to gather additional information on exact caloric intake and the composition of substrate that the infant is consuming. [47]

Prolonged intubation and repeated insertion of gavage tubes can result in aversion to oral feeding. [72] Deviations from a normal suck-and-swallow response may include a tonic bite reflex, an abnormal tongue thrust, or a hyperactive gag. More important than these findings, and too often observed, is the presence of a dysfunctional suck, swallow, and breathing pattern that results in hypoxemia, apnea, or both during oral feeding. This problem is often observed in ELBW infants.

Parental anxiety adds to the problem. In this setting, observation of infants by an occupational therapist or nurse specially trained to recognize feeding problems is an excellent method to make the diagnosis and treat these infants properly. Cineradiography of the suck-and-swallow mechanism often helps in this process. Diagnostic tests to exclude GER as a contributor to abnormal feeding behavior are also frequently necessary.

The NICU team is responsible for making these assessments and for developing a treatment plan before discharge. The PCP must be thoroughly aware of this assessment to understand the pathophysiology of the disorder and must comprehend the likelihood of success with the therapy applied.

Finally, stool passage and the composition of the stools may be useful in assessing the adequacy of nutrition. Abdominal distention and oily, mucoid, explosive, or watery stools should heighten the suspicion of epithelial absorptive problems in the intestine. Given the gene frequency in the white population, these signs and symptoms should always alert the PCP to the potential for cystic fibrosis. Most state governments in the United States have neonatal screening programs that now recognize the majority, but not all, of these afflicted children.

Carbohydrate and protein intolerance (an inability to digest food or a true allergy) must be considered in infants with abnormal patterns of stool passage, and diagnostic tests are indicated. Referral to a pediatric gastroenterologist may be appropriate if test results do not indicate a diagnosis or if interventions do not alleviate the problem.

Most infants gain weight and grow with an intake of 108 kcal/kg/day. Infants born prematurely usually require 110-130 kcal/kg/day for sustained weight gain and growth. Protein requirements may also be increased. For these preterm infants, the following simple equation may be used to calculate their increased needs:

Daily intake = 120 kcal/kg × (ideal weight for actual height/actual weight), where both weights are in kilograms.

In extraordinary circumstances, indirect calorimetry may be required to ascertain an infant's energy needs. These measurements include oxygen consumption, production of carbon dioxide, and elimination of urinary nitrogen. These techniques require the assistance of a tertiary care center.

Specific evaluations

Specific assessments of the NICU graduate include the following:

  • Fluid and acid-base balance
  • Mineral content of the blood and bone
  • Energy intake
  • Dietary nutrient composition

Fluid balance

Fluid balance is important in NICU graduates with serious pulmonary, cardiac, GI, and/or renal problems. Fluid restriction may result in poor growth unless the patient is fed high-caloric formula, fortified breast milk, or breast milk supplemented with formula intake. In the converse, excessive fluid may cause edema. The disease itself or diuretic therapy may affect the mineral content of the blood.

Acid-base balance

Assessment includes not only a determination of blood electrolyte concentrations but also a measurement of acid-base balance. Pulmonary or cardiac disease can be associated with clinically significant respiratory acidosis and renal compensation. Diuretic therapy may accentuate this problem. This is particularly true for low concentrations of potassium and chloride in the blood and a contraction alkalosis in association with severe chronic lung disease of prematurity (ie, bronchopulmonary dysplasia).

Blood and bone mineral content

Diuretic therapy may also cause further disturbances in calcium and phosphorus homeostasis (eg, low concentrations of total or ionized calcium), diminish plasma phosphorus content, and elevate alkaline phosphatase activity in the blood. These chemical findings in the blood frequently reflect osteopenia in preterm infants.

Osteopenia of prematurity is a defined disorder of diminished bone mineralization that is often observed in VLBW infants. A study in the United Kingdom revealed no consistent practices related to osteopenia in preterm infants. [73] The disease can result in rickets or fractures of the ribs and long bones.

Recommendations for the intake of calcium salts, phosphorus, and vitamin D that can diminish the risk of fracture have been established. [74] If fractures do occur after discharge, this cause must be distinguished from physical abuse.

Osteopenia of prematurity has a complex etiology that includes rapid bone growth with inadequate intake or metabolism of calcium, phosphorus, vitamin D, and protein. Correction involves treating the specific deficiencies. Renal disease with sodium wasting, excess bicarbonate losses, and poor retention of calcium and/or phosphorus can additionally complicate the clinical picture. On infrequent occasions, genetic disorders may manifest rickets.

The optimal method of monitoring bone mineralization in preterm infants with birth weights less than 1500 g is unsettled and may involve biochemical analyses, dual X-ray absorptiometry (DEXA), and/or ultrasonography. [74, 75] The PCP should be aware of the methods of assessment.

Dietary deficiencies

Other deficiency states observed in NICU graduates may involve the following:

  • Specific vitamins
  • Iron and trace minerals
  • Carnitine
  • Essential fatty acids
  • Protein

These deficiencies are not discussed in detail in this article; however, specific deficiencies are covered briefly below. Most deficiencies are avoided with the use of preterm formulas and/or fortified breast milk. Improved fortifiers, if available and if used before and after hospital discharge, enhance biochemical components associated with growth and affect postnatal growth itself. [76, 77, 78]

Vitamin K deficiency can result in a bleeding disorder from reduced synthesis of liver-related coagulation factors. [79] Folate deficiency can be associated with a megaloblastic anemia, dermatitis, and diarrhea.

Iron deficiency is the most important cause of anemia in NICU graduates. Therefore, infants with iron deficiency are often receiving iron and vitamin supplementation at the time of discharge. A recent study concluded that iron supplementation in breastfed infants is feasible and increases plasma ferritin without increasing hemoglobin. [80]

Although infrequent, zinc deficiency is associated with growth failure, defective host defenses, slow wound healing, and acrodermatitis enteropathica. Exclusive breastfeeding of ELBW infants is the scenario that most often results in zinc deficiency.

Carnitine deficiency can cause failure to thrive, cardiomyopathy, encephalopathy, and recurrent infections. This deficiency is most often recognized in infants who are receiving only parenteral nutrition. For this reason, in infants receiving long-term total parenteral nutrition, the solution should be supplemented with carnitine.

Protein-related malnutrition is among the most serious nutritional problems encountered in patients in the NICU before and after discharge. [81] Protein deficiency is associated with slow growth, hypoproteinemia, edema, lethargy, impaired wound healing, and an increased incidence of infection.

Measuring plasma prealbumin and albumin levels is useful in severe protein-deficiency states, but a strategy for early detection of the deficiency state is problematic. French pediatricians have long used a blood urea nitrogen (BUN) value of more than 5 mg/dL as an indication of adequate protein anabolism, but simple measurements of adequate protein intake and metabolic use are, for the most part, lacking.

Exclusive breastfeeding of ELBW and VLBW preterm infants creates a likelihood of protein-energy malnutrition, but the use of fortifiers and a study showing an improved neurodevelopmental outcome in ELBW infants who received human milk should allay concerns about this risk. [70] Early use of intravenous solutions containing amino acids has been shown to prevent the negative protein balance that can begin shortly after birth in the VLBW and ELBW population. [60]

A prospective, double-blind, randomized, controlled trial showed that a human milk fortifier enhanced the growth of preterm infants. [76] Use of human milk fortifier may be incorporated into the post-discharge care of preterm infants. Hay and associates wrote a comprehensive overview of the nutritional needs of ELBW infants. [82]

Delivery of preventive care

Health care maintenance is the essential function of the PCP. Assessment of growth and nutrition is an integral part of that function. Other functions include education regarding safety concerns; prevention of infectious diseases by means of immunization; and evaluations of vision, hearing, and other aspects of neurologic development. Problems with infant-parent bonding are also the PCP's concern. The neurologic and emotional aspects manifest in NICU graduates are reviewed in subsequent sections of this article.

Two important preventive measures may begin in the NICU. The first preventive therapy is immunization. To obtain current information about the immunization schedule for infants, see the AAP Red Book [20] and Immunization Schedules from the CDC. [21]

The second measure is education regarding car seat safety and the proper use of car seats. Because some preterm infants have episodes of cardiorespiratory instability when secured in a standard car seat, preterm infants should be tested before they leave the hospital for the absence of airway obstruction or apnea when they are in the seat. However, data are insufficient to confirm the value of this "car seat challenge test." [83]

There is sporadic use of the “car seat challenge test” before discharge of infants born at less than 37 weeks in the United States. Controversy abounds regarding this test in the United Kingdom; nevertheless, use of the car seat challenge test is increasing in that country. Studies have recommended standardizing the test to all infants born at less than 37 weeks' gestation to a duration of at least 90 minutes, along with a failure threshold for bradycardia of less than 80 bpm for greater than 10 seconds, and for saturation of less than 90% for more than 10 seconds. [84]

Car seats do prevent fatal vehicular injuries, so they are strongly recommended; in many states, their use may be controlled by law. Before infants are discharged from the NICU, their parents or guardians should be instructed about appropriate use of a car seat. The PCP should also review car seat safety during the initial office visit.

The PCP also has a responsibility to aid in home safely and to prevent crib death. In its Back-to-Sleep campaign, the AAP recommends supine sleeping for term infants; this positioning has significantly reduced rates of sudden infant death syndrome (SIDS). [85] The risk of SIDS is increased in preterm infants (birth weight < 2500 g) and SGA infants who sleep in a prone or lateral recumbent position versus the supine position. [86] Therefore, for preterm as well as term infants, "back is best." [87, 88]

However, parents and PCPs must be aware that "back is best" is associated with an increased risk of plagiocephaly and torticollis. Interventions to prevent (ie, tummy time with parental observation) or treat of these conditions when they occur are mandatory. [89]

If indicated, immunizations should be started prior to hospital discharge. [90] The PCP continues or starts immunizations during follow-up care. The practice of professionals who deliver in-hospital care of VLBW may lag behind current recommendations for immunizations. [91] One reason for this lag may be the concern that apnea may increase within 72 hours after immunizations [92, 93] or that febrile responses may occur. [94]

The cardiorespiratory status of infants with chronic lung disease may worsen for approximately 48 hours after immunization. [95] Whether ELBW infants can respond to immunizations within 2 months of birth (eg, 2 months after birth for an infant born at 23 weeks' gestation [ie, postconceptual age 31 wk]) is controversial. Some investigators believe that immunizations should be given at or after 35 weeks’ postconceptual age and that this timing enhances the immune response. PCPs must be aware of problems associated with the administration and the effectiveness of immunizations given to ELBW infants.

Preterm and other high-risk infants may meet guidelines for the administration of palivizumab. This monoclonal immunoglobulin G is given intramuscularly to reduce the severity of respiratory syncytial virus (RSV) infections. [96] In most communities, RSV season usually begins in late October or November and ends in March to May.

In 2009, the AAP published revised guidelines for prophylactic injections of palivizumab. [97] The use of palivizumab has been cost-effective in preterm infants born at less than 30 weeks' gestation [98] and in preterm infants with a post-conceptual age of 32-35 weeks. [99]

Health supervision and anticipatory guidance

Review of the patient's neurodevelopment is one of the PCP's primary tasks, and the provider must understand how the patient's physical and mental disabilities affect the parents and the family as a whole. The emotional well-being of the child and the family is critical to a successful life.

The PCP should coordinate care for patients leaving the NICU, [100] but in reality this seldom occurs. The PCP is also responsible for the follow-up care of any infant who has an abnormal result on a hearing screen or ophthalmologic examination before hospital discharge. Many states require that all infants undergo hearing screening before discharge.

A vital role of the PCP is assessment of anemia of prematurity. Periodic measurements of hemoglobin levels, hematocrit levels, and reticulocyte counts is an important job transferred to the PCP. The PCP also has the responsibility of keeping track of referrals to subspecialists for the numerous problems that an individual NICU graduate may have.

The PCP must ensure that health care insurance, whether funded privately or publicly, is available to the infant. The physician and the family must work together to obtain supplemental Social Security benefits based on the infant’s disabilities. In addition, the PCP must refer the infant to the proper community-based and education-based services appropriate to the patient's disabilities. Finally, the PCP must ensure that supplies and services are continuously available to technology-dependent infants.

Personal communication between the PCP and parents about their NICU graduate is essential to the physical and emotional well-being of the infant and family. Many questions are likely to arise before discharge, during the first office visit, and over the next months and years. Issues may include keeping the home warm, dressing the infant, allowing visitors, taking the infant outside, avoiding direct sun exposure, and flying to visit friends or family. Other issues may be more specific than these and related to infant's behavior or health. Sample questions include the following:

  • Why does my baby make grunting sounds ("preemie" noises)? Are these sounds abnormal?
  • Why does my baby's nose seem stuffier now than during my baby’s stay in the hospital?
  • My baby seems to sneeze and cough a lot. Is this a sign of illness?
  • When and how do I take my baby's temperature?
  • Is it bad that my baby has not had a bowel movement for 2 days?

A predischarge meeting of the NICU staff and the family can help ease the transition home. That said, the PCP must realize that questions such as those listed above are common and may seem numerous. Many answers simply involve common sense. To the authors' knowledge, no complete collection of the typical questions parents asked has been published. Therefore, few or no reference sources are available for PCPs. Practical experience is perhaps the only way to become comfortable in addressing many of these questions.

Many concerns may emanate from the parents' perception that their child is vulnerable to a number of physical and intellectual challenges. This view may be obvious to both the PCP and the parents at the time of discharge. The PCP must recognize patients at risk of becoming vulnerable children because the parents feel guilt or are overprotective. This topic is important enough that it is covered specifically in The Vulnerable Child (please see below).

Parents may wish to learn more about their baby's potential problems or disease than what time allows in the NICU or in the physician's office. The parents may seek information about community, state, or national resources to help their infant or child. Tertiary-sponsored follow-up clinics can also be resources, as can governmental or private agencies involved in specialized care or rehabilitation. Helping parents to gain access to information or resources is an important function of the PCP.

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Assessing/Managing Medical Disorders

Medical disorders in neonatal intensive care unit (NICU) graduates cover a wide range of disease states. The conditions covered in this article include disorders uniquely encountered in premature infants. [43, 44]

Anemia of prematurity

Anemia of prematurity is one of the most common and important problems that the primary care physician (PCP) must address in the NICU graduate. After birth, hemoglobin concentrations decrease more rapidly and more severely in premature infants than in term neonates, with the lowest hemoglobin levels observed in extremely low-birth-weight infants (ELBW) infants. [101]

The PCP absolutely must know the patient's hemoglobin value, hematocrit, and reticulocyte count at the time of discharge. The PCP should frequently (eg, weekly or biweekly) obtain hemoglobin levels, hematocrits, and reticulocyte counts after discharge until they suggest that the patient's anemia is resolving. In ELBW infants, hematocrits usually stabilize and begin to rise by age 3-6 months.

The PCP must be aware of the signs and symptoms of anemia and know how to manage it appropriately. In a hospital-based study, liberal use of blood may have improved neurologic outcome compared with restricted transfusion [102] and is associated with a decrease in apnea of prematurity. [97] For information on the treatment of severe anemia after hospital discharge, see the Medscape Reference article Anemia of Prematurity.

Apnea and bradycardia of prematurity

The National Institute of Child and Human Development (NICHD) has reviewed issues related to apnea of prematurity. [103] The severity of apnea and bradycardia in prematurely born infants is inversely proportional to their gestational age. Causes of apnea include immature central regulation of breathing; obstruction due to immature airway reflexes; and/or delayed coordination of sucking, swallowing, and breathing responses.

However, the primary care physician must consider other diagnoses when apnea and/or bradycardia are the presenting signs or symptoms after discharge. Such conditions include, but are not limited to, the following:

  • Profound anemia
  • Severe gastroesophageal reflux (GER)
  • Hypoxia or bronchospasm related to chronic lung disease (CLD)
  • Infection (especially respiratory syncytial virus [RSV] infection)
  • Malfunctioning or infection of a ventriculoperitoneal shunt
  • Seizures

Apnea can recur in preterm infants after they are hospitalized or after they receive general anesthesia for a surgical procedure (eg, inguinal hernia repair). Apnea and bradycardia of prematurity may occur at home, even in an infant who was free of apneic episodes for more than a week before discharge. [104] In this instance, the PCP may have to consider rehospitalization if an acute life-threatening event has occurred.

If no cause for the apnea is found and if the infant is not receiving a methylxanthine, use of caffeine may be considered in conjunction with cardiorespiratory monitoring. [105, 106] Some premature infants may be discharged with cardiorespiratory monitoring and one of these medications.

Home monitoring

Much discussion has focused on whether home monitoring helps prevent death in preterm infants. [87] Because of advertising in the lay media, parents may request or demand home monitoring. Clinicians must explain to such parents that home monitoring does not prevent sudden infant death syndrome (SIDS), although monitoring for apnea and bradycardia might avert the sequelae of hypoxia-related events. [87]

Late causes of apnea, as described above, should be excluded before an infant is discharged with home monitoring. For infants discharged with home monitoring and caffeine, the PCP may wish to have the infant outgrow medication during monitoring. Regardless of whether the caffeine is weaned in this manner or formally stopped, monitoring must be continued for at least 1-2 weeks before its cessation is considered.

One criterion for stopping home monitoring is a 4- to 8-week period with no clinical apnea, no cyanotic episodes, and no history of monitor alarms. Some PCPs may wish to download and review event recordings before stopping monitoring. A scheme for judging the cessation of home monitoring has been published. [107]

The AAP suggests that home monitoring for a preterm infant with apnea of prematurity may be stopped by 43 weeks' postmenstrual age. [87] Infants who were born at 22-24 weeks’ gestation may not be ready for discharge until this postmenstrual age. A study in Israel showed that 80% of infants with apnea of prematurity terminated monitoring at 40-44 weeks’ postconceptual age, and the authors recommended that home monitoring be discontinued at 45 weeks. [108]

Chronic lung disease of prematurity

Initially called bronchopulmonary dysplasia, chronic lung disease of prematurity and its manifestations have changed in recent years. [109] The incidence of chronic lung disease of prematurity, or the "new bronchopulmonary dysplasia," is greater than 60% for infants who weigh 500-600 g at birth, [109, 52] and its incidence approaches 100% for those who weigh less than 500 g at birth.

Investigators have reviewed the pathophysiology of the "new bronchopulmonary dysplasia," [110, 111] and comprehensive overviews have been published related to prevention and treatment. [112, 58, 113] Factors that define chronic lung disease include a typical radiographic appearance of cystic emphysema and fibrosis or subtle changes of diffuse interstitial edema and a requirement for inspired oxygen at the 28th day of life or the 36th week of post-conceptual age. The preferred definition may be an oxygen requirement at 36 weeks’ post-conceptual age.

The infant presenting in the primary care physician’s office while receiving home oxygen therapy and multiple medications is at high risk for developing cardiopulmonary complications after discharge. To prevent cor pulmonale, intermittent or persistent hypoxemia and clinically significant hypercarbia must be prevented after discharge. A pediatric pulmonologist, in addition to the PCP, should monitor infants discharged home with considerable need for oxygen and substantial renal correction of respiratory acidosis.

Home oxygen therapy is a safe and cost-effective treatment and may reduce complications (eg, cor pulmonale) in infants with chronic lung disease. [114, 115] Oxygen therapy also appears to facilitate growth in infants with bronchopulmonary dysplasia. [116] In the PCP’s office, oxygen saturation rates must be monitored with pulse oximetry. The level of inspired oxygen required to prevent hypoxemia indicates the severity of disease and whether the patient's condition is improving.

Adjunctive therapy for bronchopulmonary dysplasia may include inhaled bronchodilators and/or steroids, oral corticosteroids, and diuretics. [117, 118] Management of chronic lung disease may involve oxygen, medications, complex technologies, and superb nutrition. [119, 120, 121]

Issues have arisen regarding whether corticosteroids inhibit normal growth of the lung and brain and whether it causes future pulmonary or neurodevelopmental disabilities. Grier and Halliday propose an appropriate use of corticosteroids in severe bronchopulmonary dysplasia that may be lifesaving and that may also minimize adverse responses in the developing brain. [122]

Infants receiving diuretics to treat chronic lung disease require periodic evaluation of their electrolyte status. Bumetanide may be associated with fewer electrolyte abnormalities, especially if dosing occurs on alternate days. [117] Furosemide therapy for chronic lung disease predisposes these infants to nephrocalcinosis. The PCP may need to screen patients for nephrocalcinosis by performing serial renal ultrasonography, by assessing calcium-to-creatinine ratios in the urine, or by examining the urine for erythrocytes (microscopic hematuria).

No current therapy effectively resolves nephrocalcinosis in preterm infants. However, nephrocalcinosis spontaneously resolves in many, but not all, infants in the first year of life.

Prevention may be the key. Furosemide should be used only when this diuretic is proven effective. When nephrocalcinosis is present, every attempt should be made to stop furosemide or other drugs that cause excessive urinary excretion of calcium. Other diuretics such as spironolactone (Aldactone) and chlorothiazide (Diuril) may be used as they are effective in controlling pulmonary edema secondary to bronchopulmonary dysplasia and they conserve or prevent loss of calcium in the urine.

Infants who have chronic lung disease may require more than 120-150 kcal/kg/day for weight gain because of their increased work of breathing. [120] Therefore, either breast milk with added fortifier or a formula with high energy density may be needed for nutritional support. Infants with chronic lung disease may also need restrictions in fluid intake, a therapeutic strategy that may further impair their growth.

On occasion, infants with chronic lung disease may be discharged with home monitoring. For infants who have the most severe illness, this may include pulse oximetry. These infants are candidates for immunoglobulin therapy to prevent or ameliorate respiratory syncytial virus infection. Other pulmonary infections should also be managed with attentiveness and concern.

Gastrointestinal diseases

The 2 prominent intestinal problems encountered in premature infants discharged from the NICU are GER and complications arising from necrotizing enterocolitis (NEC). Reviews of GER [123, 124] and NEC have been published. [125, 126]

Gastrointestinal reflux

Gastrointestinal reflux (GER) can be suspected in premature and term infants who have any of the following manifestations:

  • Repeated regurgitation or emesis after feedings
  • Apnea after feedings
  • Fussiness or painful crying during or after feedings
  • Arching of the head and neck during or after feedings
  • A nasogastric or orogastric feeding tube
  • Rumination

Each of these signs or symptoms takes on additional importance if the child's growth is poor.

GER has been associated with esophageal or duodenal atresia, diaphragmatic hernia, brain injury due to hypoxic-ischemic encephalopathy, prematurity whether chronic lung disease is present or absent, and many other neonatal conditions. Data have suggested that GER has no role in apnea of prematurity, [127] whereas an endoscopic study indicated that GER may be responsible for laryngeal edema, apnea, and bradycardia. [128]

Testing to confirm GER has been the subject of contentious debate. [61] Diagnostic tests for GER include contrast-enhanced studies of the esophagus and upper GI tract, radiolabeled scanning after feedings, monitoring of esophageal pH (optimally done with dual high and low probes), and esophagoscopy with or without biopsy. Before a reliable pH probe test can be performed, medications that raise gastric pH must be withdrawn for several days (eg, 2-4 days).

In the ideal situation, the patient's gastric pH should be known before a pH probe examination is performed. Some pediatric gastroenterologists recommend adding hydrochloric acid to the feedings during pH probe testing to avoid false-negative results due to high gastric pH.

The success rate of medical therapy for GER is less than ideal. Treatments that have been tried for GER include the following:

  • Prokinetic agents (eg, metoclopramide, erythromycin)
  • H2-receptor blockers or proton-pump inhibitors
  • Thickened feedings
  • Positioning to facilitate gastric emptying

Prokinetic agents have proved problematic in the treatment of GER. A study indicated that GER was worst when preterm infants were treated with metoclopramide; in addition, long-term use of this drug has been associated with tardive dyskinesia.

Erythromycin 1.5-2.5 mg/kg given every 6 hours may effectively treat GER in some preterm infants. [129, 130] However, erythromycin therapy may increase the risk of hypertrophic pyloric stenosis, although this complication may occur more frequently when erythromycin is given intravenously at therapeutic doses than with the low oral doses used to treat GER. Nevertheless, if erythromycin is used to treat GER, the PCP should watch for symptoms associated with hypertrophic pyloric stenosis.

Thickened feedings and positioning have had mixed success in treating GER in neonatal clinical trials. Thickened feedings effectively decrease GER [131, 132] ; therefore, a resurgence in their use has occurred. Although GER may be a lifelong problem in some infants (eg, term infants with profound brain injury due to hypoxic-ischemic encephalopathy), premature infants generally have self-limited disease that improves as the gastroesophageal sphincter and gastroduodenal motility matures.

Infants with severe GER associated with hypoxic-ischemic encephalopathy may be candidates for treatment with gastric fundoplication. A pediatric gastroenterologist should collaborate with the PCP in caring for infants with severe GER.

Necrotizing enterocolitis

For graduates of the NICU who have had NEC, the primary care physician must be alert for later problems. Complications of NEC include the following:

  • Need for ostomy care
  • Malabsorption
  • Intestinal dysmotility
  • Need for parenteral nutrition despite enteral feedings
  • Infections of the ascending biliary tract
  • Biliary calculi
  • Late partial or complete bowel obstruction
  • Short bowel syndrome

The prognosis of patients with short bowel syndrome is guarded, [133] and the PCP must follow up with these infants, as should a pediatric gastroenterologist and surgeon. Some of these complications may be present at discharge from the NICU, and some require continued surveillance. Poor growth is a frequent outcome.

Dumping syndrome is observed in infants with ostomies or severe diarrhea during GI infections (eg, those due to rotavirus). Dumping syndrome may cause rapid dehydration and electrolyte imbalance. Strong evidence suggests that NEC is associated with increased occurrence of adverse neurologic outcomes. [134, 135]

The PCP must be diligent in recognizing growth failure, fluid imbalance, and electrolyte abnormalities in infants with GI disease. Scarring after neonatal GI surgery can cause partial or complete bowel obstruction after discharge. Repeated emesis, particularly if it is bilious, and/or a sudden onset of abdominal distention must always be investigated. Polymicrobial sepsis may be another indicator of partial or complete bowel obstruction after NEC or GI malformations.

When short bowel syndrome necessitates parenteral nutrition at home, catheter or gut-related bacteremia is a major risk factor. The possibility of bacteremia must be suspected even if the patient has only subtle signs of infection (eg, irritability, low-grade fever, apnea).

A pediatric gastroenterologist should monitor infants who have clinically significant complications secondary to NEC and other GI diseases. In particular, short bowel syndrome occurs in extremely low-birth-weight (ELBW) infants as a complication of NEC and has a high mortality rate. [136] These infants have complex conditions and require management at a major pediatric gastroenterology or transplant center.

Central nervous system disorders

The most common and serious CNS disorders that may be present in premature infants at the time of discharge are posthemorrhagic hydrocephalus, postmeningitic hydrocephalus, periventricular leukomalacia (PVL), and seizures. These disorders place the NICU graduate at high risk for poor long-term neurologic outcomes. Infants with these problems should be followed up in the NICU follow-up clinic.

The discussion below is limited to CNS complications that most commonly affect infants born prematurely. Other common conditions affecting the CNS of NICU graduates include developmental defects in the brain and/or spinal cord.

Posthemorrhagic complications

Despite past beliefs, ELBW preterm infants with grade I subependymal or II intraventricular hemorrhage may have poor neurodevelopmental outcomes. [137, 138] Grade III intraventricular or grade IV intracortical hemorrhage is associated with the least favorable neurodevelopmental results, but the degree of prematurity and the presence of chorioamnionitis may also be major contributors to severe long-term disabilities.

Intraventricular hemorrhage may lead to posthemorrhagic hydrocephalus. In turn, intracortical hemorrhage causes cerebral infarction and may culminate in cerebral or cerebellar porencephaly. Porencephaly and posthemorrhagic hydrocephalus are among the most devastating CNS events in premature infants. [139]

The risk of these conditions is inversely proportional to gestational age. For example, 35% of infants with birth weights of 500-600 g have severe intracranial hemorrhage. [52] If posthemorrhagic ventricular dilatation occurs after intraventricular hemorrhage, it is usually apparent on cranial ultrasonography within 2-3 weeks.

Hemorrhagic cerebral events are occasionally observed in term neonates. Such hemorrhage in term infants carries an ominous prognosis. Infants who have had intracranial hemorrhage must always be monitored in neurodevelopmental follow-up clinics. They should also be referred to community services because rehabilitation is frequently necessary.

Although ventricular dilatation may be reversible, infants with severe intraventricular hemorrhage with posthemorrhagic ventricular enlargement are at high risk for neurodevelopmental handicap. [140] The PCP must be informed of this clinical scenario.

Rapidly progressive posthemorrhagic hydrocephalus may require permanent placement of a cerebrospinal fluid shunt. [141] The long-term neurodevelopment of ELBW infants who require shunt insertion is very unfavorable compared with ELBW infants with intraventricular hemorrhage who do not have ventricular enlargement. [142]

If a ventriculoperitoneal shunt is needed, the PCP must monitor the infant for shunt infections or malfunctions. Malfunctions are typically due to an occlusion of the proximal or distal cannula, with a subsequent increase in intracranial pressure. Poor feeding, vomiting, irritability, lethargy, sleepiness, apnea, and seizures may be signs and symptoms of shunt blockage. If fever or a septic appearance is present, the PCP should suspect shunt infection and meningitis. The PCP should monitor the patient's head circumference for rapid or slow growth.

Periventricular leukomalacia

Periventricular leukomalacia (PVL) is caused by an ischemic infarction of the white matter, most commonly adjacent to the lateral ventricles. PVL can be observed in either preterm or term infants. The pathogenesis of cerebral white matter injury in preterm infants has been reviewed. [143] Antenatal or intrapartum hemorrhage and severe placental disease (eg, chorioamnionitis) have been associated with PVL. [144] Postnatal sepsis and NEC are associated with white matter abnormalities on MRI at term gestation and with adverse neurodevelopmental outcomes. [145]

Other postnatal events leading to PVL include CSF infections or intraventricular hemorrhage, life-threatening apnea and bradycardia, and cardiorespiratory arrest. The quality of general movements of preterm infants at age 3 months can often identify white matter pathology. [146] The condition is otherwise identified on cranial sonograms as echogenic areas in the periventricular white matter. Injuries in these areas evolve into cysts. [147]

PVL is highly associated with subsequent neurodevelopmental disabilities, particularly cerebral palsy (motor dysfunction of infancy or spastic paresis). Persistence of cysts is known to increase the risk of severe neuromotor abnormalities. PVL is always a reason to schedule appointments in a neurodevelopmental follow-up clinic. Affected infants should also be referred to community services that provide early intervention and rehabilitation.

Cranial imaging

Preterm infants whose cranial sonograms show reduced growth of the corpus callosum during the patient's NICU stay are at increased risk for psychomotor delays and cerebral palsy. [148] Moreover, abnormal findings on MRIs obtained at term in very preterm infants at term corrected age are predictive of adverse neurologic outcomes and are increasingly becoming standard of care prior to discharge from the NICU. [149, 150]

Some investigators believe that identification of neuroanatomic abnormalities on MRI scans can predict the need for early interventions. [151] The PCP should be advised about any imaging findings that suggest the possibility of an unfavorable neurodevelopmental outcome before the patient is discharged home, and the parents should be fully counseled about the findings. Whether this knowledge, when conferred to parents, enhances or diminishes parental involvement in their infant's rehabilitation is controversial.

Seizures

A history of neonatal seizures is associated with long-term psychomotor or neuromotor handicaps. [152, 153, 154] Reasons for neonatal seizures include hypoxic-ischemic injury, direct cerebral trauma, intracranial hemorrhage, metabolic abnormalities, malformations, and infections. Neurodevelopmental outcomes after neonatal seizures are clearly related to the etiology of the seizures. However, in some patients, a specific cause is never determined.

When known, the cause may be predictive of the ease or difficulty with which the seizures can be controlled with anticonvulsants. Phenobarbital has been the mainstay of anticonvulsant therapy for neonatal seizures [155, 156, 157] ; however, recently levetiracetam has been used safely. [158, 159]

The duration of treatment for neonatal seizures is controversial, partly because of concerns that anticonvulsants may hinder brain development. Among infants without signs and symptoms, and in the absence of electroencephalographically recorded seizures, pediatric neurologists may recommend discontinuing anticonvulsants before the patient's discharge or shortly thereafter. Before anticonvulsants are withdrawn, electroencephalography must be performed to exclude subclinical seizures.

A neurologist should evaluate any NICU graduate who has persistent or difficult-to-control seizures. These infants should also be monitored in neurodevelopmental follow-up clinics and referred to appropriate community services.

A common neurologic condition associated with neonatal seizures is hypoxic-ischemic encephalopathy. Maternal intrapartum conditions that commonly result in profound hypoxic-ischemic encephalopathy include placental abruption, uterine rupture, and prolonged cord compression. Hypoxic-ischemic encephalopathy is a devastating event that most often affects term neonates. Survivors of hypoxic-ischemic encephalopathy have long-term sequelae more commonly than survivors of extreme prematurity. All neonates with Sarnat stages 2 (moderate) and 3 (severe) hypoxic-ischemic encephalopathy should be enrolled in follow-up programs. [160] The PCP should be aware of whether the hypoxic-ischemic encephalopathy was treated with head or whole body cooling. [161]

If infants have profound damage (often in association with a burst-suppression pattern on initial electroencephalography and an abnormal MRI finding), extensive home care and community services may be required. The PCP must monitor the patient for recurrence of seizures many months after anticonvulsants are discontinued. The PCP has the responsibility for coordinating the complex care required by infants with hypoxic-ischemic encephalopathy.

Eye problems

Retinopathy of prematurity (ROP) is an important problem that the PCP must monitor. One of the most devastating complications of preterm birth is blindness secondary to ROP. This condition is complex in its pathophysiology. Certain clinical conditions, such as infections, increase the risk of ROP. Its association with oxygen therapy is long known, but this causal relationship is complex. One firmly established etiologic factor is the degree of prematurity. Therefore, the infants at highest risk are those who were born prematurely with an ELBW.

The AAP published guidelines for ROP screening. [23] However, some neonatologists take a more conservative view than the AAP and perform screening in large or relatively mature preterm infants, especially if they received prolonged oxygen therapy and/or assisted ventilation. A subspecialist trained to recognize neonatal retinal diseases must perform the screening for ROP. The subspecialist must continue to examine the patient and make recommendations for follow-up examination of the neonatal retina until it is fully vascularized.

Complete vascularization usually occurs by 44-48 weeks after conception but occasionally takes longer. Screening examinations are important because if retinal detachment (ie, threshold disease) is a risk, the infant may be referred for laser therapy of the retina to prevent it. Studies from the United Kingdom have proposed more aggressive criteria for intervention when preterm infants have ROP.

Retinal detachment can also occur relatively late in life. Again, this is particularly common in premature ELBW infants. Preterm infants who have had evidence of ROP must also be screened for refractive disorders and for amblyopia at 6 months after discharge, at age 2-3 years, before they begin school, during grade school, and during adolescence when rapid growth of the ocular globe is occurring.

Infants with a history of ROP are at increased risk for myopia and amblyopia. In later life, glaucoma may also be more common in preterm infants than in their term counterparts. When a PCP examines premature infants younger than 1 year, the PCP must assess the baby for strabismus.

Hearing problems

The incidence of hearing loss is higher in NICU graduates than in the general population of well neonates. Many factors can contribute to such hearing loss, including hypoxia, certain drugs used alone or in combination, and infections.

Silent or symptomatic congenital or postnatally acquired cytomegaloviral (CMV) infection is highly associated with hearing loss in later life. The hearing loss caused by CMV infection may progress over time. Most NICUs in the United States use only CMV-seronegative blood for RBC transfusions. This practice has lowered the incidence of acquired postnatal CMV infection among hospitalized neonates, but infants might still become infected with CMV from platelet or plasma-derived blood products.

All NICU graduates must undergo a hearing evaluation before leaving the hospital. A number of devices are now available for this purpose. The PCP should be informed at discharge whether the patient passed the hearing screen. If the infant did not, he or she must be referred to an audiologist who practices with a pediatric otolaryngology specialist.

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Developmental Follow-up

Although advances in neonatal intensive care have improved survival rates of high-risk neonates, this improvement has not been accompanied by a proportional decrease inin certain morbidities. As a result, reduction in mortality does not necessarily equal a reduction in disability rates. Cognitive deficits without major motor deficits are now the dominant neurodevelopmental sequelae.

Methodologic problems in follow-up studies have produced conflicting data about the sequelae of premature birth. [162] Areas of potential problems include sample selection procedures, cohort studied, exclusion criteria, control groups, age at assessment, social background/socio-economic status, definition of deficits, and use of birth weight or gestational age. There currently is a shift toward emphasis on both gestational age and birth weight, with the former providing a better window into the infant’s maturational status. As the total number of survivors at potential risk for neurodevelopmental morbidity increases, many clinical research questions with major ramifications on medical care have evolved. These questions can be answered only by performing long-term follow-up studies because many of the neurocognitive deficits are not identified early on. [163]

Although neurodevelopmental outcomes are increasingly used to determine the efficacy of medical interventions in infants born preterm, long-term follow-up studies are infrequently performed because of cost and subject dropout. The bias of neurodevelopmental data is that they are not as precise as radiographic or biologic measurements.

In addition, confusion occurs because of the following factors [164, 165] :

  • The frequent disconnections between adverse perinatal experiences and later outcomes
  • The moderating and mediating effects of socioeconomic status and other environmental influences
  • The long time lag necessary to complete longitudinal assessments

Besides initial biologic risks, perinatal interventions designed to address these risks may substantially affect later development. Therefore, extended follow-up is critical to identify possible negative effects that a medical intervention or the standard of care might have on the child’s brain that might not be obvious in the first years of life. This point was clearly demonstrated in the use of postnatal steroids to treat chronic lung disease and in late visual impairment and developmental delays with the use of oxygen. [166, 167]

Of interest, standardized guidelines about follow-up services for high-risk infants in tertiary care centers are lacking despite the requirement for approved neonatal fellowship training programs to include experience in follow-up clinics and the increasing number of centers involved in networks. [168]

A National Institute of Child and Human Development (NICHD)–National Institute of Neurological Disorders and Stroke (NINDS) workshop on follow-up care of high-risk infants identified surveillance and research as the 2 primary areas of responsibility for neonatal follow-up programs. [168] Surveillance involves monitoring medical care during hospitalization and serial evaluation of health and neurodevelopmental outcomes after discharge. This process is necessary for the following reasons:

  • Auditing of neonatal intensive care unit (NICU) interventions
  • Monitoring of important quality indicators for the individual NICU
  • Summarizing center-specific outcomes for selected conditions (eg, intraventricular hemorrhage, retinopathy of prematurity [ROP])
  • Summarizing annual outcome data to be combined and used in policy decisions

Provision of feedback to the family and the primary care physician (PCP) at each serial evaluation and appropriate referrals are in line with the medical home concept. Follow-up research data are necessary to evaluate the long-term effect of medical interventions and to identify previously unidentified adverse risks. Areas of concern include social issues; neurologic, cognitive, behavioral, and physical issues; health-related quality-of-life; and functional outcomes.

Outcome studies have primarily emphasized the incidence of major disabilities such as moderate-to-severe intellectual disabilities, sensorineural deficits (eg, hearing loss, blindness), cerebral palsy, and epilepsy. Babies born with LBW (< 2500 g) have a 6-8% incidence of developing these major disabilities; those born at VLBW (< 1500 g) have a 14-17% incidence; and those at ELBW (< 1000 g) have a 20-34% rate. Therefore, as birth weights decline, disabilities increase. [169, 170] The same inverse relationship is found with gestational age and the prevalence of disabilities. In comparison, major disabilities occur in 5% of full-term infants. These rates have remained relatively constant over the last decade.

The nature of impairment is changing, with notable problems found in survivors without the major disabilities just described. [171, 164] Recognition of this change may be related to lengthened follow-up, refined assessment techniques, and improved survival rates. [164]

High-prevalence, low-severity dysfunctions [171, 172, 164] appear to be increasing, particularly in premature infants of lower birth weight and gestational ages. [173, 174, 175] These abnormalities include the following:

  • Learning disabilities
  • Borderline to low-average intelligence quotients (IQs)
  • Specific neuropsychological deficits (eg, visual motor integration, executive dysfunction)
  • Behavior problems(internalizing problems, social difficulties)

High-prevalence, low-severity dysfunctions may occur in as many as 50-70% of VLBW/VPT infants, with an inverse relationship to birth weight/gestational age. VLBW/VPT and ELBW/EPT infants have the highest risk. Twenty-five to 40% of VPT/VLBW children require special education services, 20% or more need a self-contained learning disabilities placement, and 16-20% repeat a grade in school. [176] As much as 60-70% of ELBW/EPT children require special assistance in school. Conversely, 17% of late-preterm (34-36 weeks’ gestational age) require such services, this in comparison to a 2.3-8% rate in children born full-term. Moreover, these dysfunctions do not occur in isolation. Affected infants often have several concomitant problems that synergistically produce academic difficulties.

The situation is complex because the parents' social, ethnic, and educational backgrounds may also influence the prevalence of these disabilities. In addition, though major disabilities are often identified during infancy, high-prevalence/low-severity dysfunctions become more obvious as the child reaches school age. Further compounding the issue is that no good predictors of these subtle problems can be identified during infancy or preschool age.

It is extremely difficult to determine in early infancy whether problems are transient and result from continuing recovery or catch-up from the negative effects of preterm birth or whether they reflect the emergence of a permanent handicap. Many functional outcomes cannot be adequately gauged until the child encounters broad, complex demands and situations that require developmentally complex functions. Preexisting deficits in these functions become apparent only when they are challenged directly. [164]

This situation again substantiates the necessity of longitudinal follow-up. Follow-up protocols vary in terms of patients who should be followed, levels and frequencies of follow-up, and testing and outcomes of interest.

Patients who require follow-up

As mentioned previously, clinicians should consider birth weight and gestational age when determining who should be monitored. Neonates are categorized into 1 of the 3 following gestational groups:

  • Extremely premature: less than 28 weeks' gestation (EPT)
  • Very premature: 28-32 weeks' gestation (VPT)
  • Premature: 33-36 weeks' gestation (late preterm is 34-36 weeks)

Categorization on the basis of birth weight alone may result in the inclusion of infants who are relatively mature but whose growth was restricted because of an adverse intrauterine environment. In addition, use of birth weight alone may bias evaluations or biomedical findings because conditions such as periventricular leukomalacia (PVL) are relatively infrequent in growth-restricted fetuses. ELBW infants need follow-up assessments because they are subject to “2-hit” CNS involvement. The CNS of the at-risk infant is potentially subject to developmental disruption, insult, or both. Premature birth changes the spatial and temporal progression of brain structures (eg, migration, organization and differentiation, myelination) and alters brain architecture and connectivity. Also compounding the disrupted brain development are altered postnatal visual, auditory, tactile, and vestibular-proprioceptive experiences.

The CNS of high-risk fetuses and neonates are also frequently subject to insults, such as maternal infections, exposure to proinflammatory cytokines, hypoxic-ischemic encephalopathy, intraventricular hemorrhage, or PVL. Extreme prematurity (weight < 1000 g or age 28 wk or younger), regardless of other factors, increases the risk of CNS insults, including grade III or IV intraventricular hemorrhage, PVL, and seizures. Many infants have both developmental disruption and insult, with the brain areas having the greatest rapidity and complexity of developmental events being most vulnerable to negative exogenous and endogenous influences. This is analogous to so-called encephalopathy of prematurity.

Risk criteria differ depending on whether the infant was born preterm or at full term. Moreover, medical conditions can exacerbate the risk; examples of such conditions are the following:

  • Extracranial or intracranial trauma
  • Respiratory disorders
  • Infections
  • Hyperbilirubinemia that requires exchange transfusion
  • Encephalopathy
  • Fetal growth restriction

Likewise, interventions to deal with specific disease states (eg, resuscitation, prolonged ventilation, postnatal steroids, total parenteral nutrition) can also raise the risk. Also contributory are social and/or environmental risks; these often occur in conjunction with biologic risks and place infants at double jeopardy. [177]

levels of follow-up

As indicated in the National Institute of Child and Human Development–National Institute of Neurological Disorders and Stroke (NINDS) workshop, [168] levels of follow-up can differ. The workshop monograph identified 4 levels of follow-up intensity.

level I follow-up could consist of a telephone interview from a designated NICU staff member or use of a screening instrument such as the Cognitive Adaptive Test and Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS)) [178] or the Ages and Stages Questionnaire-3. [179] Subsequent referrals could be made as needed.

level II follow-up could involve a clinic visit with the use of one of the aforementioned screening instruments or a hands-on test, such as the Bayley Infant Neurodevelopmental Screener (BINS). [180] Additional allied professionals, such as a dietitian and physical, occupational, and speech therapists, may or may not also evaluate the child.

level III can entail comprehensive assessment at a single visit. level IV involves serial assessments by a multidisciplinary team.

Frequency of follow-up

The frequency of follow-up contact is superimposed on the level. Patients at high risk or those in whom neurodevelopmental problems have already been identified should be evaluated soon after discharge from the NICU and frequently thereafter. In general, serial contact is recommended whether follow-up is for surveillance (eg, quality assurance) or for research purposes, and regardless of the level of follow-up.

The frequency of follow-up involves 2 issues: the optimal ages for assessment and the intensity or level of follow-up, which also depends on constraints such as cost, personnel, and/or unique characteristics of the center. Some have found that beginning follow-up visits shortly after discharge enhances the likelihood the family will continue with subsequent visits.

Evaluation at corrected age 6 months

Evaluation at corrected age 6 months (chronologic age minus weeks born prematurely) offers a window during which indicators of severe handicaps can be identified. An evaluation at this time also provides early contact with the family, enhances continuity of contact, and helps to ensure that children are receiving early intervention services.

By this age, the influence of many medical and/or biologic issues is decreasing. However, recovery from medical procedures, feeding difficulties, and subsequent hospitalizations may still affect neurodevelopmental assessments. Tone, neurosensory (eg, auditory, visual) functions, gross and fine motor coordination, early verbal skills, interactive capacities, and some cognitive processes can be evaluated.

Use of assessment before this age is questionable. Screening with tests such as the BINS may be sufficient, but this approach again underscores the interplay between the frequency and level of follow-up.

Evaluation at corrected age 12 months

Environmental factors do not exert a major influence at this age, and biomedical issues, such as oxygen supplementation for chronic lung disease, tend to improve and have a lesser effect on testing. By 12 months’ corrected age, a varied behavioral repertoire is emerging, and cognitive processes and developing language skills can be assessed.

However, at this age, cognitive and motor functions are still highly intertwined, and this period of developmental acquisition is a time of variability as well. Some neurologic abnormalities identified in the first year of life are now transient or improving (eg, transient dystonia of prematurity). Conversely, findings in some infants worsen over time.

Evaluation at corrected age 18-24 months

By 18-24 months, environmental factors exert increasing influences on evaluation results. Cognitive and motor abilities diverge, language and reasoning skills develop, and the ability to predict early school performance improves. However, many intelligence tests have weak floors at this age, restricting assessment to only developmental tests. As a result, more subtle impairments may be underestimated. Test refusals may invalidate results or produce false-positive results.

Judging performance at 2 years on the basis of corrected age (chronologic age minus weeks born prematurely) rather than actual age is controversial but generally accepted. Standard follow-up protocols in many multicenter networks specify how the age at evaluation is calculated.

Evaluation at age 3-4 years

Intelligence can first be assessed at 3-4 years. Intelligence includes concept development, pre-academic readiness skills, early indicators of executive function and attention, and abilities in visual-motor integration. Verbal and nonverbal skills can be differentiated. Moreover, the predictability of later intelligent quotient (IQ) on the basis of scores at this age is acceptable. Environment and social support, as well as other factors, broadly influence test results most strongly from this age onward.

Evaluations at age 6 years and age 8 years

By age 6 years, additional tests and procedures can be used to access attention problems, academic skills (at approximately the first-grade level), socialization, and neuropsychological functions. The selection of possible tests that can be used is more limited at age 5 years than at age 6 years.

By age 8 years (approximately the third grade), intelligence, neuropsychological functions, learning disabilities, school performance, and social and behavioral adjustment can be adequately assessed. The predictive validity of IQ scores is highest now compared with earlier ages.

Summary of the frequency of evaluation

Evaluation after age 3-4 years is often not feasible in many follow-up programs whose main focus is surveillance. This is when the PCP should monitor the child's school performance and periodically review developmental, cognitive, academic, and behavioral concerns.

A hybrid, parallel model is used at the authors' institution. In the Developmental Continuity Clinic, infants at greatest biologic risk (eg, birth weight 1250 g or less or 32 weeks' gestation or younger) or those having significant medical complications (eg, intraventricular hemorrhage, asphyxia, prolonged mechanical ventilation, necrotizing enterocolitis, retinopathy of prematurity) are examined at age 6, 12, 24, and 36 months (with the first 3 ages corrected for prematurity).

At the first 3 appointments, the BINS is applied, and occupational-physical therapy and speech-language evaluations are made. At age 36 months, detailed developmental or cognitive evaluation substitutes for the BINS (ie, Bayley Scales of Infant and Toddler Development-III). The developmental continuity clinic serves both surveillance and research functions.

Children treated in the author's NICU who do not meet the predetermined level of risk are seen by nurses in the regular, serial follow-up clinic and screened using the BINS. This clinic has a lowered level of intensity. However, if concerns are identified during screening, the children are referred for early intervention services and for inclusion in the intensive, parallel Developmental Continuity Clinic for subsequent, ongoing assessment.

In both clinics, the PCP receives written communication about the findings and the appropriate strategies for intervention. Individual program staff must decide on the possible combinations of levels and frequency of follow-up, which depend on the needs and the constraints of a given NICU.

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Testing and Outcomes of NICU Graduates

In addition to obvious moderate-to-severe disabilities, other suboptimal outcomes can occur in high-risk infants. These include the following:

  • Motor and neurologic deficits (including developmental coordination disorder)
  • Reduced intelligence
  • Learning disorders
  • Language difficulties
  • Visuomotor problems
  • Impaired executive function
  • Attention-deficit/hyperactivity disorder (ADHD)

Motor and neurologic deficits

Motor and neurologic outcomes are major concerns when they arise as a high-risk infant matures. Different risk factors may affect these outcomes. [163, 181] The link between perinatal problems and deficits in these areas also appears to be relatively direct. The incidence of transient dystonia in preterm infants peaks at 7 months’ corrected age, with a prevalence of 21-36%. [182, 183]

Noteworthy is the fact that children with dystonia have an increased risk of later cognitive and motor problems. Often recognized are neurologic soft signs (eg, motor, sensory, or integrative functions but not localized brain dysfunctions). These deficits increase the risk of subnormal IQ or learning disabilities (particularly reading) in children with normal IQs.

Upper extremity motor tasks involving visually guided ballistic arm movements (eg, bouncing, catching, throwing) are particularly affected. [184] The proposed underlying mechanism is that parallel mental dysfunction involves circuits in the neighborhood of motor tracts that also sustain damage, but these abnormalities are not apparent until later in life. Motor injury is more self-correcting, whereas cognitive abnormalities are not.

Reduced intelligence

In a meta-analysis conducted more than 20 years ago, LBW infants had a mean IQ score that was 5-7 points below that of control children. [185] More recent comparisons that excluded children with severe disabilities revealed a comparable 0.3-0.6 standard deviation (SD) decrease in IQ scores of premature infants. This change translated to a 3.8- to 9.8-point decrement in IQ scores, although 12- to 17-point deficits have also been reported. [164, 171]

In children with birth weights of less than 1500 g and without major disabilities, mean group IQ scores were borderline to average, with most of the data suggesting that a low-average score was the mode. [164, 171, 186] These children's scores are generally 8-11 points lower than those of their full-term counterparts or even siblings. A recent meta-analysis of 16 studies indicated that VPT/VLBW infants had IQ scores that were 10.9 points below their full-term peers (0.66 SD). There is a gestational age gradient: the smaller or younger the infant, the lower the mean group IQ. Based on meta-analyses, the EPICURE study and the Bavarian Longitudinal Follow-up study, for each week of gestational age younger than 33 weeks, there is an average mean decline of 1.7-2.5 IQ points. Correlations between gestational age and cognitive scores, as well as between birth weight and cognitive scores, generally are r=.50 (accounting for 25% of the variance).

Such changes in intelligence place affected children at a distinct disadvantage when they have to compete with peers whose IQ scores are average or above. Moreover, the lowered IQ scores typically do not occur in isolation.

Learning disorders

More than half of all former VLBW/VPT infants and 60-70% of ELBW/EPT infants require special assistance in school. By middle school, ELBW/EPT infants are 3-5 times more likely than those born at term to have a learning problem in reading, spelling, writing, or mathematics, with mathematics and written language being most disrupted. [187, 176] Overall, it appears that in descending order, disabilities are found in math, written expression, spelling, and reading.

Approximately 16-20% of children born VLWB/VPT repeat a grade, and 20% are taught in self-contained classrooms for students with learning disabilities. Almost one third are in mainstream classrooms, but these children are functioning more than 1 grade below their placement. [176] Once again, there is a gestational age gradient: 25-40% at younger than 32 weeks are retained, 20-30% at 32-33 weeks, and 17% at 34-36 weeks (vs 2.3-8% ≥37 wk).

Many children born prematurely later develop nonverbal learning disabilities (NVLD). [188] Environment, sex, and heredity also have moderating effects, and learning disorders are likely related to both medical-biologic and environmental risks. The prevalence of learning disorders appears to increase 4-fold in children born at risk. [164] Despite broadly normal IQ, one third of these children have more than one learning disability.

Language difficulties

Functional MRI and event-related potential studies indicate differences in the way auditory and language functions are carried out in patients born prematurely. [189, 190] Language is also susceptible to negative environmental influences.

For preterm infants, many language functions are in the average range, particularly vocabulary, receptive language, verbal fluency, and memory for prose. However, compared with term infants, preterm infants demonstrate deficiencies in relatively complex and subtle verbal processes or measures, such as the following [191, 192] :

  • Understanding syntax
  • Abstract verbal skills
  • Verb production
  • Mean length of utterance
  • Auditory discrimination
  • Ability to follow complex instructions
  • Organization
  • Language processing and reasoning

Deficits in verbal working memory may also be present. In a recent meta-analysis, Barre (2011) found that on average children born VPT had language test scores that ranged from 5.7-11.6 points less than controls at school age. This has an impact on social and academic functioning.

Visuomotor problems

Most VLBW/VPT and ELBW/EPT babies manifest later visuomotor problems. [193, 194] The following skills may be affected [195, 196, 197] :

  • Copying
  • Perceptual matching
  • Spatial processing
  • Finger tapping
  • Pegboard performance
  • Visual memory
  • Spatial organization and visual-sequential memory
  • Handwriting speed and legibility

A greater proportion of preterm patients are left-handed than in the general population. In addition, their probability of needing glasses is 3-fold greater than that of healthy full-term infants. These deficits may contribute to problems with written expression.

Impaired executive function

Executive function is a broad term that refers to coordination of many interrelated processes. It involves purposeful, goal-directed behavior that is instrumental to cognitive, behavioral, and social functions. Executive functions are necessary to plan, execute, and update behavior in response to changing environmental demands. Problems in executive function are reported in children born prematurely, especially if they have white-matter pathology. [194, 198, 199]

Children born VLBW/VPT reportedly have 2-3 times greater difficulty initiating activities, displaying flexibility in generating ideas and strategies for problem solving (shift/switching), holding information in short-term or working memory, planning a sequence of actions, verbal fluency, and organizing information. These deficits appear to be in the so-called "cool" metacognitive processes, versus the "hot," behavioral regulation executive functions (ie, inhibition, emotional control). Deficits in executive function have an impact on IQ, academics, fluid intelligence, and social competence.

Attention-deficit hyperactivity/disorder

Symptoms suggestive of ADHD are reported to occur 2.6-4 times more frequently in children born VLBW/VPT and ELBW/EPT than in controls, with some estimates indicating a 6-fold increase. The male predominance is not as great as in the general population, and there is not strong association with oppositional defiant disorder or conduct disorder. The modal range of ADHD is 20-33% in this population. Vigilance/alertness is more of a problem than is impulsivity, with the inattentive presentation being more frequently reported than the combined presentation. The association between prematurity and ADHD is indirect and could be influenced by environmental advantages and disadvantages.

Other behavioral features have been associated with prematurity. Shyness, unassertiveness, withdrawn behavior, anxiety, depression, and social skills deficits occur more frequently in LBW children than in infants born with normal birth weights. [200, 201, 202] Johnson and Marlow (2011) report a “preterm behavioral phenotype” that includes inattention, anxiety, and social difficulties, with a 3- to 4-fold increased risk for disorders in childhood. [203]

Autism spectrum disorders

LBW and prematurity have been cited as risk factors for autism spectrum disorders (ASDs). In an earlier study by Limperopoulis et al, 20% of 91 toddlers who were VLBW infants had positive results on the Modified Checklist for Autism in Infants and Toddlers (M-CHAT). [204] Although the M-CHAT does not diagnose an ASD, positive findings raise concerns. More recently the M-CHAT was used in the EPICure2 2 study. [175] This applied to children born at 26weeks. A positive screening was found for 41% of the children, and 62% had coexisting disabilities; 95.5% of those with severe motor disabilities and 55.9% of infants with cognitive impairment screened positive, suggesting that positive screen must be viewed in light of other neurodevelopmental sequelae. [205, 206]

Currently, evidence is insufficient to implicate any one perinatal or neonatal factor in ASD etiology. Exposure to a broad range of general, negative influences on perinatal and neonatal health increases the risk, perhaps in an epigenetic fashion. Moreover, differences seem to exist between screening instruments; in the NICHD neonatal network, of infants born at less than 27 weeks, screened at 18-22 months, 20% scored positive on one screen, but only 1% scored positive on all 3 screeners that were used. The items failed were indicative of deficits found in children with language and motor delays, again suggesting confounding comorbidity issues. [207]

Schendel and Bhasin report data from a longitudinal surveillance study that indicate a 2 to 3-fold increase in ASDs in infants born as either LBW or preterm infants compared with peers. [208] An increased tendency was observed toward ASD and mental retardation, intellectual disability, and/or developmental disability compared with ASD alone.

A study by Moster et al that used data from the National Registries of Norway and followed more than 900,000 individuals into adulthood found that the prevalence of ASD in those born prematurely is 2-3 times greater than in the general population and perhaps 7-9 times greater in ELBW infants. [209] These investigators reported an odds ratio (OR) of 9.7 for ASD in those born at 23-27 weeks' gestation and an OR of 7.3 for those born at a postconceptual age of 28-30 weeks.

Finally, in a long-term outcome study of adolescents born LBW/PT, 5% were found to have an ASD, this being 5-times greater than the general population. [210]

Although further investigation is needed, this trend underscores the need for ASD screening at the 18-24 month NICU follow-up visit.

Late preterm infants

Another area of increased interest involves infants born in the late preterm range (34-36 wk), with this group comprising 75% of preterm births. In the last 6-8 weeks of gestation, a 35% increase in brain size and a 5-fold increase in white matter volume occurs. Neuronal connectivity, dendritic arborization, synaptic junction formation, and maturation of neurochemical and enzymatic processes occur. [211]

Chyi et al reported that late preterm (LPT) infants had lower reading and math scores than their term counterparts in kindergarten and first grade and had lower teacher ratings of reading from kindergarten through fifth grade. [212] The likelihood of special education involvement was 1.4 to 2.1 times higher.

Huddy et al reported that one third of their sample of children born at 32-35 weeks' gestation had special education needs. [213] Moster et al documented a risk ratio (RR) of 1.6 for mental retardation/intellectual disability and 1.5 for psychological, developmental, or emotional disorders in late preterm infants. [209]

One of the big questions is the reason for the late preterm delivery, including the possibility of multiple births. Although as a general rule, these children do better than their younger preterm counterparts, they do not do as well as their term peers. [214]

These data suggest that late preterm infants have an incidence of sequelae that falls on a continuum between those born at younger gestational ages and those born at term. Given the large number of these infants, many programs do not routinely follow them, despite the increased risk for neurodevelopmental problems. As a result, the primary care physician should monitor the cognitive and academic performance during routine surveillance.

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Areas of Assessment and Assessment Tools

Proper assessment is critical to evaluate the areas of function that may be impaired in neonatal intensive care unit (NICU) graduates. In both research and clinical surveillance programs, the ideal situation is to extend assessment beyond traditional intelligence quotient (IQ) and achievement testing. Global scores may not help in identifying subtle problems that can interfere with a child’s learning and development.

Tests or rating scales should be directed at measuring the following areas that are likely to be deficient [163, 171] :

  • Intelligence, including verbal and nonverbal function
  • Achievement
  • Attention and executive functions - Planning, organization, monitoring, inhibition, working memory
  • Language - Phonologic awareness, syntax, verbal fluency, comprehension of instructions, high-order abstracting functions
  • Sensorimotor functions - Visual-motor precision, fine motor speed
  • Visual-spatial processes: design copying, visual closure, visual-spatial planning, handwriting
  • Memory and learning - List learning, delayed recall, narrative memory, assessment of semantic, strategic, rote, and episodic verbal and visual functions
  • Behavioral adjustment - Attention-deficit/hyperactivity disorder (ADHD-I), internalizing and socialization problems

Detailed evaluation obviously raises costs, which may be difficult to justify and therefore unfeasible in many clinical or research settings. A compromise is to use representative tests to measure areas of function that are likely to be problematic in children born prematurely or to assess certain functions at different times.

Options for comprehensive cognitive-assessment protocol vary by age. [163] For ages 6 months, 12 months, and 13-24 months, the following instruments are used:

  • Bayley Scales of Infant and Toddler Development, 3rd edition: Cognitive language, motor, social-emotional, and adaptive

Adaptation to the Bayley III has been accompanied by much controversy, [215] this primarily because of inflated scores when compared with the Bayley-II MDI and PDI. Most concerning is that these differences are greater at the lower end of the continuum of neurodevelopmental functioning and this would lead to under-identification or underestimation of children with disabilities. This difference in scores may be due to a change in format (there is now a Cognitive and a Language Composite versus an MDI; the PDI now is a Gross motor and Fine motor scale) and inclusion of at-risk infants and toddlers in the normative sample (10%). The cognitive score is 6-10 points higher than the MDI, depending on the study, while the motor score is 8-14 points higher.

This raises concerns that either the Bayley-III overestimates development or that the Bayley-II was too conservative. [216] Regardless, comparability of the 2 tests is problematic in longitudinal follow-up studies. Correction for prematurity does not seem to be the main reason, as the same trends have been found in full-term samples. [217] This argues for the need to have control groups to help to determine whether improvements in test scores are due to better neonatal care or test characteristics.

For patients aged 3-4 years, the following instruments are often used:

  • Differential Ability Scales-II
  • Bayley Scales of Infant and Toddler Development (up to 42 months)
  • Stanford-Binet Intelligence Scales, 5th edition
  • Wechsler Preschool and Primary Scale of Intelligence, 3rd edition
  • Bracken Basic Concept Scale-3
  • Kaufman Assessment Battery for Children, 2nd edition
  • Behavior Rating Inventory of Executive Function-Preschool version

For patients aged 6 years, the following instruments are used:

  • Wechsler Abbreviated Scale of Intelligence-II
  • Stanford-Binet Intelligence Scales, 5th edition
  • Wechsler Intelligence Scale for Children, 4th edition
  • Developmental Test of Visual-Motor Integration
  • Developmental Neuropsychological Assessment (NEPSY-II)
  • Continuous Performance Test
  • Behavior Rating Inventory of Executive Function (BRIEF)
  • Kaufman Assessment Battery for Children, 2nd edition
  • ADHD Rating Scales

For patients aged 8 years, the following instruments are used:

  • Wechsler Intelligence Scale for Children, 4th edition
  • Wechsler Abbreviated Scale of Intelligence-II
  • Stanford-Binet Intelligence Scales, 5th edition
  • Developmental Neuropsychological Assessment (NEPSY-II)
  • Behavior Rating Inventory of Executive Function
  • Continuous Performance Test
  • Wide Range Assessment of Memory and Learning-2/Children’s Memory Scale/California Verbal Learning Test-C
  • ADHD Rating Scales

The protocols can be altered in frequency, areas assessed, depth of evaluation, and evaluation instruments. Examiners should select specific tests from these lists, tailoring the protocol to the follow-up program of the particular patient.

Scaled-down cognitive-neuropsychological protocols for use at ages 6, 12, and 18-24 months are as follows [163] :

  • Bayley Infant Neurodevelopmental Screener
  • Ages and Stages questionnaire-3
  • Cognitive Adaptive Test and Clinical Linguistic and Auditory Milestone Scale
  • Bayley Scales of Infant and Toddler Development Screener, 3rd edition

Protocols for use at age 3-4 years are as follows:

  • Kaufman Brief Intelligence Test, 2nd edition (age 4 y)
  • Stanford-Binet Intelligence Scales, 5th edition, Abbreviated Battery IQ
  • Kaufman Assessment Battery for Children-2, Short Form
  • Developmental Test of Visual-Motor Integration

Protocols for use at age 6 years are as follows:

  • Wechsler Abbreviated Scale of Intelligence-II
  • Stanford-Binet Intelligence Scales, 5th edition, Abbreviated Battery IQ
  • Kaufman Brief Intelligence Test, 2nd edition
  • Wechsler Intelligence Scale for Children, 4th edition (selected subtests)
  • Continuous Performance Test
  • Developmental Test of Visual-Motor Integration

Protocols for use at age 8 years are as follows:

  • Wechsler Abbreviated Scale of Intelligence-II
  • Stanford-Binet Intelligence Scales, 5th edition, Abbreviated Battery IQ
  • Kaufman Brief Intelligence Test, 2nd edition
  • Developmental Neuropsychological Assessment (NEPSY-II), selected subtests
  • Continuous Performance Test
  • Developmental Test of Visual-Motor Integration

Pre-academic testing can begin at age 4 years, using the Bracken 3-Receptive and expressive tests School Readiness Composite. The Kaufman Test of Educational Achievement-II is applicable for ages 4.5 years and older and has a brief and a comprehensive form; it is applicable to a comprehensive or limited follow-up protocol. [218]

The Wide Range Achievement Test-4 is a gross academic screener that can be used with either level of follow-up mentioned above, this beginning at kindergarten. [219] The Woodcock-Johnson III instrument is often used with detailed follow-up, particularly at ages 6 years and 8 years. [220] , as is the Wechsler Individual Achievement Test-III, beginning at kindergarten.

Visuomotor integrative skills can be assessed from the 3- to 4-year evaluation point using tests specifically designed to evaluate these areas or using components of broad test instruments. The same holds true for language functions. Broad- and narrow-band rating scales can be applied.

In summary, developmental follow-up is a critical component of the overall care of high-risk infants. Numerous options are available in terms of level, frequency, and patient age at the time of assessment.

Follow-up of high-risk infants can be undertaken for surveillance, research, or both. However, serial evaluation is necessary because of rapid developmental changes during infancy, silent periods, test behaviors in young children, and increasing environmental and educational demands that may uncover previously unidentified deficits. Medical status and quality of life should also be monitored. The child’s family and the primary care physician (PCP) should be active partners in this entire process.

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Counseling Caregivers During and After the NICU Stay

Whether they are dealing with extreme prematurity or serious malformations, parents of high-risk neonates typically ask 2 basic questions during counseling: "Will my baby survive?" and "Will my baby be normal?"

These questions cannot be easily addressed, and the answers depend on circumstances of the individual infant. Therefore, answers may need to be given in general terms, which can be frustrating for parents. These questions may arise again during the infant's hospital stay, although parents may be reluctant to ask them again because of their fears about the potential answers.

When an infant arrives in the neonatal intensive care unit (NICU) or in the office during follow-up visits, another important question is, "Is my baby gaining weight or growing?" Parents often reduce their fear and apprehension to these simple terms. The psychological trauma parents experience when their infant is hospitalized in the NICU cannot be underestimated. [221, 222, 223]

One of the most difficult and perhaps most overlooked aspects of care of the high-risk neonate is effective, timely, and compassionate delivery of information to parents and family members by the medical staff involved. Although most members of the medical team are well prepared to meet the physiologic and medical needs of the infants they care for, the psychological, emotional, and spiritual needs of the family members can be ineffectually met when concern is directed primarily toward the infant.

The goal of communication is to provide information to all who need it in an efficient and compassionate manner. Parents are often the first people to whom a change in the patient's status and in future plans should be directed, but others may also require this information. Social service agencies, state and federal agencies, and pastoral support staff may also require timely updates according to the desires of the particular parents.

To effectively counsel parents of high-risk neonates, caregivers should identify the specific needs of the family early in the infant’s hospitalization. Also, members of the medical team should be aware of their individual skills and responsibilities in interacting with parents and extended family members.

A helpful approach is to plan a visit occurring immediately before or soon after discharge to ascertain the expectations of parents regarding their child's outcome and follow-up care. As risk factors for later problems are identified, they must immediately be explained to parents and other care providers in a manner that is understandable and complete. Health care providers must be able to collaborate with parents who may feel isolated and who may be in various stages of grieving.

By suggesting support services or parental support groups, providers may assist parents in coping with the stresses of uncertainty regarding their individual situation and their child's anticipated outcomes. Programs for social service contacts, developmental disability services, home nursing, and economic assistance are addressed in Resources for Parents and Healthcare Professionals. [224]

By the time of discharge, the staff should have established the parents’ understanding about their infant’s status and potential outcome. Medical conditions that require close follow-up should be identified and communicated, and care plans should be developed. Providers must understand and accept cultural diversity in child care, and skilled medical interpreters should be available when necessary. Each medical provider must listen carefully to the parents’ questions to answer them, as well as any implied questions.

The medical team can anticipate that parents will have new questions regarding their child’s potential outcome and abilities at subsequent visits. Goals of medical professionals and other professionals should be to explain previous problems and risks in relation to current physical and developmental findings.

Avoiding medical jargon and using language appropriate to each parent’s level of understanding is important. For example, early use of the term cerebral palsy to explain motor dysfunction of infancy or spastic paresis desensitizes parents to it and may open avenues of explanation regarding neurologic dysfunction and therapeutic intervention.

The phrase "out of the normal range" must often be carefully explained. Although the phrase may be distressing to parents, its use may allow an infant to qualify for interventional services earlier than otherwise possible.

Providers must often explain the difference between medical and parental responsibilities. Initial counseling should be directed at the importance of maintaining the discharge plans, including preventive health care. Routine health care, including immunizations and safety counseling, should be addressed at each visit. The need for respiratory syncytial virus (RSV) prophylaxis and influenza vaccination may be important points of discussion for infants with chronic lung disease.

Regular multidisciplinary neurodevelopmental evaluation must be reinforced, as well as the necessity for preplanned or future referrals to medical specialists. The potential need for periodic or regular evaluations by gastroenterologists, nutritionists, neurologists, rehabilitation specialists, orthopedic surgeons, physical therapists, occupational therapists, and speech therapists should be introduced in the discussions about outcome.

In the process of discharge planning and follow-up, providers should stress the uncertainty of outcomes for specific neurologic risks in the extremely low-birth-weight (ELBW) infant. The propensity for later dysfunction should be discussed, even if an infant appears to be doing well during early follow-up. [169, 225] Although the relative statistical risks associated with some of the previously described conditions should be discussed, clinicians must carefully explain that definitive medical diagnoses can be appreciated only over an extended period.

Physicians must be candid in discussing abnormalities found on examinations in the NICU or during early follow-up visits, especially if they increase the risk of an adverse outcome. Providers may want to avoid sharing this information because it creates sadness and anger in the parents and other caregivers. Nevertheless, the provider must undertake this task because the parents may later claim that they were never told that their infant was affected or at risk.

Avoiding the truth about potential neuromotor and psychomotor disabilities may destroy the relationship and future collaboration between providers and caregivers. Honesty regarding an unfavorable outcome also enables parents and other caregivers to understand what interventions and therapeutic plans are needed.

The counseling process is often complicated because of denial of the apparent facts by the parents and family members. Although honesty regarding anticipated outcomes is always wise, stressing that many high-risk situations change in ways that are not initially evident is prudent. Unrealistic parental hopes can be gently addressed and should not be ridiculed or disparaged.

A stable and consistent home environment almost always improves the infant's outcomes. Guilt over a poor neuromotor and/or psychomotor outcome in an ELBW infant or an infant with a malformation syndrome that requires complex medical care can result in serious discord in the family. The provider must always be aware of this potential situation and be ready to intervene with counseling. Disruption of the family unit only potentiates unfavorable outcomes for the infant or child.

Asking parents what they want or expect, particularly early in the evaluation, is unwise. Rather, suggest to parents that, in the opinion of the multidisciplinary team, certain adverse physical or developmental outcomes might be anticipated and that therapies will be recommended if these outcomes occur. In contrast, asking parents what they feel about such outcomes and plans opens communication and helps them participate, even if they do not completely understand what is being discussed.

Each family unit eventually decides on the degree to which they wish to participate in the health care of their child. As parents learn about their child’s condition and take on advocacy roles, they may become active participants. However, some parents are never able or willing to actively participate in planning. Caution should be taken to avoid making parents feel guilty about too much, or too little, intervention.

As parents find health care providers with whom they can communicate, that individual may assume the role of coordinator of care. [39] Information must flow between this person and other providers. In the ideal situation, this individual is a primary care physician (PCP), but it is often another health care professional participating in the child's care. A primary coordinator with whom parents can feel comfortable should be identified as the team leader because such a person facilitates parent-staff and interdisciplinary communication.

Seamless communication between public and private agencies, as well as parents and physicians, helps prevent duplication of services and provides parents the emotional and medical support that they require for the optimal care of their child. In some cases, the parent may become the best care coordinator for their child. If this situation occurs, the parent must be accepted as an active participant in the health care team.

Health care providers who are unwilling to allow active participation of informed parents in their child’s care create a difficult environment for continuing and optimizing care. Indeed, providers must always remember that the major goal of both parents and providers is to achieve the best outcome for the patients and families. With regard to keeping the family intact, the risk of divorce may increase among parents of NICU graduates, and the PCP should be alert for signs of family stress. [226]

Specific recommendations

Today, counseling of parents often begins before birth. [227] The availability of prenatal biochemical screening tests, improved knowledge of genetic diseases and family histories, and the use of fetal ultrasonography have made the recognition and management of maternal and/or fetal disease more commonplace now than ever before.

Depending on the specific problems, counseling may entail one or more prenatal conferences with obstetricians, perinatologists, geneticists, surgical subspecialists, neonatologists, and the infant’s and family’s PCPs. The PCP should be kept informed about these consultations. These conferences should continue throughout the patient's NICU stay and during visits to the PCP’s office.

Parents or caregivers must actively participate in decision making. Many studies indicate that parental contribution to the physical and psychomotor welfare of their NICU graduate is the most important factor for a favorable long-term outcome. [228]

The Internet has become an important source of information to find institutions that provide specialized care for NICU graduates. Care facilities are often near the family’s home. To identify local or national support groups, parents of infants with extreme prematurity, unusual or complex malformations, or rare metabolic diseases can consult social workers, PCPs, Web-based resources, and special agencies (eg, the March of Dimes). The PCP should take a proactive role in helping parents connect with other parents in similar situations.

The vulnerable child

An interesting and perplexing management problem in the follow-up of high-risk neonates is vulnerable child syndrome. In 1964, Morris Green, MD, described this syndrome. [229] Green summarized its nature in 1986. [230] In brief, NICU graduates, especially those born prematurely, are at risk for developing behavioral problems as a consequence of excessive parental anxiety.

Some investigators have questioned whether this syndrome actually occurs. [231] Others, however, have published strong evidence of its presence in NICU graduates. [232, 233, 234, 235]

Some parents react with an overprotective response after dealing with the emotional stress of multiple medical problems or even simply the admission to the intensive care nursery. Vulnerable child syndrome is occasionally observed when children have a tentative diagnosis of a minor disorder or when physicians suggest that certain neonatal findings be reevaluated in the future.

The vulnerable child response often manifests as limitations in the child's contact with the environment. Parents may limit or prevent exposure with other people or family members. In the most severe form, parents become virtual recluses with their child, refusing to leave the child to care for himself or herself. An exaggerated fear of infection, hypoxia, injury, or ridicule may be the initiating factor for some parents.

Health care providers must reinforce the idea of normal interaction with other children, family members, and extended communities that is limited only by the child’s tolerance. A mistake practitioners frequently make is inadvertently reinforcing the parents' overprotective behavior by accentuating the risks of infection or injury in an infant with residual problems of prematurity, congenital heart disease, or neurologic injury.

Involving children with family members and with other infants is an increasingly important part of normal development. If a child is restricted because of technological dependence or a real infectious risk, a reasonable plan of participation should be designed so that he or she can be involved in as many age-appropriate activities as possible.

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