Who Is at Risk?

High-Risk Infant Follow-up

Isabell B. Purdy, PhD, NNP, CPNP; Mary Alice Melwak, PhD, CPNP


NAINR. 2012;12(4):221-226. 

In This Article

Infants With Special Health Needs

Infants With Pulmonary Conditions

Advances in treatment have also increased the survival rate for infants with pulmonary conditions but not always produced the hoped for decreases in associated morbidity. Nurses should recognize that babies with pulmonary compromises are fragile and need close follow-up with a pediatric pulmonary specialist and high risk infant team that assists with monitoring the physiologic stability, oxygen requirements, calorie expenditures, and nutrition needs throughout the first year of life and risks for reactive airway disease in early childhood. Babies with pulmonary conditions such as bronchopulmonary dysplasia (BPD), persistent pulmonary hypertension (PPHN), congenital diaphragmatic hernia (CDH), and some of the treatments used to support these patients can increase risks for long-term physical and neurologic complications. Individualized predischarge planning is needed to establish comprehensive health care and support services that will be needed post discharge for infants with special health needs with combined biologic and environmental risks.

In the past few decades, antenatal steroids, early surfactant therapy, and a shift to gentler modes of ventilation have minimized much of the severity of lung injury seen in the past. Earlier studies identified that infants with CLD had more deficits in intelligence, attention, reading, mathematics, and gross motor skills that warranted special education services.[17] Most studies conducted on school age outcomes of preterm infants born between 1995 and 2005 did not account for higher cumulative steroid exposures and they varied on dose and duration of treatment of these patients. Specifically concerns about dexamethasone's association with increase risk for neurobehavioral impairments and CP highlighted the need for long-term follow-up of postnatal steroids.[18–21]

Bronchopulmonary dysplasia, a form of chronic lung disease (CLD), has often been associated with increased risk for morbidity and mortality. CLD can result from extended positive pressure ventilation and oxygen treatment. It is more commonly seen in very immature infants, with rates ranging from 50% to 86% among infants born at 23 weeks GA.[6,7,8] Infants born at 23 to 24 weeks GA are reported to be more likely than older preterm infants to be discharged on monitors, numerous medications (eg, diuretics, bronchodilators, steroids, etc), tube feeds, and some remain on oxygen at 18 months of age.[10] Preterm infants with BPD compared to those without BPD have been shown to have higher rates of clinical morbidity, rehospitalization and incidences of developmental delay at 1 year of age[22] and also higher rates of poor cognitive and language skills, lower mean weight and head circumference, more moderate to severe CP, spastic diplegia, and quadriplegia.[23] Variations in treatment effects may account for some of the morbidity and mortality. One study reported lower rates of BPD and death, higher weights, and better developmental scores among very low-birth-weight infants who were treated with continuous positive airway pressure versus conventional mechanical ventilation.[24]

Another pulmonary condition that has been identified with increased risk is persistent PPHN. One study reported on 18 to 22 months outcomes of infants with PPHN who were randomized to early inhaled nitric oxide (iNO) versus a control group and reported no differences in neurodevelopmental impairment or incidence of hearing loss.[25] However, a different study examining three year outcomes of infants with PPHN who were treated with iNO reported an increased risk for auditory brainstem response abnormalities and abnormal neurodevelopment outcomes.[26] At this point, iNO is considered a choice treatment for PPHN, and the best practice should be to continue to follow developmental and hearing re-assessments for this population throughout 3 years of age.[27]

Infants With Intracranial Pathology

There are several intracranial conditions of infancy that may present with seizures or hypotonia in the neonatal period. These babies are always considered high-risk infants due to concern for neuromorbidities (eg, brain asphyxia, strokes, bleeds, hydrocephalus, etc). In general, the childhood outcomes of seizure disorders vary by etiology. Poorer outcomes have been reported in ELBW babies.[28] Hypotonia results from dysfunction at any level of the nervous system and generates an extensive list of differential diagnoses (eg, central or peripheral nervous system abnormalities, myopathies, genetic disorders, endocrinopathies, metabolic diseases, and acute or chronic illnesses).[29] Hypotonia is more commonly a central cause (60%-80% of cases) compared to peripheral causes (15%-30% of cases). Central injury disorders lead to development of increased tone and deep tendon reflexes that is not commonly seen in central developmental disorders.[29]

Central conditions include intracranial hemorrhage, chromosomal disorders, muscle disorders, but the most common cause is hypoxic ischemic encephalopathy (HIE).[29] HIE, often associated with inadequate oxygenation or perfusion of the fetus or infant's brain, may result from a variety of causes (e.g. impaired uteroplacental exchange, umbilical cord compression, maternal hypoxia or hypotension, placental insufficiency or abruption, birth trauma, or failed initiation of respiration).[30] In one study conducted with infants with HIE, analyses revealed that more than 90% had a low 1-minute Apgar score and that death or disability occurred in 76%, 82%, and 80% of infants with 10-minute Apgar scores of 0, 1, and 2, respectively.[31] Newer hypothermia trials for HIE are still underway to identify whether various degrees of cooling can decrease the complications and improve the often devastating outcomes such as severe CP and seizures. Recent studies suggest that seizures do not have an independent impact on babies who received hypothermia.[32]

Neonatal stroke is another devastating diagnosis that requires nurses to prepare families to understand the need for long term follow-up. Outcomes do not necessarily differ between stroke type or laterality of the infarct. During the first 2 years of life when children are expected to meet fine and gross motor milestones, the motor and functional abnormalities become more noticeable over time. One recent study reported better functional outcome at school age of surviving preterm infants with periventricular hemorrhagic infarction.[33]

Infants With Congenital Birth Defects

There are a variety of newborn birth defects, genetic syndromes and conditions (eg, Down syndrome, congenital diaphragmatic hernia, and congenital heart defects to name a few) that are multi-level complex disorders.[34] Myelomeningocele is a defect that is not uncommon to see in NICU. This congenital defect in the closure of the spinal area during embryologic development results in increased risk for psychomotor problems. A wide variety of subspecialists work together as a team to follow these babies (eg, genetics, neurology, audiologists, endocrinology, and developmental specialists and physical therapists).[35]

The most common inherited genetic syndrome is Down Syndrome which occurs at a rate of approximately 1 per 1000 live births. These infants can present with hypotonia, gastrointestinal abnormalities (e.g. duodenal or esophageal atresia, tracheal or pyloric stenosis, anorectal anomalies, Hirschsprung disease) and are at risk for feeding difficulties. Congenital heart disease (CHD) is common (eg, endocardial cushion defects, ventricular and atrial septal defects, and isolated persistent patent ductus arteriosis or Tetralogy of Fallot) and early childhood heart diseases can occur as well (eg, acquired mitral valve prolapse and aortic insufficiency).[36] Even in the absence of CHD these babies are at risk for PPHN and later development of airway obstruction. In addition, they are at high risk for immune dysfunction, thyroid diseases, hematologic disorders, hearing impairment, cataracts, strabismus, and vision problems.[36,37] Down Syndrome offers one example of a genetic condition which requires recognition of multi-organ system involvement. The complexity of genetic conditions like this necessitate a team approach between general pediatrics, many subspecialists (eg, cardiology, endocrinology, gastroenterology, audiology, ophthalmology) and HRIF to assure comprehensive post NICU care.

Babies who are born with CHD frequently undergo extensive surgeries, oxygen challenges, and growth delays that place them at increase risk for morbidities and mortalities. For example, one report points out that the increased risk seen in infants with DiGeorge Syndrome may not be solely the result of their cardiac lesions and cardiac surgery but more related to factors intrinsic to the disease such as hypocalcemia which can predispose patients to worse developmental outcomes.[38] Another CHD study examining infants born with hypoplastic left heart syndrome suggests additional variables such as early GA, genetic syndromes, and preoperative intubation had significant negative effects on development and that surgical variables alone did not affect neurologic outcomes.[39]

Congenital diaphragmatic hernia is a primary anatomical anomaly that can be associated with pulmonary hypertension as it results from embryologic herniation of the abdominal contents into the diaphragm, which can cause compression of lung development, and pulmonary hypoplasia.[40] Despite neonatal advances in surgery and medical care, the mortality rate remains around 20% to 30% with significant morbidity observed in 50% of survivors and associated anomalies in 25% to 57%, for example, heart defects, hydronephrosis, renal agenesis, intestinal atresia, and neurologic defects.[41,42,43] Several complications related to respiratory failure and the need for extensive life saving treatments, such as extracorporeal membrane oxygenation, add to the risk for early demise or latent cognitive, social, and/or neurosensory problems.[44,45] Neurology and HRIF programs should monitor patients out to 3 years of age and assess electroencephalography, neuroimaging, and brain auditory evoked potentials.[46]