Nursing Care Management

The nursing care of the infant with jaundice is discussed in the Nursing Process box and in the following section.

Part of the routine physical assessment includes observing for evidence of jaundice at regular intervals. Jaundice is most reliably assessed by observing the infant’s skin color from head to toe and the color of the sclerae and mucous membranes. Applying direct pressure to the skin, especially over bony prominences such as the tip of the nose or the sternum, causes blanching and allows the yellow stain to be more pronounced. For dark-skinned infants, the color of the sclerae, conjunctiva, and oral mucosa is the most reliable indicator. Also, bilirubin (especially at high levels) is not uniformly distributed in skin. The nurse observes the infant in natural daylight for a true assessment of color.

The TcB is a useful screening device and is used to detect neonatal jaundice in full-term infants. Because phototherapy reduces the accuracy of the instrument, its value is limited to assessments made before the initiation of phototherapy. Institutions in which the device is used set up their own criteria based on their experience with their particular instrument. Blood samples are also taken for the measurement of bilirubin in the laboratory.

With short hospital stays, jaundice may appear after discharge. A careful history from the parents may reveal significant familial patterns of hyperbilirubinemia (older siblings of the infant). Other considerations in assessment include the ethnic origin of the family (e.g., higher incidence in Asian infants); type of delivery (e.g., induction of labor); and infant characteristics such as weight loss after birth, gestational age, sex, and the presence of any bruising. The method and frequency of feeding are assessed.

Prevention of jaundice may be possible with early introduction of feedings and frequent nursing without supplementation. Every effort is made to provide an optimum thermal environment to reduce metabolic needs.

Blood Incompatibility

The membranes of human blood cells contain a variety of antigens, also known as agglutinogens, substances capable of producing an immune response if recognized by the body as foreign. The reciprocal relationship between antigens on RBCs and antibodies in the plasma causes agglutination (clumping). In other words, antibodies in the plasma of one blood group (except the AB group, which contains no antibodies) produce agglutination when mixed with antigens of a different blood group. In the ABO blood group system the antibodies occur naturally. In the Rh system the person must be exposed to the Rh antigen before significant antibody formation takes place and causes a sensitivity response known as isoimmunization.

Clinical Manifestations

Jaundice may appear shortly after birth (during the first 24 hours) in the newborn affected by HDN, and serum levels of unconjugated bilirubin rise rapidly. Anemia results from the hemolysis of large numbers of erythrocytes, and hyperbilirubinemia and jaundice result from the liver’s inability to conjugate and excrete the excess bilirubin. Most newborns with HDN are not jaundiced at birth. However, hepatosplenomegaly and varying degrees of hydrops may be evident. If the infant is severely affected, signs of anemia (notably, marked pallor) and hypovolemic shock are apparent. Hypoglycemia may occur as a result of pancreatic cell hyperplasia.

Diagnostic Evaluation

Early identification and diagnosis of Rh D sensitization are important in the management and prevention of fetal complications. A maternal antibody titer (indirect Coombs test) should be drawn at the first prenatal visit. Genetic testing allows early identification of paternal zygosity at the RHD gene locus, thus allowing earlier detection of the potential for isoimmunization and avoiding further maternal or fetal testing (Moise, 2002). Amniocentesis can be used to test the fetal blood type of a woman whose antibody screen is positive; the use of polymerase chain reaction may determine the fetal blood type and presence of maternal antibodies. The fetal hemoglobin and hematocrit can also be measured (Moise, 2002). Chorionic villus sampling has drawbacks that preclude its use, including possible spontaneous abortion of the fetus and fetomaternal hemorrhage, which would essentially makethe situation worse. With either method, if the fetus is found to be Rh negative, no further treatment is required.

Ultrasonography is considered an important adjunct in the detection of isoimmunization; alterations in the placenta, umbilical cord, and amniotic fluid volume, as well as the presence of fetal hydrops, can be detected with high-resolution ultrasonography and allow early treatment before the development of erythroblastosis. Doppler ultrasonography of fetal middle cerebral artery peak velocity has been used to detect and measure fetal hemoglobin and, subsequently, fetal anemia (Moise, 2002). Erythroblastosis fetalis caused by Rh incompatibility can also be monitored by evaluating rising anti-Rh antibody titers in the maternal circulation or by testing the optical density of amniotic fluid (delta OD₄₅₀ test), since bilirubin discolors the fluid (Mari, 2000).

The disease in the newborn is suspected on the basis of the timing and appearance of jaundice (see Table 9-3) and can be confirmed postnatally by detecting antibodies attached to the circulating erythrocytes of affected infants (direct Coombs test or direct antiglobulin test). The Coombs test may be performed on cord blood samples from infants born to Rh-negative mothers if there is a history of incompatibility or further investigation is warranted.

Therapeutic Management

The primary aim of therapeutic management of isoimmunization is prevention. Postnatal therapy is usually phototherapy for mild cases and exchange transfusion for more severe forms. Although phototherapy may control bilirubin levels in mild cases, the hemolytic process may continue, causing severe anemia between 7 and 21 days of life.

Nursing Care Management

The initial nursing responsibility is recognizing jaundice. The possibility of hemolytic disease can be anticipated from the prenatal and perinatal history. Prenatal evidence of incompatibility and a positive Coombs test result are cause for increased vigilance for early signs of jaundice in an infant.

If an exchange transfusion is required, the nurse prepares the infant and the family and assists the practitioner with the procedure. The infant receives nothing by mouth (is NPO) during the procedure; therefore a peripheral infusion of dextrose and electrolytes is established. The nurse documents the blood volume exchanged, including the amount of blood withdrawn and infused; the time of each procedure; and the cumulative record of the total volume exchanged. Vital signs, monitored electronically, are evaluated frequently and correlated with the removal and infusion of blood. If signs of cardiac or respiratory problems occur, the procedure is stopped temporarily and resumed after the infant’s cardiorespiratory function stabilizes. The nurse also observes for signs of blood transfusion reaction and maintains the infant’s blood glucose levels and fluid balance.

Throughout the procedure attention must be given to the infant’s thermoregulation. Hypothermia increases oxygen and glucose consumption, causing metabolic acidosis. Not only do these consequences hinder the infant’s overall physical ability to withstand the long procedure, but they also inhibit the binding capacity of albumin and bilirubin and the hepatic enzymatic reactions, thus increasing the risk of kernicterus. Conversely, hyperthermia damages the donor erythrocytes, elevating the free potassium content and predisposing the infant to cardiac arrest.

The exchange transfusion is performed with the infant in a radiant warmer. However, the infant is usually covered with sterile drapes that may prevent the radiant heat from sufficiently warming the skin. The blood may also be warmed (using specially designed devices, never a microwave oven) before infusion.

After the procedure is completed, the nurse inspects the umbilical site for evidence of bleeding. The catheter may remain in place in case repeated exchanges are required.

Pathophysiology

Preterm infants are born before the lungs are fully prepared to serve as efficient organs for gas exchange. This appears to be a critical factor in the development of RDS. The effects of lung immaturity are compounded by the presence of more cartilage in the chest wall, leading to increased compliance of the chest wall, which collapses inward in response to less compliant (stiffer) lung tissue.

There is evidence of fetal respiratory activity before birth. The lungs make feeble respiratory movements, and fluid is excreted through the alveoli. Because the final unfolding of the alveolar septa, which increases the surface area of the lungs, occurs during the last trimester of pregnancy, preterm infants are born with numerous underdeveloped and many uninflatable alveoli. Pulmonary blood flow is limited as a result of the collapsed state of the fetal lungs—poor vascular development in general and an immature capillary network in particular. Because of increased pulmonary vascular resistance, the major portion of fetal blood is shunted from the lungs by way of the ductus arteriosus and foramen ovale.

At birth, infants must initiate breathing and keep the previously fluid-filled lungs inflated with air. At the same time, the pulmonary capillary blood flow must be increased approximately tenfold to provide for adequate lung perfusion and to alter the intracardiac pressure that closes the fetal cardiac structures. Most full-term infants successfully accomplish these adjustments, but preterm infants with respiratory distress are unable to do so. Although numerous factors are involved, immaturity of the surfactant system plays a central role.

Surfactant is a surface-active phospholipid secreted by the alveolar epithelium. Acting much like a detergent, this substance reduces the surface tension of fluids that line the alveoli and respiratory passages, resulting in uniform expansion and maintenance of lung expansion at low intraalveolar pressure. Immature development of these functions produces consequences that seriously compromise respiratory efficiency. Deficient surfactant production causes unequal inflation of alveoli on inspiration and the collapse of alveoli on end expiration. Without surfactant, infants are unable to keep their lungs inflated and therefore exert a great deal of effort to reexpand the alveoli with each breath. With increasing exhaustion, infants are able to open fewer and fewer alveoli. This inability to maintain lung expansion produces widespread atelectasis.

In the absence of alveolar stability (normal functional residual capacity) and with progressive atelectasis, pulmonary vascular resistance (PVR) increases, whereas with normal lung expansion it would decrease. Consequently, there is hypoperfusion to the lung tissue, with a decrease in effective pulmonary blood flow. The increase in PVR causes partial reversion to the fetal circulation, with a right-to-left shunting of blood through the persisting fetal communications—the ductus arteriosus and foramen ovale.

Inadequate pulmonary perfusion and ventilation produce hypoxemia and hypercapnia. Pulmonary arterioles, with theirthick muscular layer, are markedly reactive to diminished oxygen concentration. Thus a decrease in oxygen tension causes vasoconstriction in the pulmonary arterioles that is further enhanced by a decrease in blood pH. This vasoconstriction contributes to a marked increase in PVR. In normal ventilation with increased oxygen concentration, the ductus arteriosus constricts and the pulmonary vessels dilate to decrease PVR.

Prolonged hypoxemia activates anaerobic glycolysis, which produces increased amounts of lactic acid. An increase in lactic acid causes metabolic acidosis; inability of the atelectatic lungs to blow off excess carbon dioxide produces respiratory acidosis. Acidosis causes further vasoconstriction. With deficient pulmonary circulation and alveolar perfusion, partial pressure of oxygen in arterial blood continues to fall, pH falls, and the materials needed for surfactant production are not circulated to the alveoli.

Diagnostic Evaluation

The diagnosis of RDS is made on the basis of clinical manifestations (Box 9-4) and radiographic studies. Radiographic findings characteristic of RDS include (1) a diffuse granular pattern over both lung fields that closely resembles ground glass and represents alveolar atelectasis and (2) dark streaks, or bronchograms, within the ground glass areas that represent dilated, air-filled bronchioles. It is important to distinguish between RDS and pneumonia in infants with respiratory distress. The extent of respiratory function and acid-base balance is determined by blood gas analysis. Criteria for visually evaluating the degree of respiratory distress are illustrated in Fig. 9-19. Pulse oximetry and carbon dioxide monitoring, as well as pulmonary function studies, assist in differentiating pulmonary and extrapulmonary illness and are used in the management of RDS.

Therapeutic Management

The treatment of RDS involves immediate establishment of adequate oxygenation and ventilation and supportive care and measures required for any preterm infant, as well as those instituted to prevent further complications associated with preterm birth. The supportive measures most crucial to a favorable outcome are to:

Nipple and gavage feedings are contraindicated in any situation that creates a marked increase in respiratory rate because of the greater hazards of aspiration. Nutrition is provided by parenteral therapy during the acute stage of the disease, while minimal enteral feeding is provided to enhance maturation of the neonate’s gastrointestinal system.

The administration of exogenous surfactant to preterm neonates with RDS has become an accepted and common therapy in most neonatal centers worldwide. Numerous clinical trials involving the administration of exogenous surfactant to infants with or at high risk for RDS demonstrate improvements in blood gas values and ventilator settings, decreased incidence of pulmonary air leaks, intraventricular hemorrhage, decreased deaths from RDS, and an overall decreased infant mortality rate (Merrill and Ballard, 2003; Stevens and Sinkin, 2007). The overall rates of some associated comorbidities (bronchopulmonary dysplasia, NEC, patent ductus arteriosus) have not decreased with surfactant replacement. Exogenous surfactant is derived from a natural source (e.g., porcine, bovine) or from the production of artificial surfactant.

Complications seen with surfactant administration include pulmonary hemorrhage and mucous plugging. Surfactant therapy is also being investigated for use in infants with meconium aspiration, infectious pneumonia, sepsis, persistent pulmonary hypertension, and lung hypoplasia concomitant with congenital diaphragmatic hernia (Finer, 2004; Stevens and Sinkin, 2007). Surfactant may be administered at birth as a preventive or prophylactic treatment of RDS or later on in the course of RDS as a rescue treatment. Surfactant is administered via an ET tube directly into the infant’s trachea. Nursing responsibilities with surfactant administration include assistance in the delivery of the product, collection and monitoring of arterial blood gases, scrupulous monitoring of oxygenation with pulse oximetry, and assessment of the infant’s tolerance of the procedure. After surfactant is absorbed, there is usually an increase in respiratory compliance that requires adjustment of ventilator settings to decrease mean airway pressure and prevent overinflation or hyperoxemia. Suctioning is usually delayed for an hour or so (depending on the type of surfactant and unit protocol) to allow maximum effects to occur. Research is in progress to investigate the possibility of delivering an aerosolized surfactant (Mazela, Merritt, and Finer, 2007). This method would decrease the problems associated with current delivery systems (contamination of the airway, interruption of mechanical ventilation, and loss of the drug in the ET tubing from reflux).

The goals of oxygen therapy are to provide adequate oxygen to the tissues, prevent lactic acid accumulation resulting from hypoxia, and at the same time avoid the potentially negative effects of oxygen and barotrauma. Numerous methods have been devised to improve oxygenation (Table 9-6). All require that the gas be warmed and humidified before entering the respiratory tract. If the infant does not require mechanical ventilation, oxygen can be supplied by nasal cannula or via nasal prongs in conjunction with continuous positive airway pressure (CPAP) (see Oxygen Therapy, Chapter 22). If oxygen saturation of the blood cannot be maintained at a satisfactory level and the carbon dioxide level (Paco ₂) rises, infants will require ventilatory assistance.

Nursing Care Management

Care of infants with RDS involves all of the observations and interventions previously described for high-risk infants. In addition, the nurse is concerned with the complex problems related to respiratory therapy and the constant threat of hypoxemia and acidosis that complicates the care of patients in respiratory difficulty.

The respiratory therapist, an important member of the neonatal intensive care team, is often responsible for the maintenance of respiratory equipment. Although it may be the respiratory therapist’s responsibility to regulate the apparatus, nurses should understand the equipment and be able to recognize when it is not functioning correctly. The most essential nursing function is to observe and assess the infant’s response to therapy. Continuous monitoring and close observation are mandatory because an infant’s status can change rapidly and because oxygen concentration and ventilation parameters are prescribed according to the infant’s blood gas measurements and pulse oximetry readings.

Changes in oxygen concentration are based on these observations. The amount of oxygen administered, expressed as the fraction of inspired air (Fio₂), is determined on an individual basis according to pulse oximetry or direct or indirect measurement of arterial oxygen concentration. Capillary samples collected from the heel (see Chapter 22 for procedure) are useful for pH and Paco₂ determinations but not for oxygenation status. Continuous transcutaneous or pulse oximetry readings are recorded at least hourly. Blood sampling is performed after ventilator changes for the acutely ill infant and thereafter when clinically indicated.

Mucus may collect in the respiratory tract as a result of the infant’s pulmonary condition. Secretions interfere with gas flow and predispose the infant to obstruction of the passages, including the ET tube. Suctioning should be performed only when necessary and should be based on individual infant assessment, which includes auscultation of the chest, evidence of decreased oxygenation, excess moisture in the ET tube, or increased infant irritability. During suctioning a variety of techniques can be used to minimize complications, including the use of a closed suctioning system (Cifuentes, Segars, and Carlo, 2003).

When nasopharyngeal passages, the trachea, or the ET tube is being suctioned, the catheter should be inserted gently but quickly; intermittent suction is applied as the catheter is withdrawn. It is imperative that the time the airway is obstructed by the catheter be limited to no more than 5 seconds, because continuous suction removes air from the lungs along with the mucus. It is recommended that the “two-person” suctioning procedure be used on infants who are acutely ill and who do not tolerate any procedure without profound decreases in oxygen saturation, BP, and heart rate. The object of suctioning an artificial airway is to maintain patency of that airway, not the bronchi. Suction applied beyond the ET tube can cause traumatic lesions of the trachea. The use of in-line suction catheters may decrease airway contamination and hypoxia.

The most advantageous positions for facilitating an infant’s open airway are on the side with the head supported in alignment by a small folded blanket or, when on the back, positioned to keep the neck slightly extended. With the head in the “sniffing” position, the trachea is opened at its maximum; hyperextension reduces the tracheal diameter in neonates.

Inspection of the skin is part of routine infant assessment. Position changes and the use of water pillows are helpful in guarding against skin breakdown.

Mouth care is especially important when infants are receiving nothing by mouth, and the problem is often aggravated by the drying effect of oxygen therapy. Drying and cracking can be prevented by good oral hygiene using sterile water. Irritation to the nares or mouth that occurs from appliances used to administer oxygen (e.g., nasal CPAP) may be reduced by the use of a water-soluble ointment.

The nursing care of an infant with RDS is a demanding role; meticulous attention must be given to subtle changes in the infant’s oxygenation status. The importance of attention to detail cannot be overemphasized, particularly in regard to medication administration. (See Nursing Care Plan.)

 Jitteriness is not accompanied by ocular movement as are seizures.

 The dominant movement in jitteriness is tremor, whereas seizure movement is clonic jerking that cannot be stopped by flexion of the affected limb.

 Jitteriness is highly sensitive to stimulation, whereas seizures are not.

Diagnostic Evaluation

Early evaluation and diagnosis of seizures are urgent. In addition to a careful physical examination, the pregnancy and family histories are investigated for familial and prenatal causes. Blood is drawn for glucose and electrolyte examination, and cerebrospinal fluid may be obtained for testing of cell count and differential, protein, glucose, and culture. Electroencephalography may help identify subtle seizures but is less helpful in establishing a diagnosis. Other diagnostic procedures, such as computed tomography, magnetic resonance imaging, and cerebral ultrasound, may be indicated. A video electroencephalogram may be used to identify seizure activity in some newborns. More extensive metabolic testing may be needed when initial test results do not provide a diagnosis or the history is suggestive of an inherited metabolic disorder.

Therapeutic Management

Treatment is directed toward prevention of cerebral damage and involves correction of metabolic derangements, respiratory and cardiovascular support, and suppression of the seizure activity. The underlying cause is treated (e.g., glucose infusion for hypoglycemia, calcium for hypocalcemia, antibiotics for infection). If needed, respiratory support is provided for hypoxia, and anticonvulsants may be administered, especially when the other measures fail to control the seizures. Phenobarbital, given intravenously or orally, has been the drug of choice and is used if seizures are severe and persistent. Other drugs that may be used are phenytoin (Dilantin), lorazepam, and diazepam (Valium).

A newer drug, fosphenytoin sodium, is a water-soluble prodrug and has been designed to replace phenytoin. Fosphenytoin metabolizes to form phenytoin in the body yet can easily be diluted or mixed in dextrose and normal saline and may be given via IV or intramuscular routes. In addition, fosphenytoin does not cause pain during IV administration.

Nursing Care Management

The major nursing responsibilities in the care of infants with seizures are to recognize when the infant is having a seizure so that therapy can be instituted, to carry out the therapeutic regimen, and to observe the response to the therapy and any further evidence of seizures or other symptomatology. Assessment and other aspects of care are the same as for all high-risk infants. Parents need to be informed of their infant’s status, and the nurse should reinforce and clarify the practitioner’s explanations. The infant’s behaviors need to be interpreted for the parents, and the infant’s responses to the treatment must be anticipated and their significance explained. Parents are encouraged to visit their infant and perform the parenting activities consistent with the care plan. Seizures are a frightening phenomenon and generate a great deal of anxiety and fear, which is easily compounded by the justifiable concern of the staff. Providing support and guidance is an important nursing function.

SEPSIS

Sepsis, or septicemia, refers to a generalized bacterial infection in the bloodstream. Neonates are highly susceptible to infection as a result of diminished nonspecific (inflammatory) and specific (humoral) immunity, such as impaired phagocytosis, delayed chemotactic response, minimal or absent immunoglobulin A and immunoglobulin M (IgA and IgM), and decreased complement levels. Because of the infant’s poor response to pathogenic agents, there is usually no local inflammatory reaction at the portal of entry to signal an infection, and the resulting symptoms tend to be vague and nonspecific. Consequently, diagnosis and treatment may be delayed.

Breastfeeding has a protective benefit against infection and should be promoted for all newborns. It is of particular benefit to the high-risk neonate. Colostrum contains agglutinins that are effective against gram-negative bacteria.

Sepsis in the neonatal period can be acquired prenatally across the placenta from the maternal bloodstream or during labor from ingestion or aspiration of infected amniotic fluid. Prolonged rupture of the membranes always presents a risk for this type from maternal-fetal transfer of pathogenic organisms. In utero transplacental transfer can occur with organisms and viruses such as cytomegalovirus, toxoplasmosis, and Treponema pallidum (syphilis), which cross the placental barrier during the latter half of pregnancy. Intrapartum infection may occur via contact with an infected mother; examples of such infections include herpesvirus and HIV.

Early-onset sepsis (less than 3 days after birth) is acquired in the perinatal period; infection can occur from direct contact with organisms from the maternal gastrointestinal and genitourinary tracts. The most common infecting organism is Escherichia coli, whereas group B streptococci (GBS) rates remain low (Stoll, Hansen, Higgins, and others, 2005). E. coli, which may be present in the vagina, accounts for approximately half of all cases of sepsis caused by gram-negative organisms. GBS is an extremely virulent organism in neonates, with a high (50%) death rate in affected infants. Other bacteria noted to cause early-onset infection include Haemophilus influenzae, Citrobacter and Enterobacter organisms, coagulase-negative staphylococci, and Streptococcus viridans (Stoll, Hansen, Higgins, and others, 2005). Other pathogens that are harbored in the vagina and may infect the infant include gonococci, C. albicans, herpes simplex virus (type II), and chlamydia.

Late-onset sepsis (1 to 3 weeks after birth) is primarily nosocomial, and the offending organisms are usually staphylococci, Klebsiella organisms, enterococci, E. coli, and Pseudomonas or Candida species (Stoll, Hansen, Fanaroff, and others, 2002). Coagulase-negative staphylococci, considered to be primarily a contaminant in older children and adults, is commonly found to be the cause of septicemia in ELBW and VLBW infants. Bacterial invasion can occur through sites such as the umbilical stump; the skin; mucous membranes of the eye, nose, pharynx, and ear; and internal systems such as the respiratory, nervous, urinary, and gastrointestinal systems.

Postnatal infection is acquired by cross-contamination from other infants, personnel, or objects in the environment. Bacteria that are commonly called “water bugs” (because they are able to grow in water) are found in water supplies, humidifying apparatus, sink drains, suction machines, and most respiratory equipment. Organisms such as coagulase-negative staphylococci, which usually colonize the skin, may infect indwelling venous and arterial catheters used for infusions, blood sampling, and monitoring of vital signs. Neonatal sepsis is most common in the infant at risk, particularly the preterm infant or the infant born after a difficult or traumatic labor and delivery, who is least capable of resisting such bacterial invasion. These organisms are often transmitted by personnel from person to person or object to person by poor hand washing and inadequate housecleaning.

NECROTIZING ENTEROCOLITIS

NEC is an acute inflammatory disease of the bowel with increased incidence in preterm infants. Three factors appear to play an important role in the development of NEC: intestinal ischemia, colonization by pathogenic bacteria, and substrate (formula feeding) in the intestinal lumen. The precise cause of NEC is still uncertain, but it appears to occur in infants whosegastrointestinal tract has suffered vascular compromise. Intestinal ischemia of unknown etiology, immature gastrointestinal host defenses, bacterial proliferation, and feeding substrate are now believed to have a multifactorial role in the etiology of NEC. Prematurity remains the most prominent risk factor in the development of NEC.

The damage to mucosal cells lining the bowel wall is great. Diminished blood supply to these cells causes their death in large numbers; they stop secreting protective, lubricating mucus; and the thin, unprotected bowel wall is attacked by proteolytic enzymes. Thus the bowel wall continues to swell and break down; it is unable to synthesize protective IgM, and the mucosa is permeable to macromolecules (e.g., exotoxins), which further hampers intestinal defenses. Gas-forming bacteria invade the damaged areas to produce pneumatosis intestinalis, the presence of gas in the submucosal or subserosal surfaces of the bowel.

A consistent relationship has been observed between the development of NEC and enteric feeding of hypertonic substances (e.g., formula, hyperosmolar medications). It is unclear whether this connection is a result of the formula imposing a stress on an ischemic bowel, serving as a substrate for bacterial growth, or both.

INFANTS OF DIABETIC MOTHERS

Before insulin therapy, few women with diabetes were able to conceive; for those who did, the mortality rate for both mother and infant was high. The morbidity and mortality of infants of diabetic mothers (IDMs) have been significantly reduced as a result of effective control of maternal diabetes and an increased understanding of fetal disorders. Because infants born to women with gestational diabetes mellitus are at risk for the same complications as IDMs, the following discussion of IDMs includes infants born to women with gestational diabetes mellitus.

The severity of the maternal diabetes affects infant survival. Severity of maternal diabetes is determined by the duration of the disease before pregnancy; age of onset; extent of vascular complications; and abnormalities of the current pregnancy, such as pyelonephritis, diabetic ketoacidosis, pregnancy-induced hypertension, and noncompliance. The single most important factor influencing fetal well-being is the euglycemic status of the mother. It has been found that reasonable metabolic control that begins before conception and continues during the first weeks of pregnancy can prevent malformation in an IDM. Elevated levels of hemoglobin A1c during the first trimester appear to be associated with a higher incidence of congenital malformations.

Hypoglycemia may appear a short time after birth and in IDMs is associated with increased insulin activity in the blood (see also Table 9-5, p. 285). The serum glucose level that corresponds to clinical hypoglycemia have not been well defined. Because some infants experience metabolic complications at higher levels than previously thought, some researchers recommend that serum glucose levels be maintained above 45 mg/dl (2.5 mmol/L) in infants with abnormal clinical symptoms, and as high as 50 or 60 mg/dl in other infants (Deshpande and Ward, 2005; Cornblath, Hawdon, Williams, and others, 2000).

Hypoglycemia in the IDM is related to hypertrophy and hyperplasia of the pancreatic islet cells, and thus is a transient state of hyperinsulinism. High maternal blood glucose levels during fetal life provide a continual stimulus to the fetal islet cells for insulin production (glucose easily passes the placental barrier from maternal to fetal side; insulin, however, does not cross the placental barrier). This sustained state of hyperglycemia promotes fetal insulin secretion that ultimately leads to excessive growth and deposition of fat, which probably accounts for the infants who are large for gestational age, or macrosomic. When the neonate’s glucose supply is removed abruptly at the time of birth, the continued production of insulin soon depletes the blood of circulating glucose, creating a state of hyperinsulinism and hypoglycemia within ^1/2 to 4 hours, especially in infants of mothers with poorly controlled diabetes (formerly class C diabetes or beyond [class D through R]). Precipitous drops in blood glucose levels can cause serious neurologic damage or death.

IDMs have a characteristic appearance (Box 9-8). Infants of mothers with advanced diabetes may be small for gestational age, may have IUGR, or may be appropriate for gestational age because of the maternal vascular (placental) involvement. There is an increase in congenital anomalies in IDMs in addition to a high susceptibility to hypoglycemia, hypocalcemia, hypomagnesemia, polycythemia, hyperbilirubinemia, cardiomyopathy, hypomagnesemia, and RDS. Hyperinsulinemia and hyperglycemia in the diabetic mother may be factors in reducing fetal surfactant synthesis, thus contributing to the development of RDS. Although large, these infants may be delivered before term as a result of maternal complications or increased fetal size.

DRUG-EXPOSED INFANTS^*

Maternal habits hazardous to the fetus and neonate include drug addiction, smoking, and alcohol abuse. Occasional withdrawal reactions have been reported in neonates of mothers who use to excess such drugs as barbiturates, alcohol, amphetamines, or antidepressants. Serious reactions are seen in neonates whose mothers abuse psychoactive drugs or are treated with methadone.

Narcotics, which have a low molecular weight, readily cross the placental membrane and enter the fetal system. Illicit substances may also be transmitted to the newborn through breast milk. When the mother is a habitual user of opiates, especially heroin or methadone, the unborn child may also become chemically dependent or passively addicted to the drug, which places such infants at risk during the perinatal and early neonatal periods. Neonatal abstinence syndrome (NAS) is the term used to describe the set of behaviors exhibited by the infant exposed to chemical substances in utero.

MATERNAL INFECTIONS

The range of pathologic conditions produced by infectious agents is large, and the difference between the maternal and fetal effects caused by any one agent is also great. Some maternal infections, especially during early gestation, can result in fetal loss or malformations because the fetus’s ability to handle infectious organisms is limited and the fetal immunologic system is unable to prevent the dissemination of infectious organisms to the various tissues.

Not all prenatal infections produce teratogenic effects. Furthermore, the clinical picture of disorders caused by transplacental transfer of infectious agents is not always well defined. Some microbial agents can cause remarkably similar manifestations, and it is not uncommon to test for all when a prenatal infection is suspected. This is the so-called TORCH complex, an acronym for:

To determine the causative agent in a symptomatic infant, tests are performed to rule out each of these infections. The O category may involve testing for several viral infections (e.g., hepatitis B, varicella zoster, measles, mumps, HIV, syphilis, and human parvovirus). Bacterial infections are not included in the TORCH workup because they are usually identified by clinical manifestations and readily available laboratory tests. Gonococcal conjunctivitis (ophthalmia neonatorum) and chlamydial conjunctivitis have been significantly reduced by prophylactic measures at birth (see Chapter 8). The major maternal infections, their possible effects, and specific nursing considerations are outlined in Table 9-11.

Malformation—Results from deficient formation of tissues. Example: Ventricular septal defects

Deformation—Results from the action of mechanical forces on a normal tissue. These forces may be extrinsic to the developing embryo, such as uterine constraints (especially common during the second trimester of development), or intrinsic, resulting from consequences of a primary malformation. Example: Arthrogryposes, or contraction of the lower limbs

Disruption—Results from the breakdown of previously normal tissue. Example: Amnion disruption sequence (amniotic band sequence)

Dysplasia—Results from abnormal organization of cells within a tissue. Example: Hamartomas

Diagnosis—What is the disorder?

Etiology—What caused it?

Prognosis—What are possible consequences?

Therapy—What can be done?

Recurrence risk—Will it happen again?

Prenatal diagnosis—Is testing available for future pregnancies?

GENETIC ETIOLOGY OF CONGENITAL ANOMALIES

DEFECTS CAUSED BY CHEMICAL AGENTS

Prenatal environmental influences from chemicals such as alcohol, medications, or drugs; infectious disease; or radiation or other environmental influences may be regarded as nongenetic causes of congenital anomalies because these effects can produce congenital structural, functional, or growth defects. An agent that produces congenital malformations or increases their incidence is called a teratogen.

The relationship of the fetal and maternal circulations allows for the interchange of chemical substances across the placental membrane. Many drugs have been suspected of producing congenital malformations, and some have been definitely implicated. Some of the most recognized teratogenic drugs include alcohol, tobacco, antiepileptic medications, isotretinoin (Accutane), lithium, cocaine, and diethylstilbestrol (Table 9-13).

The extent to which chemical agents affect the unborn child depends on the interplay of several factors: the nature of the agent and its accessibility to the fetus, the gestational age at which exposure occurred, the level and duration of the dosage, and the genetic makeup of the fetus. For example, fetal exposure to valproic acid in the first 3 months of pregnancy may result in congenital anomalies such as neural tube defects, congenital heart defects, and distinctive facial features. The limited metabolic capabilities of the fetal liver and its immature enzyme and transport systems render the unborn child ill equipped for maintaining homeostasis when chemical disturbances are imposed by the mother or the environment. This includes both substances produced by the mother in response to a disease state (such as diabetes) and exogenous substances ingested or inhaled by the mother.

The teratogenic effect of drugs is not believed to have an effect on developing tissue until day 15 of gestation, when tissue differentiation begins to take place. Before that time, drugs usually have little effect because they are believed to have an insignificant affinity for undifferentiated tissue. Also, until implantation takes place, at approximately 7 days after conception, the embryo is not exposed to maternal blood that contains the drug. However, some drugs may affect the uterine lining, making it unsuitable for implantation. Drugs administered between days 15 and 90 may produce an effect if the tissue for which the drug has an affinity is in the process of differentiation at that time. After 90 days, when differentiation is complete, most fetal tissues are believed to be relatively resistant to teratogenic effects of drugs. However, the impact on ongoing neurologic development is not known.

CONGENITAL HYPOTHYROIDISM

Congenital hypothyroidism (CH) may have a number of causes and can be either permanent or transient. Transient CH is frequently associated with maternal Graves disease that was treated with antithyroid drugs. The majority of cases are sporadic (nonhereditary), but approximately 15% of all cases are transmitted as an autosomal dominant trait. The most common pathogenesis is thyroid dysgenesis, mostly with unknown causes. Worldwide, the most common cause of CH resulting in endemic cretinism is iodine deficiency. However, no matter what the cause, the manifestations (Box 9-11) and management are similar. In some conditions the thyroid deficiency is severe and manifestations develop early; in others, the symptoms may be delayed for months or years. Early detection and prompt initiation of treatment are essential, since their delay will result in various degrees of mental retardation, in which the IQ loss has a direct relationship to the time treatment is initiated. If treatment is implemented from 0 to 3 months of age, the mean IQ attained is 89 (range: 64 to 107); if treatment begins at 3 to 6 months, mean IQ will reach 71 (range: 36 to 96); treatment initiated after 6 months of age will result in a mean IQ of 54 (range: 25 to 80).

Results of screening tests in the United States indicate that CH occurs in approximately 1 in 4000 to 1 in 3000 newborns (Kaye, Committee on Genetics, Accurso, and others, 2006). It affects all races and ethnicities, but it is more prevalent among Hispanic and American Indian or Alaskan Native people (1 in 2000 to 1 in 700 newborns) and less prevalent among African Americans (1 in 3200 to 1 in 17,000 newborns). Infants with Down syndrome have a much higher rate of either permanent or transient forms of the disorder (approximately 1 in 140 newborns) (Kaye, Committee on Genetics, Accurso, and others, 2006). Also, a higher incidence of other congenital abnormalities has been observed in infants with CH. In addition, many preterm infants have transient hypothyroidism (hypothyroxinemia) at birth as a result of hypothalamic and pituitary immaturity. Some screening programs target both primary (thyroid-based) and secondary (pituitary-based) hypothyroidism.

PHENYLKETONURIA

PKU, an inborn error of metabolism inherited as an autosomal recessive trait (the PAH gene is located on chromosome 12q24), is caused by a deficiency or absence of the enzyme needed to metabolize the essential amino acid phenylalanine. Classic PKU is at one end of a spectrum of conditions known as hyperphenylalaninemia. Because rarer forms are a result of a deficiency in other enzymes and are diagnosed and treated differently, the following discussion of PKU is limited to the severe, classic form.

In PKU the hepatic enzyme phenylalanine hydroxylase, which normally controls the conversion of phenylalanine to tyrosine, is deficient. This results in the accumulation of phenylalanine in the bloodstream and urinary excretion of abnormal amounts of its metabolites, the phenyl acids (Fig. 9-20). One of these phenylketones, phenylacetic acid, gives urine the characteristic musty odor associated with the disease. Another is phenylpyruvic acid, which is responsible for the term phenylketonuria.

Tyrosine, the amino acid produced by the metabolism of phenylalanine, is absent in PKU. Tyrosine is needed to form the pigment melanin and the hormones epinephrine and thyroxine. Decreased melanin production results in similar phenotypes of most individuals with PKU: blond hair, blue eyes,and fair skin that is particularly susceptible to eczema and other dermatologic problems. Children with a genetically darker skin color may be red haired or brunette.

The prevalence of PKU varies widely in the United States due to the fact that different states have different definition criteria for what constitutes hyperphenylalaninemia and PKU. The reported figures for PKU range from 1 per 19,000 to 1 per 13,500 live births. The disease has a wide variation of incidence by ethnic groups. The disease is most prevalent among individuals of Northern European ancestry, as well as American Indians and Alaskan Natives, whereas African-American, Hispanic, Jewish, and Asian individuals account for the lowest frequencies (Hellekson, 2001). Among African Americans, for instance, the incidence of PKU is 1 per 50,000 live births (McPhee and Ganong, 2006).

Clinical manifestations in untreated PKU include failure to thrive (growth failure); frequent vomiting; irritability; hyperactivity; and unpredictable, erratic behavior. Mental retardation is thought to be caused by the accumulation of phenylalanine and presumably by decreased levels of the neurotransmitters dopamine and tryptophan, which affect the normal development of the brain and CNS, resulting in defective myelinization, cystic degeneration of the gray and white matter, and disturbances in cortical lamination. Older children commonly display bizarre or schizoid behavior patterns such as fright reactions, screaming episodes, head banging, arm biting, disorientation, failure to respond to strong stimuli, and catatonia-like positions.

GALACTOSEMIA

Galactosemia is a rare autosomal recessive disorder that results from various gene mutations leading to three distinct enzymatic deficiencies. The most common type of galactosemia (classic galactosemia) results from a deficiency of a hepatic enzyme, galactose 1-phosphate uridyltransferase (GALT), and affects approximately 1 of 50,000 births. The other two varieties of galactosemia involve deficiencies in the enzymes galactokinase (GALK) and galactose 4′-epimerase (GALE); these are extremely rare disorders. All three enzymes (GALT, GALK, and GALE) are involved in the conversion of galactose into glucose.

As galactose accumulates in the blood, several organs are affected. Hepatic dysfunction leads to cirrhosis, resulting in jaundice in the infant by the second week of life. The spleen subsequently becomes enlarged as a result of portal hypertension. Cataracts are usually recognizable by 1 or 2 months of age; cerebral damage, manifested by the symptoms of lethargy and hypotonia, is evident soon afterward. Infants with galactosemia appear normal at birth, but within a few days of ingesting milk (which has a high lactose content), they begin to experience vomiting and diarrhea, leading to weight loss. E. coli sepsis is also a common presenting clinical sign. Death during the first month of life is not infrequent in untreated infants. Occasionally classic galactosemia is seen with milder, chronic manifestations, such as failure to thrive, feeding difficulty, and developmental delay. This presentation is more frequent among African-American children with galactosemia (Kaye, Committee on Genetics, Accurso, and others, 2006).

PSYCHOLOGIC ASPECTS OF GENETIC DISEASE

The diagnosis of a genetic disorder in a child can be a life-altering experience for families. They may have to reassess their perception of “self” and the loss of the dream of the perfect baby. Parents may change educational, employment, and reproductive plans after the diagnosis of a genetic disorder in their child.

Families may need to have the genetic information repeated several times. Families may also encounter ethical or moral dilemmas regarding genetic evaluation and testing options, as well as potential involvement of other family members. Nurses are pivotal caregivers in assessing the family’s understanding of the genetic disorder, psychologic responses, and coping mechanisms. Nurses may help families by providing support and attempting to alleviate possible feelings of guilt, and by helping the family make the best possible adjustment to the disorder.

It is important to stress that there is nothing shameful about an inherited or congenital defect and to emphasize any appropriate remedy. The thought of a hereditary disorder often creates intrafamily strife, hostility, and marital disharmony, sometimes to the point of family disintegration. Relatives may change reproductive plans after the diagnosis of a genetic disorder in a member, or the decision to reproduce may be postponed indefinitely on the basis of a disorder in a relative, even a remote one. Although people may understand the information on an intellectual level, they may still harbor fears on an emotional level. Nurses can help the family identify their personal strengths and offer them information about local and national support groups. (The Genetic Alliance^*is a nonprofit organization that has a database of support groups for genetic conditions.) Finally, it is important to keep in mind that the infant or child has the same basic needs after the diagnosis of a genetic disorder as he or she had before the diagnosis.

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