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Jaundice is a common clinical sign of hyperbilirubinemia in pediatrics, specifically newborns. Because hyperbilirubinemia of the newborn is so common, it is important for nurses to familiarize themselves with the physiology and the vital role nurses have in managing this condition. This course will review the pathophysiology and various types of hyperbilirubinemia in pediatrics, risk factors, screening, treatment, complications, and, most importantly, the nurses’ role in providing care to patients with the disorder. 

Introduction

Hyperbilirubinemia is a health condition in pediatrics occurs in approximately 60-80% of newborns (1). Hyperbilirubinemia occurs when there is a large amount of bilirubin present in the blood. Many newborns develop a total serum bilirubin (TSB) level >1 mg/dL, which is the upper limit of normal for adults (1). The rapid accumulation of bilirubin in the blood can cause yellowing of newborns’ skin and eyes, as also known as  jaundice (1).

Often, within the healthcare setting, hyperbilirubinemia in pediatrics and jaundice are used interchangeably, although they do not have the same meaning. However, where there is a presence of hyperbilirubinemia in pediatrics, it is likely that there is a presence of jaundice. Because hyperbilirubinemia of the newborn is so common, it is important for nurses to familiarize themselves with the physiology of the condition and the vital role nurses have in managing hyperbilirubinemia in pediatric patients (1). 

This course will review the pathophysiology and various types of hyperbilirubinemia in pediatrics as well as risk factors, how to screen for the disorder, treatment, complications, and, most importantly, the nurses’ role in caring for those with hyperbilirubinemia.  

What is Hyperbilirubinemia?

Hyperbilirubinemia is a health condition that occurs when there is too much bilirubin present in the blood. Simply put, when hyperbilirubinemia occurs in the newborn there is either increased production of bilirubin or decreased clearance of bilirubin (1). When newborns produce unconjugated hyperbilirubinemia (UHB), this is because of physiologic or pathologic causes (1).

Most cases of neonatal jaundice are physiologic in nature. Physiologic jaundice is the most common form of neonatal jaundice, occurring in approximately 75% of newborns. Physiologic jaundice occurs because of differences in the metabolism of bilirubin in the neonatal period leading to an increased bilirubin level (1). 

Bilirubin is a product of heme catabolism/heme degradation (1). Heme is a breakdown product of hemoglobin, and hemoglobin is an essential protein in red blood cells (RBCs) that carry oxygen. Since bilirubin is a result of the catabolism of heme in the reticuloendothelial system (RES), bilirubin is an essential marker of liver, blood, and skin health in the body, especially neonates (1).

It is estimated that approximately 80% of bilirubin is produced during the breakdown of hemoglobin from RBCs or from ineffective erythropoiesis (2). Because of the differences in bilirubin metabolism and other metabolic functions in the neonatal period, bilirubin levels increase in the newborn (1). This increase is because of many red blood cells with a shorter lifespan in circulation in the neonate; this is also known as ineffective erythropoiesis (2). 

Types of Hyperbilirubinemia

Physiologic Jaundice

is the most common form of hyperbilirubinemia in pediatrics and usually occurs in newborns between 24-72 hours of age (1). It is important to note that physiological jaundice never occurs within the first 24 hours of life and accounts for 75% of neonatal hyperbilirubinemia. Physiologic jaundice is typically mild in nature and easily resolves with treatment (1). Physiological jaundice occurs because of differences in the metabolism of bilirubin in the neonatal period leading to an increased bilirubin level. The increase in bilirubin is because of a high number of red blood cells (RBCs) (polycythemia) with a shorter lifespan in circulation; this is also known as immature erythropoiesis (1). Newborns have immature livers, resulting in the body not getting rid of bilirubin quickly enough, leading to an excess of bilirubin.

Pathologic Jaundice

Occurs within the first 24 hours of birth (1). Pathological jaundice is characterized by jaundice within the first 24 hours of birth, a rapidly increasing total serum bilirubin level (increasing by 5 mg/dL per day or more than 0.2 mg/dL/hr), or jaundice persists beyond two to three weeks in full-term infants  (1). 

Pathologic jaundice is often influenced by underlying conditions, such as (3): 

• Sepsis  
• TORCH (toxoplasmosis, other viruses, rubella, cytomegalovirus, herpes (simplex) viruses) 
• Isoimmune-mediated hemolysis (ABO or rH incompatibility)  
• Inherited red blood cell membrane defects (eg, hereditary spherocytosis and elliptocytosis) 
• Erythrocyte enzymatic defects (eg, glucose-6-phosphate dehydrogenase [G6PD] deficiency [20], pyruvate kinase deficiency, and congenital erythropoietic porphyria) 
• Macrosomic infants of diabetic mothers (IDM (3)] 

In cases of suspected pathologic jaundice, evaluation and assessment for dehydration, skin changes like petechiae and pallor, increased bruises, weight changes, liver function, spleen function, sleep changes, or seizures is essential to determine any co-morbid conditions or further complications or pathologic jaundice (1).  

Breastmilk jaundice is a specific type of jaundice that occurs in infants who are exclusively breastfed. This type of jaundice usually occurs between 24-72 hours of age, peaks within two weeks, and typically declines between the third and 12th week of life (4). Breastmilk jaundice differs from breastfeeding/lactation failure jaundice. Newborns who exclusively receive breastmilk typically have serum bilirubin levels of >5 mg/dL that continue for weeks (4). This form of hyperbilirubinemia is typically mild in nature and does not require medical intervention. The underlying cause of breastmilk jaundice remains unknown (4).

Lactation failure jaundice, also known as chestfeeding or breastfeeding jaundice, typically occurs within the first week of life (1). This type of jaundice occurs as a result of inadequate caloric and fluid intake in the initial days of life, leading to dehydration and inability to pass meconium in the newborn (1). This is often caused by poor latch, or poor breastmilk production, leading to an inadequate transfer of breastmilk. In newborns experiencing breastfeeding jaundice, it is encouraged to increase the frequency of feedings. It is not necessary to supplement with formula unless there is continued poor weight gain and failure to stool.

Risk Factors

There are many risk factors to consider for hyperbilirubinemia in pediatrics, including newborn and maternal (1).  

Neonatal factors include: 

• Prematurity 
• Male gender 
• Polycythemia 
• Birth trauma that causes significant bruising (such as cephalohematomas)
  

Infants with risk factors should be closely monitored for signs of hyperbilirubinemia during the first couple of weeks of life. At about 14 days of age, bilirubin levels begin to stabilize and reflect normal levels (1). 

Maternal factors include: 

• Blood type (ABO or RH incompatibility) 
• Breastfeeding 
• Maternal health conditions, specifically, gestational diabetes 
• Ethnicity (Southeast Asian, East Asian, Native American) 

Screening for Hyperbilirubinemia

Measuring the total serum or plasma bilirubin (TSB) is considered to be the gold standard for neonatal bilirubin testing (1). Transcutaneous bilirubin (TcB) is also a common and reasonable alternative (1). 

TSB values are obtained by collecting a blood sample from the infant’s tissue, typically from the their heel. This is known as a capillary stick. Following the blood sample collection, it is sent to the lab, where it is processed. The results are then compared among normal values for age to determine whether values are abnormal or within normal limits; this is also referred to as the Bhutani Nomogram (5). 

TcB devices are used on the skin’s surface to estimate TSB levels (5). TcB testing decreases the need for blood sampling and is similarly as effective as TSB testing. In some instances, TcB measurements may not accurately estimate TSB levels.  

TSB levels should be obtained if (1,5): 

• Infant is undergoing phototherapy 
• Infant has had prior exposure to sunlight or phototherapy in the last 24 hours 
• TcB exceeds the 75th percentile on the nomogram for phototherapy 
• TcB is within 3 mg/dL of the phototherapy threshold levels 
• Previously high TSB levels (>15 mg/dL)
  

Special considerations: 

• At high TB levels (>15 mg/dL), it is common for TcB to underestimate TSB levels. 
• TcB testing can reflect an overestimation of TSB levels in infants with darker skin tones and an underestimation of TSB levels in lighter skin tones. 
• If confirmatory TB testing is indicated, therapeutic intervention should be initiated while waiting for results under the orders of a health care provider(5). 

Treatment 

Treating hyperbilirubinemia in the newborn includes the following interventions (1,2): 

• Adequate nutrition 
• Phototherapy 
• Exchange transfusion
  

Phototherapy is the most common intervention used to treat jaundice and hyperbilirubinemia of the newborn. Phototherapy is the use of a light of specific wavelengths and doses to change unconjugated bilirubin in the skin into lumirubin, which can be excreted through bile and urine (1). Lumirubin is a photoproduct of bilirubin that is produced in infants undergoing phototherapy (1). The blue-green LED light has a wavelength of 460-490 nm and is the preferred light source for phototherapy to treat neonatal hyperbilirubinemia (1). The key benefit to phototherapy is reducing TSB levels and preventing TSB levels from rising to a level in which an exchange transfusion is indicated. Effective phototherapy results in a decrease of TSB levels of at least 2-3 mg/dL within the initial four to six hours of initiation (1,5). Health care providers are responsible for ordering the appropriate phototherapy dose. 

Nurses should be mindful of ways to maximize the benefits of phototherapy (1). It is important to: 

• Place infant supine. 
• Maximize skin exposure—size down on diaper 
• Cover infant’s eyes with an eye shield. 
• Monitor the infant during therapy.  
• If applicable, time spent off phototherapy for feeds. Limit to 30 minutes or less.
  

In infants with hyperbilirubinemia, it is vital to promote nutrition and hydration. This is especially important for infants undergoing phototherapy. Promoting adequate nutrition and hydration will increase urinary output, leading to an increase in the excretion of lumirubin. Lumirubin is a photoproduct of bilirubin that is produced in infants undergoing phototherapy (1). It is important to ensure that during phototherapy, infants continue feedings by breast or bottle. IV hydration is indicated in infants with dehydration, fluid imbalances, or when oral intake is inadequate (1). 

Because of the risk for excessive fluid loss, nurses should closely monitor: 

• Dose of phototherapy (irradiance) 
• Infant’s temperature 
• Hydration status (Intake and output) 
• Time of exposure 
• Daily TSB levels (1,2,5)
  

An exchange transfusion (ET) is a high-risk procedure that involves removing and replacing blood at a slow rate to lower the presence of bilirubin rapidly (1). Exchange transfusions are rarely indicated because of favorable outcomes with intensive phototherapy. However, exchange transfusions are indicated to treat symptomatic infants with clinical signs of moderate to advanced signs of severe unconjugated hyperbilirubinemia or and when intensive phototherapy is ineffective (1). 

Exchange transfusions are an inpatient procedure and should be performed by trained personnel within an intensive care unit (ICU) setting where full monitoring and resuscitation equipment is available. 

Caring for Newborns with Hyperbilirubinemia

The nurse’s role in caring for a newborn with hyperbilirubinemia is focused on assessing and managing the newborn and educating the parents. As a nurse caring for a newborn with hyperbilirubinemia, it is important to closely monitor feeding volumes and frequency to ensure adequate nutritional status. It is also important to teach parents about the importance of oral feedings and the role proper hydration plays in ridding the body of bilirubin. Nurses should encourage parents to feed at minimum every 3-4 hours. In infants undergoing phototherapy, it is important to encourage parents to limit feedings to no more than 30 minutes at a time in effort to maximize infants’ time under the phototherapy light (1,2,5).  

In addition to closely monitoring feedings, it is important for the nurse to closely monitor urine and stool output. Depending on the setting, diaper weights may be indicated, but counting the number of wet diapers suffices. This is important to ensure that the infant is properly excreting waste and hydrating.  

Complications of Severe Hyperbilirubinemia

Severe hyperbilirubinemia in pediatrics is defined as a TSB > 25 mg/dL (1). Severe hyperbilirubinemia is associated with an increased risk for developing bilirubin-induced neurologic dysfunction (BIND) and is associated with significant infant morbidity and mortality (6). BIND occurs when bilirubin crosses the blood-brain barrier binding to brain tissue, which can result in brain injury if not quickly treated. To prevent or intervene before the development of severe hyperbilirubinemia, it is important to assess for the presence of risk factors that increase the risk of developing BIND (1,6). 

These factors include: 

• Isoimmune hemolytic disease 
• Lethargy 
• Asphyxia 
• Temperature instability 
• Sepsis 
• High altitude 
• Hypoalbuminemia 
• Southeast Asian or East Asian ethnicity 
• Inadequate fluid intake with excessive weight loss (1) 

Based on the assessed risk for severe hyperbilirubinemia, either phototherapy or exchange transfusion may be indicated. Typically, once severe hyperbilirubinemia develops, it is treated by exchange transfusion (1,7). 

Case Study

Baby Smith is a 4-day old infant born to a 28-year-old East Asian mom. She reports that this was her first pregnancy and first biological child. Mom brings him to his first newborn visit at a pediatric office. She is concerned because his “eyes and skin are yellow,” and she said he has not had a bowel movement since day 1 of life. She reports that he does not have a fever or trouble eating or sleeping.  

The history on baby Smith: 

• He was born at 38 weeks via vacuum-assisted vaginal delivery.  
• He developed a caput succedaneum as a result of vacuum-assistance during delivery, but otherwise experienced no complications.  
• He received formula during his first day of life, but is now exclusively breastfeeding and is not receiving supplemental formula.  
• He has had four wet diapers in the initial 24-hours of life and passed stool a couple of hours after birth, but has not had a stool since. 
• At 27 hours of age, his TSB level is 5.9 mg/dL placing him in the low-risk zone on the Bhutani nonogram. He was discharged from the hospital at 36 hours of age with mom. 
• Mom of infant reports that since discharge, she has been exclusively breastfeeding every 4-5 hours and reports infant has three wet diapers a day.
  

Assessment: 

Baby Smith presents in the pediatric office visibly jaundiced with the presence of conjunctival icterus (yellow sclera). A TcB is obtained and measures at 13.5 mg/dL. A TSB is obtained and measures at 15.1 mg/dL. 

Based on the information provided by the mom and assessment of the infant, the health care provider determines that the infant has lactation failure jaundice and is also at increased risk for hyperbilirubinemia due to the presence of caput succedaneum. 

Since being discharged, the infant has been exclusively breastfed. Previously, in the hospital, mom was supplementing formula because this infant was adequately hydrated and urinated, and stooled appropriately. Following being sent home, the infant began to have fewer wet diapers and no stooling episodes. This is indicative of inadequate fluid and caloric intake, likely because of issues with lactation. 

Plan:  

The provider orders at home phototherapy with a fiberoptic blanket. Because of failure to stool and increasing TSB level, the provider encourages mom to supplement with formula along with her breastmilk. It is important to encourage mom to feed every 2-3 hours and to continue to count wet and soiled diapers. 

A home health nurse will come to her home to teach her how to use the fiberoptic blanket. The nurse will also return to the home daily to monitor the child’s weight, oral intake, urine and stool output, skin appearance, and bilirubin level. 

This therapy will continue for three days. The provider would like to see baby Smith back in the office on day 3. 

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