Purpose: This activity is designed to increase the knowledge level and therefore the quality of care for infants at risk for hyperbilirubinemia.

Objectives:

1. Recall pathophysiology of hyperbilirubinemia in the newborn
2. List the risk factors currently recognized with positive predictive value for hyperbilirubinemia.
3. Identify the process of evaluation of risk for hyperbilirubinemia in the term newborn

Kernicterus in the newborn:  $10,000,000 settlement against nurses and hospital.

Let's review the facts.

Plaintiffs in this case are plaintiff son Nathaniel, a newborn male infant now age four, plaintiff mother Jodie, a 38-year-old homemaker, and plaintiff father Douglas, a 37-year-old attorney. Plaintiff son was born at SJMC in Tacoma (owned by defendant). He was essentially a healthy newborn and was discharged the following day. He developed jaundice over the next several days. There were a variety of contacts between the parents, doctors and nurses.

On May 20, 1997, plaintiff mother called defendant lactation consultant for advice on what she believed was a breast-feeding problem, as the infant was not feeding well. The lactation nurse who took the telephone call recorded that the baby was "really jaundiced, lethargic, and fading fast." The nurse did not call the doctor or advise the mother about what she admitted at trial was a potential medical emergency. Instead, the nurse testified that she told plaintiff mother to call the doctor and report the symptoms, and arranged for a lactation consultation with another of defendant's lactation nurses, which took place in Federal Way, Washington (also owned by defendant).

This second nurse testified that this was the most jaundiced baby she had ever seen. She also knew this was a potential medical emergency.  However, the nurse did not direct the parents to the emergency room (one floor below), and did not call the baby's doctor to report her assessment or concerns. Nor did the nurse tell the parents that their baby was in jeopardy.  The nurse assumed that the mother was going to follow up with the pediatrician later that day. The nurse did not plan to see the pediatrician until the following day.

A routine bilirubin blood test (TSB) done earlier that morning (for review by the pediatrician the following day) was reported by page to the pediatrician as 29.2 mg/dL (extremely high). The pediatrician suspected laboratory error because the baby's clinical picture seemed inconsistent with the laboratory result. The pediatrician had not been advised of the lactation nurse's assessment and therefore arranged for a home health nurse to go out to the baby's home and get another blood draw and start phototherapy. 

The second blood result was about 32 mg/dL. The doctor told the parents to take the baby to MB Children's Hospital emergency department in Tacoma. After they arrived and while the arrangements were being made for an exchange of blood transfusion, the baby suffered severe brain injury.

Plaintiffs alleged that the nurses were negligent in failing to call the baby's doctor failing to direct the parents and baby to the emergency room; failing to tell the parents that their baby was in jeopardy; and failing to properly respond to what they knew was a potential medical emergency. In addition, plaintiffs alleged the hospital was corporately negligent in failing to develop and implement policies and procedures for handling newborn jaundice. Finally, plaintiffs alleged that under these extraordinary circumstances, the hospital failed to obtain informed consent.

Defendant denied liability, asserting that its nurses met the standard of care as they told the parents to call or see the pediatrician. Defendant denied any obligation to call the pediatrician directly or to send the baby to the emergency room. It asserted the pediatrician, not the emergency room doctor, was the proper person to handle this problem and that it would have made no difference if the pediatrician or emergency room doctor had been called because nothing would have been done until the blood tests came back. Plaintiffs called pediatric experts who testified the delay in communication and treatment caused the brain injury. Defendant also alleged the parents were contributorily negligent for not following advice to call or see the pediatrician.

Nathaniel sustained kernicterus with athetoid cerebral palsy and severe motor impairment. He is believed to be cognitively normal. He also has a non-related kidney disorder that will necessitate transplant (congenital nephrotic syndrome). The defense initially argued that this condition was a contributing factor to kernicterus but abandoned this theory at trial.

Oregon Litigation and Arbitration Reports: The jury, after four days of deliberation, found that the hospital was negligent. It awarded damages of over $10,000,000 to the minor boy, and $665,000 to the parents. As to the parents'; contributory negligence, the jury found the hospital to be 80% at fault and the parents to be 20% at fault (for a net verdict of $532,000 in favor of the parents).

What were the breakdowns in process here? Does our culture, here at Mother Frances, leave us at risk for just such a case? Perhaps not, but we need to review.

What do we know about kernicterus?

Kernicterus, a serious - but preventable - neurological syndrome, can strike otherwise healthy newborns with jaundice or high bilirubin concentrations. It is a lifelong brain syndrome that can include cerebral palsy, mental retardation, and hearing loss, caused by severe and untreated elevations of the bilirubin in the newborn.

Many newborns develop jaundice and never have a problem.  Why the sudden concern?

Neonatal jaundice affects over half of all newborns in the US. It usually appears within the first few days of life and may resolve on its own.  Without treatment, some infants develop very high levels of bilirubin, which may lead to brain damage or hearing impairment. Kernicterus had become a rare occurrence in the US when babies stayed in the hospital for several days. The jaundice, usually visible within 72 hours, was identified and treated while the infant was still an inpatient. Because most healthy newborns are now released from the hospital within no more that 48 hours of birth, and may not be closely followed, jaundice may go unnoticed. In recent years, complications of jaundice have reappeared. Better monitoring, parent education and follow-up are vital.

What occurs when an infant becomes jaundiced?

The breakdown of a red blood cell (RBC) releases hemoglobin and produces bilirubin. The rate of bilirubin production in the newborn is two to three times that of an adult. There are several causes.

1. The fetus has more RBC's per KG of body weight, due to the environment of reduced oxygen supply. At birth, respiration is established in an oxygen-rich environment and the surplus of RBC's is no longer needed. They are then metabolized.
2. Neonatal RBC's have an average life span of 70-90 days compared to that of an adult 120 days.
3. Heme, an essential molecule in oxygen-dependent metabolism and a potential source of bilirubin, is increased in the neonate.
4. Enterohepatic shunting is often increased in the neonate.

The newborn gut lacks bacterial floras needed to convert bilirubin to a form that cannot be reabsorbed and thus excreted. The newborn bowel contains 10X the adult concentration of the intestinal enzyme beta-glucuronidase, which readily hydrolyzes conjugated back into the unconjugated form. The unconjugated form is absorbed across the intestinal mucosa and returns to the liver via the enterohepatic circulation.

You can see this repeats the whole process and adds to the bili level.

 How does the bilirubin end up in the skin and sclera... even worse the brain?

When old erythrocytes (RBC's) are removed from the circulation they break down into heme, globin and iron. Heme is converted to biliverdin, which is transformed rapidly into the unconjugated form of bilirubin and released into the plasma (Halamek & Stevenson, 1997).

Unconjugated bilirubin is bound to albumin in the plasma until there are insufficient sites or unless the bilirubin has decreased affinity for the albumin.  Unbound bilirubin has potential for movement into tissues (skin, sclera, across the blood-brain barrier).

Unconjugated bilirubin cannot be excreted.  Conjugation is accomplished in the liver with the aid of hepatic ligands, which aid in transport from the albumin across the cell membrane. As luck would have it, hepatic ligands are decreased in the neonate and do not reach adult levels until about 5 days of life.  Conjugation also requires oxygen and glucose - no wonder that we see higher bili levels in newborns that have trouble maintaining oxygen and glucose levels.  Once conjugation occurs the bilirubin is water soluble and excreted into the intestine in the stool as stercobilin, a small amount will be excreted as urobilinogen by the kidneys.  When any part of this process suffers we develop the potential for higher and higher serum bilirubin.

Are there factors that predispose an infant to Hyperbilirubinemia/kernicterus?

The risk factors are well known and appear to have played a role in the recent cases. 

1. Jaundice appearing in the first 24 hours after birth
2. Inadequate nutrition/hydration through suboptimal breast-feeding
3. Near-term newborns at 35, 36, and 37 weeks of gestation, particularly if they are breast-fed.
4. Bruising and cephalohematomas (increase the production of bilirubin)
5. Unrecognized hemolysis, such as ABO incompatibility
6. Glucose-6-phosphate dehydrogenase (G6PD) deficiency
7. Genetic or ethnic risk factors include siblings with jaundice, East-Asian or Mediterranean descent.

PRESTO!!!  The root causes have been identified, also.

• Patient assessment

• The unreliability of the visual assessment of jaundice in newborns with dark skin
• Failure to recognize jaundice in an infant - or its severity-based on visual assessment, and measure a bilirubin level before the infant's discharge from the hospital or during a follow-up visit.
• Failure to measure the bilirubin level in an infant who is jaundiced in the first 24 hours.

• Continuum of care

• Early discharge (before 48 hours) with no follow-up within one to two days of discharge. Particularly important for infants less than 38 weeks gestation.
• Failure to provide early follow-up with physical assessment for infants who were jaundiced before discharge.
• Failure to provide ongoing lactation support to ensure adequacy of intake for breast-fed newborns.

• Patient and family education

• Failure to provide appropriate information to parents about jaundice and failure to respond appropriately to parental concerns about a jaundiced newborn, poor feeding, lactation difficulties and change in newborn behavior and activity.

• Treatment

• Failure to recognize, address or treat rapidly rising bilirubin.
• Failure to aggressively treat severe Hyperbilirubinemia in a timely manner with intensive phototherapy or exchange transfusion.

 How can we detect pathologic levels of bilirubin?  When does jaundice become serious?

Do you want the AWHONN version or the American Academy of Pediatrics version?  Actually they agree and AAP goes (as you can imagine) a step further.

1. Maternal prenatal testing should include ABO and Rh(D) typing and a serum screen for isoimmune antibodies.
2. A direct Coombs' test, a blood type, and an Rh(D) type on the infant's cord blood are recommended.
3. Institutions are encouraged to save cord blood for future testing, particularly when the mother's blood type is group O (most common for ABO incompatibility).
4. When family history, ethnic or geographic origin, or the timing of the appearance of jaundice suggests the possibility of glucose-6-phosphate dehydrogenase deficiency or some other cause of hemolytic disease, appropriate laboratory assessment of the infant should be performed.
5. A TSB level needs to be determined in infants noted to be jaundiced in the first 24 hours of life.
6. In newborn infants, jaundice can be detected by blanching the skin with digital pressure, revealing the underlying color of the skin and subcutaneous tissue. The clinical assessment of jaundice must be done in a well-lighted room. (If you can't picture this and have never done it, seek a demo from a "nursery" nurse.) Dermal icterus is seen first in the face and progresses caudally to the trunk and extremities. As the TSB level rises, the extent of cephalocaudad progression may be helpful in quantifying the degree of jaundice; use of an icterometer or transcutaneous jaundice meter may also be helpful. Special care must be taken in evaluation of dark-skinned infants as the jaundice may be masked by skin color. Observe the sclera carefully and watch for behavior changes.
7. Evaluation of newborn infants who develop abnormal signs such as feeding difficulty, behavior changes (irritable, fussy, lethargic), apnea, or temperature instability is recommended - regardless of whether jaundice has been detected - to rule out underlying illness.
8. Follow-up should be provided to all neonates discharged less than 48 hours after birth by a health care professional in an office, clinic, or at home within 2 to 3 days of discharge
9. Approximately one third of healthy breast-fed infants have persistent jaundice after 2 weeks of age. A report of dark urine or light stools should prompt a measurement of direct serum bilirubin. If the history (particularly the appearance of the urine and stool) and physical examination results are normal, continued observation is appropriate. If jaundice persists beyond 3 weeks, a urine sample should be tested for bilirubin, and a measurement of total and direct serum bilirubin obtained.

Can breast-feeding continue?

When a mother nurses a newborn, it takes at least 2-3 days for the breast to start making enough milk. If the baby goes home at 36 hours and is not nursing well, there is a chance that the baby will not get adequate nutrition. When this happens, jaundice is much more likely to occur.
In every study of jaundiced infants, most cases of severe jaundice have occurred in breast fed infants. However, it is VERY IMPORTANT TO EMPHASIZE THAT ALMOST ALL OF THESE CASES HAVE OCCURRED IN INFANTS IN WHOM BREAST-FEEDING HAS BEEN INADEQUATELY OR POORLY MANAGED. THIS IS ABSOLUTELY NOT A REASON TO DISCOURAGE BREAST FEEDING. BREAST FEEDING IS, WITHOUT QUESTION, THE BEST WAY TO FEED A BABY.
(AAP, 2002)
To prevent jaundice, strategies to promote early effective breastfeeding are important. Breastfeeding should be started early-at birth or soon after - and separation of the newborn and mother avoided barring maternal or neonatal complications. The AAP (1997) recommends early and frequent breastfeeding without water, other fluids or glucose supplementation unless medically indicated. Early frequent feedings can ensure an adequate volume of colostrum and milk in the intestine to encourage peristalsis and passage of meconium and provide adequate caloric needs (Sater, 1995).

The AAP discourages the interruption of breast-feeding in healthy term newborns and encourages continued and frequent breast-feeding (at least eight to ten times every 24 hours). Supplementing nursing with water or dextrose water does not lower the bilirubin level in jaundiced, healthy, breast-feeding infants. Depending on the mother's preference and the physician's judgment, however, a variety of options are presented for possible implementation beyond observation, including supplementation of breast-feeding with formula or the temporary interruption of breast-feeding and substitution with formula, either of which can be accompanied by phototherapy.

Treatment Options for Jaundiced Breast-fed Infants

What treatment can we expect?

1. Infants <24 hours old are excluded, because jaundice occurring before age 24 hours is generally considered "pathologic" and requires further evaluation. Although some healthy infants appear slightly jaundiced by 24 hours, the presence of jaundice before 24 hours requires (at least) a serum bilirubin measurement and, if indicated, further evaluation for possible hemolytic disease or other diagnoses. Phototherapy and/or exchange transfusion may be indicated for rapidly rising TSB levels in the first 24 hours of life.
2. For the treatment of 25-48 hour old infant, phototherapy may be considered when TSB level is >12 mg/dL. Phototherapy should be implemented when TSB level is >15mg/dL. If intensive phototherapy fails to lower a TSB level of >20mg/dL, exchange transfusion is recommended. If the TSB level is >25mg/dL when the infant is first seen, intensive phototherapy is recommended while preparations are made for an exchange transfusion. If intensive phototherapy fails to lower the TSB level, exchange transfusion is recommended. The higher TSB levels in a 25-48 hour old infant suggest that the infant may not be healthy and indicate the need for investigation into the cause of hyperbilirubinemia, such as hemolytic disease.
3. Phototherapy may be considered for the 49-72 hour old jaundiced infant when the TSB level is >15mg/dL. Phototherapy is recommended when the TSB level reaches 18mg/dL. If intensive phototherapy fails to lower the TSB level when it reaches or is predicted to reach 25 mg/dL, an exchange transfusion is recommended. If the TSB level is >30 mg/dL when the infant is first seen, intensive phototherapy is recommended while preparations are made for exchange transfusion. If intensive phototherapy fails to lower the TSB level, and exchange transfusion is recommended.
4. For the infant >72 hours old, phototherapy may be considered if the TSB level reaches 17 mg/dL. Phototherapy needs to be implemented at a TSB level of >20mg/dL. If intensive phototherapy fails to lower a TSB level of >25mg/dL, exchange transfusion is recommended. If the TSB level is >30mg/dL when the infant is first seen, intensive phototherapy is recommended while preparations are made for an exchange transfusion. If intensive phototherapy fails to lower the TSB level, an exchange transfusion is recommended.

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AAP algorithm

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Management of Hyperbilirubinemia in the Healthy Term Newborn

We discharge early. Is it up to the parents to follow up?
The most important outcome of parental education is ensuring that the parent understands the importance of and adherence with the infant's follow-up care plan. Lack of follow up has been one of the greatest challenges faced by practioners following the AAP Practice Parameter guidelines for early discharge (Brown et al., 1999; Seidman et al., 1995). Health care providers should remember that 10% or more of parents may not seek follow-up care for their infants at the recommended time even when they have agreed to do so or have a scheduled appointment for their child (Seidman et al., 1995).
Breastfeeding should be evaluated carefully before discharge by a professional qualified to assess latch, suck and the mother's perception of feeding. Mothers should voice their understanding of and agreement with the definition of "frequent" feedings. 
Parents should be instructed to recognize a difference in the infant's color appearance, behavior or feeding and to seek professional care if concerns arise. Understand that it is difficult for parents to recognize jaundice and relying on their observation for jaundice cannot be considered adequate follow-up.

Conclusion

In light of the serious nature of this condition the nurse cannot over assess. The key to risk reduction is:

1. Assess early and often. Report any jaundice or abnormal behavior to the provider.
2. Educate parents and family to the signs and symptoms of hyperbilirubinemia. Stress the importance of follow-up, following the recommended appointment schedule.
3. Assess the latch and suck of breast-feeding infants. Learn the signs of a good latch and suck/swallow indicators.
4. Document, Document. Documentation of assessments, teaching, consultations with the provider and lactation consultant is defensive documentation.
5. Review your unit policy for hyperbilirubinemia and bilirubin protocol. Notify the provider of any abnormal values and findings.
6. Many hospitals are taking steps to develop a process for thorough screening of all infants for risk of hyperbilirubinemia prior to discharge

This nomogram is useful to decide which babies may need more follow-up and can be used with TcB or TSB.

The following guideline is useful in the determination of which infants should receive phototherapy. Following the graph is an example of the clinical pathway recommended by the American Academy of Pediatrics.

Resources

Clinical Practice Guideline. (2004). Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 114, (1). 

Holcomb, S. S. (2005). Managing jaundice in full-term infants. The Nurse Practitioner 30, (1).

Joint Commission on Accreditation of Healthcare Organizations.
(2001). Kernicterus threatens healthy newborns.  Sentinel Event Alert, 18.

NSO September 2002 Legal Case Study. Failure to timely diagnose and treat jaundice-kernicterus brain injury with athetoid cerebral palsy and severe motor impairment of a newborn boy. Accessed at: http://www.nso.com 12 November 2002.

Reiser, D. (2001). Hyperbilirubinemia: identification and management in healthy term and near term newborns. Association of Women's Health, Obstetric and Neonatal Nurses.