Kernicterus threatens healthy newborns

Kernicterus is a condition of newborns that leads to severely disabling brain damage or death. It results from hyperbilirubinemia that can be caused by a number of factors. Kernicterus is preventable with techniques currently available. Nevertheless, in recent years cases of kernicterus have continued to be reported. One registry includes 90 cases in the United States from 1984 to the present in which three of the newborns died and all others sustained brain damage.1 "This is probably happening more than clinicians know about," says Sue Sheridan, spokesperson for the advocacy, educational and support group PICK, Parents of Infants and Children with Kernicterus. "With these recent cases, risk assessments were inadequate and unreliable, and bilirubin levels were not measured--or measured in time." PICK has been instrumental in drawing attention to the reemergence of kernicterus and its prevention.

Hyperbilirubinemia is characterized by jaundice, and while jaundice in

"This is probably happening more than clinicians know about. With these recent cases, risk assessments were inadequate and unreliable, and bilirubin levels were not measured--or measured in time." - Sue Sheridan

the newborn is common, extreme hyperbilirubinemia that causes kernicterus is rare. "To make sure that we do not miss these rare newborns, we need to follow and measure serum bilirubin levels on many newborns who will never go on to develop severe hyperbilirubinemia," says Dr. Jeffrey Maisels, pediatrics chairman at William Beaumont Hospital and chairman of the American Academy of Pediatrics' (AAP) subcommittee on neonatal hyperbilirubinemia. In order to identify these rare newborns, certain organization systems and processes should be in place. Dr. Phyllis A. Dennery and co-authors have provided a recent comprehensive review of neonatal hyperbilirubinemia.2

Risk factors for recent cases

The risk factors for severe hyperbilirubinemia are well known2, 3, 4, 5 and also appear to have played a role in the recent cases:

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

Root causes identified

Examination of the recent cases has identified a pattern of root causes related to four patient care processes: 

• 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. This is particularly important for infants less than 38 weeks of gestation.
  • Failure to provide early follow-up with physical assessment for infants who are 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.

Available risk reduction strategies

AAP Practice Guidelines for Management of Hyperbilirubinemia in the Healthy Term Newborn5, which are based on available data and expert consensus, provide guidelines for identifying at-risk newborns, their diagnosis and their treatment. These guidelines include specific recommendations to evaluate all cases of jaundice appearing in the first 24 hours, appropriate assessment of maternal and infant blood types, and detailed treatment strategies for specific levels of bilirubin at different ages. Other strategies to consider include:

• Predischarge bilirubin measurement with use of a percentile based nomogram to predict the risk of hyperbilirubinemia in newborns and to guide follow-up.3
• Policies and procedures or standing orders allowing nurses to order TSB (total serum bilirubin) or TcB (transcutaneous bilirubin) tests for jaundiced newborns, and to provide for proper documentation of bilirubin values and a report to the pediatrician.
• Policies for assessing the risk of severe hyperbilirubinemia in all infants by history, clinical evaluation and, if necessary, by laboratory measurement. (Because clinical assessment is imprecise, if there is any doubt about the presence or severity of jaundice, a serum or transcutaneous bilirubin measurement should be obtained.)
• Procedures for follow-up of all newborns within 24 to 48 hours by a physician or pediatric nurse. If this cannot be achieved, decisions regarding timing of discharge or other follow-up must be based on risk assessment.
• Policies and procedures on jaundice management that specifically cover the nurse's role, documentation, charting requirements, and monitoring of jaundice predischarge. PICK's medical advisory board recommends these policies also cover the ER and Newborn ICU, where newborns with dangerous levels of bilirubin may be brought after discharge.
• Provide parents with adequate educational materials about newborn infants that includes information about jaundice.
  Provide adequate equipment--bilirubin lights and blankets, and non-invasive TcB measurement device or lab services for timely TSB test.

Recommendations

The Joint Commission recommends that organizations (1) take steps to raise awareness among neonatal caregivers of the potential for kernicterus and its risk factors; (2) review their current patient care processes with regard to the identification and management of hyperbilirubinemia in newborns; and (3) identify strategies from the above list of available risk reduction strategies that could enhance the effectiveness of these processes.

References

1. Audrey K. Brown and Lois Johnson: Loss of Concern About Jaundice and the Reemergence of Kernicterus in Full-Term Infants in the Era of Managed Care, 1996 Yearbook of Neonatal and Perinatal Medicine; xvii-xxviii.
2. Phyllis A. Dennery, Daniel S. Seidman, and David K. Stevenson: Neonatal Hyperbilirubinemia, The New England Journal of Medicine, 2001; 344(8):581-590.
3. Vinod K. Bhutani, Lois Johnson, and Emidio M. Sivieri: Predictive Ability of a Predischarge Hour-specific Serum Bilirubin for Subsequent Significant Hyperbilirubinemia in Healthy Term and Near-term Newborns, Pediatrics, 1999; 103(1):6-14.
4. Thomas B. Newman, Blong Xiong, Veronica M. Gonzales, Gabriel J. Escobar: Prediction and Prevention of Extreme Hyperbilirubinemia in a Mature Health Maintenance Organization, Archives of Pediatrics and Adolescent Medicine, 2000; 154: 1140-1147.
5. American Academy of Pediatrics Practice Guidelines for Management of Hyperbilirubinemia in the Healthy Term Newborn: Pediatrics, 1994; 94(4):558-565. 

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