Originally published June 2016
Severe hyperbilirubinemia — defined as a total serum bilirubin of greater than 340 µmol/L in the first 28 days of life — can lead to serious complications such as encephalopathy and kernicterus. Despite the condition being largely preventable with the proper testing and diagnosis, infants today continue to suffer debilitating complications.1,2
Almost 10 years ago clinical practice guidelines were published to assist healthcare providers and hospitals manage the condition and lower the number of incidents.3 And, while overall numbers have fallen since that publication,4 potentially preventable cases are still occurring.
In an analysis of its medical-legal files involving jaundice in newborns, the CMPA has identified two recurring risk themes and, based on the peer experts’ opinions, has also identified actions hospitals and providers can take to reduce these risks to susceptible newborns.
Case example: The baby at risk for hyperbilirubinemia
A woman prepares to leave the hospital with her newborn after a two-day stay post-delivery. The baby, born late pre-term, appears healthy with mild jaundice and a bilirubin level of 168 µmol/L at 42 hours. The mother is a glucose-6-phosphate dehydrogenase (G6PD) deficiency carrier and the baby’s brother has the condition. At discharge, the pediatrician advises the mother to watch for increased jaundice and signs that the baby is unwell.
Two days later the mother brings her baby to a follow-up appointment. Her regular physician is away, so she sees a second pediatrician who is covering for her regular physician. The second pediatrician notes that the baby is slightly jaundiced, but that the mother reports the infant is breastfeeding well and voiding adequately. He instructs the mother to seek medical care if the jaundice worsens and to follow-up with her regular pediatrician.
Three days later, the mother is concerned that her baby is lethargic and not feeding well. She takes him to her regular pediatrician. The physician remarks that the baby is very jaundiced and orders a serum biluribin test. The level is over 500 µmol/L. He immediately admits the baby to the hospital for phototherapy and IV fluids, and arranges for transfer to a tertiary care hospital for exchange transfusion. The infant is diagnosed with severe hyperbilirubinemia with related encephalopathy. Screening reveals G6PD deficiency and an MRI is consistent with kernicterus. Despite aggressive treatment, the child is left with profound hearing loss and cerebral palsy.
The CMPA pays a settlement in response to a legal action, on behalf of the second pediatrician. Experts are critical of this doctor for failing to order a serum bilirubin test at the initial follow-up appointment; knowing the baby’s bilirubin level would have been helpful in the management of his jaundice. Furthermore, a peer expert states that the baby’s risk factors, namely late preterm birth and family history of G6PD deficiency, required that he be followed very closely for a potential increase in serum bilirubin.
The state of hyperbilirubinemia care today
Hyperbilirubinemia (jaundice) is common in newborns and usually does no harm. But failure to identify and monitor severe hyperbilirubinemia ranks in the top 15 risks in healthcare based on total claims costs compiled by the Healthcare Insurance Reciprocal of Canada (HIROC).1 Canada has previously reported the highest incidence of severe hyperbilirubinemia (1 in 2480 live births) in the developed world.2
In 2007, the Canadian Paediatric Society (CPS) published guidelines for the detection, management, and prevention of hyperbilirubinemia.3 However, not all Canadian hospitals or physicians have adopted them. By 2012, about 79% of Ontario hospitals reported having implemented the guidelines.5 And a national survey found that only about 75% of pediatricians, 69% of midwives, and 40% of family physicians were using them.6
During the years from 2000–2014, the CMPA identified 16 medical-legal cases (11 legal actions and 5 complaints to a regulatory authority [College]) involving hyperbilirubinemia or kernicterus. While recent surveillance data demonstrate a decrease in the minimum estimated incidence of severe neonatal hyperbilirubinemia,4 six of these cases occurred after publication of the national guidelines. Ten cases had major or catastrophic injuries, such as severe hearing loss and cerebral palsy. Six cases involved peer expert criticism of a physician’s clinical care.
Themes found in CMPA files point to either a poor understanding of newborn jaundice or poor adherence to the published guidelines. These included:
- failure to consider patient risk factors, including prematurity, Rh incompatibility, ethnicity, and G6PD carrier status
- inadequate monitoring or follow-up of bilirubin levels, including inadequate discharge instructions for follow-up blood work
- delay in transfer of the patient to a tertiary care hospital for treatment or assessment by a specialist
- delay in treating the patient with phototherapy or an exchange transfusion
- early discharge of a premature infant
Some of these themes were highlighted in a report on the U.S.A. Kernicterus Registry, which also found that a lack of understanding of the potential neurotoxicity from hyperbilirubinemia contributed to cases of kernicterus.7 Additionally, several system issues were identified such as multiple providers providing services at multiple sites; early discharge (<72 h of age) associated with lack of pre-discharge screening and/or failure to have the bilirubin level checked post-discharge; and inadequate breastfeeding support.
Using the guidelines, identifying babies at risk
Although rare, kernicterus is a serious complication that can be prevented in most cases by using organizational checks and balances8 such as plotting bilirubin values on the hourly nomogram available with the Canadian guidelines.
Importantly, all providers who care for mothers and newborns should be aware that the Canadian guidelines were written for the prevention, detection, and management of jaundice in otherwise healthy term and near-term infants. Babies who have significant risk factors for pathologic hyperbilirubinemia need more focused care. Providers must carefully interpret the prenatal history of these at-risk infants and take appropriate action.
To determine their readiness for care for newborns with jaundice, providers and hospitals may ask themselves:
- Are we aware of the risk factors for jaundice?
- Are we aware that some babies are at greater risk for pathologic jaundice and should be identified as needing an even more focused approach to their care?
Risk reduction considerations based on experts’ opinions in the CMPA cases
- Keep up-to-date on current guidelines for the detection, management, and prevention of hyperbilirubinemia.
- Complete an appropriate history, noting relevant risk factors for hyperbilirubinemia, including comorbidities and family history.9
- Be aware of the delivery and neonatal characteristics (e.g. prematurity [< 38 weeks], higher birth weight, exclusive breastfeeding) that can place an infant at higher risk of hyperbilirubinemia.8
- Consider whether additional diagnostic tests or consultations are necessary to establish or confirm the diagnosis.
- Have a systematic approach for the detection and timely follow-up of hyperbilirubinemia and consider whether further investigation or referral is indicated.
- Provide clear discharge instructions including follow-up instructions and when to seek further medical attention.
How strong are your systems, what are your vulnerabilities?
- Have you or your hospital implemented the CPS’s Guidelines for the detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants?3
- Do you have access to maternal prenatal records so you can identify risk factors?
- Does your hospital use appropriate hour-specific bilirubin nomograms?
- Is there a process to ensure that bilirubin results are reported in a timely fashion to the most responsible physician?
- Can you and your hospital identify and treat babies in need of intervention in a timely fashion, including having systems for the transfer of babies who require exchange transfusions and tertiary care?
- Do you or your hospital review discharge instructions with the parent(s) and then provide them with supporting written material?
- What resources are available to breastfeeding mothers? How do they find out about these resources?
- Are adequate post-discharge processes in place to ensure appropriate follow-up and testing of newborns, including contact information for the parent in need of advice?
- How do you communicate with other healthcare professionals who will be providing follow-up?
- Noble, J. Preventing Kernicterus in Canadian Hospitals. Healthscape [Internet]. Toronto (ON); Ontario Hospital Association newsletter; 2015 Feb 19 [cited 2016 Feb 1]. Available from: www.healthscape.ca/Pages/news-02192015-HIROCperspectiveonkernicterus.aspx
- Sgro M, Campbell D, Shah V. Incidence and causes of severe neonatal hyperbilirubinemia in Canada. CMAJ. 2006;175:587-90.
- Barrington KJ, Sankaran K. Canadian Paediatric Society Fetus and Newborn Committee. Guidelines for the detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants. Paediatr Child Health. 2007;12(Suppl B):1B-12B.
- Sgro M, Kandasamy S, Shah V, Ofner M, Campbell D. Servere neonatal hyperbilirurubinema decreased after the 2007 Canadian Guidelines. J Pediatr [Internet]. 2016 Feb [cited 2016 March 2];S0022-3476(15):01657-1. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26852177 doi: 10.1016/j.jpeds.2015.12.067
- Darling EK, Guttmann A, Sprague AE, et al. Implementation of the Canadian Paediatric Society’s hyperbilirubinemia Guidelines: A survey of Ontario hospitals. Paediatr Child Health. 2014;19(3):133-7.
- Mateo PC, Lee K-S, Barozzino M, Sgro M. Management of neonatal jaundices varies by practitioner type. Can Fam Physician. 2013;59:e379-86.
- Bhutani VK, Johnson L. Synopsis report from the pilot USA Kernicterus Registry. J Perinatol. 2009;29,S4-7.
- National Patient Safety Consortium. Never Events for Hospital Care in Canada Safer Care for Patients [Internet]. Edmonton: Canadian Patient Safety Institute; 2015 Sep. 11 p. [cited 2016 Feb 01]. Available from: http://www.patientsafetyinstitute.ca/en/toolsResources/NeverEvents/Documents/Never%20Events%20for%20Hospital%20Care%20in%20Canada.pdf
- Norman M, Aberg K, Holmsten K, et al. Predicting nonhemolytic neonatal hyperbilirubinemia. Pediatrics. 2015 Dec;136(6):1087-94.