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Risk Management of Severe Neonatal Hyperbilirubinemia to Prevent Kernicterus
Clin Perinatol 32 (2005) 125 – 139
Risk Management of Severe Neonatal Hyperbilirubinemia to Prevent Kernicterus Vinod K. Bhutani, MDa,b,*, Steven M. Donn, MDc, Lois H. Johnson, MDa a
Department of Pediatrics, University of Pennsylvania School of Medicine, Center for Research on Reproduction and Women’s Health, 1315, Biomedical Research Building II/III, 421 Curie Boulevard, Philadelphia, PA 19104, USA b School of Nursing, University of Pennsylvania, Philadelphia, PA, USA c Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI, USA
Case report A 3.11-kg male infant was delivered to a 32-year-old Caucasian woman with an essentially uncomplicated pregnancy until the spontaneous onset of labor and rupture of membranes at 36 completed weeks of gestation. Maternal blood type was O negative; Rh immune globulin was not administered. Delivery was uneventful with smooth perinatal transition, and the newborn appeared normal in all respects. Mother and baby were discharged 24 hours after delivery, although the baby was noted to be visibly jaundiced. The baby, who was breastfeeding, appeared more jaundiced at home. After repeated phone calls to the pediatrician’s office, he was brought back to the hospital emergency room on the third postnatal day because of lethargy and poor feeding. He had a serum bilirubin of 32 mg/dL at age 76 hours. His neurologic examination revealed a high-pitched cry, ophisthotonic posturing, limited upward gaze, and a reverse Moro response. He was admitted to the neonatal ICU several hours later; a repeat serum bilirubin concentration was 38 mg/dL. He was treated
This work was supported in part by CDC/AAMC PERT Grant No. MM-0488-02/03. * Corresponding author. Department of Pediatrics, University of Pennsylvania School of Medicine, Center for Research on Reproduction and Women’s Health, 1315, Biomedical Research Building II/III, 421 Curie Boulevard, Philadelphia, PA 19104. E-mail address: [email protected] (V.K. Bhutani). 0095-5108/05/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.clp.2004.11.002 perinatology.theclinics.com
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only with intensive phototherapy, to which he responded slowly, and eventually was discharged. Subsequently, he was determined to have ABO incompatibility. At age 5 years, he is profoundly impaired. He has severe choreoathetoid cerebral palsy, sensorineural hearing loss, paralysis of upward gaze, and dental enamel hypoplasia. These signs were diagnosed as kernicterus after a diagnostic odyssey of 3 years.
Root causes of kernicterus Kernicterus, the ultimate adverse manifestation of severe hyperbilirubinemia, is the most easily preventable form of brain injury in the neonatal period, among term and near-term infants. Preventive management—early and effective use of intensive phototherapy—provides for safe bilirubin reduction strategies such that the potentially hazardous use of an exchange transfusion to reduce the bilirubin load dramatically is minimized [1,2]. Screening and prevention are key to
Box 1. Root cause analysis for occurrence of kernicterus Failure to recognize the clinical significance of jaundice within the first 24 hours after birth Failure to recognize the limitations of visual recognition of jaundice Failure to recognize clinical jaundice and document its severity by bilirubin measurement before discharge from the hospital Failure to ensure postdischarge follow-up based on the severity of predischarge hyperbilirubinemia Failure to respond to parental concerns of newborn jaundice, poor feeding, lactational difficulties, and change in newborn’s behavior and activity Failure to provide ongoing lactational support in breastfeeding babies to ensure adequacy of intake Failure to recognize the impact of race, ethnicity, and family history on severity of newborn jaundice and risk of brain damage Failure to diagnose the cause of hyperbilirubinemia Failure to institute interventional strategies to prevent severe hyperbilirubinemia when bilirubin is increasing more rapidly than expected Failure to treat severe hyperbilirubinemia aggressively with intensive phototherapy or exchange transfusion for ‘‘hazardous’’ bilirubin levels Failure to educate parents and health care providers about the potential irreversible risks of jaundice during the newborn period and infancy
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the management of severe hyperbilirubinemia [3–6], because there are no treatment options for kernicterus (a timely double volume exchange transfusion in the early stages of acute bilirubin encephalopathy may prevent or minimize the extent of neurologic sequelae). Risk management of severe hyperbilirubinemia that seeks to implement a practice that prevents kernicterus in the United States needs to meet the expectations of diverse communities, including: (1) the health care providers and clinicians who aim to prevent disabilities through health education, early diagnosis, and the use of effective and safe treatment options; (2) the public health community, to monitor a reduction in the incidence of extreme hyperbilirubinemia (thresholds of hyperbilirubinemia that necessitate an exchange transfusion) through provider and public education; (3) society-at-large, to seek to prevent kernicterus in infants who are discharged as healthy and are anticipated to have benign and healthy outcomes; and (4) the community of families who demand a safer experience for their infants who have neonatal jaundice [7–9]. Gaps in delivery of newborn health services that are related to the management of newborn jaundice have been identified in infants who were discharged as healthy, yet subsequently succumbed to a tragic outcome of kernicterus. The root cause analyses of these cases of kernicterus have been listed in the Joint Commission on Accreditation of Health care Organizations (JCAHO) Sentinel Alerts [10,11], updates and guidelines from the American Academy of Pediatrics (AAP) Subcommittee of Newborn Jaundice [12,13], and a recent analysis of cases from the
Goal Conflicts And Job Pressures
Lapses Incomplete Procedures
Mixed Messages
Regulatory Narrowness
Inadequate Training Production Pressures
Attention Distractions
Oversight Deferred Maintenance Clumsy Technology
Responsibility Shifting
Newborn Jaundice
Ke
r
te nic
rus
Fig. 1. Potential lapses in the management of newborn jaundice. (Modified from Reason J. Human error. In: Latent errors and systems disasters. New York: Cambridge University Press; 1990. p. 173–216; with permission.)
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Pilot Kernicterus Registry, using the principles that were enunciated by the Institute of Medicine [14]. These factors (Box 1) may be attributed to a variety of goal conflicts and work performance at the specific levels of health care delivery. As illustrated in Fig. 1, the triggers that lead to lapses and oversight to provide safe care represent potential ‘‘gaps’’ in the system. Risk management of these quality gaps may be addressed best by a systems approach that is focused on patient safety.
Principles of risk management and patient safety Over the past decade, the concepts and practice of risk management have evolved. Risk management is a systematic process that helps health care providers to identify, evaluate, and address problems that may injure patients, lead to medical negligence claims, and cause financial loss to health care entities. It is not merely a system of defensive medicine, but one which aspires to improve the safety and quality of health care. There are some unique aspects of ‘‘risk’’ in neonatal practice. Neonatologists deal with constant changes in the patient’s weight and physiologic maturation; these influence adverse outcomes greatly. There is no capacity for cooperation on the part of the patient, which places a dependency on others, particularly parents, nursing, and ancillary personnel. Importantly, there frequently is no preexisting physician–parent relationship; this can undermine communication and the establishment of trust. Successful risk management programs meet four basic requirements: attitude, knowledge, skill, and commitment. Attitude must include an awareness of potential risk and liability. It must develop effective systems of communication. There must be a disciplined approach to documentation. There must be an appreciation of the impact of ‘‘other forces’’ on risk management, such as the unique aspects of neonatology that were mentioned above. Knowledge refers to multiple elements, including an understanding of the physician–parent relationship, the process of informed consent or assent to care, a comprehension of communication systems and skills, the requirements for successful documentation, and knowledge of areas of neonatal practice vulnerability and medical negligence claims. Risk management also is a process. It seeks to identify areas of real or potential risk. It calculates the probability of an adverse effect from a risk situation. It estimates the impact of the adverse effect. It attempts to control the risk. It also has an ongoing process [15,16]. How quality is measured remains a subjective function. Donabedian [17] described it as an interaction of structure (the setting and practice of care), process (the delivery of care), and outcome (the result of care). Objective studies, such as the Harvard Malpractice Study, concluded that 0.3% of deaths in hospitalized patients are preventable; in the United States, this represents almost 100,000 deaths per year and surpasses the
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number of patients who die of ‘‘natural causes’’ [18]. Quality of neonatal care on the scale of the Harvard Malpractice Study has yet to be evaluated; nevertheless, results of the Harvard study are worthy of comment. The study concluded that the incidence of medical negligence is shockingly high; that bad medical care is found more frequently among the underprivileged, and that many people never know that they or family members received incompetent or substandard medical services; that complaints filed with the state’s Department of Health rarely are effective; and that in 1984, there were 98,000 adverse events that caused or contributed to 13,451 deaths [19]. The experience of the parent’s organization, Parents of Infants and Children with Kernicterus (PICK), is a case study of how a societal role can affect a change in the health care community (see later discussion). Goals for improving patient safety standards for neonatal jaundice Application of industry standards for safety, such as those that are used to define aviation safety, are particularly relevant and appropriate to this specific aspect of newborn care. Infants who are born after a successful, well-screened, and healthy pregnancy and who experience a safe birthing experience and a subsequent discharge from the well-baby nursery represent a population that is anticipated by pediatricians to enjoy a benign and healthy outcome. Death or preventable brain injury would be attributed to unforeseen catastrophic adverse events. Most clinicians will attest to the benign nature of neonatal jaundice— often labeled as physiologic—and none would tolerate the preventable occurrence of kernicterus. Unmonitored and untreated severe hyperbilirubinemia was shown to lead to cases of excessive hyperbilirubinemia, in which the only available option was to perform a potentially hazardous exchange transfusion to save lives and minimize brain damage. There are no public health indicators for the occurrence of kernicterus; use of surrogate indices has been recommended [2,14]. Using the need for an exchange transfusion as an index adverse outcome (akin to rescue by crash landing), we can estimate the occurrence of defect per critical encounters (using critical-to-quality characteristics). Industry safety experts have used ‘‘sigma six’’ as a means to define safety standards for each critical encounter. Events that occur beyond six standard deviations or do not occur in 99.99996% of the encounters are considered to have achieved a six-sigma status. Thus, if one considers the clinical services to a well baby during the first week after birth as a ‘‘critical encounter’’, the goal is to achieve a benign outcome of neonatal jaundice. The expectation is that families are not presented with an untenable choice to consent for an exchange transfusion in order to prevent kernicterus when their infant manisfests severe hyperbilirubinemia. The incidence of parental decision to consent for an exchange transfusion, based on an incidence of 1:700 for a total serum bilirubin (TSB) of 25 mg/dL [15], provides a basis to determine the frequency of ‘‘infants at risk for kernicterus’’ and calculate ‘‘critical encounter’’ sigma value of 4.45. This predicts an occurrence of critical encounters in 1428 infants who
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are at risk for kernicterus per million well-baby discharges. A risk management approach that improves the ‘‘sigma’’ status to 6.0 or decreases the occurrence of critical encounters to 3.4 per million well-baby discharges is a successful health care strategy for this newborn population; it is family-centered and newborn safety-centered (such that it focuses on adverse experience rather than disease incidence) and provides substantial long-term savings for the health care community and society. Application of a risk management approach to prevent kernicterus A breakthrough strategy to prevent tragic outcomes of unmonitored and untreated severe hyperbilirubinemia has evolved in the past 3 years. It includes identification of the problem by society and the health care community, a characterization of the adverse outcome in a tangible manner, and recommendations for optimal solutions that are achieved best through a systems change [2,7,9,14]. Measurement of the impact of this approach is now in the domain for institutional risk management and public health surveillance. We outline a five step process for systems risk management to prevent kernicterus in the United States: (1) the societal imperative; (2) the clinical mandate; (3) the institutional imperative; (4) the public health imperative; and (5) continuing care of infants who sustain kernicterus. The societal imperative PICK has best vocalized the societal imperative that ‘‘one case of kernicterus is one too many . . .. prevent this’’. PICK was initiated by a small group of mothers of children who had kernicterus, who wished to share the lessons they learned while caring for their infants and help other families from the devastating condition of kernicterus. Over the 3 years since its formation, PICK has expanded its membership greatly and developed powerful partnerships with several components of the health care system to prepare a national campaign for the prevention of kernicterus in the United States [20,21]. In September 2000, as the mother of a son with kernicterus, Susan Sheridan was asked to testify at the Agency for Health Care Policy and Research’s First National Summit on Patient Safety and Medical Errors which received national media coverage. In October 2000, families who were included in a video documentary of children who had kernicterus (selected from the Pilot Kernicterus Registry) attended the first showing of this video (prepared by LJ and VKB) at a satellite meeting at the AAP National Convention. The parents who attended this meeting believed that more should be done by the health care organizations to protect newborns from kernicterus. They formed a group—PICK—and recruited a nationally renowned medical and patient safety advisory board. Their mission has been to partner with the health care system to: (1) prevent kernicterus through the establishment of a systems-based approach for the management of newborn jaundice that includes a universal predischarge bilirubin screen with the
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use of an evidence-based nomogram; (2) provide comfort, support, and information on treatment options for families of children who have kernicterus; and (3) establish a model for a family-centered, system-based approach for a constructive parent–health care partnership to address preventable disabilities. Guided by this mission and their efforts, in February 2001, PICK coordinated a health care agency–consumer workshop that included representatives from the JCAHO; AAP; National Institutes of Health (NIH); Health Care Financing Administration (then HCFA, now Center for Medicare and Medicaid Services or CMS); Maternal and Child Health care Bureau (MCHB) of the Health Resources and Services Administration (HRSA); Office of the Department of Health and Human Service’s Assistant Secretary for Planning and Evaluation (ASPE); Centers for Disease Control (CDC); Association of Women’s Health, Obstetrics and Neonatal Nurses (AWHONN); National Association of Neonatal Nurses (NANN); Harvard School of Public Health; Boston’s Children’s Hospital; Pennsylvania Hospital; some of the leading researchers in the country. and six mothers of children who had kernicterus. PICK presented a call to action for the prevention of kernicterus. PICK, as a family advocacy group, has since worked with the JCAHO to issue a Sentinel Event Alerts on kernicterus prevention (May 2001 and August 2004); with the CDC to publish an alert on the re-emergence of kernicterus in Morbidity/Mortality Weekly Report (June 2001); and with the National Quality Forum in classifying kernicterus as a ‘‘never event’’ in their January 2002 report on preventable adverse outcomes [22]. In addition, the CDC has identified kernicterus as an emerging public health threat and has granted funds for kernicterus research and public education to the University of Pennsylvania in collaboration with PICK. Also, the U.S. Food and Drug Administration, NIH, HRSA/MCHB, and several professional societies and hospital organizations have welcomed PICK’s leadership to voice their vision to the science of prevention of kernicterus. Societal activities, through PICK, continue to focus on: (1) increasing the awareness of the dangers of kernicterus through presentations to health care and consumer audiences; (2) voicing the urgent need for a system-wide standard of care that includes universal predischarge bilirubin screening as a standing order and a fundamental component of jaundice management; (3) being a constructive force in driving change in jaundice management practices by partnering with health care workers; specialty societies; industry, state and federal policy makers; government agencies; and the media to disseminate information, tools, and best practices; (4) launching a comprehensive national education campaign; (5) advocating that JCAHO include the prevention of kernicterus as one of its national patient safety goals, which is necessary to ensure that health care organizations are surveyed for their bilirubin management programs; and (6) articulating in all available forums, the need for all professional and health care organizations to endorse a universal bilirubin screen as the safest means for preventing kernicterus. This societal mission of prevention, promise, and partnership is ongoing and expanding and is based on the recognition that eradication of
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kernicterus only can be achieved through a dedicated partnership and equity between consumers and the health care community [23]. The clinical mandate In July 2004, the AAP, following an extensive review of evidence, deliberation, and consensus building, published a clinical practice guideline for the management of hyperbilirubinemia in the newborn infant of at least 35 weeks of gestation [2]. The overall aim of this guideline is to promote an approach that will reduce the frequency of severe neonatal hyperbilirubinemia and bilirubin encephalopathy, while minimizing the risk of unintended harm, such as increased anxiety, decreased breastfeeding, or unnecessary treatment for the general population. The AAP concluded that the analysis of the reported cases of kernicterus suggests that if health care personnel follow the recommendations that are listed in the proposed guideline, kernicterus largely would be preventable. These guidelines emphasize the importance of universal systematic assessment for the risk of severe hyperbilirubinemia, close follow-up, and prompt intervention when indicated. More importantly, the AAP seeks to further the aims that were defined by the Institute of Medicine as appropriate for health care: safety, effectiveness, efficiency, timeliness, patient centeredness, and equity [24,25]. They specifically emphasize the principles of patient safety and the key role of timeliness of interventions to prevent adverse outcomes that result from neonatal hyperbilirubinemia. Lapses in care can be ascertained by an analysis of the introductory case report (Table 1). This mandate, endorsed by JCAHO, empowers the clinicians to: (1) promote and support successful breastfeeding; (2) establish nursery protocols for the identification and evaluation of hyperbilirubinemia; (3) measure the TSB or transcutaneous bilirubin (TcB) level on infants who are jaundiced in the first 24 hours; (4) recognize that visual estimation of the degree of jaundice can lead to errors, particularly in darkly pigmented infants; (5) interpret all bilirubin levels according to the infant’s age in hours; (6) recognize that infants who were less Table 1 Review of a case of kernicterus that was reported to the Pilot Registry Patient centeredness
Safety
Effective care
Timeliness
Lack of jaundice teaching
Lack of jaundice recognition for TSB or TcB
Lack of recognition of critical risk factors
Lack of on-site lactational consultation Lack of intake documentation and response of laboratory staff Lack of consistent discharge plan
Lack of communication among professionals
Lack of response by physician to parent’s report
Lack of recognition of jaundice as a vital sign Lack of hospital-based breastfeeding
Based on matrix from Institute of Medicine. Abbreviation: TcB, transcutaneous bilirubin.
Lack of TSB/TcB measure of jaundice progression
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than 38 weeks’ gestation, particularly those who are breastfed, are at a higher risk of developing hyperbilirubinemia and require closer surveillance and monitoring; (7) perform a systematic assessment on all infants for the risk of severe hyperbilirubinemia before discharge; (8) provide parents with written and oral information about newborn jaundice; (9) provide appropriate follow-up based on the time of discharge and the risk assessment; and (10) treat newborns, when indicated, with phototherapy or exchange transfusion [2,11]. This clinician mandate, which is endorsed by a respected and venerated professional organization, sets the stage for a health and societal partnership, a shared responsibility, and goals for a national standard to prevent kernicterus. Implementation of these guidelines will build on earlier experiences of the AAP to develop a program to ensure that the strategies that are suggested in the bilirubin guidelines are put into practice. This effort is the initial and key component of a broader initiative, ‘‘Ensuring Safe and Healthy Beginnings,’’ which will address management of several issues that are critical to a seamless and safe transition from the birth hospital to home and family during the first week of age [26]. The AAP program ‘‘Ensuring Safe and Healthy Beginnings’’ will make available a toolkit of practical tools and strategies for clinicians to use in the hospital and office, such as examples of medical order sheets for use in nurseries and hospital policies for recognition and evaluation of jaundice. AAP also will facilitate the development of this toolkit through AAP chapters and residency programs. In addition, participation with other national organizations in a parent education and public awareness campaign would help to develop a program to advocate for needed policy changes [27]. The institutional imperative The root cause analysis of the clinical cases of kernicterus at U.S. health care facilities suggested several areas for improvement based on a systems approach (Box 2). JCAHO authored a Sentinel Event Alert (Issue 18, published in April 2001) to discuss ‘‘the risk factors, root causes, risk reduction strategies and follow up recommendations to guide health care organizations in the prevention, early detection, and treatment of hyperbilirubinemia leading to kernicterus—a highly preventable condition of newborns that leads to severe brain damage or death’’. In a Revised guidance to help prevent kernicterus (Issue 31, August 31), the Joint Commission recommended that all hospitals and health care professionals caring for newborn infants both inside the hospital and after discharge from the hospital observe the recommendations cited in the updated clinical practice guideline. These include specific guidance for hospital policies and procedures. ‘‘All hospitals should provide written and oral information for parents at the time of discharge. This should include an explanation of jaundice, the need to monitor infants for jaundice, and advice on how this should be done [11]. By endorsing the Institute of Medicine’s recommendation for a dramatic change in the way in which the American health care system ensures the safety of patients, the AAP recommended that ‘‘the perspective of safety as a purely
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Box 2. Seven steps to facilitate a safer experience with newborn jaundice in the United States Increase level of concern (patient and provider education) Asses for absence or presence of jaundice objectively Estimate for risk of severe hyperbilirubinemia Target follow-up for the at-risk population Timely intervention for infants whose hour-specific TSB is greater than the 95th percentile (in the high-risk zone) Peer review of ‘‘close calls’’ Rapid response to ‘‘never events’’ Goal: To implement clinical pathways for a safe and seamless experience for newborns who have jaundice, including transition from birthing hospital to home.
individual responsibility must be replaced by the concept of safety as a property of systems.’’ Safe systems—characterized by shared knowledge, a culture that emphasizes safety and promotes an individual’s ability to act in a manner that promotes safety—should minimize the use of memory and emphasize the use of standard procedures (eg, checklists) [2]. Successful implementation of a safe system requires parent and family involvement as equal partners. These principles can be applied to the challenge of preventing severe hyperbilirubinemia and kernicterus. A systematic approach to the implementation of these guidelines should result in greater safety. The AAP recommended that such approaches might include; (1) the establishment of standing protocols for nursing assessment of jaundice, including testing TcB and TSB, without requiring physician orders; (2) checklists or reminders that are associated with risk factors, age at discharge, and laboratory test results that provide guidance for appropriate follow-up; and (3) explicit educational materials for parents (a key component of all AAP guidelines) concerning the identification of newborns who have jaundice [2]. Institutionalization of these recommendations and implementation of a systems approach at birthing hospitals in the United States will empower clinicians and set a new safety standard to achieve goals that are akin to aviation safety. Complemented by a peer review, an internal root-cause analysis of ‘‘closecalls’’ would promote a culture of patient safety, family participation, and clinician empowerment. The public health imperative The first step in developing a surveillance system is to identify the occurrence of kernicterus or its surrogate (eg, extreme hyperbilirubinemia). The next step is case confirmation; who will confirm cases and how and the reporting
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sources must be determined. The usual data recipients at the primary level are county health departments; at the secondary level, it is the state health departments; and at the tertiary level, the CDC is involved. Development of a good and effective surveillance system is rooted in its simplicity, flexibility, acceptability, representativeness, usefulness, and timeliness. At a recent National Institute of Child Health and Human Development meeting, experts posed six questions that are relevant for the public health imperative for surveillance: (1) Is kernicterus a matter of public health concern? (2) What is the relationship between severe hyperbilirubinemia and kernicterus? (3) What are the characteristics and the root causes of recent cases of kernicterus? (4) What should be the target indicator for public health surveillance of kernicterus or hyperbilirubinemia? (5) Have systems-based approaches for the management of newborn jaundice been validated? (6) Are there biologic or genetic markers that can be used to better define infants who are at risk for severe hyperbilirubinemia? [14]. To this end, experts proposed to define the severity thresholds for hyperbilirubinemia [14] based on TSB levels: significant ( 17.0 mg/dL), severe ( 20.0 mg/dL), extreme ( 25.0 mg/dL), and hazardous ( 30.0 mg/dL). These definitions are applicable to infants over 72 hours of age because bilirubin can be more toxic because of limitations of its albumin-binding properties in younger infants. A recommendation was made to obtain data on prevalence, incidence, and mortality of severe hyperbilirubinemia and associated neurologic injury, including kernicterus. In Fig. 2, we propose surveillance indices that use threshold TSB levels for clinical interventions as suggested by recent AAP guidelines.
Never Event
"Close Call"
30 mg/dL
25 mg/dL
Rapid Response Approach
Risk Management
to 18 mg/dL >95th percentile (>72 hours age)
At-Risk Population
>75th percentile (<72 hours age)
Ensure Followup/Intervention
Pre-discharge TSB /TcB screening for all infants * Represents TSB thresholds for exchange transfusion at age >72 hours (AAP 2004)
Fig. 2. Outcome assessment: performance standards. (Data from American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 2004;114:297–316.)
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Because of the spectrum of neuronal injury that is associated with hyperbilirubinemia, neither bilirubin induced neurologic dysfunction (BIND) nor kernicterus can be defined on the basis of TSB alone. Thus, standardization of the definitions of severe hyperbilirubinemia, BIND, acute bilirubin encephalopathy, and kernicterus is essential before making these reportable conditions. In the meantime, the incidence of extreme hyperbilirubinemia serves as a surrogate index for public health surveillance. A systematic, centralized review process, including root cause analysis, for prospective cases of extreme hyperbilirubinemia can be used for education and systems improvements. There are several ways in which a condition becomes a reportable condition. One is through Congressional mandate, another is through state legislative mandates, and the third is through the Council of State and Territorial Epidemiologists action. To achieve a national goal that prevents all cases of kernicterus, more epidemiologic data are needed. With the collaboration of health care systems and organizations for health care quality, regulation, and professional education, the public health community could initiate surveillance by two mechanisms: 1. National and regional surveillance for infants who have extreme hyperbilirubinemia. Using the AAP definition of extreme hyperbilirubinemia as a level at which an exchange transfusion is recommended [25 mg/dL for healthy term infants and decreased levels for those at additional risk], a national reporting system can be established for review by the primary and secondary levels of data recipients. Reporting of these levels should initiate a self-review process by the reporting institution that leads to a system appraisal and correction. 2. Rapid response action for a ‘‘never event’’ should be initiated at a tertiary level when it is reported as a total serum bilirubin level of greater than 30 mg/dL. Occurrence of such an event could lead to the following: a. Direct communication with institutional representatives to initiate an impartial root cause analysis of the event b. Full disclosure to the family c. Enrollment of the infant in an early neurodevelopment evaluation and intervention program for the potential, but often unknown, risk of kernicterus d. Reinforcing the community-wide education regarding the management of newborn jaundice as a collaborative activity of the health care institution and the public health community e. Enrollment of the case in national registry for subsequent long-term follow-up to define accurately the actual risk and incidence of kernicterus A potential downside of public awareness of hyperbilirubinemia and kernicterus is increased liability and litigation. The recent concerns of vaginal births after Cesarean sections and concerns of liability have led to a resurgence of Cesarean sections.
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The medico-legal imperative Medical negligence falls under civil litigation, where an injured party may sue one or more individuals or institutions for causing or contributing to the injury. Under the present tort system, a patient/family (plaintiffs) has the right to seek compensatory damages if they believe that there has been a breach in the applicable standards by those who provided care (defendants), and that it is causally related to the injury [28]. Thus, the family of an infant who has kernicterus can sue those who might be held responsible for improper management of the hyperbilirubinemia—including the hospital—if it can be shown that negligent care resulted in the disabilities manifest as kernicterus. The standard of care is defined as that which a prudent health care provider of similar training and background would do under the same or similar circumstances. It is generally held that the standard of care is a national one, and thus, the management of neonatal jaundice should be the same anywhere in the country. There is no question that the fear of being sued alters health care provider behavior. It may result in ‘‘defensive medicine,’’ with errors of commission (eg, too many tests) occurring more commonly than errors of omission (eg, not doing the appropriate test). The medico-legal imperative prompts most health care providers to conform to prevailing standards [29]. It requires appropriate documentation in the medical record and is intended to assure patient safety and to offer a means of redress when substandard care is provided. Sometimes, the medico-legal imperative is subverted by third-party payers, who may be more concerned with reducing costs [30]. Early discharge, fewer diagnostic tests, and decreased follow-up may contribute to an increased risk of a poor outcome and subsequent litigation. Health care providers may be caught in the middle. It is not clear whether the present tort system has accomplished its goals. Its purpose is essentially threefold: (1) to compensate the injured; (2) to penalize wrong-doers; and (3) to deter future wrong-doing. A substantial percentage of settlements or verdict awards never reaches the patient/family. It has not deterred negligent behavior appreciably; if it did, lawsuits should be a thing of the past. It does not seem to have had much impact on improving quality or safety; it might be argued that it is directly responsible for the ‘‘conspiracy of silence’’ to which lawyers allude, and it inhibits constructive dialog and quality improvement.
Summary Kernicterus, the only pediatric disease to be listed as a ‘‘never event’’ by the National Quality Forum, now impacts all institutions and clinicians who care for newborns. An optimal risk management approach that builds on collaboration between the health care community and society can be successful, if the goals are for a concise definition of adverse encounters, clarity in identification of a population at risk, clinician empowerment, family participation, institutional oversight, and public health surveillance. As a systems approach is institution-
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alized to manage newborn jaundice, occurrence of this ‘‘never event’’ should lead to a collaborative rapid response that results in full disclosure, a critical analysis to distinguish preventable from a truly nonpreventable event, a remediation, early neurodevelopment intervention for the potential brain injury, and nonadversarial support for the infant and family. The potential risk of kernicterus in the newborn is a biologic certainty because the progression of hyperbilirubinemia is monitored or treated inadequately or if there is unpredictable predilection. Reassurance that an infant has had a safe experience with newborn jaundice through systems approach and institutionalized practices, based on seamless care by multiple clinicians at multiple sites as infants transition from birthing hospital to home, would prevent adverse encounters and allow for nonadversarial solutions. The partnerships that are being built among health care providers and societal advocates have the potential to be a viable model for risk management that meets a common purpose of quality and equity.
References [1] American Academy of Pediatrics. Provisional Committee for Quality Improvement. Practice Parameter: management of hyperbilirubinemia in the healthy term newborn. Pediatrics 1994; 94(4):558 – 65. [2] American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 2004;114: 297 – 316. [3] Bhutani VK, Johnson L, Sivieri EM. Predictive ability of a pre-discharge hour-specific serum bilirubin for subsequent significant hyperbilirubinemia in healthy term and near-term newborns. Pediatrics 1999;103(1):6 – 14. [4] Stevenson DK, Fanaroff AA, Maisels MJ, et al. Prediction of hyperbilirubinemia in term and near-term newborn infants. Pediatrics 2001;108:31 – 9. [5] Newman TB, Xiong B, Gonzales VM, et al. Prediction and prevention of extreme neonatal hyperbilirubinemia in a mature health maintenance organization. Arch Pediatr Adolesc Med 2000;154:1140 – 7. [6] Ip S, Chung M, Kulig J, et al. An evidence-based review of important issues concerning neonatal hyperbilirubinemia. Pediatrics 2004;114:e130 – 53. [7] Johnson L, Brown AK, Bhutani VK. System-based approach to management of neonatal jaundice and prevention of kernicterus. J Pediatr 2002;93:488 – 94. [8] Bhutani VK, Johnson LH. Newborn jaundice and kernicterus-health and societal implications. Indian J Pediatr 2003;70(5):407 – 16. [9] Palmer RH, Clanton M, Ezhuthachan S, et al. Applying the ‘‘10 simple rules’’ of the institute of medicine to management of hyperbilirubinemia in newborns. Pediatrics 2003;112:1388 – 93. [10] JCAHO. Kernicterus threatens healthy newborns. Sentinel Event Alert 2001;18:1 – 4. [11] JCAHO. Revised guidance to help prevent kernicterus. Sentinel Event Alert 2004;31(31):1 – 2. [12] Centers for Disease Control and Prevention. Kernicterus in full-term infants—United States, 1994–1998. JAMA 2001;286:299 – 300. [13] AAP Subcommittee on Neonatal Hyperbilirubinemia. Neonatal jaundice and kernicterus. Pediatrics 2001;108:763 – 5. [14] Bhutani VK, Johnson LH, Maisels MJ, et al. Kernicterus: epidemiological strategies for its prevention through systems-based approaches. J Perinatol 2004;24(10):650 – 62.
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[15] Berger JE, Deitschel Jr CH, editors. Medical liability for pediatricians. 6th edition. Elk Grove Village (IL)7 American Academy of Pediatrics; 2004. [16] Donn SM, Fisher CW, editors. Risk management techniques in perinatal and neonatal practice. Armonk (NY)7 Futura Publishing; 1996. [17] Donabedian A. The quality of care: how can it be assessed? JAMA 1988;260:1743 – 8. [18] Brennan TA, Leape LL. Adverse events, negligence in hospitalized patients: results from the Harvard Malpractice Study. Perspec Healthc Risk Manage 1991;11:2 – 8. [19] Brennan TA, Leape LL, Laird NM, et al. Incidence of adverse events and negligence in hospitalized patients. Results of the Harvard Medical Practice Study I. N Engl J Med 1991; 324:370 – 6. [20] Sheridan SE. Kernicterus parents’ group [letter to the editor]. J Pediatr 2002;141(4):597. [21] Dixon KT. Newborn jaundice and kernicterus. Greater awareness and action needed. Adv Nurse Pract 2004;12:43–6, 63. [22] Schyve PM. Microsystems, macrosystems, and kernicterus. Jt Comm J Qual Saf 2004;30:591 – 2. [23] Grimm D. Baby pigment peril. US News World Rep 2003;135(3):39. [24] Institute of Medicine. To err is human: building a safer health system. Washington, DC7 National Academy Press; 2000. [25] Institute of Medicine. Crossing the quality chasm: a new health system for the 21st century. Washington, DC7 National Academy Press; 2001. [26] Lannon C, Stark AR. Closing the gap between guidelines and practice: ensuring safe and healthy beginnings. Pediatrics 2004;114(2):494 – 6. [27] Blackmon LR, Fanaroff AA, Raju TN. National Institute of Child Health and Human Development Research on prevention of bilirubin-induced brain injury and kernicterus: National Institute of Child Health and Human Development conference executive summary. 2003. Pediatrics 2004;114(1):229 – 33. [28] Dawson MC, Cikanek RW. Prospective risk management. In: Donn SM, Fisher CW, editors. Risk management techniques in perinatal and neonatal practice. Armonk (NY)7 Futura Publishing; 1996. p. 663 – 83. [29] Schuster MA, McGlynn EA, Brook RH. How good is the quality of health care in the United States? Milbank Q 1998;76:509, 517–63. [30] Physician Insurers Association of America. Neurologic impairment in newborns. Rockville (MD)7 PIAA Press; 2002.
Risk Management of Severe Neonatal Hyperbilirubinemia to Prevent Kernicterus Vinod K. Bhutani, MDa,b,*, Steven M. Donn, MDc, Lois H. Johnson, MDa a
Department of Pediatrics, University of Pennsylvania School of Medicine, Center for Research on Reproduction and Women’s Health, 1315, Biomedical Research Building II/III, 421 Curie Boulevard, Philadelphia, PA 19104, USA b School of Nursing, University of Pennsylvania, Philadelphia, PA, USA c Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI, USA
Case report A 3.11-kg male infant was delivered to a 32-year-old Caucasian woman with an essentially uncomplicated pregnancy until the spontaneous onset of labor and rupture of membranes at 36 completed weeks of gestation. Maternal blood type was O negative; Rh immune globulin was not administered. Delivery was uneventful with smooth perinatal transition, and the newborn appeared normal in all respects. Mother and baby were discharged 24 hours after delivery, although the baby was noted to be visibly jaundiced. The baby, who was breastfeeding, appeared more jaundiced at home. After repeated phone calls to the pediatrician’s office, he was brought back to the hospital emergency room on the third postnatal day because of lethargy and poor feeding. He had a serum bilirubin of 32 mg/dL at age 76 hours. His neurologic examination revealed a high-pitched cry, ophisthotonic posturing, limited upward gaze, and a reverse Moro response. He was admitted to the neonatal ICU several hours later; a repeat serum bilirubin concentration was 38 mg/dL. He was treated
This work was supported in part by CDC/AAMC PERT Grant No. MM-0488-02/03. * Corresponding author. Department of Pediatrics, University of Pennsylvania School of Medicine, Center for Research on Reproduction and Women’s Health, 1315, Biomedical Research Building II/III, 421 Curie Boulevard, Philadelphia, PA 19104. E-mail address: [email protected] (V.K. Bhutani). 0095-5108/05/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.clp.2004.11.002 perinatology.theclinics.com
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only with intensive phototherapy, to which he responded slowly, and eventually was discharged. Subsequently, he was determined to have ABO incompatibility. At age 5 years, he is profoundly impaired. He has severe choreoathetoid cerebral palsy, sensorineural hearing loss, paralysis of upward gaze, and dental enamel hypoplasia. These signs were diagnosed as kernicterus after a diagnostic odyssey of 3 years.
Root causes of kernicterus Kernicterus, the ultimate adverse manifestation of severe hyperbilirubinemia, is the most easily preventable form of brain injury in the neonatal period, among term and near-term infants. Preventive management—early and effective use of intensive phototherapy—provides for safe bilirubin reduction strategies such that the potentially hazardous use of an exchange transfusion to reduce the bilirubin load dramatically is minimized [1,2]. Screening and prevention are key to
Box 1. Root cause analysis for occurrence of kernicterus Failure to recognize the clinical significance of jaundice within the first 24 hours after birth Failure to recognize the limitations of visual recognition of jaundice Failure to recognize clinical jaundice and document its severity by bilirubin measurement before discharge from the hospital Failure to ensure postdischarge follow-up based on the severity of predischarge hyperbilirubinemia Failure to respond to parental concerns of newborn jaundice, poor feeding, lactational difficulties, and change in newborn’s behavior and activity Failure to provide ongoing lactational support in breastfeeding babies to ensure adequacy of intake Failure to recognize the impact of race, ethnicity, and family history on severity of newborn jaundice and risk of brain damage Failure to diagnose the cause of hyperbilirubinemia Failure to institute interventional strategies to prevent severe hyperbilirubinemia when bilirubin is increasing more rapidly than expected Failure to treat severe hyperbilirubinemia aggressively with intensive phototherapy or exchange transfusion for ‘‘hazardous’’ bilirubin levels Failure to educate parents and health care providers about the potential irreversible risks of jaundice during the newborn period and infancy
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the management of severe hyperbilirubinemia [3–6], because there are no treatment options for kernicterus (a timely double volume exchange transfusion in the early stages of acute bilirubin encephalopathy may prevent or minimize the extent of neurologic sequelae). Risk management of severe hyperbilirubinemia that seeks to implement a practice that prevents kernicterus in the United States needs to meet the expectations of diverse communities, including: (1) the health care providers and clinicians who aim to prevent disabilities through health education, early diagnosis, and the use of effective and safe treatment options; (2) the public health community, to monitor a reduction in the incidence of extreme hyperbilirubinemia (thresholds of hyperbilirubinemia that necessitate an exchange transfusion) through provider and public education; (3) society-at-large, to seek to prevent kernicterus in infants who are discharged as healthy and are anticipated to have benign and healthy outcomes; and (4) the community of families who demand a safer experience for their infants who have neonatal jaundice [7–9]. Gaps in delivery of newborn health services that are related to the management of newborn jaundice have been identified in infants who were discharged as healthy, yet subsequently succumbed to a tragic outcome of kernicterus. The root cause analyses of these cases of kernicterus have been listed in the Joint Commission on Accreditation of Health care Organizations (JCAHO) Sentinel Alerts [10,11], updates and guidelines from the American Academy of Pediatrics (AAP) Subcommittee of Newborn Jaundice [12,13], and a recent analysis of cases from the
Goal Conflicts And Job Pressures
Lapses Incomplete Procedures
Mixed Messages
Regulatory Narrowness
Inadequate Training Production Pressures
Attention Distractions
Oversight Deferred Maintenance Clumsy Technology
Responsibility Shifting
Newborn Jaundice
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Fig. 1. Potential lapses in the management of newborn jaundice. (Modified from Reason J. Human error. In: Latent errors and systems disasters. New York: Cambridge University Press; 1990. p. 173–216; with permission.)
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Pilot Kernicterus Registry, using the principles that were enunciated by the Institute of Medicine [14]. These factors (Box 1) may be attributed to a variety of goal conflicts and work performance at the specific levels of health care delivery. As illustrated in Fig. 1, the triggers that lead to lapses and oversight to provide safe care represent potential ‘‘gaps’’ in the system. Risk management of these quality gaps may be addressed best by a systems approach that is focused on patient safety.
Principles of risk management and patient safety Over the past decade, the concepts and practice of risk management have evolved. Risk management is a systematic process that helps health care providers to identify, evaluate, and address problems that may injure patients, lead to medical negligence claims, and cause financial loss to health care entities. It is not merely a system of defensive medicine, but one which aspires to improve the safety and quality of health care. There are some unique aspects of ‘‘risk’’ in neonatal practice. Neonatologists deal with constant changes in the patient’s weight and physiologic maturation; these influence adverse outcomes greatly. There is no capacity for cooperation on the part of the patient, which places a dependency on others, particularly parents, nursing, and ancillary personnel. Importantly, there frequently is no preexisting physician–parent relationship; this can undermine communication and the establishment of trust. Successful risk management programs meet four basic requirements: attitude, knowledge, skill, and commitment. Attitude must include an awareness of potential risk and liability. It must develop effective systems of communication. There must be a disciplined approach to documentation. There must be an appreciation of the impact of ‘‘other forces’’ on risk management, such as the unique aspects of neonatology that were mentioned above. Knowledge refers to multiple elements, including an understanding of the physician–parent relationship, the process of informed consent or assent to care, a comprehension of communication systems and skills, the requirements for successful documentation, and knowledge of areas of neonatal practice vulnerability and medical negligence claims. Risk management also is a process. It seeks to identify areas of real or potential risk. It calculates the probability of an adverse effect from a risk situation. It estimates the impact of the adverse effect. It attempts to control the risk. It also has an ongoing process [15,16]. How quality is measured remains a subjective function. Donabedian [17] described it as an interaction of structure (the setting and practice of care), process (the delivery of care), and outcome (the result of care). Objective studies, such as the Harvard Malpractice Study, concluded that 0.3% of deaths in hospitalized patients are preventable; in the United States, this represents almost 100,000 deaths per year and surpasses the
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number of patients who die of ‘‘natural causes’’ [18]. Quality of neonatal care on the scale of the Harvard Malpractice Study has yet to be evaluated; nevertheless, results of the Harvard study are worthy of comment. The study concluded that the incidence of medical negligence is shockingly high; that bad medical care is found more frequently among the underprivileged, and that many people never know that they or family members received incompetent or substandard medical services; that complaints filed with the state’s Department of Health rarely are effective; and that in 1984, there were 98,000 adverse events that caused or contributed to 13,451 deaths [19]. The experience of the parent’s organization, Parents of Infants and Children with Kernicterus (PICK), is a case study of how a societal role can affect a change in the health care community (see later discussion). Goals for improving patient safety standards for neonatal jaundice Application of industry standards for safety, such as those that are used to define aviation safety, are particularly relevant and appropriate to this specific aspect of newborn care. Infants who are born after a successful, well-screened, and healthy pregnancy and who experience a safe birthing experience and a subsequent discharge from the well-baby nursery represent a population that is anticipated by pediatricians to enjoy a benign and healthy outcome. Death or preventable brain injury would be attributed to unforeseen catastrophic adverse events. Most clinicians will attest to the benign nature of neonatal jaundice— often labeled as physiologic—and none would tolerate the preventable occurrence of kernicterus. Unmonitored and untreated severe hyperbilirubinemia was shown to lead to cases of excessive hyperbilirubinemia, in which the only available option was to perform a potentially hazardous exchange transfusion to save lives and minimize brain damage. There are no public health indicators for the occurrence of kernicterus; use of surrogate indices has been recommended [2,14]. Using the need for an exchange transfusion as an index adverse outcome (akin to rescue by crash landing), we can estimate the occurrence of defect per critical encounters (using critical-to-quality characteristics). Industry safety experts have used ‘‘sigma six’’ as a means to define safety standards for each critical encounter. Events that occur beyond six standard deviations or do not occur in 99.99996% of the encounters are considered to have achieved a six-sigma status. Thus, if one considers the clinical services to a well baby during the first week after birth as a ‘‘critical encounter’’, the goal is to achieve a benign outcome of neonatal jaundice. The expectation is that families are not presented with an untenable choice to consent for an exchange transfusion in order to prevent kernicterus when their infant manisfests severe hyperbilirubinemia. The incidence of parental decision to consent for an exchange transfusion, based on an incidence of 1:700 for a total serum bilirubin (TSB) of 25 mg/dL [15], provides a basis to determine the frequency of ‘‘infants at risk for kernicterus’’ and calculate ‘‘critical encounter’’ sigma value of 4.45. This predicts an occurrence of critical encounters in 1428 infants who
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are at risk for kernicterus per million well-baby discharges. A risk management approach that improves the ‘‘sigma’’ status to 6.0 or decreases the occurrence of critical encounters to 3.4 per million well-baby discharges is a successful health care strategy for this newborn population; it is family-centered and newborn safety-centered (such that it focuses on adverse experience rather than disease incidence) and provides substantial long-term savings for the health care community and society. Application of a risk management approach to prevent kernicterus A breakthrough strategy to prevent tragic outcomes of unmonitored and untreated severe hyperbilirubinemia has evolved in the past 3 years. It includes identification of the problem by society and the health care community, a characterization of the adverse outcome in a tangible manner, and recommendations for optimal solutions that are achieved best through a systems change [2,7,9,14]. Measurement of the impact of this approach is now in the domain for institutional risk management and public health surveillance. We outline a five step process for systems risk management to prevent kernicterus in the United States: (1) the societal imperative; (2) the clinical mandate; (3) the institutional imperative; (4) the public health imperative; and (5) continuing care of infants who sustain kernicterus. The societal imperative PICK has best vocalized the societal imperative that ‘‘one case of kernicterus is one too many . . .. prevent this’’. PICK was initiated by a small group of mothers of children who had kernicterus, who wished to share the lessons they learned while caring for their infants and help other families from the devastating condition of kernicterus. Over the 3 years since its formation, PICK has expanded its membership greatly and developed powerful partnerships with several components of the health care system to prepare a national campaign for the prevention of kernicterus in the United States [20,21]. In September 2000, as the mother of a son with kernicterus, Susan Sheridan was asked to testify at the Agency for Health Care Policy and Research’s First National Summit on Patient Safety and Medical Errors which received national media coverage. In October 2000, families who were included in a video documentary of children who had kernicterus (selected from the Pilot Kernicterus Registry) attended the first showing of this video (prepared by LJ and VKB) at a satellite meeting at the AAP National Convention. The parents who attended this meeting believed that more should be done by the health care organizations to protect newborns from kernicterus. They formed a group—PICK—and recruited a nationally renowned medical and patient safety advisory board. Their mission has been to partner with the health care system to: (1) prevent kernicterus through the establishment of a systems-based approach for the management of newborn jaundice that includes a universal predischarge bilirubin screen with the
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use of an evidence-based nomogram; (2) provide comfort, support, and information on treatment options for families of children who have kernicterus; and (3) establish a model for a family-centered, system-based approach for a constructive parent–health care partnership to address preventable disabilities. Guided by this mission and their efforts, in February 2001, PICK coordinated a health care agency–consumer workshop that included representatives from the JCAHO; AAP; National Institutes of Health (NIH); Health Care Financing Administration (then HCFA, now Center for Medicare and Medicaid Services or CMS); Maternal and Child Health care Bureau (MCHB) of the Health Resources and Services Administration (HRSA); Office of the Department of Health and Human Service’s Assistant Secretary for Planning and Evaluation (ASPE); Centers for Disease Control (CDC); Association of Women’s Health, Obstetrics and Neonatal Nurses (AWHONN); National Association of Neonatal Nurses (NANN); Harvard School of Public Health; Boston’s Children’s Hospital; Pennsylvania Hospital; some of the leading researchers in the country. and six mothers of children who had kernicterus. PICK presented a call to action for the prevention of kernicterus. PICK, as a family advocacy group, has since worked with the JCAHO to issue a Sentinel Event Alerts on kernicterus prevention (May 2001 and August 2004); with the CDC to publish an alert on the re-emergence of kernicterus in Morbidity/Mortality Weekly Report (June 2001); and with the National Quality Forum in classifying kernicterus as a ‘‘never event’’ in their January 2002 report on preventable adverse outcomes [22]. In addition, the CDC has identified kernicterus as an emerging public health threat and has granted funds for kernicterus research and public education to the University of Pennsylvania in collaboration with PICK. Also, the U.S. Food and Drug Administration, NIH, HRSA/MCHB, and several professional societies and hospital organizations have welcomed PICK’s leadership to voice their vision to the science of prevention of kernicterus. Societal activities, through PICK, continue to focus on: (1) increasing the awareness of the dangers of kernicterus through presentations to health care and consumer audiences; (2) voicing the urgent need for a system-wide standard of care that includes universal predischarge bilirubin screening as a standing order and a fundamental component of jaundice management; (3) being a constructive force in driving change in jaundice management practices by partnering with health care workers; specialty societies; industry, state and federal policy makers; government agencies; and the media to disseminate information, tools, and best practices; (4) launching a comprehensive national education campaign; (5) advocating that JCAHO include the prevention of kernicterus as one of its national patient safety goals, which is necessary to ensure that health care organizations are surveyed for their bilirubin management programs; and (6) articulating in all available forums, the need for all professional and health care organizations to endorse a universal bilirubin screen as the safest means for preventing kernicterus. This societal mission of prevention, promise, and partnership is ongoing and expanding and is based on the recognition that eradication of
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kernicterus only can be achieved through a dedicated partnership and equity between consumers and the health care community [23]. The clinical mandate In July 2004, the AAP, following an extensive review of evidence, deliberation, and consensus building, published a clinical practice guideline for the management of hyperbilirubinemia in the newborn infant of at least 35 weeks of gestation [2]. The overall aim of this guideline is to promote an approach that will reduce the frequency of severe neonatal hyperbilirubinemia and bilirubin encephalopathy, while minimizing the risk of unintended harm, such as increased anxiety, decreased breastfeeding, or unnecessary treatment for the general population. The AAP concluded that the analysis of the reported cases of kernicterus suggests that if health care personnel follow the recommendations that are listed in the proposed guideline, kernicterus largely would be preventable. These guidelines emphasize the importance of universal systematic assessment for the risk of severe hyperbilirubinemia, close follow-up, and prompt intervention when indicated. More importantly, the AAP seeks to further the aims that were defined by the Institute of Medicine as appropriate for health care: safety, effectiveness, efficiency, timeliness, patient centeredness, and equity [24,25]. They specifically emphasize the principles of patient safety and the key role of timeliness of interventions to prevent adverse outcomes that result from neonatal hyperbilirubinemia. Lapses in care can be ascertained by an analysis of the introductory case report (Table 1). This mandate, endorsed by JCAHO, empowers the clinicians to: (1) promote and support successful breastfeeding; (2) establish nursery protocols for the identification and evaluation of hyperbilirubinemia; (3) measure the TSB or transcutaneous bilirubin (TcB) level on infants who are jaundiced in the first 24 hours; (4) recognize that visual estimation of the degree of jaundice can lead to errors, particularly in darkly pigmented infants; (5) interpret all bilirubin levels according to the infant’s age in hours; (6) recognize that infants who were less Table 1 Review of a case of kernicterus that was reported to the Pilot Registry Patient centeredness
Safety
Effective care
Timeliness
Lack of jaundice teaching
Lack of jaundice recognition for TSB or TcB
Lack of recognition of critical risk factors
Lack of on-site lactational consultation Lack of intake documentation and response of laboratory staff Lack of consistent discharge plan
Lack of communication among professionals
Lack of response by physician to parent’s report
Lack of recognition of jaundice as a vital sign Lack of hospital-based breastfeeding
Based on matrix from Institute of Medicine. Abbreviation: TcB, transcutaneous bilirubin.
Lack of TSB/TcB measure of jaundice progression
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than 38 weeks’ gestation, particularly those who are breastfed, are at a higher risk of developing hyperbilirubinemia and require closer surveillance and monitoring; (7) perform a systematic assessment on all infants for the risk of severe hyperbilirubinemia before discharge; (8) provide parents with written and oral information about newborn jaundice; (9) provide appropriate follow-up based on the time of discharge and the risk assessment; and (10) treat newborns, when indicated, with phototherapy or exchange transfusion [2,11]. This clinician mandate, which is endorsed by a respected and venerated professional organization, sets the stage for a health and societal partnership, a shared responsibility, and goals for a national standard to prevent kernicterus. Implementation of these guidelines will build on earlier experiences of the AAP to develop a program to ensure that the strategies that are suggested in the bilirubin guidelines are put into practice. This effort is the initial and key component of a broader initiative, ‘‘Ensuring Safe and Healthy Beginnings,’’ which will address management of several issues that are critical to a seamless and safe transition from the birth hospital to home and family during the first week of age [26]. The AAP program ‘‘Ensuring Safe and Healthy Beginnings’’ will make available a toolkit of practical tools and strategies for clinicians to use in the hospital and office, such as examples of medical order sheets for use in nurseries and hospital policies for recognition and evaluation of jaundice. AAP also will facilitate the development of this toolkit through AAP chapters and residency programs. In addition, participation with other national organizations in a parent education and public awareness campaign would help to develop a program to advocate for needed policy changes [27]. The institutional imperative The root cause analysis of the clinical cases of kernicterus at U.S. health care facilities suggested several areas for improvement based on a systems approach (Box 2). JCAHO authored a Sentinel Event Alert (Issue 18, published in April 2001) to discuss ‘‘the risk factors, root causes, risk reduction strategies and follow up recommendations to guide health care organizations in the prevention, early detection, and treatment of hyperbilirubinemia leading to kernicterus—a highly preventable condition of newborns that leads to severe brain damage or death’’. In a Revised guidance to help prevent kernicterus (Issue 31, August 31), the Joint Commission recommended that all hospitals and health care professionals caring for newborn infants both inside the hospital and after discharge from the hospital observe the recommendations cited in the updated clinical practice guideline. These include specific guidance for hospital policies and procedures. ‘‘All hospitals should provide written and oral information for parents at the time of discharge. This should include an explanation of jaundice, the need to monitor infants for jaundice, and advice on how this should be done [11]. By endorsing the Institute of Medicine’s recommendation for a dramatic change in the way in which the American health care system ensures the safety of patients, the AAP recommended that ‘‘the perspective of safety as a purely
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Box 2. Seven steps to facilitate a safer experience with newborn jaundice in the United States Increase level of concern (patient and provider education) Asses for absence or presence of jaundice objectively Estimate for risk of severe hyperbilirubinemia Target follow-up for the at-risk population Timely intervention for infants whose hour-specific TSB is greater than the 95th percentile (in the high-risk zone) Peer review of ‘‘close calls’’ Rapid response to ‘‘never events’’ Goal: To implement clinical pathways for a safe and seamless experience for newborns who have jaundice, including transition from birthing hospital to home.
individual responsibility must be replaced by the concept of safety as a property of systems.’’ Safe systems—characterized by shared knowledge, a culture that emphasizes safety and promotes an individual’s ability to act in a manner that promotes safety—should minimize the use of memory and emphasize the use of standard procedures (eg, checklists) [2]. Successful implementation of a safe system requires parent and family involvement as equal partners. These principles can be applied to the challenge of preventing severe hyperbilirubinemia and kernicterus. A systematic approach to the implementation of these guidelines should result in greater safety. The AAP recommended that such approaches might include; (1) the establishment of standing protocols for nursing assessment of jaundice, including testing TcB and TSB, without requiring physician orders; (2) checklists or reminders that are associated with risk factors, age at discharge, and laboratory test results that provide guidance for appropriate follow-up; and (3) explicit educational materials for parents (a key component of all AAP guidelines) concerning the identification of newborns who have jaundice [2]. Institutionalization of these recommendations and implementation of a systems approach at birthing hospitals in the United States will empower clinicians and set a new safety standard to achieve goals that are akin to aviation safety. Complemented by a peer review, an internal root-cause analysis of ‘‘closecalls’’ would promote a culture of patient safety, family participation, and clinician empowerment. The public health imperative The first step in developing a surveillance system is to identify the occurrence of kernicterus or its surrogate (eg, extreme hyperbilirubinemia). The next step is case confirmation; who will confirm cases and how and the reporting
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sources must be determined. The usual data recipients at the primary level are county health departments; at the secondary level, it is the state health departments; and at the tertiary level, the CDC is involved. Development of a good and effective surveillance system is rooted in its simplicity, flexibility, acceptability, representativeness, usefulness, and timeliness. At a recent National Institute of Child Health and Human Development meeting, experts posed six questions that are relevant for the public health imperative for surveillance: (1) Is kernicterus a matter of public health concern? (2) What is the relationship between severe hyperbilirubinemia and kernicterus? (3) What are the characteristics and the root causes of recent cases of kernicterus? (4) What should be the target indicator for public health surveillance of kernicterus or hyperbilirubinemia? (5) Have systems-based approaches for the management of newborn jaundice been validated? (6) Are there biologic or genetic markers that can be used to better define infants who are at risk for severe hyperbilirubinemia? [14]. To this end, experts proposed to define the severity thresholds for hyperbilirubinemia [14] based on TSB levels: significant ( 17.0 mg/dL), severe ( 20.0 mg/dL), extreme ( 25.0 mg/dL), and hazardous ( 30.0 mg/dL). These definitions are applicable to infants over 72 hours of age because bilirubin can be more toxic because of limitations of its albumin-binding properties in younger infants. A recommendation was made to obtain data on prevalence, incidence, and mortality of severe hyperbilirubinemia and associated neurologic injury, including kernicterus. In Fig. 2, we propose surveillance indices that use threshold TSB levels for clinical interventions as suggested by recent AAP guidelines.
Never Event
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30 mg/dL
25 mg/dL
Rapid Response Approach
Risk Management
to 18 mg/dL >95th percentile (>72 hours age)
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>75th percentile (<72 hours age)
Ensure Followup/Intervention
Pre-discharge TSB /TcB screening for all infants * Represents TSB thresholds for exchange transfusion at age >72 hours (AAP 2004)
Fig. 2. Outcome assessment: performance standards. (Data from American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 2004;114:297–316.)
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Because of the spectrum of neuronal injury that is associated with hyperbilirubinemia, neither bilirubin induced neurologic dysfunction (BIND) nor kernicterus can be defined on the basis of TSB alone. Thus, standardization of the definitions of severe hyperbilirubinemia, BIND, acute bilirubin encephalopathy, and kernicterus is essential before making these reportable conditions. In the meantime, the incidence of extreme hyperbilirubinemia serves as a surrogate index for public health surveillance. A systematic, centralized review process, including root cause analysis, for prospective cases of extreme hyperbilirubinemia can be used for education and systems improvements. There are several ways in which a condition becomes a reportable condition. One is through Congressional mandate, another is through state legislative mandates, and the third is through the Council of State and Territorial Epidemiologists action. To achieve a national goal that prevents all cases of kernicterus, more epidemiologic data are needed. With the collaboration of health care systems and organizations for health care quality, regulation, and professional education, the public health community could initiate surveillance by two mechanisms: 1. National and regional surveillance for infants who have extreme hyperbilirubinemia. Using the AAP definition of extreme hyperbilirubinemia as a level at which an exchange transfusion is recommended [25 mg/dL for healthy term infants and decreased levels for those at additional risk], a national reporting system can be established for review by the primary and secondary levels of data recipients. Reporting of these levels should initiate a self-review process by the reporting institution that leads to a system appraisal and correction. 2. Rapid response action for a ‘‘never event’’ should be initiated at a tertiary level when it is reported as a total serum bilirubin level of greater than 30 mg/dL. Occurrence of such an event could lead to the following: a. Direct communication with institutional representatives to initiate an impartial root cause analysis of the event b. Full disclosure to the family c. Enrollment of the infant in an early neurodevelopment evaluation and intervention program for the potential, but often unknown, risk of kernicterus d. Reinforcing the community-wide education regarding the management of newborn jaundice as a collaborative activity of the health care institution and the public health community e. Enrollment of the case in national registry for subsequent long-term follow-up to define accurately the actual risk and incidence of kernicterus A potential downside of public awareness of hyperbilirubinemia and kernicterus is increased liability and litigation. The recent concerns of vaginal births after Cesarean sections and concerns of liability have led to a resurgence of Cesarean sections.
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The medico-legal imperative Medical negligence falls under civil litigation, where an injured party may sue one or more individuals or institutions for causing or contributing to the injury. Under the present tort system, a patient/family (plaintiffs) has the right to seek compensatory damages if they believe that there has been a breach in the applicable standards by those who provided care (defendants), and that it is causally related to the injury [28]. Thus, the family of an infant who has kernicterus can sue those who might be held responsible for improper management of the hyperbilirubinemia—including the hospital—if it can be shown that negligent care resulted in the disabilities manifest as kernicterus. The standard of care is defined as that which a prudent health care provider of similar training and background would do under the same or similar circumstances. It is generally held that the standard of care is a national one, and thus, the management of neonatal jaundice should be the same anywhere in the country. There is no question that the fear of being sued alters health care provider behavior. It may result in ‘‘defensive medicine,’’ with errors of commission (eg, too many tests) occurring more commonly than errors of omission (eg, not doing the appropriate test). The medico-legal imperative prompts most health care providers to conform to prevailing standards [29]. It requires appropriate documentation in the medical record and is intended to assure patient safety and to offer a means of redress when substandard care is provided. Sometimes, the medico-legal imperative is subverted by third-party payers, who may be more concerned with reducing costs [30]. Early discharge, fewer diagnostic tests, and decreased follow-up may contribute to an increased risk of a poor outcome and subsequent litigation. Health care providers may be caught in the middle. It is not clear whether the present tort system has accomplished its goals. Its purpose is essentially threefold: (1) to compensate the injured; (2) to penalize wrong-doers; and (3) to deter future wrong-doing. A substantial percentage of settlements or verdict awards never reaches the patient/family. It has not deterred negligent behavior appreciably; if it did, lawsuits should be a thing of the past. It does not seem to have had much impact on improving quality or safety; it might be argued that it is directly responsible for the ‘‘conspiracy of silence’’ to which lawyers allude, and it inhibits constructive dialog and quality improvement.
Summary Kernicterus, the only pediatric disease to be listed as a ‘‘never event’’ by the National Quality Forum, now impacts all institutions and clinicians who care for newborns. An optimal risk management approach that builds on collaboration between the health care community and society can be successful, if the goals are for a concise definition of adverse encounters, clarity in identification of a population at risk, clinician empowerment, family participation, institutional oversight, and public health surveillance. As a systems approach is institution-
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alized to manage newborn jaundice, occurrence of this ‘‘never event’’ should lead to a collaborative rapid response that results in full disclosure, a critical analysis to distinguish preventable from a truly nonpreventable event, a remediation, early neurodevelopment intervention for the potential brain injury, and nonadversarial support for the infant and family. The potential risk of kernicterus in the newborn is a biologic certainty because the progression of hyperbilirubinemia is monitored or treated inadequately or if there is unpredictable predilection. Reassurance that an infant has had a safe experience with newborn jaundice through systems approach and institutionalized practices, based on seamless care by multiple clinicians at multiple sites as infants transition from birthing hospital to home, would prevent adverse encounters and allow for nonadversarial solutions. The partnerships that are being built among health care providers and societal advocates have the potential to be a viable model for risk management that meets a common purpose of quality and equity.
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