Kernicterus Surveillance

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Kernicterus Surveillance

• Presented at the FDA Pediatric Advisory Committee
  Meeting on
• Current Epidemiology and Therapeutic
  Interventions Relevant to Hyperbilirubinemia in
  the Term Near-term Newborn
• Washington DC June 11, 2003

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Overview Framework for DD surveillance

• Monitor prevalence rates, trends, prevention
  programs
• Provide registry of cases

Surveillance Systems

• Identify risk protective factors
• Address public concerns

Epidemiological Studies

• Health education prevention strategies
• Public policy

Prevention Programs
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Overview Complexities of DD surveillance

• Definitions
• Attributable to an impairment in physical,
  cognitive, speech or language, psychological, or
  self-care areas
• Measurement
• Not always straight forward, especially for
  conditions that rely on a behavioral patterns
• Population-based
• Limited surveillance and not always well defined
• Above issues can make generalizing results
  difficult or impossible to interpret

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Issues Complexities of KI surveillance

• Range of impairment and associated conditions
• Brain damage that is associated with athetoid
  cerebral palsy, hearing loss, vision impairment,
  dental dysplasia, and sometimes mental
  retardation
• Changes in level of awareness and use of
  diagnosis over time
• Variability in how cases are diagnosed
• Physical markers and behavioral pattern utilized
  for identification
• Early onset but often delayed diagnosis

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Historical perspectives KI surveillance

• No systematic population-based surveillance in
  place to monitor kernicterus or
  hyperbilirubinemia
• Case reports from convenience samples or select
  populations (e.g., medical insurance records)
• No accepted standard for surveillance definition

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Prevalence of kernicterus

• True population estimates unknown to date
• Case reports from a convenience sample
• Johnson, Bhutani, Brown (2002). 90 cases reported
  from 1984-Jan 2001
• Rates versus numbers

• Rate of people with condition
• of people in the population
• Is kernicterus on the rise?

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Summary of issues for KI surveillance

• Case definition
• Debate over cut-offs for inclusion
• Low prevalence condition
• Requires a substantial population to detect cases
• Lack of recognition
• Acute event with specific features
• Permanent damage, long-term clinical features
• Litigation
• Possible deterrent for case identification

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Preliminary actions CDC

• Evaluation of extant databases
• National Hospital Discharge Data
• Preponderance of hyperbilirubinemia codes
• KI codes not readily used
• MADDSP surveillance
• Examined athetoid CP cases
• Kernicterus is not a reportable condition
• CSTE approached regarding national reporting
• Managed Care System
• Proposed cooperative agreement
• Limited interest

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Current activities CDC surveillance efforts

• PERT announcement 2002
• Objectives
• Applicant should seek to review cases of extreme
  jaundice in otherwise healthy full-term infants
• Provide a body of evidence to inform why cases of
  extreme jaundice may lead to kernicterus and why
  kernicterus may be re-emerging
• A forum of concerned scientists and health-care
  professionals will be convened to develop a
  strategic plan for national kernicterus prevention

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Objectives of award recipient

• UMDNJ Robert Wood Johnson Medical School
• Principal Investigators T. Hegyi, B. Ostfeld, A.
  Petrova
• Infant mortality and morbidity of kernicterus
• Surveillance
• Identification of risk factors (clinical
  systemic)
• Support network

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Progress to date

• UMDNJ Robert Wood Johnson Medical School
• Principal Investigators T. Hegyi, B. Ostfeld, A.
  Petrova
• IRB submissions and approvals
• Initial discussion process for population-based
  surveillance with the NJ Dept of Health
• Analyses of extant data on infant morbidity and
  mortality due to kernicterus

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Preliminary results Morbidity

• UMDNJ Robert Wood Johnson Medical School
• Principal Investigators T. Hegyi, B. Ostfeld, A.
  Petrova
• 1992 2001(NJ Hospital Discharge Data)
• 82 cases of kernicterus
• 7.5/100,000 live births (cumulative incidence)
• Significant variation by race and ethnicity with
  the lowest rate among Hispanics and the highest
  rate among Asians

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Objectives of award recipient

• Pennsylvania Hospital, University of Pennsylvania
• Principal Investigators V. Bhutani L. Johnson
• Surveillance
• Identification of risk factors (clinical
  systemic)
• Prevention Taskforce/Steering Committee
• National Prevention Campaign

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Progress to date

• Pennsylvania Hospital, University of Pennsylvania
  Principal Investigators V. Bhutani L. Johnson
• Initial Advisory Board teleconference
• Peer Review Group meeting for consensus on
  kernicterus definition (planned)
• Database development
• Collaboration with PICK on kernicterus
  educational video

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Future directions

• Partner for prevention
• American Academy of Pediatrics, AWHONN, HRSA,
  JCAHO, March of Dimes, NICHD, PICK, University
  researchers, others
• Forum for developing consensus on surveillance
  case definition
• Identify a mechanism for population-based
  surveillance
• State
• National

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Working to prevent kernicterus
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