32 nd California SIDS Conference. Los Angeles, California. October 30, 2012.

1. 32nd California SIDS Conference. Los Angeles, California. October 30, 2012. Diagnosis of Infants Dying Suddenly: Is it SIDS, SUDI, or Undetermined? Does it Matter? Thomas G. Keens, M.D. Chair, California SIDS Advisory Council Professor of Pediatrics, Physiology and Biophysics Keck School of Medicine of the University of Southern California Division of Pediatric Pulmonology, Children’s Hospital Los Angeles No Conflicts of Interest to Disclose

2. “And this woman's son died in the night ...” 1 Kings 3: 19 (950 B.C.) Antoon Claeissens, The Judgment of Solomon, ~1600.

3. “What greater pain can mortals bear than this: to see their children die before their eyes?” Euripides 480-406 B.C.E. Russell-Jones, D.L. Arch. Dis. Child., 60: 278-281, 1985.

4. The Problem • Diagnosing the cause and manner of death for infants who die suddenly and unexpectedly has become challenging. • There is a lack of consistency in the way these diagnoses are made between counties in California. • Infants with similar death scene and autopsy findings may be “SIDS” in one county, “Undetermined” in another, and “Positional asphyxia” in still another.

5. Sudden Deaths in Infants • Ancient Greece and Rome, and in the Middle Ages, the cause of sudden infant death was generally assumed to be maternal overlaying. • 12th Century, Britain’s Children and Young Person’s Act criminally punished adults who “in a drunken state” slept in a bed with an infant who was later found dead. • 1862, Anthony Leared, M.D.: “Nor must we forget a frequent cause of death, that in which an infant is overlaid in its slumbers by a careless, perhaps drunken, nurse or mother.” Russell-Jones, D.L. Arch. Dis. Child., 60: 278-281, 1985.

6. In 18th century France, a wood and metal cage --- arcuccio --- was placed over a bedsharing infant to prevent overlaying. • Charles Dickens, “Of all things in life, there should be nothing so preventable, as there is nothing on the face of it so unnatural, as the death of a little child.” Arcuccio, ~1730

7. Status Thymico Lymphaticus • In 1889, Professor A. Paltauf of Vienna hypothesized that SIDS was due to Status Thymico Lymphaticus. • An enlarged thymus might compress the airway, causing death. • In 1906, Professor Hamar showed that Paltauf’s findings were normal, and did not cause death. • Blame returned to caregivers. Russell-Jones, D.L. Arch. Dis. Child., 60: 278-281, 1985. Dally, A. Medical History, 41: 70-85, 1997.

8. Early Study of Maternal Overlaying • In 1892, a Scottish police surgeon, Templeman, drew attention to the potential role of excessive alcohol consumption and overlaying. • 258 cases of suffocation in infants. • More than half of deaths occurred Saturday night. • Postulated that intoxication impaired arousal responses of parents sleeping with infants, thus increasing the risk of accidental suffocation. Templeman, C. Edinburgh Med. J., 38: 322-329, 1892.

9. Infant Deaths in the 20th Century • Social changes and increased standard of living, most infants had a separate bed; no overlaying. • Deaths were blamed on soft pillows and bedclothes causing suffocation; but some babies died face up and not covered by pillows. • By mid-century, autopsies were performed more consistently, and pathologists began to believe these were natural causes of death, rather than suffocation. • Diagnoses “unascertainable” or “unknown”. • Knowledge of the epidemiology of SIDS was unknown prior to 1960. Russell-Jones, D.L. Arch. Dis. Child., 60: 278-281, 1985.

10. Second International Conference on the Cause of Sudden Death in Infants, 1969 Definition of Sudden Infant Death Syndrome (attributed to J. Bruce Beckwith): “The sudden death of an infant or young child, which is unexpected by history, and in which a thorough post mortem examination fails to demonstrate an adequate cause of death.” Bergman, A.B., J.B. Beckwith, and C.G. Ray, eds. Proceedings of the second international conference on the causes of sudden death in infants, Seattle Washington. University of Washington Press, 1970.

11. Second International Conference on the Cause of Sudden Death in Infants, 1969 • This original definition was developed by pathologists for two reasons: • to give a consistent name to the group of babies which were receiving widely disparate diagnoses at that time; • to focus research activities on this group of babies in an attempt to find a cause and/or cure. • At the time this definition was developed, it was intended to mean essentially what “undetermined” means today; that is, that the death is unexplained. Bergman, A.B., J.B. Beckwith, and C.G. Ray, eds. Proceedings of the second international conference on the causes of sudden death in infants, Seattle Washington. University of Washington Press, 1970.

12. Sudden Infant Death Syndrome • Sudden Infant Death Syndrome Act of 1974 (U.S. Public Health Law 93-270) authorized funding for SIDS services and research, and defined SIDS. • In 1974, California passed AB 409: Crown Act: Sudden Infant Death Syndrome, mandating use of the diagnosis “sudden infant death syndrome” when appropriate. • In 1979, the World Health Organization created a category for “Sudden Infant Death” (code 798.0) in the International Classification of Diseases.

13. SIDS in California in the 1980’s • First responders often mistook natural post-mortem pooling of blood for bruising. Parents were arrested for killing their babies. • Lack of pathology expertise in many jurisdictions. Diagnoses were inaccurate and inconsistent. • Little public education about SIDS. Most SIDS parents had never heard of SIDS before their baby died. • Parent support was provided entirely by SIDS parent support groups. • There was little information available where SIDS families could turn for answers.

14. SIDS in California in the 1980’s • Southern and Northern California Regional SIDS Councils identified four needs: • Need for first responder education about SIDS, to prevent SIDS parents from being unjustly arrested. • Need to increase support for services to SIDS families and for SIDS public education. • Need for uniform autopsy and death scene investigation protocols to improve the accuracy of the causes of death in these infants. • Need for a State SIDS Advisory Council to provide input, information, and guidance to the California Department of Public Health on SIDS issues.

15. Boatwright SIDS Bills of 1989 • SB 1067, Boatwright: Police, Firefighter, and Paramedic Training. • SB 1068, Boatwright: Support SIDS services and training. • SB 1069, Boatwright: Standardized autopsy protocol for sudden unexplained infant deaths. • SB 1070, Boatwright: State SIDS advisory council.

16. Boatwright Bill SB-1069 (Chapter 955; Government Code 27491.41) • SIDS definition “the sudden death of any infant which is unexpected by the history of the infant and where a thorough post-mortem examination fails to demonstrate an adequate cause of death.” • Requires an autopsy to be performed within 24-hours of death or as soon as is reasonably possible. • Autopsy to be performed according to a standardized protocol by all Coroners. The legislation made provisions for the standardized protocol to be developed.

17. Boatwright Bill SB-1069 (Chapter 955; Government Code 27491.41) • Uniform autopsy and death scene investigation protocols developed. • Interdisciplinary committee chaired by Doctor Henry Krous. • Hoped it would provide Coroner investigators and pathologists with a roadmap to make appropriate observations. • Hoped it would improve the accuracy and consistency of diagnoses.

19. The majority of SIDS victims have ≥1 Risk Factor 244 SIDS victims from New Jersey, 1996-2000. Ostfeld, B.M., et al. Pediatrics, 125: 447-453, 2010.

20. SIDS in 1990 to the present • The nature of the SIDS victim is changing: • SIDS Risk Reduction campaigns have decreased the number of babies dying. • SIDS babies are often found in unsafe sleeping environments or have other risk factors. • Pathologists, armed with more information from autopsy and death protocols, are making observations which they think may contribute to the death. • Pathologists are less inclined to accept a natural manner of death, and are concerned that accidental causes of death may be present. • SIDS diagnosis is used less frequently.

21. Infant Vulnerability Hannah Kinney SIDS Development Environment Filiano, J.J., and H.C. Kinney. Biol. Neonate, 65: 194-197, 1994.

22. Spectrum of Infant Deaths Biology Interacts with Environment Known Cause of Death “True” SIDS Clear evidence of suffocation, entrapment, etc. Dx: Accidental Some Risk Factors, but would not cause death in all infants. Dx: Variable No Risk Factors. Dx: SIDS

23. Biology Interacts with Environment Known Cause of Death “True” SIDS V V V SIDS SIDS E SIDS D D E D E

24. Biology Interacts with Environment Known Cause of Death “True” SIDS Clear evidence of suffocation, entrapment, etc. Dx: Accidental Some Risk Factors, but would not cause death in all infants. Dx: Variable No Risk Factors. Dx: SIDS V V V SIDS SIDS E SIDS D D E D E

26. SIDS Summit, 2011 • October 19, 2011, in Studio City. • 32 Coroners and Pathologists from around California attended. • Working conference to explore whether or not it is possible to achieve better consistency between counties on diagnosing the cause and manner of death in babies dying suddenly and unexpectedly. • Attendees voted on cause of death and manner of death on a number of cases, to bring out areas of common ground and of difference.

27. SIDS Summit, 2011 “Pristine SIDS” Baby Some pathologists were persuaded to use “SUID” because it is recommended by the CDCP.

28. SIDS Summit, 2011 • Other cases were reviewed which had a variety of findings. • Complete consensus was not achieved on any case, but those with a positive finding had better agreement. • The conference illustrated the complexity of cases Coroners currently see. • Increased observations revealed more questions. • Difficult to come to a definitive diagnosis. • Achieve “probable cause” or “certainty”?

29. SIDS Summit, 2011 • Coroners and medical examiners face considerable challenges in determining the cause and manner of death in infants dying suddenly. • While coroners are doing their best to determine the cause of death, certainty is rarely possible. • It will not be possible to achieve consensus on the cause of death for all babies dying suddenly in California. • In the eyes of Coroners, the diagnoses of SIDS, SUDI, SUID, and Undetermined all mean that the cause of death is unknown, and they are used interchangeably.

30. Recommendations • The California SIDS Community … • Should take the lead to educate SIDS parents, public health nurses, and others that these diagnoses mean the same thing. • Specifically, a diagnosis of “Undetermined” is equivalent to a diagnosis of SIDS, and should not be viewed as tainting the death. • This is our responsibility to take the initiative, lead by personal example, and educate others.

31. Recommendations • Coroners should refer all baby deaths to public health departments, whether diagnosed as SIDS, SUDI, Undetermined, etc. • Families of all of these babies are entitled to the same supportive services offered to SIDS families. • County health departments should send public health nurses to visit all of these families to provide support and education.

32. Recommendations • Some parents have interpreted an “Undetermined” diagnosis as tainting the death compared to a “SIDS:” diagnosis. • This is not the intent of Coroners in California, and we need to reinforce this. • It is our responsibility to emphasize that these deaths are similar in the eyes of coroners. • Parents of all of these babies are entitled to the same support, education, empathy, and respect.

33. When theories compete in profusion Then, the experts conclude in confusion, There’ll be flaws in all laws Of this unexplained cause Till the problem is solved by exclusion. Lady Sylvia Limerick SIDS Parent 1976 Russell-Jones, D.L. Arch. Dis. Child., 60: 278-281, 1985.

34. SIDS