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Thomas G. Keens, M.D. Professor of Pediatrics, Physiology and Biophysics

Turkish Thoracic Society 15 th Annual Congress Side-Antalya, Turkey April 13, 2012. Turkish Thoracic Society. Turkish Thoracic Society. Apnea and Sudden Infant Death Syndrome. Thomas G. Keens, M.D. Professor of Pediatrics, Physiology and Biophysics

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Thomas G. Keens, M.D. Professor of Pediatrics, Physiology and Biophysics

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  1. Turkish Thoracic Society 15th Annual Congress Side-Antalya, Turkey April 13, 2012 Turkish Thoracic Society Turkish Thoracic Society Apnea and Sudden Infant Death Syndrome Thomas G. Keens, M.D. 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. Thank You! Refika Hamutcu Ersu, M.D. Division of Pediatric Pulmonology Marmara University Istanbul, Turkey Formerly, Postdoctoral Fellow in Pediatric Pulmonology Children’s Hospital Los Angeles

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

  4. Emergency Responders Sudden Death of an Infant Coroner's Investigation Determination of Cause of Death Autopsy

  5. 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

  6. Sudden Infant Death Syndrome The sudden unexpected death of an infant, under one-year of age, with onset of the fatal episode apparently occurring during sleep, that remains unexplained after a thorough investigation, including performance of a complete autopsy, and review of the circumstances of death and the clinical history. Krous, H.F., J.B. Beckwith, R.W. Byard, T.O. Rognum, T. Bajanowski, T, Corey, E. Cutz, R. Hanzlick, T.G. Keens, and E.A. Mitchell. Pediatrics, 114: 234-238, 2004.

  7. Infant Deaths by Age of Death California 2003 Carrie Florez California 2002-2003 Birth & 2003 Death Statistical Master Files & SUID Database, 2003. California Department of Health Services, MCAH/OFP, September 2005.

  8. Infant Deaths by Race/EthnicityCalifornia 2003 SUID Data Carrie Florez California 2002-2003 Birth & 2003 Death Statistical Master Files & SUID Database, 2003. California Department of Health Services, MCAH/OFP, September 2005.

  9. SIDS Autopsy Findings • No identifiable cause of death. • No signs of severe illness. • No signs of significant stress.

  10. Kinney, H.C., and B.T. Thach. N. Eng. J. Med., 361: 795-805, 2009.

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

  12. Kinney, H.C., and B.T. Thach. N. Eng. J. Med., 361: 795-805, 2009.

  13. 5-HT1A Receptor Binding Density in the Mid-Medulla from SIDS vs Control Hannah Kinney Paterson, D.S., et al. J. Amer. Med. Assoc., 296: 2124-2132, 2006.

  14. P <0.05 P <0.04 Hannah Kinney Duncan, J.R., et al. J. Amer. Med. Assoc., 303: 430-437, 2010.

  15. Brainstem Neurotransmitters in SIDS • 5-HT abnormalities may be developmental in origin. • SIDS victims may have abnormal neurologic control of cardiac, respiratory, and/or arousal function. • Confirms a biological basis for SIDS. • Supports risk reduction strategies. Professor Hannah Kinney. Neuropathologist. Harvard Medical School. Panigrahy, A., et. al. J. Neuropath. Exp. Neurol., 59: 377-384, 2000. Kinney, H.C., et al. J. Neuropath. Exp. Neurol., 60: 228-247, 2001. Kinney, H.C., et al. J. Neuropath. Exp. Neurol., 62: 1178-1191, 2003. Paterson, D.S., et al. J. Amer. Med. Assoc., 296: 2124-2132, 2006. Duncan, J.R., et al. J. Amer. Med. Assoc., 303: 430-437, 2010.

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

  17. 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

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

  19. 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

  20. SIDS AAP Policy Statement. Pediatrics, 128: 1030-1039, 2011.

  21. Supine Prone SIDS Risk Reduction: Curriculum for Nurses, NICHD, 2006. NIH Publication No. 06-6005.

  22. U.S. Prone Sleeping and SIDS Rate 1.5 1.0 SIDS Rate per 1,000 Prone Sleeping (%) 0.5 0 M. Willinger, et al. J. Amer. Med. Assoc., 280: 329-335, 1998. Colson, E.R., et al. Arch. Pediatr. Adolesc Med., 163: 1122-1128, 2009.

  23. Infant Apnea SIDS

  24. Post-neonatal Apnea and SIDS Total (P<0.001) Birthweight (gm) Hoffman, H.J., et al. Ann. N.Y. Acad. Sci., 533: 13-30, 1988.

  25. Apparent Life-Threatening Event(ALTE) An event, which is frightening to the observer, with: • Color change (cyanosis or pallor). • Tone change (limpness). • Apnea. • Requirement for intervention. Kahn, A. Eur. J. Pediatr., 163: 108-115, 2004.

  26. Do all ALTE need to be Hospitalized? • Attempt to identify criteria for safe discharge from ED. • Prospective study of ALTE seen in ED over 3-years. • Information on presentation and outcome obtained. • 59 infants <1-year of age were studied. • 55 were hospitalized. Ilene Claudius, M.D. Claudius, I., and T. Keens. Pediatrics, 119: 679-683, 2007.

  27. Do all ALTE need to be Hospitalized? Critical Outcome Criteria: • Subsequent events requiring resuscitation. • Identified cause of ALTE requiring hospitalization. • Diagnosis that would have put the child at risk if discharged (i.e., sepsis, child abuse). • Development of life-threatening condition (i.e., hypoxia). Ilene Claudius, M.D. Claudius, I., and T. Keens. Pediatrics, 119: 679-683, 2007.

  28. Do all ALTE need to be Hospitalized? • 8 infants (14%) had Critical Outcomes, and should have been hospitalized. • 3 multiple apneas. • 2 required treatment for infection or neurologic problem. • 2 required PICU care. • 1 developed hypoxia. • All were hospitalized. Ilene Claudius, M.D. Claudius, I., and T. Keens. Pediatrics, 119: 679-683, 2007.

  29. Do all ALTE need to be Hospitalized? • None of the remaining 51 infants had Critical Outcomes. • 47 of these were hospitalized. • 4 of these were not hospitalized. • None had serious sequelae. • These results suggest that some ALTE need not be hospitalized, but how do you predict which ones? Ilene Claudius, M.D. Claudius, I., and T. Keens. Pediatrics, 119: 679-683, 2007.

  30. Do all ALTE need to be Hospitalized? Admitting all infants age <1-month and/or who had multiple ALTE included all infants who had critical outcomes. Ilene Claudius, M.D. Claudius, I., and T. Keens. Pediatrics, 119: 679-683, 2007.

  31. When an ALTE Infant is Hospitalized • Continuous cardiorespiratory monitoring and/or pulse oximetry. • Preferably with memory capability. • Diagnostic evaluation to identify medical cause for the ALTE. • No cookbook diagnostic evaluation. • Diagnostic testing should be individualized. • 50%-70% of ALTE can be explained. Kahn, A. Eur. J. Pediatr., 163: 108-115, 2004.

  32. Most Common Causes of ALTE % Kahn, A. Eur. J. Pediatr., 163: 108-115, 2004.

  33. Management of ALTE • When specific cause for ALTE is found, treat the specific cause. • Respiratory stimulants (methylxanthines) are not effective and have side effects. • Home apnea-bradycardia monitoring is used most commonly when a specific cause can not be found. • No universally accepted indications for home monitoring. Kahn, A. Eur. J. Pediatr., 163: 108-115, 2004.

  34. Home Apnea Bradycardia Monitors Can Detect Central Apnea Flow Rib Cage Abdomen Time

  35. Home Apnea Bradycardia Monitors Can Not Detect Obstructive Apnea Flow Rib Cage Abdomen Time

  36. Home Apnea Bradycardia MonitorAlarm Thresholds

  37. Instructions to Monitoring Parents • Monitor when sleeping and whenever the baby is otherwise unobserved. • Caregivers must be trained in infant CPR and graded response to monitor alarms. • Must be able to hear the alarm (No shower, vacuum, loud stereo if alone). • Trained babysitters and child care.

  38. Graded Response to Monitor Alarms

  39. Inborn Errors of -Oxidation of Fatty Acids • Rare cause of ALTE, but more likely if: • Apneas persist --- do not resolve in 3-months. • Severe apneas --- require resuscitation. • Family history of consanguinity, ALTE, seizures, SIDS, or other infant deaths. • Serum ammonia elevated in all cases. • 4% of infants with severe ALTE. Arens, R., et al. J. Pediatr., 123: 415-418, 1993.

  40. When to discontinue HomeApnea-Bradycardia Monitoring • No true alarms requiring intervention for 2-months. • 6-weeks since the last true alarm requiring intervention. • Testing is not helpful. Ramanathan, R., and CHIME. J. Amer. Med. Assoc., 285: 2199-2207, 2001.

  41. CHIME Steering Committee, NICHD Bethesda, Maryland, U.S.A. July, 1992.

  42. The CHIME Home Monitor • Respiratory Inductance Plethysmography. • Central and Obstructive Apneas. • Electrocardiogram. • Pulse Oximeter. • Body Position. • Computer to record events and normative data. Neuman, M.R., et al., and CHIME. Physiol. Meas., 22: 267-286, 2001. Ramanathan, R., and CHIME. J. Amer. Med. Assoc., 285: 2199-2207, 2001.

  43. Neuman, M.R., et al., and CHIME. Physiol. Meas., 22: 267-286, 2001.

  44. CHIME Study -- Event Definitions Neuman, M.R., et al., and CHIME. Physiol. Meas., 22: 267-286, 2001. Ramanathan, R., and CHIME. J. Amer. Med. Assoc., 285: 2199-2207, 2001.

  45. CHIME -- Conventional Events 6,958 Conventional Events in 444 of 1,079 infants (41%) 4,937 (78%) 6,958 769 (12%) Ramanathan, R., and CHIME. J. Amer. Med. Assoc., 285: 2199-2207, 2001.

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