DEPARTMENT

Practice Guidelines

Sudden Infant Death Syndrome Polina Gelfer, MD, & Michelle Tatum, MSN, BSN, RN

KEY WORDS Infant death, SIDS

BACKGROUND Sudden infant death syndrome (SIDS) is defined as the sudden, unexpected death of an infant before 1 year of age that cannot be explained after a thorough case investigation, including a scene investigation, autopsy, and review of the clinical history

Section Editors Robert J. Yetman, MD Corresponding Editor University of Texas School of Medicine Houston, Texas Polly F. Cromwell, MSN, RN, CPNP Yale-New Haven ChildrenÕs Hospital Bridgeport Hospital Campus Bridgeport, Connecticut Polina Gelfer, Assistant Professor, Neonatal-Perinatal Division, Department of Pediatrics, University of Texas at Houston, Houston, TX. Michelle Tatum, Clinical Nurse Educator, Neonatal Intensive Care Unit, Children’s Hermann Memorial Hospital, Houston, TX. Conflicts of interest: None to report. Correspondence: Polina Gelfer, MD, Neonatal-Perinatal Division, Department of Pediatrics, University of Texas at Houston, 6431 Fannin St, MSB 3.020, Houston, TX 77030; e-mail: polina.gelfer@ uth.tmc.edu. J Pediatr Health Care. (2014) 28, 470-474. 0891-5245/$36.00 Published by Elsevier Inc. on behalf of the National Association of Pediatric Nurse Practitioners. Published online June 16, 2014. http://dx.doi.org/10.1016/j.pedhc.2014.04.007

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(Willinger, James, & Catz, 1991). In the United States, about 2,100 infants die of SIDS each year (Mathews & MacDorman, 2013). Risk factors for SIDS have been identified through epidemiologic studies, which demonstrate a strong correlation between infant sleep position, sleeping environment, and SIDS (American Academy of Pediatrics [AAP], 2005). The Back to Sleep campaign started in 1994 with the goals of spreading safe sleep messages within communities and educating caregivers and health care providers on strategies to reduce the incidence of SIDS. The accomplishment of the campaign has been associated with more than a 50% decline in deaths from SIDS since the beginning of the campaign until 2000, but during the past decade, the SIDS rate has remained constant (Centers for Disease Control and Prevention, 2006). Despite the success of the Back to Sleep campaign, SIDS remains the third-leading cause of infant mortality in the United States (Mathews & MacDorman, 2013), and the majority of these deaths still occur when safe sleep recommendations are not followed (Li et al., 2003; Texas Department of State Health Services, 2011; Trachtenberg, Haas, Kinney, Stanley, & Krous, 2012). The expanded campaign, called ‘‘Safe to Sleep,’’ was launched by the National Institute of Child Health and Human Development in 2012. The goal of the campaign is to continue to incorporate new and evolving science-based information on safe infant sleep. As part of their activities, Safe to Sleep campaign personnel revised the educational materials to include the latest AAP recommendations. The newly expanded campaign created the Champion initiative to enlist people across the United States to help share safe sleep messages in their local areas. Safe to Sleep Champions are spokespersons for the campaign who are reaching out to the media and their community, as well raising awareness about the campaign and distributing key information about SIDS and other sleep-related causes of infant death (Safe to Sleep, 2013). Journal of Pediatric Health Care

HISTORY AND RISK FACTORS Epidemiology  The highest risk is between 2 to 4 months (91% of deaths occur between 1 and 6 months of age).  Preterm and low-birth-weight infants are at increased risk.  SIDS has a seasonal trend, with more deaths happening in winter months (overheating may play a role). Demographic Risk Factors  Racial and ethnic disparities: African American and American Indian/Alaska native infants have the highest risk.  SIDS is more likely to occur in male infants at a 3:2 ratio. Maternal Risk Factors  Teenage mother (< 20 years old)  Low educational level  Lower socioeconomic status  Poor prenatal/postnatal care  Maternal smoking during pregnancy and after birth  Use of illicit substances Sleeping Position and Sleeping Environment  Prone and side sleeping: The prone sleep position has been recognized as a major risk factor for SIDS. A side sleep position also confers The prone sleep an increased risk position has been (Hauck et al., 2010; Li et al., recognized as a 2003). The side major risk factor for position is not SIDS. very stable; it is easier for the infant to roll to the prone position from the side then from the back (AAP, 2005, 2011).  Infants who were usually placed supine but were placed on their side or prone for the last sleep (i.e., unaccustomed prone sleeping) are at an even higher risk of SIDS (Li et al., 2003).  Use of soft bedding or pillows in close proximity to the sleeping infant is a risk factor for SIDS (AAP, 2011).  Overheating: The risk of SIDS may be associated with the amount of clothing on an infant, the room temperature, and the season of the year. It is unclear whether extra clothing and a cold climate are independent factors or a reflection of the use of more potentially asphyxiating soft objects in the sleeping environment during the cold weather.

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 Co-sleeping with an adult or another child: The risk of SIDS seems to be particularly high when an infant is co-sleeping with multiple people and may increase when the bed sharer is under the influence of alcohol or is overtired. In contrast, room sharing without bed sharing was associated with a reduced risk of SIDS (AAP, 2005).  Environmental tobacco smoke exposure Triple-Risk Model The triple-risk model is the interplay of multiple factors that lead to SIDS.  A vulnerable infant  A critical period of development (0 to 12 postnatal months)  Exogenous stressors (sleeping position and sleeping environment) EVALUATION Physical Examination SIDS is a diagnosis of exclusion, and a thorough postmortem examination, the infant’s clinical history, the family history, and an evaluation of the death scene should fail to demonstrate an adequate cause of death. Investigators must inquire about previous nutritional or developmental abnormalities, previous unexpected sibling death, history of domestic violence, and involvement with Child Protective Services or law enforcement. A full skeletal survey is performed to detect possible fractures, particularly metaphyseal and rib fractures, which are suspicious signs for child abuse. Death Scene Investigation The death scene investigation is pivotal for delineating factors that may have contributed to the infant’s death. As part of the scene investigation, families and other relevant witnesses are interviewed in an effort to reconstruct the events prior to the infant’s death. Questions include at what time the infant was put to sleep, in what position the infant was placed, and in what position the infant was found. A re-enactment using a doll to represent the infant can be helpful. The scene investigation is best performed soon after the infant’s death so that bedding is still in place. Bedding should be examined and photographed because the presence of blood-tinged infant secretions on sheets may indicate evidence of airway occlusion (Berkowitz, 2012). Autopsy An autopsy is performed on all infants who die suddenly to eliminate any other possible cause of death. As part of the procedure, the eyes are evaluated for the presence of retinal hemorrhages. Blood samples

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should be sent for toxicology and electrolyte levels (Berkowitz, 2012). Differential Diagnosis  Trauma/child abuse: Comprehensive physical examination, radiographic skeletal surveys, and toxicology studies performed before the autopsy may help to reveal evidence of child abuse. Common postmortem findings in cases of child abuse are cranial injuries, abdominal trauma (e.g., liver laceration, organ perforations, or hematomas), burns, or skeletal fractures. All sites of suspected skeletal injury require additional investigations through high-detail specimen radiography or histologic analysis (Hymel, 2006). It is difficult to distinguish between SIDS and accidental or deliberate suffocation with a soft object (Hymel, 2006). Certain circumstances could point out the possibility of intentional suffocation, which include: recurrent cyanosis or apnea happening only while in the care of the same caregiver, age of death older than 6 months, previous unexpected or unexplained death of another sibling, concurrent deaths of twins, previous death of infants under the care of the same caregiver, or evidence of an old pulmonary hemorrhage on autopsy (Hymel, 2006).  Inborn errors of metabolism: Fatty acid metabolism disorders such as medium chain acylcoenzyme A dehydrogenase deficiency and very long-chain acyl-coenzyme A dehydrogenase deficiency are associated with sudden unexpected death in the neonatal period. A history of neonatal deaths of previous siblings, although a concern for child abuse, should also initiate a search for possible inborn errors of metabolism.  Certain genetic conditions: As proposed by the triple-risk model, certain genetic conditions may occur with an increased frequency in certain populations, accounting for the increased prevalence of SIDS within that group. For example, Alaska natives have a high incidence of abnormal variant of gene coding for carnitine palmitoyltransferase, and the rate of infant deaths in those infants was found to be the highest compared with infants without the variant (Berkowitz, 2012).  Infection: Some research studies proposed that severe gene polymorphism may affect the immune response in the presence of a mild infection and may also play a role in the infant’s unexpected death. The data are equivocal and require additional investigations (Berkowitz, 2012).  Cardiac channelopathies: Prolonged QT syndrome may account for 5% to 10% of sudden unexpected infant deaths, but identification of the condition in a noninvasive, cost-effective way prior to death is challenging (Berkowitz, 2012). 472

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SIDS RISK REDUCTION STRATEGIES Sleeping Practice  The supine position should be used during every sleep by every caregiver until 1 year of life. Side sleeping should not be advised because it is not safe (AAP, 2011). No evidence indicates that the supine sleep position increases the risk of choking or aspiration (Tablizo et al., 2007).  A crib, bassinet, or portable crib/play yard that conforms to the safety standards of the Consumer Product Safety Commission should be used.  Use of a firm sleep surface: A firm crib The crib should be mattress should be free from loose used, covered by a fitted sheet. bedding and soft  The crib should be objects, such as free from loose extra blankets, bedding and soft objects, such as pillows, or toys, extra blankets, pilbecause of the risk lows, or toys, beof asphyxiation. cause of the risk of asphyxiation.  Avoid overheating. The infant should be dressed appropriately for the environment, usually with only one additional layer more than an adult would wear to be comfortable in that environment.  Consider offering a pacifier at nap and bedtime. Use of the pacifier should not be forced if the infant refuses it. Data suggest that a pacifier may decrease SIDS risk. The protective effect was not associated with finger sucking behavior (AAP, 2011).  Sitting devices, such as car seats, strollers, swings, and infant carriers, are not recommended for routine sleep. Infants who fall asleep in these devices should be moved to the supine position in an appropriate sleeping environment as soon as possible.  Room sharing without bed sharing is recommended. The infant’s crib should be placed in the parents’ bedroom close to the parents’ bed.  Use of devices promoted to make bed sharing protected (e.g., in bed co-sleepers, wedges, or positioners) is not recommended (AAP, 2011). Feeding Practices  Breastfeeding is recommended and associated with a reduced risk of SIDS. The protective effect of breastfeeding increases with exclusivity (AAP, 2011). Health Maintenance  Pregnant women should receive regular prenatal care. Evidence links a higher risk of SIDS for infants whose mothers did not receive prenatal care (Getahun, Amre, Rhoads, & Demissie, 2004). Journal of Pediatric Health Care

 Parents should be counseled on the association between smoke exposure, alcohol, illicit drug use during pregnancy and after birth, and SIDS.  Infants should be immunized in accordance with AAP recommendations. No evidence exists that links immunizations to SIDS. Indeed, data suggest that immunizations may have a protective effect against SIDS (Fleming, Blair, Platt, Tripp, & Golding, 2001).  Supervised, awake tummy time is recommended. It will facilitate development and decrease positional plagiocephaly. Home Monitoring  Home cardiorespiratory monitors should not be used as a strategy to reduce the risk of SIDS. Although these monitors can be used to detect apnea or bradycardia, no evidence has shown that use of such devices decreases the incidence of SIDS. In addition, no evidence exists that routine in-hospital cardiorespiratory monitoring can identify infants at risk of SIDS (AAP, 2011). CLINICIANS’ ROLE IN PARENTAL EDUCATION Health care professionals, staff in hospital nurseries, and all health care providers should receive education on safe infant sleep and endorse the SIDS riskreducing recommendations from birth (AAP, 2011). Parental knowledge and acceptance of safe sleep practices is key to decreasing the risk of SIDS. Although parents learn from multiple sources, including their family, friends, and the media, studies have indicated that neonatal nurses and other health care providers play critical roles in parental education. The way infants are positioned in the hospital strongly influences parental practices at home (Colson & Joslin 2002; Gelfer, Cameron, Masters, & Kennedy, 2013; Vernacchio et al., 2003). Safe sleep practices and SIDS reduction strategies are typically introduced to parents in the hospital as part of routine newborn care. However, a significant knowledge gap between SIDS prevention measures and their implementation exists among clinicians. Hospital surveys showed that about 90% of nurseries are using the side-sleeping position as an acceptable practice, and only 42% to 64% of nurses (depending on the component of safe sleeping practices) identified themselves as always following SIDS prevention recommendations when preparing infants for discharge (Grazel, Phalen, & Polomano, 2010; Hein & Pettit, 2001). Another survey revealed that just half of neonatal nurses advised parents to place their infants exclusively in the supine position for sleep after discharge (Aris et al., 2006). Nurses should always practice safe sleep practices while caring for infants prior to discharge and provide

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appropriate education for their patients. Moreover, preterm infants require special attention. In its updated recommendations from 2011, the AAP stressed that neonatal intensive care nurseries should endorse and model the SIDS risk-reduction recommendations as soon as the infant is medically stable and significantly before the infant’s anticipated discharge, by 32 weeks’ postmenstrual age (AAP, 2011). SIDS education should continue at all infant health care visits, whether well child care or sick visits, until the infant is 1 year of age. Indeed, improvement is required in this area. During one cross-sectional survey, almost all clinicians admitted to the imporSIDS education tance of SIDS educashould continue at tion, but 30% reported not discussing it with all infant health care their patients at all visits, whether well (Eron et al., 2011). To child care or sick continue to decrease the SIDS rate, health visits, until the care professionals infant is 1 year of must take responsibilage. ity for educating themselves and caregivers about safe sleep practices and SIDS-reducing strategies. They should also become leaders in promoting breastfeeding and smoking secession. The ultimate goal is for all infants to sleep in a safe sleep position and environment and to lessen the SIDS death rate even further. REFERENCES American Academy of Pediatrics, Task Force on Sudden Infant Death Syndrome. (2005). The changing concept of sudden infant death syndrome: Diagnostic coding shifts, controversies regarding the sleeping environment, and new variables to consider in reducing risk. Pediatrics, 116, 1245-1255. American Academy of Pediatrics, Task force on Sudden Infant Death Syndrome. (2011). SIDS and other sleep-related infant deaths: Expansion of recommendations for a safe infant sleeping environment. Pediatrics, 128(5), 1030-1039. Aris, C., Stenes, T., LeMura, C., Lipke, B., McMullen, S., Cote-Arsenault, D., & Consenstein, L. (2006). NICU nursesÕ knowledge and discharge teaching related to infant sleep position and risk of SIDS. Advances in Neonatal Care, 6(5), 281-294. Berkowitz, C. (2012). Sudden infant death syndrome, sudden unexpected infant death, and apparent life-threatening events. Advances in Pediatrics, 59, 183-208. Centers for Disease Control and Prevention, National Center for Health Statistics. (2006). National Infant Sleep Position Study. Retrieved from http://www.nichd.nih.gov/SIDS/Documents/ SIDS_rate_back_sleep_2006.pdf Colson, E., & Joslin, S. (2002). Changing nursery practice gets innercity infants in the supine position for sleep. Archives of Pediatric & Adolescent Medicine, 156(7), 717-720. Eron, N., Dygert, K., Squillace, C., Webster, N., Andrianos, A., Crockett, E., & Consenstein, L. (2011). The physicianÕs role in reducing SIDS. Health Promotion Practice, 12(3), 370-378. Fleming, P., Blair, P., Platt, M., Tripp, J., & Golding, J. (2001). The UK accelerated immunization programme and sudden unexpected death in infancy: Case-control study. BMJ, 322(7290), 822.

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Gelfer, P., Cameron, R., Masters, K., & Kennedy, K. (2013). Intergrating ‘‘Back to Sleep’’ recommendations into neonatal ICU practice. Pediatrics, 131, e1264. Getahun, D., Amre, D., Rhoads, G., & Demissie, K. (2004). Maternal and obstetric risk factors for sudden infant death syndrome in the United States. Obstetrics & Gynecology, 103(4), 646-652. Grazel, R., Phalen, A., & Polomano, R. (2010). Implementation of the American Academy of Pediatrics recommendations to reduce sudden infant death syndrome risk in neonatal intensive care units: An evaluation of nursing knowledge and practice. Advances in Neonatal Care, 10(6), 332-342. Hauck, F., Moore, C., Herman, S., Donovan, M., Kalelkar, M., Christoffel, K., . Rowley, D. (2010). The contribution of prone sleeping position to the racial disparity in sudden infant death syndrome, the Chicago infant mortality study. Pediatrics, 110, 772-780. Hein, H., & Pettit, S. (2001). Back to Sleep: Good advice for parents but not for hospitals? Pediatrics, 107, 537-539. Hymel, K. (2006). Distinguishing sudden infant death syndrome from child abuse fatalities. Pediatrics, 118, 421-427. Li, D., Petitti, D., Willinger, M., McMahon, R., Odouli, R., Vu, H., & Hoffman, H. (2003). Infant sleeping position and the risk of sudden infant death syndrome in California, 1997-2000. American Journal of Epidemiology, 157(5), 446-455.

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Mathews, T. J., & MacDorman, M. F. (2013). Infant mortality statistics from the 2010 period linked birth/infant death data set. National Vital Statistics Reports, 62(8). Retrieved from http://www.cdc. gov/nchs/data/nvsr/nvsr62/nvsr62_08.pdf Safe to Sleep. (2013). Public Educational Campaign. Retrieved from http://www.nichd.nih.gov/sts/Pages/default.aspx Tablizo, M., Jacinto, P., Parsley, D., Chen, M., Ramanothan, R., & Keens, T. (2007). Supine sleeping position does not cause clinical aspiration in neonates in hospital newborn nurseries. Archives of Pediatric & Adolescent Medicine, 161(5), 507-510. Texas Department of State Health Services. (2014). Maternal & Child Health—Texas Child Fatality Review. Retrieved from http:// www.dshs.state.tx.us/mch/child_fatality_review.shtm Trachtenberg, F., Haas, E., Kinney, H., Stanley, C., & Krous, H. (2012). Risk factor changes for sudden infant death syndrome after initiation of Back-to-Sleep campaign. Pediatrics, 129(4), 630-638. Vernacchio, L., Corwin, M., Lesko, S., Vezina, R., Hunt, C., Hoffman, H., . Mitchell, A. (2003). Sleep position of low birth weighted infants. Pediatrics, 111(3), 633-640. Willinger, M., James, L., & Catz, C. (1991). Defining the sudden infant death syndrome (SIDS): Deliberations of an expert panel convened by the national Institute of Child health and Human Development. Pediatric Pathology, 11(5), 677-684.

Journal of Pediatric Health Care

Sudden infant death syndrome.

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