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Bundock EA, Corey TS, Andrew TA, et al., editors. Unexplained Pediatric Deaths: Investigation, Certification, and Family Needs [Internet]. San Diego (CA): Academic Forensic Pathology International; 2019.
JACOB
“Jacob was 2 years old with a 5-year-old loving big sister at the time of his death. ‘Jakey’ was vibrant, sweet, kind and so happy. He loved playing hide and seek, which we called ‘Dragon,’ where he would hide together with his sister while his mom and I would take our time trying to find them. Once we did, we would scare them with our dragon roar. They would scream and then all four of us would burst out laughing. His wispy white blond hair would catch everyone’s eye when he walked into a room and his smile was so big that his eyes would close when he flashed you a good one. It was a foggy and surreal time, but I remember the police and first responders gave us space and time to hold Jacob before they took him away. We are forever grateful for their compassion in those dark early hours.”
– Jacob’s Dad
OUTLINE.
Prevention efforts for sudden unexpected deaths in childhood have almost exclusively been based on identification and avoidance or amelioration of risk factors. Risk factors for sudden unexpected death in infants (i.e., <365 days of age) have been most thoroughly studied, and best practices for decreasing risk of these deaths include avoidance of these identified risk factors. There is less clarity about the risk factors and prevention strategies for sudden unexpected deaths in children after the first year of life. Infants should always sleep in the supine position, in a safety-approved crib located next to the parents’ bed, without bedding, and in a smoke-free environment. Infants should be breastfed; a pacifier can be offered at sleep time after breastfeeding has been established. Smoking, drinking alcohol, and consumption of illicit drugs during pregnancy should be avoided. The infant should not be exposed to secondhand smoke. In addition, the infant should not sleep on the same surface as others, especially when any of the others are smokers, have ingested alcohol, or have consumed illicit drugs or arousal-altering medications. Infants should never sleep on cushioned surfaces, including sofas, couches, and armchairs.
This chapter will discuss the latest evidence regarding risk factors and prevention strategies, including genetic, metabolic, and cardiac evaluation for specific categories of sudden unexpected deaths. Topics that will be discussed include prevention of sudden unexpected deaths in infants, athletes, children with epilepsy, children with febrile seizures, and siblings/relatives of infants and children who have died suddenly and unexpectedly.
Best practices for decreasing risk of sudden unexpected infant deaths include avoidance of known risk factors. The American Academy of Pediatrics’ most recent recommendations, published in 2016, state that infants should always sleep in the supine position, in a safety-approved crib located next to the parents’ bed, without bedding, and in a smoke-free environment (“Alone, on their Back, in their own Crib”) (1). Infants should be breastfed; a pacifier can be offered at sleep time after breastfeeding has been established. Smoking, drinking alcohol, and consumption of illicit drugs during pregnancy should be avoided. The infant should not be exposed to secondhand smoke. In addition, the infant should not sleep on the same surface as others, especially when any of the others are smokers, have ingested alcohol, or have consumed illicit drugs or arousal-altering medications. Infants should never sleep on cushioned surfaces, including sofas, couches, and armchairs. Examples of safe sleep environments are shown in Image 14.1.
Infants should always sleep on their back, in their own crib or sleep-approved area, with a firm surface, without soft objects, bumpers, or loose bedding, and in a smoke-free environment (2).
There are approximately 3700 infants who die suddenly and unexpectedly during sleep each year in the US (3). While the 1994 “Back to Sleep” public awareness campaign and the subsequent “Safe to Sleep” campaign have been associated with declining rates of prone infant sleeping and reductions in mortality rates, these decreases have plateaued since 2000 (3). Some caregivers, including parents, relatives, child care providers, and health care professionals, continue to resist adoption of safe infant sleep recommendations, such as placing infants supine, avoidance of smoke exposure, avoidance of parent-infant bed-sharing, and avoidance of soft bedding (including blankets, pillows, and bumper pads). The rate of prone positioning remains at approximately 30% (4), bed-sharing rates have doubled (5), and rates of soft bedding use remain at approximately 50% (6).
Information about barriers to safe sleep practices comes largely from qualitative studies. Parents and caregivers frequently express concerns about the infant’s comfort (usually inferred from the duration and perceived quality of infant sleep) and safety (7, 8). Thus, parents are more likely to place infants prone, sleep with their infants, and use soft bedding to improve perceived infant comfort. If parents worry about the infant being uncomfortable because of the cold air, blankets and comforters are often used (9). With regards to the infant’s safety, parents worry that infants will choke or aspirate if placed supine (7, 8) or will injure themselves on the crib sides if bumper pads are not used (9). Bed-sharing facilitates breastfeeding, and some parents view bed-sharing as a time to bond with infants; in addition, parents often view bed-sharing as a strategy to maintain vigilance and ensuring the infant’s safety while sleeping (10). Parental fatigue must not be underestimated as a barrier to safe sleep practices. In their desperation for longer sleep, tired parents will often place their infants prone and bedshare as strategies to sleep for longer duration (8, 10). Financial considerations also are important in that some parents may bedshare with the infant if there is no crib or limited space/room(s) in the home.
Barriers to breastfeeding are well documented and include perceived inadequate supply, societal norms about breasts as sexual organs, and lack of support from family, friends, and coworkers (11).
Social and cultural norms with regards to safe sleep practices can be barriers or facilitators of safe sleep practices. If trusted sources, including senior family members, partners, and health care professionals, encourage safe sleep practices, there is a higher likelihood of adherence (4, 11).
Although randomized controlled trials, in which participants are randomly assigned to an intervention or control, are the gold standard for assessing effectiveness of interventions, they are not often used in studies of sudden unexpected pediatric deaths because they are expensive and time-consuming to conduct and because of ethical considerations (e.g., one cannot randomize infants to sleep supine or sleep prone). Therefore, most sudden unexpected pediatric deaths interventional studies are observational studies; these are limited in that there may be confounding factors, and it is difficult to attribute causality to the intervention. Finally, most interventions use self-report measures as outcomes and are, thus, limited by how forthcoming the participants are about their actual practices, particularly when these practices are inconsistent with recommended practice.
Randomized controlled trials testing improved messaging that focuses on known barriers to safe sleep practices have shown improvement in parental knowledge, attitudes, and intention with regards to supine positioning (12, 13). Hospital quality improvement programs, aimed at improving staff safe sleep practices and counseling, have shown improvements in hospital (14) and parental self-reported practices (15). One intervention that provided free portable cribs and safe sleep education to low-income families found that parental knowledge about sleep position and intended use of supine positioning increased, while bed-sharing the night prior decreased (16). A Scottish randomized control trial randomized pregnant smokers to receive usual care only or usual care plus a maximum of 400 British pounds for achieving specific smoking cessation goals. In this study 23% of intervention participants quit smoking, compared with 9% of control participants (17). In New Zealand, sudden unexpected death in infancy rates are amongst the highest for developed countries (18); sudden unexpected death in infancy rates are particularly high in the indigenous population (19) and these deaths are often associated with bed-sharing (19). A randomized control trial evaluating use of the wahakura, a Maori (indigenous) low-sided (6 inches tall) infant bed woven from flax, which is usually placed in the adult bed next to the parent as a separate infant sleep space within the adult bed, found that rates of use, quantity and quality of maternal sleep, breastfeeding, and head covering events were similar for the wahakura and a standard bassinet placed next to the parents’ bed or in the parents’ room (20). A recent US four-armed randomized control trial tested two separate, complementary interventions (nursing quality improvement and mobile health intervention) and found that, compared with mothers who received a control intervention, mothers who received the mobile health intervention with regular text or email messages and safe sleep videos reported higher rates of placing their infant supine, room sharing without bed-sharing, no soft bedding use, and any pacifier use. The nursing quality improvement intervention alone did not impact outcomes but was synergistic with the mobile health intervention for supine sleep (21).
A sudden, unexpected death in a child athlete, while uncommon, is often widely publicized. It is an anathema to see a vibrant, strong, and energetic youth with a promising future die. Physical activity, especially organized athletics starting in youth, is strongly encouraged and promotes numerous psychological and social advantages. Athletics is often part of a healthy lifestyle and encouraged in the fight against the obesity epidemic.
For most chronic health conditions, physical activity and athletics are encouraged and likely do not present an unacceptably high risk of morbidity or mortality. The American Academy of Pediatrics Policy Statement on “Medical Conditions Affecting Sports Participation” outlines medical conditions, diagnoses, and possible contributing situational factors which require consideration before counseling and qualifying children for sports participation (22, 23). The risk of sudden cardiac death during athletics is generally accepted to be increased for certain cardiac conditions, such as hypertrophic cardiomyopathy and long QT syndrome, for which guidelines for sports participation are outlined (24). The importance of well-informed, shared decision making for participation in athletics (and to what extent) and respect for patient and family autonomy is highlighted in policies and guidelines.
Knowledge gaps on the incidence of sudden death for prepubertal athletes during and shortly after athletic participation or exercise persist in part due to a lack of standardized postmortem procedures. Procedures for investigating, classifying, and reporting these sudden deaths of young athletes vary across the US. Mandatory regional and national event reporting structures could further our understanding of the risk factors and conditions involved and would allow for more targeted preventive measures and interventions.
There is ongoing discussion and controversy about which measures should be used to try to prevent sudden death in young athletes and their cost effectiveness (25–32). Barriers to prevention strategies include identification of those children or potential child athletes at greater risk of sudden death and identification of the interventions most likely to significantly reduce risk without causing undue harm. Electrocardiogram screening for asymptomatic youth is cost-effective in most settings but research has not thoroughly addressed the harm of false-positives and false negatives (25–27, 32–34).
Primary prevention of sudden death in the young athlete begins with a pre-participation physical examination, for those 12 years and older, this should include cardiovascular screening, even though no nationwide requirement for this exists (26, 29). The objective of cardiovascular screening is to detect silent cardiovascular abnormalities that can lead to sudden death (35). The Pre-Participation Physical Evaluation, Fourth Edition (PPE-4), is not used by all secondary school athletic programs in the US and, when used, is not always used in its entirety (35, 36). Expansion of pre-participation physical evaluations and cardiovascular screenings to include younger athletes and organized athletics in all its various forms has the potential to improve identification of high-risk conditions with little cost. Child athletes with certain cardiac diagnoses can decrease their risk of sudden death by following best practice recommendations, such as limitation or modification of sports participation, adherence to prescribed medical therapy, and prophylactic lifestyle restrictions (24, 37).
Prompt identification of children at increased risk for sudden death is critical. Immediate medical assessment by appropriately trained providers should be required for any child who experiences syncope or seizure during exercise or excitement, for children who have consistent or unusual chest pain and/or shortness of breath during exercise, and for children found to have a family history of an unexpected, unexplained sudden death in the young (38). Standardization of the postmortem cardiac diagnostic procedures used in cases of sudden death in the young, including provision of economic support for the genetic/metabolic tests needed to identify heritable causes, would help identify families in need of further testing. A standard approach to cascade screening of the surviving family members would lead to prompt identification of immediate risk and improved access to potentially life-saving therapies (39–46).
Improved understanding of the clinical characteristics of sudden death in the young can inform best practices for preventive clinical methods as well as strategic allocation of prevention resources in the community (47, 48). School-based automated-external defibrillator (AED) programs have had particularly strong support, likely due to the social prominence of sporting events and community support for prevention programs (49–51). Increasing cardiopulmonary resuscitation (CPR) training provided to the community has been shown to more than double bystander CPR rates and more than triple survival rates from out-of-hospital cardiac arrest (52). Despite the rarity of an event, school programs aimed at training children on cardiopulmonary resuscitation/automatic external defibrillator use appear to be effective and should be supported and promoted (53–55).
Febrile seizures in infants and children (which is not a form of epilepsy) are relatively common, occurring in 2 to 4% of all children under the age of five years (56–61). Febrile seizures occur in association with an elevated temperature without a history of nonfebrile seizures or epilepsy and without central nervous system or systemic inflammation or metabolic abnormality. Typically, they occur after the age of six months and before the age of five years. They may be associated with a viral infection, particularly human herpesvirus type 6 (62). There is a genetic susceptibility to febrile seizures, although the exact inheritance mechanism is unknown (63), and various genetic loci are implicated. Febrile seizures are classified as simple when they last less than 15 minutes, do not recur in a 24-hour period, and are tonic-clonic seizures. Complex seizures are those that are prolonged or recurrent, often with a focal onset. Although long-considered clinically innocuous with a favorable prognosis, febrile seizures carry a small risk for sudden death particularly when complex (64, 65). The mechanism of sudden death in children after febrile seizures and children and adults with epilepsy after afebrile seizures may be very similar.
Little is known about the small subset of the pediatric population who suffer sudden death after a febrile seizure. Knowledge gaps abound. In addition to performing the investigation elements detailed elsewhere in this publication, inquiring about the child’s history or the family’s history of febrile seizures is essential. As with any sudden infant death, a thorough scene investigation, including doll reenactment and documentation of the sleep environment should be performed when there is a sudden sleep-related death in a young child. Careful history should be taken with respect to febrile or other seizures occurring in the child, including description of the seizure and any medical or other follow-up. Family history of seizures should also be obtained.
Deaths following a febrile seizure or associated with a febrile seizure history are poorly understood and likely result from mechanisms different and potentially unrelated to other causes of sudden pediatric death. Similarities exist in risk factors (prone or unsafe sleeping, hyperthermia, infections, prematurity, exposure to tobacco smoke, and male gender) (64), and anatomic findings are frequently, but not uniformly, similar (e.g., subtle neuropathologic findings, focal bronchiolitis, or other minor diagnostic findings) (66–70). For those professionals involved in counseling families and sharing information regarding cause of death, it is important to inform families that these are rare tragedies and there are no known prevention strategies.
The state of knowledge with respect to sudden unexpected death in epilepsy (SUDEP) has advanced in the past two decades and there is increased potential to pursue preventive measures. Most publications during this period have focused on education, potential risk factors, and the mechanism of sudden death due to epilepsy (71). However, the current state of knowledge in prevention of sudden death due to epilepsy is limited. A review of literature finds that, although there are a number of proposed preventive measures, none as of yet are proven effective (72–78). Measures proposed to prevent sudden death due to epilepsy include various monitoring arrangements and/or interventions such as sleeping with others in a room or application of monitoring devices, such as automated seizure detection systems or implantable cardiac event recorders (72, 74, 76, 79–81). As there is strong evidence that frequency of generalized tonic-clonic seizures is one of the most significant risk factors for sudden death due to epilepsy, some authors propose lifestyle counseling for the purpose of attaining better medication adherence (71, 79), as well as use of physical stimulation and selective serotonin reuptake inhibitors (SSRI’s) to reduce central hypoventilation (79). In a related vein, recent publications indicate that polytherapy (i.e., treating with more than one antiepileptic medication) is also an important risk factor for sudden death due to epilepsy and, accordingly, some authors advocate therapies specifically tailored to the seizure type and individual epilepsy syndrome (82). A study performed in 2000 by Annegers et al. theorized that vagal nerve stimulation would decrease the incidence of sudden death due to epilepsy (77), and recent work by Granbichler supports this hypothesis (78). There may be other ways to reduce the risk of sudden death associated with epilepsy or febrile seizures. Nocturnal monitoring with a sound monitor, a watch that detects motion and/or autonomic activation, motion detectors under the mattress, or sharing the room with an adolescent or adult could potentially be lifesaving (83, 84). However, the risks of false alarms and caregiver and patient stress must be balanced with the likelihood of benefit when considering these interventions. A recent Cochrane review of several treatments to prevent sudden death due to epilepsy, including early presurgical evaluation for lesions responsible for epilepsy, educational interventions, seizure-monitoring devices, safety pillows, nocturnal supervision, serotonin-selective uptake inhibitors, opiate antagonists, and adenosine antagonists, found that there was very low-quality evidence for the effectiveness of nocturnal supervision and no evidence supporting use of any of the other listed treatments (79).
Thom et al. advise that gaps in our knowledge about preventing sudden death due to epilepsy could be addressed with more rigorous autopsy investigations including specialized neuropathology and cardiac pathology examinations. Such thorough investigations would likely yield more information regarding contributing factors and, thereby, make it possible to develop preventive measures (71). Similarly, other authors argue that research in sudden death due to epilepsy – particularly genetic studies – may lead to effective prevention but will be dependent on collaborative and multidisciplinary efforts between researchers, neurologists, cardiologists, and forensic pathologists (81). In this regard, the role of the forensic pathologist is crucial as they alone engage in the collection of postmortem specimens invaluable for research analysis (71, 81). To that end, it is important to pursue change in forensic pathology practice, specifically 1) routine collection and storage of biospecimens and 2) ethical and compassionate discussion with the family regarding potential genetic testing and research (71).
Given that research funding for public health initiatives and prevention programs is tied to public and governmental awareness of sudden death due to epilepsy, neurologists, cardiologists, and forensic pathologists are key in raising the profile of sudden death due to epilepsy (85). Neurologists and cardiologists are instrumental in educating their patients and patients’ families regarding sudden death due to epilepsy and its risk factors; forensic pathologists are critical in recognizing and diagnosing sudden death due to epilepsy and, in doing so, are responsible for the extent to which these deaths are reported in statistical and epidemiological analyses (71, 81).
To prevent sudden death due to epilepsy, the current body of literature supports proactively counseling patients with epilepsy and their families about the potential for sudden death and its associated risk factors (72, 85–87). The actual practice, however, according to a study by Louik et al., is that most counseling is retroactive, with families first learning about fatal risk upon death of their family member (86). This retrospective knowledge is stressful and a complaint from bereaved families. Most papers endorse counseling all patients with epilepsy about possible sudden death (85–87), while one author, in an effort to protect patients from undue anxiety, recommends counseling only those patients with epilepsy who are at high risk (i.e., those with seizure onset at a young age, frequent generalized, tonic-clonic seizures, and those with medically intractable seizures) (72). Gayarti et al. and Tonberg et al., however, found that providing information on sudden death in epilepsy to patients does not have any sort of negative impact on them in the near or long term (85, 87). Lastly, Tonberg et al. found that, although provision of information on sudden death due to epilepsy is valued by patients and their families, there is no evidence that such education and information sharing impacts medical adherence (85).
Informing a surviving sibling of their sister’s or brother’s sudden unexpected death is challenging for both parents and providers. Cepeda created a guide for clinicians who want to help parents communicate with surviving children after the sudden death of their sibling (88). The author divided the paper into developmental age ranges of children, described each age range’s concept of death, and related that concept to suggestions on how death should be communicated (Table 14.1) (63, 88). Beyond this, communicating with surviving siblings concerning the genetic risk factors that may be associated with the dead sister’s/brother’s sudden unexpected death is fraught with ethical and practical dilemmas (89–92).
Summary of Developmental Age and Perceived Meaning of Death with Suggested Provider Action.
McGuire et al. provide a list of recommendations surrounding molecular autopsies, autopsies that are conducted to identify potential genetic causes of death. They address the process of obtaining consent, confidentiality of causes of death, analysis, and disclosure (79). The importance of communicating potential genetically-confirmed or even negative findings to the first-degree relatives was discussed. Importantly, the authors recommended that prior to any molecular autopsy, a multidisciplinary group needs to convene and plan how to collaborate with the family.
Garstang reports that families preferred to be made aware of modifiable risk factors, specifically in regard to infant deaths (93). Limited research is available regarding sibling perceptions of being informed of a cause of death with potential genetic implications. Even less is known about the long-term sequela of such disclosures.
Research has not conclusively identified a “best approach” for communicating with surviving siblings regarding death. However, several recommendations can be made: establish agency policies for pediatricians and death investigators that address communication of genetic testing results, establish a multidisciplinary team to communicate with family/members, and ensure that communication is conducted at an age- and developmentally-appropriate level.
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