The Impact of Emergency Care Planning for Children With Medical Complexity: Nora’s Story

Nora was born in September 2020 amidst a global pandemic. During pregnancy, the birthing team at our local hospital detected some abnormalities and recommended transferring our care 2 hours away to a larger, level 1 hospital that could better manage any potential challenges.Diagnosed with intrauterine growth restriction (IUGR) at 31 weeks’ gestation, we were prepared for Nora’s small size. She was born on September 9, 2020, at 37 weeks and 5 days, weighing only 2210 g (4 lbs, 14 oz), and was immediately rushed to the neonatal intensive care unit (NICU). Nora spent the first 21 days of her life there, facing numerous hurdles. She was diagnosed with IUGR, reduced movement, and a short umbilical cord that had avulsed during delivery. She also developed persistent pulmonary hypertension of the newborn, respiratory failure, and a pneumothorax. Thankfully, on September 28, 2020, we brought her home, ready to start life as a family. During our NICU stay, the pediatric genetics team evaluated Nora, as her appearance and medical issues suggested a genetic condition. Although they couldn’t identify the exact condition then, we knew it was something we’d learn more about in the future.In February 2021, our world changed when Nora experienced her first seizure. COVID-19 protocols were still in place, and despite their best efforts, our local emergency department (ED) in rural Vermont was not equipped to handle a seizing infant of her size. We were transferred to a nearby children’s hospital for specialized care, which is 70 miles (and 2 hours) away by car from our local ED. Following this first seizure, a full genetic screening revealed that Nora had a rare genetic disorder called Wolf-Hirschhorn syndrome (WHS). In her first 4 years, Nora endured 18 seizures, with 12 requiring transport to children’s hospitals with level 1 trauma centers. Six times, her emergency antiseizure medications caused respiratory arrest, leading to intubation. For Nora, a seizure often signals illness and comes without warning.Living in a rural area with a medically complex child poses unique challenges: Fragmented emergency care: Nora’s care has involved 3 different EDs, leading to fragmented care and inconsistent medical records. Many local hospitals lack pediatric expertise and necessary resources, often requiring medical transport to facilities with pediatric specialists. Our local ED does not treat pediatric patients, and Nora has been transported 12 times by ambulance, helicopter, or medical bus.Medication availability: Ensuring that specialized medications are available on short notice can be difficult in our rural area. Some of Nora’s intravenous medications are not regularly stocked at our local ED, resulting in treatment delays. Her small size also makes vein access challenging, which has further delayed treatment and prolonged seizures.Port and G-tube challenges: To address her vein access issues, Nora underwent surgery for a port and a gastrostomy tube. However, our local ED lacked the specific needles required to access her port, and few staff members were trained to use pediatric ports, leading to further complications in her emergency care.Dr Christian Pulcini: I first met Bo, Katie, and Nora in the ED when Nora was an infant. She had a seizure in the lobby of the hospital while attending outpatient appointments, and she was rushed to the ED while I was on a shift. During this encounter, we were able to stop her seizures (using a standardized, evidence-based approach), and despite some respiratory depression, we were able to avoid intubation with a noninvasive ventilation strategy that had not been previously attempted (“Bubble” continuous positive airway pressure). The medical details of this encounter are far less important, however, than the relationship formed with the McDougalls through conversations about Nora’s emergency and acute care leading up to that presentation. It was after this encounter that we were able to connect on an ongoing basis, establish an emergency care action plan1 with her “health neighborhood,” and provide direct guidance and assistance to the local ED for her anticipated emergency care needs.Katie and Bo McDougall: Nora’s sixth seizure in less than 5 months occurred in the registration lobby of the hospital. The call for a “Code Blue” was terrifying, but seeing all the staff rushing our way was beyond relief. After assessing her, Dr Pulcini remained with Nora throughout her ER stay before being admitted to the pediatric intensive care unit (PICU). After talking with him about her genetic disorder, medication conditions, and our lack of a plan, he was able to generate an emergency care plan for Nora, which addressed not only her seizures but also her respiratory distress. The comprehensive plan included protocols for her tracheomalacia, contingencies for loss of airway and ventilator settings, and a list of required equipment for intubation. This emergency care plan was shared on our medical platform via digital and paper copies to our local rural ED, and a paper copy remained with Nora at all times. The establishment of this plan not only gave us a specific sequence of events but also the confidence to be a valuable part of her medical team. We knew what was going to happen and could advocate based on our own specific knowledge of Nora and her medical history. Her care plan was further enhanced by her neurologist to include a seizure action plan. All of these vital components explicitly written down with updated measurements and rates have allowed Nora to have consistent care despite varying emergency providers.Nora represents a small population of children (less than 5% of the pediatric population) often referred to as children with medical complexity (CMC). CMC are generally defined as “children with multiple significant chronic health problems (including multiple organ systems) which result in functional limitations, high health care needs or utilization, and often require need for, or use of, medical technology.”2 CMC are a growing population that represent a subset of children and youth with special health care needs (CYSHCN), yet account for high use of outpatient and inpatient health care resources, including up to 20% of pediatric ED visits.2,3 Nora’s story reflects the often unavoidable need for CMC to utilize the ED and raises concerns regarding the ability of CMC to receive high-quality emergency care when it is needed. The challenges described by the McDougall family are also reflective of the current medical literature.4–7 The challenges are especially pertinent to Nora and her family given that they reside in a rural area far removed from the nearest children’s hospital, and they often present to a general or critical access ED as the most accessible option for emergency care. The experience of the McDougalls is also reflective of most CYSHCN across the United States, whereas 80% of all children present to general EDs as opposed to freestanding children’s hospitals for emergency care.8Despite known challenges in providing high-quality emergency care for CMC, there is a lack of interventional evidence-based strategies to support children, families, and clinicians who care for CMC in the ED. In 1999 and 2010, the American Academy of Pediatrics (AAP) and American College of Emergency Physicians (ACEP) released joint policy statements recognizing the importance of advanced care planning for all CYSHCN and encouraged use of emergency information forms (EIFs).9 Despite the expert recommendations, dissemination and uptake of the EIF has been limited, and there has been little research on their real-time effectiveness.10–12 An updated policy statement from the AAP and ACEP reaffirmed the recommendation of EIFs and further delineated the challenges and importance of providing critical health information in emergencies for all children in emergency settings and disasters.12 Recently, an article describing an emergency care action plan (ECAP) was released in Pediatrics as an attempt to address ongoing implementation challenges of emergency care planning tool (such as EIFs).1 Effectiveness testing is currently under way. Nora’s emergency care plan used an earlier version of the ECAP.Despite challenges, as Nora’s parents, we have enacted several solutions and recommended support strategies for children like Nora. Systematic ED care plan: After Nora’s sixth seizure, her medical team created a comprehensive ED care plan. In addition to the information described previously, the plan also lists contact information for her care team at both our local hospital and Boston Children’s Hospital, ensuring that any clinician can reach her specialists as needed.Ready-to-go access kits: Due to the rarity of her condition and her small size, Nora’s necessary treatment tools were often unavailable. With help from her infusion team, we created port access kits containing everything required to treat her during a seizure. These kits travel with our family everywhere and are brought into the ED with Nora, allowing the team to begin treatment immediately without waiting for supplies or sourcing them from other hospital areas.On-site respiratory care box: In collaboration with our pulmonary team at the children’s hospital, the local ED assembled a respiratory care box. This box, stored in the ED, contains properly sized equipment for oxygen support and intubation. The local ED team provided us with a direct access number to reach the charge nurse, enabling us to alert them of Nora’s arrival. This allows the ED team to prepare her room with the necessary supplies for efficient treatment.Care coordination meetings: Living 2 hours from the nearest children’s hospital, we rely heavily on local coordination. After Nora’s first birthday, we began holding regular care team meetings that included her full care team: her case manager, primary care physician (PCP), multiple specialists, therapists (physical therapists, occupational therapists, and speech-language pathologists), PICU clinicians, and representatives from each local ED that had cared for Nora. In all, service providers from 5 different hospitals have participated in these care team meetings. These meetings allow us to adjust her seizure action plan, coordinate medications, ensure each localized team is prepared to follow the established care plan, and determine observation needs after each seizure. Our PCP, as the leader of the medical home/neighborhood for Nora, alongside us parents, is the leader of the care coordination meetings (as the meetings often involve care for Nora beyond emergencies).Extending beyond, yet paralleling the role of advanced emergency care planning is the importance of pediatric readiness across our emergency care systems in the United States. Evidence suggests that high pediatric readiness is associated with lower mortality risk for children and that children with complex chronic conditions may experience adverse outcomes/low-quality of care in EDs with low pediatric readiness.13,14 Therefore, it is of the upmost importance that EDs who treat all children are, at baseline, “pediatric ready” and are provided direct guidance through advanced emergency care planning in collaboration with all key partners for the emergency care of CMC. This is best achieved by partnering directly with children, families, and local EDs to (1) assess the ability to care for the child, (2) arrive at a shared plan of care, and (3) ensure it is maintained. This work is continuously supported on the regional and national level by several key organizations such as the National Pediatric Readiness Project/Emergency Medical Services for Children program and the Pediatric Pandemic Network. Although best practices on how to achieve these goals have not yet been published, a solid understanding of local context and family preferences and needs is necessary. This type of collaboration should be viewed as both urgent and vital because, as evidenced by the recent pediatric readiness data, the lives of children like Nora may depend on it.As we reflect on Nora’s journey, we are thrilled to share that she has started school this fall—a significant milestone for our family. Thanks to her comprehensive ED care plan, we have gained the confidence to send her to school knowing she is surrounded by a team that is prepared to respond to her needs. Her entire school team has embraced her plan, reviewed it in detail, and even requested relevant training to ensure they are ready to assist if an emergency arises. This collaboration is a testament to the power of proactive planning and teamwork in providing a safe and supportive environment for CMC and builds confidence that our collaborative efforts can extend far beyond the emergency department and hospital for CMC and their families.Katie and Bo McDougall: At the same time, we recognize the privileges that have enabled us to advocate effectively for our daughter. As well-educated professionals with advanced degrees and the means to bear the financial demands of her care, we have been able to navigate this journey with less strain than many families might face. We know there are families who lack access to education, financial resources, local support systems, or other advantages that have been critical to our ability to advocate for Nora.Dr Christian Pulcini: The current literature reflects this sentiment as well: many families caring for CMC also face concurrent socioeconomic challenges that require intervention to improve their care. This should be considered for all efforts aimed at improving care for CMC (and their families).15,16Through sharing our joint story, we as parents and as a pediatric emergency physician hope to shine a light on the importance of advanced emergency care planning, care coordination, and advocacy for all families of children with medical complexity. Our hope is that care providers, policymakers, and others who read this will be inspired to continue improving systems of care so that every family, regardless of their circumstances, can benefit from the incredible support and partnership that has made such a difference for Nora.We want to extend our deepest gratitude to the many individuals and teams who have supported Nora and our family along the way. To Nora’s primary care pediatrics team at Rutland Pediatrics, the ED team at Rutland Regional Medical Center, the Dartmouth Hitchcock Advanced Response Team (transport), the University of Vermont Medical Center ED, PICU, and Child Specialty Clinic teams, the Pediatric Neurology teams at both University of Vermont Medical Center and Boston Children’s Hospital, and, specifically, Ms Leah Pence for her care coordination and dedication to Nora and our family—we thank you all. We feel so fortunate to live in a community with a level 3 trauma center and to have access to 2 level 1 trauma centers within a few hours’ drive. The coordinated efforts of these teams and individuals have been instrumental in ensuring Nora’s well-being, and we are eternally grateful for their expertise and care.