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

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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 (

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