It’s 2 AM, and the Pediatric Emergency Department (ED) at a community hospital in New York is overflowing with children and caregivers. A young Nigerian boy is being transported down the center of a hallway, past a long line of doors to patient rooms. The porter is calm and walks briskly, determined to bring this boy to get immediate care. The boy winces, his hands outstretched next to him, rigid, and frozen in space, and while he is seated in the wheelchair, his legs bent at the knees are thin frames, held in place with his feet planted on the wheelchair pedestals. He is afraid to move any of his extremities; tears are rolling down his face; he is fighting the urge to grimace and furrow his brow. He cries how much it hurts to move. He knows he needs help. Behind him, his mother follows close holding a one-year-old baby in her arms, and behind her, five other young children aged 3 through to thirteen stream in. There is quiet concern on all of their faces. The older siblings have seen this before. We learn that he has Sickle Cell Disease (SCD). He has been in excruciating pain for the past 4 hours and is now presenting with dactylitis. This case has not been the first in this ED, and like other EDs across the United States and in the world, the number of cases presenting with SCD will increase.
Sickle Cell Disease (SCD)
SCD is a condition that causes red blood cells to morph from a biconcave dumbbell-shaped disc, into a rigid semi-circular shape. This disease is inherited genetically by receiving two sickle genes, one from each parent and risk for complications are attributed to a variety of factors, including deoxygenation, dehydration. It is most common in African Americans as well as Latinos and people of Middle Eastern, Indian, Asian and Mediterranean backgrounds. In the United States, SCD is the most common genetic blood disorder and affects approximately 100,000 Americans(1) and although babies are screened at birth, management plans vary with the degree of disease progression and exacerbation severity, as well as with the availability of resources and education.
Why Emergency Physicians need to be Familiar with SCD
SCD affects both pediatric and adult patients, and it has been reported that patients between the ages of 18 to 30 years old have increased emergency department utilization. A major reason for this is due to the transition by young adults from pediatric to adult care in the management of SCD, and this population is simultaneously also learning to navigate the health care system and community resources (pediatric to adult care, insurance, independent decision making, housing, education, workforce) as discussed further below(2). In addition, the use of community health workers is important as they can act as liaisons between the health care systems and patients to disseminate information and resources. However, despite the awareness of the disproportionate use of the ED among patients with SCD, the social factors that impact care remain unknown(3) and more research and investigation is needed to understand this patient population.
Often when a complication or crisis occurs in patients with SCD, patients seek immediate care in the Emergency Department. Included in the potential list of complications include infections, such as those with encapsulated bacteria; sepsis; stroke; splenic sequestration, and early treatment is essential in managing patients. Of these complaints, the emergent cases to be aware of in the ED include vaso-occlusive crisis and pain, sickle cell anemia (SCA)(4) central nervous system such as stroke, and acute chest syndrome (ACS), where ACS due to blocked capillaries in the lungs, may be caused by infections, asthma exacerbations and/or pulmonary embolisms, and is the leading cause of morbidity in patients with SCD. Further, the Emergency Severity Index (ESI) Version 4 triage system, commonly used in the majority of EDs in the United States, suggest that patients with SCD be triaged as ESI level 2, indicating a very high priority, and that rapid placement be facilitated(5).
Although the discussion of complications of SCD including the presentation and management is a complex topic, and will be covered in detail in future posts, information and algorithms for clinicians are available online for reference. One such resource is a treatment algorithm that acts as a how-to guide for SCD and is available online in the Annals of Emergency Medicine(6). This approach is based on the point-of-care hemoglobin level, and discusses issues such as myonecrosis, aplastic crisis, ACS.
Pain in SCD
When tissues and organs are not adequately perfused with oxygen, in part due to the sickled shape of RBCs, tissue damage and death can occur. Patient management of vaso-occulusive crisis and pain varies by practices and the medications available for use around the world, however it is important to note that pain in patients with SCD is often extreme and may require treatment with opioids. In a response to the American Society of Hematology (ASH) draft recommendations to Sickle Cell Disease-Related Pain in May 2019(7), emDOCs.net published a response to the drafted recommendations and offered insight to pain management and includes an algorithm(8). The insight provided is essential in decreasing the suffering experienced by patients during an SCD crisis, and notes the use of Dilaudid, Ketamine, Dexmedetomidine, and Lidocaine. Further, the understanding of limiting the use of NSAIDS due to impaired renal function caused by the disease is also outlined in the response.
Management of pain in pediatric patients with SCA and vaso-occulsive pain also varies according to hospital and individual provider practices, and scientific investigation and patient research is needed to provide proper care to this population. An example includes a study by PECARN addressing the use of a normal saline bolus in pediatric emergency departments found an association with poorer pain control(9). Identifying and implementing results from research studies is important in understanding and managing SCD in both adult and pediatric patients.
Emergency Physicians around the world should be aware of strategies for identifying SCD, and management, specifically in areas around the world where refugees from countries with SCD prevalence is common. Countries where refugees and migrants are commonly are known to disembark, such as those in southern Europe(10) and certain areas in the United States and Canada would benefit from in-depth analysis of the issue and could allow for appropriate and accessible health care to vulnerable populations, as well as educate providers who are unexposed to managing emergencies in SCD patients while setting in place integrated and individual health plans away from emergency room dependence(11). In developing countries with SCD populations, such as Nigeria, there is a high prevalence of pediatric emergency cases, and the proper management of the disease as well as policy and hospital organization for high volume and off-hour admissions, may reduce hospital stays(12). Further, the self-efficacy of adult patients with SCD, from education, pro-active efforts, understanding of disease management, also can allow for decreased ED visits and hospitalizations for pain(13).
Investigations, Resources, Education
A number of investigative studies, clinical trials and research is being conducted around the world for a better understanding of SCD, including patient care in adult and pediatric patients, genetic factors, supportive services, associated co-morbidities, and search for cures. Investigations around the world include collaborations and information sharing between academic researchers, patients, clinical providers, and health care providers and officials around the world.
The National Heart, Lung, and Blood Institute hosted a series of Webinars in September 2018, during Sickle Cell awareness month from experts in blood science and sickle science research and are available to watch for free online(14). Some of the key highlights from two of the webinars: Serving the Sickle Cell Disease Community Here and Abroad; Sickle Cell Transitional Care from Childhood to Adulthood, are discussed here.
Webinar Overview Serving the Sickle Cell Disease Community Here and Abroad
Presented by Dr. Keith Hoots, Director of Division of Blood Diseases and Resources, NHLBI
- Prevalence of the disease is so much larger in Africa than most places in the world. There are as many babies born with SCD born in Nigeria there are babies born with SCD, by estimate, as there almost are total people with SCD in the United States.
- There is a need to share research and practices in the developed world with the developing world.
Three New Research Initiatives in Africa:
- The Sickle Pan-African Research Consortium (SPARCO)
Overview: The study sites for this research include East Africa (Tanzania), West Africa (Ghana, Nigeria) and central Africa (Cameroon, Democratic Republic of Congo) with the goal to later include 20 sites in 15 countries. SPARCO’s aim is to develop an SCD database, standards of care, and strengthen research investigation.
- Realizing Effectiveness Across Continents With Hydroxyurea (REACH):
Overview: Safety and dosing of hydroxyurea therapy for SCA in pediatric patients in sub-Saharan Africa; sponsored by the Children’s Hospital Medical Center, Cincinnati
- Sickle Cell Disease Genomics of Africa (SickleGenAfrica)
Overview: The purpose is to develop strategies to predict, prevent and treat organ damage in SCD and to investigate biomarkers associated with the development of organ damage, including molecules released during red blood cell damage in sub-Saharan African populations.
Webinar Overview: Sickle Cell Transitional Care from Childhood to Adulthood
Part 1 Presented by Dr. David Wong, MD, FAAP, Medical Officer, Office of Minority Health
- SCD is no longer a childhood disease. Young adults are at a higher risk for hospitalization due to illness and pain.
- Treatment and management examples in childhood include annual transcranial dopplers to assess for risk of stroke; vaccinations; hydroxyurea; L-glutamine; opioids for pain management; penicillin prophylaxis; RBC transfusions; water intake to avoid exacerbations due to dehydration; splenectomy. The cure available is bone marrow transplant.
- Prior to July 2017, Hydroxyurea was the only FDA approved therapy for 20 It is used in adults and children. It has been shown to reduces hospital admissions, pain crisis, and ACS however barriers to hydroxyurea use exist. These include difficulty with communicating the use to patients and caregivers, issues with frequent monitoring, lack of adherence, lack of provider knowledge and comfort with its use.
- Community Health Workers (CHWs) are key players in effective patient care. CHW can provide information affected by social and health determinants from local economic and environmental (housing, employment), local communities (families, safety, support), activities (learn, work, play, move, shop), lifestyles (alcohol, drugs, smoking, sexual health, physical activity, and individual needs (age, genetics). CHW are experts in condition-specific information and navigating complex health systems, including accessing care in a medical home (the approach to providing comprehensive care). This is particularly important when care is not always contained or organized by one organization, where care should be accessible, continuous, comprehensive, family-oriented, coordinated, compassionate and culturally competent. Pediatric medical home principles include family-centered partnerships, community-based systems, transition care, value. Interventions for education such as warning signs and treatment options and links to care are important.
- The SCD Newborn screening program, and the Sickle Cell Disease Treatment Demonstration Program for patients who solely rely on the ED for SCD care, aid the care options for patients with SCD.
Follow this iEM story for part two which will include information on adult and pediatric management of SCD in the ED, as well as an overview of four NHLBI webinars: Holistic Health and Sickle Cell Disease A Focus on Mental and Behavioral Health; Genetic Therapies in Sickle Cell Disease; Bone Marrow Transplants, Other Therapies, and Sickle Cell; Improvement Initiatives and Ongoing Research.
Emergency Department Sickle Cell Care Coalition: Resources
National Institute of Health’s Cure Sickle Cell Initiative:
2019 sickle cell disease guidelines by the American Society of Hematology: methodology, challenges, and innovations: https://www.ncbi.nlm.nih.gov/pubmed/31794603
Sickle Cell Disease Training And Mentoring Program (STAMP): https://www.minorityhealth.hhs.gov/sicklecell/#stamp
Episode 68 Emergency Management of Sickle Cell Disease: https://emergencymedicinecases.com/emergency-management-of-sickle-cell-disease/
Practice Variation in Emergency Department Management of Children With Sickle Cell Disease Who Present With Fever. https://www.ncbi.nlm.nih.gov/pubmed/30020250
10 Lucia De Franceschi, Caterina Lux, Frédéric B. Piel, Barbara Gianesin, Federico Bonetti, Maddalena Casale, Giovanna Graziadei, Roberto Lisi, Valeria Pinto, Maria Caterina Putti, Paolo Rigano, Rossellina Rosso, Giovanna Russo, Vincenzo Spadola, Claudio Pulvirenti, Monica Rizzi, Filippo Mazzi, Giovanbattista Ruffo, Gian Luca Forni; Access to emergency departments for acute events and identification of sickle cell disease in refugees. Blood 2019; 133 (19): 2100–2103
12 Robert M Cronin, Tim Lucas Dorner, Amol Utrankar, Whitney Allen, Mark Rodeghier, Adetola A Kassim, Gretchen Purcell Jackson, Michael R DeBaun, Increased Patient Activation Is Associated with Fewer Emergency Room Visits and Hospitalizations for Pain in Adults with Sickle Cell Disease, Pain Medicine, Volume 20, Issue 8, August 2019, Pages 1464–1471
13 Enyuma, Callistus Oa et al. “Patterns of paediatric emergency admissions and predictors of prolonged hospital stay at the children emergency room, University of Calabar Teaching Hospital, Calabar, Nigeria.” African health sciences vol. 19,2 (2019): 1910-1923. doi:10.4314/ahs.v19i2.14
* Images from The Sickle Cell Disease Tool Kit.