An 18-year-old adolescent girl with sickle cell disease presents to the ED stating she has severe bilateral thigh and shoulder pain. On examination, she has normal vital signs and appears comfortable lying on her gurney. She reports mild pain when her shoulder and thighs are palpated. A nurse comments that she had also presented 2 days ago, and is possibly seeking drugs. How do you decide whether this is drug-seeking behavior or pain from vaso-occlusive crisis? What pain medication can you offer her? How do you decide whether she will need to be admitted for pain control?
A 12-year-old boy with sickle cell disease is brought in by EMS because he is having trouble breathing. He is coughing and complains of right-sided chest pain. On examination, he appears to be in severe respiratory distress. His vital signs are: respiratory rate, 50 breaths/ min; oxygen saturation, 89% on room air; temperature, 37°C (98.6°F); heart rate, 110 beats/min; and blood pressure, 110/70 mm Hg. On auscultation of the lungs, you hear crackles bilaterally. A chest x-ray shows a right-sided infiltrate. The resident is placing him on a nonrebreather mask. What is the diagnosis of this patient? What treatment should be started? Is there any role for a blood transfusion?
A 4-year-old boy with sickle cell disease is brought in by his father because the child developed a fever today. Prior to fever onset, the child was in school, and was his usual, active self. On examination, he has a fever of 38.9°C (102°F), but all other vital signs are normal. He appears well and is playful for the rest of the examination. Should you obtain any laboratory studies? Does he need antibiotics?
An 18-month-old toddler with sickle cell disease is brought in by his mother because he looks pale and tired. His vital signs are: respiratory rate, 30 breaths/min; oxygen saturation, 97% on room air; temperature, 37°C (98.6°F); heart rate, 142 beats/ min; and blood pressure, 88/60 mm Hg. He appears alert and comfortable. You note a large spleen on examination. What steps should you take next? Does this patient need to be admitted or can he be discharged home if all tests are normal?
Sickle cell disease (SCD) refers to a family of genetic blood disorders caused by the sickling of red blood cells secondary to atypical hemoglobin molecules, known as hemoglobin S (HbS). This disease is seen primarily in individuals of African, Indian, Mediterranean, or Saudi Arabian ancestry.1 Annually, 200,000 children are born with SCD worldwide. Ninety percent of these births are in sub-Saharan Africa. In the United States, 1 in 2474 births are children with SCD. The national prevalence is between 70,000 to 140,000 patients, mostly in those of African ancestry.2
SCD has wide-ranging implications to human physiology. Newborns inherit the autosomal recessive genes from their biological parents. The pathophysiological processes that alter the properties of the red blood cell begin in infancy, with effects seen through adulthood. The following common acute complications (listed in order or prevalence) will be discussed:
Emergency clinicians are first-line providers in the care of patients with acute SCD complications. As such, an understanding of the pathophysiology, clinical features, and management is essential to providing high-quality care. This issue of Pediatric Emergency Medicine Practice provides an overview of SCD and recommendations for the management of some of the most common complications seen in pediatric patients with SCD.
A literature search was performed in PubMed using the terms sickle cell, pain, vaso-occlusion, hemolysis, fever, sepsis, wheeze, acute chest syndrome, stroke, splenic sequestration, transient red cell aplasia, and priapism. Over 500 articles from 1961 to 2015 were reviewed. The Cochrane Database of Systematic Reviews was searched using the key words sickle cell; 52 studies were reviewed. The American College of Emergency Physicians (ACEP) and American Society of Hematology (ASH) websites were reviewed; neither have issued SCD policy statements or guidelines. The American Academy of Pediatrics (AAP) Section on Hematology/Oncology and Committee on Genetics issued a policy statement on health supervision for children with SCD that was reaffirmed in 2006 and 2011.10 The June 2014 publication by the National Institutes of Health National Heart, Lung, and Blood Institute (NHLBI) expert panel report on SCD was reviewed.11 The AAP endorsed this report in November 2014. The comprehensive report included consensus statements and evidence-based guidelines. Using a modified Grading of Recommendation, Assessment, Development, and Evaluation (GRADE) standard evidence scale, there was only 1 high-quality, strongly recommended finding for management of acute SCD complications.12 All other recommendations used moderate- to low-quality evidence. Between 1995 and 2010, only 12 randomized controlled trials were conducted for acute complications of SCD in children.13 Reasons for this lack of high-quality evidence for management of acute complications are likely due to the rarity of the genetic disorder, poor funding for research, and the discovery of successful breakthrough health main-tenance interventions (ie, immunizations, penicillin prophylaxis, regular blood transfusions, and hydroxyurea use).14,15
Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.
To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study is included in bold type following the references, where available. The most informative references cited in this paper, as determined by the authors, are noted by an asterisk (*) next to the number of the reference.
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Sathyaseelan Subramaniam, MD; Jennifer H. Chao, MD, FAAP
November 2, 2016
December 2, 2019
4 AMA PRA Category 1 Credits™, 4 ACEP Category I Credits, 4 AAFP Prescribed Credits, 4 AOA Category 2-A or 2-B Credits. Specialty CME Credits: Included as part of the 4 credits, this CME activity is eligible for 0.5 Pharmacology CME credits
Upon completion of this article you should be able to:
Date of Original Release: November 1, 2016. Date of most recent review: October 15, 2016. Termination date: November 1, 2019.
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