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Managing Acute Complications of Sickle Cell Disease in Pediatric Patients (Pharmacology CME)
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Managing Acute Complications of Sickle Cell Disease in Pediatric Patients (Pharmacology CME) - $49.00

Publication Date: November 2016 (Volume 13, Number 11)

CME Credits: 4 AMA PRA Category 1 Credits™, 4 ACEP Category I Credits, 4 AAFP Prescribed Credits, 4 AOA Category 2-A or 2-B Credits. CME expires 11/1/2020

Specialty CME Credits: Included as part of the 4 credits, this CME activity is eligible for 0.5 Pharmacology CME credits, subject to your state and institutional approval.

Authors
 
Sathyaseelan Subramaniam, MD
Pediatric Emergency Medicine Fellow, Pediatric Emergency Medicine Department, SUNY Downstate Medical Center/Kings County Hospital Center, New York, NY
 
Jennifer H. Chao, MD, FAAP
Clinical Assistant Professor, Pediatric Emergency Medicine Department, SUNY Downstate Medical Center/Kings County Hospital Center, New York, NY
 
Peer Reviewers
 
Ilene Claudius, MD
Associate Professor, Department of Emergency Medicine and Pediatrics, USC Keck School of Medicine, Los Angeles, CA
 
Jeffrey Glassberg, MD, MA
Assistant Professor of Emergency Medicine, Hematology and Medical Oncology; Associate Director of the Mount Sinai Comprehensive Sickle Cell Program; Department of Emergency Medicine, Mount Sinai Medical Center, New York, NY
 
P. David Sadowitz, MD
Associate Professor of Pediatrics and Pediatric Emergency Medicine, Department of Emergency Medicine, SUNY Health Science Center, Syracuse, NY
 
Abstract
 
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management. Patients with sickle cell trait generally have a benign course, but are also subject to serious complications. This issue provides a current review of evidence-based management of the most common acute complications of sickle cell disease seen in pediatric patients in the emergency department.
 

Excerpt From This Issue

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?

 

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