Cooperative Study of Sickle Cell Diseases. Factors predicting future ACS episodes in children with sickle cell anemia. Quinn CT. Sickle cell disease in childhood: From newborn screening through transition to adult medical care. Current issues in sickle cell pain and its management. Use of a clinical pathway to improve the acute management of vaso-occlusive crisis pain in pediatric sickle cell disease. Recurrence of splenic sequestration is common, recurring in 50-75% of cases. Hydroxyurea and blood transfusions have been the gold standard of therapy for the management of sickle cell disease. Glassberg JA, Wang J, Cohen R, et al. N Engl J Med 1995;332: 1317-1322. Ogunlesi F, Heeney MM, Koumbourlis AC. Olujohungbe A, Burnett AL. Interpreting the CBC of children treated with hydroxyurea (HU) is increasingly important. Sickled red blood cells trapped in the splenic sinusoids cause mechanical obstruction leading to the clinical signs of sequestration: abdominal distension, pallor and hemodynamic instability with tachycardia, and hypotension. Bivalacqua TJ, Musicki B, Kutlu O, Burnett AL. Sickle cell disease as a cause of osteonecrosis of the femoral head. In high-risk SCD patients, such as those with HbSS, a low threshold for brain imaging is warranted. Priapism in children: A comprehensive review and clinical guideline. Piel FB, Tatem AJ, Huang, et al. 2013;10:e1001484. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. In young children especially, physical exam may be insufficient to distinguish these etiologies from causes of acute abdomen common in all children vs those particular to children with SCD. These specialists should also be notified of any child with a likely stroke. Darbari DS, Catro O, Taylor JG 6th, et al. Ischemia and infarction of the metacarpals and phalanges cause painful and often symmetric swelling of hands and/or feet and sometimes fever. In the absence of an additional indication for transfusion, such as severe anemia or worsening clinical status, transfusions are not emergently indicated. Transfusions should be administered in small aliquots. Booth C, Inusa B, Obaro SK. Recurrent, untreated episodes cause long-term damage to penile structure and function. Impact of hydroxyurea on clinical events in the BABY HUG trial. Comparison of automated red cell exchange transfusion and simple transfusion for the treatment of children with sickle cell disease acute chest syndrome. Children having a CVA may present with seizures and motor deficits, and posterior circulation lesions may present with ataxia. Ethical issues in the management of sickle cell pain. How I use hydroxyurea to treat young patients with sickle cell anemia. This publications provides an overview of major sickle cell disease (SCD)-related health complications, as well as the nursing implications for each. Surgery and anesthesia in sickle cell disease. Lovett PB, Sule HP, Lopez BL. Gladwin MT, Kato GJ, Weiner D, et al. Association of hospital and provider types on sickle cell disease outcomes. Donaldson JF, Rees RW, Steinbrecher HA. Risk factors for increased ED utilization in a multinational cohort of children with sickle cell disease. MRI and MRA with diffusion-weighted images are the gold standard for identifying the timing and location of ischemic stroke and should be performed for all children with SCD in whom stroke is a differential concern. Angelucci E, Matthes-Martin S, Baronciani D, et al. Lemonne N, Lamarre Y, Romana M, et al. National Heart, Lung, and Blood Institute. Progression and prognostic indicators of bronchial disease in children with sickle cell disease. Initial management is conservative with hydration, oxygen, and pain control with opiates. doi: 10.1371/journal.pmed.1001484. Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease. Piel FB, Hay SI, Gupta S, et al. From the Sickle Cell Information Center, 1991. Outcomes of acute chest syndrome in adult patients with sickle cell disease: Predictors of mortality. This may occur in isolation or during a VOC event, and, although dactylitis may cause fever, blood culture and parenteral antibiotics are indicated (see: Fever). Topley JM, Rogers DW, Stevens MC, Serjeant GR. The Multicenter Study of Hydroxyurea (MSH) compared hydroxyurea with placebo in adults with HbSS and more than three acute painful crises per year (not necessarily requiring treatment in hospital). Pediatric Emergency Medicine Reports 2015-02-01 Recent ACS, fever or exchange transfusion within 2 weeks of stroke Cognitive functioning and brain magnetic resonance imaging in children with sickle cell disease. Sickle cell disease in the emergency department: Atypical complications and management. Koshy M, Weiner SJ, Miller ST, et al. Endothelial activation and inflammation biomarkers in children and adolescents with sickle cell disease.