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manifestations-of-sickle-cell-disease?search=sickle cell anemia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank =1. Painful vaso-occlusive crisis (VOC) is the most frequent complication of Sickle Cell Disease. Common sites of pain include bone pain which can be in the extremities causing dactylitis or hand/foot syndrome and abdominal pain. Bone pain is the most common type of VOC and may or may not be accompanied by swelling, low-grade fever, redness, and warmth, as presented with Miah in the case study this week. Dactylitis is a common presentation in infants
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and toddlers; where back and abdominal pain are more common in older children. Abdominal pain in children with sickle cell disease is usually a simple VOC, but other diagnoses may present which include splenic sequestration, liver sequestration, appendicitis, pancreatitis, biliary colic and cholecystitis, urinary tract infection, and pelvic inflammatory disease and should be ruled out. In addition, pneumonia and chest crisis may present as, or accompany abdominal pain. During a severe pain crisis, a patient may also develop an acute chest syndrome, or a central nervous system event. Pain should be treated early and as aggressive as possible (“Acute Painful Episodes Vaso-occlusive Crisis,” 2019). In 2017, the Food and Drug Administration (FDA) granted regular approval to hydroxyurea to reduce the frequency of painful crises and the need for blood transfusions in pediatric patients from 2 years of age and older with sickle cell anemia with recurrent moderate to severe painful crises(Center for Drug Evaluation and Research, 2017 ). Benefits associated with the use of hydroxyurea in sickle cell patients include (Agrawal, Patel, Shah, Nainiwal, & Trivedi, 2014 ): Single-agent, inexpensive, orally administered, once-daily dosing Increases HbF and total Hb, reduces WBC and reticulocytes, and lowers LDH Leads to fewer vaso-occlusive events and hospitalizations Works in all age groups Prevents acute events and chronic organ dysfunction Benefits continue over time without medication resistance or tolerance Reference Acute Painful Episodes Vaso-occlusive Crisis: Guidelines for Management in Children with Sickle Cell Disease. (2019, April 30). Retrieved from - practice-guidelines/clinical-practice-guidelines/Export/CLINS214/Main Document.pdf. Agrawal, R. K., Patel, R. K., Shah, V., Nainiwal, • L., & Trivedi, B. (2014). Hydroxyurea in Sickle Cell Disease: Drug Review. Indian Journal of Hematology and Blood Transfusion , 30 (2), 91–96. doi: 10.1007/s12288-013-0261-4 Center for Drug Evaluation and Research. (2017, December 21). FDA approves hydroxyurea for treatment of pediatric patients with sick. Retrieved from - treatment-pediatric-patients-sickle-cell-anemia.
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  • Summer '17
  • Sickle cell anemia, Sickle-cell disease

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