Abstract
Background: Sickle cell disease could be a common cluster of serious genetic disorders caused by the synthesis of abnormal hemoglobin. It has a wide range of clinical manifestations and complications, one of them being vaso-occlusive crisis (VOC).
The most prominent symptom of VOC is acute episodes of pain. In addition to the pain, there are several clinical consequences, such as acute chest syndrome, hepatic and renal involvement, cerebrovascular accident, and multiorgan failure resulting in death.
Aim: To evaluate the degree of adherence of pediatric physicians of Assiut University Children's Hospital to the international guidelines for the management of acute VOC in patients with sickle cell anemia.
Patients and Methods: A clinical audit prospective study was done in the Hematology Unit at Assiut University Children's Hospital over 1 year, from May 1, 2019, to the end of April 2020.
All children diagnosed with VOC of sickle anemia were enrolled. Data concerning the outline of the management of acute pain were collected in the form of clinical history, examination, investigations, and medications.
Results: All clinical data were asked for in all cases, such as pain, fever, cough, and diarrhea, but skin ulcer was asked only in 19 cases, shortness of breath was asked in 28 cases, abdominal pain in 32, headache in 25, convulsion in 24, and weakness in 26. All physical and general examinations were checked in all cases except skin ulcers. Regarding medications, I.V. fluids, antibiotics, and analgesics were prescribed in all patients; 33 received NSAIDS, and 25 received I.V. paracetamol.
Blood transfusion was needed in 24 cases. Folic acid and hydroxyurea were prescribed for all cases, but prophylactic antibiotics and vaccination were prescribed for 25 and 28 cases, respectively.
Conclusion: The National Heart, Lung, and Blood Institute guidelines were followed by the Hematology Department of Assiut University Children's Hospital for the management of sickle cell disease pediatric patients with VOC.