Behavior of Sickle Cell Disease (SCD) and it's Management in Adult Patients

Manal Elhabbash, Isra Errayes
2018 The International Annals of Medicine  
Sickle cell disease SCD is an inherited chronic hemolytic anemia. Clinical manifestation arise from the tendency of hemoglobin (Hb s or sickle hemoglobin) to polymerize and deform red blood cells into the characteristic sickle shape. Aim of study: To study the behavior and complications of sickle cell anemia in adult life and to assess the benefit of Hydroxyurea in selected patients. Patients and Methods: All patients who were referred from pediatric hematology department to medical hematology
more » ... epartment in Tripoli Medical Center as a case of sickle cell anemia proved by hemoglobulin electrophoresis in the period between Jan 2012-Dec2017 were included in our study. Results: 34 patients were included in our study. The Median age was 30.5 years. 64.7% (22) were males and 35.3% (12) were females.91.2% were black. 47% of patients had strong family history of S C D. Mean hemoglobin level 7 g/dl. Jaundice occurred in 88.2%. The causes of admission were infection in 50% and bone pain crisis in 73%. One patient had bone tuberculosis. The mean frequency of admissions was 3 times /year . All our patients were on folic acid tab. 5 mg daily and 20(60%) patients received Hydroxyurea capsules 1g /day. 70% (14/20) of patients who received Hydroxyurea had hemoglobin concentration between 9-11 g/ dl and the admission frequency became less than one/year. During follow up of all patients, one patient died because of acute chest syndrome & one patient died because of complication of cerebrovascular accident CVA. Conclusion: Painful crisis, hemolytic crisis and infections were the most common causes of admission in our patients. The frequency of admissions to the hospital were less in adult life compared to childhood indicating the quiescent behavior of SCD. The use of Hydroxyurea increases hemoglobin F concentration with less attacks of painful and hemolytic crisis.
doi:10.24087/iam.2018.2.8.589 fatcat:tq5kbhg6cnfu7oviuwkwf5juka