Contributions
Abstract: EP1216
Type: E-Poster Presentation
Session title: Sickle cell disease
Background
Sickle cell disease (SCD) is a significant health problem for Turkey. The official register of the Ministry of Health includes 1200 patients with SCD residents in Turkey. However, there has been no data including the demographic and clinical features of this cohort
Aims
The National Hemoglobinopathy Register of the Turkish Society of Hematology (TSH) aims, to evaluate the geographic distribution and disease characteristics of patients with SCD and the need for delivering a standard of care to the patients.
Methods
Thirty-five hemoglobinopathy centers entered a total of 656 patients' data, 627 of whom were alive, into the web-based system from the hospital records. The database was composed of 480 SCD (75%), 160 (25%) HbSB patients, and only 16 HbS with other abnormal beta-globin chain variants. The 640 of 656 registered patients were born in Turkey, and 80% were born in only three provinces (Adana, Hatay, and Mersin).
Results
The patients (305 males, 335 females) were 32 ± 15 years-old.The mean age of diagnosis was 11±10.7 months (HbSS; 10±8.2, HbSB; 23±14). The physicians prescribed penicillin prophylaxis for 46.5% of patients at the mean age of 15±14 months. The pneumococcal, meningococcal, and Hib vaccination was administered to 91.7%, 66.8%, and 54.6%, respectively, and 48.5% of those received booster dose of pneumococcal vaccine. 75% of patients were receiving hydroxyurea, the last amount used was mean 15 ± 6.7 mg/kg/day, the physician reported compliance with the treatment was 69%.
The 33% of patients received simple transfusions for anemia (63%) or controlling painful crises (20%) in the last year. The 8.4% of the patients were on chronic exchange programs. Erythrocytapheresis was the method of choice in the majority of those (81.4%). The rate of transfusion with ABO, Rh(CcDEe), Kell compatible products was 23%. Iron chelation therapy (ICT) has been initiated in 22% of the patients at the mean age of 7.7±11.2 years, with a median serum ferritin level of 2500±470 ug/L. Deferasirox was the first-line iron chelator in 52.5% of the patients. Deferasirox use increased to 85.6% among patients on ICT over the years. The median serum ferritin decreased to 1400±176 ug/L in the last year. The patients who had at least one acute chest syndrome and stroke were 16% and 9.5%, respectively. At least one painful crisis was recorded in 81% of patients in the last year
Conclusion
Current treatment options for reducing morbidity and mortality in SCD are limited to adherence to penicillin prophylaxis and recommended vaccination schedule, using hydroxyurea, erythrocyte transfusions, and ICT whenever indicated. The data showed, starting hydroxyurea and providing patients' compliance to the therapy were our positive aspects. However, the physicians should reconsider the adjustment of the hydroxyurea dose. The initiating penicillin prophylaxis, use of ABO, Rh(CcDEe), Kell compatible erythrocyte transfusions, and timely initiation of ICT were the aspects we needed to improve delivering a standard of care to the patients.
Keyword(s):
Abstract: EP1216
Type: E-Poster Presentation
Session title: Sickle cell disease
Background
Sickle cell disease (SCD) is a significant health problem for Turkey. The official register of the Ministry of Health includes 1200 patients with SCD residents in Turkey. However, there has been no data including the demographic and clinical features of this cohort
Aims
The National Hemoglobinopathy Register of the Turkish Society of Hematology (TSH) aims, to evaluate the geographic distribution and disease characteristics of patients with SCD and the need for delivering a standard of care to the patients.
Methods
Thirty-five hemoglobinopathy centers entered a total of 656 patients' data, 627 of whom were alive, into the web-based system from the hospital records. The database was composed of 480 SCD (75%), 160 (25%) HbSB patients, and only 16 HbS with other abnormal beta-globin chain variants. The 640 of 656 registered patients were born in Turkey, and 80% were born in only three provinces (Adana, Hatay, and Mersin).
Results
The patients (305 males, 335 females) were 32 ± 15 years-old.The mean age of diagnosis was 11±10.7 months (HbSS; 10±8.2, HbSB; 23±14). The physicians prescribed penicillin prophylaxis for 46.5% of patients at the mean age of 15±14 months. The pneumococcal, meningococcal, and Hib vaccination was administered to 91.7%, 66.8%, and 54.6%, respectively, and 48.5% of those received booster dose of pneumococcal vaccine. 75% of patients were receiving hydroxyurea, the last amount used was mean 15 ± 6.7 mg/kg/day, the physician reported compliance with the treatment was 69%.
The 33% of patients received simple transfusions for anemia (63%) or controlling painful crises (20%) in the last year. The 8.4% of the patients were on chronic exchange programs. Erythrocytapheresis was the method of choice in the majority of those (81.4%). The rate of transfusion with ABO, Rh(CcDEe), Kell compatible products was 23%. Iron chelation therapy (ICT) has been initiated in 22% of the patients at the mean age of 7.7±11.2 years, with a median serum ferritin level of 2500±470 ug/L. Deferasirox was the first-line iron chelator in 52.5% of the patients. Deferasirox use increased to 85.6% among patients on ICT over the years. The median serum ferritin decreased to 1400±176 ug/L in the last year. The patients who had at least one acute chest syndrome and stroke were 16% and 9.5%, respectively. At least one painful crisis was recorded in 81% of patients in the last year
Conclusion
Current treatment options for reducing morbidity and mortality in SCD are limited to adherence to penicillin prophylaxis and recommended vaccination schedule, using hydroxyurea, erythrocyte transfusions, and ICT whenever indicated. The data showed, starting hydroxyurea and providing patients' compliance to the therapy were our positive aspects. However, the physicians should reconsider the adjustment of the hydroxyurea dose. The initiating penicillin prophylaxis, use of ABO, Rh(CcDEe), Kell compatible erythrocyte transfusions, and timely initiation of ICT were the aspects we needed to improve delivering a standard of care to the patients.
Keyword(s):