Management and Complications of Thalassemic Patients in Palestine: Retrospective Study

In Palestine little data concerning current thalassemia management and complication is available. This study aimed to evaluate the existing management protocol administrated to the registered β-thalassemia major (BTM) patients in comparison with internationally used ones. In addition, to identify commonly developed complications and their association with iron overload. This cross-sectional study was conducted retrospectively from January 2012 to September 2014. The study included all reported BTM patients admitted for treatment and care at Al-Wattani thalassemic ward. Medical files were reviewed and data was collected in a specially designed questionnaire. Missing data was obtained by direct interview with patients or their guardians. Statistical analysis was performed using the IBM SPSS v.21. Conformation of diagnosis was carried out for five cases. A total of 54 patients includes 26 (48.1%) males and 28(51.9%) females, with average age of 17.07 ± 1.8 ranged from 1-68 years. 84.4% of them were born to relative parents. Excluding five miss-diagnosed patients, the main age of the rest reported BTM patients was 13.96 ± 7.75, ranged from 1-28 years. The annual haemoglobin mean was below 7.6g/dL in three studied years (2012- 2014) indicating a sever anaemic state among the study group. Hepatitis infection (one with B the other with C) were reported in two cases (3.7%). Splenectomy was carried out for 29(53.7%) patients. Serum ferritin mean was 2721±2204.1ng/ml in 2012 and 2069.6± 1770.6 ng/ml in 2014 with significant decrease (P-value= 0.001). In 2014, 73.5% of patients were with levels above the internationally recommended cut off value, ≤1000ng/ml, reflecting iron overload. Biochemical characteristics strongly indicate deteriorated liver, kidney and endocrine functions. Elevated ALT and AST levels were reported among 43.4% to 93.5% of tested patients over the study period. Comparative analysis of the levels of these enzymes showed an increased level with increased age for both enzymes. Variations in the levels of ALT were of no significant value, however variations in AST levels were statistically significant (P-value= 0.001). A strong positive correlation was observed between SF and hepatic enzymes (P- value= 0.00, rs>0.5 for both enzymes). Elevated ALP levels were reported among 32.7% to 67.5% of tested patients over the study period. Variation in the levels of this enzyme with elapsed time were significant (P-value= 0.002). Hypercalcemia was reported among 58.7% and 17.3% of the tested patients in 2012 and 2013, respectively with significant decrease in calcium level between those years (P-value= 0.001). Kidney function test showed low creatinine levels among 84.3% of tested patients in 2012 and in 59.2% for those tested in 2014, with significant decrease (P-value= 0.024) . High serum urea levels were reported among 12% patients in 2012 and among 23.6% of those tested in 2014 with significant increase in urea levels with elapsed time (P- value= 0.00). Poor endocrine follow up was reported as testing for endocrine function only and partially started in 2014. The most common complication among the tested patients was hypogonadism in 46.7%, hypothyroidism was reported in 13.3%, and hypercortisolism was reported in 5.5%. Poor monitoring for bone profile and cardiovascular function were also reported. Cardiac complications were reported in 9.3% of the patients. This study is the first to address a detailed profile of the health situation of β-thalassemia major patients. The observed impaired biochemical and hormonal levels reflect a deteriorated clinical picture. Such findings emphasize the need for reinforcement and importance of adopting effective strategic policy for management and care thalassemic patients in Palestine.