Results
During this exercise ,a total of 114 patients of SCD were reviewed. There were 68 males and 46 female patients with a median age group of 19 years (2-70 years). Majority of patients (n=60) were younger than 20 years.
HU was prescribed on average considering age of the patient and average weight. 104 children were taking hydroxyurea. Exact calculations for dosing were not done. The dose averages between 10 mg/kg to15 mg/kg. As there was only one formulation available , the required daily dosing was changed to fixed dose scheduling , alternate day or five times in seven days, so as to keep the overall dose to near to the planned dose. For example a very young child;3-5 years of age would get about 125 mg per day. A child o 5-10 year would get 250 mg per day (or 500 mg alternate day) and an elder child received 5oo mg/day or 500 mg 5/7 days. Patients were on HU for variable period of time averaging to few years. Dosing modifications were minimal and were done only with change of age .No modifications were done using regular blood reports.
Previous records of Hb electrophoresis were available in 75 patients. The average value of HbS at diagnosis was 67 % and Hb F levels were 20.26 %.A random fresh sampling on the day of examination was done in 98 individuals , it showed an average of 71.91 % Hb S levels ( range-46% to 91%) and 27.63 %Hb F levels (range-9- 54%).
For 64 individuals(43 male, 21 female), pretreatment and post treatment data was, available for comparision. There was considerable time gap between the two readings depending on the age of the patient but was always few years, except in very young patients up to 3 years of age. No difference in report was less than 3 years.
There was no significant change in the levels of HbS between two readings. In age group of patients below 10 years of age, mean +/- standard deviation was 61.89 +/- 17.56 % at the time of diagnosis and 68.09 =/- 8.09 at point of present study, with a p value of 0. 222.In age group of 10-19 years the p value (0.031) was slightly significant. In all other groups HbS values changed insignificantly (Table 1).
HbF levels were also analyzed using available first report and reports performed on the day of this examination. In male population (n=43 ) the initial Hb F levels were 19.62± 8.0 and present review revealed the levels to be 27.66 ± 8.21, with a p- value of 0.001;In female population (n=21), the initials HbF levels were 21.71±9.30 which increased to 26.09±8.29 ,with p value of 0.005.Both the genders showed significant increase in HbF levels and average level of whole group was always higher than 20 %, range.
HbF levels were compared for various age groups (Table 2). In the age group of 1-10 HbF changed from 20.9 ± 8.68 to 31.90 ± 8.09 , which is statistically significant. In the age group of 10-19 years the value changed from 21.27 ± 8.06 to 26.41±8.16 (p =0.031). Similar changes were observed in all other age groups (Table-1) .In eldest group(age 40years - 49 years) ,interestingly, higher percentile change of HbF was very evident. It changed average from 19.02 ± 6.86 to 35.4±6.80 with significance p-value value of 0.001.
Overall, there was statistically significant increase in the levels of Hb F in all age groups. The Hb F levels lowest recorded were 5.6 % and the highest had gone to 46 %. There was change towards higher side in every single case. The change was evident in all age groups. All patients had reached values higher than 20 % of HbF. The highest values were as high as 46 %