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 %