Sammendrag
Background: Sickle cell disease (SCD) is
characterized by frequent crises requiring
hospitalization. The objective of this study is to
determine the types and frequencies of the various
crises in adult sickle cell disease patients.
Methods: This is a cross-sectional study of adult
SCD patients accessing emergency care in a
Nigerian tertiary hospital over a three-month
period.
Results: The mean age and age at diagnosis of the
73 patients seen were 31.8±11.4 and 7.3±6.6 years
respectively. HbSC patients were older at
diagnosis than HbS patients (14.8±3.6yrs vs
6.5±0.9yrs; P=0.007). Steady state haematocrit
was significantly higher than haematocrit at
emergency visit (24% vs 20.7%; P<0.001). Forty
eight (65.8%) patients had bone pain crisis (BPC),
twenty-four (32.9%) had haemolytic crisis with
dark brown urine occurring in 14 (58.3%) while
15 (20.5%) had both BPC and haemolytic crises.
Nineteen (27.9%) admitted to rarely/never having
BPC, 32 (47%) had <1BPC in a year, 13(19.1%)
had 1-3 BPC per year while 4(5.8%) had >3
episodes. There was no association between the
number of BPC per year and blood transfusion
(P=0.51) but BPC per year was associated with
hospital admission (P=0.04). Blood transfusion
was also associated with hospital admission
(P=0.001). The age at diagnosis was associated
with the steady state haematocrit (P=0.045) but
not with BPC per year (P=0.1).
Conclusion: The coinheritance of G6PD prevalent
in the community may play a significant role in
the associated intravascular haemolysis of the
haemolytic crisis. The late age at diagnosis is an
urgent call for newborn screening of the disorder.
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