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BACKGROUND: Sickle cell disease (SCD) refers to any of syndromes in which the sickle mutation is co-inherited with a mutation at the other beta globin allele that reduces or abolishes normal beta globin production.The major features are hemolytic anemia,vaso-occlusion, which can lead to acute and chronic pain and tissue ischemia or infarction.These pregnancies are at increased risk of obstetrical and fetal complications,medical complications of SCD are due,to metabolic demands, hypercoagulable state, and vascular stasis associated with pregnancy.
CASE REPORT:22Y old G2A1(MTP) with history of multiple blood transfusions (12-15) since the age of 12,on hydroxyurea pre-pregnancy,admitted in view of severe anaemia (Hb-5.7) with DCDA twin.Conception was spontaneous with 6 units blood transfusions in first trimester.7 units transfusion were done,but haemoglobin was declining.Extensive workup was done involving multidisciplinary team. She had fever episode . Dengue IgM+.4 UNITS of FFP transfusion due to deranged INR(6.9)..She was ANA+,DCT +,HPLC of SCD with Beta Thal trait.On USG ,hepatosplenomegaly with hemangioma and gammagandy body in spleen.Immune antibody profile showed minor blood group antibody.Ecosprin,Enoxaparin,vitcofol and methylprednisolone pulse therapy with antibiotic coverage and blood transfusion was done after complete crossmatch testing minor blood groups. Connective tissue disorder,APLA profile investigations negative. Her haemoglobin was stabilised (hb6-7).Readmitted at 28 weeks with joint pain.Haemoglobin 3.6 and pulmonary oedema, severe hypertension Dexamethasone,2 units of blood transfusion and 9 units of IVIG transfusion were done along with MgSo4.LSCS was done(30W) with 1 unit of intraop blood transfusion,Hypertension managed with medications and live male and female of 1.235 kg and 1.15 kg were born.Babies are stable in NICU.The patient is stable with post-op haemoglobin of 7.6g/dl and is on hydroxyurea.
CONCLUSION: Early identification of SCD women with twin pregnancy with vigilant surveillance, well-defined care plan and extensive collaboration with multidisciplinary team highlights the advantage of improved clinical outcomes in complicated cases.
