CASE STUDY OF A SUCCESSFUL TOTAL HIP REPLACEMENT,IN A HIGH RISK RARE BLOOD CONDITION- SICKLE CELL DISEASE
28 year old girl with sickle cell disease HBS 73 % HBA 24%- with bilateral avascular necrosis of hips , presented with hip
Sickle-cell disease (SCD), or sickle-cell anaemia (or anemia, SCA) or drepanocytosis, is anautosomal recessive genetic blood disorder with overdominance, characterized by red blood cellsthat assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the hemoglobin gene.
challenges for surgery and surgeon - 1. sickle crisis- during or after surgery
2.bleeding - increased risks
3. Deep venous thrombosis - higher risk
4. infection - higher risks
5. loosening - due to repetitive microinfarctions .
sickling is precipitated by due to acidosis, dehydration and hypoxia.
surmounting the obstacles-
1.Preop -infection work up
2. Preop - MRI to rule out any abscess/ infection source
3. Intraoperative OT temperature around 24 degrees to prevent hypothermia precipitating a crisis
4. Intraoperative patient warmer
5. Intraoperative NAHCO3 infusion to keep blood alkaline
6. Preoperative haematologist advise
7. Preoperative hydroxyurea admininstration
8. Preoperative packed cell tranfusion - raised Hb from 9.2 to 11 +
9. Pre and post NAHCO3 administration
10. Intraoperative local antibiotic wash and antibiotic impregnated collagen sponge instilled
11. Postoperative warmer
12.Post op dvt prophylaxis
13. post op early mobilization
14 . post operative pneumatic calf compression pump and stockings.
We performed an UNCEMENTED TOTAL HIP REPLACEMENT - METAL ON POLY MEGA HEAD ( depuy / J & J) successfully , her post op xray