Abstract

End Stage Kidney Disease (ESKD) in patients with Sickle Cell Nephropathy is marked with an inexorable clinical deterioration with an increase in frequency of painful crisis, transfusion requirement and hypertension. This makes Kidney Transplant in these set of patients quite challenging. A 38 year old known HbSS patient presented in our clinic on referral from her haematologist. She has been managed for hypertension, recurrent anaemia and generalized body weakness of three year duration. Electrolytes, Urea and Creatinine assay at presentation reveals significantly elevated Potassium, Urea and Creatinine levels. An assessment of chronic kidney disease stage V was made. She was previously diagnosed in another facility of End Stage Renal Disease secondary to Sickle Cell Nephropathy for which she has been receiving twice weekly maintenance haemodialysis for the past 2 years. She has also previously had right Hip replacement surgery done 3 years prior to presentation due to avascular necrosis of the head of femur. She was subsequently counselled for renal transplant to which she consented and a non-related voluntary donor was sought. Intervention She was worked up for renal transplant and a living non-related voluntary donor was sought. Meanwhile, maintenance hemodialysis was continued ahead of the kidney transplant. At PCV of 11.8% on 3/11/15, 8000IU of Erythropoietin was given on account of severely low PCV. A renal transplant was subsequently done on the 8th November, 2015. Renal Transplant Protocol/Procedure Pre-operatively Both donor and recipient were routinely screened for compatibility and against possible infections. In addition, antibiotics cover was given and the drug of choice Ceftriaxone given intravenously 12 hourly ahead of the transplant. Pre exchange Hb electrophoresis quantitation done revealed a HBS level of 80% and HbA1 level of 5%. Four (4) exchange blood transfusions were done with fresh, genotype AA blood until the concentration of haemoglobin A1, was above 60% and HBS was below 20%. We administered albendazol 400mg and ondasetron 4mg tablets in statum dose. For immunosuppression, Tabs Tacrolimus 4mg and Tab MMF 1g were both given as statum doses 6 hours pre-operatively. In theatre IV Basiliximab 20mg was diluted in 50ml of Normal saline. Also, methylprednisolone 500mg was given in the theatre. This was continued till 2 days post operation. Thereafter, Tab prednisolone was given 40mg daily and subsequently tapered down to 5mg daily by 8 days post-operation. Post Operatively Urine output was monitored hourly and this was essentially satisfactory. Vital signs were monitored half hourly and adjusted accordingly. Fluid replacement was done at ratio 1:1 Tacrolimus level was assessed by day 3 post operation and IV Basiliximab repeated 20mg in 50ml Normal saline on day 4 The drain was then removed on day 5, followed by urinary catheter which was removed on day 8 together with stitches and staples removal same day. Kidney Transplantation in Sickle Cell Nephropathy patients can be rewarding for the recipient if successful. Especially considering the various complications that these set of patients do have. However, data is scarce and so more studies are required.

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