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Success Story – Raja Umer

raja umer

Age: 12 years, Beta Thalassemia Major Class III

Raja Umer

Contributed By: Dr. Tariq Ghafoor

Raja Umer Shahid presented with fever and progressive pallor at 06 months of age. Hb Electrophoresis was consistent with Beta thalassemia major. He received first transfusion at 6 months of age since then he was transfusion dependent

Oral Iron chelation was started at the age of 01 year and was irregular. When he presented at AFBMTC on 25/08/2021, his serum ferritin was 9175 ng/ml. He was advised intensive iron chelation with three drugs (desferrioxamine, Deferasirox and deferiprone). His serum ferritin was controlled over one year of iron chelation

Raja Umer Shahid was fully matched with his younger sister .He underwent   Matched Sibling Donor Bone marrow transplant on 10/11/2021 with Flu120, Bu16 IV, TG5, Cy160 used as conditioning protocol. Neutrophil Engraftment was achieved on day +13

Post transplant period was complicated by febrile neutropenia, Mucositis III, Hematuria II and CSA induced Hypertension. He developed Skin GVHD stage 1 grade 1 on Day +28. He was started on oral Prednisolone at 2mg/kg and tropical betamethasone. His skin rashes settled on day+32. He was started on IV Methylprednisolone 50mg OD and IV Meropenem.

Raja Umer was admitted on Day +34 as frequency of loose stools increased (4-5 episodes / day), watery in consistency, greenish in colour with abdominal cramps but with no blood. He was started on IV Meropenem, IV cyclosporine, IV Omperazole, oral ruxolitinib   on same day along with IV fluids. He was already on I/V methylprednisolone 50mg IV OD for two days. He was kept NPO for 2 days. Acyclovir, Mycophenolate and Norvasc were also continued. His stool R/E and stool for cryptosporidium were negative.

His loose stool volume continued to increase (2000ml / day, Day +35). He was started on oral budesonide 3mg TDS. Inj Infliximab 10mg/kg (300mg) 1st dose was given on 14/01/22 (Day +35) and 2nd dose on (Day +42). Frequency of loose stool gradually decreased over next 2 days and was allowed clear fluids orally.

He developed facial puffiness and swelling over hands and both feet on (day+37). Serum Albumin was 20 g/l. He was given Inj Albumin 20% 100ml for two days. His peripheral oedema improved with Albumin replacement.

IV methylprednisolone and Meropenem were stopped on (Day+39). IV cyclosporine was switched to oral cyclosporine on same days. However MMF (Cellcept), Ruxolotinib (Jakavi) and oral Budesonide were continued.

Umer had fever spike of 101F and was started on Meropenem on (Day +46).

During routine surveillance his COVID PCR became positive on 25/01/2022. Repeat covid-19 PCR next day was also positive. He was given intravenous immunoglobulin 15gram on 27/01/22 as he was immunocompromised.