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Success Story – Muhammad Abdullah Riaz

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Age 04 years, Beta Thalassemia Major Class III

Muhammad Abdullah Riaz

Contributed By: Dr. Tariq Ghafoor

Abdullah presented with pallor at five months of age and had 1st RCC transfusion. His diagnosis was confirmed at nine months of age with Hb electrophoresis. He had been on regular RCC transfusion since then and had received around 90 blood transfusions before going to transplant.

Iron chelation was regular and was started at the age of two years. He was fully matched with is sister and underwent bone marrow transplant on 18/08/2017 with Flu150, BuIV, Cy160 used as conditioning protocol. Neutrophil engraftment was achieved on Day +16. Post-transplant period was complicated with problems like febrile neutropenia, Platelet Refractoriness, VOD, ARDS, CSA induced hypertension and Haemorrhagic cystitis

Abdullah developed fever on (Day +3) and was started on Amikacin and Piperacillin / tazobactam. Amphotericin B was added to the regimen on (Day +5) as he continued to have fever spikes. His fever settled within 48 hours but again developed fever on (Day +10). Piperacillin / Tazobactam was swapped with Meropenem for better anaerobic cover. He again had fever spike on day +20 for which Meropenem was started again.

On day +7, Abdullah gained 1 kg weight with tender hepatomegaly (liver palpable 6 cm below costal margin) and hyperbilirubinemia. He gradually developed ascites with abdominal girth of 64 cm and later on he developed jaundice.

Abdullah developed platelets refractoriness on (day + 4). Despite continuous platelets transfusion his platelets count remained <10 X10^9/l. On day +9, he had oozing from injection site, gum bleeding, hematemesis, melena, haemoptysis and haematuria suggestive of platelet refractoriness and DIC. He was managed with single donor platelets, FFP, Cryoprecipitate transfusion and Inj Novo7 (factor 7) along with Sandostatin. He remained very critical for next 4 days due to VOD and DIC with gradual improvement from day +13 onward.

Abdullah had very difficult time due to HC. He developed haematuria on Day+10. He was initially managed with platelet transfusion and hydration. It gradually worsened and he started passing blood clots in urine. He also had severe pain at suprapubic region which was managed with frequent administration of Tramadol and Nalbuphine. His urine PCR for BK/ JC virus was negative.

Urologist was involved in the care and he underwent open clot evacuation 3 times under GA on day +36 day +38 and then on day +42. Supra-pubic Catheter (SPC) was inserted for continuous bladder irrigation and urethral catheter for urine output. With recurrent blockade of urethral catheter, he underwent Vesicostomy on day +48. He was started on IV Teicoplanin on day +38 when he had cystostomy for blood clot evacuation from bladder (ongoing haemorrhagic cystitis). Teicoplanin was stopped after 5 days on day +42.

On day +59 he had pus discharge from vesicostomy wound. Piperacillin / Tazobactam and Teicoplanin were started empirically. Pus C/S showed growth of Acinobacter and Stenotrophomonas for which antibiotic cover was switched to Colistin on day +64. Colistin was then stopped on day +74 before discharge.

Repeated blood and line fluid C/S revealed no growth. Pus C/S had growth of Acinobacter and Stenotrophomonas sensitive to Polymyxin B.