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Suffering from hemophilia type a. Had abdominal pain, vomitting. Given bolus of Novoseven medicine. Suggestions?

Hello Sir/ Madam I am Ravi Patel from Indore, Madhya Pradesh, India. I am facing a very serious problem in my family. My uncle Mr. Paresh Patel is Hemophilia Patient having “Hemophilia Type A”. Presently he is in very serious condition and so I need your help or opinion. Here with giving brief history of Mr. Paresh Patel (Age 43) by his Doctor. On 20th morning at around 2.00 AM he had a pain in abdomen and vomiting .he was infused with 2000 i.u of AHF immediately.after 6 Hrs. pain is increased and he was uncomfortable. Radio logically it was diagnosed with retro peritoneal hemetoma. he was infused regularly doses of factor at regular interval.but coagulation was not achieved for 24 the time he had a bleed in lungs and he was intuubated and supported with respirator. His Haemoglobin was falling and maitained by RCC transfusions same evening we received the report of presence of inhibitors.started FEIBA and his renal function was impaired and creatinine was moree than 7.his weight is 60 Kg.and he is maintain on FEIBA accordingly. on 2nd December he had a nasal bleed which was controlled by FEIBA. Today his creatienine 2.5mg/dl , APTT 96 sec. and Hemoglobin 9.2 Again he had same nasal bleed at around 5 AM.and he is applied in neutral nasal packs. at present his blood pressure is maintained and hemoglobin is maintained and heis given first bolus of Novo seven 5.2 mg Infusion Details FACTOR Date Time Factor- VIII I.U 19.11.2012 12;30 am 2000 2011.2012 09;00 am 2000 20.11.2012 08;00 pm 3000 21;11;2012 11;30 pm 2000 21.11.2012 09;00 am 750 21.11.2012 11;00 am 716 21.11.2012 03;30 am 1074 21.11.2012 06;15 am 716 21.11.2012 08;30pm 716 22.11.2012 03;30 am 2000 22.11.2012 07;30 am 2000 21.11.2012 11;30 pm 2000 22.11.2012 03.30 am 2000 22.11.2012 07;30 am 2000 22.11.2012 11;30 am 2000 23.11.2012 03;30 am 2000 23.11.2012 7;30 am 2000 23.11.2012 10.00 am 2000 FEIBA Date Time FEIBA I.U 23.11.2012 10;15 am 6000 23.11.2012 11;00 pm 3000 24.11.2012 06;00 am 3000 24.11.2012 04;00 pm 6000 25.11.2012 04;00 am 6000 25.11.2012 01;30 pm 6000 26.11.2012 01.30 am 6000 26.11.2012 03.30 pm 6000 27.11.2012 03.00 am 6000 27.11.2012 03 5736 28.11.2012 03.00 am 4500 28.11.2012 05.00 pm 4500 29.11.2012 5.00 am 3000 29.11.2012 5.00 pm 3000 30.11.2012 3.00 am 3000 30.11.2012 3.00 pm 3000 1.12.2012 4.oo am 3000 1.12.2012 7.30 pm 3000 2.12.2012 10.00 am 4500 2.12.2012 10.00pm 4500 3.12.2012 10.00 am 4500 3.12.2012 10.00 pm 4500 4.12.2012 4..00 am 3000 4.12.2012 5.00 am 3000 NOVO SEVEN 5.4 mg on 4 dec.2012 pl. opine thanks. Mo no. 91 0000
Asked On : Sat, 8 Dec 2012
Answers:  2 Views:  43
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General & Family Physician 's  Response
start porcine factor VIII or recombinant factor VIIa or start immune tolerance therapy by infusing large dose (25-100 iu/kg/day) of factor VIII daily/alternate days for months till the inhibitor level become un detectable, it can be combined with immunosuppressive agents, keep on doing factor assay to monitor the treatment and to prevent any further bleeding episode and also keep on checking inhibitor status whether its concentration is decreasing or not.
Answered: Thu, 3 Jan 2013
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General & Family Physician Dr. Mamun Kabir's  Response
than you.hemophilia A is due to factor eight deficiency & it is treated by factor eight concentrate.but at this stage your uncle is not responding to it probably due to development of antibody against it.but FEIBA is a good option in this case which is continuing repeated epistaxis is a problem.gradually factor seven is to be increased.
Answered: Mon, 31 Dec 2012
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