PREOPERATIVE PREPARATION AND ANAESTHETIC MANAGEMENT OF A PATIENT WITH HAEMOPHILIA A

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2 Int. J. Pharm. Med. & Bio. Sc Manjunath, 2012 Case Report ISSN Vol. 1, No. 1, July IJPMBS. All Rights Reserved PREOPERATIVE PREPARATION AND ANAESTHETIC MANAGEMENT OF A PATIENT WITH HAEMOPHILIA A Manjunath 1* *Corresponding Author: Manjunath, mcpatil52@rediffmail.com This case report describes the successful management of a 13 year old boy who presented with continued bleeding from post-fasciotomy wound on right forearm. The patient was investigated and diagnosed Haemophilia A. Later the patient was posted for skin grafting. The literature on the pre-operative preparation and anaesthetic management of patient with Haemophilia A posted for split thickness skin grafting is reviewed. Keywords: Haemophilia A, Factor VIII concentrates, Anaesthetic management INTRODUCTION Haemophilia A is characterized by hereditary deficiency of factor VIII (anti-haemophilic factor) and is most common hereditary disorder of coagulation, transmitted as a X-linked trait with variable expression, it is ordinarily carried by females who are unaffected. Thus it is a disease of males (Mannucci and Tuddenham, 2001). CASE REPORT A 13 year old boy weighing 40 kilograms presented to the casualty with continued bleeding from the post-fasciotomy wound on right forearm which was done to release compartment syndrome following post traumatic haematoma, performed in the referring hospital. On admission to our hospital, blood investigations were done and history noted. Past history revealed easy bruising and delayed clotting since childhood. There was no history suggestive of haemarthrosis. Family history was insignificant. On admission, his pulse rate was 128/min, BP- 90/60 mm of Hg, patient was pale, conscious and oriented. Systemic examination was insignificant. Blood investigations revealed Haemoglobin of 5.7 gm/dl, total WBC count of 9900/cc, platelet count of 1,62,000/cc. Two packs of whole blood were transfused, coagulation profile revealed Prothrombin Time 20 s. (control 14.8 s), INR 1.56, activated Partial Thromboplastin Time (aptt) 55.2 sec (control 32 s) following this Factor VIII and Factor IX assay were sought for 1 Department of Anaesthesiology, J.N.Medical College, Belgaum , Karnataka. 118

3 Int. J. Pharm. Med. & Bio. Sc Manjunath, 2012 and two packs of fresh frozen plasma (FFP) transfused. Factor VIII was 6.28% (normal %) (DiMichele and Neufeld, 1998), Factor IX was 72.6% (normal %) (DiMichele and Neufeld, 1998). Reviewing all these investigations the diagnosis of Haemophilia A was made. Additional two packs of FFP were transfused, bleeding was under control. Then the patient was started on Desmopressin nasal sprays on alternate days. Seven days later when oedema was reduced, the patient was posted for wound debridement and split thickness skin grafting. On the day before surgery Factor VIII was 8% and on the day of surgery with the aim of achieving the factor VIII level to near normal (100%) patient was transfused with 50 IU/kg (2000 IU) of factor VIII concentrates and taken up for surgery. Intra-operatively, heart rate, oxygen saturation and electrocardiogram were monitored. The patient was premedicated with inj.glycopyrrolate 0.004mg/kg, inj. midazolam 0.05 mg/kg, inj.fentanyl 2 g/kg. Preoxygenated for 3 minutes, anaesthesia was induced with inj.thiopentone 5mg/kg and inj.succinylcholine 1.5 mg/kg. Intubated with 7.0mm portex cuffed endotracheal tube. Anaesthesia was maintained with sevoflurane in oxygen and nitrous oxide, Inj.Vecuronium 0.1 mg/kg and ventilated mechanically. Intraoperatively 1.5 liters of I.V. fluids were transfused. Lasted for 1 hr 30 mins. Blood loss was insignificant. Patient was extubated after reversal using 0.05 mg/kg neostigmine, 0.01 mg/kg glycopyrrolate, and extubated. Postoperatively, the patient was haemodynamically stable, shifted to postoperative ward. 25 to 30 U/kg of Factor VIII concentrates were repeated every 8 to 12 hourly to keep the plasma factor VIII level above 50%. Desmopressin nasal spray one puff on alternate days, tab.tranexamic acid 500mg thrice a day, iron supplements were given. The treatment was continued for two weeks later on only iron supplementation, he was observed for any excessive bleeding and was discharged home on twentieth post-operative day. DISCUSSION 1 in 5000 males suffer from Haemophilia A. Nearly one third of cases represent new mutations with no family history and most frequently presenting in childhood as spontaneous haemorrhage involving joints or deep muscles or both (DiMichele and Neufeld, 1998). In general symptoms are internal or external bleeding episodes, patients with severe haemophilia suffer from severe and frequent bleeds, while patients with mild haemophilia typically have minor symptoms except after surgery or serious trauma. Moderate haemophiliacs have variable symptoms along a spectrum between severe and mild forms. In mild cases, patients may not be identified until later in life, often after unexplained bleeding following surgery or trauma (William and David Glass, 1990). Classically, laboratory testing in patients with haemophilia reveals prolongation of the aptt, whereas the prothrombin time (PT) and bleeding time remain within normal limits (Brinkhaus, 1964). Specific measurement of factor VIII:C is required to confirm the diagnosis and to clarify the severity of factor VIII deficiency (Brinkhaus, 1964). Individuals with less than 1% active factor are classified as having severe haemophilia, those with 1-5% active factor as moderate haemophilia and mild haemophiliacs have 5-40% of normal levels (Cahill and Colvin, 1997). 119

4 Int. J. Pharm. Med. & Bio. Sc Manjunath, 2012 Our patient was diagnosed mild Haemophiliac as he presented at 13 years of age following trauma, with prolonged aptt and reduced factor VIII levels. Desmopressin is useful in the treatment of mild Haemophiliacs (DiMichele and Neufeld, 1998), hence we used nasal sprays on alternate days. Tachyphylaxis may develop on repeated dosing of desmopressin. In order to increase the preoperative levels of factor VIII to 100%(Gihan and Nasar, 2010), dose of factor VIII concentration was calculated using the formula(valder Arruda and Katherine, 2008), FVIII dose (IU) = (Target F VIII level Baseline F F VIII level) x Body Wt. (kg) x 0.5 Accordingly, dose of factor VIII concentrates is 2000 IU in our patient, since the half life of factor VIII is approximately 12 hours repeat doses were administered for 2 weeks. Peak and trough factor VIII levels should be measured to confirm the appropriate dosing level and dosing interval. Therapy must be continued for up to 2 weeks to avoid postoperative bleeding that disrupts wound healing. Longer periods of therapy (4 to 6 weeks) may be required in patients who undergo bone or joint surgery. Up to 30% of severe Haemophilia A patients exposed to factor VIII concentrate or recombinant product will eventually develop inhibitor antibodies, some within 10 to 12 days of first exposure. Hence a patient receiving factor VIII concentrate has unusually prolonged bleeding an assay of the antibody titers have to be done (Schraf et al., 1996). REFERENCES 1. Brinkhaus K M (1964), The Hemophilias, Chapel Hill, University of North Carolina Press, p Cahill M R and Colvin B T (1997), Haemophilia, Postgrad. Med. J., Vol. 73, pp DiMichele D and Neufeld E J (1998), Hemophilia: A New Approach to an Old Disease, Hematol. Oncol. Clin. North Am., Vol. 12, pp Gihan E L and Nasar M D (2010), Anaesthetic Management of Patient with Haemophilia A, Ain Shams Journal of Anesthesiology, Vol. 3-2, July, pp Mannucci P M and Tuddenham E G (2001), The Hemophilias from Royal Genes to Gene Thepary, N. Engl. J. Med., Vol. 344, pp Schraf R, Kucharski W, Nowak T (1996), Surgery in Hemophilia A Patients with Factor VIII Inhibitor: 10-Year Experience, World J. Surg., Vol. 20, pp Valder Arruda and Katherine A (2008), High- Coagulation Disorders, in Fauci A S, Braunwald E, Kasper D L, Hauser S L, Longo D L, Jameson J L et al. (Eds.), Harrison s Principles of Internal Medicine, Vol. 1, 17 th Edtion, pp , Mc Graw Hill Inc., New York. 8. William E D and David Glass D (1990), Haematological Diseases, in Katz Benumof, Kadis, Anesthesia and Uncommon Diseases Philadelphia, pp

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