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Management of severe haemophilia A during surgery: case report


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Image: Haemophilia specialist nurses play a vital role in planning and coordinating care for people with haemophilia undergoing surgery. The surgical team, the wider multidisciplinary team and patients all gain significantly from their involvement in ensuring the safe and effective delivery of care.© Shutterstock
Image: Haemophilia specialist nurses play a vital role in planning and coordinating care for people with haemophilia undergoing surgery. The surgical team, the wider multidisciplinary team and patients all gain significantly from their involvement in ensuring the safe and effective delivery of care.© Shutterstock

Figure 1

FVIII levels during continuous infusion
FVIII levels during continuous infusion

Comorbidities and current treatment

DIAGNOSISMEDICATIONDOSE
HIVTenofovir/emtricitabine RaltegravirOne daily 400 mg twice daily
Chronic hepatitis B co-infectiontenofovir/emtricitabine
Haemophilia AFVIII Tranexamic acid40 IU/kg (3000 IU) on alternate days, increased to daily administration, March 2017 1 g three times daily
EpilepsyPhenytoin300 mg once daily
Joint pains (mainly ankles)Celecoxib (Taken with Gaviscon as required)
HypertensionRamipril5 mg once daily

Postoperative management

POSTOPERATIVEDAYTIMEFVIII BOLUSDOSES (IU)FVIII CONTINUOUSINFUSION RATE(ML/HR)DOSAGE(IU/KG)FVIII LEVEL(IU/DL)CLINICAL ACTIVITY
Day 007:154000Pre – 43.1Post – 152.6
08:243.755CVP insertion, epidural, catheter, NGT
09:30105
10:4510004.56
11:45114.4
12:301000
13:40114End of theatre – 3 abdominal drains(silicone drain, Abdovac and Redivac)
14:505.257Transfer to intensive care unit
17:00126
Day 108:00160Extubated
09:554.56
13:00116
17:00119TPN infusion started
Day 208:00150
10:3034Clear fluids only
15:00144
17:302.253
Day 308:00147
10:451.52NGT out
16:00123.5
Day 408:00100.8Epidural out
17:0086.6
19:002.253
Day 508:00385.8
19:0091.8
Day 608:0088.8
19:0079.9
Day 708:0079
09:00Stop FVIII
17:003000Pre – 78.7Post – 133.2
Day 808:302000Pre – 65.4Post – 107.4
20:301500
Day 909:001500Pre – 68Post – 100.5
21001500
Day 1009:001500Pre – 70.4Post – 102.5Neck swelling – right side / painwhen swallowing. USS revealed clotoccluding right internal jugular vein.No collection.Enoxaparin 60 mg twice daily(administer 2 hours before FVIII)Bilateral leg Doppler USS – normal
21:001500Staph aureus growth at Abdovacdrain – start Flucloxacillin 1g fourtimes daily
Day 1109:001500
21:001500
Day 1209:001500
21:001500
Day 1309:001500Pre – 57Post – 87Swelling reduced; swallowing easier.Continue Clexane.
21:001500
Day 1409:001500Repeat neck USS – right internaljugular vein remains completelyoccluded with thrombus
21:001500
Day 1509:001500
21:001500
Day 1609:001500109.3 (45 minspost-FVIII)
21:001500
Day 183000 dailyDischarge homeClexane to continue at smaller dose:80 mg OD for 2 weeks.( to be injected2-3 hours before FVIII)Repeat neck USS in Bournemouth 2/52
3000 alt daysStop Clexane
Total FVIII used99,500From admission to discharge (day 1 to 18)
eISSN:
2055-3390
Language:
English
Publication timeframe:
Volume Open
Journal Subjects:
Medicine, Basic Medical Science, other, Clinical Medicine, Pharmacy, Pharmacology