HAEMOPHILIA IN ORTHOPAEDICS PRESENTER: DR. LEMAYIAN DISCUSSANT: DR.KARANU

B.O. MALE 35YRS OLD DISTRICT OF ORIGIN: MARSABIT DATE OF ADMISSION: 2/3/2012 DATE OF DISCHARGE: 21/8/2012

Referral from Marsabit District Hospital as a known haemophiliac 1/12 h/o septic wound dorsal aspect of right foot Involved in an RTA 1/12 prior sustaining degloving injury to the dorsal aspect of the right foot

Reason for refferal Surgical debridement done twice at the hospital resulted in excessive bleeding FURTHER MANAGEMENT

PMHx/PSHx Excessive bleeding during circumcision Recurrent swelling of the knee joints since he was 5yrs old Occurred with trivial falls and while playing Small cuts and bruises that would bleed for long Admitted severally to Marsabit D.H. with DX of BLEEDING DISORDER Multiple whole blood transfusions Progressive joint deformation esp RT. Worsened in 1997 resulting in pathological fusion

FSHx 1st born in a family of 5 siblings(2 other brothers and 2 sisters) Both brothers are also known haemophiliacs DX made at KNH in one of the younger brothers in 1990 when he was referred with similar severe bleeding disorder Patient works as a shop attendant in Marsabit

Maternal Side-9 of his mother’s relatives had died due to excessive bleeding post circumcision

EXAMINATION UPON ADMISSION FGC , wasted, Mildly pale BP-109/67mmHg, PR-60/min MSS: Fused right knee in fixed extension, valgus deformity Wasted LL musculature bilaterally Necrotic wound on the dorsal aspect of right foot L knee also deformed in fixed flexion No signs of any soft tissue swellings Sensation was intact OTHER SYSTEMS: Essentially normal

INVX AT ADMISSION CBC- Hb 9.38g/dl -WBC 5.23; Neutrophil 50% -platelets 294 Coagulation profile -PT test 19s -PTI 73.7% -INR 1.36

-APTT test >120s -APTT control 30s U/E/CR-normal XRAY L KNEE-complete joint fusion -subchondral cysts -disuse osteopaenia

SUBSEQUENT TESTS p24-Reactive CD4+-205 cells/microlitre Viral load-? Substitution Tests Normal plasma+ pt’s plasma(1:1)—39.5s FVIII def plasma+ pt’s plasma(1:1)—75.1s FIX def plasma + pt’s plasma(1:1)—50.4s DEFINITIVE DX: FVIII def (Haemophilia A)

Progressively: Sepsis worsened despite several surgical debridements Metartarsals became exposed Nutritionist involvement—nutritional supplementation Haematologist review Initial Decision-midfoot amputation

In view of worsening and ascending infection (as well as patient preference) decision made to do BKA

Pre-Op blood workup CBC- Hb 7.38g/dl; WBC 4.47; Neutrophils- 44.3%; Platelets- 320 U/E/CR-normal(Na+ 131; k+ 3.52; Urea 2.3 mmol/l) GXM 4 Units of cryoprecipitate

Transfused 2 units pre-op, 1 unit intra-op and 1 unit post-op BKA done on 13/8/12 Post-op medication: i.v. tramadol 50mg BD i.v ceftriaxone 1g BD Discharged through both orthopaedic and haematology clinics