It is a X-linked defective gene transmission which affects the production of clotting factor protein, it may be either factor VIII ( hemophilia A ) or factor IX ( hemophilia B ). Females have two X chromosome, of which if one X chromosome is defective, then she is a carrier. This state mostly goes un-noticed but when that defective X chromosome gets transmitted to generation in a male child, then that child suffers from Hemophilia, a bleeding disorder. Bleeding can occur at any site with or without injury ( spontaneous), internally or externally.
Joints are commonly affected. Repeated joint bleed events may lead to chronic degenerative changes. Therefore, gradual functional impairment progresses to disability and established deformity. All bleeds are not due to hemophilia. There can be deficiency of fibrinogen, factor XIII, Von Willebrand activity or reduced platelet count or platelet function defect which needs a proper investigation.
1) Acute bleed management : Should start from the point of time when the PWH senses the bleed. The quicker the action, less will be the suffering.
2) Quick modality of treatment ( i.e. within two hours of onset of bleed) with factor infusion and follow the protocol of “ P R I C E “ for 24 hrs. Demand dose of factor concentrates to be given as per body weight and the site of bleed along with its severity.
