Management Haemophilia

1 management

1.1 clotting factors
1.2 other
1.3 contraindications

management

while there no cure haemophilia, treatment improves outcomes.

clotting factors

commercially produced factor concentrates such advate , recombinant factor viii, come white powder in vial must mixed sterile water prior intravenous injection.

clotting factors not needed in mild haemophilia. in moderate haemophilia clotting factors typically needed when bleeding occurs or prevent bleeding events. in severe haemophilia preventive use recommended 2 or 3 times week , may continue life. rapid treatment of bleeding episodes decreases damage body.

factor viii used in haemophilia , factor ix in haemophilia b. factor replacement can either isolated human blood serum, recombinant, or combination of two. people develop antibodies (inhibitors) against replacement factors given them, amount of factor has increased or non-human replacement products must given, such porcine factor viii.

if person becomes refractory replacement coagulation factor result of circulating inhibitors, may partially overcome recombinant human factor vii.

in 2008, food , drug administration (fda) approved anti-haemophilic factor, genetically engineered genes of chinese hamster ovary cells. since 1993 recombinant factor products (which typically cultured in chinese hamster ovary (cho) tissue culture cells , involve little, if human plasma products) have been available , have been used in wealthier western countries. while recombinant clotting factor products offer higher purity , safety, are, concentrate, extremely expensive, , not available in developing world. in many cases, factor products of sort difficult obtain in developing countries.

clotting factors either given preventively or on-demand. preventive use involves infusion of clotting factor on regular schedule in order keep clotting levels sufficiently high prevent spontaneous bleeding episodes. on-demand (or episodic) treatment involves treating bleeding episodes once arise. in 2007, trial comparing on-demand treatment of boys (< 30 months) haemophilia prophylactic treatment (infusions of 25 iu/kg body weight of factor viii every other day) in respect effect on prevention of joint-diseases. when boys reached 6 years of age, 93% of in prophylaxis group , 55% of in episodic-therapy group had normal index joint-structure on mri. prophylactic treatment, however, resulted in average costs of $300,000 per year. author of editorial published in same issue of nejm supports idea prophylactic treatment not more effective on demand treatment suggests starting after first serious joint-related haemorrhage may more cost effective waiting until fixed age begin.

other

desmopressin (ddavp) may used in mild haemophilia a. tranexamic acid or epsilon aminocaproic acid may given along clotting factors prevent breakdown of clots.

pain medicines, steroids, , physical therapy may used reduce pain , swelling in affected joint.

contraindications

anticoagulants such heparin , warfarin contraindicated people haemophilia these can aggravate clotting difficulties. contraindicated drugs have blood thinning side effects. instance, medicines contain aspirin, ibuprofen, or naproxen sodium should not taken because known have side effect of prolonged bleeding.

also contraindicated activities high likelihood of trauma, such motorcycling , skateboarding. popular sports high rates of physical contact , injuries such american football, hockey, boxing, wrestling, , rugby should avoided people haemophilia. other active sports soccer, baseball, , basketball have high rate of injuries, have overall less contact , should undertaken cautiously , in consultation doctor.