Hemophilia is a rare bleeding disorder

Treatments

        In minor  disease factor  level  should  be  raised  to 20 %  - 30%  .

        In  severe  bleeding , it raised  at least  50% . For  the  major  surgery , it  raised to  100 % . preoperatively and  maintained  above 50%  until  healing .

         To  prevent  secondary hemorrhage  ,  maintain  10 -25% of  normal by 10 -5 ml/ kg /  12 h .

         Intravenous  infusion  of  factor  VIII  concentrate /FFP / cryoprecipitate .  

-         T/2  is about 12 h  ( at  least  twice  daily )

         >  30  ml / kg within 24  hrs  course  circulatory  overload .

-  1ml  of  plasma  contain 1u  of  factor  VIII 

-  stored  in  domestic  refrigerator  at 4 *c

               - immediately   after  bleeding starts  to  infuse 

         To  severely  affected  patients,  give  prophylaxis  3/52  time

 

Recombinant  factor  VIII  concentrated

-         reduced  risk  of  transmission  of  infection

-         safe  - but limited  supply

-         effective  - high  cost 

-                          -   similar incidence  of  antibodies  development

 

Synthetic  vasopressin ( DDAVP )   IV ,  S.C. or  intra  nasal

-         It avoid  complication  associated  with blood  products

-         to  mild  cases 

-         as prophylaxis  before miner  surgery 

-          

Never  give  aspirin  to  the  patient

prevent  trauma      

physiotherapy  give  to  pressure muscle  strength  and  avoid  damage  from             immobilization

Social  and  psychological  support .

Good  dental  care 

Avoid IM  injection

Each  person  with  hemophilia  carries  a  medical  card  which  gives  details of  the  disorder  and  its  treatment .

              Factor viii  1u /kg  increase 2 %  of  factor  level

                                15u/kg  increase  30%   ( haemostasis in joint bleeding )

                               30u/kg   increase  60%   (  required  in ICH and  to  cover  the  surgery )

tranexamic   acid  is  given  in  mucosal  bleeding .

              should not  used in  haematuria .

 

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smcopywrite 5 years ago from all over the web

hemophilia is a genetic disorder that is passed on from one generation to another. most hemophiliacs are male-nearly 98 percent.

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