Bolnik S Hemofilijo v Sloveniji: Od Leta 1967 Do Danes

Abstract

Izvleček:Celostna oskrba bolnikov s hemofilijo in drugimi prirojenimi motnjami strjevanja krvi, ki sodijo med zelo redke bolezni, mora biti premišlje-no organizirana. Zdravljenje krvavitev, njihovih posledic in zapletov ter preprečevanje krvavitev pri teh bolnikih je namreč zelo drago, vendar je neprimerno ali zgolj neustrezno organizirano zdravljenje poleg zdravstvene škode za bolnika še dražje, in sicer za bolnika in njegovo družino ter celotno skupnost. S pravilno obravnavo lahko danes bolnik s hemofilijo in drugimi prirojenimi motnjami strjevanja krvi dejansko živi normalno ustvarjalno življenje. Edino pojav inhibitorjev proti manjkajočemu faktorju strjevanja krvi lahko zelo zaplete zdravljenje, vendar danes ne usodno. Bolnika in njegovo družino je potrebno dobro seznaniti z naravo bolezni, s celostno obravnavo, in organiziranostjo obravnave, saj si lahko le malo zdravnikov in drugih zdravstvenih delavcev pridobi potrebne osebne izkušnje pri obravnavi teh bolnikov. AbstractThe comprehensive management of patients with rare diseases, such as haemophilia and other bleeding disorders, must be organized with extreme care and systematically. The treatment of bleedings, their common consequences and complications as well as the enforcement of basic prevention measures can be very costly. However, the costs of subsequent consequences of an improper and inadequately organized treatment are much higher. Besides its negative medical impact on the patient himself, harm to the patient's family and the society as a whole can be substantial and severe. Today, with proper treatment, the patient with haemophilia or other bleeding disorders can lead a completely normal and creative life.A potential cause of severe complications in patients with haemophilia and other bleeding disorders is the development of inhibitors against the missing coagulation factors, but today even this complication can be managed successfully.