Abstract

HEMOPHILIA-INHERITED BLEEDING DISORDER: AN OVERVIEW

S.B. Somwanshi*, V.B. More, S.N. Hiremath1, R.T. Dolas, K.B. Kotade,V.M.Gaware

ABSTRACT

Hemophilia is an inherited bleeding disorder where one of the bloods clotting proteins is absent or present in a reduced amount. People with Hemophilia, do not bleed faster than anyone else; but will bleed continuously at the normal rate until they are treated. Hemophilia has an estimated frequency of approximately one in 10000 births. Estimations based on the World Federation of Haemophilia’s (WFH) annual global surveys indicate that the number of people with Hemophilia in the world is approximately 400000. The history of Hemophilia shows the human mind attempting to define and encompass a mysterious yet fascinating phenomenon; and also the human heart responding to the challenge of repeated adversity. There is obviously a need to establish facilities and treatment options that will help the patient with Hemophilia, to manage their life with ease. As this is a genetic disorder no complete cure is possible as of now. The available treatment for Hemophilia is by replacing the missing clotting factor in the blood through an intravenous infusion of clotting factor concentrate. Several new technologies are also being implemented to advance Hemophilia, treatment. Multiple trials and studies are underway to examine the possibility to use gene therapy to replace the defective genes in Hemophilia. To date, stable and sustained production of the deficient clotting factors has not been achieved in humans, but this is an area of active investigation that holds great promise for the new delivery systems of the existing therapies or new treatment options has to be driven by pharmacy professionals. The present review broadly describes an overview of the management of various aspects of Hemophilia in order to draw the attention of medical as well as pharmacy professionals for the benefit of millions of hemophilic patients.

Keywords: Hemophilia, Clotting factors, Hemostasis, Inheritance, Prophylaxis, Treatment