HEALTH-RELATED QUALITY OF LIFE OF PATIENTS WITH SICKLE CELL DISEASE

ABSTRACT

Sickle cell anaemia is a homozygous form of HbS (HbSS). This result from single point replacement of glutamine by valine at position 6 of β-globin chain.This reduces solubilty of the red cells which in turn leads to polymerisation and vaso-occlussion in the vasculature. The βglobin gene is found on the short arm of chromosome 11. This project aims at describing the pathiophysiology, diagnosis, and health related quality of life of patient with sickle cell disease on hydroxyurea. Materials from this project work were gotten from relevant publications, journal articles, PubMed, Google Scholar, Science Direct. Findings from the seminar showed that Some Therapy for Sickle cell disease include hematopoietic stem cell transplant, gene therapy and hydroxyurea which has been shown to be efficacious for the treatment of sickle cell anemia (SCA). It also showed that Hydroxyurea, a myelosuppressive agent, is the only effective drug proven to reduce the frequency of painful episodes. It raises the level of HbF and the haemoglobin level. It usually decreases the rate of painful episodes by 50 %. It was first tested in sickle cell disease in 1984. It also decreases the rate of Acute Chest Syndrome episodes and blood transfusions by -50 % in adults. In conclusion, Health-Related Quality of life of patients with sickle cells disease has greatly improved with the use of hydroxyurea.