PB2214: SCD POPULATION SURVEY IN ALBANIA, A PROSPECTIVE NON-INTERVENTIONAL MULTI-CENTER STUDY EVALUATING DISEASE AND PATIENTS CHARACTERISTICS OF SICKLE CELL DISEASE IN ALBANIAN SCD POPULATION

Abstract

Background: SCD is a group of inherited disorders affecting hemoglobin (Hb) synthesis. Sickle cell erythrocytes have a mutant form of Hb, sickle Hb (HbS), which turns normally pliable erythrocytes into rigid, sickle-shaped cells. We conducted a survey project for generation of local data on SCD, to deeper understanding disease burden, patient journey treatment pathways and QoL and get insights on SCD epidemiology on national level, in our country Aims: The study answered the following main research question: What is the frequency (number per year) of vaso-occlusive crises (VOCs) and their overall QoL in patients with Sickle Cell Disease within routine treatment regimens in the real-world setting in Albania and different regions among permanent resident SCD patients? Methods: This study was a cross-sectional primary data collection NIS focused on disease that enrolled 242 patients with confirmed SCD diagnosis who signed patient consent forms. 10 selected physicians from Albanian Association of Hematology from 7 centers across the country fulfilled a questionnaire (survey) for each SCD patient, based on patient medical history (retrospective data) and interviewing the patient live (collecting primary data-EQ-5D instruments). Results: The study demonstrated in fatigue, 11% of patients reported “severe” and “very severe”; in pain/discomfort, 8% reported “severe” and “very severe”, while in mobility only 3.7% reported “severe” and “very severe”; in self-care, the majority of patients reported “no”, “mild” or “moderate” (respectively 86%, 11%, 2.5%); and usual activity dimensions, most of patients reported “no”, “mild”, “moderate” (respectively 64%, 26%, 7.4%). The data we received from our study corrected with the data from literature. Our study reported 37% of patients reported more than 2 VOCs requiring medical care (hospitalization or other medical facility visit and treatment with oral or parenteral narcotic agents or parenteral NSAIDs), while 67% of patients reported more than 2 VOCs in last 12 months managed at home. Image:Summary/Conclusion: This is the first study conducted in Albania in SCD disease area. The study demonstrated the local burden of SCD, the impact of the disease on QoL of SCD patients, one overall of prevalence, geographical distribution of SCD patients, clinical characteristics of sickle cell patients, treatment landscape and clinical practice in Albania. According to our results we concluded that in Albania the burden is considered moderate. The study concluded also some very important findings about the role of treatment and quality of life of SCD patients and the need for other treatment options to improve the outcomes of SCD patients.