5597199 AGEING WITH SICKLE CELL ANEMIA AND HOW TO FACE IT

Abstract

Background: Sickle Cell Anemia, a hereditary monogenic disorder is caused by homozygous sickle mutation (Hb SS). Sickle erythrocytes promote vaso-occlusion and haemolysis, which are two major hallmarks of the disease. Vaso-occlusive crisis results from the sickle red cells obstructing and reducing blood flow to the vital organs leading to ischemia, necrosis and pain(2). Data show that other blood cells i.e. leukocytes and thrombocytes are involved in this process as well, and the process starts from up regulation of P-selectin which contributes in cellular interaction and vaso-occlusion later on(3,4). All these cells stick together and can’t easily move through the blood vessels. This situation can block small blood vessels and the movement of healthy, normal oxygen-carrying blood(5,6). Repeated episodes lead to bone and organ infarction and necrosis; organs damage occur over time. Hemochromatosis occurs as well, as a result of blood transfusions or/and persistent hemolysis. Patients suffering from this kind of anemia feel exhausted and need strictly monitoring for every health issues they represent, especially when they get older(8). Because of this pathologic condition’s complexity, the best method of treatment is a combined approach(1). Sickle Cell Disease is a very polymorphous disease and its severity varies largely in each patient, thats why the management has to be in a personalized model(7). Aims: To give an overview and our considerations related to Sickle Cell Disease in Albania Methods: To achieve the goal of treating and managing medically people living with Sickle Cell Disorder, we monitor every single sign and laboratory fluctuation happening to these patients in every clinical visit. F.A. is a female with Sickle Cell Disorder, 41 years old, diagnosed in early infancy and since then she has been treated with blood transfusions initially(in her hometown until 19 years old) and later on with pure red blood cells and iron chelation therapy in our clinic. This treatment was performed once per month. She was receiving Hydroxycarbamide as well. She started to be non compliant to ICT (iron chelation therapy) and as a consequence she has been presented with very high value of ferritin level (above 5000 ng/ml). On 2013 she gave birth to a baby. A severe haemolysis was seen over time, she was put in transfusions twice per month. No changes in ultrasound or images. Two years ago she demonstrated a severe anaemia, arterial hypertension and proteinuria. She was diagnosed with Chronic Kidney Disease grade 3. Since then she had a personalised supportive therapy and this year was put in dialysis. Results: According to our experience and our point of view Sickle Cell Disease seems to have a moderate burden in our country. A new era of novel drugs seem to encourage and realize better achievement in SCD management. Sharing best experiences and our clinical cases between us, may affect positively the quality and management of these diseases. Summary: A better understanding and a strictly monitoring of patients suffering from Sickle Cell Disease could help health professionals and patients themselves to cooperate and develop new modalities towards managing these polymorphous conditions.