The RBCs of a patient with sickle cell disease has 40% abnormal beta hemoglobin chains which form the hemoglobin S (HbS), inherited from one or both parents thus possessing two of these alleles and a total hemoglobin of “80-100%.” A carrier of this genetic disorder would have one HbS and HbA allele. In decreased oxygen conditions, the RBCs of the SCD individual “assume a sickle shape, become rigid, and clump together” (sticky and fragile) blocking blood flow and causing “a vaso-occlusive event (VOE)” and pain in affected joints. This obstruction leads to reduced oxygen capacity of hemoglobin reaching the tissue and other parts of the body leading to ischemia. When ischemia repeatedly occurs due to deficient oxygen in the blood, it leads to “progressive organ damage from anoxia and infarction.” (Ignatavicius & Workman, …show more content…
Early signs include general fatigue and swelling of hands and feet which occur as a result of blood vessel occlusion common in the joints of the body due to decreased oxygen levels causing the RBCs to have the sickle shape which inflicts pain and weakness on the individual. The SCD patient will exhibit psychosocial and behavioral changes such as agitation, frayed nerves, negative thinking and confusion which are “early manifestations of cerebral hypoxia from poor tissue perfusion.” Poor perfusion of oxygen in body systems due to decreased levels lead to further symptoms which include increased heart murmurs, heart rate, low blood pressure, decreased pulse pressure, delayed capillary refill, jaundice, ulcers, Chronic Kidney Disease, and dark yellow or amber colored urine. (Ignatavicius & Workman, 2013). Many of these later symptoms occur simultaneously in the body systems and are more common during acute pain episodes. (Porth, 2013). As a result, there is an increased risk for infection due to damage to major organs such as the spleen, liver and the central nervous system due to “low-grade fever” leading to seizures and or