Answer
People with sickle cell disease produce an abnormal hemoglobin because of inheritance of variant hemoblobin genes. The variant hemoglobin is called hemoglobin S and it causes red blood cells that contain it to behave abnormally. In addition the RBS of a person with sicle cell disease have much shorteneed life span . The result is that subjects with this condtion suffer oxygen deficits whic impacts tissues negatively gererates characterstic signs, symptoms and sybndroems
Some of the signs and symptoms of sicle cell disease are shortness of breath, fatigue, paleness. bone pain, fever, tachycardia, and excessive urination, painful and prolonged erection in males.
Work Step by Step
Som of the more serious manifestation of sickle cell disease include damage to brain, lungs, spleen and kidneys . Also, some subjects develop leg ulcers, jaundice, swelling and inflammation, In some cases children experience delayed growth and mental development
These problems result because the hemoglobin in this disease is not hemoglobin A, but hemoglobin S, an abnormal hereditary hemoglobin. When RBCs with hemoglobin S exchange oxygen with interstitial fluid (ISF) these RBCs lose their round biconcave shape and morph into a crescentic ( sickle shaped) structure . This structures rupture easily and spill their hemoglobin; as a consequence the blood loses oxygen- carrying capacity .These sickled RBC's live an average of about 50 days, whereas the life of an RBC with normal hemoglobin is 100 to 120 days. The erythropoietic tissues try to keep up with the losses by increasing the output of new RBCs .But it is a losing game. The RBC loss usually, in time, outpaces replacement and when that happens the Sickle cell subject experiences a health crises due to symptoms caused by oxygen deficit.
