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Signs and Symptoms of Sickle Cell Anemia

Sickle cell disease usually begins in early childhood. The severity of symptoms varies from person to person and is especially determined by nutrition, genetic factors, stress, ambient temperature, air pressure and other factors. Some people have mild symptoms, while others are frequently hospitalized for more serious complications. Beginning in childhood, just before the first birthday, patients suffer unpredictable and recurrent episodes severe VOCs due to blocked blood flow to organs and extremities, often leading to psycho-social and physical disability. The constant destruction and rupture of red blood cells releases their content into the blood, often leading to damage and diseased blood vessels, further exacerbating blood flow and multi-organ damage. SCA can lead to hemolytic anemia (the destruction of red blood cells within blood vessels), vaso-occlusion (blocked blood flow to tissues), progressive multi-organ damage and early death. Patients with characteristic anemia experience fatigue, weakness, shortness of breath, dizziness, headaches, and coldness in the hands and feet. Anemia can also cause delayed growth and development in children. Deprivation of oxygen-rich blood is especially deleterious to the lungs, kidneys, spleen, and the brain. A particularly serious complication of SCA is pulmonary hypertension linked to blockages in the blood vessels that supply the lungs. Pulmonary hypertension occurs in about one-third of adults with SCD and can lead to heart failure. Other serious consequences are strokes, cognitive impairment, disappearance of the spleen, ulcers of the lower extremities, impaired vision and hearing, and priapism. Blockage of the blood vessels supplying the spleen may lead to failure of that organ, which results in serious infectious conditions such as bone infection, inflammation of the gall bladder, pneumonia and urinary tract infection. Infections in sickle cell disease may also be linked to effects of the disease on other components of the immune system, such as white blood cells. As a result of the fatty acid imbalance, VOCs and organ damage, sickle cell patients are often in a near-continuous state of inflammation. They have elevated states of certain proteins that are markers of inflammation. Sickle cell patients often have near-continuous obstructive blood clotting activity inside the blood vessels, low level most of the time, but spiking during VOCs. Ultimately, SCA causes multi-organ dysfunction and early death. Many succumb to complications of chronic organ dysfunction and eventual organ failure.