Description:

Respond to the following: Sickle Cell Anemia with a Vaso-occlusive crisis (primary): is due to an abnormally sickle shaped hemoglobin that blocks the flow of blood that circulates through the tissues (Adams,2017). Hemoglobin S or Hb S is formed by a genetic mutation in which one amino acid (valine) replaces another (glutamic acid). Hb S reacts to deoxygenation and dehydration by solidifying and stretching the erythrocyte into an elongated sickle shape. Deoxygenation is probably the most important variable in determining the occurrence of sickling. Sickled erythrocytes are stiff and cannot change shape as easily as normal cells when they pass through the microcirculation. As a result, sickled erythrocytes tend to plug the blood vessels, causing vascular occlusion, pain, and organ infarction. The anemia that follows triggers erythropoiesis in the marrow and, in extreme cases, in the liver. Sickle cell anemia is a homozygous inherited autosomal recessive disorder. The gene is more common in families that come from Africa, India, Mediterranean countries, Saudi Arabia, the Caribbean islands, and South and Central America. In the United States, it most commonly affects blacks. When sickling occurs, the general manifestations includes pallor, fatigue, jaundice, and irritability and sometimes are accompanied by acute manifestations called crises. Vaso-occlusive crisis or thrombotic crisis begins with sickling in the microcirculation. As blood flow is obstructed by tangled masses of rigid, sickled cells, vasospasm occurs and a “log jam” effect brings all blood flow through the vessel to a halt. Vaso-occlusive crisis is extremely painful and may last for days or even weeks, with an average duration of 4 to 6 days. In older children and adults the large joints and surrounding tissue become painful and swollen. The pain you feel in a sickle cell crisis is often in one or a few areas such as your bones, legs, hips, lower back, belly, or chest. Some people have pain and throbbing over their whole body. Severe abdominal pains often are caused by infarction in abdominal structures. This diagnosis seems to fit with what is occurring with the patient.