Mediterranean, Middle Eastern, Caribbean,

Mediterranean, Middle Eastern, Caribbean,

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Post 1

Etiology

Sickle cell anemia (SCA) is an inherited genetic abnormality of the oxygen-carrying protein found in red blood cells called hemoglobin S and is characterized by crescent-shaped (sickle) red blood cells and chronic anemia caused by excessive destruction of the abnormal red blood cells. Sickle cell anemia and the term sickle cell disease (SCD) are often used interchangeably; however, SCD is an umbrella term while SCA is one of 3 main genotypes (Foster & Ellis, 2018).  SCA is usually diagnosed within the first 2 years of life.  Mennella and Parks-Chapman (2018) state that SCA and sickle cell trait occur primarily in persons who are of African desceAsian Indian ancestry can have SCA. nt, persons with Mediterranean, Middle Eastern, Caribbean, and

Pathophysiology

In SCA, the red blood cells contain an abnormal form of hemoglobin called hemoglobin S.  When red blood cells contain a large amount of hemoglobin S, these red blood cells can become deformed into a “sickle” shape and are less flexible and become more prevalent when individuals have infections or decreased levels of oxygen in the blood.  The sickle cells are brittle and break apart easily.  Because the sickle cells are stiff, they have difficulty traveling through the smallest blood vessels which are called the capillaries, thus blocking blood flow and reducing oxygen supply to tissues in areas where these tiny vessels are blocked.  This blockage of blood flow can cause pain and, over time, cause damage organs such as the spleen, kidneys, brain, bones, and other organs as well as causing renal failure and heart failure.

Hallmark Signs

Although there is no hallmark sign for an individual with SCA, the symptoms they tend to experience are generally fatigue or feeling weak.  Some individuals can even have jaundiced eyes.  The hallmark symptoms for an individual undergoing a sickle cell crisis include severe bone pain, chest pain, fever, and shortness of breath.  Some common lab results would reveal a low hemoglobin and an elevated white blood cell count, platelet count, and reticulocyte count.

Complications