Sickle Cell Crisis

Sickle cell disease (SCD) is a group of inherited red blood cell disorders affecting about 1 in 500 African American children and 1 in 36,000 Hispanic American children. SCD results in anemia and a "sickle cell crisis" (SCC). The main clinical feature of sickle cell disease is the "acute painful crisis," which often requires hospitalization. The term "sickle cell crisis" is used to describe several acute conditions such as the vaso-occlusive crisis (acute painful crisis), aplastic crisis, splenic sequestration crisis, hyperhemolytic crisis, hepatic crisis, dactylitis, and acute chest syndrome. Other acute complications include pneumonia, meningitis, sepsis and osteomyelitis, stroke, avascular necrosis, priapism, and venous thromboembolism.

SCC is primarily characterized by severe acute pain and impaired end-organ function. Symptoms of SCC depend on the areas involved. For instance, a vaso-occlusive crisis, which most patients with SCD experience by the age of 6 years, can cause severe pain and swelling of both hands and feet (ie, dactylitis), primarily in young children. Pain can begin from any part of the body but frequently affects the extremities, back, and chest areas. SCD is diagnosed when laboratory evaluation identifies the presence of abnormal hemoglobin S (HbS). Genetic testing is also used to confirm the diagnosis. In a patient with SCC, evaluation is primarily performed to determine the severity of the acute disease flare. Treatment and management of SCD primarily consists of managing symptoms and associated complications.

About a third of sickle cell disease-related deaths occur within the home setting or 24 hours of hospitalization.[5]] Generally, adults are most likely to die from chronic complications, while children die acutely. The foremost causes of death in sickle cell disease are vaso-occlusive crises, cardiopulmonary threats, infections, and renal issues. Hypoxemia only compounds these problems.