microcirculation and obstructs
microcirculation and obstructs
Clinical manifestations of sickle cell anemia include the typical signs of hemolytic anemia – “pallor, fatigue, jaundice, and irritability” (McCance et al., 2013, p. 1066) and are sometimes accompanied by an acute manifestation or crises. There are four types of crises that can be triggered by extensive sickling: vaso-occlusive crisis, aplastic crisis, sequestration crisis, or hyperhemolytic crisis (McCance et al., 2013). Vaso-occlusive crisis is when sickling occurs in microcirculation and obstructs blood flow, causing vasospasm, and can lead to painful swelling of the hands and feet, priapism, severe abdominal pain, and even stroke (McCance et al., 2013). Aplastic crisis typically occurs as a result of a viral infection. Parvovirus B19 is a virus that almost always causes a person with sickle cell anemia to go into an aplastic crisis and causes the production of red blood cells to be shut down temporarily, which leads to a severe hemoglobin drop. Sequestration crisis occurs when blood becomes pooled in the liver/spleen and can lead to death if not treated with hydration and a blood transfusion. Treatment for recurrences of this crisis is the removal of the spleen after the age of 5. Hyperhemolytic crisis is characterized by red blood cell destruction at an accelerated rate, and symptoms include anemia, jaundice, and reticulocytosis.
Supportive care, which is aimed at the avoidance of these crises, is one of the key factors in the treatment and management of sickle cell disease. “Crises can be prevented by avoiding fever, infection, acidosis, dehydration, constricting clothes, and exposure to cold” (McCance et al., 2013, p. 1068). Additionally, it is recommended that all routine childhood immunizations, along with the pneumococcal and meningococcal vaccine series, and an annual influenza vaccine be given to all patients with a sickle cell disease.
For this patient, it is important to ensure that she understands the severity of her disease and how to avoid going into a sickle cell crisis, as well as the potentially life-threatening consequences if she’s not careful.
