Hallmark Signs
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
Potential complications of SCA include ischemic cerebrovascular accident, vaso-occlusion of the pulmonary vasculature, pneumonia, splenic sequestration crisis, severe infection resulting from impaired splenic function, renal dysfunction, chest pain syndrome, enlarged heart, osteomyelitis, retinopathy, blindness, transfusion-related illness due to low hemoglobin, gallstones, chronic pain, enlarged liver, bloodstream infection and aplastic crisis.
Patient Teaching
Because there is no cure for SCA, supportive treatment is the only therapy available. I would educate the patient to refrain from flying at high altitudes, prevent infection such as proper handwashing techniques and receiving necessary vaccinations, maintain hydration, and avoid extreme temperature fluctuations as these actions can help prevent a sickle cell crisis. I would stress to the patient to adhere to his or her prescribed medications as indicated. In terms of diet, I would advise the individual with SCA to consume a diet high in calories with special attention to protein, minerals such as zinc, copper, folate, and vitamins A, C, E all while spaced out into smaller, frequent meals.
Post 2
Sickle cell disease is a group of disorders of which sickle cell anemia is the most severe. Sickle cell anemia is an autosomal recessive disorder that is caused due to a genetic mutation where the amino acid valine replaces glutamic acid to cause an abnormal form of hemoglobin within the red blood cells. “The resulting substitution of the hydrophilic amino acid glutamic acid at the sixth position by the hydrophobic amino acid valine leads to the production of hemoglobin S (HbS)” (Wun & Brunson, 2016, p. 640). When deoxygenation and dehydration occur, the red blood cell changes its shape into an elongated crescent (sickle) shape which can lead to a variety of consequences (McCance et al., 2013). Vascular occlusion, organ infarction, and pain are among the complications caused by this disorder. According to McCance et al. (2013) “sickling is an occasional, intermittent phenomenon” (p. 1065) and can be triggered by hypoxemia, a decreased pH, an increase in plasma osmolality, a decrease in plasma volume, or a low temperature. If the sickled red blood cells are not reoxygenated and rehydrated appropriately, irreversible plasma membrane damage may occur which leads to irreversible sickling.
