A nurse is caring for a patient with sickle cell disease (SCD)
The patient asks, “Why do my red blood cells become sickle-shaped?” Which response by the nurse is most accurate?
A “Your red blood cells become sickle-shaped due to a genetic mutation that produces abnormal hemoglobin S (HbS)”
B “Sickle-shaped red blood cells occur because of an autoimmune reaction in your body.”
C “The sickle shape of your red blood cells is caused by a viral infection.”
D “Your diet and lifestyle choices contribute to the sickle shape of your red blood cells.”
The correct answer and Explanation is :
The correct answer is:
A. “Your red blood cells become sickle-shaped due to a genetic mutation that produces abnormal hemoglobin S (HbS).”
Explanation:
Sickle cell disease (SCD) is a genetic disorder that affects the hemoglobin protein in red blood cells. Hemoglobin is responsible for carrying oxygen throughout the body. In individuals with SCD, there is a mutation in the gene that codes for hemoglobin, leading to the production of an abnormal form called hemoglobin S (HbS). When red blood cells are exposed to low oxygen levels, HbS molecules stick together and cause the red blood cells to become rigid and form a characteristic sickle (crescent) shape.
These sickle-shaped cells are less flexible and can get stuck in small blood vessels, blocking blood flow. This can lead to pain, tissue damage, and complications such as stroke or organ damage. Unlike normal round red blood cells, sickle cells have a shorter lifespan, leading to a constant shortage of red blood cells, also known as anemia.
Sickle cell disease is inherited in an autosomal recessive pattern, meaning that a person must inherit two copies of the abnormal gene (one from each parent) to have the disease. If only one abnormal gene is inherited, the person is considered a carrier and may have sickle cell trait but not the full-blown disease.
Did you know? Some people with sickle cell trait have a protective advantage against malaria, as the presence of the sickle cell trait can make it harder for the malaria parasite to survive in red blood cells!