NSG 430 Exam 4: (Latest 2026/2027 Update) Sickle Cell Disease and Crisis
| Q&A | Grade A | 100% Correct (Verified Answers) – Nursing Program
Subject: NSG 430 – Advanced Medical-Surgical / Hematology
Source: NSG 430 Exam 4 Blueprint 2026/2027 Format: Q&A Guide with Rationale | Verified Grade A
1. What is sickle cell disease (SCD)?
Correct Answer: An inherited blood disorder that causes a change in the primary structure of
hemoglobin, causing red blood cells to assume a sickle shape
1. SCD is an autosomal recessive disorder caused by a mutation in the β-globin gene (Glu→Val at position 6).
2. Abnormal hemoglobin S (HbS) polymerizes under low oxygen, distorting RBC shape.
3. This leads to hemolysis, vaso-occlusion, and chronic organ damage.
2. What organs are primarily affected in sickle cell disease?
Correct Answer: Lungs, kidneys, brain, retina, and bones
1. Vaso-occlusion affects any organ but lungs (acute chest syndrome), kidneys (renal failure), brain (stroke), retina
(blindness), and bones (avascular necrosis) most vulnerable.
2. Functional asplenia increases infection risk.
3. Chronic hemolysis leads to gallstones, leg ulcers, and priapism.
3. What does the sickle cell shape cause?
Correct Answer: Vascular occlusion leading to hemolysis
1. Sickled RBCs are rigid and stick to endothelium, causing vaso-occlusion.
2. Occlusion leads to ischemia, infarction, necrosis, and eventual shock.
3. Hemolysis releases free hemoglobin, causing nitric oxide scavenging, vasoconstriction, and pulmonary
hypertension.
4. What is a sickle cell crisis?
Correct Answer: A severe, painful, acute exacerbation of RBC sickling causing a vaso-occlusive crisis
1. Vaso-occlusive crisis is most common type; presents with severe bone pain (back, chest, extremities).
2. Other types: aplastic crisis (parvovirus B19), sequestration crisis (spleen/liver), hemolytic crisis (rapid drop in Hgb).
3. Triggers include infection, dehydration, hypoxia, cold, high altitude, stress.
5. What does sickle cell crisis lead to?
Correct Answer: Tissue ischemia, infarction, necrosis → shock
1. Ischemia results from occluded microvasculature; repeated episodes cause cumulative organ damage.
2. Infarction can occur in bone (avascular necrosis of femoral head), spleen (autosplenectomy), and lungs (acute chest
syndrome).
3. Shock may result from severe pain, dehydration, sepsis, or sequestration crisis.
6. Which populations are most affected by sickle cell disease?
Correct Answer: Africans, Sub-Saharan Africans, Mediterranean populations, and Hispanics
1. SCD is most prevalent in malaria-endemic regions because sickle cell trait provides malaria protection.
2. In the US, SCD affects approximately 1 in 365 African American births and 1 in 16,300 Hispanic births.
3. Carrier frequency: 1 in 13 African Americans has sickle cell trait.
| Q&A | Grade A | 100% Correct (Verified Answers) – Nursing Program
Subject: NSG 430 – Advanced Medical-Surgical / Hematology
Source: NSG 430 Exam 4 Blueprint 2026/2027 Format: Q&A Guide with Rationale | Verified Grade A
1. What is sickle cell disease (SCD)?
Correct Answer: An inherited blood disorder that causes a change in the primary structure of
hemoglobin, causing red blood cells to assume a sickle shape
1. SCD is an autosomal recessive disorder caused by a mutation in the β-globin gene (Glu→Val at position 6).
2. Abnormal hemoglobin S (HbS) polymerizes under low oxygen, distorting RBC shape.
3. This leads to hemolysis, vaso-occlusion, and chronic organ damage.
2. What organs are primarily affected in sickle cell disease?
Correct Answer: Lungs, kidneys, brain, retina, and bones
1. Vaso-occlusion affects any organ but lungs (acute chest syndrome), kidneys (renal failure), brain (stroke), retina
(blindness), and bones (avascular necrosis) most vulnerable.
2. Functional asplenia increases infection risk.
3. Chronic hemolysis leads to gallstones, leg ulcers, and priapism.
3. What does the sickle cell shape cause?
Correct Answer: Vascular occlusion leading to hemolysis
1. Sickled RBCs are rigid and stick to endothelium, causing vaso-occlusion.
2. Occlusion leads to ischemia, infarction, necrosis, and eventual shock.
3. Hemolysis releases free hemoglobin, causing nitric oxide scavenging, vasoconstriction, and pulmonary
hypertension.
4. What is a sickle cell crisis?
Correct Answer: A severe, painful, acute exacerbation of RBC sickling causing a vaso-occlusive crisis
1. Vaso-occlusive crisis is most common type; presents with severe bone pain (back, chest, extremities).
2. Other types: aplastic crisis (parvovirus B19), sequestration crisis (spleen/liver), hemolytic crisis (rapid drop in Hgb).
3. Triggers include infection, dehydration, hypoxia, cold, high altitude, stress.
5. What does sickle cell crisis lead to?
Correct Answer: Tissue ischemia, infarction, necrosis → shock
1. Ischemia results from occluded microvasculature; repeated episodes cause cumulative organ damage.
2. Infarction can occur in bone (avascular necrosis of femoral head), spleen (autosplenectomy), and lungs (acute chest
syndrome).
3. Shock may result from severe pain, dehydration, sepsis, or sequestration crisis.
6. Which populations are most affected by sickle cell disease?
Correct Answer: Africans, Sub-Saharan Africans, Mediterranean populations, and Hispanics
1. SCD is most prevalent in malaria-endemic regions because sickle cell trait provides malaria protection.
2. In the US, SCD affects approximately 1 in 365 African American births and 1 in 16,300 Hispanic births.
3. Carrier frequency: 1 in 13 African Americans has sickle cell trait.
