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Quizzes > Health & Medicine > Diseases & Conditions

Take the Sickle Cell Anemia NCLEX Practice Quiz Now!

Ready to boost your NCLEX exam prep? Tackle these sickle cell anemia nursing questions!

Difficulty: Moderate
2-5mins
Learning OutcomesCheat Sheet
Paper art illustration for NCLEX sickle cell anemia quiz showing Challenge free quiz ten timed questions on teal background

Hey future RNs, grab your stethoscopes and sharpen your pencils! Think you know "a client with sickle cell anemia has a" from pathophysiology to crisis management? Compete against the clock and fine-tune your clinical decision-making under pressure. Our free Quick NCLEX Practice Quiz on sickle cell anemia is your chance to level up with 10 questions in a timed NCLEX quiz designed for real exam pressure. Test your nursing diagnosis sickle cell skills, from identifying triggers of vaso-occlusive crises to prioritizing pain relief strategies. Enhance your NCLEX exam prep by blending this sickle cell anemia nursing quiz with our curated sickle cell disease nclex questions and next-level hematology nclex questions . Ready, set, ace it - start now to boost your confidence!

What genetic mutation causes sickle cell anemia?
A substitution of valine for glutamic acid in the beta-globin chain
A deletion of the alpha-globin gene
A substitution of lysine for glutamic acid in the beta-globin chain
An insertion mutation in the delta-globin gene
Sickle cell anemia is caused by a point mutation in the HBB gene resulting in substitution of valine for glutamic acid at position six of the beta-globin chain. This single amino acid change leads to hemoglobin polymerization under deoxygenated conditions. The polymerized hemoglobin distorts RBCs into a sickle shape, causing hemolysis and vaso-occlusion. NCBI: Sickle Cell Disease
Which inheritance pattern does sickle cell anemia follow?
Autosomal recessive
Autosomal dominant
X-linked recessive
Mitochondrial
Sickle cell anemia follows an autosomal recessive inheritance pattern, meaning an individual must inherit two mutated HBB alleles to express the disease. Carriers with only one mutated allele (sickle cell trait) are usually asymptomatic. Offspring of two carrier parents have a 25% chance of disease, 50% chance of trait, and 25% chance of normal hemoglobin. CDC: Sickle Cell Data & Statistics
Which variant of hemoglobin polymerizes under low-oxygen conditions in sickle cell disease?
Hemoglobin S
Hemoglobin A
Hemoglobin F
Hemoglobin C
Hemoglobin S results from the mutated beta-globin chain and polymerizes when deoxygenated, causing red blood cells to sickle. Hemoglobin A is normal adult hemoglobin and does not sickle. Fetal hemoglobin (HbF) inhibits sickling, which is why hydroxyurea therapy aims to increase HbF levels. Hemoglobin C is a different variant that can cause mild hemolytic anemia but does not polymerize like HbS. NCBI: Hemoglobinopathies
What is the characteristic finding on a peripheral blood smear in sickle cell anemia?
Sickle-shaped red blood cells
Howell-Jolly bodies
Heinz bodies
Target cells
The hallmark on smear is sickle-shaped red cells due to polymerization of HbS in deoxygenated states. Howell-Jolly bodies indicate splenic dysfunction and may be seen later but are not specific. Heinz bodies form in G6PD deficiency. Target cells are seen in liver disease and thalassemia. Life in the Fast Lane: Sickle Cell Anaemia
Which screening test is commonly used to detect sickle cell trait or disease in newborns?
Hemoglobin electrophoresis
Coombs direct antiglobulin test
Serum haptoglobin
G6PD enzyme assay
Hemoglobin electrophoresis separates hemoglobin variants by charge, identifying HbS in both trait and disease. The Coombs test detects immune-mediated hemolysis. Serum haptoglobin levels decrease with hemolysis but do not identify variants. G6PD assay screens for enzyme deficiency anemia. CDC: Newborn Screening for Sickle Cell
Which protective effect is conferred by sickle cell trait (heterozygous HbAS)?
Resistance to severe malaria
Resistance to tuberculosis
Resistance to HIV infection
Resistance to typhoid fever
Individuals with sickle cell trait have reduced risk of severe Plasmodium falciparum malaria, likely due to enhanced parasite clearance in early RBC sickling. There is no evidence of protection against tuberculosis, HIV, or typhoid. This selective advantage explains the high allele frequency in malaria-endemic regions. NCBI: Sickle Cell Trait and Malaria
Which of the following commonly triggers a vaso-occlusive pain crisis in sickle cell patients?
Dehydration
Hyperhydration
Hypothermia
High altitude without dehydration
Dehydration increases blood viscosity and promotes red cell sickling, triggering vaso-occlusion and pain crises. Hyperhydration reduces viscosity. Hypothermia can also trigger some sickling but is less common than dehydration. High altitude may precipitate crisis mainly if dehydration occurs. NCBI: Sickle Cell Vaso-Occlusive Crisis
Which organ undergoes functional asplenia early in sickle cell disease?
Spleen
Liver
Kidney
Pancreas
Repeated splenic infarctions from sickled cells cause functional asplenia by early childhood, increasing infection risk from encapsulated organisms. The liver, kidney, and pancreas can be affected by other complications but do not undergo early functional failure like the spleen. UpToDate: Splenic Sequelae
What is the first-line therapy for managing acute vaso-occlusive pain crisis?
Aggressive IV hydration and opioid analgesics
High-dose corticosteroids
NSAIDs alone
Exchange transfusion immediately
Management of vaso-occlusive crises centers on hydration to reduce blood viscosity and opioids for pain control. NSAIDs may be added but are insufficient alone for severe pain. Corticosteroids are not standard due to rebound pain risk. Exchange transfusion is reserved for severe complications, not routine pain episodes. NCBI: Sickle Cell Crisis Management
How does hydroxyurea benefit patients with sickle cell disease?
Increases fetal hemoglobin production
Enhances iron absorption
Blocks beta-globin synthesis
Stimulates white blood cell production
Hydroxyurea increases HbF levels, which inhibits HbS polymerization and reduces sickling events. It does not enhance iron absorption or block beta-globin synthesis. Although it can cause mild leukocytosis initially, its primary therapeutic effect is on HbF induction. NCBI: Hydroxyurea in Sickle Cell Disease
Which finding is most consistent with acute chest syndrome in sickle cell disease?
New pulmonary infiltrate on chest X-ray with fever
Elevated liver enzymes and jaundice
Isolated splenic enlargement
Increased creatinine and oliguria
Acute chest syndrome presents with fever, respiratory symptoms, and a new pulmonary infiltrate on imaging. It may mimic pneumonia and requires antibiotics, oxygen, and often exchange transfusion. Liver enzyme elevation and jaundice suggest hepatic sequestration. Splenic enlargement indicates splenic sequestration crisis. Renal issues present differently. NCBI: Acute Chest Syndrome
What is the typical presentation of splenic sequestration crisis?
Sudden splenomegaly and acute drop in hemoglobin
Chronic pain in the left upper quadrant
Iron overload due to repeated transfusions
Elevated reticulocyte count without splenomegaly
Splenic sequestration crisis presents with rapid enlargement of the spleen and acute hemoglobin drop due to pooling of sickled cells. It is an emergency requiring transfusion. Chronic LUQ pain is not typical of sequestration crisis. Iron overload is a complication of frequent transfusions but not the acute presentation. Elevated reticulocytes occur in hemolysis but without splenomegaly it’s not sequestration. CDC: Splenic Sequestration
Aplastic crisis in sickle cell disease is most often triggered by which infectious agent?
Parvovirus B19
Epstein-Barr virus
Cytomegalovirus
Influenza A
Parvovirus B19 infects erythroid precursors, halting red cell production and causing aplastic crisis in patients dependent on high erythropoiesis. EBV, CMV, and influenza may cause anemia but do not specifically trigger aplastic crisis the way parvovirus does. NCBI: Aplastic Crisis
What is dactylitis in infants with sickle cell disease?
Painful swelling of hands or feet due to bone infarction
Joint subluxation secondary to ligament laxity
Osteomyelitis of the metacarpals
Serous effusion in finger joints
Dactylitis is due to small bone infarctions in the hands or feet, causing painful swelling in infants with sickle cell disease. It is not caused by ligament laxity, osteomyelitis, or joint effusions. This is typically one of the first signs of sickling in young children. UpToDate: Clinical Manifestations
Which prophylactic measure reduces stroke risk in children with sickle cell disease?
Regular blood transfusions based on transcranial Doppler velocity
Daily aspirin therapy
Supplemental vitamin D
High-dose intravenous immunoglobulin
Chronic transfusion therapy guided by transcranial Doppler ultrasound velocities reduces stroke risk by lowering HbS concentration. Aspirin is not proven for stroke prevention in sickle cell. Vitamin D supports bone health but does not prevent stroke. IVIG is not used for stroke prophylaxis. NCBI: Stroke Prevention
Why is folic acid supplementation recommended for sickle cell patients?
To support increased erythropoiesis from chronic hemolysis
To prevent iron overload
To enhance hemoglobin S polymerization
To reduce pain crises
Chronic hemolysis drives increased red cell turnover and erythropoiesis, depleting folate stores. Supplementation prevents megaloblastic anemia in these patients. It does not affect iron overload, HbS polymerization, or directly reduce pain crises. NCBI: Folic Acid in Hemolytic Anemias
What is the primary treatment for iron overload in chronically transfused sickle cell patients?
Deferoxamine chelation therapy
Phlebotomy
High-dose vitamin C
Erythropoietin injections
Deferoxamine binds excess iron for urinary and fecal excretion, preventing organ damage from iron overload due to repeated transfusions. Phlebotomy is not feasible in anemic patients. Vitamin C may increase iron absorption. Erythropoietin does not remove excess iron. NCBI: Iron Chelation Therapy
In a patient with priapism secondary to sickle cell disease, what is the initial management step?
Intracavernosal phenylephrine injection
Oral sildenafil
High-dose NSAIDs
Warm sitz baths only
Intracavernosal alpha-agonist injection such as phenylephrine is first-line to induce detumescence. Oral sildenafil is contraindicated as it can worsen priapism. NSAIDs and warm baths provide symptomatic relief but are insufficient for acute episodes. Prompt intervention prevents fibrosis and erectile dysfunction. NCBI: Priapism Management
Which definitive therapy can cure sickle cell disease but is limited by donor availability and risks?
Allogeneic hematopoietic stem cell transplant
Chronic exchange transfusions
Hydroxyurea dose escalation
Splenectomy
Allogeneic stem cell transplantation can be curative by replacing defective hematopoietic cells, but donor matching and graft-versus-host risks limit use. Chronic transfusions manage disease but are not curative. Hydroxyurea modifies disease severity, and splenectomy treats sequestration but does not cure. NCBI: Stem Cell Transplant
What complication arises from chronic hemolysis in sickle cell disease leading to pulmonary hypertension?
Release of free hemoglobin scavenging nitric oxide
Excess carbon monoxide production
Direct sickling of pulmonary capillaries
Immune complex deposition in pulmonary arteries
Free hemoglobin from hemolysis binds nitric oxide, reducing vasodilation and causing pulmonary vascular remodeling and hypertension. CO production is not a primary mechanism. Sickling does occur but the NO scavenging is key to pulmonary hypertension. Immune complexes are not implicated. NCBI: Pulmonary Hypertension in SCD
What is the recurrence risk of sickle cell disease in a couple where both parents have sickle cell trait?
25%
50%
75%
100%
When both parents carry one HbS allele, each pregnancy has a 25% chance of an affected child (HbSS), 50% chance of trait (HbAS), and 25% chance of normal HbAA. Genetic counseling uses these probabilities for family planning. CDC: Inheritance of Sickle Cell
Which newly approved therapy reduces oxidative damage in red blood cells of sickle cell patients?
L-glutamine
Voxelotor
Crizanlizumab
Hydroxyurea
L-glutamine is thought to reduce oxidative stress in sickled RBCs, decreasing vaso-occlusive events. Voxelotor increases hemoglobin oxygen affinity, and crizanlizumab targets P-selectin to reduce adhesion. Hydroxyurea increases HbF. NCBI: L-glutamine Therapy
What molecular event initiates red blood cell sickling at the microvascular level?
Polymerization of deoxygenated HbS forming long fibers
Oxidative denaturation of the red cell membrane
Activation of complement on the red cell surface
Calcium influx mediated by NMDA receptors
Deoxygenated HbS molecules stick together, forming rigid polymers that distort red cells into the sickle shape and impede microvascular flow. Oxidative damage and complement activation can occur but are not the primary sickling mechanism. NMDA-mediated calcium influx is not involved. NCBI: Sickle Cell Pathophysiology
Which pattern on hemoglobin electrophoresis distinguishes sickle cell trait from sickle cell disease?
Trait: HbA and HbS bands; Disease: mostly HbS with no HbA
Trait: only HbS band; Disease: HbA and HbS bands
Trait: HbF only; Disease: HbA only
Trait: HbA2 elevated; Disease: HbA2 decreased
In trait (HbAS), electrophoresis shows both HbA and HbS. In disease (HbSS), HbS predominates with little or no HbA and increased HbF. HbA2 is modestly increased in beta-thalassemia but not diagnostic here. NCBI: Hemoglobin Electrophoresis
CRISPR-based gene therapy for sickle cell disease typically targets which gene to induce a curative effect?
BCL11A enhancer to increase fetal hemoglobin expression
HBA1 promoter to silence alpha-globin
EPOR gene to enhance erythropoietin signaling
G6PD gene to improve red cell stability
CRISPR strategies often disrupt the BCL11A erythroid-specific enhancer, derepressing gamma-globin genes to boost HbF and ameliorate sickling. Targeting HBA1, EPOR, or G6PD is not part of current curative approaches for sickle cell. NEJM: CRISPR in Sickle Cell
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Study Outcomes

  1. Analyze crisis presentation -

    Analyze how a client with sickle cell anemia has a crisis presentation by identifying key assessment findings and prioritizing critical nursing interventions.

  2. Apply nursing diagnosis principles -

    Apply nursing diagnosis sickle cell guidelines to develop comprehensive, patient-centered care plans during the sickle cell anemia nursing quiz scenarios.

  3. Interpret clinical and laboratory data -

    Interpret clinical signs and laboratory values to recognize vaso-occlusive crises early and implement measures to prevent further complications.

  4. Formulate pain management strategies -

    Formulate evidence-based interventions to manage acute pain and improve patient comfort during sickle cell anemia episodes.

  5. Utilize time-management techniques -

    Utilize time-management strategies and test-taking tips during the timed NCLEX quiz to enhance answer accuracy under pressure.

  6. Evaluate quiz performance -

    Evaluate NCLEX practice questions results to identify knowledge gaps, reinforce learning, and strengthen your NCLEX exam prep.

Cheat Sheet

  1. Understanding Pathophysiology -

    In a client with sickle cell anemia crisis, deoxygenated hemoglobin S polymerizes and distorts red cells, causing vaso-occlusion and ischemia. Use the mnemonic "S.O.S." (Sickling, O2 deficit, Stasis) to recall triggers of a crisis (NIH). This foundation is tested often in NCLEX practice questions related to SCD pathogenesis.

  2. Pain Management Interventions -

    Acute pain is the hallmark of a vaso-occlusive crisis, so initiate PCA opioids for consistent analgesia (American Pain Society) and combine with NSAIDs and relaxation techniques. Warm compresses promote vasodilation and pain relief. These strategies frequently appear in a sickle cell anemia nursing quiz scenario.

  3. Hydration and Oxygenation -

    A client with sickle cell anemia has a high risk of increased blood viscosity, so maintaining IV hydration (1.5 - 2 L/day) reduces sickling (CDC). Provide supplemental oxygen to keep saturation above 95%, reversing polymerization of hemoglobin S. These interventions are key in timed NCLEX quiz questions on crisis management.

  4. Laboratory Monitoring Essentials -

    Monitor hemoglobin (6 - 8 g/dL during crisis), reticulocyte count (elevated in hemolysis), and peripheral smear for sickled cells (UpToDate). Check renal function and liver enzymes to detect organ damage early. Accurate lab interpretation is a common focus in NCLEX practice questions on a client with sickle cell anemia has multiple complications.

  5. Patient Education and Prevention -

    Teach patients nursing diagnosis sickle cell care: avoid temperature extremes, stay hydrated, and get daily folic acid for erythropoiesis (WHO). Stress importance of pneumococcal and influenza vaccines to reduce infection-triggered crises. Empowering self-management is a cornerstone of NCLEX exam prep and enhances long-term outcomes.

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