Sickle Cell Presentation

What assessments may indicate hematologic or immunologic dysfunction in a child?

• Complete blood cell count

• History and physical exam

• Parent comments about child’s lack of energy

• Food diary showing poor iron sources

• Frequent infections

• Bleeding

What is the most common hematologic disorder of childhood?

Anemia

• Decrease in number of RBCs, hemoglobin concentration, or both

• Leads to decreased oxygen-carrying capacity of blood

What is sickle cell anemia?

• Group of diseases termed hemoglobinopathies

• Partial or complete replacement of normal hemoglobin (Hgb A) with abnormal hemoglobin (Hgb S)

• Types include:

  • Sickle cell anemia

  • Sickle cell–C disease

  • Sickle cell–hemoglobin E disease

  • Sickle cell ischemia disease

What is the inheritance pattern of sickle cell anemia?

Autosomal recessive

• Both parents carry the gene mutation

• Offspring probabilities:

  • 25% chance unaffected

  • 50% chance carrier (sickle trait)

  • 25% chance affected (disease)

What populations are most affected by sickle cell disease?

• 90,000 Americans affected

• Primarily affects African-Americans

• Also affects Hispanic populations (lower incidence in other groups)

• 9% of African-Americans are carriers (trait)

• 40% of native Africans are carriers

Why does sickle cell trait provide a survival advantage in some regions?

In areas where malaria is common, sickle cell trait provides resistance to malaria, giving carriers a survival advantage.

What are the primary clinical features of sickle cell anemia pathophysiology?

• Obstruction from sickled RBCs

• Vascular inflammation

• Increased RBC destruction

• Abnormal adhesion and entanglement of cells

• Local hypoxia

• Cellular death

What complications can result from sickle cell anemia?

• Stroke (CVA), paralysis

• Retinopathy, blindness, hemorrhage

• Avascular necrosis (shoulder, hip)

• Hepatomegaly, gallstones, obstructive jaundice

• Splenomegaly, sequestration, autosplenectomy

• Hematuria, hyposthenuria (dilute urine)

• Abdominal pain

• Dactylitis (hand-foot syndrome)

• Priapism (males)

• Pain, osteomyelitis

• Chronic leg ulcers (rare in children)

What are the four types of sickle cell crises?

• Vaso-occlusive crisis

• Sequestration crisis

• Hyperhemolytic crisis

• Aplastic crisis

Sickle Cell Crises: Vaso-occlusive crisis

• Blood stasis, ischemia, infarction

• Severe pain (joints, abdomen, chest), swelling (hands/feet), stroke, acute chest syndrome

Sickle Cell Crises: Sequestration crisis

• Pooling/clumping of blood in spleen (hypersplenism)

• Profound anemia, hypovolemia, shock

• May require splenectomy → ↑ infection risk

Sickle Cell Crises: Hyperhemolytic crisis

• Accelerated RBC destruction → anemia, jaundice, liver failure, hematuria, ↑ reticulocytes

• May need transfusions

Sickle Cell Crises: Aplastic crisis

• ↓ RBC production + ↑ destruction

• Triggered by viral infection or folic acid depletion

• Average RBC life span only 10–20 days (vs normal 120 days)

• Folic acid supplementation

What are key nursing management priorities during a sickle cell crisis?

• Pain control

• Oxygen therapy

• IV fluids (to improve circulation and prevent clumping)

• Antibiotics if infection present

• Blood transfusions as needed

• Folic acid supplementation (especially with aplastic crisis)

How is sickle cell disease (SCD) diagnosed in the U.S.?

• Universal newborn screening

• Early diagnosis prevents complications and allows for early management

What are the main goals of management for sickle cell disease?

• Prevent sickling

• Rest and minimize energy expenditure

• Hydration (oral or IV)

• Electrolyte replacement

• Analgesia for pain

• Blood replacement for anemia

• Antibiotics for infection

What history should be collected during a sickle cell assessment?

• Previous crises

• Causative factors, severity, usual management

• Activities in last 24 hrs (hydration, fever, exposures)

• Behavioral changes

What should be included in the clinical assessment for sickle cell disease?

• Pain (joints, extremities, abdomen)

• SOB & fatigue

• Murmurs (S3)

• Priapism (males)

• Skin color changes (jaundice, cyanosis, pallor)

• Abnormal abdominal exam (hepatomegaly, splenomegaly)

• CNS changes (LOC, seizures, fever)

What is the prognosis of sickle cell anemia?

• No cure (possible bone marrow transplant under study)

• Supportive care/prevention of sickling episodes

• Frequent bacterial infections → immunocompromise

• Bacterial infection = leading cause of death in young children with SCD

• Usual life span: into 5th decade (50s)

What nursing interventions are important in SCD care?

• Seek early intervention for problems

• Give prophylactic penicillin as ordered

• Recognize signs of stroke & respiratory problems

• Treat child normally (promote normal growth & development)

• Prevent sickling

What factors can precipitate a sickle cell crisis?

• Increased oxygen need / decreased oxygen transport

• Trauma

• Infection, fever

• Physical or emotional stress

• Dehydration (↑ blood viscosity)

• Hypoxia

What is the therapeutic management of a sickle cell crisis?

• Rest (reduce energy expenditure)

• Hydration (oral/IV therapy)

• Electrolyte replacement

• Analgesia for pain (often under-medicated, IV opioids if needed)

• Blood replacement for anemia

• Antibiotics for infection

What additional interventions may be used in SCD crisis management?

• Prophylactic antibiotics (2 months–5 years)

• Monitor reticulocyte count (bone marrow function)

• Early blood transfusion to reduce ischemia

What key teaching should be provided to patients/families with SCD?

• Recognize early signs/symptoms of crises

• Maintain hydration (avoid dehydration, especially in heat)

• Keep immunizations up to date (pneumococcal, meningococcal, influenza, COVID)

• Be aware of hereditary aspects (genetic counseling if planning children)

• Splenectomy may be needed with recurrent sequestration crises

What are general management considerations for SCD?

• Avoid constrictive clothing

• Keep extremities extended (avoid knee-chest position)

• Elevate HOB >30°

• Maintain moderate room temp (~72°F)

• Avoid blood pressure cuffs on affected extremities

• Monitor circulation in extremities hourly

• Continuous O2 sat monitoring

What medications are commonly used in the pharmacologic management of SCD?

• Hydroxyurea: ↑ fetal Hgb (Hgb F), ↓ crises, ↓ acute chest syndrome, ↓ need for transfusions/hospitalizations; requires monitoring, teratogenic, avoid in pregnancy, avoid missed doses

• NSAIDs: first-line for mild/moderate pain (ibuprofen; avoid if kidney disease → use acetaminophen instead)

• Opioids: for severe pain during crisis (IV morphine preferred; avoid Demerol due to seizure risk)

What drug should you avoid in sickle cell crisis?**

AVOID DEMEROL (due to seizure risk)

A child with sickle cell disease is admitted in vaso-occlusive crisis. Which nursing intervention is the priority?
A. Apply cold compresses to affected joints
B. Encourage increased oral fluids
C. Limit activity and provide rest
D. Administer opioid analgesics as prescribed

D. Administer opioid analgesics as prescribed

• Rationale: Pain is the hallmark of vaso-occlusive crisis and must be managed promptly. Cold compresses worsen vasoconstriction (use warmth instead). Fluids and rest are important but not the immediate priority.

The nurse teaches parents of a child with sickle cell disease about preventing sickle cell crises. Which statement indicates the need for further teaching?
A. “We will avoid letting our child get dehydrated.”
B. “We should avoid very strenuous physical activity.”
C. “We will give our child aspirin for pain.”
D. “We will call the doctor if our child has a fever.”

C. We will give our child aspirin for pain.

• Rationale: Aspirin is avoided in children due to the risk of Reye’s syndrome. Acetaminophen, NSAIDs, or opioids are preferred.

A nurse is reviewing lab results of a child with sickle cell anemia. Which finding is expected?
A. Decreased reticulocyte count
B. Elevated hemoglobin and hematocrit
C. Increased bilirubin level
D. Decreased WBC count

C. Increased bilirubin level

• Rationale: Chronic hemolysis releases bilirubin → jaundice. Reticulocyte count is increased, not decreased. Hemoglobin is low. WBC count may be elevated if infection is present.

Which of the following are common complications of sickle cell anemia? (Select all that apply)

• A. Stroke

• B. Splenic sequestration

• C. Acute chest syndrome

• D. Hyperglycemia

• E. Priapism

A, B, C, E

• Rationale: Stroke, sequestration crisis, acute chest syndrome, and priapism are complications. Hyperglycemia is not related.

The nurse is planning care for a 6-year-old child with sickle cell disease. Which intervention should be included?
A. Restrict fluids to reduce cardiac workload
B. Administer prophylactic penicillin as ordered
C. Limit vaccines due to immune compromise
D. Encourage high-intensity exercise

B. Administer prophylactic penicillin as ordered

• Rationale: Penicillin is prescribed to prevent infection. Fluids should be encouraged, vaccines are critical, and high-intensity exercise should be avoided.

Which dietary teaching is most appropriate for a client with sickle cell disease?
A. “Increase foods high in folic acid.”
B. “Avoid foods high in iron.”
C. “Increase calcium-rich foods.”
D. “Limit protein intake.”

A. Increase foods high in folic acid.

• Rationale: Folic acid supports RBC production. Iron intake is not typically restricted unless iron overload occurs from transfusions.

Which nursing assessments are critical during a sickle cell crisis? (Select all that apply)

• A. Pain level and location

• B. Lung sounds and oxygen saturation

• C. Urine output

• D. Pupillary reaction to light

• E. Peripheral circulation (cap refill, pulses)

A, B, C, E

• Rationale: Pain, O2 status, urine output (renal perfusion), and circulation are priority assessments. Pupillary reaction is not specifically relevant unless CNS involvement suspected.

A client with sickle cell disease is prescribed hydroxyurea. Which statement by the client indicates a need for further teaching?
A. “This medicine helps increase my fetal hemoglobin.”
B. “I need regular blood tests while taking this medication.”
C. “I should use birth control while on this medication.”
D. “This medicine will cure my sickle cell disease.”

D. This medicine will cure my sickle cell disease.

• Rationale: Hydroxyurea reduces crises and improves outcomes but does not cure sickle cell disease.