Sickle Cell Anemia Nursing Care and Management: Study Guide

Sickle cell anemia is a lifelong hemoglobin disorder, and most of what you do for these patients is managing pain, preventing crisis, and catching the complications early. Keep them warm, hydrated, and oxygenated, treat infection fast, and respect that the patient knows their own pain better than the scale does.

What Is Sickle Cell Anemia?

Sickle cell anemia is an inherited, severe hemolytic anemia caused by inheriting the sickle hemoglobin gene. The sickle hemoglobin (HbS) gene appears in people of African descent and, to a lesser extent, in people from the Middle East, the Mediterranean, and aboriginal tribes in India. It is the most severe form of sickle cell disease.

Pathophysiology

The HbS gene makes the hemoglobin molecule defective. Exposed to low oxygen tension, sickle hemoglobin takes on a crystal-like formation. Venous oxygen can drop low enough that the erythrocyte loses its round, pliable, biconcave shape. These long, rigid cells adhere to the endothelium of small vessels and to each other, cutting blood flow to a region or organ. If the cell meets adequate oxygen again before its membrane stiffens too far, it can revert to normal shape.

Causes

Cold aggravates sickling because vasoconstriction slows blood flow. Tissue hypoxia and necrosis trigger the sickle crisis. Infection with human parvovirus causes aplastic crisis. Sequestration crisis occurs when organs such as the spleen pool the sickled cells (splenic infarction).

Clinical Manifestations

Symptoms vary and only loosely track the amount of HbS. Anemia is always present, with hemoglobin usually 7 to 10 g/dL. Jaundice is characteristic and usually obvious in the sclerae. Dysrhythmias and heart failure may occur in adults. In childhood the marrow expands to offset anemia, sometimes enlarging the bones of the face and skull.

Complications

These patients are unusually susceptible to infection, especially pneumonia and osteomyelitis. Reduced oxygen from sickling can cause stroke. Reduced flow to the kidneys can drive renal failure. The heart compensates for poor distribution by pumping harder and may eventually fail.

Assessment and Diagnostic Findings

• CBC: Reticulocytosis (count may vary from 30% to 50%); leukocytosis (especially in vaso-occlusive crisis), with counts over 20,000 indicating infection; decreased Hb (5 to 10 g/dL) and total RBCs; elevated platelets; normal to elevated MCV.
• Stained RBC examination: Partially or completely sickled crescent-shaped cells; anisocytosis; poikilocytosis; polychromasia; target cells; Howell-Jolly bodies; basophilic stippling; occasional nucleated RBCs (normoblasts).
• Sickle-turbidity tube test (Sickledex): Routine screen for hemoglobin S (HbS); does not differentiate sickle cell anemia from trait.
• Hemoglobin electrophoresis: Identifies abnormal hemoglobin types and differentiates sickle cell trait from sickle cell anemia. Results may be inaccurate if the patient received a blood transfusion within 3 to 4 mo before testing.
• ESR: Elevated.
• Erythrocyte fragility: Decreased (osmotic or RBC fragility); RBC survival time decreased (accelerated breakdown).
• ABGs: May reflect decreased Po2 (defects in alveolar capillary gas exchange); acidosis (hypoxemia and acidic states in vaso-occlusive crisis).
• Serum bilirubin (total and indirect): Elevated (increased RBC hemolysis).
• Acid phosphatase (ACP): Elevated (release of erythrocytic ACP into serum).
• Alkaline phosphatase: Elevated during vaso-occlusive crisis (bone and liver damage).
• LDH: Elevated (RBC hemolysis).
• Serum potassium and uric acid: Elevated during vaso-occlusive crisis (RBC hemolysis).
• Serum iron: May be elevated or normal (increased absorption from excessive RBC destruction).
• Total iron-binding capacity (TIBC): Normal or decreased.
• Urine/fecal urobilinogen: Increased (more sensitive than serum levels for RBC destruction).
• Intravenous pyelogram (IVP): May be done to evaluate kidney damage.
• Bone radiographs: May show skeletal changes (osteoporosis, osteosclerosis, osteomyelitis, avascular necrosis).
• X-rays: May indicate bone thinning, osteoporosis.

Medical Management

Treatment remains an active research target. Peripheral blood stem cell transplant may cure sickle cell anemia but reaches only a small subset of patients because of donor incompatibility or because severe organ damage contraindicates it. Chronic RBC transfusion therapy can prevent or manage complications. Supportive therapy leans on adequate hydration during a painful sickling episode; aspirin helps mild to moderate pain, and NSAIDs help moderate pain or combine with opioid analgesics. Monitor pulmonary function, treat pulmonary hypertension early, and treat infections and acute chest syndrome promptly since both precipitate crisis. Use incentive spirometry to prevent pulmonary complications and bronchoscopy to identify the source of pulmonary disease.

Pharmacologic Therapy

Hydroxyurea increases fetal hemoglobin levels. Arginine has antisickling properties and boosts nitric oxide, the most potent vasodilator, lowering pulmonary artery pressure.

Nursing Management

Nursing Assessment

Ask the patient to identify what precipitated previous crises and what they do to prevent and manage them. Monitor pain levels on a pain intensity scale. Assess the quality and frequency of pain and what aggravates or relieves it. Because these patients are prone to infection, assess for any infectious process.

Nursing Diagnosis

• Acute pain related to tissue hypoxia from agglutination of sickled cells within blood vessels.
• Risk for infection.
• Risk for powerlessness related to illness-induced helplessness.
• Deficient knowledge regarding sickle cell crisis prevention.

Nursing Care Planning and Goals

Major goals are relief of pain, decreased incidence of crisis, enhanced self-esteem and sense of power, and absence of complications.

Nursing Interventions

Managing Pain

Use the patient's own description and pain rating to guide analgesics. Support and elevate any acutely swollen joint until swelling diminishes. Teach relaxation, breathing exercises, and distraction. Once the acute episode eases, push function aggressively (physical therapy, whirlpool baths, transcutaneous nerve stimulation).

Preventing and Managing Infection

Monitor for signs and symptoms of infection and start prescribed antibiotics promptly. Assess for dehydration. Teach the patient to take prescribed oral antibiotics at home if indicated and to finish the entire course.

Promoting Coping Skills

Manage pain well to build a therapeutic relationship on mutual trust. Focus on the patient's strengths rather than deficits. Give the patient decisions to make about daily care to increase their sense of control.

Increasing Knowledge

Teach what precipitates a crisis and how to prevent or blunt it (stay warm, maintain hydration, avoid stress). If hydroxyurea is prescribed for a woman of childbearing age, warn that the drug can cause congenital harm and counsel on pregnancy prevention.

Monitoring and Managing Potential Complications

For leg ulcers, protect the leg from trauma and contamination, use scrupulous aseptic technique against nosocomial infection, and refer to a wound-ostomy-continence nurse to aid healing and prevention. For priapism leading to impotence, teach the patient to empty the bladder at onset, exercise, and take a warm bath, and to seek care if an episode persists more than 3 hours. For chronic pain and substance abuse, stress adherence to the treatment plan, build trust through good acute-pain management during crisis, and steer the patient toward a single consistent provider rather than rotating emergency department staff. When a crisis arises, emergency department staff should contact the patient's primary provider for optimal management. Promote continuity of care and establish written contracts with the patient.

Promoting Home and Community Based Care

Involve the patient and family in teaching about the disease, treatment, assessment, and monitoring for complications, including vascular access device management and chelation therapy. Have providers, patients, and families communicate regularly. Give clear guidelines on when to seek urgent care. Provide followup for patients with vascular access devices as needed.

Evaluation

Expected outcomes are relief of pain, decreased incidence of crisis, enhanced self-esteem and power, and absence of complications.

Discharge and Home Care Guidelines

Outpatient or home care nurses may need to provide followup for patients with vascular access devices. Every provider serving these patients and families should communicate regularly.

Documentation Guidelines

Document the client's description of and response to pain, the acceptable level of pain, prior medication use, signs and symptoms of infectious process, the plan of care, the teaching plan, responses to interventions and teaching, attainment of or progress toward outcomes, and long-term needs.