sickle cell disease (SCD)

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PRINCE2

Robin Leysen‑k egina

rugby

MOHD ALHAFIZ‑k egina

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sickle cell disease (SCD)

Patient Education

Genetic counseling

Avoiding high altitudes and extreme temperatures

Recognizing early signs of crises and complications

Infection prevention (vaccinations, hand hygiene)

Hydration and proper nutrition

Importance of regular follow-up care

Treatment & Management

Oxygen therapy during acute crises

Folic acid supplements

Hydration

Bone marrow transplant (potential cure)

Hydroxyurea (reduces crises)

Blood transfusions

Pain management (opioids, NSAIDs)

Complications

Leg ulcers

Eye problems (retinopathy)

Increased risk of infection

Pulmonary hypertension

Organ damage (especially spleen, liver, kidneys)

Stroke

Acute chest syndrome

Causes

Carrier state: Sickle Cell Trait (one gene)

Must inherit two sickle cell genes (one from each parent) to have the disease

Autosomal recessive inheritance

Genetic mutation (HBB gene)

Prognosis

Regular medical care can prevent or manage complications

Early detection and proper management improve outcomes

Lifelong condition with variable severity

Nursing Care

Educate on signs of complications

Monitor vital signs, oxygen saturation, and lab results (Hgb levels)

Provide support during pain crises

Educate patient on avoiding triggers

Assess for signs of infection

Monitor for pain and administer medications

Diagnosis

Genetic testing

Complete blood count (CBC)

Hemoglobin electrophoresis

Newborn screening (heel-prick test)

Symptoms

Delayed growth and development

Frequent infections

Swelling in hands and feet (dactylitis)

Fatigue

Pain crises (vaso-occlusive episodes)

Chronic anemia

Definition & Pathophysiology

Block blood flow and cause ischemia

Causes red blood cells to become rigid, sticky, and sickle-shaped

Abnormal hemoglobin (Hemoglobin S)

Inherited blood disorder