sickle cell disease (SCD)

Inherited blood disorder

Abnormal hemoglobin (Hemoglobin S)

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

Block blood flow and cause ischemia

Chronic anemia

Pain crises (vaso-occlusive episodes)

Fatigue

Swelling in hands and feet (dactylitis)

Frequent infections

Delayed growth and development

Newborn screening (heel-prick test)

Hemoglobin electrophoresis

Complete blood count (CBC)

Genetic testing

Monitor for pain and administer medications

Assess for signs of infection

Educate patient on avoiding triggers

Provide support during pain crises

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

Educate on signs of complications

Lifelong condition with variable severity

Early detection and proper management improve outcomes

Regular medical care can prevent or manage complications

Genetic mutation (HBB gene)

Autosomal recessive inheritance

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

Carrier state: Sickle Cell Trait (one gene)

Acute chest syndrome

Stroke

Organ damage (especially spleen, liver, kidneys)

Pulmonary hypertension

Increased risk of infection

Eye problems (retinopathy)

Leg ulcers

Pain management (opioids, NSAIDs)

Blood transfusions

Hydroxyurea (reduces crises)

Bone marrow transplant (potential cure)

Hydration

Folic acid supplements

Oxygen therapy during acute crises

Importance of regular follow-up care

Hydration and proper nutrition

Infection prevention (vaccinations, hand hygiene)

Recognizing early signs of crises and complications

Avoiding high altitudes and extreme temperatures

Genetic counseling