Luokat: Kaikki - symptoms - treatment - complications - management

jonka Aysha Abdulla Ahmed Jasim Mahmood 1 kuukausi sitten

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sickle cell disease (SCD)

Sickle cell disease (SCD) is an inherited blood disorder characterized by the production of abnormal hemoglobin known as Hemoglobin S. This abnormality leads red blood cells to become rigid, sticky, and sickle-shaped, causing blockages in blood flow and subsequently leading to ischemia.

sickle cell disease (SCD)

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