Sickle Cell Disease

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Temple University *

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512

Subject

Nursing

Date

Nov 24, 2024

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docx

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4

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Sickle Cell Disease Hereditary, severe, chronic condition. Pathophysiology: Abnormal hemoglobin. Erythrocytes distorted. Increases fragility of erythrocytes. Causes cells to become sickled in shape and rigid. Anemia occurs. Periods of exacerbation called vasoocclusive crises. Indications: Systemic. Chronic anemia - hemoglobin, 6 to 9 g/dL (60 to 90 g/L). Possible growth retardation. Delayed sexual maturity. Pain - often especially focused on joints. Swelling. Jaundice. Priapism. Impaired kidney function. Cardiac murmurs. Altered pulmonary function. Increased susceptibility to infection. Interventions: Rest. Oxygen. IV fluids. Electrolytes. Sedation. Analgesia. Possible transfusion. Nursing care: Promote rest. Administer analgesia as prescribed. Teach use of patient-controlled analgesia (PCA) pump as indicated. Administer oxygen. Maintain I and O. Ensure adequate fluid intake. Monitor for infection. Encourage activity as tolerated when not in crisis. Vaso-occlusive crisis: Most common complication of sickle cell disease. Painful due to hypoxia and necrosis of tissues or organ. Occurs when sickled cells block blood flow to an organ or body part. Aplastic: Human parvovirus infection. Hemoglobin levels decrease faster than bone marrow can create reticulocytes to become red blood cells (RBCs). Sequestration:
Pooling of sickled cells in an organ - spleen and lungs in children. Most likely to occur with: High altitude. Sudden change in ambient temperature. Illness. Dehydration. Stress. Nursing considerations: Manage pain. Prevent infection. Encourage strengths. Client education: Take medication as ordered. Teach new coping skills. Discuss pregnancy with health care provider. Learn situations that lead to crises. Seek medical assistance if priapism occurs. Seek medical assistance for leg/ankle wounds that do not heal. Avoid alcohol or illegal drugs to manage pain. Nursing Focus & Concepts Sickle Cell Disease Definition: Severe hereditary form of anemia. A mutated form of hemoglobin distorts red blood cells into crescent shape at low oxygen levels. Most common among those of African descent. Concepts: Cellular Regulation. Sickle Cell Disease. Assessment: Signs and symptoms of sickle cell disease: Anemia. Dark urine. Jaundice. Swelling of hands and feet. Stunted growth. Stroke. Frequent pain episodes. Fatigue. Painful hands and feet. Client/family knowledge: Disease. Ability to manage chronic condition. Diagnosis: Ineffective gas exchange. Decreased tissue perfusion. Pain from the inflammatory response. Lack of knowledge.
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