Nursing care of children with alterations in health status of oxygen transport.
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Autosomal recessive genetic disorder of inadequate production of normal hemoglobin. found in meditteranean ethnic groups.
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Clinical Manifestation
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Asymptomatic | Major retardation | Life threatening
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Splenomegaly, Heptomegaly
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Pathophysiology
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Genetic defect, synthesis of one of a or b globin chains, production of abnormal Hgb and RBC, Hemolysis, Anemia
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Collaborative Care
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• No specific drug or diet are effective for treatment
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• Minor (hetero-beta) : body adapts to low Hgb
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• Major (Homo-beta) : Blood transfusions with IV deferoxamine (used to remove excess iron from the body)
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A net increase in the total number of Red blood cells. Overproduction may be due to:
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- myeloproliferative syndrome in bone marrow
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- reaction to chronically low oxy lvls or malignancy
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Pathophysiology
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Myeloproliferative due to hypoxia, the stem cell grow uncontrollably, BM becomes Hypercellular, Blood becomes thick to sluggish circulation, BM becomes fibrotic.
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Complications
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- Increased viscosity of blood
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- Hemorrhage and thrombosis
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Treatment
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- Phlebotomy
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- Myelosuppressive agents : Intron A, Agrylin, Aspirin
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Disorder of decreased platelets (below 150,000)
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Causes
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•Low production of platelets
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•Increased breakdown of platelets
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Symptoms
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• Bruising, Nosebleeds, Petechiae
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Immune Thrombocytopenic Purpura
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• Abnormal destruction of circulating platelets
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• Autoimmune disorder
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• Destroyed in hosts' spleen by macrophages
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Thrombotic Thrombocytopenic Purpura
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• Increase agglutination of platelets, form microthrombi
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Diagnostic Studies
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•Platelet count, Hgb/Hct, prothrombin time (PT)
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•Activated partial thromboplastin time (aPTT)
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Treatments
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•Corticosteroids (Bethamethasone, prednisone, prelone)
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• Plasmapheresis, Splenectomy, platelet transfusion
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