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note pain in a joint before they are aware of swelling and limitation of motion. ⇒Spontaneous hematuria and GI bleeding can occur. ⇒Bleeding is also common in other mucous membranes such as nasal passage. ⇒The most dangerous site of hemorrhage is in the head. ⇒Now factor VIII, IX concentrates are available to all blood banks. Patients are given concentrates when they are actively bleeding or as a preventive measure before traumatic procedures. The patient and family are taught how to administer the concentrate by IV at home at the first sign of bleeding. Desmopressin induces a transient rise in factor VIII levels; the mechanism for this response is unknown. In patients with hemophilia A, desmopressinis extremely useful, significantly reducing the amount of blood products required. ⇒The patients are encouraged to be self-sufficient and to maintain independence by preventing unnecessary trauma that can cause acute bleeding episodes and temporarily interfere with normal activities. ⇒Patients with hemophilia are instructed to avoid any agents that interfere with platelet aggregation such as aspirin, NSAIDS, herbs, nutritional supplements and alcohol. These restrictions apply to OTC medications such as cold remedies. Applying pressure may be sufficient to control bleeding. ⇒Splints and other orthopedic devices may be useful in patients with joint or muscle hemorrhages. All injections should be avoided; invasive procedures should be minimized or performed after administration of appropriate factor replacement. Patients should be encouraged to carry a medical identification bracelet. During hemorrhagic episodes, the extent of bleeding must be assessed carefully. ⇒During bleeding episodes, heat is avoided because it can accentuate bleeding. ⇒Analgesics are used for pain management. DISSEMINATED INTRAVASCULAR COAGULATION (DIC) ⇒DIC is not a disease but a sign of an underlying condition. DIC may be triggered by sepsis, trauma, cancer, shock, abruptio placentae, toxins, or allergic reactions. It is potentially life-threatening ⇒Normal hemostatic mechanisms are altered in DIC so that a massive amount of tiny clots forms in the microcirculation. There is a paradoxical result of excessive clotting resulting in bleeding. The clinical manifestations of DIC are reflected in the organs, which are affected
27either by excessive clot formation or by bleeding. The excessive clotting triggers the fibrinolytic system to release fibrin degradation products, which are potent anticoagulants, furthering the bleeding. The bleeding is characterized by low platelet and fibrinogen levels; prolonged PT, PTT and thrombin time, and elevated fibrin degradation products (D-dimers). ⇒These patients bleed from mucous membranes, venipunctures sites, and the GI and urinary tracts. The bleeding can range from minimal occult internal bleeding to profuse hemorrhage from all orifices. The patient may also develop organ dysfunction, such as renal failure and pulmonary and multifocal central nervous system infarctions as result of hemorrhage.