HEMOPHILIA

- Inherited Bleeding Disorder - X-linked, affected are males. Types: Hemophilia A- deficient/defective factor VIII Hemophilia B - deficient/defective factor IX Clinical Manefestation a. Hemorrhage in various body part. Mostly in joints ( weight bearing joint). Can also be GI, mucous membrane intra/extra cranial b. Poor wound healing Medical management a. Fresh frozen plasma infusion b. Factor VIII or IX concentrates infusion. c. Aminocaproic acid Adjunctive measure after oral surgery. d. Demopressin (DDAVP) transient increase in factor VIII levels. e. Nursing Interventions a. Prevent trauma by activity restriction and self care measures. b. Avoid anti-platelet agents. ( aspirin, NSAIDS, herbs supplements, alcohol.) c. Dental hygiene is important to prevent extractions. d. Apply pleasure in case of bleeding in minor trauma. e. No injections, invasive procedure should be minimized. f. Close assessment during hemorrhagic episodes. g. Assess surgical site for bleeding. h. Administer for Analgesic for pain. i. Screen blood product for infectious agents. Diagnostic Exam - Factor VIII assay reveals 0%-25% of a normal factor VIII - APT T prolonged - Factor

Von Willerbrands -common bleeding disorder affecting males and females and is usually inherited as
a dominant trait -disease caused by a deficiency of Von Willerbrands factor, w/c is necessary for factor VIII activity. Von Willerbrand factor is also necessary for platelet adhesion at site of vascular injury Clinical Manifestations epistaxis heavy menses prolonged bleeding farom cuts and postoperative bleeding Diagnostic Exams Normal platelet but prolonged BT and PTT Ristocetin cofactor/VWF collagen binding assay means VWF activity

Medical Management a. Replace the deficient protein (VWF or factor VIII) with replacements products like Humate P and Alphanate commercial concentrate of VWF and Factor VIII b. Desmopressin (DDAVP vasopressin analogue) prevent bleeding associated w/ dental or surgical procedures >contra: w/ patients having unstable coronary disease and MI c. Aminocaproic Acid (Amicor) form of mucosal bleeding d. Platelet transfusion Nursing management (Same interventions with hemophilia)

DISSEMINATED INTRAVASCULAR COAGULATION (DIC)

- a.k.a consumption coagulopathy or defribrination syndrome - In DIC, clotting and hemorrhage occur in the vascular system at the same time. Clinical manifestation a. Abnormal bleeding w/o a history of serious hemorrhagic d/o b. petechia c. bleeding in surgical/ IV site d. bleeding from GI tract e. cyanosis of extremities f. n/v, seizures g. oliguria, shock, confusion Diagnostic exams - Thrombocytopenia - Fibrinogen < 150mg/dl - PT > 60-80 s - Fibrin degradation increase > 45mg/ml - + fibrin, monomers, decrease factor V & VIII - + D-dimer test