The common side effects of DDAVP include facial warmth and flushing during the infusion. Insignificant variations in blood pressure may be noted. Headaches may occur as late as 6–8 hours after the infusion. Abdominal cramping with diarrhea and generalized myalgias have rarely been noted. In very ill patients, fluid retention resulting in congestive heart failure has been reported. MLID85223521 44 In small children receiving large fluid volumes, seizures induced by severe hyponatremia after DDAVP infusion have recently occurred. MLID90053541 45 In the young pediatric age group serum sodium levels should be monitored and large amounts of intravenous fluid avoided if DDAVP is used, especially if repeated doses are given. Myocardial infarction temporally related to DDAVP infusion has been reported, MLID88094560 46 although whether DDAVP truly causes a hypercoagulable state is unclear. These serious side effects are very uncommon. In summary, DDAVP is the treatment of choice in persons with mild hemophilia A if they respond adequately.
Surgery And Hemophilia
Both elective and emergency surgery can be done in a patient with hemophilia A unless an inhibitor is present. Before surgery (1) a hematologist and diagnostic coagulation laboratory should be available; (2) the surgeon should feel comfortable handling a patient with a coagulation disorder; (3) there should be a blood bank or pharmacy capable of providing adequate amounts of the appropriate replacement material; (4) an appropriate rehabilitation team should be available for postoperative management, especially with orthopaedic surgery; and (5) no inhibitor should be present. An inhibitor level must be checked immediately prior to surgery. Surgery should be scheduled on Monday or Tuesday to allow for availability of laboratory services for factor level assays and best access to consultants.
Preoperative orders should include “No IM medication” and “No ASA-containing compounds such as Darvon, Empirin or percodan.” For major surgery, the factor VIII level should be brought to the 80 - 100% range (40–50 U/kg) about 1 hour prior to surgery and then kept >30–50% for 10–14 days. The theoretical calculations should be checked every 2–3 days with factor VIII assays and the dose adjusted accordingly.
Postoperatively, pain management should be aggressive, with patient-assisted narcotic delivery systems (PCA) or constant infusional narcotic dosing.
For oral surgery such as impacted wisdom teeth removal, the factor level prior to surgery is raised to 100% with infusions. Postoperatively e-aminocaproic acid (1 g PO every 4 hours) for 7–10 days is given. If the dental procedure is minor, e-aminocaproic acid may be used alone.
Patients with mild hemophilia may be able to utilize DDAVP. If there is a poor response to DDAVP, some physicians use cryoprecipitate. However, if DDABP cannot be used, we recommend factor concentrate since it can be treated with viricidal methods and is thus currently safer than cryoprecipitate.
Complications of factor use include hepatitis C, which has largely been eliminated by the new production methods and hemolysis due to anti-A or anti-B in the concentrate preparation. If hemolysis occurs, blood loss should be replaced by type O packed red cells, and concentrates with low isoagglutinin titers should be obtained and utilized.
Health Care Delivery
The keystone of therapy in hemophilia is to provide the patient with access to immediate and adequate correction of the hemostatic defect at the earliest symptom suggestive of hemorrhage. For most persons with severe and moderately severe hemophilia, the achievement of this goal is through a combination of intensive education of the patient and family, plus the institution of a carefully supervised self-therapy program.
Self-Therapy Program
Уважаемый посетитель!
Чтобы распечатать файл, скачайте его (в формате Word).
Ссылка на скачивание - внизу страницы.