Further Outpatient Care
Ideally, individuals with severe factor VII (FVII) deficiency should be monitored by a comprehensive hemophilia care team that has experience in the diagnosis and management of inherited bleeding disorders.
Deterrence/Prevention
Individuals who may require plasma-derived coagulation factor concentrates should be immunized with hepatitis A and hepatitis B vaccines.
Patients should avoid use of aspirin and other drugs, including alcohol, that affect platelet function.
Consider prophylactic therapy in patients with recurrent bleeding episodes or CNS hemorrhage.
Patients should maintain good dental hygiene to prevent dental or periodontal disease.
Individuals should participate in appropriate physical activity to maintain muscle strength, and they should wear appropriate protective gear for activities.
Complications
Complications may include the following:
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Recurrent bleeding, particularly hemarthroses, which can result in progressive damage to joints and chronic arthropathy. Management of recurrent hemarthroses applies the principles used in hemophilia care.
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A risk of thrombosis can be associated with the combination of surgery and factor VII replacement therapy using prothrombin complex concentrate or high doses of factor VII concentrates.
Patient Education
Patients and families should be given instruction and educational materials to enable them to understand FVII deficiency, to recognize the symptoms and signs of bleeding, and to identify emergency situations.
Patients should know how to contact their treatment center for immediate treatment, and they should know where to receive emergency care.
Patients should wear a MedicAlert bracelet or carry other identification showing their bleeding disorder and recommended therapy.
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Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin.
