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KEY CONCEPTS

KEY CONCEPTS

  • image Hemophilia is an inherited bleeding disorder resulting from a congenital deficiency in factor VIII or IX.

  • image The goal of therapy for hemophilia is to prevent bleeding episodes and their resulting long-term complications and to arrest bleeding if it occurs.

  • image Recombinant factor concentrates usually are the first-line treatment of hemophilia because they have the lowest risk of infection.

  • image Inhibitor formation is the most significant treatment complication in hemophilia and is associated with significant morbidity and decreased quality of life.

  • image Recombinant factor VIIa is effective for the treatment of acute bleeds in patients with hemophilia A or B who have developed inhibitors.

  • image The goal of therapy for von Willebrand disease (vWD) is to increase von Willebrand factor (vWF) and factor VIII levels to prevent bleeding during surgery or arrest bleeding when it occurs.

  • image vWF concentrates are the agents of choice for treatment of type 3 vWD and some type 2 vWD, and for serious bleeding in type 1 vWD.

  • image Desmopressin acetate often is effective for the treatment of type 1 vWD. It also may be effective for the treatment of some forms of type 2 vWD in addition to mild-to-moderate hemophilia A.

PATIENT CARE PROCESS

Patient Care Process for Coagulation Disorders

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Collect

  • Patient characteristics (age, sex, etc.)

  • Patient medical history (including family history and bleeding history)

  • Social history (level of activity, extracurricular activities)

  • Medication list, especially aspirin, nonsteroidal anti-inflammatory drugs [NSAIDs], other antiplatelet medications and anticoagulants

  • Labs—clotting factors

Assess

  • Presence of active bleeding (see “Clinical Manifestations and Diagnosis” section and Table 123-1)

  • Presence of common clinical manifestations in bleeding disorders (easy bruising, bleeding after surgery, mucocutaneous bleeding, prolonged menses, etc., and Table 123-1).

  • Factor level and the need for prophylactic management

Plan*

  • Determine drug therapy regimen (dose, route, frequency, and duration) (see Table 123-3 and “Treatment: Hemophilia” section)

  • Monitoring parameters (signs/symptoms of bleeding, frequency of bleeding episodes)

  • Patient education/counseling (control of bleeding episodes, administration of drug therapy, when to seek medical attention)

  • Continued care at a Hemophilia Treatment Center (or comparable clinic with a comprehensive care team)

Implement

  • Provide education and reinforcement of the treatment plan

  • Improve adherence through motivational interviewing and open discussions about patient care and the treatment plan

  • Schedule regular clinic appointments to assess adherence, bleeding episodes, and to tailor patient therapy

Follow-up: Monitor and Evaluate

  • Control and resolution of bleeding signs and symptoms

  • Number of bleeding episodes (improvement since beginning care, need for adjustment in the treatment plan)

  • Factor level activity (tailor factor doses to patient-specific levels)

  • Adherence to the treatment plan (prophylactic and as-needed therapy, seeking medical attention)

*Collaborate with patients, caregivers, and other healthcare professionals.

BEYOND THE BOOK

BEYOND THE BOOK

Create a Venn diagram to compare hemophilia A and B. Within the diagram, be sure to include pertinent disease state information such as clinical manifestations, incidence, and recommended treatment. ...

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