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Comprehensive Overview of Hemophilia Treatment: Drugs and Management Strategies

Hemophilia is a genetically-based bleeding disorder predominantly affecting males, with severe and moderate cases stemming from defective genes inherited from mothers or spontaneous mutations. This condition hinders normal hemostasis, impacting fibrin production. Effective management includes factor VIII and IX concentrates, pain management strategies, and immunization. Innovative therapies like desmopressin and antifibrinolytic agents are also key in preventing bleeding. Patients with inhibitors face challenges, requiring specialized treatments. Understanding these facets is essential for optimal patient care.

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Comprehensive Overview of Hemophilia Treatment: Drugs and Management Strategies

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  1. Chapter 54 Drugs for Hemophilia

  2. Hemophilia • Genetically based bleeding disorder • Almost exclusively seen in males • 70% of cases result from inheriting defective gene from mother • 30% of cases result from spontaneous gene mutation • Hemophilia A and hemophilia B • Prognosis is good with good management

  3. Basic Considerations • Pathophysiology • Failure of hemostasis • Normal process • Formation of a platelet plug followed by production of fibrin • Hemophilia • Fibrin production does not proceed normally

  4. Basic Considerations • Inheritance pattern • Recessive gene (both factors VIII and IX) • Carried on the X chromosome • Females can have hemophilia in some cases • Clinical features • Severe hemophilia • Moderate hemophilia • Mild hemophilia

  5. Basic Considerations • Overview of therapy • Pain management • Mild pain: Tylenol • Moderate to severe pain:opioids • Avoid NSAIDs and aspirin • More research needed for COX-2 use • Immunization • Normal immunization schedule • SubQ rather than IM injections • Vaccinated for hepatitis A and B

  6. Fig.54–1. Outline of the coagulation cascade showing clotting factors used to treat hemophilia.

  7. Preparations Used to Treat Hemophilia • Factor VIII concentrates • Production methods and product safety • Plasma-derived factor VIII (low risk of viral contaminants) • Recombinant factor VIII • Adverse effects: allergic reactions • Dosage and administration • On-demand therapy • Prophylactic therapy

  8. Preparations Used to Treat Hemophilia • Factor IX concentrates • Nearly identical pharmacology to that of factor VIII • No risk of HIV/AIDS transmission • Small risk of hepatitis A transmission • On-demand therapy • Prophylactic therapy

  9. Preparations Used to Treat Hemophilia • Desmopressin • Stops or prevents bleeding (mild hemophilia) • Antifibrinolytic agents • Used to prevent recurrent bleeding, less useful to stop ongoing bleeding • Aminocaproic acid (Amicar), tranexamic acid (Cyklokapron)

  10. Managing Patients Who Develop Inhibitors • Inhibitors:antibodies that develop against factor VIII or IX • Drugs for patients with inhibitors • Factor VIII inhibitors • Activated factor VII (factor VIIa) • Anti-inhibitor coagulant complex (AICC) • Porcine factor VIII • Factor IX inhibitors • Limited options • Immune tolerance therapy (ITT) often fails

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