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The Basics of Hemophilia Nursing Working Group National Hemophilia Foundation.

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Presentation on theme: "The Basics of Hemophilia Nursing Working Group National Hemophilia Foundation."— Presentation transcript:

1 The Basics of Hemophilia Nursing Working Group National Hemophilia Foundation

2 National Hemophilia Foundation Mission Statement The National Hemophilia Foundation is dedicated to finding the cures for inherited bleeding disorders and to preventing and treating the complications of these disordersthrough education, advocacy, and research.

3 Hemostatic System Blood vessels Blood vessels Platelets Platelets Plasma coagulation system Plasma coagulation system Proteolytic or Fibrinolytic system Proteolytic or Fibrinolytic system

4 How Bleeding Stops Vasoconstriction Vasoconstriction Platelet plug formation Platelet plug formation Clotting cascade activated to form fibrin clot Clotting cascade activated to form fibrin clot

5 Normal Hemostasis Hoffman et al. Blood Coagul Fibrinolysis 1998;9(suppl 1):S61. TF-Bearing Cell Activated Platelet Platelet TF VIIIa Va VIIIa Va Va VIIa TF VIIa Xa X II IIa IX VVa II VIII/vWF VIIIa II IXa X IX X IXa IXa VIIa Xa IIa IIa Xa

6 Types of Bleeding Disorders Hemophilia A (factor VIII deficiency) Hemophilia A (factor VIII deficiency) Hemophilia B (factor IX deficiency) Hemophilia B (factor IX deficiency) von Willebrand Disease (vWD) von Willebrand Disease (vWD) Other Other

7 What is Hemophilia? Hemophilia is an inherited bleeding disorder in which there is a deficiency or lack of factor VIII (hemophilia A) or factor IX (hemophilia B) Hemophilia is an inherited bleeding disorder in which there is a deficiency or lack of factor VIII (hemophilia A) or factor IX (hemophilia B)

8 Inheritance of Hemophilia Hemophilia A and B are X-linked recessive disorders Hemophilia A and B are X-linked recessive disorders Hemophilia is typically expressed in males and carried by females Hemophilia is typically expressed in males and carried by females Severity level is consistent between family members Severity level is consistent between family members ~30 % of cases of hemophilia are new mutations ~30 % of cases of hemophilia are new mutations

9 Detection of Hemophilia Family history Family history Symptoms Symptoms Bruising Bruising Bleeding with circumcision Bleeding with circumcision Muscle, joint, or soft tissue bleeding Muscle, joint, or soft tissue bleeding Hemostatic challenges Hemostatic challenges Surgery Surgery Dental work Dental work Trauma, accidents Trauma, accidents Laboratory testing Laboratory testing

10 Degrees of Severity of Hemophilia Normal factor VIII or IX level = 50-150% Normal factor VIII or IX level = 50-150% Mild hemophilia Mild hemophilia factor VIII or IX level = 6-50% factor VIII or IX level = 6-50% Moderate hemophilia Moderate hemophilia factor VIII or IX level = 1-5% factor VIII or IX level = 1-5% Severe hemophilia Severe hemophilia factor VIII or IX level = <1% factor VIII or IX level = <1%

11 Hemophilia A: 20.6 per 100,000 males Hemophilia A: 20.6 per 100,000 males Severe: 50-60% Severe: 50-60% Hemophilia B: 5.3 per 100,000 males Hemophilia B: 5.3 per 100,000 males Severe: 44% Severe: 44% U. S. Incidence of Hemophilia

12 Types of Bleeds Joint bleeding - hemarthrosis Joint bleeding - hemarthrosis Muscle hemorrhage Muscle hemorrhage Soft tissue Soft tissue Life threatening-bleeding Life threatening-bleeding Other Other

13 Joint or Muscle Bleeding Symptoms Symptoms Tingling or bubbling sensation Tingling or bubbling sensation Stiffness Stiffness Warmth Warmth Pain Pain Unusual limb position Unusual limb position

14 Life-Threatening Bleeding Head / Intracranial Head / Intracranial Nausea, vomiting, headache, drowsiness, confusion, visual changes, loss of consciousness Nausea, vomiting, headache, drowsiness, confusion, visual changes, loss of consciousness Neck and Throat Neck and Throat Pain, swelling, difficulty breathing/swallowing Pain, swelling, difficulty breathing/swallowing Abdominal / GI Abdominal / GI Pain, tenderness, swelling, blood in the stools Pain, tenderness, swelling, blood in the stools Iliopsoas Muscle Iliopsoas Muscle Back pain, abdominal pain, thigh tingling/numbness, decreased hip range of motion Back pain, abdominal pain, thigh tingling/numbness, decreased hip range of motion

15 Other Bleeding Episodes Mouth bleeding Mouth bleeding Nose bleeding Nose bleeding Scrapes and/or minor cuts Scrapes and/or minor cuts Menorrhagia Menorrhagia

16 Complications of Bleeding Flexion contractures Flexion contractures Joint arthritis / arthropathy Joint arthritis / arthropathy Chronic pain Chronic pain Muscle atrophy Muscle atrophy Compartment syndrome Compartment syndrome Neurologic impairment Neurologic impairment

17 Treatment of Hemophilia Replacement of missing clotting protein Replacement of missing clotting protein On demand On demand Prophylaxis Prophylaxis DDAVP / Stimate DDAVP / Stimate Antifibrinolytic Agents Antifibrinolytic Agents Amicar Amicar Supportive measures Supportive measures Icing Icing Immobilization Immobilization Rest Rest

18 Factor VIII Concentrate Intravenous infusion Intravenous infusion IV push IV push Continuous infusion Continuous infusion Dose varies depending on type of bleeding Dose varies depending on type of bleeding Ranges from 20-50+ units/kg. body weight Ranges from 20-50+ units/kg. body weight Half-life 8-12 hours Half-life 8-12 hours Each unit infused raises serum factor VIII level by 2 % Each unit infused raises serum factor VIII level by 2 %

19 Factor IX Concentrate Intravenous infusion Intravenous infusion IV push IV push Continuous infusion Continuous infusion Dose varies depending on type of bleeding Dose varies depending on type of bleeding Ranges from 20-100+ units/kg. body weight Ranges from 20-100+ units/kg. body weight Half-life 12-24 hours Half-life 12-24 hours Each unit infused raises serum factor IX level by 1% Each unit infused raises serum factor IX level by 1%

20 History of Clotting Factor Concentrates - 1 Prior to 1950: whole blood 1952: Hemophilia A distinguished from B 1950-1960: FFP and Cryoprecipitate Early 1970s: Commercial plasma-derived factor concentrates Mid-late 1970s: Home infusion practices 1981: First AIDS death in the Hemophilia community

21 History of Clotting Factor Concentrates - 2 Mid-1983: Factor concentrates heat treated for hepatitis 1985: All products heat treated for viral inactivation 1987: Monoclonal factor concentrates 1992: Recombinant factor VIII 1994: Recombinant factor IX-albumin free 2001: 2nd generation recombinant factor VIII

22 Infusions of Factor Concentrates Verify product with physician order. Verify product with physician order. Dose may be +/- 10% ordered. Dose may be +/- 10% ordered. Do not waste factor even if the dose is not exactly what is ordered. Do not waste factor even if the dose is not exactly what is ordered. Reconstitute factor per package insert. Reconstitute factor per package insert. Infusion rate per package insert or pharmacy instructions. Infusion rate per package insert or pharmacy instructions. Document lot number, expiration date, time of infusion, and exact dose given in units. Document lot number, expiration date, time of infusion, and exact dose given in units.

23 Prophylaxis Scheduled infusions of factor concentrates to prevent most bleeding Scheduled infusions of factor concentrates to prevent most bleeding Frequency: 2 to 3 times weekly to keep trough factor VIII or IX levels at 2-3% Frequency: 2 to 3 times weekly to keep trough factor VIII or IX levels at 2-3% Types Types primary prophylaxis primary prophylaxis secondary prophylaxis secondary prophylaxis Use of IVAD necessary in some patients Use of IVAD necessary in some patients

24 DDAVP ( Desmopressin acetate) Synthetic vasopressin Synthetic vasopressin Method of action - Method of action - release of stores from endothelial cells raising factor VIII and vWD serum levels release of stores from endothelial cells raising factor VIII and vWD serum levels Administration - Administration - Intravenous Intravenous Subcutaneously Subcutaneously Nasally (Stimate) Nasally (Stimate) Side effects Side effects

25 Stimate How supplied How supplied 1.5 mg./ ml (NOT to be confused with DDAVP nasal spray for nocturnal enuresis) 1.5 mg./ ml (NOT to be confused with DDAVP nasal spray for nocturnal enuresis) 2.5 ml bottle - delivers 25 doses of 150 mcg. 2.5 ml bottle - delivers 25 doses of 150 mcg. Dosing Dosing Every 24-48 hours prn Every 24-48 hours prn <50 kg. body weight - 1 spray (150 mcg.) <50 kg. body weight - 1 spray (150 mcg.) >50 kg. body weight - 2 sprays (300 mcg.) >50 kg. body weight - 2 sprays (300 mcg.)

26 Amicar (epsilon amino caproic acid) Antifibrinolytic Antifibrinolytic Uses Uses Mucocutaneous bleeding Mucocutaneous bleeding Dosing: 50 - 100 mg./kg. q. 6 hours Dosing: 50 - 100 mg./kg. q. 6 hours Side effects Side effects Contraindications Contraindications Hematuria Hematuria

27 Complications of Treatment Inhibitors/Antibody development Inhibitors/Antibody development Hepatitis A Hepatitis A Hepatitis B Hepatitis B Hepatitis C Hepatitis C HIV HIV

28 Inhibitors Definition Definition IgG antibody to infused factor VIII or IX concentrates, which occurs after exposure to the extraneous VIII or IX protein. IgG antibody to infused factor VIII or IX concentrates, which occurs after exposure to the extraneous VIII or IX protein. Prevalence Prevalence 20-30% of patients with severe hemophilia A 20-30% of patients with severe hemophilia A 1-4% of patients with severe hemophilia B 1-4% of patients with severe hemophilia B

29 Hepatitis Hepatitis A - small risk of transmission Hepatitis A - small risk of transmission Vaccination recommended Vaccination recommended Hepatitis B - no transmissions since 1985 Hepatitis B - no transmissions since 1985 Vaccination recommended Vaccination recommended Hepatitis C - no transmissions since 1990 Hepatitis C - no transmissions since 1990 ~90% of patients receiving factor concentrates prior to 1985 are HCV antibody positive ~90% of patients receiving factor concentrates prior to 1985 are HCV antibody positive

30 Human Immunodeficiency Virus No transmissions of HIV through factor concentrates since 1985 due to viral inactivation procedures No transmissions of HIV through factor concentrates since 1985 due to viral inactivation procedures HIV seropositive rate - HIV seropositive rate - 69.6% of patients with severe hemophilia A receiving factor concentrates prior to 1985 69.6% of patients with severe hemophilia A receiving factor concentrates prior to 1985 48.6% of patients with severe hemophilia B receiving factor concentrates prior to 1985 48.6% of patients with severe hemophilia B receiving factor concentrates prior to 1985

31 Nursing Considerations Factor replacement to be given on time Factor replacement to be given on time Laboratory monitoring Laboratory monitoring Increase metabolic states will increase factor requirements Increase metabolic states will increase factor requirements Factor coverage for invasive procedures Factor coverage for invasive procedures Document - infusions, response to treatment Document - infusions, response to treatment Avoid NSAIDS Avoid NSAIDS Utilize Hemophilia Center staff for questions / problems Utilize Hemophilia Center staff for questions / problems

32 Psychosocial Issues Guilt Guilt Challenge of hospitalizations Challenge of hospitalizations Control issues Control issues Financial / insurance challenges Financial / insurance challenges Feeling different / unable to do certain activities Feeling different / unable to do certain activities Counseling needs Counseling needs

33 Hemophilia Treatment Center Team Members Patient / Family Patient / Family Hematologist Hematologist Nurse Nurse Social Worker Social Worker Physical Therapist Physical Therapist Orthopedist Orthopedist Primary Care Infectious Disease Genetics Pharmacy Dental Hepatology

34 Role of Hemophilia Treatment Centers State-of-the-art medical treatment for persons with hemophilia through the life span State-of-the-art medical treatment for persons with hemophilia through the life span Education Education Research Research Outreach Outreach Model of comprehensive care for chronic disease Model of comprehensive care for chronic disease


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