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Hemophilia
- 1. The Basics of Hemophilia MATHEW VARGHESE V MSN(RAK),FHNP (CMC Vellore),CPEPC Nursing officer AIIMS Delhi 1mathewvmaths@yahoo.co.in
- 2. Hemostatic System • Blood vessels • Platelets • Plasma coagulation system • Proteolytic or Fibrinolytic system 2mathewvmaths@yahoo.co.in
- 3. How Bleeding Stops • Vasoconstriction • Platelet plug formation • Clotting cascade activated to form fibrin clot 3mathewvmaths@yahoo.co.in
- 7. Clotting cascade • Intrinsic Pathway – Initiated by Endothelial surface injury • Extrinsic Pathway - Trauma • Common Pathway 7mathewvmaths@yahoo.co.in
- 8. I P E P C P 8mathewvmaths@yahoo.co.in
- 10. 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) or factor XI (hemophilia C) 10mathewvmaths@yahoo.co.in
- 11. Types of hemophilia •Hemophilia A - Factor 8 •Hemophilia B - Factor 9 •Hemophilia C- Factor 11 11mathewvmaths@yahoo.co.in
- 12. Inheritance of Hemophilia • Hemophilia is a X-linked recessive disorder • Hemophilia is typically expressed in males and carried by females • Severity level is consistent between family members • ~30 % of cases of hemophilia are new mutations 12mathewvmaths@yahoo.co.in
- 16. Incidence •Hemophilia A – 1:5000 •Hemophilia B – 1:25000 •Hemophilia C- 1:50000 16mathewvmaths@yahoo.co.in
- 17. Detection of Hemophilia • Family history • Symptoms – Bruising – Bleeding with circumcision – Muscle, joint, or soft tissue bleeding • Hemostatic challenges – Surgery – Dental work – Trauma, accidents • Laboratory testing 17mathewvmaths@yahoo.co.in
- 18. Degrees of Severity of Hemophilia • Normal factor VIII or IX level = 50-150% • Mild hemophilia – factor VIII or IX level = 6-50% • Moderate hemophilia – factor VIII or IX level = 1-5% • Severe hemophilia – factor VIII or IX level = <1% 18mathewvmaths@yahoo.co.in
- 19. Types of Bleeds • Joint bleeding - hemarthrosis • Muscle hemorrhage • Soft tissue • Life threatening-bleeding • Other 19mathewvmaths@yahoo.co.in
- 20. Joint or Muscle Bleeding • EARLY SIGNS • A tingling, bubbling sensation (but no real pain) • Tightness and pain (but no visible signs) • LATER SIGNS • Swelling at the joint and a hot feeling when the skin is touched • Pain when bending or extending the joint • Worsening swelling, and difficulty moving 20mathewvmaths@yahoo.co.in
- 21. Life-Threatening Bleeding • Head / Intracranial – Nausea, vomiting, headache, drowsiness, confusion, visual changes, loss of consciousness • Neck and Throat – Pain, swelling, difficulty breathing/swallowing • Abdominal / GI – Pain, tenderness, swelling, blood in the stools • Iliopsoas Muscle – Back pain, abdominal pain, thigh tingling/numbness, decreased hip range of motion 21mathewvmaths@yahoo.co.in
- 23. Other Bleeding Episodes • Mouth bleeding • Nose bleeding • Scrapes and/or minor cuts • Menorrhagia 23mathewvmaths@yahoo.co.in
- 24. Complications of Bleeding • Flexion contractures • Joint arthritis / arthropathy • Chronic pain • Muscle atrophy • Compartment syndrome • Neurologic impairment 24mathewvmaths@yahoo.co.in
- 25. Treatment of Hemophilia • Replacement of missing clotting protein – On-demand therapy. Infusions are given as soon as possible after a bleed happens to control bleeding. This is often how joint and muscle bleeds are treated. Sometimes, especially with prompt treatment, one infusion is enough. In other cases, treatment is given over several days. For more serious bleeding, a patient may need 2 to 3 infusions a day, for 10 to 14 days. – Prophylaxis (preventative) therapy. Infusions are given once or more a week to help prevent bleeds. Often, children with hemophilia receive prophylaxis therapy to help prevent bleeds from happening at all. Making smart and safe choices about activities is also key to preventing bleeds. 25mathewvmaths@yahoo.co.in
- 26. First Aid : RICE • Rest • Ice • Compression • Elevation 26mathewvmaths@yahoo.co.in
- 27. Factor VIII Concentrate • Intravenous infusion – IV push – Continuous infusion • Dose varies depending on type of bleeding – Ranges from 20-50+ units/kg. body weight • Half-life 8-12 hours • Each unit infused raises serum factor VIII level by 2 % 27mathewvmaths@yahoo.co.in
- 28. Factor IX Concentrate • Intravenous infusion – IV push – Continuous infusion • Dose varies depending on type of bleeding – Ranges from 20-100+ units/kg. body weight • Half-life 12-24 hours • Each unit infused raises serum factor IX level by 1% 28mathewvmaths@yahoo.co.in
- 29. Infusions of Factor Concentrates • Verify product with physician order. • Dose may be +/- 10% ordered. • Do not waste factor even if the dose is not exactly what is ordered. • Infusion rate per package insert or pharmacy instructions. • Document lot number, expiration date, time of infusion, and exact dose given in units. 29mathewvmaths@yahoo.co.in
- 30. Desmopressin acetate in Hemophilia A • Synthetic vasopressin • Method of action - Release of stores from endothelial cells raising factor VIII Administration – Intravenous – Subcutaneously – Nasally (Stimate) 30mathewvmaths@yahoo.co.in
- 31. Stimate • How supplied – 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. • Dosing – Every 24-48 hours prn – <50 kg. body weight - 1 spray (150 mcg.) – >50 kg. body weight - 2 sprays (300 mcg.) 31mathewvmaths@yahoo.co.in
- 32. Tranexa & Amicar (Tranexamic acid and Aminocaproic acid ) • Antifibrinolytic • Uses – Mucocutaneous bleeding • Dosing: 50 - 100 mg./kg. q. 6 hours 32mathewvmaths@yahoo.co.in
- 33. Complications of Treatment • Inhibitors/Antibody development • Hepatitis B • Hepatitis C • HIV 33mathewvmaths@yahoo.co.in
- 34. Treating inhibitors • Managing inhibitors is complex and one of the biggest challenges facing people with hemophilia • Doctors often treat inhibitors by using something called immune tolerance therapy (ITT), which tries to “teach” the body to stop fighting factor replacement therapy. Very high doses of factor VIII are used in ITT. • Sometimes, a doctor will have to try different regimens of ITT to find optimal results. Not everyone will respond to ITT. ITT is usually not effective in people with hemophilia B. • If ITT doesn’t work, other treatment options can be tried. These treatments focus on preventing bleeding, rather than fighting the inhibitors. 34mathewvmaths@yahoo.co.in
- 35. Nursing Considerations • Factor replacement to be given on time • Laboratory monitoring • Increase metabolic states will increase factor requirements • Factor coverage for invasive procedures • Document - infusions, response to treatment • Avoid NSAIDS • Patient education and counseling 35mathewvmaths@yahoo.co.in
- 36. Psychosocial Issues • Guilt • Challenge of hospitalizations • Disease Control issues • Financial / insurance challenges • Feeling different / unable to do certain activities • Counseling needs 36mathewvmaths@yahoo.co.in
- 37. Hemophilia Treatment Center Team Members • Patient / Family • Hematologist • Nurse • Social Worker • Physical Therapist • Orthopedist • Primary Care • Infectious Disease • Genetics • Pharmacy • Dental • Hepatology 37mathewvmaths@yahoo.co.in