Bleeding disorder.pptx

BLEEDING DISORDER NURSING, MEDICAL SURGICAL NURSING

1. COLLEGE OF NURSING MADRAS MEDICAL COLLEGE, CHENNAI-03 MEDICAL SURGICAL NURSING-II bleeding disorder BY EDWIN JOSE. L., MSC ( N)., COLLEGE OF NURSING, MADRAS MEDICAL COLLEGE, CHENNAI

2. INTRODUCTION Coagulation is the process by which blood forms clots. It is an important part of hemostasis where in a damaged blood vessel wall is covered by a platelet and fibrin containing clot to stop bleeding and begin repair of the damaged vessel. Disorders of coagulation can lead to an increased risk of bleeding or thrombosis Coagulation involves both cellular (platelets) and protein (coagulation factors) component.

3. DEFINITION Bleeding disorders are a group of disorders that share the inability to form a proper blood clot characterized by Bleeding into joints, muscles and soft tissues, excessive bruising, Prolonged heavy menstrual periods (menorrhagia), Unexplained nosebleeds, Extended bleeding after minor cuts, blood draws or vaccinations, minor surgery or dental procedures. - National Hemophilia Foundation

4. TYPES OF BLEEDING DISORDER Congenital   bleeding disorder Acquired  bleeding disorder

5. Congenital   bleeding disorder Congenital bleeding disorders are caused by defects or damage in the genes and are present at birth. They can be inherited or appear due to a genetic mutation. Examples - Hemophilia A (Factor VIII deficiency) Hemophilia B (Factor IX deficiency) Hemophilia C (Factor XI deficiency) vWD (von Willebrand disease)

6. Acquired  bleeding disorder Acquired bleeding disorders encompass a heterogeneous group of conditions with varied and often complex aetiologies . Clinical evaluation of patients presenting with a bleeding disorder often provides clues as to whether the abnormality resides in coagulation factors, platelets or blood vessels Examples – 1. Severe liver disease (because hepatocytes are the major cell type involved in the synthesis of the coagulation factors) 2. DIC (Disseminated intravascular coagulation) 3. Acquired Hemophilia 4. Anticoagulant drugs (heparin, warfarin) 5. Direct oral thrombin inhibitors and direct oral Xa inhibitors 6. The antiphospholipid syndrome

7. HEMOPHILIA

8. Haemophilia Hemophilia is a rare genetic bleeding disorder in which the blood doesn't clot normally because it lacks sufficient blood-clotting proteins (clotting factors). Hemophilia is caused by a mutation or a change in one of the genes that provide instructions for forming blood-clotting proteins. Although the different types of hemophilia have very similar signs and symptoms, they are caused by mutations in different genes. There is no way to cure hemophilia, but there are ways to reduce the risk of bleeding complications.

9. DEFINITION Hemophilia is a rare genetic bleeding disorder that is caused by a deficiency or lack of blood-clotting proteins (clotting factors). - Harrison’s Principles of Internal Medicine This disorder mainly affects males. Hemophiliacs don't have as many clotting factors as there should be in their blood, so this means that they bleed for a longer time than usual after an injury. It can also lead to organ and tissue damage. The severity of the condition is determined by the amount of clotting factors present in the patient’s blood; the less clotting factors a patient has the more severe the condition.

10. Types of hemophilia Hemophilia A (classic hemophilia): It occurs when clotting factor VIII is either absent or not present in sufficient amounts, and it is the most common type. Hemophilia B : It occurs when clotting factor IX is either absent or not present in sufficient amounts. Hemophilia C : It occurs when clotting factor XI is either absent or not present in sufficient amounts. Acquired hemophilia : It is an autoimmune disorder that is not caused by inherited genetic mutations. This condition is characterized by abnormal bleeding in the skin, muscles or other tissues, and it usually starts during puberty. The exact cause is still unknown

11. Risk factors/causes Family history Gender - It affects males more than females. Pregnancy Cancer Autoimmune diseases (multiple sclerosis).

12. Signs and symptoms Pain, swelling or burning sensation in the joints. Unexplained bruises due to internal bleeding. When blood gets trapped in an injured area, it may form a solid swelling of clotted blood (hematoma). Bleeding gums especially after tooth loss. Bleeding after injury. Bleeding after vaccination. Bleeding in the head of an infant after delivery. Blood in the urine or stool. Frequent nosebleeds.

13. Screening & Diagnosis HISTORY COLLECTION: Does anyone in the family have a bleeding disorder? Did the patient have prolonged bleeding after circumcision or dental procedure? Is there a family history of menorrhagia? Does the patient have joint pain or muscle stiffness? Does the patient bruise easily or excessively? Has the patient had episodes of spontaneous bleeding or bruising without trauma? Is there a history of blood transfusions or iron replacement therapy?

14. CONT….. Prenatal Genetic testing Chorionic villus sampling is offered between 9 and 11 weeks gestation Amniocentesis is offered between 12 and 15 weeks gestation Neonatal factor assays Hemophilia A can be diagnosed from FVIII levels in cord blood collected immediately after delivery Hemophilia B requires blood be taken after 6 months of age since FIX is vitamin-K dependent and all neonates have low levels of vitamin K and FIX

15. Other screening tests aPTT is prolonged PT is normal Bleeding time and PFA are normal Diagnosis can only be made by factor assays Hemophilia A - Measure FVIII activity and level Measure von Willebrand Factor to rule out von Willebrand Disease Hemophilia B - Measure FIX activity and level

16. Treatment Management of haemophilia requires prevention and treatment of acute bleeding episodes. Acute bleeding episodes are treated with clotting factors. Patients often have a sense that they are bleeding. They may feel a trickling, warmth, or tingling sensation from blood accumulating in tissues. Clotting factors should be administered immediately to prevent hemarthroses, compartment syndrome, or ICH. For patients with severe disease prophylactic replacement therapy should be implemented. Such treatment reduces episodes of bleeding and prevents the development of arthropathy and compartment syndrome

17. CONT….. There are a variety of products that can be administered to treat and prevent bleeding episodes. Purified Plasma-derived FVIII and FIX are derived from human or porcine serum. Recombinant FVIII and FIX are synthesized from genetically modified cells. Prothrombin complex concentrate contains factors II, VII, IX and X Recombinant FVIIa is given to patients who have developed inhibitors to other factors Desmopressin acetate increases the release of FVIII from endothelial cells and is useful for treating mild-to-moderate FVIII deficiency

18. VON WILLEBRAND DISEASE

19. VON WILLEBRAND DISEASE von Willebrand disease ( vWD ) is the most common bleeding disorder, affecting nearly 1% of the population. vWF is a plasma GP that is composed of low-, intermediate-, and high-molecular weight multimers. vWF binds to platelets through GPIb -IX-V and GPIIb-IIIa on the surface of the platelets, promoting platelet adhesion and aggregation, respectively, at the injured vascular endothelium. vWF also serves as a carrier for FVIII in the circulation

20. Types of Von Willebrand Diseases Inherited Type of Von Willebrand Disease:  It has three main sub-types. The symptoms and treatment is different for each type: Type1 - most common and mildest form of vWD , in which a person has lower-than-normal levels of vWF . A person with Type 1 VWD also might have low levels of factor VIII , another type of blood-clotting protein. About 85% of people treated for VWD have Type 1.

21. CONT…. Type 2 In this the body makes normal amounts of the vWF , the factor does not work the way it should. Type 2 is further broken down into four subtypes―2A, 2B, 2M, and 2N―depending on the specific problem with the person’s vWF . T he treatment is different for each type Type 2A - the vWF is not the right size and doesn’t help the platelets attach together in order to form a clot. Type 2B - the vWF attaches to platelets at the wrong time (when there is no injury). The body removes the platelets attached to vWF , causing a reduced amount of both platelets and vWF in the blood when needed to form a clot.

22. CONT….. Type 2M - vWF does not attach to the platelets as it should, which decreases the platelets’ ability to form a clot when an injury occurs. Type 2N - vWF attaches to the platelets normally. However, the vWF does not attach to another protein, Factor VIII , which is also needed for blood to clot. This causes the body to remove the Factor VIII protein Type 3 This is the most severe form of vWD , in which a person has very little or no vWF and low levels of factor VIII. This is the rarest type of vWD . Only 3% of people with vWD have Type 3. Acquired Type Von Willebrand Disease:  It is a very rare type and occurs due to spontaneous mutation after the child’s birth.

23. Symptoms of von Willebrand disease The common symptoms observed in all types of Von Willebrand Disease are: Easy bruising Excessive nose bleeding Bleeding from gums Women face problems of abnormally heavy or prolonged bleeding during menstruation. Less commonly observed symptoms of Von Willebrand Disease, which occur in older people include Blood in feces due to bleeding in the intestine Blood in urine samples due to bleeding in the  kidneys

24. Diagnosis of Von Willebrand Disease Personal history to know the extent of bleeding on injury or  trauma . Family history to know if the same episodes of this disorder existed in the family or it is acquired.

25. CONT…. laboratory blood tests: Quantitative analysis of von willebrand factor antigen to know the amounts in which it is produced. Qualitative analysis of von willebrand factor multimers to reveal the structure of this protein. This test enables to know the type of Von Willebrand Disease. Ristocetin cofactor (Functional Von Willebrand factor) activity reveals whether this protein performs its function of clotting or not. Factor VIII activity measures the binding capacity of von willebrand factor to factor VIII and whether it maintains the levels of factor VIII in the blood. Platelet function tests to measure if the person’s platelets are functioning normally.

26. Treatments The type of treatment prescribed for VWD depends on the type and severity of the disease. For minor bleeds, treatment might not be needed. The most commonly used types of treatment are: Desmopressin Acetate Injection This medicine is injected into a vein to treat people with milder forms of vWD (mainly Type 1). It works by making the body release more vWF into the blood. It also helps increase the level of factor VIII in the blood. Desmopressin Acetate Nasal Spray This high-strength nasal spray is used to treat people with milder forms of vWD (mainly Type 1). It works by making the body release more vWF into the blood.

27. CONT….. Factor Replacement Therapy Recombinant and medicines rich in vWF and factor VIII are used to treat people with more severe forms of vWD or people with milder forms of vWD who do not respond well to the nasal spray. These medicines are injected into a vein in the arm to replace the missing factor in the blood. Antifibrinolytic Drugs Either injected or taken orally to help slow or prevent the breakdown of blood clots. Birth Control Pills Birth control pills can increase the levels of vWF and factor VIII in the blood and reduce menstrual blood loss.

28. Acquired bleeding disorders

29. Acquired haemophilia A Acquired bleeding disorders are the most common causes of bleeding which can be due to interference with the platelet plug formation, interference with blood coagulation proteins, and interference with both. 

30. Classification of acquired bleeding disorder Acquired bleeding disorders are divided into the following groups: ( i ) clotting factor deficiencies (ii) abnormalities of platelet number or function (iii) vascular defects (iv) various combinations of the three above mentioned disorders which includes liver disease, disseminated intravascular coagulation (DIC) and chronic kidney disease

31. Clotting factor deficiencies Acquired haemophilia A Vitamin K deficiency Anticoagulation and antiplatelet agents

32. Acquired haemophilia A Acquired haemophilia A (AHA) is a rare (~1 per million of the population per year), potentially life-threatening auto-immune bleeding disorder due to inhibiting auto-antibodies (inhibitors) against endogenous FVIII. AHA affects both males and females and is most common in the elderly (median age 64 - 78 years). Most cases are idiopathic, but underlying precipitating causes include pregnancy, auto-immune diseases (most commonly rheumatoid arthritis), infection, malignancy and drugs (e.g. interferon alpha). Research suggests that the breakdown of immune tolerance to FVIII is due to both genetic and environmental factors. The majority (94.6%) of patients present with bleeding, which can either be spontaneous or provoked, e.g. after surgery. The site of bleeding is most commonly subcutaneous, followed by the gastrointestinal tract, intramuscular sites and genito -urinary tract. Bleeding in other sites (intracranial and retroperitoneal) can occur.

33. management Management of patients with acquired inhibitors entails: control of bleeding with haemostatic agents, as for inherited haemophilia patients with inhibitors eradication of the inhibitor with immune modulating agents (e.g. corticosteroids and rituximab) treatment of the underlying pathogenic disease process. Thrombotic complications, including myocardial infarctions and cerebrovascular accidents, can occur in relation to haemostatic agent administration.

34. Vitamin K deficiency Vitamin K is responsible for gamma-carboxylation of FII, FVII, FIX and FX, as well as for proteins C, S and Z. Gammacarboxylation enables binding to phospholipid membranes via Ca++ bridges. Vitamin K deficiency is encountered in various clinical scenarios and causes include: haemorrhagic disease of the newborn (currently termed vitamin K deficiency bleeding), reduced dietary intake, prolonged antibiotic use, cholestatic liver disease, malabsorption, and drugs, e.g. anticonvulsants and warfarin The mode of therapy is oral or intravenous vitamin K, and patients with severe bleeding are treated with fresh-frozen plasma (FFP) or prothrombin complex concentrate (PCC).

35. Anticoagulation and antiplatelet agents Warfarin : Warfarin, a coumarin derivative, inhibits the enzyme vitamin K epoxide reductase and thereby impairs production of vitamin K-dependent coagulation factors, i.e. FII, FVII, FIX and FX, as well as proteins C, S and Z. Patients treated with coumarin derivatives have reduced concentrations of these coagulation factors, with consequent increased risk of bleeding that is amplified when the INR is supratherapeutic (particularly >5). Management of warfarin-associated bleeding depends on the severity of bleeding, the level of the INR and the indication for anticoagulation. For over- warfarinisation without bleeding, stoppage of warfarin with possible oral vitamin K administration is usually sufficient ,if this is accompanied by significant bleeding, reversal of anticoagulation with factor replacement becomes necessary. As for patients scheduled for surgery, anticoagulant reversal must be done before surgery to restore normal coagulation status

36. Heparin: Heparin is an anticoagulant that works by binding to and potentiating the activity of antithrombin, which then inhibits thrombin. Heparin is used for the treatment and prevention of thrombosis. High doses of heparin can cause severe bleeding. In this event, discontinuation of heparin is usually sufficient owing to its short half-life of 8 hours. If rapid reversal of heparin effect is required, protamine sulphate is very effective for unfractionated heparin, but only reverses ~60% of the antifactor Xa activity of low- molecularweight heparin, and has negligible effects on fondaparinux and danaparoid (a mixture of anticoagulant glycosaminoglycans used to treat heparin-induced thrombocytopenia).

37. Non-vitamin K antagonist oral anticoagulants: Non-vitamin K antagonist oral anticoagulants (NOACs) include thrombin inhibitors, e.g. dabigatran, and FXa inhibitors, e.g. rivaroxaban and apixaban. Outcomes of major bleeding are no worse than with vitamin K antagonists. Three NOAC reversal agents are in various stages of development, i.e. idarucizumab for thrombin inhibitors, andexanet for FXa inhibitors, and ciraparantag for all NOACs.

38. Antiplatelet agents: Aspirin exerts its antiplatelet effect by irreversibly binding to the enzyme cyclo-oxygenase. Other antiplatelet agents include NSAIDs and adenosine diphosphate (ADP) receptor inhibitors, such as clopidogrel (Plavix). Mild bleeding and bruising may occur in response to trauma or surgery, but are likely to be exacerbated with coexisting medical conditions, such as haemophilia , renal disease and leukaemia . More severe spontaneous bleeds, e.g. from the gastrointestinal tract, occur less frequently. The effect of aspirin and clopidogrel lasts for 5 - 7 days, i.e. the entire lifespan of the platelet

39. Abnormalities of platelet number & function Platelet defects are typically associated with mucocutaneous bleeding, with the severity depending on the degree of the thrombocytopenia. The risk of bleeding is low when platelets are >80 × /L, and significantly increased when the platelet count is < 20 x /L (where spontaneous bleeding may occur).  

40. Causes of thrombocytopenia: Diminished production (caused by viral infections, vitamin deficiencies, aplastic  anemia , drug-induced) Increased destruction (caused by drugs,  heparin  [HIT], idiopathic,  pregnancy , immune system) Sequestration (caused by an  enlarged spleen ,  neonatal , gestational,  pregnancy )

41. Thrombocytopenic symptoms may include: Petechiae  (superficial tiny areas of bleeding into the skin resulting in small reddish spots) Fatigue Purpura (easy or excessive  bruising ) Prolonged bleeding  cuts Spontaneous bleeding from the gums or nose Jaundice Heavy menstrual bleeding that's unusual for the female Blood in the urine  or stools Enlarged spleen  ( splenomegaly ) Bleeding that will not stop DVT ( deep vein thrombosis )

42. Treatment Platelet transfusion is indicated in all bleeding patients to maintain a platelet count of 50 - 100 × /L (depending on the site of blood loss), as well as in selected patients with a platelet count ˂20 × /L as bleeding prophylaxis.  

43. Immune thrombocytopenia Immune thrombocytopenia (ITP) is an acquired, auto-immune disorder with the formation of antiplatelet antibodies against platelets and megakaryocytes, resulting in increased destruction and inadequate production of platelets. older term is idiopathic thrombocytopenic purpura The threshold for ITP and for clinical thrombocytopenia is defined as a platelet count <100 x /L The incidence of ITP is ~3 - 4.5/100  000/year The presentation of ITP may be acute or insidious ITP may be primary (~80% of cases), with no identifiable cause, or secondary (~20% of cases), due to a number of causes. Secondary causes includes infections (e.g. HIV), auto-immune disorders (e.g. systemic lupus erythematosus), drugs (e.g. rifampicin, quinine and heparin), and lymphoproliferative disorders (e.g. chronic lymphocytic leukaemia, and lymphoma).  

44. Patients with ITP may be asymptomatic (where the platelet count is usually >30 × 109 /L) or may present with bleeding, which is typically of the mucocutaneous type. The incidence of major bleeding events, such as intracranial haemorrhage and cavity bleeding, is low Management- Corticosteroids Prednisone ,dexamethasone and methylprednisolone. cyclophosphamide, danazol and vincristine Alternative drugs include rituximab and thrombopoietin-receptor agonists (TPO-RAs) spleenectomy

45. Micro-angiopathic haemolytic anaemia Micro-angiopathic haemolytic anaemia (MAHA) encompasses a group of entities that are associated with red cell fragmentation haemolysis and thrombocytopenia. Although MAHA may be complicated by thrombocytopenia-related blood loss, the risk of bleeding is significantly higher among patients with DIC. In contrast, bleeding is an unusual complication in thrombotic thrombocytopenic purpura (TTP), despite the thrombocytopenia often being very severe ( <10 x /L)  

46. Disseminated Intravascular coagulation DIC is characterised by systemic activation of the coagulation cascade with production of microthrombi in the small vessels of multiple organs, resulting in organ dysfunction and consumption of coagulation factors and platelets. It can manifest with bleeding and/or thromboembolism, depending on the rate of fibrinolysis and coagulation factor consumption relative to the compensatory production of these proteins. Causes of DIC include severe sepsis, obstetric calamities, major trauma and some malignancies (particularly acute promyelocytic leukaemia ). All of these can result in systemic coagulation activation, either by exposing procoagulant proteins, or by generating procoagulant cytokines

47. It is diagnosed by demonstrating evidence of the following: consumption of blood clotting factors, leading to prolongation of the INR and/or PTT, with a decreasing fibrinogen level. Fibrinogen is, however, an acute-phase reactant, and is therefore not invariably low in patients with DIC consumption of anticoagulant molecules (such as antithrombin) accumulation of the products of fibrinolysis (such as D-dimers) a decreasing platelet count

48. Treatment of DIC is aimed at active management of the underlying cause, and symptomatic management of the associated organ and coagulation abnormalities. Treatment options include FFP and platelet transfusions in the event of bleeding, or low-molecular weight heparin therapy when thromboembolic phenomena predominate. The mortality rate associated with DIC is high, particularly in the presence of pronounced organ dysfunction or severe coagulopathy.

49. Vascular defects Scurvy. Vitamin C promotes peptidyl hydroxylation of procollagen, and its deficiency causes abnormal collagen formation with defective perivascular support. This predisposes to capillary fragility and mucocutaneous bleeding. Treatment is with vitamin C 200 mg daily

50. Henoch- Schonlein purpura. This idiopathic disorder is primarily a disease of children, but may occur at any age, and is characterised by abdominal colic, arthritis, nephritis and palpable purpura. Biopsy of the skin shows an acute immune-related vasculitis and complement/ immunoglobulin complexes. Treatment entails supportive care and steroids in more severe cases

51. Paraproteinemia and amyloidosis The mechanism of bleeding is multifactorial, including interference with coagulation factor levels/ function, impaired platelet aggregation and deposits of light chain/ amyloid fibrils in cutaneous blood vessels, with increased vessel fragility. Cryoglobulins may similarly deposit in dermal vessels and cause vasculitis and purpura. Management entails treatment of the underlying condition.

52. Senile purpura: In the elderly, there is a loss of subcutaneous collagen and elastin fibres . Bruising is usually induced by minor trauma

53. Organ dysfunction Liver disease: Haemostatic abnormalities due to liver disease include: thrombocytopenia due to thrombopoietin deficiency, hypersplenism (if enlarged spleen) and possible megakaryocytic suppression (secondary to alcohol or hepatitis B or C infection) coagulopathy due to impaired production of clotting factors by the liver, vitamin K deficiency (e.g. alcohol abuse, obstructive jaundice), production of functionally abnormal fibrinogen (dysfibrinogenaemia) increased fibrin degradation products due to: impaired hepatic clearance hyperfibrinolysis (impaired clearance of tissue plasminogen activator and decreased production of fibrinolytic inhibitors).

54. Cont … Coagulation factor, fibrinogen and platelet replacement therapy may be needed, but caution should be exercised against liberal use of FFP in liver disease, as the plasma volume expansion may elevate portal pressure and thereby paradoxically increase the risk of variceal bleeding. Bleeding due to hyperfibrinolysis, diagnosed with viscoelastic tests such as the thromboelastogram (TEG), can respond to antifibrinolytic agents, e.g. tranexamic acid.

55. Renal disease Numerous haemostatic disturbances are observed in renal disease, which may predispose to a hypo- or hypercoagulable state. There is no superior pathogenic factor to determine whether a patient would be prone to bleeding or thrombosis, where the dynamics of events are often influenced by comorbid factors. Tendency to bleed is caused by platelet dysfunction (due to accumulation of toxic metabolites, fibrinogen degradation products, anaemia , drugs, etc.) and decreased FXI/XII level. Desmopressin and/or antifibrinolytics are generally effective in controlling uraemic bleeding.

56. Nursing management Nursing Assessment History. For patients in whom the bleeding disorder is suspected, inquire about the history of haemorrhage disproportionate to trauma, history of spontaneous hemorrhage, bleeding disorders in the family, and concomitant illness (especially those associated with acquired haemophilia , such as chronic inflammatory disorders, autoimmune diseases, hematologic malignancies, and allergic drug reactions). Physical examination. Assess for joint swelling and ability to move affected limb; Assess for limited ROM, contractures, and bony changes in the joints when bleeding has stopped.

57. Nursing Diagnoses Acute pain  related to traumatic injury to the muscles. Impaired physical injury  related pain and discomfort with the onset of bleeding episodes. Compromised family coping  related to incorrect and inadequate information or understanding. Risk for bleeding  related to decreased concentration of clotting factors circulating in the blood (factor VIII and factor IX). Risk for injury  related to decreased clotting factor (VIII or IX).

58. Nursing interventions Relieve pain.  Immobilize joints and apply elastic bandages to the affected joint if indicated; elevate affected and apply a cold compress to active bleeding sites, but must be used cautiously to prevent skin breakdown. Maintain optimal physical mobility.  Provide gentle, passive ROM exercise when the condition is stable; educate on preventive measures, such as the application of protective gear and the administration of factor products; and refer for physical therapy, occupational therapy, and orthopedic consultations, as required. Assist in the coping of the family.  Encourage family members to verbalize problem areas and develop solutions on their own; encourage family members to express feelings, such as how they deal with the chronic needs of a family member and coping patterns that help or hinder adjustment to the problems.

59. Cont …. Prevent bleeding.  Monitor haemoglobin and hematocrit levels; assess for inhibitor antibody to factor VIII; anticipate or instruct in the need for prophylactic treatment before high-risk situations, such as invasive diagnostic or surgical procedures, or dental work; and provide replacement therapy of deficient clotting factors. Prevent injury.  Utilize appropriate toys (soft, not pointed or small sharp objects); for infants, may need to use padded bed rail sides on crib; avoid rectal temperatures; provide appropriate oral hygiene (use of a water irrigating device; use of a soft toothbrush or softening the toothbrush with warm water before brushing; use of sponge-tipped toothbrush); and avoid contact sports such as football, soccer, ice hockey, karate.

60. thankyou