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Practical approach to a bleeding child Peri Kamalakar,MD Director The Valerie Fund Children’s Centers For Cancer &Blood Disorders At Saint Barnabas Health.

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Presentation on theme: "Practical approach to a bleeding child Peri Kamalakar,MD Director The Valerie Fund Children’s Centers For Cancer &Blood Disorders At Saint Barnabas Health."— Presentation transcript:

1 Practical approach to a bleeding child Peri Kamalakar,MD Director The Valerie Fund Children’s Centers For Cancer &Blood Disorders At Saint Barnabas Health Care System Associate Director, Hemophilia Center Newark Beth israel Medical Center

2 Practical approach to a bleeding child OBJECTIVES: Overview of hemostasis Overview of hemostasis Clinical approach in making a diagnosis Clinical approach in making a diagnosis Review the most common bleeding conditions Review the most common bleeding conditions Discuss the current treatment strategies Discuss the current treatment strategies

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5 Overview of Haemostasis INJURY Collagen Exposure Platelet Adhesion and release reaction Platelet aggregation VASOCONSTRICTION SerotoninPlatelet Phospolipid Thromboxane A2 ADP Primary haemostatic plug Stable haemostatic plug Tissue Factor Coagulation Thrombin Fibrin Fibrinolysis

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8 Overview of Haemostasis INJURY Collagen Exposure Platelet Adhesion and release reaction Platelet aggregation VASOCONSTRICTION SerotoninPlatelet Phospolipid Thromboxane A2 ADP Primary haemostatic plug Stable haemostatic plug Tissue Factor Coagulation Thrombin Fibrin Fibrinolysis

9 KininsHMW Kininogen Kallikrein Contact Activation XII Prekallikrein XIIa XIaXI IXa Ca++ IX VIIIaVIII Ca++ Phospholipid Intrinsic Pathway XaX VaV Ca++ Phospholipid XIII IIIIa XIIa FibrinogenFibrinXIII Common Pathway VIIVIIa Ca++ Tissue Factor Coagulation cascade Extrinsic Pathway

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11 PRACTICAL APPROACH TO A CHILD WITH BLEEDING HISTORY HISTORY – HISTORY – HISTORY HISTORY – HISTORY – HISTORY >AGE OF ONSET >AGE OF ONSET > SEX > SEX >FREQUENCY >FREQUENCY >LOCATION / TYPE OF BLEEDING >LOCATION / TYPE OF BLEEDING >DURATION OF BLEEDING >DURATION OF BLEEDING > MEDICATIONS > MEDICATIONS > ASSOCIATED SYMPTOMS > ASSOCIATED SYMPTOMS > REVIEW OF SYSTEMS > REVIEW OF SYSTEMS

12 Approach to a bleeding patient –What is the type of bleeding disorder? Primary hemostasis – Vascular causes Platelets-Number vs. Platelets-Number vs. Function Function Fibrin formation – clotting factors Premature clot dissolution- post clot formation

13 . Approach to a bleeding patient –Is a bleeding tendency present? Easy Bruising Mucosal bleeding Menorrhagia Surgical Hemorrhage – Procedure vs.Diathesis Postpartum Hemorrhage Joint and Muscle bleed –Severity of trauama

14 . Approach to a bleeding patient –Is the disorder Familial or Acquired? Family history – MOTHER & OTHER FEMALE MEMBERS IN THE IMMEDIATE FAMILY – Detailed Menstrual history

15 Vascular causes – Vascular causes – First and foremost rule out infectious causes – “Meningococcemia” First and foremost rule out infectious causes – “Meningococcemia” Vasculitis – Henoch-Schonlein Purpura Hemangiomas- Kassalback-Merritt syndrome

16 Petechiae and Purpura Petechiae and Purpura Infectious Infectious – Meningococcemia – Meningococcemia – Rocky mountain spotted – Rocky mountain spotted fever fever – Group A strep – Group A strep – Atypical measles – Atypical measles – Echovirus 9, 4, 7 – Echovirus 9, 4, 7 – Epstein-Barr virus – Epstein-Barr virus – Coxsackie virus A9 – Coxsackie virus A9

17 Non-infectious Non-infectious – Normal platelets – Normal platelets HSP HSP Coagulation disorders Coagulation disorders Trauma Trauma – Low platelets – Low platelets ITP ITP Leukemia Leukemia

18 PRACTICAL APPROACH TO A CHILD WITH BLEEDING HISTORY PHYSICAL EXAMINATION- PHYSICAL EXAMINATION- > PETICHEAE > PETICHEAE >ECHYMOSES >ECHYMOSES >JOINT BLEED &DEEPSEATED HEMATOMAS >JOINT BLEED &DEEPSEATED HEMATOMAS > HEPATOSPLENOMEGALY > HEPATOSPLENOMEGALY >SIGNIFICANT LYMPHADENOPATHY >SIGNIFICANT LYMPHADENOPATHY > ACTIVE AND PLAYFUL VS. ILL LOOKING > ACTIVE AND PLAYFUL VS. ILL LOOKING > DYSMORPHIC FEATURES > DYSMORPHIC FEATURES > CAFÉ-AU-LAIT SPOTS > CAFÉ-AU-LAIT SPOTS >TELANGIECATIC VESSELS >TELANGIECATIC VESSELS >HEMANGIOMAS >HEMANGIOMAS

19 PRACTICAL APPROACH TO A CHILD WITH BLEEDING HISTORY LABORATORY WORK UP- LABORATORY WORK UP- P.M.D - > C.B.C./PLATELET COUNT > C.B.C./PLATELET COUNT >PERIPHERAL SMEAR- MORPHOLOGY >PERIPHERAL SMEAR- MORPHOLOGY > P.T. [Prothrombin time] > P.T. [Prothrombin time] > a.P.T.T. [ Activated partial thromboplastin time] > a.P.T.T. [ Activated partial thromboplastin time] Hemophilia service -- Hemophilia service -- > T.T. [Thrombin time] > T.T. [Thrombin time] > Bleeding time > Bleeding time >Platelet aggregation studies >Platelet aggregation studies > Factor assay > Factor assay

20 Pandora’s box: coagulation test  The results are as good as the sample is. Standards: Time from sample to test: PT 24 hours,PTT 4 hours. Blood/citrate ratio: 9 :1. Blood/citrate ratio: 9 :1.

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23 Bleeding disorders Platelets– Platelets– Acquired causes much more common Acquired causes much more common Thrombocytopenia more common than Thrombocytopenia more common than functional defects functional defects Inherited disorders – both number &functional defects Inherited disorders – both number &functional defects are extremely rare are extremely rare

24 PLATELETS – NUMBER Acquired causes are most common Acquired causes are most common I.T.P. I.T.P. Infections Infections CONGENITAL THROMBOCYTOPENIAS T.A.R. syndrome T.A.R. syndrome

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26 I.T.P.- I.T.P.- Most have benign &limited course Most have benign &limited course Treatment options- Conservative –wait &watch Conservative –wait &watchAggressive- Steroids Steroids IvIGG IvIGG Rhogam Rhogam Rituximab Rituximab

27 PLATELETS PLATELETS Functional disorders- Functional disorders- Acquired- Aspirin; Uremia Acquired- Aspirin; Uremia Inherited – Inherited – Glanzman’s Glanzman’s Bernard-Soulier Bernard-Soulier Gray platelet syndrome Gray platelet syndrome

28 von Willebrand Disease The most common inherited bleeding disorder The most common inherited bleeding disorder Occurs in 1% of the population Occurs in 1% of the population Less than 10% of patients have bleeding events due to vWD Less than 10% of patients have bleeding events due to vWD

29 Inheritance of Type 1 vWD

30 Functions of vWF Binds to platelet receptor GP Ib and to subendothelial structures such as collagen serving as bridge between platelets and subendothelium in damaged vessels Binds to platelet receptor GP Ib and to subendothelial structures such as collagen serving as bridge between platelets and subendothelium in damaged vessels Acts as bridge between adjacent platelets in vessels with high shear (arterioles) forming small platelet aggregates Acts as bridge between adjacent platelets in vessels with high shear (arterioles) forming small platelet aggregates Binds to circulating factor VIII protecting it and prolonging FVIII t1/2 in the circulation from 2 to 8-12 hours Binds to circulating factor VIII protecting it and prolonging FVIII t1/2 in the circulation from 2 to 8-12 hours

31 Symptoms of vWD Easy bruisability Easy bruisability Epistaxis or gingival bleeding Epistaxis or gingival bleeding Menorrhagia Menorrhagia Post-partum hemorrhage Post-partum hemorrhage Post-surgical bleeding Post-surgical bleeding Bleeding post-dental extraction Bleeding post-dental extraction

32 Classification of vWD Sub types of VW Type 1 Partial quantitative deficiency of vWF Type 2 Qualitative variants of vWF A Absence of HMW vWF multimers A Absence of HMW vWF multimers B Same as 2A and increased affinity for platelet gp Ib B Same as 2A and increased affinity for platelet gp Ib M Abnormal function not caused by absence of HMW multimers M Abnormal function not caused by absence of HMW multimers N Decreased affinity for factor VIII N Decreased affinity for factor VIII Type 3 Complete deficiencey of vWF & Behave as Severe Hemophilia A Hemophilia A

33 Treatment Guidelines in VWD TYPE TYPE 1 2A 2A 2B 2B 2M 2M 2N 2N 3 TREATMENT TREATMENT DDAVP DDAVP DDAVP/FVIII-VWF DDAVP/FVIII-VWF FVIII-VWF FVIII-VWF

34 DDAVP (1-desamino-8-D-arginine vasopressin) Parenteral form: DDAVP (for IV or SC use, 0.3 ug/kg) Parenteral form: DDAVP (for IV or SC use, 0.3 ug/kg) Highly concentrated intranasal spray form: Stimate nasal spray ( ug ) Highly concentrated intranasal spray form: Stimate nasal spray ( ug )

35 Hemophilia Hemophilia

36 Hemophilia Caused by an absence or decreased amount of a procoagulant – Caused by an absence or decreased amount of a procoagulant – VIII -Hemophilia A affects ~ 1:5000 males VIII -Hemophilia A affects ~ 1:5000 males XI -Hemophilia B affects ~ 1:30000 males XI -Hemophilia B affects ~ 1:30000 males XI –Hemophilia C – Rare /Ethnicity XI –Hemophilia C – Rare /Ethnicity

37 Epidemiology Hemophilia A Other Hemophilia B Incidence: Hemophilia A - 1:5,000 Hempohilia B – 1: 30, 000

38 Inheritance

39 Inheritance

40 Woman can have hemophilia Lyonization of the normal X chromosome Lyonization of the normal X chromosome Turner syndrome ( XO) Turner syndrome ( XO) Father with hemophilia/ mom as a carrier Father with hemophilia/ mom as a carrier vW type 2 N ( Normandy) vW type 2 N ( Normandy)

41 HEMOPHILIA SEVERITY LEVELS Severe <1% activity level - Spontaneous bleeds Moderate 1 to 5% activity --Trauma/surgery bleeds Occasional joint bleeds Mild 5 to 30% activity - Major trauma/surgery Rare joint bleeds

42 Factor replacement 1 u/kg raises FVIII levels 2% 1 u/kg raises FVIII levels 2% 1/2 life : 12 hrs 1/2 life : 12 hrs 1 u/kg raises FIX levels 1 % 1 u/kg raises FIX levels 1 % 1/2 life hrs 1/2 life hrs

43 Minor Bleeding Episodes Early joint bleeds Early joint bleeds Soft tissue & muscle bleeds Soft tissue & muscle bleeds Nose & gum bleeding not responding to local measures Nose & gum bleeding not responding to local measures Treatment of minor bleeding episodes Treatment of minor bleeding episodes – % correction –FVIII : 25 units / kg –FIX : 50 units / kg

44 Major Bleeding Episodes Advanced soft tissue & muscle bleeds Advanced soft tissue & muscle bleeds Head & neck injuries Head & neck injuries Gastrointestinal bleeding Gastrointestinal bleeding Advanced joint bleeding Advanced joint bleeding Treatment of major bleeding episodes Treatment of major bleeding episodes 80 – 100 % correction80 – 100 % correction FVIII : 50 units / kgFVIII : 50 units / kg FIX : 100 units / kgFIX : 100 units / kg

45 Current Products Plasma Products: plasma-derived factor VIII concentrate Plasma Products: plasma-derived factor VIII concentrate Porcine Factor: Porcine Factor: –Use was halted due to parvovirus/retrovirus sequences discovered Recombinant products: Recombinant products: –First Generation: Recombinate, Kogenate, Helixate –Second Generation: Kogenate FS, Helixate FS –Third Generation: Advate DDAVP: DDAVP: –Causes release of factor VIII/vWF –Increased factor activity in 30-60” –For mild hemophiliacs and mild bleeding symptoms

46 Replacement therapy: Joint disease Prophylaxis Prophylaxis –Primary –Secondary Intensive infusion therapy Intensive infusion therapy Dose escalation modified prophylaxis Dose escalation modified prophylaxis

47 Clinical Severity

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50 Chronic Joint

51 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 Primary Care Infectious Disease Infectious Disease Genetics Genetics Pharmacy Pharmacy Dental Dental Hepatologist Hepatologist

52 Basis for Comprehensive Care Hematologist Hematologist –Assumes overall care Musculoskeletal Musculoskeletal –Orthopedic Surgeon, Physical therapist Nursing Nursing –Coordination of home/clinic care for rapid treatment at the earliest symptoms suggestive of a bleed Dental Dental Genetic Counseling Genetic Counseling Infectious Disease Infectious Disease Psychosocial Psychosocial –social worker

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

54 The Past…

55 …the promise of achieving your potential Present

56 Made possible by a STRONG & Dedicated Hemophilia parent association and dedicated NJHA staff

57 Thank you Thank you


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