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Basics of Bleeding Disorders

Hemophilia & von Willebrand Disease

INTRO

Hemophilia Outreach Center

INTRO

  • Speaker introductions

TODAY'S SCHEDULE

1

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Learning Objectives

Describe what the Hemophilia Outreach Center offers to bleeding disorders & medical community.

Describe normal hemostasis & its 3 major steps

Describe various types of hemophilia, prevalence, genetics, diagnosis, types of bleeds & treatments

Describe von Willebrand Disease, prevalence, genetics, diagnosis, bleeding manifestation & treatment

ABOUT

Hemophilia Outreach Center

  • Federally funded Hemophilia Treatment Center
  • Comprehensive Treatment under one roof

HISTORY

HISTORY

How it all began...

  • Founded over 40 years ago by 2 pediatric nurses
  • Services were initially rendered out of their home, infusing many patients at Peggy's dining room table.
  • Now located: 2060 Bellevue Street in Green Bay, WI

TEAM

Comprehensive Care Team

  • Physicians
  • Nurses
  • Clinical Staff
  • Behavioral Health Specialist
  • Dental Hygienist
  • Financial Coordinator
  • Genetic Counselor
  • Dietitian
  • Pharmacy
  • Physical Therapist
  • Program Coordinator

Ancillary Services

HEMOPHILIA

Factor VIII & Factor IX deficiencies

  • A genetic disorder that is characterized by a deficiency or absence of one of the clotting proteins in the plasma

HEMOPHILIA

  • The result is delayed clotting
  • Patients do not bleed faster, rather they bleed longer.

CLOTTING

SEQUENCE

CLOTTING SEQUENCE

  • Hemostatic System
  • Blood Vessels
  • Platelets
  • Plasma Coagulation System
  • Proteolytic or Fibrinolytic System
  • How Bleeding Stops
  • Vasoconstriction
  • Platelet plug formation
  • Clotting Cascade activated to form fibrin clot

TYPES OF

HEMOPHILIA

TYPES OF HEMOPHILIA

HEMOPHILIA "A" & HEMOPHILIA "B"

  • Hemophilia A
  • Affects about 1:5000 males
  • Carriers- females may be symptomatic

  • Hemophilia B
  • Also referred to as Christmas Disease
  • Affects about 1:20,000 males
  • Carrier- females may be symptomatic

DEGREES OF

SEVERITY

DEGREES OF SEVERITY

Normal, Mild, Moderate, Severe

  • Mild Hemophilia
  • expect few problems with bleeding except during surgery and after severe trauma, bleeds uncommon
  • Moderate Hemophilia
  • bleeding after incidental trauma, ~4-6 bleeds/year
  • Severe Hemophilia
  • factor VIII or IX level-frequent bleeding episodes and spontaneous bleeding, ~1 bleed per week

WOMEN'SCARE

WOMEN'S CARE

CARRIER OR MILD HEMOPHILIA?

Mild Hemophilia

  • expect few problems with bleeding except during surgery and after severe trauma, bleeds uncommon

MENSTRUAL CYCLE

  • Symptomatic Carriers
  • There can be several genetic reasons for why these carriers are symptomatic.
  • Treatment options

BABY

LABOR & DELIVERY

  • HOC RNs will provide a full plan of care for the mother and the baby during and after delivery.
  • Epidurals
  • Third trimester factor level check
  • Vaginal delivers are considered to be safe
  • Post delivery hemorrhaging
  • Instruments to avoid during delivery
  • Forceps
  • Vacuum suction
  • Scalp monitoring

  • Test baby's factor levels
  • Cord blood (Males)

  • Procedures to avoid prior to knowing baby's factor levels
  • Circumcision
  • Heel stick/IM injections

INHERITANCE

  • Hemophilia A & B are X-linked recessive disorders
  • Hemophilia is typically expressed in males and carried by females
  • Severity level is consistent between family members

INHERITANCE OF HEMOPHILIA

  • 30% of cases of severe hemophilia result from spontaneous mutations/no family history
  • When diagnosis of a new patient with mild/moderate/severe disease, test all other at-risk family members regardless of prior history

TYPES OF BLEEDS

  • #1 Joint Bleeds (hemarthrosis), “Target Joint” term used to indicate 3-4 bleeds to same joint in 6 month period, most common target joints are knees, ankles and elbows
  • Muscle hemorrhage
  • Soft Tissue
  • Mucous Membranes (Nosebleeds/Mouth Bleeds)
  • Life Threatening Bleeds-CNS (Head & Spinal Cord)
  • Gastrointestinal
  • Urinary tracked bleed
  • Limb Threatening bleeds

TYPES OF BLEEDS

BLEED DETECTION

ACTIVE JOINT BLEEDS

JOINT BLEEDS

LOOK INSIDE THE KNEE

  • With bleeds the space around the joint fills with blood
  • This causes the synovium to become irritated and bleed again

LOOK INSIDE

  • The body makes enzymes remove blood from the joint
  • These enzymes damage the cartilage
  • After many bleeds, the cartilage wears away

MUSCLE BLEED

Major Hemorrhage

MAJOR HEMORRHAGE

  • This is less common but requires immediate assessment and intervention.
  • #1 cause of death is intracranial hemorrhage
  • Intracranial, spinal cord, airway and ocular.
  • Bleeding into an enclosed space – compartment syndrome
  • Compression of vital organs
  • Potential for life or limb threatening

INHIBITORS/ ACQUIRED HEMOPHILIA

  • People with hemophilia can develop antibodies to factor medications.
  • Time consuming/ Costly
  • Factor products are not interchangeable
  • People without hemophilia can develop acquired hemophilia

INHIBITORS

HISTORY OF TREATING BLEEDS

TREATING BLEEDS

1960’s

-Dr. Judith Graham Pool- factor-rich plasma (cryoprecipitate)

1970’s

Commercial plasma derived factor concentrates

1980's

2010’s

Monoclonal antibodies

Heat treated factor concentrates

(Clotting factor concentrates : Freeze dried powder. ***)

1990’s

New recombinant factors

2020

Gene therapy clinic trials (Hemophilia A & B)

"PROPHY"

  • Preventative treatment is the best treatment
  • Prophy is the standard of care for the hemophilia population
  • Prophy schedule
  • Extended half-life v. standard half-life
  • Primary v. secondary
  • Insurance approval/denial

PROPHYLAXIS

BAD BLOOD

"BAD BLOOD"

Therapy & Treatment

  • Early and appropriate treatment of each bleeding episode is imperative
  • IV Factor Replacement
  • DDAVP IV/Stimate Nasal Spray for Hemophilia A only
  • RICE-Rest, Ice, Compression and Elevation.
  • Amicar for mucosal bleeding. Avoid for GI/GU bleeds.

Replacement of the absent or deficient

clotting protein is imperative.

THERAPY & TREATMENT

FACTOR & VON WILLEBRAND PRODUCT

  • Factor VIII {Recombinant}
  • ADVATE*
  • Helixate
  • Kogenate
  • KOVALTRY
  • Novoeight
  • NUWIQ
  • RECOMBINATE
  • XYNTHA

PRODUCTS

  • Von Willebrand Factor {Recombinant}
  • VONVENDI*
  • Desmopressin Acetate
  • Stimate
  • Factor IX {Human}
  • AlphaNine SD
  • Mononine
  • Factor IX {Recombinant}
  • BeneFix*
  • IXINITY
  • RIXUBIS
  • Factor XI {Recombinant}, Long-acting
  • ALPROLIX*
  • IDELVION

  • Factor VIII {Recombinant}, Long-acting
  • ADYNOVATE
  • AFSTYLA
  • ELOCTATE*
  • Factor VIII {human}
  • HEMOFIL M
  • KOATE-DVI
  • MONOCLATE-P
  • Factor VIII/ von Willebrand Factor Complex {human}
  • ALPHANATE
  • HUMATE-P*
  • WILATE*

Factor & von Willebrand

Products (Continued)

MORE PRODUCTS

  • Anti-Inhibitor Coagulation Complex
  • FEIBA
  • Coagulation Factor VIIa {Recombinant}
  • NovoSeven RT*
  • SevenFact
  • Antihemophilic Factor {Recombinant} Porcine Sequence
  • OBIZUR*
  • Factor IX Complex
  • BEBULINE
  • PROFILNINE
  • Prothrombin Complex Concentrate
  • KCENTRA
  • Coagulation Factor X {Human}
  • COAGADEX
  • Factor XIII Concentrate {Human}
  • Corifact
  • Coagulation Factor XIII A-Subunit {Recombinant}
  • Tretten
  • Fibrinogen Concentrate {Human}
  • RiaSTAP
  • Non-Factor Replacement
  • Hemlibra*

FUNDAMENTAL PRINCIPLES OF HEMOPHILIA TREATMENT

PRINCIPLES OF TREATMENT

  • Treat bleeds early to prevent acute and chronic complications.
  • Believe the child/patient who thinks he/she is bleeding.
  • Recognize that various somatic complains may be due to hemorrhage.
  • Communicate with the local hemophilia treatment center

SCENARIOS

  • Patient presents with
  • Pain in left ankle
  • Played soccer yesterday
  • What other signs of bleeding would you look for?

SCENARIOS

  • Patient who is a carrier of hemophilia A severe
  • has a factor level of 11%
  • calls saying she is pregnant
  • What questions would you ask this patient?
  • What do you think the plan of care should be?
  • Patient's mom calls
  • Patient has hemophilia A severe
  • Patient having more bleeds than usual
  • What other questions would you ask?
  • What do you think is going on with the patient?

QUESTIONS ABOUT HEMOPHILIA?

QUESTIONS

VON WILLEBRAND DISEASE

VON WILLEBRAND DISEASE

TYPES

Most common, presents in 75%-80% of people with vWD, mild symptoms

VWF protein does not work properly

Present in 15%-20% of patients with vWD; exhibits mores severe bleeding (Type 2A, 2B, 2M, & 2N)

Occurs in 1 out of 1,000,000

Most severe form, < 1% of patients with vWD, resulting no vWF

Factor VIII levels also very low so patients may have symptoms similar to mild hemophilia

TYPE I

TYPE II

TYPE III

CLASSIFICATION OF vWD

TYPE I

  • Partial quantitative VWF deficiency
  • Prevalence in vWD- 70%-80%
  • Autosomal dominant- 50% chance of inheritance

TYPE II

  • Qualitative VWF defect
  • Four subtypes: 2A, 2B, 2M, 2N
  • Prevelance in vWD- 20%
  • Autosomal dominant- 50% chance of inheritance

TYPE III

  • Virtually complete VWF deficiency & decreased factor VIII
  • Prevalence in vWD < 5%
  • Two Type I's pass on their genetic mutations

vWD Type II(s)

TYPE II

  • Qualitative VWF defect
  • Four subtypes: 2A, 2B, 2M, 2N
  • Prevelance in vWD- 20%
  • Autosomal dominant- 50% chance of inheritance

VWD Type 2A

Decreased platelet binding. Loss of HMWM

VWD Type 2N

Decrease FVIII binding

Low FVIII levels

VWD Type 2B

Increased plataelet binding

Thrombocytopenia

VWD Type 2M

Decreased platelet binding

Normal multimers

Ng C, et al. Blood. 2015;125:2029-37 .

vWD CLINICAL PRESENTATION

TREAMENT OF vWD

  • DDAVP IVPB or Stimate Nasal Spray
  • Used to treat patients with Hemophilia A (Mild) and VWD Type 1 & sometimes 2A.
  • Not recommended in the perinatal period. Oral fluid restrictions for 24 hours.
  • Higher Concentrate
  • VWF Replacement:
  • Plasma derived VWF/Factor VIII concentrates: Humate-P, Alphanate, Wilate
  • Recombinant VWF: VONVENDI.
  • Adjunctive therapies
  • Hormonal control of menstruation (OCP, depoprovera, hormone IUD)
  • Antifibrinolytics (Amicar and Lysteda-(tranexamic acid)

MUCOSAL BLEEDING

  • Epistaxis/excessive nosebleeds
  • not always externally visible
  • Gingival bleeding
  • Menorrhagia
  • GI bleeding

EASY OR EXCESSIVE BRUISING

POST-PROCEDURE BLEEDING

  • Bleeding following tooth extraction
  • Postoperative bleeding
  • Postpartum hemorrhage

TREATMENT OF VWD

TREAMENT OF vWD

DDAVP IVPB or Stimate Nasal Spray

Used to treat patients with Hemophilia A (Mild) and VWD Type 1 & sometimes 2A.

Not recommended in the perinatal period. Oral fluid restrictions for 24 hours.

Higher Concentrate

VWF Replacement:

Plasma derived VWF/Factor VIII concentrates: Humate-P, Alphanate, Wilate

Recombinant VWF: VONVENDI.

Adjunctive therapies

Hormonal control of menstruation (OCP, depoprovera, hormone IUD)

Antifibrinolytics (Amicar and Lysteda-(tranexamic acid)

ANTIFIBRINOLYTIC AGENTS

  • Inhibits plasminogen activation thereby preventing normal clot destruction. Ensures the stability of the clot and allows for better control of the bleeding.

  • Amicar-(Aminocaproic acid):
  • Can be administered orally to persons with nasal, oral and uterine bleeding
  • Oral (elixer or tablet)
  • Aminocaproic Acid IV Solution
  • Amicar is commonly used following tooth extraction and oral surgery to prevent clot lysis.

  • Lysteda-(tranexamic acid)
  • Used to treat heavy menstrual bleeding

SCENARIOS

  • Patient calls saying
  • nose bleeding
  • What other questions would you ask this patient?
  • What would be your next steps?
  • Patient calls saying
  • heavy period
  • tiredness
  • craving ice chips
  • What other questions would you ask this patient?
  • What would be your next steps?
  • Patient calls saying
  • dark stool (x3)
  • gurgling stomach
  • lightheaded
  • What kind of bleed do you think this patient is having?
  • What other questions would you ask this patient?
  • What would be your next steps?

HEMOPHILIA OUTREACH CENTER

HOC

  • Please do not hesitate to call if there are ANY questions.
  • Phone 1-920-965-0606
  • You may fax reports to the Center at 1-920-965-0607
  • We are available 24 hours/7 days a week.

ROLE OF AN HTC

  • State-of-the-art medical treatment for persons with a bleeding disorder through their life span
  • Education
  • Research
  • Outreach
  • Model of comprehensive care for chronic disease

ROLE OF AN HTC

PERCEPTION OF AN HTC

PROGRAMS

SOURCES

CITATIONS

  • Banks, RN, D. (2012). Introduction to Bleeding Disorders. Retrieved Jan. & Feb., 2018, from https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-1-Introduction-to-Bleeding-Disorders.pdf
  • Hemophilia A. National Hemophilia Foundation website. http://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A. Accessed October 14, 2016.
  • Fast facts. National Hemophilia Foundation website. http://www.hemophilia.org/About-Us/Fast-Facts. Accessed October 14, 2016.
  • McDaniel, RN, M. (2013). Treatment of Hemophilia A and B. Retrieved November, 2016, from https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-6-Treatment-of-Hemophilia-A-B.pdf
  • N. (2010, April 1). How Hemophilia is inherited [Photograph]. The Royal College of Midwives, N/A. Infographic
  • White, RN, MSN, E., Christie, RN, ACRN, B., & Orto, RN, BSN, MPA, C. (2013). Common Bleeding Episodes. Retrieved November, 2016, from https://www.hemophilia.org/sites/default/files/document/files/Nurses-Guide-Chapter-4-Common-Bleeding-Episodes.pdf
  • W. (n/a). Introduction to Hemophilia [Degrees of Severity ]. Retrieved February, 2018, from http://www.hemophiliabangalore.org/images/gallery/part.1.4.html Infographic
  • N. (2016, July 11). Hemophilia. Retrieved February 22, 2018, from https://www.cdc.gov/ncbddd/hemophilia/data.html
  • N. (2011, September 13). Hemophilia. Retrieved November 23, 2017, from https://www.cdc.gov/ncbddd/hemophilia/diagnosis.html
  • W. (n.d.). Symptoms and diagnosis. Retrieved November 23, 2017, from https://www.wfh.org/en/page.aspx?pid=640
  • N. (n.d.). How Hemophilia Affects Blood Clotting [Normal Clotting Process v. Clotting with Hemophilia]. Retrieved November 23, 2017, from http://brokenblood.co.uk/haemophilia-explained/how-haemophilia-affects-blood-clotting/
  • NuFactor Specialty Pharmacy. (2016). Coagulation Products Reference [Brochure]. Temecula, CA: Author. Reflects prescribing information for products as of July 2016, BR149
  • National Hemophilia Foundation. (2016, July). The Basics of Hemophilia. Lecture presented at Nursing Working Group in NHF Annual Meeting .
  • Ness, M. (Director). (2010). Bad Blood: A Cautionary Tale [Video file]. United States: Necessary Films. Retrieved from http://www.BadBloodDocumentary.com
  • Lysteda. (2012, September). Retrieved February 23, 2018, from http://www.lysteda.com/
  • World Hemophilia Foundation. (2008, April). Protocols for the Treatment of Hemophilia & von Willebrand Disease [PDF]. Georgia: WFH.
  • PRODUCTS. (2004). Retrieved February 23, 2018, from http://www.itxm.org/tmu/tmu2004/issue2004-1.htm
  • CSL Behring. (2010, December). CSL Behring Stimate Package Insert [PDF]. FDA.
  • Sanofi-aventis U.S. LLC. (2007). DDAVP Nasal Spray Package Insert [PDF]. Bridgewater: FDA.
  • Zappa, RN , S. C., Lacasse, RN, L., Jacobson, RN, R., Purcell, RN, S., Wulff, RN, K., Lillicrap, MD, FRCPC, D., & Torres, MD, M. (2006). Emergency Care for Patients with von Willebrand Disease [PDF]. ZLB Behring.
  • Nati

HAPPY HEMOSTASIS

Remember to consult the local HTC

QUESTIONS

Q&A

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