The Student with Hemophilia

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The Student with Hemophilia

• Ellen White RN BSN
• Yvette Menga LSW

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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)

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Clinical Characteristics

• Internal bleeding into joints, muscles and major
  organs
• Depending on the factor level bleeding can be
  spontaneous or caused by trauma
• Bleeds longer not faster
• Soft tissue bleeds
• Hematomas

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Hemophilia Treatment Center Team Members

• Patient / Family
• Hematologist
• Pediatric
• Adult
• Nurse
• Social Worker
• Physical Therapist

• Orthopedist
• Primary Care
• Genetics
• Home Care Company
• Dental

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Role of Hemophilia Treatment Center

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

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Services

• Medical
• Nursing
• Psychosocial
• Physical therapy
• Dental services
• Diagnostic laboratory services
• Genetic counseling and testing

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Types of Bleeding Disorders

• Hemophilia A (factor VIII deficiency)
• Hemophilia B (factor IX deficiency)

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Incidence

• Hemophilia A 15000 male births
• Hemophilia B 130,000 male births

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School Issues

• Frequent absences
• Hard to catch up
• Decreased peer support
• Activity restrictions
• No contact sports
• Immobilization during bleeding episodes
• Pain
• Chronic/acute

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How Blood Clots

• Blood vessels
• Platelets
• Plasma coagulation system
• Proteolytic or Fibrinolytic system

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Hemostasis

• Vasoconstriction
• Platelet plug formation
• Clotting cascade activated to form fibrin clot

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Coagulation Cascade
II
X
VIII/vWF
TF
VIIa
Xa
IIa
Va
VIIIa
TF-Bearing Cell
TF
V
Va
VIIa
IX
Platelet
II
IXa
X
IIa
Xa
VIIIa
IXa
Va
Activated Platelet
VIIa
IXa
Va
IIa
Xa
VIIIa
II
IX
X
Hoffman et al. Blood Coagul Fibrinolysis
19989(suppl 1)S61.
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Bleeding in Hemophilia

• Vasoconstriction
• Platelet plug formation
• The clotting cascade is not activated a fibrin
  clot does not form
• Bleeding will continue

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Inheritance

• Hemophilia A and B are X-linked recessive
  disorders
• 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
• Affects all races and ethnic groups equally
• Moderate mild deficiencies under-diagnosed

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Genetics
XX
XY

• Affected males
• All daughters are carriers
• No sons are affected
• Female carrier
• 50 risk for carrier daughter
• 50 risk for affected son

XY
XX
XY
XX
XY
XX
XY
XX
XX
XY
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Clinical Aspect
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Type and Severity

• Normal factor VIII or IX level 50-150
• Mild hemophilia
• factor VIII or IX level 5-50
• Moderate hemophilia
• factor VIII or IX level 1-5
• Severe hemophilia
• factor VIII or IX level lt1

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First Bleed/Diagnosis

• Mild
• Often has bleeds at an earlier age but not
  identified till later in life, 3 to 14 years or
  older
• Moderate
• usually before 2 years
• Severe
• within first year

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Bleeding Pattern Mild

• Once a year
• Joint and muscle bleed unusual except with
  significant trauma
• Trauma-induced or contact sports significant
  hematomas
• Internal deep bleeding only with significant
  trauma
• Post op bleeding

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Carriers

• Carriers may have low factor levels
• Carriers may experience bleeding symptoms seen in
  mild or moderate deficient states
• Treat carriers as potential bleeders

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Bleeding Pattern Moderate

• Bleeds once a month
• Minor trauma causes joint and muscle bleed
• may have target joints
• Post surgical wound hematoma or oozing

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Bleeding Pattern Severe

• Bleeds once a week
• Spontaneous joint and muscle bleed
• Target joints

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Types of Bleeds Treatment and Management
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Types of Bleeds

• Joint bleeding - hemarthrosis
• Muscle hemorrhage
• Soft tissue
• Life threatening-bleeding
• Other common bleeding

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Joint or Muscle Bleeding

• Symptoms
• Tingling or bubbling sensation
• Stiffness
• Warmth
• Pain
• Unusual limb position

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Treatment of Hemophilia

• Replacement of missing clotting protein
  Intravenous infusion
• On demand
• Prophylaxis Primary/Secondary

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Factor VIII Concentrate

• Intravenous infusion
• IV push
• 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

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DDAVP (Desmopressin acetate)

• Synthetic vasopressin
• Method of action -
• release of stores from endothelial cells raising
  factor VIII and vWD serum levels
• Administration -
• Intravenous
• Subcutaneously
• Nasally (Stimate)

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Stimate

• Dosing
• Every 24-48 hours prn not to be used more than
  three days in a row
• lt50 kg. body weight - 1 spray (150 mcg.)
• gt50 kg. body weight - 2 sprays (300 mcg.)

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Factor IX Concentrate

• Intravenous infusion
• IV push
• 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

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Minor Bleeding Episodes

• Early joint bleeds
• Soft tissue muscle bleeds
• Nose gum bleeding not responding to local
  measures
• Treatment of minor bleeding episodes
• 40 - 50 correction
• FVIII 20 - 25 units / kg
• FIX 40 - 50 units / kg

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Major Bleeding Episodes

• Head neck injuries
• Advanced soft tissue muscle bleeds
• Abdominal bleeding
• Advanced joint bleeding
• Treatment of major bleeding episodes
• 100 correction
• FVIII 50 units / kg
• FIX 100 units / kg

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Primary Prophylaxis

• Scheduled infusion therapy at an early age before
  bleeding has regularly occurred to convert
  patient from severe deficient state to moderate
  deficient
• Goal suppression of spontaneous bleeding
  episodes
• Frequency 2 to 3 times weekly to keep trough
  factor VIII or IX levels at 2-3
• Use of IVAD necessary in some patients

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Secondary Prophylaxis

• Scheduled infusion therapy at any age after
  bleeding has regularly occurred or after injury
  to convert patient from severe deficient state to
  moderate deficient
• Prior to sports activity
• Goal suppression of spontaneous bleeding
  episodes or rebleeding
• Frequency 2 to 3 times weekly to keep trough
  factor VIII or IX levels at 2-3
• Use of IVAD necessary in some patients

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Morbidity of Chronic Disease

• School absenteeism increased in children with
  severe Hemophilia compared to normal population
• Difficulty attending to task if in pain
• Ability to achieve potential and find a
  productive appropriate place in work force
• Number of bleeding episodes impacts ability to
  achieve potential
• Prophylaxis decrease morbidity of chronic disease

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Adjunctive Therapy

• RICE
• Rest /Replacement
• Ice/Immobilization
• Compression
• Elevation
• Antifibrinolytic Agents
• Amicar (aminocaproic acid)
• Used for mucocutaneous bleeding
• Dosing 50 mg./kg. q. 6 hours po

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Bleeding Episodes
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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

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Joint Bleeds

• Most common bleeding manifestation
• Most common joint Knees, Ankles, Elbows
• Collection of blood in joint space may cause
  joint to feel hot
• Initial symptoms of tingling or bubbling
  sensation
• Early sign reluctance to move, swelling and
  joint pain as bleeding progresses
• Affected joint held in flexed position
• Usually no visible cutaneous bruising
• Treat with replacement factor, rest,
  ice,compression and immobilization

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Advanced Joint Bleed
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Complications Joint Bleeds

• Flexion contractures
• Joint arthritis / arthropathy
• Chronic pain
• Muscle atrophy

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X-Ray of severe joint damage from recurrent
hemarthrosis
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Muscle Bleeding

• Second common bleeding manifestation
• Bleeding leg, thigh, calf, forearm, and groin
  create pressure on nerves
• Early sign reluctance to move, swelling and pain
  as bleeding progresses
• Affected extremity held in flexed position
• Usually no visible cutaneous bruising
• Treat with replacement factor, rest, ice ,
  compression and immobilization

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Advanced joint and muscle bleed
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Complications Muscle Bleeds

• Compartment syndrome
• Neurologic impairment

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Other Common Bleeds

• Bruises
• Superficial bleeding into soft tissues
• Usually raised bruises or hematomas
• Scrapes, minor cuts and/or Lacerations
• Mucous-membrane bleeding
• Bleeding from tissues of mouth or nose
• Can cause nausea and vomiting if blood swallowed
• Blood loss can be insidious
• Bleeding with loss of primary teeth usually not a
  problem
• Hematuria

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School Issues
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Sports

• Category I Can participate safely
• Category II Benefits out weigh risk
• Category III Risk outweigh the benefits

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ResponsibilitiesStudent

• Learns to Communicate
• Reports bleeding episodes
• Completes assignments on time
• Makes the same effort as students without
  Hemophilia

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Responsibility Parent

• Communicate child's condition, activity and
  treatment
• Obtains makeup work
• Assist the child in maintaining a positive
  healthy attitude toward school
• Provide the school nurse with factor treatment
  plan

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Responsibility School

• Monitor school performance and remain alert for
  changes in motivation, personality, or
  performance and inform parents of these changes
• Communicate to parents/child observable signs of
  a bleed
• Stress the importance of completing assignments
  on time
• Promote ability and success not inability
• Respect privacy and confidentiality

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• Individualized Healthcare Plan (IHP)

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School Issues

• Acute management of bleed
• Factor replacement
• Joint support
• RICE
• Pain medications
• Absences
• Activity

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IHP Nursing Diagnosis

• Potential for injury related to factor deficiency
• Potential for alteration in comfort
• Potential for alteration in mobility
• Knowledge deficit related to disease/management
• Potential for noncompliance
• Potential for alteration in student role

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IHP Goals

• Prevent injuries/safety measures
• Not always able to prevent bleeds
• Develop emergency plan
• Comply with medical treatment
• Prevention Treatment
• Good decision making
• Good attendance
• Maximum participation

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IHP Nursing Interventions

• The Nurse will
• Keep accurate records
• Discuss bleeding prevention with staff
• Inservices as needed with HTC
• Provide/coordinate health education opportunities
• Choose and implement motivators to compliance
• Remove barriers
• Monitor attendance patterns/academic performance

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• Environmental assessment to prevent injury
• Safety measures
• Emergency plans for bleeding episodes at school
• Adequate knowledge of medications and side
  effects
• Appropriate authorizations
• Obtain needed equipment/supplies

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IHP Plan Outcomes

• The student will
• Describe a bleed and treatment
• Identify healthy lifestyle decisions
• Take appropriate safety measures/protective
  equipment
• Maintain good attendance
• Be a normal kid!

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