The Student with Hemophilia
Ellen White RN BSN
Yvette Menga LSW
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)
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
Hemophilia Treatment Center Team
Members
Patient / Family
Orthopedist
Hematologist
Primary Care
Pediatric
Adult
Nurse
Social Worker
Physical Therapist
Genetics
Home Care
Company
Dental
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
Services
Medical
Nursing
Psychosocial
Physical therapy
Dental services
Diagnostic laboratory services
Genetic counseling and testing
Types of Bleeding Disorders
Hemophilia A (factor VIII deficiency)
Hemophilia B (factor IX deficiency)
Incidence
Hemophilia A: 1:5000 male births
Hemophilia B: 1:30,000 male births
School Issues
Frequent absences
– Hard to catch up
– Decreased peer support
Activity restrictions
– No contact sports
– Immobilization during bleeding episodes
Pain
– Chronic/acute
How Blood Clots
Blood vessels
Platelets
Plasma coagulation system
Proteolytic or Fibrinolytic system
Hemostasis
Vasoconstriction
Platelet plug formation
Clotting cascade activated to form fibrin
clot
Coagulation Cascade
II
X
VIII/vWF
TF VIIa
Xa
Va
IIa
VIIIa
TF-Bearing Cell
TF
VIIa
IXa
IX
VIIIa
Va
Platelet
II
X
IXa
VIIa
V
IX
Xa
Activated Platelet
IXa
VIIIa
Xa
X
IIa
Va
Va
II
IIa
Hoffman et al. Blood Coagul Fibrinolysis 1998;9(suppl 1):S61.
Bleeding in Hemophilia
Vasoconstriction
Platelet plug formation
The clotting cascade is not activated a
fibrin clot does not form
Bleeding will continue
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
Genetics
Affected males
– All daughters are carriers
– No sons are affected
Female carrier
– 50% risk for carrier
daughter
– 50% risk for affected son
Clinical Aspect
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 <1%
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
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
Carriers
Carriers may have low factor levels
Carriers may experience bleeding
symptoms seen in mild or moderate
deficient states
Treat carriers as potential bleeders
Bleeding Pattern Moderate
Bleeds once a month
Minor trauma causes joint and muscle
bleed
may have target joints
Post surgical: wound hematoma or oozing
Bleeding Pattern Severe
Bleeds once a week
Spontaneous joint and muscle bleed
“Target joints”
Types of Bleeds Treatment and
Management
Types of Bleeds
Joint bleeding - hemarthrosis
Muscle hemorrhage
Soft tissue
Life threatening-bleeding
Other common bleeding
Joint or Muscle Bleeding
Symptoms
– Tingling or bubbling sensation
– Stiffness
– Warmth
– Pain
– Unusual limb position
Treatment of Hemophilia
Replacement of missing clotting protein
Intravenous infusion
– On demand
– Prophylaxis Primary/Secondary
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 %
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)
Stimate ®
Dosing
– Every 24-48 hours prn not to be used
more than three days in a row
– <50 kg. body weight - 1 spray (150
mcg.)
– >50 kg. body weight - 2 sprays (300
mcg.)
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%
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
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
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
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
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
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
Bleeding Episodes
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
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
Advanced Joint Bleed
Complications Joint Bleeds
Flexion contractures
Joint arthritis / arthropathy
Chronic pain
Muscle atrophy
X-Ray of severe joint damage from recurrent
hemarthrosis
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
Advanced joint and muscle bleed
Complications Muscle Bleeds
Compartment syndrome
Neurologic impairment
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
School Issues
Sports
Category I: Can participate safely
Category II: Benefits out weigh risk
Category III: Risk outweigh the benefits
Responsibilities:Student
Learns to Communicate
Reports bleeding episodes
Completes assignments on time
Makes the same effort as students without
Hemophilia
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
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
Individualized Healthcare Plan (IHP)
School Issues
Acute management of bleed
– Factor replacement
– Joint support
– RICE
– Pain medications
– Absences
– Activity
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
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
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
– 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
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!
…