The Student with Hemophilia Ellen White RN BSN

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 TF VIIa Xa Va IIa VIII/v. WF VIIIa TF-Bearing Cell TF VIIa 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): S 61.

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 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 v. WD 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!