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Hemophilia and Bleeding Disorders

August 16, 2018

Presented by

Trinna Bloomquist, RN, BSN Personal Health Partner

Mary Bridge Children’s Hematology/Oncology

Content provided in partnership with:Barbara Corwin Oldenburger, RN, BSN

Bloodworks NW

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Quick view Hemostasis:1. Injury to blood vessel2. Injured vessel constriction3. Tissue factor activates platelets4. Von Willebrand protein binds them

together and to collagen5. Clotting Cascade to convert fibrinogen

to fibrin, a mesh or “band aid”6. Vessel and tissue heals, pulls together7. Clot torn down by fibrinolytic enzymes

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Von Willebrand Disease: insufficient or ineffective Von Willebrand factor

Hemophilia A: Missing or insufficient quantity of Factor VIII protein

Hemophilia B: Missing or insufficient quantity of Factor IX protein

When something goes wrong….

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Incidence of Hemophilia:

• 1 in 5000 Males has Hemophilia A

• 1 in 10,000 Males has Hemophilia B

• Inherited, X linked recessive disorder

• Expressed in males carried in females

• Severity level is consistent between family members

• Affects all races/ethnic groups equally

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Incidence of Von Willebrand

1/100 men and women effected equally (CDC)

Type 1: most common (85%); not enough VW

Type 2 functional issue with VW protein

Type 3 most severe, little or no VWF, causes secondary factor VIII deficiency; incidence 1/500,000-1,000,000

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Von Willebrand cont.

Interesting fine points: • Difficulty in testing: VWf an acute phase reactant-

distress during lab draws, crying, pain, fear, stress etc.

• Effected by hormone fluctuations menstrual cycle

• By third trimester production of factor VIII and VWftriples in production and falls back to previous baseline levels within hours after delivery

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Hemophilia• Severe: activity level is less than 1% at risk for

spontaneous bleeding: ICH, joint bleeds, muscle bleeds

• Moderate: factor activity level is less than 1-5% bleeds with injury or invasive procedures

• Mild: factor VIII or Factor IX activity level is 6-30% bleeds with injury or invasive procedures

AS COMPARED TO NORMAL LEVELS 50-150%

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Most people have 100% of the ingredients in their clotting system that lets them make the band-aids they need.

“Hemophilia A is like missing the flour, Hemophilia B is like missing the sugar.”

Either way, you’re missing the ingredients to make a strong “band aid”

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What Bleeding Looks Like in Kids with Hemophilia • Deep bleeding into joints and muscles• Can’t see it until late stage of bleeding• Bruises can look impressive but not the big worry• Classic hemophilia bruise will have a knot in the center• Joints will become warm and swollen• The sooner a child is treated, normalizing their factor activity

levels, the sooner the bleed is cleaned up and less overall factor will be required in the long run

• A boy with hemophilia doesn’t bleed faster than other kids, but he bleeds longer

• Effects of repetitive bleeding into joint space-hemarthrosis

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What Bleeding Looks Like in Kids with Von Willebrand Disease

• MUCOTANEOUS BLEEDING

• Nosebleeds

• Mouth bleeds

• Trouble with even minor surgeries

• Girls: menorrhagia

• Severity depends on type of Von Willebrand

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Joint Bleeds• Most Common Bleed in Hemophilia• Knees, Hips, Ankles, Shoulders, Elbows• May be caused by trauma or can be spontaneous

in severe hemophilia• No early visible signs (i.e. no bruising)

• Tingling, bubbling warm sensation• Pain: Limping, favoring• Late signs: swelling, firm, flexed• R.I.C.E. Ice Ice Ice Ice Ice Ice…• Factor Infusion as soon as possible• Synovium damage

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Muscle Bleeds• Second most common bleeding

issue in hemophilia

• Large muscles can = blood volume loss

• Compartment Syndrome

• Contractures

• Goal: raise FACTOR level to normal 100% and not let it fall below 50%

• R.I.C.E. Ice Ice Ice Ice Ice Ice…

• Several days of treatment, can take much longer than a joint bleed to heal

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Minor Injuries

Bruising

Mouth bleeds

Nosebleeds

Scrapes

First aid

Potential Emergent

Head Injuries

Eye Injury

Airway Trauma

GI Bleed

Iliospoas Bleed

Other uncontrolled bleeding (i.e. menorrhagia etc)

Other Bleeding

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1. If a child is unconscious, call 9-1-1 as you would for any other student.

2. Call Parents.3. Call Mary Bridge Children’s Hematology Clinic at

253-403-3481 M-F, 8am-4pm.

Remember, a boy with hemophilia is not going to bleed faster than other children, but longer.

Call to Triage Bleeding Issues:

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Factor InfusionInfusion Skills:• Port-a-cath training of parents • PIV skills kids ages 7 and up are

taught venous access • Camp IVY

Dose is based on assays +/- 10%

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Sports and Play: kids need to be kids

• Importance of Physical Activity

• Strong muscles protect joints

• Social

• No contact sports

• Prophylaxis before

• Ice

• Learning to self infuse

• Medical Alert

• Education with coaches, communication with parents

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Hemophilia Treatment Center (HTC) of Washington

• Washington Center for Bleeding Disorders• BloodworksNW Pharmacy• Mary Bridge Children’s Health Center• Seattle Children’s Medical Center• Sacred Heart Medical Center• Research• Outreach• Camp• 340B Comprehensive care for rare bleeding disorders

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Hemophilia at School: A regular kid with a bleeding disorder

What do school personnel need to know most?

Blood disorders are treatable and most bleeding problems preventable. The key is communication with the child and parent. Though a bump may seem minor, the internal bleed can show up later. It’s important to start treatment early on and report all school incidents to parents. Always believe a child when he says “he has a bleed”.

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What is Hemophilia?

Hemophilia is a disorder of the clotting system mostly affecting boys. They lack a protein required to stabilize a blood clot. Kids with hemophilia don’t bleed faster, they bleed longer. There are two main types of hemophilia based on the protein that is either missing or low, either clotting protein factor 8 (Hemophilia A) or clotting protein factor 9 (Hemophilia B). A lack of either one of these proteins causes hemophilia. Some people with hemophilia make a little bit of the clotting factor, some make none. The symptoms and severity of hemophilia depend on how much clotting factor is missing.

Hemophilia at School: A regular kid with a bleeding disorder

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Are there any activity restrictions for this child?

It is every child’s job to explore, test their strength, socialize through play and organized activities, and simply be a child. We want kids with bleeding disorders to be healthy and active which means pursuing all kinds of sports and activities. We understand that there is no way to ensure that a child won’t ever get hurt. However, some sports are just not worth the risks that they bring. For example, intentionally high impact contact sports such as contact football, wrestling, hockey, lacrosse, are advised against since the risks outweigh the benefits.

Hemophilia at School: A regular kid with a bleeding disorder

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Common first aid needsMost children with bleeding disorders respond to general first aid like any other child would. Treat minor scrapes and bruises like you would any other child. R.I.C.E. = Rest Ice Compression Elevation, is advised. Do not apply warm packs to a child with bruising or warm drink to child with a mouth bleed. Early intervention is very important:

• Rest the injured area. If moving the injured area causes pain, this is the body's way of saying stop. Rest the affected area. Do not use or bear weight (such as standing or walking).

• Ice packs to the injured area will help to prevent or reduce swelling. Swelling causes more pain and can slow healing. Apply a cloth-covered ice pack to the injured area for no more than 20 minutes at a time, 4 to 8 times a day.

• Compression (i.e. use of an ace wrap) also helps to prevent or reduce swelling. Wrap the injured area with an ace bandage snuggly but not tight. It should not hurt or throb. Fingers or toes beyond the bandage should remain pink and not become "tingly." The ace wrap should be taken off every 4 hours and reapplied.

• Elevation means raising the injured area above the level of the heart. The affected part should be elevated so it is 12 inches above the heart, to help reduce swelling.

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Problem: Recommendation

Bruising R.I.C.E. May not need intervention if not painful. Call parent if hematoma is forming or if bruising affects movement of extremity

Nose bleeds Wear gloves. Tilt head forward, pinch nose 10-15 minutes. Call parent if heavy bleeding does not stop after 15 minutes

Cuts and scrapes Wear gloves. Clean with soap/water as per school protocol

Mouth bleeds Wear gloves. Apply direct pressure with gauze. Tongue cuts can be more serious. Give cold items like ice or popsicle. Call parent if bleeding does not stop.

Menorrhagia (painful menstruation) Girls should be excused from PE class temporarily as necessary. Contact parent if necessary.

Head injury Call 911 for loss of consciousness, nausea, vomiting, severe pain, blurred vision or sleepiness. Notify parent immediately of any

head trauma.

Throat neck injury Call 911 for any breathing trouble due to trauma to neck. Call parent right away for any trauma to throat or neck injury. Internal bleeds to neck area can be serious.

Stomach/abdominal injury Call parent. Watch for skin color changes, rigidity of the abdomen, increased pulse, decreased blood pressure.

Fever If a child has a central line, call parent immediately for ANY fever.

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CENTRAL LINE: YES ___ No ____ Type_______________(A central line is an IV catheter placed into an arm or chest used to give medicines.)

Hemophilia Factor replacement can only be administered by a trained persons. Due to complexity of mixing and administering, specific requirements for storage, expiration time of medication, and extreme cost of the medication, factor should not be stored at school, even for the emergency 3 day supply. If factor is needed, the family should be notified and will determine if 911 should be called to transport the patient or if it can wait until the family can arrive with factor.

! CALL 911 FOR BREATHING TROUBLE OR LOSS OF CONSCIOUSNESS !

Call parents or emergency contacts right away when the student has symptoms such as:

1. Unable to stop bleeding2. Fever over 101.5 F3. Any trauma to head4. If child is complaining of any pain that could indicate

bleeding5. Other:

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Signs and Symptoms of bleeding:A. Child reports “tingling”, warmth, swelling or guarding B. Arm or leg held in an abnormal positionC. Obvious signs of discomfort or painD. Area of bleed is warm to touchE. Swelling (a late sign of a bleed)F. Firmness and tenderness on palpationG. Restriction of all range of motion indicates bleeding within a

joint

Signs and symptoms of infection of a central line:A. Fever greater than 101.5 F B. Redness, swelling, pain or draining of port (central line) site

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