MANAGEMENT OF BLEEDS

Nairobi, Kenya

June 24, 2013

OBJECTIVES

• List the general principles of treatment

• Identify signs and symptoms of bleeds

• Discuss the management and treatment of each type of bleeding episode

• Describe emergencies in hemophilia and their management

• Understand the causes of synovitis and management options

• Explore complications of bleeds

GENERAL PRINCIPLES OF TREATMENT

• Main goal is to prevent and treat bleeding with the missing clotting factor

• Whenever possible, treat specific factor deficiency with specific factor concentrate

• PWH are best managed in a comprehensive care setting

• Bleeds can occur at different sites and each is managed in a particular way

• Acute bleeds should be treated as quickly as possible, preferably within two hours. If in doubt, treat!

• All severe bleeds should be treated immediately, even before diagnostic assessment is complete

GENERAL PRINCIPLES OF TREATMENT (CONT’D)

• For large bleeds, monitor hemoglobin and transfuse if necessary

• Patients should carry easily accessible medical ID to facilitate treatment in an emergency

• Treat veins with care

• Adjunctive therapies can be used to control bleeding, particularly if concentrates are not available

• Prophylaxis helps prevent bleeding

• Home therapy can be used to manage mild/moderate bleeding episodes

GENERAL PRINCIPLES OF TREATMENT

• All PWH should be routinely vaccinated against hepatitis A and hepatitis B.

• Regular exercise should be encouraged to improve general fitness and protect joints.

• Drugs that affect platelet function (Aspirin, NSAIDs) should be avoided.

• Good oral hygiene is important.

For suggested plasma factor peak levels for various bleeds, consult the WFH Guidelines for the Management of Hemophilia (tables on pages 71 and 72 in chapter 7)

SURGERY AND INVASIVE PROCEDURES

• Need to be planned with multidisciplinary team

• Adequate laboratory support needed prior to surgery and for post-op monitoring:

− Factor levels

− Inhibitor testing

• Sufficient concentrates needed for surgery and post-op

– Hemophilia A: 1 IU/kg = 2% rise in factor VIII level

– Hemophilia B: 1 IU/kg = 1% rise in factor IX level  

JOINT BLEEDS

• A joint bleed usually occurs inside a joint e.g. knee, ankle, elbow

• Symptoms include:

− a tingling/bubbling sensation when it starts

− decreased ROM

− pain

− swelling

− heat

• If untreated: swelling, heat, and pain ↑; ROM ↓E

JOINT BLEEDS

• A re-bleed is a bleed that worsens on treatment or within 72 hours of stopping treatment

• A target joint is a joint that has had 3 or more bleeds in the last 6 months

• The goal of treatment is to stop bleeding as soon as possible: Protection-Rest-Ice-Compression-Elevation (PRICE)

• Administer factor dose 10-20 IU/dl

JOINT BLEEDS

Target joints

• boggy+++

• +/- warmth

• have ↓ ROM

• muscle wasting

• pain free (not as painful as a bleed)

• doesn’t respond to factor

TREATMENT OF JOINT BLEEDS

• Avoid weight bearing, PRICE

• Immobilize if possible

• Ice packs around the joint: 5 mins on, 10 mins off

• 2nd infusion may be necessary in first 12 hours if not improving

• Should improve in 3 days - if not needs to be revaluated

• Rehabilitation most important

ADDITIONAL TEXT EXAMPLE

RESPONSE TO TREATMENT OF ACUTE JOINT BLEED

Excellent Complete pain relief within 8 hours and/or complete resolution of signs of bleeding after the initial injection and not requiring any further replacement therapy within 72 hours.

Good Significant pain relief and/or improvement in signs of bleeding within approximately 8 hours after a single injection, but requiring more than one dose of replacement therapy within 72 hours for complete resolution.

Moderate Modest pain relief and/or improvement in signs of bleeding within approximately 8 hours after the initial injection and requiring more than one injection within 72 hours but without complete resolution.

None No or minimal improvement, or condition worsens, within approximately 8 hours after the initial injection.

ISTH Definitions in hemophilia

SYNOVIUM

Synovium = lining of the joint capsule

•Fragile

•Very vascular

•Easily damaged

– Iron deposit

– Physical activity

– Trauma

NORMAL JOINT

BLEEDING JOINT

• Pain

• Swelling

• Limited ROM

• Warmth

– Blood in joint

– Iron release

– Synovial irritation

– Inflammatory cytokines

• Persistent swelling

• Muscle wasting

• Axial deformity

• Crepitation

• Limited ROM

• Instability

• Flexion contracture

BLEEDING JOINT: SEQUELAE

• Blood reabsorption

• Cartilage damage

• Chronic synovitis

SYNOVITIS

• Aim of management is to break the cycle of bleeds

• Raise the factor level above 5 %, with secondary prophylaxis for 3 months to allow the bleed to settle, prevent additional damage, and allow the synovium to clear all the products of bleeding completely

• Static exercises to strengthen muscle without provoking a bleed

• Intensive rehabilitation of ROM and strength

MANAGEMENT OF A TARGET JOINT/SYNOVITIS

• If the above fails, surgical intervention is advised

• Treatment of choice is radioactive synovectomy

• This substance injected intra-articularly decreases the volume and activity of the synovial tissue.

• The aim is to break the cycle of bleeding – synovitis – new blood vessel formation

MANAGEMENT OF A TARGET JOINT/SYNOVITIS

• Radioisotopic synovectomy with yttrium90 is safe and effective

• Radiation from isotopes causes sclerosis of synovial tissue

• Yttrium 90 does not penetrate the growth plate

• Scarring of the synovium prevents further hemorrhages

• Only successful if done before joint destruction has occurred and if there is still joint space

• Must be done at a HCCC

• No pain associated with procedure

SYNOVECTOMY

OUTCOME• Marked reduction in frequency of bleeding

• Reduction of pain

• Limited improvement of ROM

• Does not halt course of the disease

SYNOVECTOMY: OUTCOME

JOINT ASPIRATION/ARTHROCENTESIS

• Removal of blood from a joint

• Need factor cover prior to procedure

• Indications

– Bleeding, tense and painful joint; no improvement after 24 hours

– Severe pain not improving

– Evidence of threatened limb

– Unusual increase in temp/evidence of infectionE

JOINT ASPIRATION / ARTHROCENTESIS (CONT’D)

• May reduce pain and articular damage

• Must be done sooner than later under strict aseptic technique

• Must make sure inhibitors are negative

Afterwards

• Ensure joint is immobilized with mild compression

• No weight bearing for 24-48 hours

• Physio and rehabilitation to follow

ADDITIONAL TEXT EXAMPLE

JOINT BLEEDING SEQUELAE

MUSCLE BLEEDS

• Can occur after direct blow, sudden injury or stretch

• Seen clinically e.g. calf swollen, hot, tender; ↓ ROM

• Early identification and treatment needed to prevent permanent contracture, re-bleeding, and pseudo-tumours.

• Neurovascular compromise or threatened limb can occur in iliopsoas, lower leg, and forearm

• Bleeding can also occur in superficial muscles e.g. biceps, hamstring, quadriceps, and gluteal muscles

MUSCLE BLEEDS: COMMON SITES

MUSCLE BLEED: SYMPTOMS

• Pain/aching in the muscle

• Keeping area of bleed in comfortable position

• Severe pain if muscle is stretched

• Pain on contraction of muscle

• Tension and tenderness upon palpation; possible swelling

• Pain

• Swelling

• Limited ROM

• Warmth

ACUTE MUSCLE BLEEDING

ANATOMY OF A MUSCLE BLEED

ANATOMY OF A MUSCLE BLEED (CONT’D)

ANATOMY OF A MUSCLE BLEED (CONT’D)

ANATOMY OF A MUSCLE BLEED: ILIOPSOAS

MUSCLE BLEEDS

Compartment syndrome•forearm flexors

− median and ulnar nerve− radial artery− risk of Volkmann’s ischemic contracture

•psoas− femoral artery and nerve = paresis of quads = ?knee

•deep flexor compartment of leg− posterior tibial artery

TREATMENT OF MUSCLE BLEEDS

• Raise the factor level asap to 10-20 IU/dl for superficial muscle; 20-40 IU/dl for deep muscle with neurovascular compromise

• Rest injury and elevate if possible

• Splint in a position of comfort (pillows, splint, etc.) and adjust to position of function as pain allows

• Ice packs 15-20 mins every 4-6hrs

TREATMENT OF MUSCLE BLEEDS (CONT’D)

• Repeat infusions often required

• If there is neurovascular compromise, fasciotomy may be required

• Check hemoglobin as muscle bleeds can result in loss of large amount of blood

• Begin rehabilitation as soon as pain allows; may need factor cover

ILIOPSOAS MUSCLE BLEEDS

Symptoms•Pain in lower abdomen, groin, and lower back•Pain at extension of hip joint•Paraesthesia (pins and needles) on the medial aspect of thigh•Signs may mimic acute appendicitis

Treatment•Treat as per muscle bleed; sonar may be useful•Limit activity and slow rehabilitation process to follow with prophylaxis if possible

MUSCLE BLEEDS: HEALING AND REHABILITATION

• Muscles heal by fibrosis

• The muscle fibers change to scar tissue

• They lose their elasticity and are thus more vulnerable to re-bleed

• If blood isn’t resorbed completely, the remaining blood becomes toxic to muscle

• Increased scarring in muscle and inadequate reabsorption can result in pseudotumors

COMPLICATIONS OF MUSCLE BLEEDS

Dropped foot

Flexed hip

Volkmann’s contracture

Restricted movement of limb

ADDITIONAL TEXT EXAMPLE

COMPLICATIONS OF MUSCLE BLEEDS

HEAD TRAUMA/BLEEDS

• This is a medical emergency! Treat before evaluating, don’t wait for results

• Give factor immediately and for up to 14 days after the bleed: 50-80 IU/dl first 3 days, then reduce

• IC hemorrhage may require continuous prophylaxis, especially if chance of recurrence

• Immediate medical evaluation plus CT scan or MRI

• Severe headache may also be associated with meningitis in immunocompromised PWH

ADDITIONAL TEXT EXAMPLE

Cephalohematoma

HEAD TRAUMA/BLEEDS

HEAD TRAUMA

• All bumps/knocks to the head need to be taken seriously

• Manage with factor replacement therapy

THROAT AND NECK BLEEDS

• This is a medical emergency! Treat before evaluating

• Can lead to airway obstruction

• Immediately raise the factor level 30-50 IU/dl for the first 1-3 days, then reduce

• Hospitalization and evaluation by a specialist

• In a PWH with tonsillitis, factor may have to be given with the antibiotics

ADDITIONAL TEXT EXAMPLE

ACUTE GASTROINTESTINAL BLEEDS

• Immediately raise the factor level to 30-50 IU/dl

• May present as hematemesis or melena

• Hospitalization and evaluation by a specialist

• Hb needs to be checked regularly, blood given

• Treat origin of the bleed

• Can use cyklokapron/tranexamic acid in hemophilia A and hemophilia B if on pure FIX (not a PCC)

ACUTE ABDOMINAL BLEEDS

• Acute abdominal or retroperitoneal bleed can present with acute abdominal pain and distension.

• Can be confused with other infectious or surgical conditions

• May also have a paralytic Ileus

• Need factor as soon as possible 60-80 IU/dl

• Will need X-rays and diagnosis; treatment by a specialist

EYE BLEEDS

• Uncommon but can be associated with trauma or infection

• Need factor as soon as possible 50-80 IU/dl

• Will need X-rays and diagnosis; treatment by a specialist (ophthalmologist)

• If optic nerve is damaged with pressure from a bleed in the eye, vision can be lost

KIDNEY BLEEDS

• Treat painless hematuria with hydration/fluids and bed rest

• Raise the factor level if there is pain or persistent hematuria 20-40IU/dl; watch for clots/urinary obstruction

• Do not use cyklokapron/tranexamic acid

• Evaluate by a urologist if bleeding persists

MOUTH BLEEDS

• Early consult with dentist or oral surgeon• Causes related to trauma, dental extraction, and poor

oral hygiene with gingivitis• Local treatments can be used:

− Direct pressure with a swab− Suture if you can− Application of local hemostatic agents e.g. fibrin glue− Antibiotics− Cyklokapron/tranexamic acid

MOUTH/ ORAL BLEEDS

• If persistent give factor 30-50 IU/dl

• Do not use tranexamic acid with a PCC (hemophilia B)

• Use oral tranexamic acid (“swish and swallow”)

• Check Hb and treat if bleed is on-going

• Tell PWH not to swallow the blood

• Avoid mouthwashes until a day after the bleeding has stopped

• Eat a soft diet

ADDITIONAL TEXT EXAMPLE

MOUTH BLEED

ADDITIONAL TEXT EXAMPLE

MOUTH BLEED

DENTAL MANAGEMENT • PWH have the usual dental

problems but extra care should be taken because of bleeding and replacement therapy required

• 2X day teeth brushing and dental floss wherever possible

• Toothpaste containing fluoride if not present in water supply

DENTAL CARE AND MANAGEMENT

• Regular 6 monthly dental examination

• In children, bleeding from mouth, teeth, and gums is common

• Factor replacement required for regional block

EPISTAXIS (NOSE BLEED)

• Place the head forward to avoid swallowing any clots or blood, and ask PWH to gently blow out any weaker clots

• Hold gauze soaked in ice to the anterior part of the nose for 10-20 minutes

• Factor may need to be given if persistent and bleeding is not controlled

• Evaluate for anemia and treat if necessary• Antihistamines and decongestants may help if bleeds are

related to allergies and URTI• Tranexamic acid soaked gauze applied locally may be

helpful

NOSE BLEED

ADDITIONAL TEXT EXAMPLE

SOFT TISSUE BLEEDS

• Symptoms will depend on the site of the bleed• Factor replacement is not always needed for superficial

soft tissue bleeding• Evaluate severity of bleed keeping in mind head and

abdominal bleeds• Open compartment bleeding (e.g. retroperitoneal

space, scrotum, buttocks, or thighs) can result in extensive blood loss

• Treat with factor asap• Monitor Hb and vital signs

SUPERFICIAL BRUISING

SCROTAL BLEED

ADDITIONAL TEXT EXAMPLE

SOFT TISSUE BLEED

LACERATIONS AND ABRASIONS

• Treat superficial lacerations by cleaning the wound, apply pressure with steri-strips if possible

• For deep lacerations, raise the factor level 20-40IU/dl and then suture. Continue to treat for 5-7 days

• Sutures need to be removed after 8-10 days with factor cover

Why do you think this boy is standing with his leg flexed?

ALEXANDER, SON OF THE TSAR OF RUSSIA

SUMMARY

• Every bleed needs to be assessed and managed. When in doubt, treat!

• The longer a bleed goes untreated, the longer it will take to resolve.

• Treatment depends on the resources available.

• The protocols listed here are optimal for “on demand” / “episodic” treatment.

• Doctor needs to prescribe the clotting factor concentrates.

• Always remember to check for inhibitors.

• Skills required for management and care of PWH come with experience.

WFH RESOURCES

• Guidelines for the Management of Hemophilia, 2nd ed

• Emergency Care Issues in Hemophilia

• Treatment Options in the Management of Hemophilia in Developing Countries

• Oral Care for People with Hemophilia or a Hereditary Bleeding Tendency

• Chronic Hemophilic Synovitis: The Role of Radiosynovectomy

• Rehabilitation of Muscle Dysfunction in Hemophilia

ANNE-LOUISE CRUICKSHANKHaemophilia Nurse Coordinator

Western Cape South Africa

Pictures supplied by:

Professor Johnny Mahlangu, University of the Witwatersrand

Professor David Stones University of the Free State

MERGER AVEC SLIDE 1