Transcript Management of bleeds

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-CompressionElevation (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
BLEEDING JOINT: SEQUELAE
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•
•
•
•
•
•
Persistent swelling
Muscle wasting
Axial deformity
Crepitation
Limited ROM
Instability
Flexion contracture
SYNOVITIS
• Blood reabsorption
• Cartilage damage
• Chronic synovitis
MANAGEMENT OF A TARGET JOINT/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
SYNOVECTOMY
• 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
OUTCOME
• Marked reduction in frequency of bleeding
• Reduction of pain
• Limited improvement of ROM
• Does not halt course of the disease
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
E – Unusual increase in temp/evidence of infection
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
JOINT BLEEDING SEQUELAE
ADDITIONAL TEXT EXAMPLE
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 pseudotumours.
• 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
ACUTE MUSCLE BLEEDING
•
•
•
•
Pain
Swelling
Limited ROM
Warmth
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
COMPLICATIONS OF MUSCLE BLEEDS
ADDITIONAL TEXT EXAMPLE
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
HEAD TRAUMA/BLEEDS
ADDITIONAL TEXT EXAMPLE
Cephalohematoma
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
MOUTH BLEED
ADDITIONAL TEXT EXAMPLE
MOUTH BLEED
ADDITIONAL TEXT EXAMPLE
DENTAL CARE AND MANAGEMENT
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
• 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
SOFT TISSUE BLEED
ADDITIONAL TEXT EXAMPLE
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
ALEXANDER, SON OF THE TSAR OF RUSSIA
Why do you think this
boy is standing with
his leg flexed?
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
MERGER AVEC SLIDE 1
ANNE-LOUISE CRUICKSHANK
Haemophilia Nurse Coordinator
Western Cape South Africa
Pictures supplied by:
Professor Johnny Mahlangu, University of the Witwatersrand
Professor David Stones University of the Free State