South African Haemophilia Foundation (SAHF) Medical and Scientific Advisory Council (MASAC) Treatment Guidelines - Treatment of Bleeding Episodes

Types of Bleeding in Haemophilia

Major bleeding episodes

Central nervous system
Gastrointestinal
Neck/throat
Severe injury
Hip or iliopsoas
Advanced joint/muscle
Forearm compartment

Important

May cause death or deformity. Advice should be sought from a Haemophilia Treatment Centre physician.
Start appropriate Factor replacement urgently. Hospitalisation is usually required to maintain adequate Factor levels.
If the patient has an inhibitor, the Haemophilia Treatment Centre must be consulted for major bleeding problems.

Minor bleeding episodes

Joint (early)
Muscle/soft tissue
Mouth/gums
Epistaxis
Painless haematuria

Important

Although considered minor bleeds, complications may occur. If there are uncertainties about medical management, consult a Haemophilia Treatment Centre.
Treat early to avoid long term complications.

Commonly affected joints

Knees, elbows, ankles, (shoulder, hip and other joint bleeds are rare).

Important

After one or several haemarthrosis with synovitis, a joint may become ‘targeted’ for recurrent bleeding and damage.
These patients should be referred for synovectomy.

Symptoms & Signs

Tingling sensation (early)
Stiffness
Pain
Limited range of motion
Swelling
Limp or refusal to use limb

Important

Do not delay treatment.
Early bleeding can be felt by the patient before signs are apparent.
Obvious joint swelling is a late sign of bleeding.

Treatment Guidelines

Replace missing Factor, see the sections on: Factor VIII, Factor IX

Important

Treat early.
Repeat infusions in 12 to 24 hours.
Ice packs may reduce bleeding: Apply 5 minutes on 10 minutes off.
No circumferential casting.
X-ray not indicated - only if fracture is suspected
Rest the affected joint/limb
posterior splint
sling for arm
no weight bearing

Dangerous Areas

Quadrats femoris (thigh)
Forearm compartment
Neck/throat
Iliopsoas/retroperitoneal
Popliteal
Gastrocnemius (calf)

Important

Aggressive treatment may be indicated. Consult a Haemophilia Treatment Centre for advice.
Hospitalisation may be required.

Symptoms & Signs

Muscle tightness (early)
Pain
Swelling
Limited range of motion
Bruising
Warmth
Refusal to use limb (young child)

Important

Bruising may be absent with deep muscle bleeding.
Muscle compartment bleeding (calf, forearm) may cause nerve damage or vascular compromise.

Treatment Guidelines

Replace missing Factor, see the sections on: Factor VIII, Factor IX
Ultrasound or CT scan to confirm diagnosis and get baseline clot size.

Important

Treat early.
Repeat infusions in 12 to 24 hours.
Ice packs may reduce bleeding: Apply 5 minutes on 10 minutes off.
No circumferential casting.
Rest the affected joint/limb
posterior splint
sling for arm
no weight bearing
Medical Emergency

History

Minor head trauma can lead to CNS bleeding.
Unrecognised trauma is common in children.
“Spontaneous” bleeds can occur.

Important

Treat as a major bleed.
Toddlers and young children are prone to head injury.
Onset may be delayed

Symptoms & Signs

Headache
Vomiting
Irritability/convulsions
Lethargy/drowsiness
Vision disturbance
Focal neurologic deficits
Ataxia
Loss of consciousness
Paralysis

Important

Any of these with or without a history of trauma is highly suggestive of CNS bleeding. These are late features secondary to mass effect or brain irritation.
NB: Symptoms may mimic a viral infection.

Treatment Guidelines

1. Urgent Factor replacement - measure level and maintain above 80 - 100% for 7 days. Then maintain plasma factor level at 50% for haemophilia A and 30% for haemophilia B for a further 14 days.
2. CT scan of head
3. Call a Haemophilia Treatment Centre

Important

If in doubt - treat
Admit to hospital
Factor replacement for possible subarachnoid bleeding is indicated even with a normal CT scan.
For proven intracranial bleeding treatment is required for 21 days.
Anti-epileptic medication as soon as bleed is confirmed.

Dental / Gum Bleeding / Epistaxis / Tongue

Bleeding can be profuse
Swallowing blood: vomiting blood, dark tarry stools
May need Hb and HCT

Important

Torn frenulum (upper lip) bleeding is problematic in the young child.
Dental injection or extraction requires prior therapy to raise Factor levels.

Treatment Guidelines

Replace missing Factor as per minor bleed, see the sections on: Factor VIII, Factor IX
Tranexamic acid (See Treatment of Bleeding Episodes)
Local pressure.
Ice 5 minutes on, 10 minutes off.
If tooth extracted use deep silk or Vicryl suture.
Red cell transfusion if necessary.

Important

Cool, soft, or liquid diet following gum bleeding or dental extraction.
Tranexamic acid solution: give 5 - 10 ml (500 mg / 5 ml) 6-hourly, holding in mouth for 2 minutes before swallowing. Tranexamic acid tablets can also be crushed in warm water before swallowing.
Continue factor infusion and tranexamic acid until bleeding stops.

Potential Emergency

Bleeding can be profuse
Blood / coffee-ground emesis
Dark / tarry stools

Important

Abdominal pain - regard as a bleed until proven otherwise: INFUSE FACTOR FIRST, then investigate as for all GIT bleeds

Treatment Guidelines

Immediate Factor replacement, see the sections on: Factor VIII, Factor IX
Consult a Haemophilia Treatment Centre
Check BP, Hb & HCT
Concomitant tranexamic acid therapy.

Important

Hospitalise to maintain Factor level and monitor ongoing blood loss.
Investigate for site of bleeding.
May need red cell transfusion.
Usually spontaneous

Symptoms & Signs

May have renal angle tenderness
Red or dark urine
Usually no dysuria

Important

Persistent or recurrent haematuria should be investigated

Treatment Guidelines

Increase fluid intake (1 glass/hour)
Bed rest
If not resolved in 24 hours treat with Factor replacement, see the sections on: Factor VIII, Factor IX
Continue to increase fluids and bed rest for 7 days

Important

Tranexamic acid is contraindicated

Management of Patients

Types of surgical interventions:

  • Minor surgery, which includes endoscopy, skin biopsy, bronchoscopy, lumbar puncture, dental procedures, etc.
  • Major surgery, which includes laparotomy, arthroplasty.

Preoperative assessment and preparation:

  • Consultation between surgeon, haematologist and blood centre.
  • Check FBC, liver function, renal function and inhibitor level.
  • Do Factor recovery studies.
  • Prepare a written management plan and communicate this to all stakeholders.

Surgery treatment goals:

  • Raise Factor level to 50 – 80% for minor surgery and 80 – 100% for major surgery.
  • Maintain Factor level at 50% for major surgery for at least 7 – 14 days.
  • Avoid intraoperative and postoperative blood loss.

Surgery treatment approach:

Haemophilia A

  • for major surgery, give 40 – 50 IU/kg FVIII
  • for minor surgery give 20 – 40 IU/kg FVIII, 30 minutes before surgery, 6 hours postoperatively and then 12-hourly thereafter.

Haemophilia B

  • for major surgery, give 60 – 80 IU/kg FIX
  • for minor surgery 20 – 40 IU/kg, 30 minutes before surgery. Repeat the same dose 6 hours postoperatively and then daily thereafter.

  • Factor infusion for major surgery should continue for 7 – 14 days. Venous thromboembolism (VTE) prophylaxis using elastic stockings should be considered in all high-risk surgery.
  • Keep peak maintenance Factor level at 50% until healing has started.
  • Introduce postoperative rehabilitation and mobilisation gradually under Factor prophylaxis.
  • Continuous infusion of Factor with a pump may be used.
  • Use of antibiotics postoperatively is mandatory. NB: Check for allergies e.g. penicillin
  • Ensure that patient receives adequate analgesia – NB avoid intramuscular analgesia.

Managment of Chronic Synovitis and Target Joints:

Synovitis is the inflamed state of the synovium

  • Blood vessels proliferate.
  • These vessels are more prone to recurrent bleeding and target joint occurs.

Chronic synovitis

  • A condition that persists for 6 months or more.
  • There is a predilection for large joints

Treatment:

  • Secondary prophylaxis (see section on Prophylaxis ).
  • Raise plasma level above 5 %.
  • Exercises to strengthen joint and muscle.
  • Ablation of the synovium using Yttrium Synoviorthesis (only at HCCC).

Procedure for Synoviorthesis:

  • To prevent cycle of bleeding commence secondary prophylaxis
  • Raise Factor level to 50% prior to procedure
  • Give Factor daily x 3 days thereafter
  • Intra-articular injection of local anesthetic and steroids given at the same time
  • Immobilize for 3 days

Management of the Haemophilia Carrier and Pregnancy:

  • Check Haemophilia carrier baseline Factor level
  • Symptomatic carriers managed according t o severity of symptoms
  • May be treated with DDAVP, Tranexamic acid or Factor replacement

  • Level done to establish bleeding risk
  • Symptomatic carriers should wear MedicAlert® bracelets
  • Menorrhagia can be controlled using hormonal, haemostatic or surgical methods

Pre- Pregnancy:

  • Provide pre – pregnancy genetic counselling to all carriers
  • Establish gene abnormality

At Pregnancy:

  • Take family and personal bleeding history
  • Plan management with obstetrician and haematologist
  • Measure Factor level at 28 and 34 weeks
  • If bleeding do not use DDAVP

  • If not sure of carriers status check sex of fetus

At delivery:

  • Plan for vaginal delivery
  • Avoid scalp monitor
  • Avoid vacuum and forceps
  • Take cord blood for Factor levels
  • Avoid heel pricks
  • Give oral Vit K
  • If bleed, use Factor replacement

  • Watch for bleeding in mother and child
  • FIX assay unreliable in new born

Post Partum:

  • Watch for post partum bleeding
  • Treat with Factor replacement or DDAVP

  • Factor levels fall to baseline in first week

Genetic testing for Haemophilia A and B is important for:

  • Definitive carrier testing
  • Prenatal counselling and testing

Definitive carrier testing:

All females who are at risk of being haemophilia carriers (mother, sisters, maternal aunts and maternal aunts’ daughters of a person with haemophilia) should be offered genetic counselling and testing, so that their carrier status can be determined definitively. This can be done in early childhood, so that pre-emptive management is possible, but with appropriate consent and genetic counselling.

  • Females who are shown to be carriers or high-risk can then be managed appropriately for bleeding complications
  • Females who are non-carriers or at low risk would be at very low risk of bleeding complications

Prenatal counselling and testing:

Females who are shown to be carriers or high-risk should be offered genetic counselling when they reach child-bearing age to discuss their risks and options for prenatal testing and pregnancy management.

Genetic testing:

  • Is complex
  • May be done by direct mutation analysis or gene tracking (linked marker) analysis
  • May require blood samples from a number of family members (including unaffected individuals)
  • Consult with a Genetics Centre to determine from which family members samples are required

Prophylaxis:

  • Primary prophylaxis is aimed at stopping spontaneous bleeds in people who have severe Haemophilia
  • Secondary prophylaxis is given when there is a high requirement for on demand treatment
  • Single dose prophylaxis is given prior t o an event known to cause bleeding

  • Rationale for prophylaxis is to maintain Factor activity above 1% converting a bleeding pattern from severe to mild / moderate
  • Target joints are a high requirement and secondary prophylaxis is used

Treatment:

  • Haemophilia A : give 25 – 40 IU/kg, 2 – 3 times per week
  • Haemophilia B: give 25 – 40 IU/kg, twice per week

Management of Pain in Haemophilia:

  • The most effective pain management is early appropriate Factor replacement.

  • Avoid giving analgesic agents for every bleed

Pain may be caused by:

  • Joint capsular stretching as a result of haemarthroses
  • Haemophilia arthropathy
  • Compartment syndrome

  • Aim to relieve pain without risk of bleeding

Treatment:

  • COX-2 inhibitors are favoured due to favourable side effects, good analgesic effects, anti-inflammatory and anti-angiogenic effects
  • Other agents know to relieve pain without increasing bleeding risk are:
    • opiates
    • paracetamol

  • Aspirin and other antiplatelet agents must be avoided
  • Analgesia requiring intra muscular injections must be avoided