Types of Bleeding in Haemophilia

• 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.

• 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.

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.

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

• 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

• 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

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

• 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

• 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

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

• Factor levels fall to baseline in first week

• Definitive carrier testing
• Prenatal counselling and 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

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.

• 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

• 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

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

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

• Avoid giving analgesic agents for every bleed

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

• Aim to relieve pain without risk of bleeding

• 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