Major bleeding episodes
Minor bleeding episodes
Commonly affected joints
Symptoms & Signs
Symptoms & Signs
Onset may be delayed
Symptoms & Signs
Dental / Gum Bleeding / Epistaxis / Tongue
Symptoms & Signs
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:
- 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.
- 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.
- 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
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
- 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
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.
- 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
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
- 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:
- Aspirin and other antiplatelet agents must be avoided
- Analgesia requiring intra muscular injections must be avoided