Care of patients with hereditary bleeding disorders
Adapted from Haemostasis Guidelines, National Centre for Hereditary Coagulation Disorders, St James’s Hospital, Haemophilia Council Treatment Guidelines
In CUH, please Contact the haematology Registrar on call when the patient arrives (agreement)
If a patient presents to an emergency department in Cork with a similar card or a history of bleeding disorder, please contact the coagulation centre in CUH on (021)4920347 (via switchboard out of hours) and ask for the doctor on call for Haematology.
Treatment of bleeds
- Identify site of bleed and assess clinically. All bleeds or suspected bleeds must be treated immediately prior to any other investigations.
- Check medical records and with patient for type of factor deficiency.
- Check for history of inhibitor
Calculate dose of factor required
FVIII ; k = 2
NOTE : 100%
= 100 IU/dl
= 1.0 IU/ml
A 50kg patient with FVIII level of 10% needs a rise of 70%, therefore rise required = 70 -10 = 60%, FVIII dose required = (60 x 50) ÷2 = 1,500iu rFVIII concentrate (Advate®)
FIX ; k = 0.8
A 50kg patient with FIX level of 25% needs a rise of 100%, therefore rise required = 100 – 25 = 75%, FIX dose required = (75 x 50) ÷ 0.8 = 6,125iu ~ round to 6,000iu rFIX concentrate (Benefix®).
Bleeding Site |
Target post treatment FVIII & FIX factor levels |
Major bleed |
1.0 IU/ml (100%) + commence CI* |
CNS or bleed involving peripheral nerve |
1.0 IU/ml (100%) + commence CI* |
Ileopsoas / retroperitoneal |
1.0 IU/ml (100%) + commence CI* |
Tongue / neck / retropharyngeal |
1.0 IU/ml (100%) + commence CI* |
Gastrointestinal |
1.0 IU/ml (100%) + commence CI* |
Haemarthrosis |
0.5 – 0.7 IU / ml (50% - 70%) |
Minor bleed |
0.5 IU / ml (50%) |
Laceration requiring suturing % removal of sutures |
0.4 IU / ml (40%) |
Haematuria |
High fluid intake +/- rise to 0.3-0.5 IU/ml (30%-50%) |
Minor surgery (angiogram, lumbar puncture) |
1.0 IU/ml (100%) with further dosing if required |
Liver biopsy or central venous catheter |
1.0 IU (100%) + commence CI* x 3 days |
Major surgery |
1.0 IU (100%) + commence CI* consider treatment x 5-14 days (usually switch to bolus dosing by day 5-7) |
*CI = continuous infusion only used in severe haemophilia as may increase risk of inhibitor formation in mild haemophilia (especially FVIII deficiency) |
|
Recombinant activated factor VII: (Novoseven®)
- Haemarthrosis / muscle bleeds - 90µg/kg (4.5KIU/kg) every 2 hrs for 3 doses.
- Major bleeds i.e.. CNS and Illeopsaos bleeds - 90µg/kg (4.5KIU/kg) repeat 2 – 4hourly until bleed resolves.
- If patient with mild FVIII deficiency is known to be DDAVP responsive, consider DDAVP for certain bleeds
- Factor may be obtained from the blood bank in the CUH on a named patient basis
- Always round dose up or down to use the entire vial. Never discard factor
- Do not give IM injections, non-steroidal anti-inflammatories or Aspirin
- Tranexamic acid (Cyclokapron) may be used for mucosal bleeds in conjunction with factor concentrate
INHIBITORS:Discuss all management decisions with Consultant Haematologist on call
Von Willebrands Disease (vWD):
VWD= A quantitative &/or qualitative defect of von Willebrand factor.
Treatments: Tranexamic acid, DDAVP or FANHDI (vWF concentrate)
