Canterbury DHB

Context

Mild Haemophilia

Non-blood product treatment with desmopressin is used for minor bleeds and minor surgical procedures in mild to moderate haemophilia A and von Willebrand Disease.

Treatment Regimen

  • 0.3 microgram/kg IV over 30 min or subcutaneously.
  • Repeat once at 24 hours if necessary to control bleeding, then at intervals >48 hours. Monitor sodium and fluid restrict if a second dose is given. Consult with Haematologist.
  • Tranexamic acid 1 g four times a day, orally.

Desmopressin

Desmopressin is a synthetic analogue of the antidiuretic hormone Vasopressin. It is an established treatment for mild haemophilia and von Willebrand Disease (VWD) and releases FVIII:C and VWF from endothelial storage sites. VWF and VWC levels increase 3-4 times following desmopressin and in VWD, the bleeding time is minimally shortened. The effect may last for 12-24 hours, but patients may become unresponsive to desmopressin after 2-3 days if more than two doses are given within a 2-3 day period. Responsiveness to desmopressin may return in 2-3 days after discontinuation.

See the Desmopressin Data Sheets on Medsafe for dosage, precautions, side effects and contraindications. In particular, beware of the potential for desmopressin to cause hyponatraemia, especially if a repeated dose is given. Restrict fluids to the minimum necessary for 24-48 hours after dosing.

Haemophilia A

Desmopressin is especially useful in patients with mild haemophilia who have a baseline FVIII:C >10%. A three fold rise in factor VIII:C after desmopressin enables a factor VIII target level of at least 30% to be achieved, which is sufficient for normal haemostasis in minor surgical procedures. In both mild and more moderately severe haemophiliacs, the use of desmopressin may be useful as an adjuvant therapy with factor VIII concentrate to achieve adequate haemostasis. It is recommended that a desmopressin trial is carried out prior to surgery to assess the response, as some individuals respond poorly to treatment. Do not do a desmopressin trial within four days of planned surgery.

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Factor VIII Concentrates

For more major bleeding episodes or surgery in patients with mild haemophilia, target factor levels should be similar to those recommended for severe or moderate deficiency patients. In the majority of mildly deficient factor VIII patients, especially those who are previously untreated, recombinant factor VIII is the product of choice.

Note: Beware of the high potential risk for mild haemophilia A patients to develop inhibitory antibodies at times of high antigen exposure (e.g. approximately 10,000 units per treatment episode or period).

About this Canterbury DHB document (5264):

Document Owner:

Mark Smith (see Who's Who)

Issue Date:

August 2016

Next Review:

August 2019

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 5264