Revisions: 244; 186; 173; 196
a. DDAVP and Stimate should be administered no more often than once every 24 hours.
b. DDAVP and Stimate should be used for no more than three consecutive days unless directed to do so by Hemophilia Treatment Center medical staff.
c. DDAVP and Stimate should not be used in children under the age of two years.
d. DDAVP and Stimate should be used with caution in the elderly and in individuals with a history of heart disease, hypertension, or stroke.
- If a patient is treated with DDAVP before surgery, the anesthesiologist should be advised to avoid fluid overload and dilutional hyponatremia.
- DDAVP should be used with caution in pregnant women in the peripartum and immediate postpartum period, with careful attention to fluid management to avoid hyponatremia.
- Oral fluids should be restricted to maintenance for 24 hours following treatment.
Persons with type 2B and type 3 VWD, and those with type 1, 2A, 2M, and 2N who have been shown to be nonresponsive to DDAVP, should be treated with a factor VIII/VWF concentrate that is known to contain the higher molecular weight multimers of von Willebrand factor and that has been virally attenuated to eliminate transmission of HIV and hepatitis A, B, and C. Human plasma-derived products Alphanate, Humate P, and Wilate have been approved by the FDA for use in VWD. A recombinant VWF concentrate, Vonvendi, has also been approved. Another plasma-derived product, Koate DVI, may also be effective in these patients, but it has not been approved by the FDA for use in VWD. For further information, see MASAC Document #263, “MASAC Recommendations Concerning Products Licensed for the Treatment of Hemophilia and Other Bleeding Disorders.”
Because of the increased risk of HIV and hepatitis A, B, and C transmission, cryoprecipitate should not be used except in an emergency situation when none of the above-mentioned products are available and delay of treatment would be life- or limb-threatening.
In patients with VWD with a history of major and frequent bleeds, the ASH ISTH NHF WFH guideline panel suggests using long-term prophylaxis with factor replacement rather than no prophylaxis.(3) Prophylaxis in VWD is defined as a period of at least 3 months of treatment of VWF concentrate at least once weekly, or for women with HMB, use of VWF concentrate at least once per menstrual cycle.