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DDAVP for von Willebrand Disease

Stimate Nasal Spray stimulates von Willebrand factor and factor VIII

By Stephanie Stephens | 02.07.2011
Originally Published September 2008 | Updated June 2010
Woman dancing

“One and two and flip.” Lilliam Bervis thinks out loud, concentrating on her partner’s precise and rhythmic steps as the pair dances the salsa. Attentive “spotters” remain focused, just in case, on the athletic couple’s next moves. They’re in a practice studio, diligently rehearsing for an upcoming nightclub competition.

A fall would likely result in serious bruising for Lilliam, or Lily, 23, of Miami, who was diagnosed at four years old with type 1 von Willebrand disease (VWD), the most common bleeding disorder in women and girls. It is caused by a defect or deficiency of an essential blood clotting protein called von Willebrand factor (VWF). Lily, a former member of the National Hemophilia Foundation’s National Youth Leadership Institute, also has a mild factor VIII deficiency.

To shorten the duration of her irregular menstrual periods, Lily uses Stimate Nasal Spray®, a highly concentrated form of desmopressin acetate—also called simply desmopressin, or DDAVP. For treating bleeding disorders, the hormone may also be injected intravenously or subcutaneously. Storing Stimate has become more convenient. The nasal spray can now be stored at room temperature for up to six months without compromising stability, making it easier to carry with you. The nasal spray should always be stored in an upright position.

Most commonly prescribed for those with type 1 VWD or mild hemophilia A, DDAVP works by stimulating release of both VWF and factor VIII found in storage sites lining the blood vessels, says Lisa Michaels, MD, director of pediatric hemostasis and thrombosis for the New Jersey Regional Hemophilia Program in New Brunswick. DDAVP is a synthetic analogue of vasopressin, a naturally occurring hormone in the body. It carries no risk of infectious disease. Vasopressin helps balance the amount of water and salt in the body.

The medication is recommended on an individual basis for a variety of conditions, says Barbara Konkle, MD, Penn Comprehensive Hemophilia Program, University of Pennsylvania Medical Center in Philadelphia. She may prescribe the spray for women prone to any of the following: frequent nosebleeds, serious bruising or heavy menstrual periods (menorrhagia). Additionally, DDAVP is used prior to procedures involving mucous membranes, such as dental work, for home treatment of minor injuries and for minor or moderate surgeries. However, individual response should first be assessed in a preliminary treatment trial, Konkle says.

Taking a Break

Subcutaneous administration of DDAVP was used occasionally before intranasal dosing was available, says Konkle. She finds patients respond quicker to intravenous administration than to intranasal; it is often a better choice for in-patient surgery. Outpatient procedures usually work well with intranasal administration, Konkle says. Highly concentrated Stimate for bleeding disorders is delivered in the adult dose of two sprays of 150 micrograms, or 300 micrograms total; patients weighing less than 50 kilograms (110 pounds) receive one 150-microgram squirt.

The rule “more is better” does not apply here, Michaels says. Using the medication for a short time results in a progressively lowered response. For example, on the first day, a big boost of VWF level may occur, but on the second day it’s lower, and so on. That diminishing response, called tachyphylaxis, mandates a break between consecutive doses, Michaels says. Since responses are always variable, Michaels says they should be continually monitored. “Some patients enjoy complete and total correction of their VWF levels, while some achieve symptomatic relief.”

Conserving Water

The water retention properties of DDAVP must be carefully managed. Retention of free water by the kidneys, according to Konkle, causes hyponatremia, an abnormally low concentration of sodium in the blood, which could result in a seizure. She advises people not to drink more than 24 ounces of fluid in 24 hours. Further, if a patient is undergoing surgery and receiving intravenous DDAVP, Konkle asks surgeons to keep IV fluid administration to a minimum. “The risk of developing low blood sodium is thought to be greater in the very young and very old, so DDAVP is used more cautiously in those groups,” Konkle says.

“Taking DDAVP is not the time to drink those requisite ‘eight glasses of water,’” adds Michaels. In general, people will begin to urinate one to eight hours after administration of DDAVP. Once urination occurs, the risk of free water retention is decreased.

Side effects of DDAVP may include headaches, nausea and facial flushing or redness, says Konkle.

Citing the convenience and portability of the medication, Lily says she usually sprays before a dance as a preventive measure. She and her partner have won some competitions, but this is a fun hobby—she’s not counting on winning “Dancing with the Stars.” Lily graduated in 2009 with a biology degree from Florida International University. She recently started her own business, which provides customer service to a major cruise line.