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IN BRIEFYO U R G U I D E TOvon Willebrand DiseaseIf you often have large, lumpy bruises;frequent or difficult-to-stop nosebleeds;bleed a lot after a fairly minor cut; or area woman who has very heavy or longmenstrual periods, you should ask yourdoctor about an inherited bleedingdisorder called von Willebrand disease(VWD). Named for Dr. Erik vonWillebrand, a Finnish doctor who firstdescribed the condition in 1926, VWDaffects your blood's ability to clot and canlead to heavy, hard-to-stop bleeding afteran injury. The bleeding from VWD canlead to damage of your internal organs oreven be life threatening, but this is rare.In VWD, you either have low levels ofa certain protein in your blood or theprotein doesn't work the way it should.The protein is called von Willebrandfactor (VWF). It is made in the wallsof your blood vessels and released intoyour blood.

Normally, when one of your blood vesselsis injured, you start to bleed. As soon asthis happens, small cells in your blood thatare called platelets clump together to plugthe hole in the blood vessel and stop thebleeding. Von Willebrand factor acts likeglue to help the platelets stick together andform a blood clot.Von Willebrand factor also carries with itclotting factor VIII (8), another importantprotein that helps your blood clot. FactorVIII is the protein that is inactive ormissing in hemophilia, another clottingdisorder.Von Willebrand disease, like hemophilia, isan inherited bleeding disorder, but VWDis more common and usually milder. Infact, VWD is the most common of all theinherited bleeding disorders. It occurs inabout 1 out of every 100 to 1,000 people.It also affects both males and females,while hemophilia mainly affects males.There are three major types of vonWillebrand disease. In type 1 VWD, you have a low level ofthe von Willebrand factor, and you mayhave lower levels of factor VIII thannormal. This is the mildest and mostcommon form of VWD. About 3 out ofevery 4 people with VWD have type 1VWD. In type 2 VWD, the von Willebrandfactor does not work the way it'ssupposed to. Type 2 VWD is dividedinto subtypes 2A, 2B, 2M, and 2N.Each type is caused by different genemutations and treated differently. Thismakes knowing the exact type of VWDthat you have very important.2Your Guide to von Willebrand Disease In type 3 VWD, you usually have novon Willebrand factor and low levels offactor VIII. Type 3 is the most seriousform of VWD, but very rare.Von Willebrand disease cannot be cured,but it can be treated. Early diagnosis isimportant, and with the right treatmentplan, even people with type 3 VWD can behelped to live active lives.What Causes von Willebrand disease?Von Willebrand disease is almost alwaysinherited. Your parents pass the gene forthe disease on to you. You can developtypes 1 or 2 VWD when only one of yourparents carries the gene for it. You usuallyinherit type 3 VWD only if both of yourparents pass the gene on to you. Yoursymptoms may be different from yourparent’s.Some people develop a form of the diseaselater in life as a result of other medicalconditions. This is called Acquired vonWillebrand Syndrome (AVWS).What Are the Signs and Symptoms ofvon Willebrand Disease?The signs and symptoms of VWD dependon the type and severity of the disease.Many people have such mild symptomsthat they may not know they have the disorder. Some people have the gene for thedisease but don't have any symptoms.If you have type 1 or type 2 VWD, you mayhave the following mild to moderatesymptoms:

Frequent large bruises from minorbumps or injuries. Frequent or difficult-to-stop nosebleeds. Extended bleeding from the gums aftera dental procedure. Heavy or extended menstrual bleedingin women.* Blood in your stools from bleeding inyour intestines or stomach. Blood in your urine from bleeding inyour kidneys or bladder. Heavy bleeding after a cut or otheraccident. Heavy bleeding after surgery.*Heavy menstrual bleeding is the mostcommon symptom in women. If it isn’ttreated, it can lead to iron deficiency andanemia. (Not all heavy menstrualbleeding is due to VWD.)If you have type 3 VWD, you may have anyor all of the symptoms listed above, as wellas the following: Severe bleeding episodes for no reason.These bleeding episodes can be lifethreatening if not treated right away. Bleeding into soft tissue or joints,causing severe pain and swelling.How is von Willebrand DiseaseDiagnosed?Von Willebrand disease is sometimesdifficult to diagnose. People with type 1or type 2 VWD may not have majorbleeding problems; as a result, they maynot be diagnosed until they have heavybleeding after surgery or some othertrauma.On the other hand, type 3 VWD can causemajor bleeding problems during infancyand childhood. As a result, children withtype 3 VWD are usually diagnosed duringtheir first year of life.To find out if you have VWD, your doctorwill take a complete medical history anddo a physical exam. For the history, heor she will likely want to know aboutyour personal and family history -–in particular: Any episodes of bleeding from a smallwound that lasted more than 15 minutesor started up again within the first sevendays following the injury. Any episodes of extended, heavy orrepeated bleeding after surgery or dentalextractions that required medicalattention. Any episodes of bruising with little orno apparent trauma, especially if youcould feel a lump under the bruise. A nosebleed that occurred for noapparent reason and lasted more than10 minutes despite pressure on the noseor a nosebleed that needed medicalattention. Any episode of blood in your stool forno apparent reason. Any heavy menstrual bleeding inwomen (usually with clots or lastinglonger than 7 to 10 days). Any history of muscle or joint bleeding.Your Guide to von Willebrand Disease3

Any medicines you've taken that mightcause bleeding or increase the risk ofbleeding – for example, aspirin, othernonsteroidal anti-inflammatory drugs(NSAIDs), clopidogrel (Plavix ),warfarin, or heparin.Your doctor may order these tests morethan once to confirm the diagnosis. He orshe may also refer you to a hematologist(a doctor who specializes in treating blooddiseases) to confirm the diagnosis and forfollow-up care. Any history of liver or kidney disease,blood or bone marrow disease, or highor low blood platelet counts.Early diagnosis is important to make sureyou are treated effectively and can live anormal, active life.The doctor will also do a physical examination to look for:How is von Willebrand DiseaseTreated? Unusual bruising or other signs ofrecent bleeding.Your doctor will decide what treatmentyou need, based on the type of VWD youhave and how severe it is. Most cases ofvon Willebrand disease are mild, andyou may need treatment only if you havesurgery, tooth extraction, or an accident. Evidence of liver disease or anemia.No single test exists for diagnosing VWD.As a result, your doctor will order acombination of blood tests to diagnosethe disease. These tests may include: Von Willebrand factor antigen. This testmeasures the amount of von Willebrandfactor in your blood. Von Willebrand factor ristocetin(ris-toe-SEE-tin) cofactor activity. Thistest shows how well the von Willebrandfactor works. Test for factor VIII clotting activity.Some people with von Willebranddisease have low levels of factor VIIIactivity, while others have normal levels. Von Willebrand factor multimers. Thistest is performed if one or more of the3 tests above are abnormal. It showsthe makeup or structure of the vonWillebrand factor. It helps your doctordiagnose what type of VWD you have. Platelet function test. This testmeasures how well your platelets areworking.4Your Guide to von Willebrand DiseaseTreatments for von Willebrand diseaseinclude medicines to: Increase release of von Willebrandfactor and factor VIII into the bloodstream. Replace von Willebrand factor. Prevent breakdown of clots. Control heavy menstrual bleeding inwomen.Specific treatments include: Desmopressin (DDAVP). This is asynthetic hormone that you usually takeby injection or nasal spray. It makesyour body release more von Willebrandfactor and factor VIII into your bloodstream. DDAVP works for mostpatients with type 1 and some with type2 VWD.

Tips for Living with von Willebrand DiseaseI t ' s i m p o r t a n t t h a t y o u t r y t o p re v e n t b l e e d i n ga n d s t a y h e a l t h y. Yo u s h o u l d :Av o i d o v e r- t h e - c o u n t e r m e d i c i n e s t h a t c a na f f e c t b l o o d c l o t t i n g, i n c l u d i n g a s p i r i n ,I b u p ro f e n , a n d o t h e r n o n s t e ro i d a lanti-inflammatory drugs (NSAIDs). A l w a y s c h e c k w i t h y o u r d o c t o r b e f o ret a k i n g a n y m e d i c i n e s. Te l l y o u r d o c t o r o r d e n t i s t t h a t y o u h a v ev o n W i l l e b ra n d d i s e a s e. Yo u r d e n t i s t c a ntalk to your doctor about whether youn e e d m e d i c i n e b e f o re d e n t a l w o r k t or e d u c e b l e e d i n g. C o n s i d e r w e a r i n g a m e d i c a l I D b ra c e l e t o rnecklace if you have a serious form of VWF replacement therapy. Thisinvolves getting an infusion of a concentrate of von Willebrand factor andfactor VIII into a vein in your arm. Thistreatment can be used if you: Can’t take DDAVP or need extendedtreatment.Have type 1 VWD that doesn’trespond to DDAVP.Have type 2 or type 3 VWD. Oral contraceptives, or birth controlpills, can help women who have heavymenstrual bleeding. The hormones inthe pills can increase the amount ofVWF and factor VIII in the bloodstream. Antifibrinolytic drugs help prevent thebreakdown of blood clots. They areused mostly to stop bleeding after minorsurgery, tooth extraction, or an injury.They may be used alone or togetherwith DDAVP and replacement therapy.V W D ( f o r e x a m p l e, t y p e 3 V W D ) , s o t h a t i ncase of serious injury or accident thed o c t o rs c a r i n g f o r y o u w i l l k n o w y o u h a v eV W D. E x e rc i s e re g u l a r l y a n d m a i n t a i n a h e a l t h yw e i g h t . E x e rc i s e h e l p s ke e p m u s c l e sf l e x i b l e. I t a l s o h e l p s p re v e n t d a m a g e t om u s c l e s a n d j o i n t s. A l w a y s s t re t c h b e f o ree x e rc i s i n g.S o m e s a f e e x e rc i s e s o r a c t i v i t i e s a res w i m m i n g, b i k i n g, a n d w a l k i n g. F o o t b a l l ,h o c ke y, w re s t l i n g, a n d l i f t i n g h e a v yw e i g h t s a re n o t s a f e a c t i v i t i e s i f y o u h a v eb l e e d i n g p ro b l e m s. A l w a y s c h e c k w i t h y o u rd o c t o r b e f o re s t a r t i n g a n y e x e rc i s e p ro g ra m . Fibrin glue is medicine that is placeddirectly on a wound to stop thebleeding.Frequently Asked Questions1. Is there a cure for von Willebranddisease?No, von Willebrand disease is a lifelongdisorder. However, most people have amild form that causes little or nochange in their lives. Even those withtype 3 von Willebrand disease can livenormal, active lives once they aretreated.2. Who should know that I have vonWillebrand disease?People like your doctor, dentist,employee health nurse, gym trainer,and sports coach should be aware ofyour condition. If you have a severeform of VWD (for example, type 3Your Guide to von Willebrand Disease5