Von Willebrand diseaseVVon Willebrand diseaseVon Willebrand diseaseEnglishHaematologyChild (0-12 years);Teen (13-18 years)BodyCardiovascular systemConditions and diseasesCaregivers Adult (19+)NA2017-05-08T04:00:00ZVanessa Bouskill, MN, RN(EC), NP;Manuel Carcao, MD, FRCP(C), FAAP, MScsusan.ball@sickkids.ca | Susan Ball | 693A30232E777C7369636B6B6964735C737573616E2062616C6C i:0#.w|sickkids\susan ball7.5000000000000066.70000000000001410.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Von Willebrand disease is a condition that affects the blood’s ability to clot properly. Learn about the different types of this disease and how they are treated.</p><h2>What is von Willebrand disease?</h2><p>Von Willebrand disease (VWD) is an inherited condition that affects the blood’s ability to clot effectively. It is a very common bleeding disorder that affects girls and boys equally.</p><p>When a person gets a cut, a type of cell in the blood called a platelet, and a protein called von Willebrand factor (VWF), stick to the wound to plug the cut. Proteins in the blood called clotting factors then bind together to form chains called fibrin. These chains then form a net around the platelets and VWF. This holds the clot together and helps to seal the wound.</p><p>In a person with VWD, there is either not enough VWF or it does not work properly. In either case, a blood clot cannot form properly, and the injured area will continue to bleed.</p> <p>VWF also protects and maintains levels of clotting factor VIII, one of the proteins involved in blood clotting. Therefore, people with VWD may have low levels of factor VIII in addition to low levels of VWF.</p><h2>Key points</h2><ul><li>The first signs of VWD are usually prolonged nosebleeds, easy bruising and in females heavy periods.</li><li>VWD is a lifelong condition that is usually passed down from parents to children. It equally affects both girls and boys.</li><li>Severity of VWD can vary, and thus treatments do as well. Options will be discussed between you and your child’s comprehensive health-care team.</li></ul><h2>Signs that your child may have von Willebrand disease</h2><p>If your child experiences any of the following symptoms, you should talk to your doctor about the possibility that your child may have VWD:</p><ul><li>bleeding from the gums</li><li>easy bruising</li><li>prolonged bleeding after cuts or bloodwork</li><li>frequent and prolonged nosebleeds</li><li>blood in the stool or urine</li><li>heavy or prolonged menstrual bleeding (menorrhagia) in girls</li><li>soft tissue/joint bleeding (in more severe forms).<br></li></ul><p>As VWD is usually inherited, it is possible that after a child in a family is diagnosed with VWD that other family members may be tested as well.</p><h2>How is von Willebrand disease inherited?</h2><p>VWD is a genetic condition and it is most often passed on from one or both parents to their children. Occasionally, VWD may occur when one of the child’s genes changes randomly; this is called a spontaneous genetic mutation.</p><div class="akh-series"><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 1, 2A, 2B and 2M von Willebrand disease</span><img alt="Inheritance pattern of Type 1, 2A, 2B and 2M von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_1_2A_2B_2M_EN.jpg" /> </figure> <p>The type of VWD depends on the mutation in the VWF gene. The parent may have mild symptoms or no symptoms at all. Types 1, 2A, 2B and 2M are usually inherited when one parent passes on the mutated VWF gene to their child.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 2N and 3 von Willebrand disease</span><img alt="Inheritance pattern of Type 2N and 3 von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_2N_3_EN.jpg" /> </figure> <p>Types 2N and 3 VWD are usually inherited when both parents pass on the mutated gene to their child. A child receives two mutated genes, one from each parent. Each parent may have only mild symptoms or have no symptoms at all.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 2N von Willebrand disease</span><img alt="Inheritance pattern of Type 2N von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_2N_EN.jpg" /> </figure> <p>Type 2N VWD occurs when a child inherits one Type 1 (or Type 2N) gene from one parent and one Type 2N gene from the other parent.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 3 von Willebrand disease</span><img alt="Inheritance pattern of Type 3 von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_3_EN.jpg" /> </figure> <p>Type 3 VWD occurs when a child inherits a gene responsible for Type 1 VWD from both parents. In most (but not all) cases parents of a child with Type 3 VWD, despite having a gene for type 1 VWD, usually have normal levels of VWF and show no signs of bleeding.</p></div></div></div><h2>Diagnosis of von Willebrand disease</h2><p>While VWD has a genetic cause, VWF can also be affected by many other things such as blood type, age, stress, medications and hormone levels.</p><p>Often, the symptoms of Type 1 VWD are very mild. For this reason, it can take time to diagnose.</p><p>A diagnosis of VWD involves a series of blood tests measuring VWF levels and function as well as factor VIII levels in the blood. A physical exam, a review of family medical history and a bleeding questionnaire will also be performed.</p><h2>Treatment of von Willebrand disease</h2><p>Children diagnosed with VWD are cared for by a comprehensive team of health care specialists. This team will include a nurse co-ordinator, hematologist (blood doctor), physiotherapist, social worker, dentist and, for female teens and adults, a gynecologist.<br></p><h3>Medications may be used to treat all types of von Willebrand disease</h3><p><em><strong>Tranexamic acid</strong></em></p><p>Tranexamic acid is used to hold a blood clot together once it has already formed. It is often used when a person with VWD is undergoing a surgical or dental procedure, and for nosebleeds or mouth bleeds. For females, it can also be used for heavy periods.</p><p><em><strong>Fibrin glue</strong></em></p><p>Fibrin is the final product when a blood clot is formed. Fibrin glue is made up from several blood clotting agents. It is applied directly to wounds to stop bleeding. Fibrin glue is often used when people with VWD undergo surgical or dental procedures.</p><p><em><strong>Desmopressin (DDAVP)</strong></em></p><p>DDAVP is a chemical that helps to release factor VIII and VWF that is already stored in the blood. Before using this method, your child’s comprehensive care team will perform a “DDAVP Challenge”. This test is done to see if the use of DDAVP releases enough factor VIII and VWF in your child’s body to treat bleeds. If it does, your child is a "responder". Treatment of VWD with DDAVP is not recommended until your child is at least three years of age because of potential side effects. DDAVP does not work in some Type 2 subtypes and Type 3 VWD.</p><p><em><strong>Von Willebrand factor product</strong></em></p><p>Von Willebrand factor product is a plasma derived factor concentrate (a powder) that has been engineered from human plasma. It undergoes viral inactivation steps to make sure the product is safe. VWF product is used in more moderate or severe bleeds, to replace the VWF and FVIII in the body immediately to stop bleeding.</p><h3>Treatment by type</h3><p>Medical treatment of VWD will vary by patient, and should be discussed with your child’s healthcare team.</p><table class="AKH-zebra-table"><thead><tr><th style="width:50%;">Type of von Willebrand disease</th><th style="text-align:center;width:50%;">Most common treatment options</th></tr></thead><tbody><tr><td><strong>Type 1</strong></td><td>DDAVP (if a responder); von Willebrand factor product</td></tr><tr><td><strong>Type 2</strong></td><td>DDAVP (if a responder); von Willebrand factor product</td></tr><tr><td><strong>Type 3</strong></td><td>von Willebrand factor product</td></tr></tbody></table>
Maladie de von WillebrandMMaladie de von WillebrandVon Willebrand diseaseFrenchHaematologyChild (0-12 years);Teen (13-18 years)BodyCardiovascular systemConditions and diseasesAdult (19+) CaregiversNA2017-05-08T04:00:00ZVanessa Bouskill, MN, RN(EC), NP;Manuel Carcao, MD, FRCP(C), FAAP, MScHealth (A-Z) - ConditionsHealth A-Z<p>La maladie de von Willebrand est une maladie héréditaire qui affecte la capacité du sang à coaguler efficacement. Découvrez les différents types de cette maladie et comment ils sont traités.<br></p><h2>Qu'est-ce que la maladie de von Willebrand?</h2><p>La maladie de von Willebrand est une maladie héréditaire qui affecte la capacité du sang à coaguler efficacement. Ce trouble hémorragique très fréquent touche autant les filles que les garçons.</p><p>Lorsqu'une personne se coupe, un type de cellules sanguines, appelées plaquettes, et une protéine, appelée facteur de von Willebrand (FVW), se collent à la plaie afin de boucher la coupure. Des protéines sanguines nommées facteurs de coagulation se lient alors les unes aux autres afin de former des chaînes appelées fibrines. Ces chaînes forment ensuite un filet autour des plaquettes et des FVW. Ceci maintien la cohésion du caillot et aide à colmater la plaie.</p><p>Chez une personne atteinte de la maladie de von Willebrand, il n'y a pas suffisamment de FVW ou ils ne fonctionnent pas correctement. Dans les deux cas, le caillot sanguin ne peut se former normalement et la zone blessée continuera de saigner.</p><p>Les FVW protègent et maintiennent aussi les niveaux de facteurs de coagulation VIII, l'une des protéines responsables de la coagulation sanguine. Par conséquent, les personnes atteintes de la maladie de von Willebrand peuvent présenter un faible niveau de facteurs VIII en plus d'avoir un faible niveau de FVW.</p> <h2>À retenir</h2><ul><li>Les signes avant-coureurs de la maladie de von Willebrand sont habituellement des saignements de nez qui perdurent, une tendance aux ecchymoses et, chez les femmes, des saignements menstruels abondants.</li><li>La maladie de von Willebrand est une maladie chronique qui est transmise des parents aux enfants dans la plupart des cas. Sa fréquence est la même chez les filles et les garçons.</li><li>La gravité de la maladie de von Willebrand varie, tout comme les traitements. Vous discuterez des options avec l'équipe globale de soins de santé de votre enfant.</li></ul> <h2>Signes indiquant que votre enfant pourrait être atteint de la maladie de von Willebrand</h2><p>Si votre enfant présente l'un des symptômes suivants, consultez votre médecin sur la possibilité que votre enfant soit atteint de la maladie de von Willebrand :</p><ul><li>saignements des gencives;</li><li>tendance aux ecchymoses;</li><li>saignements qui perdurent à la suite d'une coupure ou d'une prise de sang;</li><li>saignements de nez fréquents et prolongés;</li><li>présence de sang dans les selles ou les urines;</li><li>saignements menstruels abondants ou prolongés (ménorragie) chez les filles;</li><li>hémorragies articulaires ou des tissus mous (chez les formes graves).</li></ul><p>Comme il s'agit habituellement d'une maladie héréditaire, il est possible qu'après avoir diagnostiqué la maladie de von Willebrand chez un enfant, les autres membres de la famille soient testés eux aussi.</p><h2>Comment la maladie de von Willebrand est-elle transmise?</h2><p>La maladie de von Willebrand est une maladie génétique et elle est le plus souvent transmise par l'un des parents ou par les deux. À l'occasion, la maladie de von Willebrand apparaît lorsque l'un des gènes de l'enfant mute de manière aléatoire; ce phénomène se nomme mutation génétique spontanée.</p><div class="akh-series"><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Mode de transmission de Type 1, 2A, 2B et 2M de la maladie de von Willebrand</span><img alt="Mode de transmission de Type 1, 2A, 2B et 2M de la maladie de von Willebrand." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_1_2A_2B_2M_FR.jpg" /> </figure> <p>Le type de la maladie de von Willebrand dépend du type de mutation du gène FVW. Le parent peut présenter peu de symtômes ou aucun symptôme. Les Types 1, 2A, 2B et 2M sont généralement hérités lorsque l'un des parents transmet le gène FVW muté à son enfant.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Mode de transmission de Type 2N et 3 de la maladie de von Willebrand</span><img alt="Mode de transmission de Type 2N et 3 de la maladie de von Willebrand." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_2N_3_FR.jpg" /> </figure> <p>Les Types 2N et 3 de la maladie de van Willebrand sont généralement hérités lorsque les deux parents transmettent le gène muté à leur enfant. Un enfant reçoit deux gènes mutés, un de chacun de ses parents. Chaque parent peut présenter peu de symtômes ou aucun symptôme.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Mode de transmission de Type 2N de la maladie de von Willebrand</span><img alt="Mode de transmission de Type 2N de la maladie de von Willebrand." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_2N_FR.jpg" /> </figure> <p>Le Type 2N de la maladie de von Willebrand apparaît lorsqu'un enfant hérite d'un gène de Type 1 (ou de Type 2N) de l'un de ses parents et d'un gène de Type 2N de l'autre parent.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 3 von Willebrand disease</span><img alt="Inheritance pattern of Type 3 von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_3_FR.jpg" /> </figure> <p>Le Type 3 de la maladie de von Willebrand apparaît lorsque les deux parents transmettent à leur enfant un gène responsable du Type 1 de cette maladie. Dans la plupart des cas (mais pas tous), les parents d'un enfant atteint du Type 3 de la maladie de von Willebrand présentent des niveaux normaux de FVW et ne montrent aucun signe d'hémorragie, malgré qu'ils soient porteurs du gène du Type 1 de cette maladie.</p></div></div></div><h2>Diagnostiquer la maladie de von Willebrand</h2><p>Bien que la maladie de von Willebrand ait une cause génétique, la présence de la maladie peut dépendre de plusieurs autres facteurs comme le groupe sanguin, l'âge, le stress, les médicaments et les niveaux d'hormones.</p><p>Bien souvent, les symptômes de Type 1 de la maladie de von Willebrand sont très bénins. C'est pour cette raison qu'elle peut prendre du temps à être diagnostiquée.</p><p>Le diagnostic de la maladie de von Willebrand nécessite une série d'analyses sanguines mesurant les niveaux et la fonction de FVW de même que les niveaux du facteur VIII dans le sang. Un examen physique, une étude des antécédents médicaux de la famille et un questionnaire portant sur les épisodes hémorragiques seront aussi réalisés.</p> <h2>Traitement de la maladie de von Willebrand</h2><p>Les enfants chez qui on a diagnostiqué la maladie de von Willebrand sont pris en charge par une équipe globale de spécialistes en soins de santé. Cette équipe comprend un coordonnateur en soins infirmiers, un hématologue (un médecin du sang), un physiothérapeute, un travailleur social, un dentiste et, pour les adolescentes et les femmes adultes, un gynécologue.</p><h3>Des médicaments peuvent être employés afin de traiter tous les types de la maladie de von Willebrand.</h3><p> <em> <strong>Acide tranexamique</strong></em></p><p>L'acide tranexamique aide à maintenir la cohésion du caillot sanguin une fois qu'il est formé. Il est souvent employé lorsqu'une personne atteinte de la maladie de von Willebrand doit subir une intervention chirurgicale ou dentaire de même que contre les saignements de nez et les hémorragies buccales. Chez les femmes, il peut être utilisé en cas de saignements menstruels abondants.</p><p> <em> <strong>Colle à la fibrine</strong></em></p><p>La fibrine est le résultat final de la formation d'un caillot sanguin. La colle à la fibrine est constituée de plusieurs agents de coagulation sanguine. Elle est appliquée directement sur les plaies pour arrêter le saignement. La colle à la fibrine est souvent employée chez les personnes atteintes de la maladie de von Willebrand qui subissent une intervention chirurgicale ou dentaire.</p><p> <em> <strong>Desmopressine (DDAVP)</strong></em></p><p>La DDAVP est un produit chimique qui aide à libérer les facteurs VIII et FVW qui sont déjà emmagasinés dans le sang. Avant d'employer cette méthode, l'équipe globale de soins de votre enfant fera un « test à la DDAVP ». Ce test est fait afin de vérifier si l'utilisation de la DDAVP libère suffisamment de facteurs VIII et FVW dans l'organisme de votre enfant pour traiter ses saignements. Si c'est le cas, votre enfant est un « sujet répondant ». Le traitement de la maladie de von Willebrand à la DDAVP n'est pas recommandé avant que votre enfant n'atteigne l'âge de trois ans en raison des effets secondaires potentiels. La DDAVP ne fonctionne pas pour certains sous-types du Type 2 et pour le Type 3 de la maladie de von Willebrand.</p><p> <em> <strong>Produit du facteur de von Willebrand</strong></em></p><p>Le produit du facteur de von Willebrand est un concentré de facteur (une poudre) dérivé du plasma qui a été conçu à partir de plasma humain. Il subit des étapes d'inactivation virale afin de s'assurer que le produit est sûr. Le produit de FVW est employé dans les cas d'hémorragies modérées ou sévères afin de remplacer immédiatement les FVW et les FVIII de l'organisme et arrêter les saignements.</p><h3>Traitement par type</h3><p>Le traitement médical de la maladie de von Willebrand varie en fonction du patient et devrait être abordé avec l'équipe de soins de santé de votre enfant.</p><table class="AKH-zebra-table"><thead><tr><th style="width:50%;">Type de la maladie de von Willebrand</th><th style="text-align:center;width:50%;">Options de traitement les plus fréquentes</th></tr></thead><tbody><tr><td> <strong>Type 1</strong></td><td>DDAVP (sujet répondant); produit du facteur de von Willebrand.</td></tr><tr><td> <strong>Type 2</strong></td><td>DDAVP (sujet répondant); produit du facteur de von Willebrand.</td></tr><tr><td> <strong>Type 3</strong></td><td>Produit du facteur de von Willebrand.</td></tr></tbody></table>

 

 

Von Willebrand disease524.000000000000Von Willebrand diseaseVon Willebrand diseaseVEnglishHaematologyChild (0-12 years);Teen (13-18 years)BodyCardiovascular systemConditions and diseasesCaregivers Adult (19+)NA2017-05-08T04:00:00ZVanessa Bouskill, MN, RN(EC), NP;Manuel Carcao, MD, FRCP(C), FAAP, MScsusan.ball@sickkids.ca | Susan Ball | 693A30232E777C7369636B6B6964735C737573616E2062616C6C i:0#.w|sickkids\susan ball7.5000000000000066.70000000000001410.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Von Willebrand disease is a condition that affects the blood’s ability to clot properly. Learn about the different types of this disease and how they are treated.</p><h2>What is von Willebrand disease?</h2><p>Von Willebrand disease (VWD) is an inherited condition that affects the blood’s ability to clot effectively. It is a very common bleeding disorder that affects girls and boys equally.</p><p>When a person gets a cut, a type of cell in the blood called a platelet, and a protein called von Willebrand factor (VWF), stick to the wound to plug the cut. Proteins in the blood called clotting factors then bind together to form chains called fibrin. These chains then form a net around the platelets and VWF. This holds the clot together and helps to seal the wound.</p><p>In a person with VWD, there is either not enough VWF or it does not work properly. In either case, a blood clot cannot form properly, and the injured area will continue to bleed.</p> <p>VWF also protects and maintains levels of clotting factor VIII, one of the proteins involved in blood clotting. Therefore, people with VWD may have low levels of factor VIII in addition to low levels of VWF.</p><h2>Types and severity of von Willebrand disease</h2><p>People with VWD have increased bleeding episodes depending on the severity of their disease. When they have a bleeding episode, they bleed for a longer period of time than a person without VWD.</p><p>There are several types of VWD: Type 1, Type 2 and Type 3. These vary in severity of symptoms and not all people will experience all symptoms. Some people will have minimal symptoms or have no symptoms at all.</p><table class="AKH-zebra-table"><thead><tr><th style="width:25%;"></th><th style="text-align:center;width:25%;">Diagnosis</th><th style="text-align:center;width:25%;">Severity</th><th style="text-align:center;width:25%;">Symptoms</th></tr></thead><tbody><tr><td><strong>Type 1</strong></td><td>Low levels of VWF; may have slightly low factor VIII levels</td><td>Mild bleeding symptoms</td><td>Nosebleeds, mouth bleeds and heavy menstrual bleeding</td></tr><tr><td><strong>Type 2</strong></td><td>Levels of VWF may be normal, but it does not work properly</td><td>Mild to severe bleeding symptoms</td><td>Same as in Type 1, but more severe; Type 2N can experience joint and muscle bleeds</td></tr><tr><td><strong>Type 3</strong></td><td>Absent or extremely low levels of VWF; factor VIII levels will also be very low</td><td>Severe bleeding symptoms</td><td>In addition to Type 1 symptoms, will experience joint and muscle bleeds</td></tr></tbody></table><h3>Type 2 subtypes</h3><p>Type 2 VWD has four subtypes; treatment varies for each of these subtypes.</p><table class="AKH-zebra-table"><thead><tr><th style="width:25%;">Type 2 subtype</th><th style="text-align:center;width:75%;">Description</th></tr></thead><tbody><tr><td><strong>Type 2A</strong></td><td>This is the most common subtype. In this subtype, VWF does not work properly to bind platelets together.</td></tr><tr><td><strong>Type 2B</strong></td><td>In people with Type 2B, the VWF is “extra sticky” and it binds to platelets in the blood stream rather than at the site of the wound. This causes a shortage of platelets in areas where they are needed. Sometimes this type of VWD is misdiagnosed as a low platelet count such as <a href="/Article?contentid=842&language=English">idiopathic thrombocytopenia purpura (ITP)</a>.</td></tr><tr><td><strong>Type 2N</strong></td><td>In this subtype, the VWF binds to platelets at the site of the wound in the same way that it would in healthy people. However, while VWF normally transports clotting factor VIII, this does not occur in people with Type 2N. As a result, low levels of factor VIII lead to poor blood clot formation. Because of low levels of factor VIII, Type 2N VWD may be misdiagnosed as <a href="/Article?contentid=844&language=English">hemophilia</a>.</td></tr><tr><td><strong>Type 2M</strong></td><td>In this subtype, VWF does not bind properly to platelets. This subtype is similar to type 2A but is distinguished through various specialized laboratory tests.</td></tr></tbody></table><h2>Key points</h2><ul><li>The first signs of VWD are usually prolonged nosebleeds, easy bruising and in females heavy periods.</li><li>VWD is a lifelong condition that is usually passed down from parents to children. It equally affects both girls and boys.</li><li>Severity of VWD can vary, and thus treatments do as well. Options will be discussed between you and your child’s comprehensive health-care team.</li></ul><h2>Signs that your child may have von Willebrand disease</h2><p>If your child experiences any of the following symptoms, you should talk to your doctor about the possibility that your child may have VWD:</p><ul><li>bleeding from the gums</li><li>easy bruising</li><li>prolonged bleeding after cuts or bloodwork</li><li>frequent and prolonged nosebleeds</li><li>blood in the stool or urine</li><li>heavy or prolonged menstrual bleeding (menorrhagia) in girls</li><li>soft tissue/joint bleeding (in more severe forms).<br></li></ul><p>As VWD is usually inherited, it is possible that after a child in a family is diagnosed with VWD that other family members may be tested as well.</p><h2>How is von Willebrand disease inherited?</h2><p>VWD is a genetic condition and it is most often passed on from one or both parents to their children. Occasionally, VWD may occur when one of the child’s genes changes randomly; this is called a spontaneous genetic mutation.</p><div class="akh-series"><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 1, 2A, 2B and 2M von Willebrand disease</span><img alt="Inheritance pattern of Type 1, 2A, 2B and 2M von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_1_2A_2B_2M_EN.jpg" /> </figure> <p>The type of VWD depends on the mutation in the VWF gene. The parent may have mild symptoms or no symptoms at all. Types 1, 2A, 2B and 2M are usually inherited when one parent passes on the mutated VWF gene to their child.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 2N and 3 von Willebrand disease</span><img alt="Inheritance pattern of Type 2N and 3 von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_2N_3_EN.jpg" /> </figure> <p>Types 2N and 3 VWD are usually inherited when both parents pass on the mutated gene to their child. A child receives two mutated genes, one from each parent. Each parent may have only mild symptoms or have no symptoms at all.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 2N von Willebrand disease</span><img alt="Inheritance pattern of Type 2N von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_2N_EN.jpg" /> </figure> <p>Type 2N VWD occurs when a child inherits one Type 1 (or Type 2N) gene from one parent and one Type 2N gene from the other parent.</p></div></div><div class="row"><div class="col-md-12"> <figure> <span class="asset-image-title">Inheritance pattern of Type 3 von Willebrand disease</span><img alt="Inheritance pattern of Type 3 von Willebrand disease." src="https://assets.aboutkidshealth.ca/akhassets/von_willebrand_inheritance_3_EN.jpg" /> </figure> <p>Type 3 VWD occurs when a child inherits a gene responsible for Type 1 VWD from both parents. In most (but not all) cases parents of a child with Type 3 VWD, despite having a gene for type 1 VWD, usually have normal levels of VWF and show no signs of bleeding.</p></div></div></div><h2>Diagnosis of von Willebrand disease</h2><p>While VWD has a genetic cause, VWF can also be affected by many other things such as blood type, age, stress, medications and hormone levels.</p><p>Often, the symptoms of Type 1 VWD are very mild. For this reason, it can take time to diagnose.</p><p>A diagnosis of VWD involves a series of blood tests measuring VWF levels and function as well as factor VIII levels in the blood. A physical exam, a review of family medical history and a bleeding questionnaire will also be performed.</p><h2>Treatment of von Willebrand disease</h2><p>Children diagnosed with VWD are cared for by a comprehensive team of health care specialists. This team will include a nurse co-ordinator, hematologist (blood doctor), physiotherapist, social worker, dentist and, for female teens and adults, a gynecologist.<br></p><h3>Medications may be used to treat all types of von Willebrand disease</h3><p><em><strong>Tranexamic acid</strong></em></p><p>Tranexamic acid is used to hold a blood clot together once it has already formed. It is often used when a person with VWD is undergoing a surgical or dental procedure, and for nosebleeds or mouth bleeds. For females, it can also be used for heavy periods.</p><p><em><strong>Fibrin glue</strong></em></p><p>Fibrin is the final product when a blood clot is formed. Fibrin glue is made up from several blood clotting agents. It is applied directly to wounds to stop bleeding. Fibrin glue is often used when people with VWD undergo surgical or dental procedures.</p><p><em><strong>Desmopressin (DDAVP)</strong></em></p><p>DDAVP is a chemical that helps to release factor VIII and VWF that is already stored in the blood. Before using this method, your child’s comprehensive care team will perform a “DDAVP Challenge”. This test is done to see if the use of DDAVP releases enough factor VIII and VWF in your child’s body to treat bleeds. If it does, your child is a "responder". Treatment of VWD with DDAVP is not recommended until your child is at least three years of age because of potential side effects. DDAVP does not work in some Type 2 subtypes and Type 3 VWD.</p><p><em><strong>Von Willebrand factor product</strong></em></p><p>Von Willebrand factor product is a plasma derived factor concentrate (a powder) that has been engineered from human plasma. It undergoes viral inactivation steps to make sure the product is safe. VWF product is used in more moderate or severe bleeds, to replace the VWF and FVIII in the body immediately to stop bleeding.</p><h3>Treatment by type</h3><p>Medical treatment of VWD will vary by patient, and should be discussed with your child’s healthcare team.</p><table class="AKH-zebra-table"><thead><tr><th style="width:50%;">Type of von Willebrand disease</th><th style="text-align:center;width:50%;">Most common treatment options</th></tr></thead><tbody><tr><td><strong>Type 1</strong></td><td>DDAVP (if a responder); von Willebrand factor product</td></tr><tr><td><strong>Type 2</strong></td><td>DDAVP (if a responder); von Willebrand factor product</td></tr><tr><td><strong>Type 3</strong></td><td>von Willebrand factor product</td></tr></tbody></table>https://assets.aboutkidshealth.ca/AKHAssets/von_willebrand_disease.jpgVon Willebrand diseaseFalse

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