Materiały źródłowe

• #1 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Von Willebrand disease is a common autosomal inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor, a multi-adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in circulation. […] The aim of treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced von Willebrand factor and the concomitant deficiency of factor VIII. […] Desmopressin is the treatment of choice for type 1 von Willebrand disease patients with factor VIII and von Willebrand factor levels of 10 U/dL or over who have proved responsive to a test-infusion with the compound. […] Von Willebrand factor/factor VIII concentrates are needed when desmopressin is ineffective (mainly type 2 and 3 von Willebrand disease). […] The severity of the bleeding tendency is usually proportional to the degree of the primary deficiency of VWF and to that of the secondary deficiency of FVIII, as VWF is the carrier of FVIII in circulating plasma.

• #1 Von Willebrand Disease Treatment Guidelines | NBDF

  https://www.bleeding.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-266-masac-recommendations-regarding-the-treatment-of-von-willebrand-disease

  von Willebrand disease (VWD) is the most common inherited bleeding disorder and affects males and females equally in up to 1% of the general population. VWD is associated with mucous membrane bleeding, excessive bruising, and bleeding from cuts. It can result in excessive bleeding with invasive dental work, during surgical procedures, and after accident or injury. In women, heavy menstrual bleeding is often the major symptom. Women with VWD are also at risk of postpartum hemorrhage, particularly delayed postpartum hemorrhage. […] The following are current recommendations for treating bleeding in individuals with VWD. They are adapted from the ASH, ISTH, NHF, WFH 2021 VWD guidelines. […] Prior to surgery in a patient with VWD, consultation should be obtained with a pediatric or adult hematologist who specializes in the management of individuals with inherited bleeding disorders. This consultation should cover risk of bleeding with procedure and duration of risk. Treatment plan should be developed including such issues as the need for a DDAVP trial; type of fluid replacement or fluid restriction; dose and duration if DDAVP is to be used; appropriate dose, timing, and duration of factor replacement therapy; and use of adjunctive medications (antifibrinolytics and topical agents).

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  VON WILLEBRAND DISEASE TREATMENT […] If you are diagnosed with von Willebrand disease (VWD), you should be referred to a hematologist. They usually recommend that you avoid medications that interfere with the function of your platelets. These might include aspirin and medications known as nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (sample brand names: Advil, Motrin) and naproxen (sample brand name: Aleve). If you need a medication for pain or fever relief, acetaminophen (sample brand name: Tylenol) is a good alternative to aspirin and NSAIDs. If you take one of these medicines for another reason such as heart disease or stroke, do not stop them until you speak with the doctor who prescribed them. […] Depending on the severity of your VWD, you may also have to take special precautions to avoid injuries, such as avoiding contact sports. However, such precautions may not be necessary for most people with mild type 1 VWD.

• #1 Pediatric Von Willebrand Disease Treatment & Management: Medical Care, Consultations, Activity

  https://emedicine.medscape.com/article/959825-treatment

  Avoid medications with known antiplatelet effects. Although aspirin is rarely taken by children, over-the-counter compounds containing acetylsalicylic acid often are used by adolescents. Ibuprofen and other nonsteroidal anti-inflammatory drugs (NSAIDs) are reversible cyclooxygenase inhibitors and may cause intestinal bleeding. The risks of these and other medicines with antiplatelet effects should be considered in light of the severity of the von Willebrand disease. Provide patients with von Willebrand disease a list of prescription and nonprescription medications to avoid. This list should include the following. […] Over-the-counter medications to avoid include the following: Aspirin, Ibuprofen, Naproxen, Antihistamines, Ethanol. […] Antiplatelet agents to avoid include the following: Dipyridamole, Ticlopidine, Prescription nonsteroidal anti-inflammatory compounds.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  If you learn that you need a medical procedure or surgery, including dental surgery, you should discuss your VWD with everyone involved in the procedure ahead of time (hematologist, dentist, surgeon, anesthesiologist). Your type of VWD, previous history of bleeding, and options to control bleeding will be discussed. […] People with VWD will generally need closer-than-average monitoring during and after surgery. […] Some people will not need any additional treatment, depending on their type of VWD and the type of procedure. […] DDAVP – Some people can be treated with a medicine called DDAVP (also called desmopressin). This medicine helps your body release extra von Willebrand factor (VWF) into the bloodstream. However, this effect lasts only for 6 to 24 hours and may not be sufficient for some people. DDAVP can be given as a nasal spray, injected under the skin, or infused through an intravenous line. Doses are given 24 hours apart and should generally not be used for more than three days in a row.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  The only way to know if DDAVP will work for you is to do a test after your diagnosis is established and when you are not bleeding or otherwise sick. During the test, you are given a dose of DDAVP and your blood is checked before and after the dose to see if your VWF and factor VIII levels increase sufficiently, usually near or into the normal range. […] If you are helped by the DDAVP nose spray, it is important to use the form intended for VWD (there are other forms intended to treat other conditions, such as fluid disorders or bedwetting, which are not effective for treating VWD). […] It is also important to limit fluid intake for 12 to 24 hours while taking DDAVP, as DDAVP can cause water retention that can lead to serious symptoms, including seizures. Some older people or people with heart disease or a history of stroke may also need to avoid using DDAVP. DDAVP is generally avoided in patients <2 years of age.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  VWF concentrates – Some people will need a stronger VWF replacement treatment such as VWF concentrates; these products are purified from human plasma or produced in a laboratory. Examples of plasma-derived product brand names include Humate P, Alphanate, Koate, and Wilfactin. An example of a product manufactured in the laboratory is Vonvendi. These treatments are injected into a vein, often in the hospital or in a health care provider’s office, but some patients are taught how to self-inject this treatment at home. […] Treatment of another disorder – People with acquired VWS often benefit with improvement in their acquired von Willebrand syndrome (AVWS) when they are treated for the associated condition. The other measures listed above, and some additional medications, may also be given.

• #1 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Thus, in VWD the aim of therapy is to correct the dual defect of hemostasis, i.e. the abnormal platelet adhesion-aggregation and the abnormal intrinsic coagulation due to low FVIII levels. […] Desmopressin (DDAVP) and replacement therapy with VWF/FVIII concentrate or VWF concentrates devoid of FVIII are the mainstay of treatment. […] Those patients in whom a test infusion with desmopressin is not able to achieve clinically useful FVIII and/or VWF levels are candidates for replacement therapy. […] Candidates for replacement treatment should be vaccinated against hepatitis B, although the risk of infection by commercial concentrates nowadays is very small. […] The goal of treatment in patients undergoing major surgery is to maintain FVIII plasma levels around 80-100 U/dL for at least a couple of days and trough level above 50 U/dL for an additional 5-7 days thereafter.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  Medications that prevent clot breakdown – Individuals with VWD may be treated with a medication that prevents blood clots from dissolving, referred to as an antifibrinolytic agent. Examples of these types of medications include aminocaproic acid (sample brand name: Amicar) and tranexamic acid (sample brand name: Cyklokapron). These medications can be given in pill form, as a mouthwash, or as an injection into a vein. […] These medications are especially useful for managing bleeding from mucosal surfaces, such as nosebleeds, heavy menstrual bleeding, or bleeding from the mouth following dental work, and they are often given in addition to other treatments for VWD. […] Treatment at the bleeding site – Some people will also benefit from application of a foam or gel to the bleeding site that stimulates clots to form right at that spot.

• #1 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Some patients with severe forms of VWD (FVIII:C levels 5 U/dL) may have frequent hemarthroses or recurrent spontaneous bleeding (e.g. gastrointestinal bleeding and epistaxis) which can benefit from secondary long-term prophylaxis. […] Life-threatening anaphylactic reactions may occur in rare patients with type 3 VWD (typically homozygous for gene deletions or nonsense mutations) who develop alloantibodies when treated with concentrates containing VWF. […] Antifibrinolytic agents (i.e. tranexamic acid and epsilon aminocaproic acid), given orally, intravenously or topically, are useful alone or as adjuncts to replacement therapy for the prevention or treatment of bleeding in mucosal tracts, characterized by a high fibrinolytic activity. […] Estrogens-progestogen preparations may be useful to reduce the severity of menorrhagia in women with VWD, including those with type 3.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  Treatment for control of menstrual bleeding – Your doctor or nurse may refer you to a gynecologist who can determine whether the bleeding is caused by VWD or by something gynecologic such as a polyp. […] People with VWD who have heavy periods are sometimes treated with hormones, such as birth control pills or a progestin-releasing intrauterine device (IUD). […] Some people also benefit from DDAVP or an antifibrinolytic medicine that prevents the breakdown of clots (for example, tranexamic acid; sample brand name Cyklokapron). These treatments can reduce heavy menstrual bleeding. In some patients for whom these measures are not effective, a VWF concentrate is given. […] Treatment during pregnancy and childbirth – Many people with VWD who become pregnant can have a normal pregnancy without bleeding complications.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  However, as with surgery, it is important to have proper monitoring and to know what treatments will work well for you if you do have excessive bleeding. If you get pregnant or want to try to get pregnant, you should discuss your VWD with your hematologist. A specialist with expertise in managing pregnancy in people with VWD should be involved, and close monitoring during the pregnancy is appropriate. […] These discussions will help your health care team develop a plan to control any excessive bleeding before it becomes a problem. […] Many people will not need treatment while they are pregnant because VWF levels naturally increase during pregnancy. After delivery, however, levels of VWF can fall quickly and lead to more prolonged or heavier bleeding. One or more of the treatments described above may be used for one to three weeks following delivery.

• #1 Diagnosis and Management of Von Willebrand Disease: Guidelines for Primary Care | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1201/p1261.html

  Humate-P and Alphanate are currently the only plasma-derived VWF concentrates approved by the U.S. Food and Drug Administration for the treatment of VWD. […] The oral antifibrinolytic agents epsilon-aminocaproic acid (Amicar) and tranexamic acid (oral formulation no longer available in the United States) are very useful for mucous membrane bleeding, such as with dental procedures. […] Menorrhagia may be the first sign of VWD in women, although other causes of menorrhagia must be ruled out first. […] If menorrhagia is caused by VWD and the patient is not trying to get pregnant, combined oral contraceptives are the treatment of choice. […] Before or during pregnancy, women with VWD should be referred to a genetic counselor to discuss the inheritance of the disease, and to a pediatric hematologist for evaluation of the infant after delivery. […] Pregnant women with VWD who have factor VIII or VWF ristocetin cofactor (VWF:RCo) levels of less than 50 IU per dL, or a history of severe bleeding should be referred to a perinatal center that has a hemophilia treatment center or a hematologist with expertise in hemostasis.

• #1 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Pregnant women with VWD are at increased risk of postpartum hemorrhage if untreated. […] In type 3 VWD women, VWF and FVIII do not increase during pregnancy and thus VWF/FVIII concentrates may be required during pregnancy to control intermittent vaginal bleeding and at delivery or for Cesarean section.

• #1

  https://haematologica.org/article/view/6652

  Life-threatening anaphylactic reactions may occur in rare patients with type 3 VWD who develop alloantibodies when treated with concentrates containing VWF. […] Platelet concentrates may be of help in the very rare situations in which bleeding continues despite adequate replacement therapy. […] Antifibrinolytic agents, given orally, intravenously or topically, are useful alone or as adjuncts to replacement therapy for the prevention or treatment of bleeding in mucosal tracts. […] Estrogens-progestogen preparations may be useful to reduce the severity of menorrhagia in women with VWD, including those with type 3. […] Pregnant women with VWD are at increased risk of postpartum hemorrhage if untreated. […] In type 3 VWD women, VWF and FVIII do not increase during pregnancy and thus VWF/FVIII concentrates may be required during pregnancy to control intermittent vaginal bleeding and at delivery or for Cesarean section.

• #1 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  Treatment of severe bleeding – If you are in an accident or have severe bleeding with surgery despite receiving the treatments above, you may be given additional VWF, other medications, and/or platelet transfusions. It may be necessary to receive treatment for several days when severe bleeding occurs. […] It is important to carry information about your VWD, including what type of VWD you have (see 'Types of von Willebrand disease’ above) and a list of any other medical problems and medications on a medical identification card or bracelet. This will help your health care providers know what type of treatment is likely to work best for you. […] Treatment of recurrent bleeding – Some individuals with severe VWD who have repeated, frequent bleeding in an area such as in a joint or from the gastrointestinal tract may benefit from intravenous injections of a VWF concentrate on a regular basis, often two to three times weekly. This is referred to as „prophylaxis.”

• #1

  https://haematologica.org/article/view/6652

  Desmopressin (DDAVP) and replacement therapy with VWF/FVIII concentrate or VWF concentrates devoid of FVIII are the mainstay of treatment. […] Those patients in whom a test infusion with desmopressin is not able to achieve clinically useful FVIII and/or VWF levels are candidates for replacement therapy. […] Candidates for replacement treatment should be vaccinated against hepatitis B, although the risk of infection by commercial concentrates nowadays is very small. […] The goal of treatment in patients undergoing major surgery is to maintain FVIII plasma levels around 80-100 U/dL for at least a couple of days and trough level above 50 U/dL for an additional 5-7 days thereafter. […] Some patients with severe forms of VWD (FVIII:C levels 5 U/dL) may have frequent hemarthroses or recurrent spontaneous bleeding which can benefit from secondary long-term prophylaxis.

• #1 Von Willebrand Disease in Women | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2013/12/von-willebrand-disease-in-women

  Von Willebrand disease, the most common inherited bleeding disorder among American women, is a common cause of heavy menstrual bleeding and other bleeding problems in women and adolescent girls. […] A multidisciplinary approach to management, which involves obstetriciangynecologists and hematologists, results in optimal treatment outcomes. […] Many treatment options are available for women with von Willebrand disease and heavy menstrual bleeding, including hormonal and nonhormonal therapies. […] Collaboration with a hematologist is recommended to aid in the planning for gynecologic surgery and obstetric management. […] Patients should be reminded that products that prevent platelet adhesion, such as aspirin or nonsteroidal antiinflammatory drugs, should be avoided once von Willebrand disease is diagnosed.

• #1 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  Intramuscular injections should be avoided, something which should be remembered when vaccinations are given. […] If it is considered essential to vaccinate via an intramuscular injection, the bleeding disorder should be corrected first. […] The key to successful surgical management of a patient with a bleeding disorder is a multidisciplinary approach. […] Additionally, please seek advice from the haemophilia team regarding post-operative thromboprophylaxis requirements. […] The haemophilia specialist nurses will visit patients when they are on a ward, sometimes to take bloods or teach patients to self-infuse, and other times simply to offer support. […] We also provide informal teaching sessions and resources.

• #1 von Willebrand Disease Treatment & Symptoms Management | University of Utah Health | University of Utah Health

  https://healthcare.utah.edu/bleeding-clotting-disorders/von-willebrand-disease

  Von Willebrand disease (VWD) is a genetic bleeding disorder that is passed down from parent to child and affects both men and women. […] We treat von Willebrand disease using a multidisciplinary team approach: Hematologist, Nurse practitioner, Nurse, Physical therapist, Social worker, Pharmacist, Genetic counselor, Research coordinator. […] We treat von Willebrand disease using medications like DDAVP (desmopressin acetate). This drug will release von Willebrand factor into the bloodstream. […] Most people who respond well to DDAVP use a form that is squirted into the nose. It is known by the brand name Stimate. […] In general, people with mild to moderate VWD will experience minimal bleeding problems except when they have surgery or serious trauma. […] If you and your provider decide that surgery is the best treatment option, you will need to fill out a pre-surgery form.

• #1 von Willebrand Disease: What It Is, Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17709-von-willebrand-disease

  Most people with von Willebrand disease have mild or moderate symptoms. For them, living with von Willebrand disease may mean they should: […] Tell all healthcare providers, including their dentists, that they have the disease. That way, their healthcare providers can plan how to manage bleeding after surgery or dental surgery. […] Consider medical alert identification. Wearing a medical alert bracelet or carrying identification may help people get appropriate medical care in an emergency. […] Cleveland Clinic Childrens providers can accurately diagnose von Willebrand disease, a blood disorder. Well help your child manage symptoms and stay healthy.

• #1 Von Willebrand Disease (VWD)

  https://www.nationwidechildrens.org/conditions/health-library/von-willebrand-disease-vwd

  Menstruating girls with VWD may be treated with birth control pills. This can help control heavy menstrual bleeding. […] You can take these steps to reduce your child’s risk for bleeding: Don’t give your child medicines such as aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen. These can cause bleeding in children with VWD. Ask your child’s healthcare provider for a list of medicines and supplements to avoid. […] Educate all caregivers, from teachers to sports coaches, about the disease and treatment in case of an emergency. […] Call your child’s healthcare provider if your child has bleeding that you can’t stop. If your child has pain or swelling, call the provider right away. […] VWD is diagnosed with blood tests. […] Medicines may be used to stop bleeding or to prevent bleeding with planned procedures. […] People with VWD should not take aspirin or NSAIDs. […] All caregivers should be educated about your child’s condition and what to do in an emergency.

• #1 Medical IDs for Von Willebrand Disease | MedicAlert Foundation

  https://www.medicalert.org/medical-conditions/von-willebrand-disease/?srsltid=AfmBOopkEHKLUfkqqLl3lObgdIcY6Cu8o_RXOqA0UL2kbnT4avoiAKn5

  Share the information thats important to your care, such as use of rescue medications or contraindication for tests like MRIs. […] Having a medical ID for Von Willebrand disease helps calm your nerves in situations where emergency medical care is required. Let MedicAlert bolster your voice and give doctors instant access to your medical history for the fastest best possible treatment. […] Help others help you. Wear a medical alert ID bracelet or necklace engraved with important information for emergency responders and healthcare providers. […] Treatment of von Willebrand disease depends on the type of VWD a person has and how severe their symptoms are. Some treatments may be used in particular as prevention of complications due to surgery or dental work. […] Its important to work with your doctor on a treatment plan that works to keep you safe in an emergency. This includes wearing medical IDs for Von Willebrand disease at all times so that your history of VWD and any on-demand medications you use is available to first responders in an emergency.

• #1 Pediatric Von Willebrand Disease Treatment & Management: Medical Care, Consultations, Activity

  https://emedicine.medscape.com/article/959825-treatment

  In general, a patient’s responsiveness to DDAVP prior to its use for these purposes can be determined. Once determined, such responsiveness is generally consistent in patients over time and within families. In patients with serious bleeding, prompt treatment is important in order to decrease the possibility of complications. […] Remember that in type 2B von Willebrand disease, DDAVP may cause a paradoxical drop in the platelet count and should not be used in a therapeutic setting without prior testing to see how the patient responds. Some other forms of type 2 von Willebrand disease may not be responsive to DDAVP either. […] Consult a pediatric hematologist. […] No evidence suggests that extensive activity restrictions are necessary for most patients with mild type 1 von Willebrand disease. Patients with more severe forms of von Willebrand disease should follow guidelines developed for patients with severe hemophilia, including considering a prophylactic regimen in patients with severe type 3 who are active or have repeated bleeding.

• #1 Articles :: The Health Plan

  https://www.healthplan.org/library/articles/hw183231

  Von Willebrand disease is a bleeding disorder. When you have this problem, it takes longer for your blood to form clots, so you bleed for a longer time than other people. […] If you have von Willebrand disease, your treatment may include: Desmopressin medicine (such as DDAVP, Stimate) which helps your body release more of the von Willebrand clotting factor into your blood. […] If you have von Willebrand disease, you most likely will need to take extra care to treat and prevent bleeding episodes. Avoid NSAIDs and blood thinners. Try to stay at a healthy weight and keep active. This helps keep your muscles and joints healthy. Avoid sports or activities where injury and bleeding are likely, such as football and hockey. […] Tell all your doctors and other health professionals, such as your dentist, that you have this disease. Doctors need to know about it before you have any procedures, because you may be at risk for dangerous bleeding. […] Most large hospitals have a „bleeding disorders” resource center. Learn the phone number to the center closest to you, and carry it with you.

• #1 Von Willebrand disease | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/von-willebrand-disease

  If you have VWD, you can still go to school, have adventures, play sports, raise a family, work, travel, and do the things that inspire you or enjoy the fun activities you love. […] Exercising regularly and being physically active is a great way to keep muscles and joints strong and stay in good health. Exercise can help to boost VWF levels and may even reduce the number of bleeding problems and episodes you experience. […] If you are planning to have a child, talk to your haemophilia treatment service and your obstetrician before you get pregnant, and to ask them to liaise with each other. […] If you are a woman or girl with VWD, a holistic or comprehensive care approach to your health care can help you to achieve better health and quality of life. Specialist gynaecological care over your lifetime, including during family planning, is important to manage any gynaecological issues that occur.

• #1 Von Willebrand disease – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/diagnosis-treatment/drc-20354984

  Mild forms of von Willebrand disease can be difficult to diagnose because bleeding is common, and, for most people, doesn’t indicate a disease. […] Your doctor might suggest one or more of the following treatments to increase your von Willebrand factor, strengthen blood clots or control heavy menstrual bleeding: […] Many doctors consider desmopressin (DDAVP) the first treatment for managing von Willebrand disease. It can be used before minor surgical procedures to help control bleeding. […] If your condition is mild, your doctor might recommend treatment only when you’re having surgery or dental work or when you’ve had a trauma, such as a car accident. […] These self-care tips can help you manage your condition: […] Let your doctors or dentist know that you have von Willebrand disease before you have surgery, start a new medication or give birth. […] While you wait for your appointment, avoid pain relievers that can increase your risk of bleeding, such as aspirin, ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve).

• #1 Von Willebrand disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/symptoms-causes/syc-20354978

  Von Willebrand disease can’t be cured. But with treatment and self-care, most people with this disease can lead active lives. […] Contact your doctor if you have bleeding that lasts a long time or is hard to stop. […] If you plan to have children and have a family history of von Willebrand disease, consider genetic counseling. If you carry the gene for von Willebrand disease, you can pass it on to your offspring, even if you don’t have symptoms.

• #1 von Willebrand disease 101

  https://www.myamericannurse.com/von-willebrand-disease-101/

  Most people with VWD live normal daily lives, but complications can arise with surgery or trauma. It’s important for caregivers in the hospital and other healthcare facilities to be familiar with VWD and the steps to take to ensure hemostasis and decrease the risk of complications related to the bleeding disorder. This is effectively done through education and collaboration with a hematologist. […] Patients with VWD should avoid medications that can increase bleeding risks, for example, aspirin and nonsteroidal antiinflammatory drugs (NSAIDS). Pain relievers such as acetaminophen are a better choice because they don’t increase the risk of bleeding. […] Treatment depends on the type of VWD and should be decided by a hematologist. Options include the following: Hormonal treatments such as oral contraceptives and some intrauterine devices are highly effective in controlling menorrhagia. In fact, 88% of women with VWD report improvement in bleeding symptoms when treated only with oral contraceptives.

• #1 43 | Help, my patient is bleeding! How to care for patients with von Willebrand’s Disease — Veterinary Internal Medicine Nursing

  https://www.veterinaryinternalmedicinenursing.com/blog/episode-43

  Alongside increasing their vWF levels and providing supportive care, our nursing aims to prevent further bleeding. Careful handling, minimal restraint, and careful venepuncture/sample collection are needed. […] Certain medications also impact platelet function (such as NSAIDs, certain antibiotics and certain antihistamines), and their use should be minimised in patients with vWD, as they can increase the risk of spontaneous bleeding in these patients. […] Lastly, client education is one of the most significant long-term areas where nurses and technicians make a difference to these patients. With some lifestyle adjustments to prevent injury or wounds and careful planning ahead of surgical procedures, these patients can have a completely normal life expectancy, and the veterinary nurse is vital in supporting clients throughout their pets life. […] As nurses and technicians, were instrumental in that managementfrom administering transfusions to monitoring, careful handling, and client support, our role in supporting these patients is vast.

• #1 Von Willebrand Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/v/von-willebrand-disease

  Children with von Willebrand disease have good long-term outcomes. With excellent care and proper treatment, most people with von Willebrand disease can lead normal, healthy lives. […] People with von Willebrand disease should not use nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen, naproxen and aspirin. These drugs can make you bleed more.

• #2

  https://haematologica.org/article/view/6652

  Von Willebrand disease is a common autosomal inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor, a multi-adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in circulation. […] The aim of treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced von Willebrand factor and the concomitant deficiency of factor VIII. Desmopressin is the treatment of choice for type 1 von Willebrand disease patients with factor VIII and von Willebrand factor levels of 10 U/dL or over who have proved responsive to a test-infusion with the compound. […] The severity of the bleeding tendency is usually proportional to the degree of the primary deficiency of VWF and to that of the secondary deficiency of FVIII, as VWF is the carrier of FVIII in circulating plasma.

• #2 Von Willebrand Disease Treatment Guidelines | NBDF

  https://www.bleeding.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-266-masac-recommendations-regarding-the-treatment-of-von-willebrand-disease

  The ASH ISTH NHF WFH 2021 guidelines on the management of VWD suggest the following: Desmopressin should not be used for major surgery. […] In patients undergoing minor surgery or minor invasive procedures, the panel suggests increasing VWF activity levels to 0.50 IU/mL with desmopressin or factor concentrate with the addition of tranexamic acid over raising VWF levels to 0.50 IU/mL with desmopressin or factor concentrate alone.

• #2 von Willebrand disease 101

  https://www.myamericannurse.com/von-willebrand-disease-101/

  Most people with VWD live normal daily lives, but complications can arise with surgery or trauma. It’s important for caregivers in the hospital and other healthcare facilities to be familiar with VWD and the steps to take to ensure hemostasis and decrease the risk of complications related to the bleeding disorder. This is effectively done through education and collaboration with a hematologist. […] Patients with VWD should avoid medications that can increase bleeding risks, for example, aspirin and nonsteroidal antiinflammatory drugs (NSAIDS). Pain relievers such as acetaminophen are a better choice because they don’t increase the risk of bleeding. […] Treatment depends on the type of VWD and should be decided by a hematologist. Options include the following: Hormonal treatments such as oral contraceptives and some intrauterine devices are highly effective in controlling menorrhagia. In fact, 88% of women with VWD report improvement in bleeding symptoms when treated only with oral contraceptives.

• #2 Pediatric Von Willebrand Disease Treatment & Management: Medical Care, Consultations, Activity

  https://emedicine.medscape.com/article/959825-treatment

  Avoid medications with known antiplatelet effects. Although aspirin is rarely taken by children, over-the-counter compounds containing acetylsalicylic acid often are used by adolescents. Ibuprofen and other nonsteroidal anti-inflammatory drugs (NSAIDs) are reversible cyclooxygenase inhibitors and may cause intestinal bleeding. The risks of these and other medicines with antiplatelet effects should be considered in light of the severity of the von Willebrand disease. Provide patients with von Willebrand disease a list of prescription and nonprescription medications to avoid. This list should include the following. […] Over-the-counter medications to avoid include the following: Aspirin, Ibuprofen, Naproxen, Antihistamines, Ethanol. […] Antiplatelet agents to avoid include the following: Dipyridamole, Ticlopidine, Prescription nonsteroidal anti-inflammatory compounds.

• #2 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Thus, in VWD the aim of therapy is to correct the dual defect of hemostasis, i.e. the abnormal platelet adhesion-aggregation and the abnormal intrinsic coagulation due to low FVIII levels. […] Desmopressin (DDAVP) and replacement therapy with VWF/FVIII concentrate or VWF concentrates devoid of FVIII are the mainstay of treatment. […] Those patients in whom a test infusion with desmopressin is not able to achieve clinically useful FVIII and/or VWF levels are candidates for replacement therapy. […] Candidates for replacement treatment should be vaccinated against hepatitis B, although the risk of infection by commercial concentrates nowadays is very small. […] The goal of treatment in patients undergoing major surgery is to maintain FVIII plasma levels around 80-100 U/dL for at least a couple of days and trough level above 50 U/dL for an additional 5-7 days thereafter.

• #2 von Willebrand Disease: What It Is, Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17709-von-willebrand-disease

  Healthcare providers treat this disorder with medication to help with blood clotting. […] Healthcare providers may treat this condition with different medications: […] Desmopressin: This hormone boosts the levels of von Willebrand factor in your bloodstream. This is the most common treatment for von Willebrand disease. […] von Willebrand factor infusions: Some people may receive infusions of von Willebrand factor to stop bleeding episodes. […] Antifibrinolytics: These medications keep blood clots from breaking down. […] Birth control pills: This medication helps people with menstrual bleeding. […] Most people have Type 1 or Type 2 von Willebrand disease and may only need treatment if they’re injured or need surgery. People with Type 3 von Willebrand disease may need ongoing medical treatment to manage bleeding.

• #2 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  The only way to know if DDAVP will work for you is to do a test after your diagnosis is established and when you are not bleeding or otherwise sick. During the test, you are given a dose of DDAVP and your blood is checked before and after the dose to see if your VWF and factor VIII levels increase sufficiently, usually near or into the normal range. […] If you are helped by the DDAVP nose spray, it is important to use the form intended for VWD (there are other forms intended to treat other conditions, such as fluid disorders or bedwetting, which are not effective for treating VWD). […] It is also important to limit fluid intake for 12 to 24 hours while taking DDAVP, as DDAVP can cause water retention that can lead to serious symptoms, including seizures. Some older people or people with heart disease or a history of stroke may also need to avoid using DDAVP. DDAVP is generally avoided in patients <2 years of age.

• #2 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Von Willebrand disease is a common autosomal inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor, a multi-adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in circulation. […] The aim of treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced von Willebrand factor and the concomitant deficiency of factor VIII. […] Desmopressin is the treatment of choice for type 1 von Willebrand disease patients with factor VIII and von Willebrand factor levels of 10 U/dL or over who have proved responsive to a test-infusion with the compound. […] Von Willebrand factor/factor VIII concentrates are needed when desmopressin is ineffective (mainly type 2 and 3 von Willebrand disease). […] The severity of the bleeding tendency is usually proportional to the degree of the primary deficiency of VWF and to that of the secondary deficiency of FVIII, as VWF is the carrier of FVIII in circulating plasma.

• #2 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  Medications that prevent clot breakdown – Individuals with VWD may be treated with a medication that prevents blood clots from dissolving, referred to as an antifibrinolytic agent. Examples of these types of medications include aminocaproic acid (sample brand name: Amicar) and tranexamic acid (sample brand name: Cyklokapron). These medications can be given in pill form, as a mouthwash, or as an injection into a vein. […] These medications are especially useful for managing bleeding from mucosal surfaces, such as nosebleeds, heavy menstrual bleeding, or bleeding from the mouth following dental work, and they are often given in addition to other treatments for VWD. […] Treatment at the bleeding site – Some people will also benefit from application of a foam or gel to the bleeding site that stimulates clots to form right at that spot.

• #2 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  However, as with surgery, it is important to have proper monitoring and to know what treatments will work well for you if you do have excessive bleeding. If you get pregnant or want to try to get pregnant, you should discuss your VWD with your hematologist. A specialist with expertise in managing pregnancy in people with VWD should be involved, and close monitoring during the pregnancy is appropriate. […] These discussions will help your health care team develop a plan to control any excessive bleeding before it becomes a problem. […] Many people will not need treatment while they are pregnant because VWF levels naturally increase during pregnancy. After delivery, however, levels of VWF can fall quickly and lead to more prolonged or heavier bleeding. One or more of the treatments described above may be used for one to three weeks following delivery.

• #2

  https://haematologica.org/article/view/6652

  Life-threatening anaphylactic reactions may occur in rare patients with type 3 VWD who develop alloantibodies when treated with concentrates containing VWF. […] Platelet concentrates may be of help in the very rare situations in which bleeding continues despite adequate replacement therapy. […] Antifibrinolytic agents, given orally, intravenously or topically, are useful alone or as adjuncts to replacement therapy for the prevention or treatment of bleeding in mucosal tracts. […] Estrogens-progestogen preparations may be useful to reduce the severity of menorrhagia in women with VWD, including those with type 3. […] Pregnant women with VWD are at increased risk of postpartum hemorrhage if untreated. […] In type 3 VWD women, VWF and FVIII do not increase during pregnancy and thus VWF/FVIII concentrates may be required during pregnancy to control intermittent vaginal bleeding and at delivery or for Cesarean section.

• #2 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  Treatment for control of menstrual bleeding – Your doctor or nurse may refer you to a gynecologist who can determine whether the bleeding is caused by VWD or by something gynecologic such as a polyp. […] People with VWD who have heavy periods are sometimes treated with hormones, such as birth control pills or a progestin-releasing intrauterine device (IUD). […] Some people also benefit from DDAVP or an antifibrinolytic medicine that prevents the breakdown of clots (for example, tranexamic acid; sample brand name Cyklokapron). These treatments can reduce heavy menstrual bleeding. In some patients for whom these measures are not effective, a VWF concentrate is given. […] Treatment during pregnancy and childbirth – Many people with VWD who become pregnant can have a normal pregnancy without bleeding complications.

• #2 Principles of care for the diagnosis and treatment of von Willebrand disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3640108/

  Some patients with severe forms of VWD (FVIII:C levels 5 U/dL) may have frequent hemarthroses or recurrent spontaneous bleeding (e.g. gastrointestinal bleeding and epistaxis) which can benefit from secondary long-term prophylaxis. […] Life-threatening anaphylactic reactions may occur in rare patients with type 3 VWD (typically homozygous for gene deletions or nonsense mutations) who develop alloantibodies when treated with concentrates containing VWF. […] Antifibrinolytic agents (i.e. tranexamic acid and epsilon aminocaproic acid), given orally, intravenously or topically, are useful alone or as adjuncts to replacement therapy for the prevention or treatment of bleeding in mucosal tracts, characterized by a high fibrinolytic activity. […] Estrogens-progestogen preparations may be useful to reduce the severity of menorrhagia in women with VWD, including those with type 3.

• #2 Von Willebrand Disease in Women | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2013/12/von-willebrand-disease-in-women

  Therapies generally prescribed in conjunction with a hematologist once a diagnosis of von Willebrand disease is established include desmopressin acetate, recombinant factor VIII, and vWF complex infusion. […] A multidisciplinary approach to management, which involves both obstetriciangynecologists and hematologists, results in optimal treatment outcomes. […] Many resources on bleeding disorders exist for patients and health care providers through the National Heart, Lung, and Blood Institute; National Hemophilia Foundation; and the American Society of Hematology.

• #2 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  VON WILLEBRAND DISEASE TREATMENT […] If you are diagnosed with von Willebrand disease (VWD), you should be referred to a hematologist. They usually recommend that you avoid medications that interfere with the function of your platelets. These might include aspirin and medications known as nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (sample brand names: Advil, Motrin) and naproxen (sample brand name: Aleve). If you need a medication for pain or fever relief, acetaminophen (sample brand name: Tylenol) is a good alternative to aspirin and NSAIDs. If you take one of these medicines for another reason such as heart disease or stroke, do not stop them until you speak with the doctor who prescribed them. […] Depending on the severity of your VWD, you may also have to take special precautions to avoid injuries, such as avoiding contact sports. However, such precautions may not be necessary for most people with mild type 1 VWD.

• #2 Von Willebrand Disease (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/vwd.html

  Von Willebrand disease, or VWD, is a genetic (inherited) bleeding disorder that prevents blood from clotting properly. […] The most common treatment for VWD is desmopressin. This synthetic (manmade) hormone causes a temporary increase in the Von Willebrand factor and factor VIII levels. It can be given as an injection or a nasal spray. But it doesn’t work for everyone and may not be helpful in treating type 2. Some patients will need treatment with an intravenous (IV, given into a vein) form of Von Willebrand factor. […] If bleeding happens, apply pressure to the area. […] Tell your child’s hematologist if any surgery or procedures are planned. […] Call your doctor right away if your child has any excessive or unexplained bleeding.

• #2 Caring for patients with inherited bleeding disorders | Nursing Times

  https://www.nursingtimes.net/haematology/caring-for-patients-with-inherited-bleeding-disorders-04-09-2020/

  Intramuscular injections should be avoided, something which should be remembered when vaccinations are given. […] If it is considered essential to vaccinate via an intramuscular injection, the bleeding disorder should be corrected first. […] The key to successful surgical management of a patient with a bleeding disorder is a multidisciplinary approach. […] Additionally, please seek advice from the haemophilia team regarding post-operative thromboprophylaxis requirements. […] The haemophilia specialist nurses will visit patients when they are on a ward, sometimes to take bloods or teach patients to self-infuse, and other times simply to offer support. […] We also provide informal teaching sessions and resources.

• #2 43 | Help, my patient is bleeding! How to care for patients with von Willebrand’s Disease — Veterinary Internal Medicine Nursing

  https://www.veterinaryinternalmedicinenursing.com/blog/episode-43

  Alongside increasing their vWF levels and providing supportive care, our nursing aims to prevent further bleeding. Careful handling, minimal restraint, and careful venepuncture/sample collection are needed. […] Certain medications also impact platelet function (such as NSAIDs, certain antibiotics and certain antihistamines), and their use should be minimised in patients with vWD, as they can increase the risk of spontaneous bleeding in these patients. […] Lastly, client education is one of the most significant long-term areas where nurses and technicians make a difference to these patients. With some lifestyle adjustments to prevent injury or wounds and careful planning ahead of surgical procedures, these patients can have a completely normal life expectancy, and the veterinary nurse is vital in supporting clients throughout their pets life. […] As nurses and technicians, were instrumental in that managementfrom administering transfusions to monitoring, careful handling, and client support, our role in supporting these patients is vast.

• #2 Von Willebrand disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/symptoms-causes/syc-20354978

  Von Willebrand disease can’t be cured. But with treatment and self-care, most people with this disease can lead active lives. […] Contact your doctor if you have bleeding that lasts a long time or is hard to stop. […] If you plan to have children and have a family history of von Willebrand disease, consider genetic counseling. If you carry the gene for von Willebrand disease, you can pass it on to your offspring, even if you don’t have symptoms.

• #3 Pediatric Von Willebrand Disease Treatment & Management: Medical Care, Consultations, Activity

  https://emedicine.medscape.com/article/959825-treatment

  Antimicrobials to avoid include the following: High-dose penicillins, Cephalosporins, Nitrofurantoin, Hydroxychloroquine. […] Cardiovascular medications to avoid include the following: Propranolol, Furosemide, Calcium channel blockers, Quinidine. […] Other agents to avoid include the following: Caffeine, Tricyclic antidepressants, Phenothiazines, Valproate, Heparin.

• #3

  https://haematologica.org/article/view/6652

  Desmopressin (DDAVP) and replacement therapy with VWF/FVIII concentrate or VWF concentrates devoid of FVIII are the mainstay of treatment. […] Those patients in whom a test infusion with desmopressin is not able to achieve clinically useful FVIII and/or VWF levels are candidates for replacement therapy. […] Candidates for replacement treatment should be vaccinated against hepatitis B, although the risk of infection by commercial concentrates nowadays is very small. […] The goal of treatment in patients undergoing major surgery is to maintain FVIII plasma levels around 80-100 U/dL for at least a couple of days and trough level above 50 U/dL for an additional 5-7 days thereafter. […] Some patients with severe forms of VWD (FVIII:C levels 5 U/dL) may have frequent hemarthroses or recurrent spontaneous bleeding which can benefit from secondary long-term prophylaxis.

• #3 Patient education: von Willebrand disease (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/von-willebrand-disease-beyond-the-basics

  Treatment for control of menstrual bleeding – Your doctor or nurse may refer you to a gynecologist who can determine whether the bleeding is caused by VWD or by something gynecologic such as a polyp. […] People with VWD who have heavy periods are sometimes treated with hormones, such as birth control pills or a progestin-releasing intrauterine device (IUD). […] Some people also benefit from DDAVP or an antifibrinolytic medicine that prevents the breakdown of clots (for example, tranexamic acid; sample brand name Cyklokapron). These treatments can reduce heavy menstrual bleeding. In some patients for whom these measures are not effective, a VWF concentrate is given. […] Treatment during pregnancy and childbirth – Many people with VWD who become pregnant can have a normal pregnancy without bleeding complications.

Zobacz więcej