What is the treatment for von willebrand disease

PROFESSIONAL HIGHLIGHTS. She has been trained in Internal Medicine, Pulmonary Disease, Critical Care Medicine, and Anxiety Medicine. In addition, she was also trained in Thoracic Transplantation Medicine and Pulmonary Arterial Hypertension. CERTIFICATIONS Dr. Sarah Edwards is Board Certified in the following: • Internal Medicine • Child Diseases • Critical Medicine • She is also a Diplomate of The American Board of Anxiety Medicine. EDUCATION Postgraduate: • University of Nevada School of Medicine • Residency: Internal Medicine

Association of von Willebrand disease :

The von Willebrand disease (VFW) is a genetic disorder caused by missing defective von Willebrand Factor VWF a clotting protein VWF binds to factor eight, a key clotting protein, and platelets in the blood vessel walls which helps form a platelet plug during the clotting process. The condition is named after the physician Erik Von Willebrand who first described it in the 1920s. vWD is the most common bleeding disorder affecting the population worldwide. It is carried on chromosome 12 and occurs more frequently in men and women.
Symptoms with vWdD experience frequent nosebleeds, easy bruising, and excessive bleeding during and after invasive procedures such as tooth extraction and surgery. Women often experience heavy menstrual bleeding (heavy menstrual periods that last longer than average and hemorrhage after childbirth.[1]

Medical history is important to help determine if other relatives have been diagnosed with a bleeding disorder or have experienced symptoms. Tests that evaluate the clotting time of patients’ ability to form a clot may be ordered. Treatment depends on diagnosis and severity All these factors should be considered when diagnosed with von Willebrand’s disease and fruitful measures should be taken with chitosan hemostatic agents.

What are the symptoms of von Willebrand disease?

Some people have no symptoms. The most common symptom is mucocutaneous bleeding. This includes easy bruising, gum bleeding, nosebleeds, heavy menstrual periods, and unexpected bleeding after childbirth and surgery. Most people with type 1 or type 2 will have one or two of these symptoms. type 3 results are spontaneous, severe, and prolonged bleeding.

Two other conditions cause symptoms similar to type 1 and type 2 Von Willebrand disease: platelet function disorders and collagen vascular disorders. Type 3 Von Willebrand disease symptoms are similar to those seen in hemophilia. Besides problems unrelated to VWF and platelets can lead to mucocutaneous bleeding. Abnormal blood vessels and allergies may result in frequent nosebleeds and hormonal disruption can cause heavy menstrual periods.
Many people with von Willebrand disease have mild symptoms or none at all depending on the genetic defect. It may be only noticed after injury or medical procedure. Symptoms usually begin in childhood or waver throughout life.

This severity will vary from person to person. common symptoms include :

  • Easy bruising
  • Frequent nosebleeds
  • Prolonged bleeding from cuts or minor cuts or gums
  • Bloody urine

Prolonged bleeding after surgery injury childbirth and invasive dental procedures
Type 3 Von Willebrand disease will also result in severe bleeding with no apparent cause. This type can cause bleeding into the joints. This can cause pain and swelling in joints.

What causes von Willebrand’s disease?

The Von Willebrand Factor is a very large protein that helps platelets stick together to injured blood vessel walls. This makes the protein critical for clot formation. Inherited genes result in low amounts of VWF (type 2 disease )or near absence of VWF (type 3 disease ) Type1 is the most common and result in the mildest symptoms. Type 3 is rare and severe.
Von Willebrand’s disease is a genetic defect. The specific defect varies by the type of vWD

  • Type 1 – results in low levels of Von Willebrand factor
  • Type 2 – results in von Willebrand factor that does not work well
  • Type 3 -results in no von Willebrand factor.

This genetic defect is often inherited from the parent. A child can develop type 1 or type 2 vWD if just one parent has the defect. For type 3 both parents must have a defective gene. some people may have a gene without having symptoms. They can still pass the gene to the offspring. Von Willebrand’s disease can also develop from other medical conditions or medications. This is called von Willebrand syndrome.

Von Willebrand disease risk factors :

Having family members with Von Willebrand disease increases the risk of getting these diseases.

How is Von Willebrand disease diagnosed?

Diagnosing Von Willebrand Disease can be difficult. No one test definitively says yes or no to diagnosis. Instead, a panel of tests are run which looks at how much VWF is present and how well it is working. Stress, health, and physical activity may affect these numbers so borderline results often need to be repeated to be sure of the diagnosis. The best place to get these tests run is a children’s hospital laboratory with dedicated pediatric pathologists and hematologists to interpret the results.

Your doctor will ask you about symptoms and medical history. You will be specifically asked for bleeding events. Your doctor will suspect based on Von Willebrand’s disease. The blood test will confirm the diagnosis and determine which type of vWD you have. These tests will look at the different factors related to blood clotting. This also measures the amount of von Willebrand factor in your blood.

Complication associated with Von Willebrand disease.

Complications with type 1 and type 2 von Willebrand disease are rare. However significant trauma and head injury can result in abnormal bleeding into joints, the abdomen, and the brain so medical attention following injuries is important. Complications are common in kids with type 3 disease and these kids should avoid contact sports and activities associated with a high likelihood of injury.

Type 1 and type 2 Von Willebrand Disease does not affect day to day life or life expectancy. A patient needs to anticipate when problems could occur (surgery, dental procedures, menstruation, traumatic injury ) and follow their doctor’s advice on what to do in these situations. There is no way to prevent Von Willebrand Disease. It is an inherited disorder and cannot be prevented. At this time there is no cure, but research in gene therapy might provide a cure for those with severe disease in the future.

Preventative therapy

Those patients under the higher risk of the wound can take the help of chitosan which is the best hemostatic agent as described above. Now that you know what is Von Willebrand disease understanding platelet thrombogenicity is quite easily analyzed as compared to those not suffering from Von Willebrand disease. Chitosan gel can be applied to delayed wound healing topically for a faster process. Studies have revealed the wound healing progression of an ulcerated area using ISDN(isosorbide dinitrate ) plus chitosan was better at 30 – 45 days compared to placebo or ISDN.

There are various combination therapy that can be used to control the bleeding with chitosan or the progression of wound healing. Chitosan enhances the endothelial cells for cellular adhesion and helps in creating a clot by delaying the process of wound healing. These nanofibrous material works like a miracle in wound healing and helps in the process of antibacterial activity. These nanofibrous materials of chitin and chitosan have tremendous potential for wound healing.

Useful resources

  • https://www.cdc.gov/ncbddd/vwd/facts.html
  • https://ghr.nlm.nih.gov/condition/von-willebrand-disease
  • https://www.aafp.org/afp/2009/1201/p1261.html
  • https://rarediseases.org/rare-diseases/von-willebrand-disease/
  • https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=166081
  • https://www.nhs.uk/conditions/von-willebrand-disease/
  • https://www.sciencedirect.com/topics/medicine-and-dentistry/von-willebrand-disease
  • https://medlineplus.gov/ency/article/000544.htm
  • https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/von-willebrand-disease
  • https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2013/12/von-willebrand-disease-in-women
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