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von willebrand disease | What are the 3 types of von Willebrand disease?

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Von Willebrand disease (VWD)

Von Willebrand disease (VWD) could be a blood disease during which the blood doesn't clot properly. Blood contains several proteins that facilitate the grume once required. one amongst these proteins is termed Erik Adolf von Willebrand issue (VWF). individuals with VWD either have an occasional level of VWF in their blood or the VWF supermolecular doesn’t work the approach it ought to.

Normally, once an individual is bruised and starts to bleed, the VWF within the blood attaches to tiny blood cells referred to as platelets. This helps the platelets rest, like glue, to create a clot at the location of injury and stop the haemorrhage. once an individual has VWD, as a result of the VWF doesn’t work the approach it ought to, the clot may take longer type|to make|to create} or form incorrectly and haemorrhage may take longer to prevent. this may cause significant, hard-to-stop haemorrhage. though rare, the haemorrhage may be serious enough to wreck joints or internal organs, or perhaps be serious.


Who is Affected by Von Willebrand disease (VWD)? 


VWD is that the commonest haemorrhage disorder, found in up to a quarter of the U.S. population. this suggests that three.2 million (or regarding one in each 100) individuals within the u.  s. have the disease. though VWD happens among men and girls equally, girls square measure additional seemingly to note the symptoms because of significant or abnormal haemorrhage throughout their discharge periods and once parturition. There square measure three major styles of VWD: sort one, Type 2, and Type 3.


Types of Von Willebrand disease (VWD)


🔵Type 1

This is the foremost common and mildest variety of VWD, during which an individual has lower-than-normal levels of VWF. an individual with sort one VWD conjointly may need low levels of U Hemofil (8), another form of blood-clotting supermolecular. regarding eighty fifth of individuals treated for VWD have sort one.

🔵Type 2

With this kind of VWD, though the body makes traditional amounts of the VWF, the issue doesn't work the approach it ought to. sort two is more attenuated into four subtypes―2A, 2B, 2M, and 2N―depending on the precise downside with the person’s VWF. as a result of the treatment is completely different for every sort, it’s necessary that an individual recognize that subtype he or she has.

In Type 2A, the VWF isn't the correct size and doesn’t facilitate the platelets attach along so as to create a clot.

In Type 2B, the VWF attaches to platelets at the incorrect time (when there's no injury). 

In Type 2N, the VWF attaches to the platelets unremarkably. However, the VWF doesn't attach to a different supermolecular, Hemofil (8), that is additionally required for blood to clot. This causes the body to get rid of the Hemofil (8) supermolecular.

🔵Type 3

This is the foremost severe variety of VWD, during which an individual has little or no VWF and low levels of Hemofil. this is often the rarest form of VWD. solely three-d of individuals with VWD have sort three.


Causes of Von Willebrand disease (VWD)


Most people World Health Organization have VWD square measure born with it. It nearly always is familial, or passed down, from a parent to a toddler. VWD may be passed down from either the mother or the daddy, or both, to the kid.

While rare, it's doable for an individual to induce VWD while not a case history of the disease. this may happen if a spontaneous mutation occurs. which means there has been a amendment within the person’s sequence. whether or not a toddler receives the affected sequence from a parent or as a results of a mutation, once the kid has it, the kid will later pass it on to his or her kids.


Also, it is rare, however doable, for an individual to induce or acquire VWD (they didn’t receive the affected sequence from their parent or as a results of a mutation) later in life due to AN underlying medical condition.  utilization of a drugs or as a results of another disease. If VWD is non-heritable during this approach, it can't be passed on to any kids.


Signs and Symptoms of Von Willebrand disease (VWD)


The major signs of VWD are:


☑️Frequent or Hard-to-Stop Nosebleeds

☑️People with VWD may need nosebleeds that

☑️Start while not injury (spontaneous)

☑️Occur usually, sometimes 5 times or additional in an exceedingly year

☑️Last over ten minutes

☑️Need packing (gauze placed within the nose) or cautery (a procedure to burn and seal blood vessels) to prevent the haemorrhage

☑️Easy Bruising

☑️People with VWD may expertise straightforward bruising that

☑️Occurs with little or no trauma or injury

☑️Occurs usually (one to fourfold per month)

☑️Is larger than the scale of 1 / 4

☑️Is not flat and features a raised lump

☑️Heavy discharge haemorrhage


Women with VWD may have heavy discharge periods during that they expertise


☑️Soaking through a pad or tampon each 1-2 hours (or additional often) on the heaviest day(s)

☑️Menstrual haemorrhage that lasts longer than seven days from the time haemorrhage starts till the time it ends

☑️Flooding or gushing of blood

☑️Passing blood clots (tissue) larger than the scale of grapes or strawberries


☑️A diagnosing of anemia (not having enough red blood cells) is formed as a results of haemorrhage from significant periods

☑️Longer than traditional haemorrhage once Injury, Surgery, Childbirth, or Dental Work

☑️People with VWD may need longer than traditional haemorrhage once injury, surgery, or parturition. This haemorrhage could also be characterised within the following ways:

☑️After a move the skin, the haemorrhage lasts over five minutes

☑️Heavy or longer haemorrhage happens once surgery. haemorrhage generally stops however starts up once more hours or days later.

☑️Heavy haemorrhage happens throughout or once parturition

☑️People with VWD may need longer-than-normal haemorrhage throughout or once dental work, for example:

☑️Heavy haemorrhage happens throughout or once odontology

☑️The surgery website oozes blood longer than three hours once the surgery

☑️The surgery website wants packing or cautery to prevent the haemorrhage

☑️The amount of haemorrhage depends on the kind and severity of VWD. different common haemorrhage events include:

☑️Blood within the stool (feces) from haemorrhage into the abdomen or intestines

☑️Blood within the excretory product from haemorrhage into the kidneys or bladder

☑️Bleeding into joints or internal organs in severe cases (Type three VWD)


Diagnosis of Von Willebrand disease (VWD)


To find out if an individual has VWD, the doctor can raise questions about personal and family histories of haemorrhage. The doctor conjointly can check for uncommon bruising or different signs of recent haemorrhage and order some blood tests to live however the blood clots. The tests can offer data regarding the quantity of curdling proteins gift within the blood and if the curdling proteins square measure operating properly. as a result of bound medications will cause haemorrhage, even among individuals while not a haemorrhage disorder, the doctor can raise regarding recent or routine medications taken that would cause haemorrhage or create haemorrhage symptoms worse.


Treatments of Von Willebrand disease (VWD)


The type of treatment prescribed for VWD depends on the kind and severity of the disease. For minor bleeds, treatment may not be required.


The most normally used styles of treatment are:


🔵Desmopressin Acetate Injection


This drugs (DDAVP®) is injected into a vein to treat individuals with milder sorts of VWD (mainly sort 1). It works by creating the body unharness additional VWF into the blood. It conjointly helps increase the extent of Hemofil within the blood.

🔵Desmopressin Acetate Nasal Spray

This high-strength nasal spray (Stimate®) is employed to treat individuals with milder sorts of VWD (mainly sort 1). It works by creating the body unharness additional VWF into the blood.

🔵Factor Replacement medical aid

Recombinant VWF (such as Vonvendi®) and medicines made in VWF and Hemofil (for example, Humate P®, Wilate®, Alphanate®, or Koate DVI®) square measure accustomed treat individuals with additional severe sorts of VWD or individuals with milder sorts of VWD World Health Organization don't respond well to the nasal spray. These medicines square measure injected into a vein within the arm to switch the missing think about the blood.

🔵Antifibrinolytic medicine

These medicine (for example, Amicar®, Lysteda®) square measure either injected or taken orally to assist slow or stop the breakdown of blood clots.

🔵Birth Control Pills

Birth control pills will increase the amount of VWF and Hemofil within the blood and cut back blood loss. A doctor will impose these pills for ladies World Health Organization have significant discharge haemorrhage.

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