Von Willebrand Disease (VWD)
It is a common, inherited hemorrhagic disorder that is caused by a dysfunction or deficiency of the protein termed von Willebrand factor (vWF).
vWF helps blood cells stick together when you bleed. Therefore, if there is not sufficient von Willebrand factor or if it doesn’t work correctly, it takes substantially longer for bleeding to stop.
Von Willebrand disease was first described in the medical literature in 1926 by Dr. Erik von Willebrand (a Finnish physician), who differentiated VWD from classic hemophilia.
In the United States, the disorder affects up to 1 percent of people.
VWD has several forms:
In this most common form of VWD, individuals have low levels of vWF and may also have low levels of factor VIII.
Moreover, individuals with type 1 Von Willebrand disease can bleed a lot with surgery, injuries, or when they have a tooth pulled and they can have nosebleeds or mild bruising.
The patients will most likely experience mild bleeding problems, however, they will be able to live a normal life.
It is caused by different gene mutations. Signs and symptoms tend to be more significant in this type of VWD. Also, patients receive different medical treatments.
In this rare type, levels of factor VIII are low and vWF is absent. It affects 5 to 10% of people with the condition. This is the rarest and most severe form of Von Willebrand disease.
Signs and symptoms may be severe, like – bleeding into the muscles and joints, occasionally without injury.
Note – individuals with type 2 and 3 require a medicine called Humate-P which contains both factor VIII and vWF. The medicine is injected into a vein.
This type of Von Willebrand disease isn’t inherited from the parents, but it develops later in life.
If you have VWD, you might experience:
- easy bruising or lumpy bruises;
- bleeding into joints or internal organs;
- blood in your urine or stool;
- blood in the urine from bleeding into the bladder or kidneys;
- blood in the stool from bleeding into the intestines or stomach;
- long-lasting or heavy bleeding after a tooth surgery or removal;
- heavy periods and bleeding during or after labor (for women);
- heavy or long menstrual bleeding (for women);
- nosebleeds which don’t stop within 10 minutes;
- excessive bleeding from an injury.
Excessive bleeding may occur when you have a tooth pulled or after surgery.
Von Willebrand disease is an inherited condition. A parent with Von Willebrand disease has a 50% chance of passing the affected gene on to each child.
Tests which may be done to diagnose VWD include:
- Von Willebrand factor specific tests;
- bleeding time;
- ristocetin cofactor test (evaluates the functioning of the protein vWF);
- platelet count;
- platelet function analysis;
- factor VIII level;
- blood typing.
There are 3 main drugs which can help stop the bleeds:
- vWF concentrate – it is available as an injection;
- tranexamic acid – it is available as a mouthwash, tablets, or an injection;
- desmopressin – it is available as an injection or nasal spray.
Consider these steps to reduce your risk of bleeding:
- make pharmacists, surgeons, and doctors aware of your condition;
- wear a medical ID bracelet;
- try to avoid participating in strenuous sports such as hockey, football, and weightlifting;
- avoid the use of blood-thinning medications such as ibuprofen and aspirin.
It is a rare disorder that prevents blood from clotting normally.
Hemophilia A, also referred to as classic hemophilia, occurs when clotting factor VIII is either not present in enough amounts or absent. Without enough factor VIII, the human body cannot form a stable blood clot. Hemophilia A is approximately 4 times more common than hemophilia B.
Hemophilia B, also referred to as Christmas disease, occurs when clotting factor IX is either not present in enough amounts or absent. Without enough factor IX, the human body cannot form a stable blood clot.
Hemophilia C, also known as Rosenthal syndrome or plasma thromboplastin antecedent deficiency, occurs when there are low levels of factor XI, a blood protein needed for clot formation. Hemophilia C is inherited differently than Hemophilia A or B.
The main symptom is bleeding longer than normal. Other symptoms include:
- bleeding gums;
- nosebleeds which won’t stop;
- bloody urine or stool;
- excessive bruising and hematoma;
- bleeding in the muscles and joints.
When to Call the Doctor
Seek emergency care for:
- signs of blood in stools or urine;
- extreme exhaustion;
- sudden or severe swelling or pain;
- bleeding which can’t be controlled.
It is a genetic disease that is caused by a mutation within the genes for coagulation factors VIII or IX. Approximately 70% of all patients with hemophilia A or B inherited the disorder. The other 30% develop from a spontaneous genetic mutation.
Risk factors are:
- inflammatory bowel disease;
- autoimmune disorders;
- age >60 years;
- male gender;
- a family history of hemophilia.
Treatment for this disorder may involve a slow injection of desmopressin (helps to release more clotting factor to stop the bleeding) by your healthcare professional into one of the veins.
To lower your chances of bleeding:
- avoid certain pain medicines;
- take care of your teeth;
- do not use blood thinners;
- avoid contact sports;
- protect your head from injuries.
Von Willebrand disease is a bleeding disorder that is caused by a deficiency of vWF. Numerous patients with Von Willebrand disease may not realize that they have the blood disorder as their symptoms are very mild. Patients who do may find that they:
- bleed excessively from the mouth;
- have heavy menstrual flow (women);
- bruise easily;
- have lots of nosebleeds.
Hemophilia is a condition in which an individual’s blood does not clot correctly since the body does not produce sufficient, or is missing, the blood clotting proteins, factor IX, or factor VIII. The bleeding caused by hemophilia can range from mild to severe. In severe cases, patients can even bleed to death.
In conclusion, hemophilia is caused by a problem with a specific blood clotting factor (factor VIII in hemophilia A; factor IX in hemophilia B), while Von Willebrand disease is caused by a problem with vWF.
Another difference is that hemophilia mainly affects males, whereas Von Willebrand disease affects both females and males. Also, Von Willebrand disease is more common and typically milder than hemophilia.