Von Willebrand Disease

Von Willebrand Disease General Information

Von Willebrand Disease (a medical condition commonly abbreviated as VWD) is one of the most widely known hereditary coagulation disorders; however, it is important for all patients to know that this clinical illness can also develop as a direct result of the presence of other medical disorders. Scientists have determined that this medical condition is mainly due to a deficiency of a special multimeric protein (called the Von Willebrand Factor) which is needed for platelet adhesion – this deficiency can be either quantitative or qualitative. Besides humans, this illness can also affect dogs. Physicians have determined that this medical condition can appear in 4 forms. The severity of the patient’s Von Willebrand Disease is known to depend on the individual’s ABO blood group. As such, patients who have blood type O are prone to developing a more severe form of this medical condition.

The Von Willebrand Factor is mostly active in cases of shear stress and also high blood flow. A deficiency of this medical factor is mainly present in the case of organs that have extensively small blood vessels (this includes the uterus, the gastro-intestinal tract, the skin, and so on). In the case of human individuals, the incidence of this coagulation medical condition is 1 in 100 patients. Most forms of this clinical disorder are mild conditions; this disorder is more often reported in the case of females, as in such cases, the bleeding tendency shows during the patient’s menstruation. The actual abnormality (all patients must be fully aware of the fact that the presence of this abnormality does not necessarily lead to the development of the disease) is present in about 0.9-3% of the world’s entire population.

Von Willebrand Disease Symptoms

As we have stated in the previous chapter, this medical condition can appear in more than 1 form; the different types of this disorder are characterized by different degrees of bleeding tendency. In most cases, the presence of this blood coagulation medical condition (Von Willebrand Disease) determines the following clinical manifestations: easy bruising or easy bleeding, bleeding gums, nose-bleeds, and so on. Female patients who are suffering from this coagulation disorder are more prone to experiencing unusually high loss of blood during childbirth and heavy menstrual periods. Type 3 of this clinical condition can trigger several severe clinical symptoms; among them: severe joint bleeding, internal bleeds, etc. Luckily, the development of such symptoms is quite rare, and only a few patients experience it. In some cases, the presence of skin rash can also be a symptom characteristic for Von Willebrand Disease.

The most common medical exams that can be performed in other to accurately diagnose the presence of this condition include: bleeding time (the results indicate prolonged bleeding time), platelet count, platelet aggregation test, Von Willebrand Factor level (the results show a reduced level of this protein), Ristocetin cofactor test (this is the primary clinical test that is employed in the diagnosis of this medical condition). Furthermore, the presence of Von Willebrand Disease can also alter the results of these clinical exams: Von Willebrand Factor multimers count, Factor VIII level. All individuals must know that the presence of this coagulation medical condition can be quite challenging to diagnose. Unusually low levels of the Von Willebrand Factor do not necessarily mean that the patient is suffering from this blood medical condition.

Von Willebrand Disease Treatment

Individuals who are suffering from this clinical disorder do not normally require a specific treatment for their condition even if they are at all times at a higher risk of experiencing (severe) bleeding. Females who present disturbingly heavy menstrual bleeding (due to the presence of the Von Willebrand Disease) should discuss with their main medical prescribers the possibility of starting a therapy course with a combined oral contraceptive pill (studies have shown that the intake of such a medical product can be very effective in the reduction of the frequency and length of the patient’s periods and also in the reduction of bleeding due to other causes).

Patients who are suffering from this medical condition and who are scheduled for a certain type of surgery may have to follow a special prophylactic therapy course. Such a therapeutic approach normally includes the administration of human derived factor VIII (of medium purity) and of the Von Willebrand Factor. Patients who are suffering from a mild form of Von Willebrand Disease can follow a treatment trial with Desmopressin (Stimate) which is able to raise the patient’s own plasma levels of the Von Willebrand Factor as it is known to determine the release of the supplies of this protein that are present in the endothelial cells’ Weibel-Palade bodies. Studies have shown that the administration of such a therapy course is effective in reducing the patient’s tendency towards heavy bleeding. However, not all types of the Von Willebrand Disease effectively respond to a therapy course with Desmopressin. For this reason, patients who are interested in following this type of treatment routine should undergo specific medical examination in order for the individual’s physician to accurately determine the type of the Von Willebrand Disease of which the patient is suffering.

A therapy course with an antihemophilic factor (such as Alphanate) is approved in order to help decrease the severity of bleeding in the case of patients who are to undergo an invasive medical procedure. Possible complications of this clinical condition (Von Willebrand Disease) may occur after the patient undergoes surgery. Patients who are suffering from this medical disorder are not allowed to use any type of NSAIDs (non-steroidal anti-inflammatory medicines) including Ibuprofen and Aspirin without discussing this matter with their personal health care specialists. If you are interested in finding out more about the most effective therapy routines that can be applied in the case of patients who are suffering from Von Willebrand Disease (a blood coagulation clinical disorder) you should contact your personal health care prescriber. Your local pharmacist can give you further information regarding the drugs that we have listed in this chapter of our medical presentation.