A Patient was admitted with the complaints of severe bleeding that it was not reduced or stopped by antifibrinolytics (Tranexamic acid) and was diagnosed as VWD
It is an inherited blood disorder that keeps your blood from clotting. It happens when you don’t have enough functioning von Willebrand factor (VWF)
Von Willebrand factor is a protein that acts as a bridge that helps blood platelets bind together to form clots, and it protects Factor VIII from premature breakdown. Patients with VWD suffer from a deficiency or dysfunction of this factor If a person has this condition they may bleed more than usual.
The most common clinical symptoms in VWD patients are mild to moderate mucocutaneous bleeding, epistaxis, bruising, menorrhagia, gastrointestinal bleeding, and prolonged bleeding after haemostatic challenges.
There are 3 types, in which Type 1 is most common and has mild and moderate symptoms.
Type 2 occur when the factor doesn’t work as it should.
Type 3 is a rare and severe form of Von Willebrand disease and leads to severe bleeding issues. In severe forms of VWD, patients may experience hemarthrosis episodes and severe gastrointestinal bleeding requiring long-term prophylactic treatment
It occurs when you inherit abnormal genes, that keep your body from making von Willebrand factor (VWF) like it should.
VWD differ from Haemophilia A by their deficient protein, VWF and Clotting factor VIII.
If they inherited a faulty gene from either their biological mother or their biological father. This is called autosomal dominant inheritance.
If they inherit a faulty gene from both biological parents to have the condition it is autosomal Recessive.
Diagnosis:
CBC, Platelet Aggregation Tests, Clotting Tests, on Will brand Factor tests, Factor VIII Factor test.
Treatment given -FANHDI
Fanhdiis a plasma-derived human coagulation Factor VIII and von Willebrand factor (VWF) complex. It is used in VWD when Desmopressin is ineffective or contraindicated.
Fanhdi 500 IU is supplied as a powder vial that must be reconstituted and administered intravenously (IV), Special instruction is given for its reconstitution by the manufacturer.
Share your thoughts on Haemophilia A disease.
MBH/AB