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Suggest Treatment For Von Willebrand Disease With Mucosal Bleeding
Question: Von Willebrand Disease Type 3 disease with mucosal bleeding in infant less than 1 year old
Brief Answer:
It is a difficult disease but treatable
Detailed Answer:
Hi
Thanks for your query.
Von willebrand disease type 3 is the most severe variety and VW Factor is almost undetectable. As this is a genetic disorder and severe, it is almost always detected in infancy, like your patient. It is quite rare, like 1 in 1 million.
As it is due to deficiency of VWF, supplementation of VWF is the most effective treatment, along with supportive measures like pause or desmopressin. Desmopressin is quite effective in VWD type 1 and 2 but not so in type 3. However, after VWF factor supplement (like immunate), desmopressin can help it to act better. These therapies are usually given whenever there is any bleeding or before any surgery, dental extraction, etc. If there are repeated bleeding episodes, then frequent supplementation to prevent such episodes may be required. But still, life-threatening episodes may happen.
As it is a rare disorder, not too much data is available in literature but it is possible to lead a normal life with proper treatment. Genetic counseling and prenatal diagnosis should be offered to the parents to prevent further children having this disease.
Hope I have answered your query. I will be available to answer further followup queries, if any.
It is a difficult disease but treatable
Detailed Answer:
Hi
Thanks for your query.
Von willebrand disease type 3 is the most severe variety and VW Factor is almost undetectable. As this is a genetic disorder and severe, it is almost always detected in infancy, like your patient. It is quite rare, like 1 in 1 million.
As it is due to deficiency of VWF, supplementation of VWF is the most effective treatment, along with supportive measures like pause or desmopressin. Desmopressin is quite effective in VWD type 1 and 2 but not so in type 3. However, after VWF factor supplement (like immunate), desmopressin can help it to act better. These therapies are usually given whenever there is any bleeding or before any surgery, dental extraction, etc. If there are repeated bleeding episodes, then frequent supplementation to prevent such episodes may be required. But still, life-threatening episodes may happen.
As it is a rare disorder, not too much data is available in literature but it is possible to lead a normal life with proper treatment. Genetic counseling and prenatal diagnosis should be offered to the parents to prevent further children having this disease.
Hope I have answered your query. I will be available to answer further followup queries, if any.
Note: For further queries related to kidney problems Click here.
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
Answered by
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