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Diagnosed with Hereditary Angioedema Type III. Receiving Cinryze medication therapy. Have swelling. Continue medication? Guide?

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Orthopaedic Surgeon
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Hello! My child was diagnosed with Hereditary Angioedema Type III. Symptoms started around the age of 7, so far no treatment has been successful. She has been receiving Cinryze medication therapy every three days for about a month, however we are not sure if the medication is helping. Primarily because she still has swelling every few days even on the days she receives the Cinryze. Frequency of the swelling is sporadic. She has not tested positive for the Hereditary Angioedema, however other females in the family have symptoms and one tested positive for the Hereditary Angioedema. How long should the medicine be taken to know if it is working? Do you know how or what information we should compare to determine if the Cinryze is helping? If our findings show the Cinryze is not helping, could that mean she doesn't have Hereditary Angioedema. What could this illness be?
Posted Tue, 25 Jun 2013 in Blood Disorders
Answered by Dr. Praveen Tayal 2 hours later
Thanks for posting your query.
Angioedema is an allergic condition characterized by swelling just below the surface of the skin, usually around the lips and eyes. During an allergic reaction lots of chemical mediators of inflammation are released, histamine is one of them, which causes blood vessels to swell. The cause is unknown, but certain food and environmental conditions can trigger an attack of angioedema.
The non responsiveness to Cetrizine does not exclude the diagnosis of angioedema. The clinicxal presentation that you have described is consistent with angioedema.
It is best to identify and avoid the exposure to the triggering factors for best response.
Sometimes it's not possible to pinpoint the cause of hives and angioedema, especially when these conditions become chronic or recur. These are usually caused by an allergic reaction, to food, medication, pollen or insect stings.

Some doctors suggest skin patch test (allergen test) to find the triggering allergens which can be avoided. You can get it done from her dermatologist.

I hope this answers your query.
In case you have additional questions or doubts, you can forward them to me, and I shall be glad to help you out.
Please accept my answer in case you do not have further queries.
Wishing you good health.
Dr. Praveen Tayal.
Above answer was peer-reviewed by
Follow-up: Diagnosed with Hereditary Angioedema Type III. Receiving Cinryze medication therapy. Have swelling. Continue medication? Guide? 2 hours later
The skin patch test has been performed, results showed environmental allergies no significant food sensitivity. What's the difference between having Angioedema and Hereditary Angioedema type III? Do you know how long it takes for Cinryze medication to show if it's improving the episodes? This swelling occurs in her sleep and usually has several hours before she can consume food or beverages. Swelling is not responsive to allergy medications or steroids.
Answered by Dr. Praveen Tayal 3 hours later
Thanks for writing again.
Angioedema can happen due to environmental factors - allergic in nature or due to genetic make up- hereditary angioedema.
The cause cannot be eliminated in hereditary type and the symptoms are more severe and persistent in problem due to genetic cause.
Cetrizine and steroids do show a response and prevent further episodes after 2-3 weeks of treatment. Immunomodulatory treatments need to be considered after consulting an immunologist in unresponsive cases.
Hope my answer is helpful.
Do accept my answer in case there are no further queries.
Above answer was peer-reviewed by
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