Question of the Week

Does weather and in particular heat or barometric pressure have any role in causing attacks of HAE?
Jun 24, 2015

Dr. C:First of all for those who qualify Happy Father’s Day. I would like to thank Lois Perry of the HAEA for providing this question. As is often the case I’m afraid that we don’t have a precise answer. This topic has come up frequently however on patient surveys. We have included questions about weather […]

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Are there any special concerns for HAE during pregnancy?

Dr. C: This is certainly a topic of interest for all of our HAE patients. The initial onset of symptoms during pregnancy is rare for HAE type I & II however not unusual for HAE with normal C1 inhibitor. It is important to know that laboratory tests can be difficult to accurately interpret during pregnancy […]

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Should people with HAE be listed for potential bone marrow transplant donors? Should they give blood?

Dr. C: We would like to thank Janet Long for passing along this question from the HAEA. I believe that it was pointed out by one of the patients on the website that HAE may be too rare for people to think about with regard to these questions. Indeed checking the criteria for both bone […]

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Is there any co-morbidity known with HAE?

Dr C: First of all, we would like to wish you all Happy Memorial Day. We honor all those who have served. By co-morbidity, our questioner is referring to whether there are any other diseases that are linked to HAE. In HAE due to C1 inhibitor deficiency, patients show excessive activation of several pathways, including […]

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If you are taking danazol do you need an ultrasound and/or MRI yearly to monitor your liver?

Dr C: Greetings from Budapest where we are attending an international meeting on HAE. Prior to 2008 with the exception of antifibrinolytics, attenuated androgens such as danazol were all that we had available for prophylaxis of angioedema attacks in the United States. While effective for many patients, attenuated androgens have a myriad of potential associated […]

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Are African Americans less likely to have HAE? Is race a factor?

Dr C: As best we know, all ethnic groups are affected by HAE type 1&II. The C1 inhibitor gene appears to be particularly vulnerable to a variety of mutations; however the specific mutation does not seem to affect the severity of disease. I do not believe that we have a complete understanding however regarding interaction […]

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Biocryst showed the PK profile of their oral kallikrein inhibitor in one of their investor presentations showing that the max drug absorption is achieved within an hour or two after administration. Does this mean that this also has the potential of being used as an acute treatment therapy?

Dr C: This is an exciting new area of drug development using an oral agent for inhibition of plasma kallikrein to prevent generation of bradykinin and thereby attacks of angioedema. Given the kinetics of the drug I did check with the investigators at Biocryst to see what their take on the possibility of usage for […]

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Can you comment about the influence of age on the frequency of angioedema attacks?

Dr. C: I would like to start by encouraging all of you to sign up for our patient registry and join the database. We began this project over 5 years ago and it has become a repository of information to allow us to answer questions such as these. It is invaluable to have the direct […]

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Is there research for a cure going on right now?

SC: This is of course the ultimate goal. While many of the new treatments have made great strides in normalizing the lives of patients with HAE the hope is to find a pathway to cure the disease. There has been a case reported where a 7 year old boy with Evan’s syndrome and concomitant HAE […]

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Is it possible for a patient to become so adjusted to the medicines such as Berinert or Cinryze that their effectiveness is greatly diminished?

Dr C: Interesting question. If this were to occur we would expect it to be most common in the patients treated with regular injections of replacement C1 inhibitor for prophylaxis. In the open label study for Cinryze several of the patients were actually able to reduce the frequency of use from twice a week to […]

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