Question of the Week

“I was diagnosed with C1 deficiency last year, at the same time they diagnosed me with B cell lymphoma. Is this common or rare?”
Sep 8, 2015

Dr. C:  First of all I would like to wish our readers a Happy Labor Day. In answer to our reader’s question, it is indeed very rare to have these two conditions simultaneously diagnosed but not, in all likelihood, a coincidence. It is probable that our reader has a condition that is even more unusual […]

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“Why can’t Icatibant be used in children? What can happen?”
Sep 1, 2015

Dr. C: We would like to thank our reader for introducing this important topic in the management of HAE—children. The limited available pediatric treatment options comprise a serious unmet need. The FDA approved on-demand drugs include: Ecallantide, for ages 12 and older; Icatibant, for ages 18 and older; plasma derived C1 inhibitor and recombinant human […]

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What is the likelihood of dental work triggering an HAE attack?
Aug 24, 2015

“My daughter has tested positive for HAE type II. She has not had any issues to date but needs dental work done. They want to infuse her first. Can infusing for precautions trigger attacks to start?” Dr. C: This is a question within a question. The first is what is the likelihood of dental work […]

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“Why does HAE localize in certain area of the body; ie feet vs face or gut?”
Aug 18, 2015

Dr C: This is a fascinating question. I don’t think that we will be able to provide a definitive answer, but we can speculate. As our readers know, bradykinin has been established as the mediator of swelling in HAE. Current understanding suggests that bradykinin is generated in the blood, and then acts on specific bradykinin […]

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Should Firazyr only be used for stomach or throat attacks due to the cost of treatment?
Aug 4, 2015

 “My doctor keep stressing that I am only to use Firazyr for a severe stomach attack or a throat attack due to how much it costs.  I keep hearing others say they use it for any attack.  What is your opinion?” Dr C:I thought that this ‘left over’ question from our last patient summit would […]

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Given the multiple treatments for acute attacks of HAE do we need to use more than one of the medications? What happens if I have recurrent, continued swelling after a treatment?
Jul 27, 2015

Dr C:Excellent question—it is indeed fortunate to have an array of choices for ‘on demand’ treatment of acute attacks. We now have four approved effective products: 1) Kalbitor an inhibitor of the enzyme plasma kallikrein which is responsible for generation of bradykinin which drives the vascular leak and swelling, 2) Firazyr which blocks the receptor […]

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What is the role for FFP in the treatment of HAE? Do patients have a role in participating in their choice of therapy?
Jul 20, 2015

“Given the current on demand treatment options in the United States, what is the role for FFP?  Do patients have a role in participating in their choice of therapy?” Dr. C:I would like to thank Janet Long for rescuing the stack of question cards from a previous patient summit–one of which raised this topic. As […]

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What is the best way to approach exercise for those with HAE?
Jul 13, 2015

Dr. C:  This is an excellent topic to promote our new event this year at the Patient Summit—‘The HAE in motion 5K Walk/Run’. We hope to see you all in motion in Denver. Exercise is one of the most beneficial things that we can do for ourselves. A recent study confirmed that just doing something […]

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Is there any need for re testing (additional laboratory) once a diagnosis of HAE is established?
Jul 6, 2015

Dr. C:  A belated Happy Fourth of July. Thank you to Lois Perry of the HAEA for bringing up this topic. It had come to her attention that some physicians were requiring patients to come in for annual testing as a condition for renewing their prescriptions. As we reviewed in our last ‘Question of the […]

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Can multiple family members have different types of HAE or are they normally the same type?
Jun 29, 2015

Dr. C: Thank you to Janet Long of the HAEA for forwarding this question. Certainly with all of the numbers attached to HAE it does get confusing. The genetics of HAE type I and II are very simple. Their inheritance pattern is autosomal dominant with near complete penetrance. What this means is that if one […]

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