Hereditary Angioedema in Pregnancy
Background:
Hereditary angioedema (HAE) is a rare but serious disease that is inherited. Due to a congenital genetic defect, the C1-esterase inhibitor is only partially effective in HAE patients.
This leads to increased permeability of the blood vessels and, as a result, to mostly unpredictable, spontaneously occurring swelling of the hands, feet or face, colicky abdominal pain, often accompanied by vomiting and diarrhea, or shortness of breath if the swelling affects the throat.
Hormonal influences can affect the course of the disease. For example, estrogen-containing contraceptives are contraindicated for HAE patients because they regularly lead to a worsening of the clinical picture. The hormonal influences of pregnancy also change the disease activity in most cases, so that an adjustment of the previous HAE therapy is often necessary. The nature and extent of the changes as well as their timeline (before/during/after pregnancy) are contradictory in the literature. Real-life data on treatment/adjustment during pregnancy are only sparsely available, as well.
Study type:
Questionnaire (physician and patient)
Aims:
A questionnaire for patients with HAE and their physicians will be used to retrospectively collect and evaluate details on the influence of pregnancy on HAE. The time period surveyed will cover the periods before, during and after the pregnancies/breastfeeding periods and should include, among other things, disease activity, medication, contraception, concomitant diseases and outcome of the pregnancies,
The data will be collected anonymously and analyzed electronically. Publication of the data is planned.
- The pregnancy can be any length of time ago.
- The physician no longer has to sign the consent form. This makes it easier for doctors who know HAE patients who have been pregnant in the past to distribute the survey. It has now become an anonymous survey.
Project lead:
Thomas Buttgereit (Berlin, Germany), Carolina Vera (Berlin, Germany)
Steering Committee:
Markus Magerl (Berlin, Germany)
Petra Staubach-Renz (Mainz, Germany)
Status:
Global Rollout
If you are an ACARE physician treating HAE or a HAE patient and would like to participate in this project, please reach out to Thomas Buttgereit at thomas.buttgereitATcharite.de
HAPY is supported by CSL Behring
