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Published in: Allergy, Asthma & Clinical Immunology 1/2010

Open Access 01-12-2010 | Research

On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience

Authors: Emel Aygören-Pürsün, Inmaculada Martinez-Saguer, Eva Rusicke, Thomas Klingebiel, Wolfhart Kreuz

Published in: Allergy, Asthma & Clinical Immunology | Issue 1/2010

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Abstract

Background

Manifestation of acute edema in hereditary angioedema (HAE) is characterized by interindividual and intraindividual variability in symptom expression over time. Flexible therapy options are needed.

Methods

We describe and report on the outcomes of the highly individualized approach to HAE therapy practiced at our HAE center in Frankfurt (Germany).

Results

The HAE center at the Frankfurt University Hospital currently treats 450 adults with HAE or AAE and 107 pediatric HAE patients with highly individualized therapeutic approaches. 73.9% of the adult patients treat HAE attacks by on-demand therapy with pasteurized pd C1-INH concentrate, 9.8% use additional prophylaxis with attenuated androgens, 1% of the total patient population in Frankfurt has been treated with Icatibant up to now. In addition adult and selected pediatric patients with a high frequency of severe attacks are instructed to apply individual replacement therapy (IRT) with pasteurized pd C1-INH concentrate. Improvement on Quality of Life items was shown for these patients compared to previous long-term danazol prophylaxis. Home treatment of HAE patients was developed in the Frankfurt HAE center in line with experiences in hemophilia therapy and has so far been implemented over a period of 28 years. At present 248 (55%) of the adult patients and 26 (24%) of the pediatric patients are practicing home treatment either as on demand or IRT treatment.

Conclusions

In conclusion, the individualized home therapies provided by our HAE center, aim to limit the disruption to normal daily activities that occurs for many HAE patients. Furthermore, we seek to optimize the economic burden of the disease while offering a maximum quality of life to our patients.
Literature
1.
go back to reference Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, Farkas H, Varga L, Zingale LC, Binkley K, Wagner E, Adomaitis P, Brosz K, Burnham J, Warrington R, Kalicinsky C, Mace S, McCusker C, Schellenberg R, Celeste L, Hebert J, Valentine K, Poon MC, Serushago B, Neurath D, Yang W, Lacuesta G, Issekutz A, Hamed A, Kamra P, Dean J, Kanani A, Stark D, Rivard GE, Leith E, Tsai E, Waserman S, Keith PK, Page D, Marchesin S, Longhurst HJ, Kreuz W, Rusicke E, Martinez-Saguer I, Aygören-Pürsün E, Harmat G, Füst G, Li H, Bouillet L, Caballero T, Moldovan D, Späth PJ, Smith-Foltz S, Nagy I, Nielsen EW, Bucher C, Nordenfelt P, Xiang ZY: Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Managment of Hereditary Angioedema. Ann Allergy Asthma Immunol. 2008, 100: S30-40. 10.1016/S1081-1206(10)60584-4.CrossRefPubMed Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, Farkas H, Varga L, Zingale LC, Binkley K, Wagner E, Adomaitis P, Brosz K, Burnham J, Warrington R, Kalicinsky C, Mace S, McCusker C, Schellenberg R, Celeste L, Hebert J, Valentine K, Poon MC, Serushago B, Neurath D, Yang W, Lacuesta G, Issekutz A, Hamed A, Kamra P, Dean J, Kanani A, Stark D, Rivard GE, Leith E, Tsai E, Waserman S, Keith PK, Page D, Marchesin S, Longhurst HJ, Kreuz W, Rusicke E, Martinez-Saguer I, Aygören-Pürsün E, Harmat G, Füst G, Li H, Bouillet L, Caballero T, Moldovan D, Späth PJ, Smith-Foltz S, Nagy I, Nielsen EW, Bucher C, Nordenfelt P, Xiang ZY: Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Managment of Hereditary Angioedema. Ann Allergy Asthma Immunol. 2008, 100: S30-40. 10.1016/S1081-1206(10)60584-4.CrossRefPubMed
2.
go back to reference Kreuz W, Martinez-Saguer I, Aygören-Pürsün E, Rusicke E, Heller C, Klingebiel T: C1-inhibitor concentrate for individual replacement therapy in patients with severe hereditary angioedema refractory to danazol prophylaxis. Transfusion. 2009, 49: 1987-1995. 10.1111/j.1537-2995.2009.02230.x.CrossRefPubMed Kreuz W, Martinez-Saguer I, Aygören-Pürsün E, Rusicke E, Heller C, Klingebiel T: C1-inhibitor concentrate for individual replacement therapy in patients with severe hereditary angioedema refractory to danazol prophylaxis. Transfusion. 2009, 49: 1987-1995. 10.1111/j.1537-2995.2009.02230.x.CrossRefPubMed
3.
go back to reference Bork K, Barnstedt SE: Treatment of 193 episodes of laryngeal Edema With C1 Inhibitor Concentrate in patients With Hereditary Angioedema. Arch Intern Med. 2001, 161: 714-718. 10.1001/archinte.161.5.714.CrossRefPubMed Bork K, Barnstedt SE: Treatment of 193 episodes of laryngeal Edema With C1 Inhibitor Concentrate in patients With Hereditary Angioedema. Arch Intern Med. 2001, 161: 714-718. 10.1001/archinte.161.5.714.CrossRefPubMed
4.
go back to reference Bork K, Meng G, Staubach P, Hardt J: Treatment with C1 Inhibitor Concentrate in Abdominal Pain Attacks of Patients with Hereditary Angioedema. Transfusion. 2005, 45: 1774-1784. 10.1111/j.1537-2995.2005.00602.x.CrossRefPubMed Bork K, Meng G, Staubach P, Hardt J: Treatment with C1 Inhibitor Concentrate in Abdominal Pain Attacks of Patients with Hereditary Angioedema. Transfusion. 2005, 45: 1774-1784. 10.1111/j.1537-2995.2005.00602.x.CrossRefPubMed
5.
go back to reference Bork K, Staubach P, Hardt J: Treatment of Skin Swellings with C1-Inhibitor Concentrate in patients with Hereditary Angio-oedema. Allergy. 2008, 63: 751-757. 10.1111/j.1398-9995.2007.01577.x.CrossRefPubMed Bork K, Staubach P, Hardt J: Treatment of Skin Swellings with C1-Inhibitor Concentrate in patients with Hereditary Angio-oedema. Allergy. 2008, 63: 751-757. 10.1111/j.1398-9995.2007.01577.x.CrossRefPubMed
6.
go back to reference Craig TJ, Levy RJ, Wasserman RL, Bewtra AK, Hurewitz D, Obtulowicz K, Reshef A, Ritchie B, Moldovan D, Shirov T, Grivcheva-Panovska V, Kiessling PC, Keinecke H-O, Bernstein JA: Efficacy of Human C1 esterase Inhibitor concentrate compared with Placebo in Acute Hereditary Angioedema Attacks. J Allergy Clin Immunol. 2009, 124: 801-808. 10.1016/j.jaci.2009.07.017.CrossRefPubMed Craig TJ, Levy RJ, Wasserman RL, Bewtra AK, Hurewitz D, Obtulowicz K, Reshef A, Ritchie B, Moldovan D, Shirov T, Grivcheva-Panovska V, Kiessling PC, Keinecke H-O, Bernstein JA: Efficacy of Human C1 esterase Inhibitor concentrate compared with Placebo in Acute Hereditary Angioedema Attacks. J Allergy Clin Immunol. 2009, 124: 801-808. 10.1016/j.jaci.2009.07.017.CrossRefPubMed
7.
go back to reference Cicardi M, Zingale L, Zanichelli A, Lambertenghi Deliliers D: Established and new Treatments for Hereditary Angioedema: An Update. Molecular Immunology. 2007, 44: 3858-3861. 10.1016/j.molimm.2007.06.004.CrossRefPubMed Cicardi M, Zingale L, Zanichelli A, Lambertenghi Deliliers D: Established and new Treatments for Hereditary Angioedema: An Update. Molecular Immunology. 2007, 44: 3858-3861. 10.1016/j.molimm.2007.06.004.CrossRefPubMed
8.
go back to reference CIOMS III: Guidelines for Preparing Core Clinical Safety Information on Drugs. Assessed online as: 20090519100059324.pdf CIOMS III: Guidelines for Preparing Core Clinical Safety Information on Drugs. Assessed online as: 20090519100059324.pdf
9.
go back to reference Bork K: Pasteurized C1 Inhibitor Concentrate in Hereditary Angioedema: Pharmacology, Safety, Efficacy and Future Directions. Expert Rev Clin Immunol. 2008, 4: 13-20. 10.1586/1744666X.4.1.13.CrossRefPubMed Bork K: Pasteurized C1 Inhibitor Concentrate in Hereditary Angioedema: Pharmacology, Safety, Efficacy and Future Directions. Expert Rev Clin Immunol. 2008, 4: 13-20. 10.1586/1744666X.4.1.13.CrossRefPubMed
10.
go back to reference Martinez-Saguer I, Rusicke E, Aygören-Pürsun E, Heller C, Klingebiel T, Kreuz W: Characterization of Acute Hereditary Angioedema Attacks During Pregnancy and Breast-feeding and Their Treatment with C1 Inhibitor Concentrate. Am J Gyn Obstet. 2010, Martinez-Saguer I, Rusicke E, Aygören-Pürsun E, Heller C, Klingebiel T, Kreuz W: Characterization of Acute Hereditary Angioedema Attacks During Pregnancy and Breast-feeding and Their Treatment with C1 Inhibitor Concentrate. Am J Gyn Obstet. 2010,
12.
go back to reference Martinez-Saguer I, Rusicke E, Aygören-Pürsün E, von Hentig N, Klingebiel T, Kreuz W: Pharmacokinetic Analysis of Human Plasma-derived Pasteurized C1-inhibitor Concentrate in Adults and Children with Hereditary Angioedema: a Prospective Study. Transfusion. 2010, 50: 354-360. 10.1111/j.1537-2995.2009.02394.x.CrossRefPubMed Martinez-Saguer I, Rusicke E, Aygören-Pürsün E, von Hentig N, Klingebiel T, Kreuz W: Pharmacokinetic Analysis of Human Plasma-derived Pasteurized C1-inhibitor Concentrate in Adults and Children with Hereditary Angioedema: a Prospective Study. Transfusion. 2010, 50: 354-360. 10.1111/j.1537-2995.2009.02394.x.CrossRefPubMed
13.
go back to reference Teitel JM, Barnard D, Israels S, Lillicrap D, Poon MC, Sek J: Home Management of Haemophilia. Haemophilia. 2004, 10: 118-133. 10.1046/j.1365-2516.2003.00853.x.CrossRefPubMed Teitel JM, Barnard D, Israels S, Lillicrap D, Poon MC, Sek J: Home Management of Haemophilia. Haemophilia. 2004, 10: 118-133. 10.1046/j.1365-2516.2003.00853.x.CrossRefPubMed
Metadata
Title
On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience
Authors
Emel Aygören-Pürsün
Inmaculada Martinez-Saguer
Eva Rusicke
Thomas Klingebiel
Wolfhart Kreuz
Publication date
01-12-2010
Publisher
BioMed Central
Published in
Allergy, Asthma & Clinical Immunology / Issue 1/2010
Electronic ISSN: 1710-1492
DOI
https://doi.org/10.1186/1710-1492-6-21

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