Management, vaccination status and COVID-19 morbidity of patients with Gaucher disease in Germany during the COVID-19 pandemic

Author:

Niederau Claus1,Regenbogen Claudia2,Fruehauf Hans-Martin3,Merkel Martin4,Ziagaki Athanasia5,Mengel Eugen6,Baerwald Christoph7,Muschol Nicole8,Staufner Christian9,Lampe Christina10,Gillessen Anton11,Koehler Jan Philipp12,vom Dahl Stephan12ORCID

Affiliation:

1. Postfach 10 12 03, Oberhausen, Germany

2. Department of Nephrology, Technical University of Munich, School of Medicine, München, Germany

3. Klinik f. Innere Medizin und Geriatrie, AMEOS Klinikum St. Marien, Oberhausen, Germany

4. Endokrinologikum, Hamburg, Germany

5. Medizinische Klinik f. Endokrinologie und Stoffwechselmedizin, Charité Campus Virchow, Berlin, Germany

6. Clinical Science for LSD, SphinCS, Hochheim, Germany

7. Klinik f. Endokrinologie, Nephrologie und Rheumatologie, Universitätsklinikum Leipzig, Leipzig, Germany

8. Department of Pediatrics, International Center for Lysosomal Disorders (ICLD), University Medical Center Hamburg-Eppendorf, Hamburg, Germany

9. Zentrum für Kinder- und Jugendmedizin, Universitätsklinikum Heidelberg, Heidelberg, Germany

10. Zentrum für seltene Erkrankungen Gießen (ZSEGI), Abteilung Kinderneurologie, Sozialpädiatrie u. Epileptologie. Zentrum Kinderheilkunde u. Jugendmedizin, Univ.-Klinikum Giessen/Marburg; Standort Giessen, Giessen, Germany

11. Klinik für Innere Medizin, Herz-Jesu-Krankenhaus Hiltrup GmbH, Münster-Hiltrup, Germany

12. Klinik für Gastroenterologie, Hepatologie und Infektiologie, Universitätsklinikum Düsseldorf, Düsseldorf, Germany

Abstract

Abstract Background Continuation of standard management of Gaucher disease (GD) has been challenging during the COVID-19 pandemic, resulting in infrequent/missed infusions and follow-up appointments. Little data are available on the consequences of these changes and on the SARS-CoV-2 vaccinations in German GD patients. Methods A survey with 22 questions about GD management during the pandemic was sent to 19 German Gaucher centres. It was answered by 11/19 centres caring for 257 GD patients (almost ¾ of the German GD population); 245 patients had type 1 and 12 had type 3 GD; 240 were ≥ 18 years old. Results Monitoring intervals were prolonged in 8/11 centres from a median of 9 to 12 months. Enzyme replacement therapy (ERT) was changed to home ERT in 4 patients and substituted by oral substrate reduction therapy (SRT) in 6 patients. From March 2020 to October 2021, no serious complications of GD were documented. Only 4 SARS-CoV-2 infections were reported (1.6%). Two infections were asymptomatic and two mild; all occurred in adult type 1, non-splenectomized patients on ERT. Vaccination rate in adult GD was 79.5% (95.3% mRNA vaccines). Serious vaccination complications were not reported. Conclusions The COVID-19 pandemic has lowered the threshold for switching from practice- or hospital-based ERT to home therapy or to SRT. No major GD complication was documented during the pandemic. Infection rate with SARS-CoV-2 in GD may rather be lower than expected, and its severity is mild. Vaccination rates are high in GD patients and vaccination was well tolerated.

Publisher

Georg Thieme Verlag KG

Subject

Gastroenterology

Reference18 articles.

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