Management, vaccination status and COVID-19 morbidity of patients with Gaucher disease in Germany during the COVID-19 pandemic
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Management, vaccination status and COVID-19 morbidity of patients with Gaucher disease in Germany during the COVID-19 pandemic. / Niederau, Claus; Regenbogen, Claudia; Fruehauf, Hans-Martin; Merkel, Martin; Ziagaki, Athanasia; Mengel, Eugen; Baerwald, Christoph; Muschol, Nicole; Staufner, Christian; Lampe, Christina; Gillessen, Anton; Koehler, Jan Philipp; Vom Dahl, Stephan.
In: Z GASTROENTEROL, Vol. 61, No. 4, 04.2023, p. 375-380.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Management, vaccination status and COVID-19 morbidity of patients with Gaucher disease in Germany during the COVID-19 pandemic
AU - Niederau, Claus
AU - Regenbogen, Claudia
AU - Fruehauf, Hans-Martin
AU - Merkel, Martin
AU - Ziagaki, Athanasia
AU - Mengel, Eugen
AU - Baerwald, Christoph
AU - Muschol, Nicole
AU - Staufner, Christian
AU - Lampe, Christina
AU - Gillessen, Anton
AU - Koehler, Jan Philipp
AU - Vom Dahl, Stephan
N1 - Thieme. All rights reserved.
PY - 2023/4
Y1 - 2023/4
N2 - 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.
AB - 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.
KW - Adult
KW - Humans
KW - Adolescent
KW - Gaucher Disease/complications
KW - Glucosylceramidase/therapeutic use
KW - COVID-19/complications
KW - Pandemics
KW - SARS-CoV-2
KW - Morbidity
U2 - 10.1055/a-1821-9009
DO - 10.1055/a-1821-9009
M3 - SCORING: Journal article
C2 - 37040780
VL - 61
SP - 375
EP - 380
JO - Z GASTROENTEROL
JF - Z GASTROENTEROL
SN - 0044-2771
IS - 4
ER -