Heiminfusionstherapie bei Morbus Pompe: Konsensusempfehlungen für den deutschsprachigen Raum [Home infusion therapy for Pompe disease: Recommendations for German-speaking countries]

Details

Serval ID
serval:BIB_55771BD7A28C
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Heiminfusionstherapie bei Morbus Pompe: Konsensusempfehlungen für den deutschsprachigen Raum [Home infusion therapy for Pompe disease: Recommendations for German-speaking countries]
Journal
Fortschritte der Neurologie-Psychiatrie
Author(s)
Hahn A., Lampe C., Boentert M., Hundsberger T., Löscher W., Wenninger S., Ziegler A., Lagler F., Ballhausen D., Schlegel T., Schoser B.
ISSN
1439-3522 (Electronic)
ISSN-L
0720-4299
Publication state
In Press
Peer-reviewed
Oui
Language
german
Notes
Publication types: English Abstract ; Journal Article
Publication Status: aheadofprint
Abstract
Pompe disease is a lysosomal multisystem disorder with predominant proximal myopathy. Treatment with enzyme replacement therapy (ERT) is available requiring life-long biweekly infusions of recombinant α-glucosidase. To minimize the burden of ERT patients ask for home infusion therapy.
Pompe disease experts from Germany, Austria, and Switzerland discussed in two consensus meetings in 2019 and 2020 requirements for home infusion therapy, adequate execution of treatment, and the legal situation for delegating physicians.
Home infusion therapy is principally feasible for patients with Pompe disease if certain preconditions are fulfilled, but the decision to implement has to be made on an individual basis. The treating physician delegates the execution of ERT ad personam to nursing staff but retains full legal responsibility. Home infusion therapy has to be carried out by specially trained and qualified staff. Infusion-related risks comprise mainly allergic reactions, and adequate medical treatment must be warranted. In German-speaking countries, clear rules for conducting home infusion therapy are needed to reduce psychosocial stress for patients with Pompe disease, and providing legal certainty for delegating physicians.
Pubmed
Web of science
Create date
22/02/2021 14:46
Last modification date
13/03/2021 7:22
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