Is home-based therapy in Fabry disease the answer to compelling patients' needs during the COVID-19 pandemic? Survey results from the Polish FD Collaborative Group.
Adv Clin Exp Med
; 30(4): 449-454, 2021 Apr.
Article
em En
| MEDLINE
| ID: mdl-33908195
ABSTRACT
BACKGROUND:
Fabry disease (FD) is an X-linked disorder related to a deficiency of the lysosomal enzyme alpha-galactosidase A. In Poland, enzyme replacement therapy (ERT) for FD is offered by the National Health Fund only at selected hospital infusion centers. Patients with FB are considered at a high risk of developing complications from COVID-19. Some patients omitted infusions due to fear of infection or outbreaks in hospitals. Lack of alternative infusion sites hampered the situation.OBJECTIVES:
To analyze the impact of the SARS-CoV-2 pandemic on FD patients, especially their fears and expectations, the Polish FD Collaborative Group collaborated on a survey project. MATERIAL ANDMETHODS:
Between September and November 2020, we distributed a customized survey exploring expectations and fears among FD subjects.RESULTS:
Fifty-five individuals (35 receiving ongoing ERT) from different FD centers completed the study. The median age was 40 years [IQR 25; 50], and gender distribution was almost equal (27 F; 28 M). One-fourth of FD patients reported severe disability limiting transportation for infusions that, in the opinion of the other 25% of responders, consumed >4 h. Forty-four (80%) of all would prefer home infusions performed by a nurse (n = 37, 67.3%) or by a trained non-medical person (n = 7, 12.7%), while 8 (14.5%) patients would choose a local hospital. As expected, transportation time (in one direction) was longer in those preferring home infusions (89.4 ±63 vs 36.2 ±67 min; p = 0.02). Also, those with more severe FD manifestation would prefer home infusions to treatment in FD centers (p = 0.03). The vast majority of respondents (n = 46; 83%) would not change their preferences after pandemic termination.CONCLUSIONS:
To maintain ERT, FD patients prefer home infusions or those given in the nearest hospital, especially during a pandemic.Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Doença de Fabry
/
COVID-19
Limite:
Adult
/
Humans
País/Região como assunto:
Europa
Idioma:
En
Revista:
Adv Clin Exp Med
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Polônia