Direct and indirect effects of the SARS-CoV-2 pandemic on Gaucher Disease patients in Spain: Time to reconsider home-based therapies?

Blood Cells Mol Dis. 2020 Nov:85:102478. doi: 10.1016/j.bcmd.2020.102478. Epub 2020 Jul 14.

Abstract

Objective: An analysis of the SARS-CoV-2 pandemic impact in the Spanish Gaucher Disease (GD) community is presented here.

Patients & methods: The Spanish GD foundation (FEETEF) surveyed 113 GD patients from March 30 to April 27; all patients provided a verbal consent.

Results: 110 surveys were analyzed. The median age was 47 years old (y.o.), 31 patients were ≥ 60 y.o.; and 34% of patients reported comorbidities. 46% (51/110) of patients were treated by enzyme replacement therapy (ERT), 48 of them at hospitals; 45.1% (45/110) were on substrate reduction therapy (SRT) and 9% (10/110) receive no therapy. 25% (11/48) of ERT-hospital-based patients reported therapy interruptions, while SRT-patients did not report missing doses. No bone crises were reported. However, 50% (55/110) of patients reported being worried about their predisposition to a severe SARS-COV-2 infection and 29% (16/55) of them took anxiolytics or antidepressants for this. While 6 patients reported to have contact with an infected person, another two confirmed SARS-CoV-2 infections were reported in splenectomyzed patients, one of them (a 79-year-old diabetic) died.

Conclusions: One quarter of the patients treated at hospitals reported dose interruptions. Home-based therapy may need to be considered in order to minimize the impact of the COVID-19 pandemic.

Keywords: COVID-19; ERT; Gaucher Disease; Home therapy; SARS-CoV-2.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Anti-Anxiety Agents / therapeutic use
  • Antidepressive Agents / therapeutic use
  • Anxiety / drug therapy
  • Anxiety / etiology
  • Betacoronavirus*
  • COVID-19
  • Combined Modality Therapy
  • Comorbidity
  • Continuity of Patient Care*
  • Coronavirus Infections*
  • Depression / drug therapy
  • Depression / etiology
  • Diabetes Mellitus / epidemiology
  • Disease Susceptibility
  • Enzyme Replacement Therapy* / methods
  • Female
  • Gaucher Disease / drug therapy*
  • Gaucher Disease / psychology
  • Gaucher Disease / surgery
  • Glucosylceramidase / supply & distribution
  • Glucosylceramidase / therapeutic use*
  • Home Care Services, Hospital-Based*
  • Humans
  • Immunocompromised Host
  • Male
  • Middle Aged
  • Pandemics*
  • Pneumonia, Viral*
  • SARS-CoV-2
  • Spain / epidemiology
  • Splenectomy / adverse effects
  • Young Adult

Substances

  • Anti-Anxiety Agents
  • Antidepressive Agents
  • Glucosylceramidase