The impact of the COVID-19 pandemic on Fabry Disease Patients: an examination of Mood Status, Therapy Adherence, and COVID-19 infection

Abstract

Background Fabry disease (FD) is a rare metabolic disorder, in which a lifelong enzyme replacement therapy (ERT) constitutes the cornerstone of disease-specific therapy. In this study, we examined the effects of the COVID-19 pandemic and lockdown measures on the management of FD patients. Methods We collected data in three main domains; mood status, adherence to ERT, and COVID-19 infection. We used the Hospital Anxiety and Depression Scale (HADS) to evaluate the mood statuses of FD patients and the Morisky Medication Adherence Scale (MMAS) and the Medication Adherence Report Scale (MARS) to assess patients’ adherence to non-disease specific therapy. We also examined a control group to compare the mood status data. Results A total of 67 FD patients (males: 47.8%, mean age: 37.0 years) were recruited to the study, of which 58 were receiving ERT. Both the HADS depression and anxiety scores were higher in the control group compared to FD patients. During the first wave of the pandemic, 25 patients reported to have missed an infusion for a mean of 2.3 ± 1.7 doses and half of the patients had adopted a home-based infusion treatment regimen. COVID-19 infection developed in 25 patients, of which one died. The majority of our patients (71.6%) have had at least one shot of the vaccine. Conclusion We found that FD patients were more resilient to the negative psychological effects of lockdown. Traumatic growth may be an important factor in explaining this finding. Government-supported home therapy programs might be beneficial for FD patients to increase the therapy adherence.

COVID-19 and Vaccination Status in Lysosomal Storage Diseases: A Single-Center Experience

“…However, in the experiences of the Fabry patients with COVID-19, severe illness and death were reported in only 2 individuals with comorbidities. 16 , 19 , 21 , 22 Karaca et al 7 reported that 5 out of 25 Fabry patients who had COVID-19 were hospitalized with major organ involvement, and 1 of them died. Our Fabry patient with COVID-19 had a mild disease in this study.…”

Self-Reported Medication Adherence Measured with Morisky Scales in Rare Disease Patients: A Systematic Review and Meta-Analysis

“…Of those studies, 7 were excluded to avoid redundancy, as were extracting data from the same study [29][30][31] or not showing adherence to treatment data [32][33][34][35]. Finally, 18 studies [14,[36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51][52] were included in this systematic review and meta-analysis.…”

“…In 8 of them [14,36,38,43,[46][47][48]50], they established the level of adherence to treatment in three ranges (low, medium, and high), with the mean prevalence in each of them being 41.4%, 30.4%, and 28.2%, respectively. On the other hand, 6 studies used the MMAS-4 [39,40,42,44,51,52]. The mean adherence reported for this type of scale was 52.9%.…”

“…The mean adherence reported for this type of scale was 52.9%. According to sex, 15 studies reported the overall proportion of adherence in both men and women [14,[37][38][39][40][41][43][44][45][46][47][48][49]51,52], and 3 studies only included one sex [36,42,50] (i.e., only women).…”

“…Four studies included subjects with myasthenia gravis (MG) [37,46,51,52], and two studies included subjects with congenital hypogonadotropic hypogonadism [36,50]. The rest of the subjects in the different studies had different RDs, such as congenital Wilson's disease [14], Fabry disease [44], cystic fibrosis [43], hemophilia A [42], pulmonary arterial hypertension [48], and amyotrophic lateral sclerosis [47].…”