COVID-19 Pandemic Outbreak and its Psychological Impact on Patients with Rare Lysosomal Diseases

Fiumara, Agata; Lanzafame, Giuseppina; Arena, Alessia; Sapuppo, Annamaria; Raudino, Federica; Praticò, Andrea D.; Pavone, Piero; Barone, Rita

doi:10.3390/jcm9092716

Cited by 41 publications

(71 citation statements)

References 6 publications

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“…This study was performed using an online cross-sectional design, the method of preference for other similar studies [ 19 , 26 , 27 ]. Among the weaknesses of cross-sectional studies is the inability to determine the temporal relation between outcomes and risk factors, so they are unable to establish if the exposure preceded the disease or vice versa.…”

Section: Discussionmentioning

confidence: 99%

Depression and Anxiety in Patients with Rare Diseases during the COVID-19 Pandemic

Sánchez-García

Cortés-Martín

Rodríguez-Blanque

et al. 2021

IJERPH

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Scientific knowledge on depression and anxiety in patients with rare diseases during the COVID-19 pandemic is scarce; however, it is essential to perform comprehensive management of these patients. The aim of this study was to research how the situation caused by the SARS-CoV-2 pandemic has influenced the lives of patients with rare diseases regarding depression and anxiety. This Spanish study considered a heterogeneous population sample of 86 patients with confirmed diagnosis of different rare diseases. Participants took part in a cross-sectional online study by completing specific questionnaires on the study topic. Depression was measured using the Patient Health Questionnaire (PHQ-9), and the General Anxiety Disorder Scale (GAD-7) was used for evaluating anxiety. Data collection through an online questionnaire allowed for a greater population scope and therefore the inclusion patients of other nationalities in the study sample. Finally, as a general result, this study found that, in the face of the pandemic, anxiety and depression remained at a higher level in this group than in the general population, making these patients a vulnerable population group.

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Section: Discussionmentioning

confidence: 99%

Depression and Anxiety in Patients with Rare Diseases during the COVID-19 Pandemic

Sánchez-García

Cortés-Martín

Rodríguez-Blanque

et al. 2021

IJERPH

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“…The main issues emphasized in the literature were the interruption of enzyme replacement therapy infusions in hospitals, modification of the treatments to home therapy, and the behavioral and emotional changes of LSD patients during the pandemic. [7][8][9] In terms of other IEM, main topics mentioned in the literature were the number of newly diagnosed patients, number of emergency unit or outpatients' clinic admissions, factors affecting the frequency of admission, the level of awareness of the patients about pandemic, and questionnaire studies about the difficulties they experienced. Telemedicine platforms consisting of phone calls and online communication were recommended to ensure the continuity of follow-up and treatment of IEM patients.…”

Section: Discussionmentioning

confidence: 99%

“…Literature concerning impact of COVID-19 on patients with IEM mainly included patients with lysosomal storage disorders (LSDs). [7][8][9] However, only limited data are available about the problems experienced and their solutions in the follow-up of other IEM in the COVID-19 period. [10][11][12] PKU is the most frequent IEM in Turkey with a prevalence of 1:4,200.…”

Section: Introductionmentioning

confidence: 99%

The Impact of Telemedicine for Monitoring and Treatment of Phenylketonuria Patients on Metabolic Outcome During Coronavirus Disease-19 Outbreak

Zübarioğlu

Hopurcuoğlu

Uygur

et al. 2022

Telemedicine and e-Health

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Background: The prognosis of phenylketonuria (PKU) in terms of neurocognitive outcome is directly related to lifelong phenylalanine (Phe) levels and adherence to treatment. Monitoring and treatment of PKU patients can be complicated in challenging circumstances as pandemics. This study aims to evaluate the impact of telemedicine for monitoring and treatment of PKU patients on metabolic outcome during coronavirus disease-19 (COVID-19) outbreak. Materials and Methods: Patients who were diagnosed as PKU and treated with low Phe diet, tetrahydrobiopterin (BH4), or BH4 adjunct with low Phe diet were enrolled. Study period was divided into two periods: prepandemic period wherein patients were followed up in outpatients' clinic and during pandemic wherein telemedicine was used. Demographic findings, laboratory results, and therapy responses were reviewed retrospectively and compared between the two periods. All procedures were in accordance with the ethical standards of the local ethical committee of Cerrahpasa Medical Faculty (17/11/2020-151640) and with the Helsinki Declaration of 1975, as revised in 2013. Results: Ninety-three (n = 93) patients were enrolled to this study. The ratio of the samples with Phe levels in the recommended ranges was found to be statistically higher during the pandemic wherein an online monitoring system was used in all treatment modalities (p < 0.05). The decrease in Phe washout frequency was statistically significant during the pandemic in the low Phe diet group (p < 0.05). Considering the relationship between Phe tolerance before and during the pandemic, a significant increase in Phe tolerance was noted during the pandemic in the low Phe diet group (p < 0.05). Conclusions: Telemedicine can be an appropriate and effective monitoring option for PKU patients during the COVID-19 pandemic.

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“…Finally, an important and often overlooked aspect of the holistic care of children and adults affected with inborn errors of metabolism, and speci cally LSDs, is the psychological state and wellbeing. Recently, Fiumara et al 12 evaluated the psychological impact of the COVID-19 pandemic on a cohort of 15 LSD patients in Italy. While their results did not reach statistical signi cance when compared to age-matched controls, an important nding universally shared by all participants was the reportedly increased feelings of anxiety, worry and uncertainty.…”

Section: Discussionmentioning

confidence: 99%

The Effects of The COVID-19 Pandemic on Patients With Lysosomal Storage Disorders in Israel

Kristal

Pode‐Shakked

Hazan

et al. 2021

Preprint

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Background- Severe acute respiratory syndrome coronavirus 2 (SARS CoV-2) is the causative agent of the current COVID-19 pandemic. Lysosomal storage disorders (LSD) comprise of 70 inherited inborn errors of metabolism. Affected individuals suffer from multi-systemic involvement with variable severity and rate of disease progression between different diseases. Some of the LSDs have established treatments whether parenteral, or oral therapies. The full impact of the COVID-19 pandemic together with the lockdown on the wellbeing and medical management of patients with rare diseases, such as LSDs, is widely unknown. Herein, we describe the effects of the COVID-19 pandemic and its associated mandatory home lockdown on patients with LSDs in Israel. Results- We present a prospective multi-center questionnaire study including 48 LSD patients from four medical centers in Israel. The study objective was to assess the impact of the COVID-19 pandemic restrictions on individuals with LSDs in Israel, as reported by their caregivers. Secondary objectives were to assess the morbidity from SARS CoV-2 in LSD patients and the impact of changes in mood and behavior on compliance to treatment and to assess the relationship between changes in mood to changes in cognition and behavior. Thirty one of 38 patients (82%) who received any kind of regular treatment did not miss treatments. Among patients receiving enzyme replacement therapy (ERT) in the in-hospital setting, 5 patients (20%) experienced treatment disruptions. Four patients had tested positive for SARS-Cov-2 virus infection by PCR. Seven out of the 48 patients (14%) described mood changes with cognitive and motor deterioration during the home quarantine. Conclusions- We observed high rates of treatment adherence and low morbidity through the COVID-19 pandemic in patients with LSDs in Israel. LSDs patients can be a model for patients with complex chronic diseases requiring routine treatments and surveillance during a pandemic or other disruption of daily routine.

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COVID-19 Pandemic Outbreak and its Psychological Impact on Patients with Rare Lysosomal Diseases

Cited by 41 publications

References 6 publications

Depression and Anxiety in Patients with Rare Diseases during the COVID-19 Pandemic

Depression and Anxiety in Patients with Rare Diseases during the COVID-19 Pandemic

The Impact of Telemedicine for Monitoring and Treatment of Phenylketonuria Patients on Metabolic Outcome During Coronavirus Disease-19 Outbreak

The Effects of The COVID-19 Pandemic on Patients With Lysosomal Storage Disorders in Israel

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