COVID-19 pandemic and care of transfusion-dependent patients of thalassaemia: Experience from a paediatric centre in North India.

COVID-19 has presented an unprecedented challenge to health services and has significantly affected the management of non-Covid illnesses, like thalassemia. The present study documents the impact of Covid-associated restrictions and disruptions on working of the pediatric thalassemia day care centre (TDCC), and measures taken by TDCC and blood transfusion services to adapt to and mitigate the negative impact of Covid pandemic and associated lockdown on patient care. Pre-transfusion haemoglobin and packed cell transfusion requirement were compared across three time periods, namely pre-lockdown, lockdown and post-lockdown in paediatric transfusion-dependent thalassaemia (TDT) patients. Caregivers were interviewed to document any problems faced by them. The study involved 181 TDT patients. There was a significant reduction in mean pre-transfusion haemoglobin and red cells transfused during lockdown phase as compared to pre-lockdown phase. Regular care was interrupted in 45% of patients and 76% of patients getting blood from outside could not get leukoreduced red cells. Investigations, monitoring and continuity of iron chelation were also affected. Blood centre faced 30.5% reduction in blood supply during lockdown. TDCC and blood centre took several steps, including prolongation of service hours and staggering of transfusions to ensure maximum transfusions while ensuring social distancing. The COVID-19 pandemic imposed many unprecedented challenges to the routine care of thalassaemic patients; however, some of them could be dealt with by a proactive approach and micro-planning at the institution level. Other similar resource-limited settings could learn from experiences for continued quality care for chronic medical conditions during pandemic like situations.

Parakh N ，Pahuja S ，Singh V ，Kumar N ，Chandra J ... -  《-》

Quality of Life in Transfusion-Dependent Thalassemia Patients in Greece Before and During the COVID-19 Pandemic.

The peak of the COVID-19 pandemic was a challenging situation for transfusion-dependent thalassemia (TDT) patients. The objectives of this study were to measure the quality of life (QoL) in TDT patients during the COVID-19 lockdown restriction measures, compare the results with the pre-COVID-19 era, and evaluate the influence of sociodemographic and clinical factors on QoL. This was a cross-sectional study of 110 consecutively selected adult TDT patients, during the stringent lockdown restriction measures implemented in Greece. All participants completed a combination of 2 QoL questionnaires, the generic Short-Form Health Survey 36 version 2 and the disease-specific Transfusion-Quality of life (TranQol). We used the "1/2 SD method," a distribution-based approach to calculate minimal clinically important differences and clinically compare the QoL scores between the pre-COVID-19 and post-COVID-19 era. A backward stepwise linear regression was selected to explore the influence of potential predictors on TranQol scores. The Short-Form Health Survey 36 version 2 and TranQol scores remained low but not clinically different compared with the pre-COVID-19 era. Older, married, and higher educated TDT patients exhibited significantly lower TranQol summary scores. The patients who reported a negative effect of the COVID-19 pandemic had significantly lower TranQol scores in summary and all subdomains except for school and career. During the COVID-19 pandemic, the overall QoL of TDT patients was clinically similar to the status of the pre-COVID-19 era. Nevertheless, most of the significant QoL subdomains were negatively affected, and distinct groups of TDT patients were more vulnerable.

Klonizakis P ，Klaassen RJ ，Roy N ，Papatsouma I ，Mainou M ，Christodoulou I ，Tsapas A ，Vlachaki E ... -  《-》

The impact of COVID-19 on transfusion-dependent thalassemia patients of Karachi, Pakistan: A single-center experience.

With the advent of COVID-19 in Pakistan, the already fragmented blood transfusion services (BTS) received a severe blow, putting the lives of transfusion-dependent thalassemia children on stake. This study aimed to assess the impact of the COVID-19 on blood transfusion therapy (BTT) of thalassemia patients and suggest ways to ensure safe and reliable blood supplies amid such health crises. A retrospective, cross-sectional study was conducted from October 2019 (before COVID-19) to July 2020 (during COVID-19) based on the data provided by a thalassemia center, named Help International Welfare Trust, Karachi, Pakistan. SPSS version 24.0 was used for the data analysis. Data were described in the form of means and percentages. There was a significant reduction in the consumption of PRBCs bags after the emergence of COVID-19 (P=0.002). Moreover, the number of thalassemia patients receiving BTT was dropped by 10.56% during the pandemic. There was a strong negative correlation observed between the rising cases of COVID-19 in Pakistan and the number of patients missing their therapy sessions (r=-0.914, P=0.030). A considerable decline in the reserves of all Rhesus-negative blood groups amid the COVID-19 outbreak was also observed. The COVID-19 pandemic adversely affected the already suboptimal care catered to thalassemia patients in Karachi, Pakistan. The fear of the virus contraction coupled with the lockdown and restricted mobility has disrupted the entire transfusion chain from donor to the recipient. Collaborated efforts by the government and healthcare authorities are essential to ensure sufficient blood for thalassemia patients amid the pandemic.

Arshad Ali S ，Azim D ，Hassan HM ，Iqbal A ，Ahmed N ，Kumar S ，Nasim S ... -  《-》

COVID-19 and thalassaemia: A position statement of the Thalassaemia International Federation.

Many patients with haemoglobinopathies, including thalassaemia and sickle cell disease, are at increased risk of developing severe complications from the coronavirus disease 2019 (COVID-19). Although epidemiologic evidence concerning the novel coronavirus (SARS-CoV-2) infection in these patients is currently lacking, the COVID-19 pandemic represents a significant challenge for haemoglobinopathy patients, their families and their attending physicians. The present statement summarizes the key challenges concerning the management of haemoglobinopathies, with particular focus on patients with either transfusion-dependent or non-transfusion-dependent thalassaemia, identifies the gaps in knowledge and suggests measures and strategies to deal with the pandemic, based on available evidence and expert opinions. Key areas covered include patients' risk level, adaptation of haemoglobinopathy care, safety of blood transfusions, blood supply challenges, and lifestyle and nutritional considerations. The proposed measures and strategies may be useful as a blueprint for other disorders which require regular hospital visits, as well as for the timely adaptation of patient care during similar future pandemics.

Farmakis D ，Giakoumis A ，Cannon L ，Angastiniotis M ，Eleftheriou A ... -  《-》

Clinical and health-related quality of life outcomes of transfusion-dependent thalassaemia patients in Singapore.

Transfusion-dependent thalassaemia is associated with complications related to iron overload from frequent red cell transfusions which affect quality of life. We collected data on the clinical outcomes, complications, socioeconomic status and health-related quality of life (HRQoL) of transfusion-dependent thalassaemia patients in Singapore, and analysed the associations between clinical and socioeconomic factors with development of transfusion-related complications and HRQoL scores. This was a cross-sectional study of transfusion-dependent thalassaemia patients treated at four major public hospitals in Singapore. Clinical information was obtained from retrospective reviews of medical records. Socioeconomic data and patient-reported compliance to iron chelators were obtained from prospective interviews of patients or caregivers using a questionnaire. A validated, disease-specific HRQoL instrument, the TranQOL, was administered to patients and caregivers during a routine clinic or transfusion visit. Liver iron loading was the most common transfusion-related complication and occurred in 79% of patients. Cardiac iron loading was noted in 28.3% and endocrine complications were present in 34.2%. Liver iron loading was significantly associated with higher mean ferritin level. Cardiac iron loading was significantly associated with increasing age, higher mean ferritin level and type of iron chelator. Endocrine complications were associated with increasing age, higher mean ferritin level, type of iron chelator and poorer patient-reported compliance to iron chelators. The lowest TranQOL scores were reported by caregiver parents of patients aged less than 18 years. Lower TranQOL scores were significantly associated with increasing age, especially in the 31-50 age cohort, and with reception of social assistance. The main morbidities noted in transfusion-dependent thalassaemia patients in Singapore are from complications associated with iron loading. The cohort of older thalassaemia patients aged 31-50 experienced significantly higher rates of cardiac iron loading, endocrine complications and lower TranQOL scores compared to younger age cohorts.

Lam JCM ，Lee SY ，Koh PL ，Fong SZ ，Abdul-Kadir NI ，Lim CY ，Zhang X ，Bhattacharyya R ，Soh SY ，Chan MY ，Tan AM ，Kuperan P ，Ang AL ... -  《-》