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1.
Indian Pediatr ; 59(7): 531-534, 2022 07 15.
Article in English | MEDLINE | ID: covidwho-2092983

ABSTRACT

OBJECTIVE: To evaluate pulmonary functions in children with transfusion-dependent thalassemia, and its reversal (lung dysfunction) using intensive intravenous chelation with desferrioxamine (DFO) (4 weeks). METHODS: This descriptive study enrolled 77 children with transfusion-dependent thalassemia. Pulmonary function test (PFT) and iron load (serum ferritin (SF) and T2* MRI of heart and liver) were done. PFT included spirometry, total lung capacity (TLC) by helium dilution test and diffusion capacity by carbon monoxide (DLCO). Follow-up PFT was available for 13 children with moderate to severe lung dysfunction given intravenous DFO. RESULTS: 50 (68.8%) patients had lung dysfunction, most commonly diffusional impairment (48; 96%), and reduced TLC (11; 22%); and none had obstructive pattern. 9 (81.8%) patients with restrictive defect had moderate to severely deranged DLCO. PFT and T2* MRI values were inversely correlated with serum ferritin. Among 13 patients receiving intensive chelation for 4 weeks, significant improvement was noticed in forced expiratory volume in one minute/ forced vital capacity ratio (DFEV1/FVC) (P=0.009), DDLCO (P=0.006) and DSF (P=0.01). CONCLUSIONS: Pulmonary dysfunction is common in children with multi-transfused thalassemia, and routine screening by PFT needs to be part of the management guidelines.


Subject(s)
COVID-19 , Thalassemia , beta-Thalassemia , COVID-19/epidemiology , Child , Ferritins , Humans , Lung/diagnostic imaging , Pandemics , SARS-CoV-2
2.
Medicina (Kaunas) ; 58(9)2022 Sep 15.
Article in English | MEDLINE | ID: covidwho-2043857

ABSTRACT

Sickle cell disease and thalassaemia are life-long haematological diseases that can impact the quality of life of patients. This impact on quality of life can require intermittent psychological input throughout the lifespan for management. Managing everyday life during the COVID-19 pandemic could be challenging for people with these health conditions, which could impact their health, their mood and anxiety, their perception of control, and their engagement with their regular healthcare services. This report describes the characteristics of these health conditions and discusses reflections, from a specialist psychology service working with this clinical population, about the impact of COVID-19 on patient engagement with the service. The main aim of this report is to highlight the relevance and usefulness of videoconferencing as a therapy format, suggest implications for further service development and suggest alternate ways of working therapeutically with clients.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Thalassemia , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy , Humans , Pandemics , Quality of Life , Thalassemia/complications , Thalassemia/therapy
3.
Hemoglobin ; 46(2): 143-145, 2022 Mar.
Article in English | MEDLINE | ID: covidwho-2028800

ABSTRACT

Healthcare systems around the globe are still facing the evolving threat of the coronavavirus-19 (COVID-19) pandemic. Hemoglobinopathies include a group of genetic disorders, with the two main entities being thalassemias and sickle cell disease. Due to their immunocompromised status, such patients have been protected as extremely vulnerable to COVID-19 infection. We studied patients with different hemoglobinopathies, consecutively monitored at our center, who were infected with severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) during the second and third waves of the pandemic in Greece (September 2020-April 2021), and associated the outcomes of the infection with the following factors: age, employment, blood type, liver and heart hemosiderosis, splenectomy, concomitant endocrine disorders and transfusion dependency. Among 250 patients monitored at our center, 14 were infected with COVID-19. Nine of them were hospitalized but no one required intensive care unit support and all of them responded to the generally applied treatment plan, despite their comorbidities. Notwithstanding the slightly increased prevalence of COVID-19 in patients with hemoglobinopathies compared to the general population, self-applied measures are still thought to be effective, as our patients got infected through their already sick family members.


Subject(s)
COVID-19 , Hemoglobinopathies , Thalassemia , COVID-19/epidemiology , Greece/epidemiology , Hemoglobinopathies/epidemiology , Humans , SARS-CoV-2 , Thalassemia/epidemiology , Thalassemia/therapy
4.
J Paediatr Child Health ; 58(10): 1760-1765, 2022 10.
Article in English | MEDLINE | ID: covidwho-1916257

ABSTRACT

AIM: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.


Subject(s)
COVID-19 , Thalassemia , Blood Transfusion , COVID-19/epidemiology , Child , Communicable Disease Control , Humans , Iron Chelating Agents , Pandemics , Thalassemia/complications , Thalassemia/epidemiology , Thalassemia/therapy
6.
J Cell Mol Med ; 26(9): 2520-2528, 2022 05.
Article in English | MEDLINE | ID: covidwho-1769729

ABSTRACT

Although numerous patient-specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalassaemic syndromes in COVID-19 patients remains poorly understood. We studied the outcomes of 137 COVID-19 patients with a history of transfusion-dependent thalassaemia (TDT) and transfusion independent thalassaemia (TIT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalassaemia. The mean age of thalassaemia patients included in our study was 41 ± 16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 40% of thalassaemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassaemia group compared to the matched cohort with no history of thalassaemia. Amongst thalassaemia patients in general, the TIT group exhibited a higher rate of hospitalization compared to the TDT group (p = 0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the TIT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassaemia (either TDT or TIT) was found to be independently associated with reduced all-cause mortality. The presence of thalassaemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models.


Subject(s)
COVID-19 , Iron Overload , Thalassemia , COVID-19/complications , Female , Hospitals , Humans , Iron Overload/etiology , Male , Oxygen , Registries , Thalassemia/complications , Thalassemia/therapy
7.
Br J Haematol ; 197(5): 576-579, 2022 06.
Article in English | MEDLINE | ID: covidwho-1741343

ABSTRACT

Patients with transfusion-dependent thalassaemia (TDT) are considered an at increased-risk population for severe and/or morbid coronavirus disease 2019 (COVID-19) infection. Timely vaccination is the main preventive method for severe COVID-19. Different adverse events and reactions after vaccination have been reported, with severe ones being extremely rare. Patients with TDT may have altered immunity due to chronic transfusions, iron overload and chelation therapy, and splenic dysfunction. Here, we show that adult patients with TDT following vaccination with the novel messenger RNA vaccines have mild adverse events and can produce protective antibodies comparable to the healthy population.


Subject(s)
COVID-19 , Thalassemia , Adult , Antibodies, Viral , COVID-19/prevention & control , Humans , Immunity , SARS-CoV-2 , Thalassemia/complications , Thalassemia/therapy , Vaccination/adverse effects
8.
Am J Hematol ; 97(2): E75-E78, 2022 02 01.
Article in English | MEDLINE | ID: covidwho-1565162
9.
authorea preprints; 2021.
Preprint in English | PREPRINT-AUTHOREA PREPRINTS | ID: ppzbmed-10.22541.au.163372376.68664123.v1

ABSTRACT

Patients with thalassemia syndromes(TS) affected with COVID 19 attending a thalassemia center in Sri Lanka situated in the region most affected with COVID 19 were studied over a 16- month period. To assess the collateral effects on overall thalassemia care in the center, data on transfusion, chelation, clinic attendance was analyzed. Morbidity events and deaths recorded during the COVID 19 period and a similar period before the beginning of COVID 19 infection in Sri Lanka too were reordered in all clinic registrants. Seven patients (of 502) with TS had developed COVID 19 during the 16-month period and all were minimally symptomatic and had recovered without complications. Number of monthly clinic visits reduced from 338 in pre-COVID19 to 268 during COVID-19 (P = 0.004). Iron chelators usage too reduced during pandemic period (P < 0.001). Though admissions related to morbidity reduced during the pandemic (58 vs 16, P <0.001) there were more non COVID19 deaths (8 vs 4). Numbers affected with COVID 19 and the severity of infection seemed milder in this cohort of patients with TS but the collateral effects on the management of the unit and its effects on mortality in this vulnerable population appears to have been substantial.


Subject(s)
Tourette Syndrome , Thalassemia , COVID-19
10.
Transfus Clin Biol ; 29(1): 70-74, 2022 Feb.
Article in English | MEDLINE | ID: covidwho-1317783

ABSTRACT

INTRODUCTION: Because of iron overload complications, thrombosis and infectious predisposition, patients with severe forms of thalassemia are likely to be at increased risk of COVID-19 complications. RESULTS: A national survey conducted during the year 2020 across the French reference centers for hemoglobinopathies identified 16 cases of COVID-19 confirmed by RT-PCR in beta-thalassemia patients. Their age ranged from 11 months to 60 years. 15 patients were transfusion-dependent and 6 were splenectomized. Concerning iron overload related complications, none had diabetes or cirrhosis and only one had experienced heart failure. All 4 pediatric patients were pauci-symptomatic during the viral episode. Three patients (41, 49 and 57 years old) developed COVID-19 pneumonia requiring oxygen therapy without the need for mechanical ventilation. Neutropenia (absolute neutrophils count <0.5 10 9/L) was observed in 2 patients receiving long-term treatment with hydroxycarbamide and deferiprone. No thrombosis event, organ failure or death occurred. All patients recovered. CONCLUSION: Severity of COVID-19 in this population of young and middle-aged patients appeared increased compared to the general population but remained mild to moderate as already described in the few series reported in the literature. Occurrence of adverse events related to chronic treatment administered in thalassemia disease may be favored by the infectious episode.


Subject(s)
COVID-19 , Iron Overload , Thalassemia , beta-Thalassemia , Child , Humans , Infant , Iron Overload/epidemiology , Iron Overload/etiology , Middle Aged , SARS-CoV-2 , beta-Thalassemia/complications , beta-Thalassemia/therapy
16.
Transfus Clin Biol ; 28(2): 129-131, 2021 05.
Article in English | MEDLINE | ID: covidwho-1189034
17.
Pan Afr Med J ; 38: 33, 2021.
Article in English | MEDLINE | ID: covidwho-1134472

ABSTRACT

The COVID-19 pandemic could have a major impact on the capacity of health systems to continue the delivery of essential health service. While health systems around the world are being challenged by increasing demand for care of COVID-19 patients, it is critical to maintain preventive and curative services, especially for the most vulnerable populations such people living with chronic conditions like thallasemics. In this context and since the start of the SARS-CoV-2 health crisis, the National Blood Transfusion Center of Morocco has ranked among its priorities the need to maintain transfusion management for chronic polytransfused patients, particularly those with thalassemia. We report in this paper, the case of a thallasemic patient whose transfusion management was disrupted by the restrictive measures introduced by the Moroccan authorities and for which the National Blood Center of Morocco provided effective support.


Subject(s)
Blood Transfusion , COVID-19 , Continuity of Patient Care , Thalassemia/therapy , Adolescent , Female , Humans , Morocco
18.
ssrn; 2021.
Preprint in English | PREPRINT-SSRN | ID: ppzbmed-10.2139.ssrn.3801721

ABSTRACT

Background: Although numerous patient specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalasemic syndromes in COVID-19 patients remains poorly understood.Aims: We studied the outcomes of 137 COVID-19 patients with a history of Transfusion Dependent Thalassemia (TDT) and non-Transfusion Dependent Thalassemia (NTDT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalasemia.Results: The mean age of thalassemia patients included in our study was 41±16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 25% of thalassemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to the an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassemia group compared to the matched cohort with no history of thalassemia. Amongst thalassemia patients in general, the NTDT group exhibited a higher rate of hospitalization compared to the TDT group (p=0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the NTDT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassemia (either TDT or NTDT) was found to be independently associated with reduced all-cause mortality.Conclusions: The presence of thalassemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models.Trial Registration for sources of data extraction: NCT: 04334291, 04746066Funding Statement: This study was funded by a non-conditioned grant from FUNDACIÓN INTERHOSPITALARIA PARA LA INVESTIGACIÓN CARDIOVASCULAR, FIC. (Madrid, Spain). This nonprofit institution had no role in the study design, the collection, analysis and interpretation of data, the writing of the report, or the decision to submit the paper for publication.Declaration of Interests: The authors declare no conflicts of interest.Ethics Approval Statement: The study was approved by the Ethics Committees in all of the centers involved (Banjarmasin, Bari, Cagliari, Catania, Ferrara, Gela, Genoa, Getafe, Guadalajara, Legan, Madrid, Mannheim, Milan, Monza, Naples, Olbia, Padua, Pavia, Ragusa, Rome, Salerno, Turin, Valladolid and Verona).


Subject(s)
Lung Diseases , Thalassemia , Kidney Diseases , Acute Kidney Injury , COVID-19 , Heart Diseases
19.
ssrn; 2021.
Preprint in English | PREPRINT-SSRN | ID: ppzbmed-10.2139.ssrn.3785263

ABSTRACT

Introduction: Coinfections are common in pandemics, but not in recorded patients with structural hemoglobin abnormalities. The COVID-19 pandemic struck Bangladesh at the beginning of March 2020, which is also an apt period for endemic Dengue fever in this monsoon region. Case Report: We report a 30-year-old man with hemoglobinopathies coinfected with SARS-CoV-2 and Dengue virus. Dengue virus was detected by ELISA. COVID-19 was confirmed by RT-PCR and Hemoglobin Electrophoresis revealed heterozygous beta-thalassemia. The patient was treated successfully at Dhaka Hospital in ICDDR,B during COVID-19 emergency response with symptomatic supportive treatment for COVID-19 and appropriate fluid therapy for dengue fever in response to daily hematocrit level. After 21 day, the patient’s RT-PCR for COVID-19 became negative. For thalassemia, the patient was advised to have genetic counseling and family screening on discharge.Conclusion: The possibility of coinfection should be considered during the Dengue fever epidemic along with the confirmatory tests for COVID-19, when any alarming signs supporting possibility of dengue fever are found in an individual. Fluid therapy for dengue fever must be maintained with utmost care to avoid adverse effect from COVID-19-Dengue coinfection and careful monitoring of oxygen saturation during the sick period of this type of case should be performed with importance.


Subject(s)
Coinfection , Hemoglobin C Disease , beta-Thalassemia , Thalassemia , COVID-19 , Dengue
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