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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
3.
Bone Marrow Transplant ; 57(11): 1689-1697, 2022 11.
Article in English | MEDLINE | ID: covidwho-2000873

ABSTRACT

In this retrospective study, we evaluated long-term survival and late effects in 137 patients affected by thalassemia major (TM) who received an allogeneic hematopoietic cell transplantation (HCT). Median age at HCT was 10.1 years. After a median follow-up of 30 years, 114 (83.2%) patients are living and 108 (78.8%) are cured. The cumulative incidence of nonrelapse mortality and thalassemia recurrence was 9.5% at 1 year and 10.2% at 39 years respectively. The 39-years cumulative incidence of overall survival and disease-free survival were 81.4% and 74.5%. One hundred twenty-three patients who survived more than 2 years after HCT were evaluated for late effects concerning hematological disorders, iron burden, growth, obesity, diabetes mellitus, thyroid and gonadal function, eye, heart, liver, lung, kidney, gastrointestinal, neurologic and psychiatric system, osteoarticular system, secondary solid cancer (SSC), performance status, and Covid-19 infection. Fertility was preserved in 21 males whose partners delivered 34 neonates and 25 females who delivered 26 neonates. Fifteen cases of SSC were diagnosed for a 39-year cumulative incidence of 16.4%. HCT represents a definitive cure for the majority of TM patients at the price, however, of a non-negligible early and late mortality which in the long run affects survival and disease-free survival.


Subject(s)
COVID-19 , Hematopoietic Stem Cell Transplantation , Neoplasms, Second Primary , beta-Thalassemia , Male , Female , Infant, Newborn , Humans , Child , beta-Thalassemia/therapy , Retrospective Studies , Hematopoietic Stem Cell Transplantation/adverse effects , Disease-Free Survival , Neoplasms, Second Primary/etiology , Disease Progression , Transplantation Conditioning/adverse effects
4.
Indian Pediatr ; 59(7): 531-534, 2022 07 15.
Article in English | MEDLINE | ID: covidwho-1958369

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
5.
Indian Pediatr ; 59(7): 531-534, 2022 07 15.
Article in English | MEDLINE | ID: covidwho-1857705

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
6.
Microbes Infect ; 24(6-7): 104976, 2022 09.
Article in English | MEDLINE | ID: covidwho-1773645

ABSTRACT

One of the most urgent needs worldwide is to vaccinate against SARS-CoV-2 as many people as possible. We evaluated humoral response to Comirnaty vaccine in Thalassemia Major patients (TM). We measured SARS-CoV-2-specific antibodies against Spike protein in 57 TM patients and 58 healthy blood donors (HBD). TM and HBD subjects revealed a homogeneous serological response to the Comirnaty (Mean ± SD; TM = 1917,21 ± 1384,49; HBD = 2039,81 ± 1064,44; p = 0,5884). No statistically significant differences were observed among two groups. Interestingly, we observed in 73.3% of asplenic patients Ab-S titres above 800 BAU, whereas only in 26% of non splenectomized patients showed Ab-S titres above 800 BAU). This differences were statistically significant p < 0.039. Further measurement on other Ab types was needed for better understanding humoral response to Comirnaty.


Subject(s)
COVID-19 , Viral Vaccines , beta-Thalassemia , Antibodies, Viral , BNT162 Vaccine , COVID-19/prevention & control , Humans , Immunity, Humoral , RNA, Viral , SARS-CoV-2/genetics , Spike Glycoprotein, Coronavirus/genetics , Vaccination , Vaccines, Synthetic , mRNA Vaccines
7.
Int J Mol Med ; 49(3)2022 Mar.
Article in English | MEDLINE | ID: covidwho-1643661

ABSTRACT

The pandemic caused by the severe acute respiratory syndrome coronavirus (SARS­CoV­2), responsible for coronavirus disease 2019 (COVID­19) has posed a major challenge for global health. In order to successfully combat SARS­CoV­2, the development of effective COVID­19 vaccines is crucial. In this context, recent studies have highlighted a high COVID­19 mortality rate in patients affected by ß­thalassemia, probably due to their co­existent immune deficiencies. In addition to a role in the severity of SARS­CoV­2 infection and in the mortality rate of COVID­19­infected patients with thalassemia, immunosuppression is expected to deeply affect the effectivity of anti­COVID­19 vaccines. In the context of the interplay between thalassemia­associated immunosuppression and the effectiveness of COVID­19 vaccines, the employment of immunomodulatory molecules is hypothesized. For instance, short­term treatment with mammalian target of rapamycin inhibitors (such as everolimus and sirolimus) has been found to improve responses to influenza vaccination in adults, with benefits possibly persisting for a year following treatment. Recently, sirolimus has been considered for the therapy of hemoglobinopathies (including ß­thalassemia). Sirolimus induces the expression of fetal hemoglobin (and this may contribute to the amelioration of the clinical parameters of patients with ß­thalassemia) and induces autophagy (thereby reducing the excessive levels of α­globin). It may also finally contribute to the mobilization of erythroid cells from the bone marrow (thereby reducing anemia). In the present study, the authors present the hypothesis that sirolimus treatment, in addition to its beneficial effects on erythroid­related parameters, may play a crucial role in sustaining the effects of COVID­19 vaccination in patients with ß­thalassemia. This hypothesis is based on several publications demonstrating the effects of sirolimus treatment on the immune system.


Subject(s)
COVID-19 Vaccines/therapeutic use , Sirolimus/therapeutic use , beta-Thalassemia/therapy , COVID-19/complications , COVID-19/mortality , COVID-19/pathology , COVID-19/prevention & control , Combined Modality Therapy , Humans , SARS-CoV-2/drug effects , SARS-CoV-2/immunology , Severity of Illness Index , Sirolimus/pharmacology , Treatment Outcome , Vaccination/methods , beta-Thalassemia/complications , beta-Thalassemia/immunology
8.
Ocul Immunol Inflamm ; 30(5): 1222-1227, 2022 Jul.
Article in English | MEDLINE | ID: covidwho-1637290

ABSTRACT

INTRODUCTION: Corona virus disease (COVID-19) has been associated with a variety of ophthalmic manifestations including acute macular neuroretinopathy and paracentral middle maculopathy. Posterior segment manifestations after post COVID-19 vaccinations have been reported. CASE REPORT: A 25- year-old Asian Indian female developed sequential bilateral AMN following a single dose of COVISHIELD™ vaccine. On investigations she was found to have a ß thalassemia trait. Presentation started unilaterally and progressed to the other eye after a month. On clinical examination, the fundus was apparently normal in both eyes. Significant changes suggestive of AMN were seen on multimodal imaging of the posterior segment of the respective eye at the time of involvement which resolved spontaneously in due course. CONCLUSION: We report a temporal association of COVID-19 vaccination and AMN.


Subject(s)
COVID-19 Vaccines , COVID-19 , Macula Lutea , White Dot Syndromes , beta-Thalassemia , Adult , Female , Humans , Acute Disease , beta-Thalassemia/complications , beta-Thalassemia/diagnosis , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Multimodal Imaging , Tomography, Optical Coherence/methods , Vaccination , White Dot Syndromes/chemically induced
9.
Blood Cells Mol Dis ; 92: 102627, 2021 12.
Article in English | MEDLINE | ID: covidwho-1520713

ABSTRACT

BACKGROUND: Equipoise exists regarding sickle cell disease (SCD) as a risk factor for COVID-19 disease severity and variables that increase risk of COVID-19 severity in SCD. Given our health system's large SCD patient catchment, we analyzed our own experience in this regard. STUDY METHODS: Retrospective analysis of the clinical course and factors associated with need for hospitalization and ICU admission of SCD patients diagnosed with COVID-19 through the Northwell Health system from March 1 to Dec 31, 2020. RESULTS: Of 1098 patients with SCD, 3.3% were diagnosed with COVID-19. Overall rates of hospitalization, ICU admission, cohort mortality, and in-hospital mortality were 80%, 19%, 2.5%,and 3.1%, respectively. By multivariable analysis, hospitalization risk was decreased by 60% for every 1 g/dL increase in admission Hb. ICU admission risk was increased by 84% as a health care worker; increased by 45% for every 1000/uL increase in admission immature granulocyte count; and decreased by 17% with hydroxyurea use. DISCUSSION: High hospitalization rates are compatible with worsened severity upon COVID-19 infection in SCD compared to the general population. Patients should be placed on hydroxyurea to increase their Hb and perhaps lower their neutrophil counts. Health care workers with SCD may warrant special safety precautions.


Subject(s)
Anemia, Sickle Cell/complications , COVID-19/complications , SARS-CoV-2 , Adult , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/drug therapy , Anemia, Sickle Cell/genetics , Child , Female , Genotype , Health Personnel , Hospitalization/statistics & numerical data , Humans , Hydroxyurea/therapeutic use , Intensive Care Units , Male , Prognosis , Retrospective Studies , Risk Factors , Severity of Illness Index , Sickle Cell Trait/complications , beta-Thalassemia/complications
10.
Prim Health Care Res Dev ; 22: e67, 2021 11 10.
Article in English | MEDLINE | ID: covidwho-1510538

ABSTRACT

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic and the resulting measures can impact daily life and healthcare management amongst patients with beta thalassemia major. METHODS: The Corbin and Strauss method of grounded theory was used to explore the impact of the COVID-19 pandemic on health-related quality of life (HRQoL) amongst Iranian patients with beta thalassemia major. Semi-structured interviews with 16 patients with thalassemia major in the eastern of Iran were performed. Data collection was conducted from 19 September through 18 November 2020. Collected data were recorded, transcribed, and coded to develop themes and subthemes. Paradigm components were sought to find out what happened to these patients and explore the process and events. RESULTS: Insights from these interviews led to five major themes: 'changing physical health', 'emotional and psychological reactions', 'changing the nature of relationships and the scope of social support', 'metamorphosis of ongoing healthcare, and 'functionality and adaptation to new realities.' The emerging core concept was labelled: 'maintaining well-being balance.' The COVID-19 pandemic disturbed the balance of life and health of the patients. Multiple strategies to maintain balance and reduce the negative effects of the COVID-19 pandemic on HRQoL were used by the patients, the healthcare team, and support systems. CONCLUSIONS: Due to the fear of COVID-19, the patients with beta thalassemia were less likely to contact healthcare professionals. They considered postponing blood transfusion and abandoned evaluating disease complications. Reduced access to the healthcare system and shifting resources from existing programmes to COVID-19 by the healthcare system were incompatible policies. These policies and strategies had strong and negative effects on the physical domain of HRQoL. The patients experienced a deterioration of emotional functioning. They reported a strong reduction in social functioning and felt lonely. Online interventions supporting mental health and social interactions and telemedicine can help during the times of social distancing and lockdowns.


Subject(s)
COVID-19 , beta-Thalassemia , Communicable Disease Control , Grounded Theory , Humans , Iran , Pandemics , Quality of Life , SARS-CoV-2 , beta-Thalassemia/therapy
14.
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
17.
Hemoglobin ; 45(6): 371-379, 2021 Nov.
Article in English | MEDLINE | ID: covidwho-1240824

ABSTRACT

During the coronavirus-19 disease (COVID-19) pandemic, several studies were performed to determine the mortality and incidence rates of coronavirus infection among patients with hemoglobinopathies. However, there has been no systematic approach or meta-analysis to evaluate the results worldwide. This meta-analysis summarized the existing evidence of incidence and mortality rates of COVID-19 and related risk factors among patients with hemoglobinopathies with a focus on ß-thalassemia (ß-thal) and sickle cell disease. The review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Two authors independently screened the articles, extracted eligible ones, and assessed the quality of studies using the Joanna Briggs Institute (JBI) checklist. The collected data were analyzed by the Stata software. The amount of heterogeneity was demonstrated by the I2 test. The incidence of COVID-19 among patients with a hemoglobinopathy, ß-thal and sickle cell disease was 4.44, 1.34, and 17.22 per 100,000 person-day, respectively, to June 15 2020. The mortality rate of COVID-19 in patients with hemoglobin (Hb) disorders was calculated as 1.07 per 1000 person-day in the same period. Our findings showed a higher incidence rate of COVID-19 in sickle cell disease patients compared to the general population. A slightly higher mortality rate was also observed in patients with hemoglobinopathies compared to the general population, possibly due to the associated risk factors and comorbidities in this vulnerable group, which underscore special care, timely diagnosis and management along with current immunization, were crucial in decreasing the frequency, disease severity and mortality of these patients.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Hemoglobinopathies , beta-Thalassemia , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , COVID-19/epidemiology , Hemoglobinopathies/epidemiology , Humans , Incidence , beta-Thalassemia/epidemiology
18.
Dev World Bioeth ; 22(1): 44-52, 2022 03.
Article in English | MEDLINE | ID: covidwho-1223477

ABSTRACT

In this paper, I argue that genetic screening for beta thalassemia major is ethically justified in the context of Vietnam. First, I differentiate genetic screening from the moral objections commonly associated with eugenics on the basis of the primary motive for screening (avoidance of suffering) and the preservation of voluntary choice. To lay the groundwork for ethical discussion, I explain the basics of beta thalassemia biochemistry and screening and the clinical picture of beta thalassemia major. I then elaborate on a specific example of the challenges of beta thalassemia major in Cyprus before moving on to the case of Vietnam and discuss the improbability of treatment for this disorder in Vietnam and therefore, the extensive suffering that it causes the Vietnamese people. This leads to my argument that a beta thalassemia screening in Vietnam would hold up the ethical principle of nonmaleficence and also preserves and enhances reproductive choice. I then propose that Vietnam's successful COVID-19 response can be used as a roadmap for beta thalassemia screening.


Subject(s)
COVID-19 , beta-Thalassemia , Genetic Testing , Humans , SARS-CoV-2 , Vietnam , beta-Thalassemia/diagnosis , beta-Thalassemia/genetics
19.
Cochrane Database Syst Rev ; 3: CD003427, 2021 03 08.
Article in English | MEDLINE | ID: covidwho-1135064

ABSTRACT

BACKGROUND: Sickle cell disease (SCD) is a group of inherited disorders that result in haemoglobin abnormalities and other complications. Injury to the spleen, among other factors, contribute to persons with SCD being particularly susceptible to infection. Infants and very young children are especially vulnerable. The 'Co-operative Study of Sickle Cell Disease' observed an incidence rate for pneumococcal septicaemia of 10 per 100 person-years in children under the age of three years. Vaccines, including customary pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic penicillin regimens may be advisable for this population. This is an update of a Cochrane Review which was first published in 2002, and previously updated, most recently in 2017.  OBJECTIVES: To compare the effects of antibiotic prophylaxis against pneumococcus in children with SCD receiving antibiotic prophylaxis compared to those without in relation to: 1. incidence of Streptococcus pneumoniae infection; 2. mortality (as reported in the included studies); 3. drug-related adverse events (as reported in the included studies) to the individual and the community; 4. the impact of discontinuing at various ages on incidence of infection and mortality. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which is comprised of references identified from comprehensive electronic database searches and also two clinical trials registries: ClinicalTrials.gov and the WHO International Registry Platform (not in 2020 given access issues relating to Covid-19 pandemic). Additionally, we carried out hand searching of relevant journals and abstract books of conference proceedings. Date of the most recent search: 25 January 2021. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing prophylactic antibiotics to prevent pneumococcal infection in children with SCD with placebo, no treatment or a comparator drug. DATA COLLECTION AND ANALYSIS: The standard methodological procedures expected by Cochrane were used. Both authors independently extracted data and assessed trial quality. The authors used the GRADE criteria to assess the certainty of the evidence. MAIN RESULTS: Six trials were identified by the searches, of which three trials were eligible for inclusion. A total of 880 children, who were between three months to five years of age at randomization were included. The included studies were conducted in centres in the USA and in Kingston, Jamaica. In trials that investigated initiation of penicillin on risk of pneumococcal infection, the odds ratio was 0.37 (95% confidence interval 0.16 to 0.86) (two trials, 457 children) (low-certainty evidence), while for withdrawal the odds ratio was 0.49 (95% confidence interval 0.09 to 2.71) (one trial, 400 children) (low-certainty evidence). Adverse drug effects were rare and minor. Rates of pneumococcal infection were found to be relatively low in children over the age of five years. Overall, the certainty of the evidence for all outcomes was judged to be low. The results from the risk of bias assessment undertaken identified two domains in which the risk of bias was considered to be high, these were incomplete outcome data (attrition bias) (two trials) and allocation concealment (selection bias) (one trial). Domains considered to have a low risk of bias for all three trials were selective reporting (reporting bias) and blinding (performance and detection bias). AUTHORS' CONCLUSIONS: The evidence examined was determined to be of low certainty and suggests that prophylactic penicillin significantly reduces risk of pneumococcal infection in children with homozygous SCD, and is associated with minimal adverse reactions. Further research may help to determine the ideal age to safely withdraw penicillin.


Subject(s)
Anemia, Sickle Cell/complications , Antibiotic Prophylaxis , Penicillins/therapeutic use , Pneumococcal Infections/prevention & control , Age Factors , Anemia, Sickle Cell/genetics , Antibiotic Prophylaxis/adverse effects , Bias , Child, Preschool , Hemoglobin SC Disease/complications , Homozygote , Humans , Incidence , Infant , Medication Adherence , Penicillins/adverse effects , Pneumococcal Infections/epidemiology , Pneumococcal Infections/mortality , Randomized Controlled Trials as Topic , Streptococcus pneumoniae , beta-Thalassemia/complications
20.
Transfus Clin Biol ; 28(1): 60-67, 2021 Feb.
Article in English | MEDLINE | ID: covidwho-1065637

ABSTRACT

OBJECTIVES: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.


Subject(s)
Blood Donors/supply & distribution , Blood Transfusion/statistics & numerical data , COVID-19 , Continuity of Patient Care , Pandemics , SARS-CoV-2 , beta-Thalassemia/therapy , Adolescent , Blood Grouping and Crossmatching , Child , Child, Preschool , Cross-Sectional Studies , Female , Health Services Accessibility , Humans , Male , Pakistan/epidemiology , Patient Acceptance of Health Care , Patient Compliance , Procedures and Techniques Utilization , Retrospective Studies , Rural Population , beta-Thalassemia/epidemiology
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