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1.
Am J Emerg Med ; 58: 235-244, 2022 08.
Article in English | MEDLINE | ID: covidwho-1914107

ABSTRACT

INTRODUCTION: Acute chest syndrome (ACS) in sickle cell disease (SCD) is a serious condition that carries with it a high rate of morbidity and mortality. OBJECTIVE: This review highlights the pearls and pitfalls of ACS in SCD, including diagnosis and management in the emergency department (ED) based on current evidence. DISCUSSION: ACS is defined by respiratory symptoms and/or fever and a new radiodensity on chest imaging in a patient with SCD. There are a variety of inciting causes, including infectious and non-infectious etiologies. Although ACS is more common in those with homozygous SCD, clinicians should consider ACS in all SCD patients, as ACS is a leading cause of death in SCD. Patients typically present with or develop respiratory symptoms including fever, cough, chest pain, and shortness of breath, which can progress to respiratory failure requiring mechanical ventilation in 20% of adult patients. However, the initial presentation can vary. While the first line imaging modality is classically chest radiograph, lung ultrasound has demonstrated promise. Further imaging to include computed tomography may be necessary. Management focuses on analgesia, oxygen supplementation, incentive spirometry, bronchodilators, rehydration, antibiotics, consideration for transfusion, and specialist consultation. Empiric antibiotics that cover atypical pathogens are necessary along with measures to increase oxygen-carrying capacity in those with hypoxemia such as simple transfusion or exchange transfusion. CONCLUSIONS: An understanding of ACS can assist emergency clinicians in diagnosing and managing this potentially deadly disease.


Subject(s)
Acute Chest Syndrome , Anemia, Sickle Cell , Acute Chest Syndrome/diagnosis , Acute Chest Syndrome/epidemiology , Acute Chest Syndrome/etiology , Acute Disease , Adult , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Anti-Bacterial Agents , Chest Pain/etiology , Fever/etiology , Humans , Prevalence
2.
Blood Adv ; 6(15): 4408-4412, 2022 08 09.
Article in English | MEDLINE | ID: covidwho-1910256

ABSTRACT

Venous thromboembolism (VTE) is a life-threatening complication observed among patients with sickle cell disease (SCD) and also among those with severe COVID-19 infection. Although prior studies show that patients with SCD are at risk of severe COVID-19 illness, it remains unclear if COVID-19 infection further increases VTE risk for this population. We hypothesized that patients with SCD hospitalized for COVID-19 would have higher VTE rates than those hospitalized for other causes. Using electronic health record data from a multisite research network, TriNetX, we identified 2 groups of patients with SCD hospitalized during 2020: (1) with COVID-19 and (2) without COVID-19. We compared VTE rates using risk ratios estimated based on adjusted Poisson regression model with log link and robust error variances. Of the 281 SCD patients hospitalized with COVID-19 and 4873 SCD patients hospitalized without COVID-19 , 35 (12.46%) and 418 (8.58%) had incident VTE within 6 months of the index hospitalization respectively. After adjusting for differences in baseline characteristics, no significant differences in VTE rates within 6 months were found between the 2 groups (adjusted relative risk, 1.06 [95% confidence interval, 0.79-1.41]). These data suggest that hospitalization with COVID-19 does not further increase VTE risk in patients with SCD.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Venous Thromboembolism , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , COVID-19/complications , COVID-19/epidemiology , Humans , Retrospective Studies , Risk Factors , Venous Thromboembolism/complications , Venous Thromboembolism/etiology
4.
Paediatr Int Child Health ; 42(1): 1-4, 2022 Feb.
Article in English | MEDLINE | ID: covidwho-1890707

ABSTRACT

ABBREVIATIONS: COVID-19: Coronavirus disease 2019; HIC: high-income countries; IBD: inflammatory bowel disease; LMIC: low- and middle-income countries; PUCAL: paediatric ulcerative colitis activity index; SCD: sickle cell disease; UC: ulcerative colitis.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Colitis, Ulcerative , Inflammatory Bowel Diseases , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , COVID-19/complications , Child , Colitis, Ulcerative/complications , Colitis, Ulcerative/epidemiology , Humans , Morbidity
5.
Pediatr Blood Cancer ; 69(9): e29797, 2022 09.
Article in English | MEDLINE | ID: covidwho-1864347

ABSTRACT

Youth with sickle cell disease (SCD) and their caregivers are susceptible to stress and depression, perhaps exacerbated by pandemic-associated health and economic concerns. Most of the 50 youth-caregiver dyads enrolled in the multisite trial, Hydroxyurea Adherence for Personal Best in Sickle Cell Treatment (HABIT), took an online survey of self-reported mental health symptoms and food insecurity during the 2020 COVID-19 pandemic. Compared to largely pre-pandemic results, prevalence of mental health symptoms in dyad members appeared to have shifted: fewer youth and more caregivers were affected during the pandemic; many of both groups lacked optimism. Pandemic/post-pandemic screening of youth with SCD for mental health symptoms and food insecurity appears warranted.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Adolescent , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/psychology , COVID-19/epidemiology , Caregivers/psychology , Depression/epidemiology , Depression/etiology , Depression/psychology , Humans , Mental Health , Pandemics
6.
J Gynecol Obstet Hum Reprod ; 51(3): 102328, 2022 Mar.
Article in English | MEDLINE | ID: covidwho-1654804

ABSTRACT

INTRODUCTION: The effect of coronavirus disease (COVID-19) on pregnancy outcome in women with sickle cell disease (SCD) is unknown. OBJECTIVES: To analyze the severity of the SARS-CoV-2 infection in pregnant women with SCD and its impact on pregnancy. METHODS: This retrospective cohort study included SCD pregnant women tested positive for COVID-19 between March 2020 - February 2021. The primary endpoint was the severity of the COVID-19 infection. Secondary endpoints were pregnancy complications and fetal outcomes. RESULTS: During the study period among 82 pregnant women with SCD, 8 have presented symptoms suggestive of COVID-19 and were tested positive. A common mild clinical presentation was observed in 6 women (75%), one woman was asymptomatic and one required oxygen. The latter was admitted to the Intensive Care Unit and a cesarean section was performed in the context of an ongoing vaso-occlusive crisis and acute chest syndrome together with incidental preeclampsia. Labor was induced in another patient who developed a vaso-occlusive crisis after COVID-19 remission. Fetal outcomes were good with an average Apgar score of 10 and normal umbilical blood pH at birth. Two newborns were small-for-gestational-age as expected on the ultrasound follow-up before occurrence of COVID-19. CONCLUSION: COVID-19 infection in our population of pregnant women with SCD had typical presentation and rarely triggered a sickle cell crisis or other complications. Fetal outcomes were good and did not seem to be directly influenced by the SARS-CoV-2 virus. Further studies are required to confirm these observations as compared to the population of women with SCD without COVID-19 infection.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Pregnancy Complications, Infectious , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Cesarean Section , Female , Humans , Infant, Newborn , Pregnancy , Pregnancy Complications, Infectious/diagnosis , Pregnancy Outcome/epidemiology , Pregnant Women , Retrospective Studies , SARS-CoV-2
7.
Telemed J E Health ; 28(8): 1166-1171, 2022 Aug.
Article in English | MEDLINE | ID: covidwho-1607430

ABSTRACT

Introduction: Sickle cell anemia (SCA) is a genetic condition that predominantly affects minority populations in the United States. A lack of access to care is strongly associated with poor outcomes and quality of care among children and adolescents with SCA. The use of telehealth, which has rapidly expanded during the COVID-19 pandemic, has been shown to improve access to care for many conditions. However, the adoption of telehealth among children and adolescents with SCA is unknown. Methods: We identified children 1-17 years old with SCA continuously enrolled in Michigan Medicaid from January 2019 to December 2020. The number of in-person and telehealth outpatient visits (both urgent and routine) were summarized prepandemic (January 2019-February 2020) and during the pandemic (March 2020-December 2020); National Provider Identifier was used to identify provider specialty for telehealth visits. Results: The study population comprised 493 children with SCA with a mean age of 8.7 (±4.9) years at study entry. Prepandemic, there were 4,367 outpatient visits; 4,348 (99.6%) were in-person and 19 (0.4%) were telehealth. During the pandemic, there were 2,307 outpatient visits; 2,059 (89.3%) were in-person and 248 (10.7%) were telehealth. Telehealth visits peaked in April 2020 and declined thereafter. The majority of telehealth visits were to hematology (49%), followed by adult subspecialists (27%) and pediatrics/family medicine (14%). Discussion/Conclusions: While the overall number of outpatient visits declined during the initial months of the pandemic compared with 2019, use of telehealth rapidly increased among children and adolescents with SCA. Additional research is needed to understand patient and provider preferences for telehealth and the roles that federal and state policies can play in facilitating telehealth adoption among children and adolescents with SCA.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Telemedicine , Adolescent , Adult , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/therapy , COVID-19/epidemiology , Child , Child, Preschool , Humans , Infant , Medicaid , Pandemics , United States/epidemiology
8.
Hematology Am Soc Hematol Educ Program ; 2021(1): 190-195, 2021 12 10.
Article in English | MEDLINE | ID: covidwho-1582134

ABSTRACT

In case 1, a 14-month-old male child with sickle cell disease (SCD) was referred for evaluation for an allogeneic hematopoietic stem cell transplant (HCT). The patient had a history of dactylitis 3 times in his first year of life and febrile episodes twice at the consult. His 4-year-old sister was found to be human leukocyte antigen (HLA) identical. The patient was started on hydroxyurea (HU) at 2.5 years of age. His parents again sought consultation when he was 5 years old because of concerns about his medical condition. At the time, the patient had experienced 2 vaso-occlusive pain episodes (VOEs) requiring hospitalization during the previous 2 years. He had also experienced intermittent pain crises requiring rest at home for 2 to 3 days. The child has not attended school in person due to the COVID-19 pandemic. The family is considering HCT but is ambivalent about it because of potential toxicity. In case 2, an 8-year-old female child is 3 years out from HCT for SCD from her HLA-identical sibling. Before HCT, despite receiving HU, she had experienced >5 VOEs requiring hospitalization and 2 episodes of acute chest syndromes in the previous 3 years. She had also been missing almost 50 days of school days each year. After HCT, she is now attending school regularly and participating in all normal age-appropriate activities. The parents believe that HCT has been transformative in their child's life.


Subject(s)
Anemia, Sickle Cell/therapy , Hematopoietic Stem Cell Transplantation , Age Factors , Anemia, Sickle Cell/epidemiology , Child , Child, Preschool , Evidence-Based Medicine , Female , HLA Antigens/analysis , Hematopoietic Stem Cell Transplantation/adverse effects , Hematopoietic Stem Cell Transplantation/methods , Humans , Infant , Male , Quality of Life , Risk Factors , Siblings , Tissue Donors
9.
J Opioid Manag ; 17(6): 489-497, 2021.
Article in English | MEDLINE | ID: covidwho-1572830

ABSTRACT

OBJECTIVE: The coronavirus disease 2019 (COVID-19) has led to a rapid transition to telehealth services. It is unclear how subspecialists managing painful chronic diseases-such as sickle cell disease (SCD), an inherited hemoglobinopathy with significant disparities in access and outcomes-have viewed the transition to tele-health or altered their pain management practices. This study elicits the views of sickle cell providers regarding their transition to telehealth and their opioid prescribing patterns during the COVID-19 pandemic. DESIGN: An anonymous online survey was sent to eligible sickle cell providers. SETTING: Comprehensive sickle cell centers and/or clinics across the United States. PARTICIPANTS: Physicians and advanced practice providers providing care to SCD patients. MAIN OUTCOME MEASURES: Respondents answered questions regarding their (1) views of telehealth compared to in-person encounters and (2) opioid prescribing practices during the early months of the pandemic. RESULTS: Of the 130 eligible participants, 53 respondents from 35 different sickle cell centers completed at least 90 percent of the survey. Respondents reported a significant increase in telehealth encounters for routine and acute appointments (mean difference and standard deviation: 57.6 ± 31.9 percent, p < 0.001 and 24.4 ± 34.1 percent, p < 0.001, respectively) since COVID-19. The overwhelming majority of respondents reported no changes in their opioid prescribing patterns since COVID-19, despite increased telehealth use. Only a minority copre-scribed naloxone as a risk mitigation strategy. CONCLUSION: The rapid uptake of telehealth has not suppressed ambulatory providers' prescribing of opioids for SCD. Studies assessing the impact of the COVID-19 pandemic and telehealth on opioid prescribing practices in other painful chronic diseases are needed to ensure health equity for vulnerable pain patients.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Telemedicine , Analgesics, Opioid/therapeutic use , Anemia, Sickle Cell/diagnosis , Anemia, Sickle Cell/drug therapy , Anemia, Sickle Cell/epidemiology , Chronic Disease , Humans , Pain , Pandemics , Practice Patterns, Physicians' , SARS-CoV-2 , Surveys and Questionnaires , United States/epidemiology
11.
Lancet Haematol ; 8(10): e744-e755, 2021 Oct.
Article in English | MEDLINE | ID: covidwho-1466996

ABSTRACT

Sickle cell disease can be life-threatening or chronically debilitating for both children and adults. Worldwide, more than 300 000 children are born with sickle cell disease every year, over 75% of whom in sub-Saharan Africa. Increased awareness and early interventions, such as neonate screening and comprehensive care, have led to considerable reductions in mortality in children younger than 5 years in high-income countries. However, sickle cell disease prevention and care have largely been neglected in Africa. Without intervention, 50-90% of affected children in many sub-Saharan African countries die before their fifth birthday. Fortunately, increasing initiatives in sub-Saharan Africa are piloting interventions such as neonate screening and comprehensive care, and as mortality declines, quality of life and increased life expectancy become major targets for interventions. Hydroxyurea (hydroxycarbamide) and haematopoietic stem-cell transplantation have already been shown to be effective therapies in high-income countries, but are either not widely accessible or too expensive for most African populations. These challenges are being alleviated by numerous networks evolving through international collaborations that are positively changing the outlook of sickle cell disease management in sub-Saharan Africa. In this Series paper, we describe the epidemiology, pathophysiology, clinicobiological profile, and psychosocial effects of sickle cell disease in sub-Saharan Africa. We highlight transferable strategies already used for the successful management of the condition and key strategies and recommendations for affordable and comprehensive care on the continent. TRANSLATION: For the French translation of the abstract see Supplementary Materials section.


Subject(s)
Anemia, Sickle Cell/prevention & control , Africa South of the Sahara/epidemiology , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/physiopathology , Anemia, Sickle Cell/psychology , Humans , Quality of Life
12.
J Pediatr Hematol Oncol ; 43(7): e975-e978, 2021 10 01.
Article in English | MEDLINE | ID: covidwho-1434554

ABSTRACT

BACKGROUND: In March 2020, WHO announced Coronavirus Disease 2019 (COVID-19) outbreak a global pandemic. During this pandemic, patients with sickle cell disease (SCD) have been placed in the "high-risk" category of the population. Although there are numerous publications describing COVID-19 in adult patients, pediatric data are still limited. OBSERVATION: Herein, we report case series of 5 sickle cell disease Omani children who got infected with COVID-19; illustrating their different ways of presentation, management and highlighting the outcomes. CONCLUSION: Although SCD patients are considered as a high-risk group, all of the observed patients, and whose cases are reported here, have recovered. A large scale of SCD cases should be studied to reach more conclusive results.


Subject(s)
Anemia, Sickle Cell/virology , COVID-19/complications , SARS-CoV-2/isolation & purification , Anemia, Sickle Cell/diagnosis , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/therapy , COVID-19/transmission , COVID-19/virology , Child , Child, Preschool , Female , Humans , Male , Oman/epidemiology
13.
Acta Haematol ; 145(1): 1-4, 2022.
Article in English | MEDLINE | ID: covidwho-1430269

ABSTRACT

Sickle cell disease is characterized by vaso-occlusive phenomena and haemolytic anaemia. There is a significant concern that the overlap of COVID-19 lung disease with acute chest syndrome that occurs in sickle cell patients may result in serious complications. Case reports of sickle cell patients with COVID-19 have been published. Here, we present a case series of COVID-19 infection in sickle cell patients in a developing country (Brazil). Only 10 patients tested positive so far for SARS-CoV-2 of 600 patients followed at our institution, of which 8 needed hospitalization (one in the intensive care unit), with no deaths. Even in a middle-income country, COVID-19 was reported to be relatively mild in sickle cell patients. In relation to risk factors, blood type O seems to confer some protection against developing severe COVID-19, a finding that could guide clinicians to adopt more clinical surveillance for patients with non-O blood type in sickle cell patients.


Subject(s)
ABO Blood-Group System/blood , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/therapy , COVID-19/blood , COVID-19/therapy , SARS-CoV-2/metabolism , Adult , Anemia, Sickle Cell/epidemiology , Brazil , COVID-19/epidemiology , Child , Developing Countries , Female , Humans , Male , Middle Aged
17.
Blood Adv ; 5(13): 2717-2724, 2021 07 13.
Article in English | MEDLINE | ID: covidwho-1291227

ABSTRACT

Patients with sickle cell disease (SCD) are at high risk of developing serious infections, therefore, understanding the impact that severe acute respiratory syndrome coronavirus 2 infection has on this population is important. We sought to identify factors associated with hospitalization and serious COVID-19 illness in children and adults with SCD.We established the international SECURE-SCD Registry to collect data on patients with SCD and COVID-19 illness. We used multivariable logistic models to estimate the independent effects of age, sex, genotype, hydroxyurea, and SCD-related and -nonrelated comorbidities on hospitalization, serious COVID-19 illness, and pain as a presenting symptom during COVID-19 illness. As of 23 March 2021, 750 COVID-19 illness cases in patients with SCD were reported to the registry. We identified history of pain (relative risk [RR], 2.15; P < .0001) and SCD heart/lung comorbidities (RR, 1.61; P = .0001) as risk factors for hospitalization in children. History of pain (RR, 1.78; P = .002) was also a risk factor for hospitalization in adults. Children with history of pain (RR, 3.09; P = .009), SCD heart/lung comorbidities (RR, 1.76; P = .03), and SCD renal comorbidities (RR, 3.67; P < .0001) and adults with history of pain (RR 1.94, P = .02) were at higher risk of developing serious COVID-19 illness. History of pain and SCD renal comorbidities also increased risk of pain during COVID-19 in children; history of pain, SCD heart/lung comorbidities, and female sex increased risk of pain during COVID-19 in adults. Hydroxyurea showed no effect on hospitalization and COVID-19 severity, but it lowered the risk of presenting with pain in adults during COVID-19.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Adult , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Child , Female , Hospitalization , Humans , Risk Factors , SARS-CoV-2
18.
Int J Infect Dis ; 106: 128-133, 2021 May.
Article in English | MEDLINE | ID: covidwho-1279596

ABSTRACT

OBJECTIVES: The study aimed to assess COVID-19 impact on the morbidity and mortality of vasooclusive crisis (VOC) in sickle cell anaemia (SCA) patients. METHODS: A prospective cohort study of 100 SCA patients; 50 with COVID-19 (COVID group) and 50 without (non-COVID group). All patients signed written informed consent. RESULTS: The COVID group had a significantly higher VOC episode median per year; 3 (IQR,1-6) vs 2 (IQR,2-12) (P < 0.05). The need for hospitalisation was similar in both groups. The non-COVID group had more history of culture-proven infection (P = 0.05). The COVID-group had more osteonecrosis (P < 0.05), splenic sequestration, splenomegaly and hepatic crisis (P = 0.05, 0.006, 0.02; respectively) and significantly higher (P < 0.05) symptoms of fever, cough, fatigue, abdominal pain and anosmia. Mean haemoglobin, lymphocyte subset, platelets, and reticulocytes were reduced in both groups, while lactate dehydrogenase and ferritin levels were significantly elevated. In the COVID group, the rise in white blood cell count, reticulocyte percentage, platelets and ferritin was subdued (P < 0.05). Two patients in the COVID group and 3 in the non-COVID group died; there was no statistically significant difference in mortality. CONCLUSIONS: Although COVID-19 may have triggered the onset of VOC, it did not significantly influence VOC-related morbidity or mortality in this SCA cohort.


Subject(s)
Acute Chest Syndrome/blood , Acute Chest Syndrome/epidemiology , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/epidemiology , COVID-19/blood , COVID-19/epidemiology , SARS-CoV-2 , Acute Chest Syndrome/mortality , Adult , Anemia, Sickle Cell/mortality , COVID-19/mortality , Cohort Studies , Comorbidity , Female , Ferritins/blood , Hospitalization , Humans , L-Lactate Dehydrogenase/blood , Leukocyte Count , Lymphocyte Count , Male , Platelet Count , Prospective Studies , Reticulocytes
19.
Ann Hematol ; 100(9): 2195-2202, 2021 Sep.
Article in English | MEDLINE | ID: covidwho-1241601

ABSTRACT

It is highly expected that COVID-19 infection will have devastating consequences in sickle cell disease (SCD) patients due to endothelial activation and decreased tissue and organ reserve as a result of microvascular ischemia and continuous inflammation. In this study, we aimed to compare the clinical course of COVID-19 in adult SCD patients under the organ injury mitigation and clinical care improvement program (BASCARE) with healthcare professionals without significant comorbid conditions. The study was planned as a retrospective, multicenter and cross-sectional study. Thirty-nine SCD patients, ages 18 to 64 years, and 121 healthcare professionals, ages 21 to 53, were included in the study. The data were collected from the Electronic Health Recording System of PRANA, where SCD patients under the BASCARE program had been registered. The data of other patients were collected from the Electronic Hospital Data Recording System and patient files. In the SCD group, the crude incidence of COVID-19 was 9%, while in healthcare professionals at the same period was 23%. Among the symptoms, besides fever, loss of smell and taste were more prominent in the SCD group than in healthcare professionals. There was a significant difference between the two groups in terms of development of pneumonia, hospitalization, and need for intubation (43 vs 5%, P < 0.00001; 26 vs 7%, P = 0.002; and 10 vs 1%, P = 0.002, respectively). Prophylactic low molecular weight heparin and salicylate were used more in the SCD group than in healthcare professionals group (41 vs 9% and 28 vs 1%; P < 0.0001 for both). The 3-month mortality rate was demonstrated as 5% in the SCD group, while 0 in the healthcare professionals group. One patient in the SCD group became continously dependent on respiratory support. The cause of death was acute chest syndrome in the first case, hepatic necrosis and multi-organ failure in the second case. In conclusion, these observations supported the expectation that the course of COVID-19 in SCD patients will get worse. The BASCARE program applied in SCD patients could not change the poor outcome.


Subject(s)
Anemia, Sickle Cell/complications , COVID-19/complications , Adolescent , Adult , Anemia, Sickle Cell/epidemiology , COVID-19/epidemiology , Cross-Sectional Studies , Disease Progression , Female , Health Personnel , Humans , Incidence , Male , Middle Aged , Retrospective Studies , SARS-CoV-2/isolation & purification , Young Adult
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