scholarly journals Presentation, Management and Outcomes of COVID-19 Patients with Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 29-30
Author(s):  
Nwabundo Anusim ◽  
Ruby Gupta ◽  
Hycienth O Ahaneku ◽  
Candace Franklin ◽  
Savitha Balaraman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Respiratory Symptoms ◽  
Bone Pain ◽  
Globin Gene ◽  
Morbidity And Mortality ◽  
Polymerase Chain Reaction Test ◽  
Acute Chest Syndrome ◽  
Shortness Of Breath ◽  
Cell Disease

Background Sickle cell disease (SCD) is an inherited disorder of red blood cell (RBC) caused by a mutation in the beta-globin gene resulting in abnormal hemoglobin known as hemoglobin S (HbS) or the sickle hemoglobin. Several clinical variants of SCD have been elucidated, all driven by two fundamental pathophysiologic processes: RBC hemolysis and intermittent vaso-occlusive vasculopathy resulting in tissue ischemia/infarction. These two processes underscore the many complications and eventual multi-organ damage that may develop in patients with the most severe types of SCD. Cardiopulmonary complications including heart failure, pulmonary hypertension and acute chest syndrome (ACS) are major drivers of morbidity and mortality among patients with SCD. With regards to ACS, patients often present with fever, cough and shortness of breath caused by vaso-occlusive crisis affecting the lungs. This is particular concerning in view of its similar features to symptomatic COVID-19 infection. Methods We retrospectively identified SCD patients with COVID-19 infection admitted to Beaumont hospitals in Michigan between March 1st 2020 and July 1st 2020. Data was abstracted using the ICD 10 code of U07. 1 for COVID-19, ICD 9 and 10 codes of 282.60 and D57 for sickle cell disease. We excluded patients with sickle cell trait. Data regarding the demographics, presentation, management and outcomes were abstracted. Results A total of eleven patients with sickle cell disease were identified as having a positive SARS-Cov19 polymerase chain reaction test (Table I). All were African American and predominantly female (64%) with a mean age of 44 (22-60) years and mean BMI of 30.2 kg/m2. Genotypes identified were HbSS in 5 (45%) patients, HbSC in 4 (36%), HbS/beta-thalassemia in 1 (9%) and HbS/alpha-thalassemia in 1 (9%). All of the patients had seen a haematologist since their diagnosis but none of the patients were on hydroxyurea, voxeloter, L-glutamine or crizanlizumab at admission. The predominant clinical presentation was fever, chest pain, chills, exertional shortness of breath and cough but this was not consistent across all patients. All the patients were managed with intravenous hydration, pain management as well as hydroxychloroquine/azithromycin per institutional guideline at that time. Three patients (cases 1-3) had recurrent visits to the hospital for similar symptoms and new bone pain crises. Case 1 had a pulmonary embolus which was evident on re-admission. Two patients (cases 3 and 10) succumbed to COVID-19. Two patients (cases 5 and 7) presented with bone pain crisis and no respiratory symptoms, but chest imaging was suggestive of COVID-19 infection necessitating treatment with antibiotics, possibly indicating that the virus can trigger vaso-occlusive crises without respiratory symptoms. Case 8 had a high Charlson comorbidity index and age over 60, had the lengthiest hospital stay complicated by renal failure and polyneuropathy, and was discharged to a long-term acute care facility: an outcome which is consistent with current data showing that the elderly and unfit patients are more likely to have a higher morbidity and mortality with COVID-19. Conclusion To date, there no compelling evidence to provide guidelines for the management of SCD patients with COVID-19. However, following existing recommendations in managing acute chest syndrome and those for COVID-19 symptomatic infection, is a good place to start. We continue to seek to improve management of these patients as new evidence of successful treatment emerges, and also encourage patients to participate in clinical trials. Disclosures No relevant conflicts of interest to declare.

Right-To-Left Shunts (Extra-Cardiac Arterial-Venous Malformations) Are Highly Common In Adults With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1004-1004
Author(s):  
Nathan Langer ◽  
MaryAnn O'Riordan ◽  
Santosh K. Rao ◽  
Jane A. Little ◽  
Robert Schilz
Keyword(s):  
Sickle Cell Disease ◽  
General Population ◽  
Oxygen Saturation ◽  
Sickle Cell ◽  
Morbidity And Mortality ◽  
Acute Chest Syndrome ◽  
Venous Malformations ◽  
Cell Disease ◽  
Group I ◽  
Group Ii

Abstract Introduction Cardiopulmonary complications are a major cause of morbidity and mortality in sickle cell disease (SCD) as shown by worse prognosis in patients who have experienced acute chest syndrome or who have an elevated tricuspid regurgitant jet velocity (TRV) on echocardiogram at clinical baseline. Here we describe an unexpected and novel cardio-pulmonary complication in HbSS, right-to-left shunting through extra-cardiac arterial-venous malformations (AVMs), which may contribute to pathophysiology. Extracardiac AVMs are rare in the general population, with an estimated incidence of 1/5000. Of 2111 shunt evaluation echocardiograms performed at our institution over 12 months only 81 (3.8%) of individual studies were positive. Methods We evaluated 36 HbSS patients who presented with subjective dyspnea or hypoxia with clinical exam and with echocardiogram utilizing agitated saline to assess for vascular right-to-left shunts. We compared this group with the remaining 81 HbSS patients in our database. 19 of 36 symptomatic patients were found to have an extracardiac right-to-left shunt. We then compared these 19 patients with the 17 symptomatic HbSS patients who did not have a shunt. 10 HbSC and 5 S-beta-thalassemia patients were also studied and did not have a right-to-left shunt; only HbSS are included in comparative analyses. Results Patients with symptoms did not differ in age (32.7±10.3 years vs 31.7±11.7 years) from patients who did not present with hypoxia or subjective dyspnea (n=81). Symptomatic patients were more likely to have macroalbuminuria (>300 mg/g albumin-to-creatinine, 9/36 vs 8/63 evaluable, p=0.05), more likely to have a TRV ≥3 meters/second (9/36 vs 11/74 evaluable, p=0.09), and were more hypoxic at rest (96 ±5% vs 98±2% oxygen saturation, p=0.07). We found delayed left-sided bubble visualization in 19/36 symptomatic HbSS subjects (53%) consistent with extra-cardiac AVMs. HbSS subjects with (n=19, Group I) or without (n=17, Group II) a positive bubble study were clinically and demographically similar (age, gender, WBC, total Hgb, HgbF%, LDH, eGFR, proportion with macroalbuminuria, baseline oxygen saturation, and elevated TRV). However, group I patients had a higher reticulocyte count (15.4±5.5% vs 9.8±6.7% p<0.005). Of Group I patients, 42.1% had history of acute chest while 70.6% of Group II had such history (p=0.09). Group I patients were less likely to be on hydroxyurea (52.78% vs 47.22% p<0.05). Conclusion Extra-cardiac AVMs are observed in 16% of all subjects with HbSS, compared with 3.8% of general medical patients at a tertiary center undergoing shunt evaluation and .02% in the general population. In HbSS, symptomatic subjects are more likely to have evidence for vasculopathy (macroalbuminuria, elevated TRV) and hypoxia; one-half of these symptomatic patients have extracardiac AVMs. We speculate that this finding is unlikely to be clinically silent, and a bubble-echocardiogram may be an important additional clinical evaluation for symptomatic dyspnea or hypoxia. The impact of this novel clinical finding on morbidity and mortality in this disease remains under investigation. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

2012 ◽  
Vol 2012 ◽  
pp. 1-55 ◽  
Author(s):  
Samir K. Ballas ◽  
Muge R. Kesen ◽  
Morton F. Goldberg ◽  
Gerard A. Lutty ◽  
Carlton Dampier ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Point Mutation ◽  
Sickle Cell ◽  
Globin Gene ◽  
Single Point ◽  
Acute Chest Syndrome ◽  
Single Point Mutation ◽  
Cell Disease ◽  
Exon 1 ◽  
Abnormal Hemoglobin

The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of theβglobin gene resulting in the substitution of glutamic acid by valine at position 6 of theβglobin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

Are we missing the mark? Fever, respiratory symptoms, chest radiographs, and acute chest syndrome in sickle cell disease

2016 ◽  
Vol 91 (8) ◽  
pp. E332-E333 ◽  
Author(s):  
Sarah G. Lazarus ◽  
Michael Kelleman ◽  
Olufolake Adisa ◽  
April R. Zmitrovich ◽  
Robert Hagbom ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Respiratory Symptoms ◽  
Chest Radiographs ◽  
Acute Chest Syndrome ◽  
Cell Disease

scholarly journals Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease

Blood ◽  
2012 ◽  
Vol 120 (18) ◽  
pp. 3822-3828 ◽  
Author(s):  
Christopher J. Bean ◽  
Sheree L. Boulet ◽  
Dorothy Ellingsen ◽  
Meredith E. Pyle ◽  
Emily A. Barron-Casella ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Heme Oxygenase ◽  
Sickle Cell ◽  
Globin Gene ◽  
Fetal Hemoglobin ◽  
Promoter Polymorphism ◽  
Adverse Outcomes ◽  
Acute Chest Syndrome ◽  
Heme Oxygenase 1 ◽  
Cell Disease

Abstract Sickle cell disease is a common hemolytic disorder with a broad range of complications, including vaso-occlusive episodes, acute chest syndrome (ACS), pain, and stroke. Heme oxygenase-1 (gene HMOX1; protein HO-1) is the inducible, rate-limiting enzyme in the catabolism of heme and might attenuate the severity of outcomes from vaso-occlusive and hemolytic crises. A (GT)n dinucleotide repeat located in the promoter region of the HMOX1 gene is highly polymorphic, with long repeat lengths linked to decreased activity and inducibility. We examined this polymorphism to test the hypothesis that short alleles are associated with a decreased risk of adverse outcomes (hospitalization for pain or ACS) among a cohort of 942 children with sickle cell disease. Allele lengths varied from 13 to 45 repeats and showed a trimodal distribution. Compared with children with longer allele lengths, children with 2 shorter alleles (4%; ≤ 25 repeats) had lower rates of hospitalization for ACS (incidence rate ratio 0.28, 95% confidence interval, 0.10-0.81), after adjusting for sex, age, asthma, percentage of fetal hemoglobin, and α-globin gene deletion. No relationship was identified between allele lengths and pain rate. We provide evidence that genetic variation in HMOX1 is associated with decreased rates of hospitalization for ACS, but not pain. This study is registered at www.clinicaltrials.gov as #NCT00072761.

Prevalence of Respiratory Symptoms in Children with Sickle Cell Disease Compared to Children with Other Hematological Diseases.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3754-3754
Author(s):  
Brigitta Ursula Mueller ◽  
Monica Acosta ◽  
Marlen Dinu ◽  
Marianna Sockrider ◽  
Felix Shardonofski ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Respiratory Symptoms ◽  
Airway Disease ◽  
The Other ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Respiratory Problems ◽  
Hematological Disorders ◽  
Hematological Diseases

Abstract Acute chest syndrome (ACS) and pulmonary hypertension are complications of sickle cell disease (SCD). The effects of pre-existing respiratory symptoms on the frequency of complications of SCD, such as vaso-occlusive crises and ACS, have not been fully explored. Many therapeutic options exist for patients with reactive airway disease or other recurrent respiratory problems. Control of associated symptoms may help prevent the occurrence of ACS and other sickle cell-related problems. We compared the prevalence of respiratory symptoms in SCD patients compared to patients with other hematological disorders. Methods: Two questionnaires (one for patients with SCD, one for patients with other hematological disorders) were developed, which included questions regarding respiratory symptoms (especially wheezing and cough, frequency of these symptoms, previous diagnosis of asthma), personal history and environmental exposure. The study was approved by the Institutional Review Board, and patients and their caregivers were interviewed during a routine visit to the outpatient hematology clinic after giving informed consent. Results: To date, 124 subjects have been interviewed (62 males and 58 females, ages 3–18 years, mean age 10.1yrs), and 120 questionnaires were evaluable, including 59 from patients with SCD and 61 from the comparison group. Respiratory symptoms (wheezing and cough) were more frequent in SCD patients compared to patients with other hematological diseases. Wheezing was present in 34 (58%) SCD patients and in 20 (33%) patients in the other group (p<0.01). Twelve patients (20%) in the SCD group missed school due to respiratory problems (over half of them missed more than 7 days of school) compared to 4 (6%) patients (only 1 missing school for more than 7 days) in the other group (p<0.04). Hospitalization due to respiratory problems before the age of 2 years was more common in SCD patients: 23 (39%) compared to 8 (13%) in other hematological patients (p<0.003). Conclusions: The prevalence of pulmonary symptoms is higher in patients with SCD compared to patients with other hematological disorders and associated with frequent hospitalizations and school absences.

scholarly journals Sickle Cell Disease and COVID-19 Infection: A Single-Center Experience

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 985-985
Author(s):  
Sindhu Devarashetty ◽  
Kimberly Le Blanc ◽  
Udhayvir Singh Grewal ◽  
Jacqueline Walton ◽  
Tabitha Jones ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Sickle Cell ◽  
Adult Patients ◽  
Living Alone ◽  
Morbidity And Mortality ◽  
Acute Chest Syndrome ◽  
Single Center ◽  
Cell Disease

Abstract Background and Objectives: The COVID-19 (CO19) pandemic caused by SARS-CoV-2 remains a significant issue for global health, economics, and society. Several reports have shown that African Americans (AA) have been disproportionately affected by the CO19 pandemic. Limited data have suggested that sickle cell disease (SCD) could be one of the several reasons for higher morbidity and mortality related to CO19 among AA. Recent reports have suggested higher-than-average morbidity and mortality related to CO19 among patients with SCD. We conducted a retrospective, single-institution study in adult patients with SCD who were diagnosed with CO19 infection and their outcomes. Methods: After IRB approval, we conducted a chart review of adult patients (greater than 18 years) with SCD who were diagnosed with CO19 infection between March 1st, 2020, and March 31st, 2021. We recorded demographic data including age, gender, social factors (the type of insurance, availability of primary care provider (PCP), living alone/not), clinical parameters (type of SCD, co-morbidities), outpatient management of SCD, and how CO19 infection was managed like inpatient admission and complications. In patients who were admitted or seen in the emergency department (ED), we collected additional data including vitals, labs, the severity of illness, complications, length of stay, and outcomes. Computations were performed using statistical software SAS 9.4 for Windows. Results: We found a total of 51 patients with SCD diagnosed with CO19 infection in the above period. The median age of patients was 30 years. 61% were females and 39 % were males. All of them were AA. 11.76% were living alone, 49.02% were living with family, 1.96% (1 patient) was institutionalized, and the living situation was unknown in 37.25%. Most of the patients had Medicaid Insurance (52.94%), Medicare in 33.3%, private insurance in 13.73 % and 2% were uninsured. Only 64.71% of patients had a PCP. 60% had HbSS disease, 32% had HbSC disease, 4% had HbS-beta thalassemia, one patient each had HbSS with hereditary persistence of HbF and HbS/HbD. Comorbidities and previous history included acute chest syndrome in 65.96%, avascular necrosis in 36.96%, leg ulcers in 8.7%, hypertension in 8.7%, sickle cell retinopathy in 14.57%, cerebrovascular disease in 26.19%, chronic kidney disease in 7.69%, venous thromboembolism (VTE) in 20.41%, 10.41% were on anticoagulation, history of HIV and hepatitis C infection in 6.38%. 28.21% of patients were maintained on partial exchange transfusions as an outpatient for various indications. 72.73% were on hydroxyurea, 7.5% were on crizanlizumab, 5.26% were on voxelotor and 26.83% were on iron chelation. Vitals and pertinent lab values on initial assessment were recorded and many patients had missing data. On presentation, 25.53% were febrile, 29.17% of patients were tachycardic, 31.25% were hypoxic (SpO2 &lt; 95%), 38.46% were tachypneic, 59.18% had a body mass index (BMI) of &gt; 24.9. Median hemoglobin and hematocrit were 8.9/27.4 g/dL. The median white blood cell count was 9490/uL and platelets were 315,000/uL. Median ferritin was 1573 ug/L. Median bilirubin and creatinine were 2.05 mg/dL and 0.86 mg/dL. The patients were further stratified based on the clinical location where CO19 infection was managed (Table 1). 39.3% were diagnosed in the outpatient setting/ED and 60.3% in the inpatient setting. Among 51 patients, 5.71% (n=2) required ICU admission and was mechanically ventilated. 17.5% received dexamethasone, 7.69% received remdesivir, 2.76% received convalescent plasma, 17.07% had infections and 47% received antibiotics. Only one patient received an exchange transfusion during admission. One patient developed a new VTE after CO19 infection. On statistical analysis, the only factor which impacted the clinical location of management was tachycardia (P=0.007). Of the 51 patients, only 3.9% (2 patients) died of complications of CO19 infection, one with hypoxic respiratory failure, disseminated intravascular coagulation, shock, and the other one with pulmonary embolism. 13% were readmitted within a month, one of them was admitted with a new pulmonary embolism and the others were admitted for acute painful episodes. Conclusion: We found a mortality rate of 3.9% in our single-center study of patients with SCD and CO19 infection. This mortality rate is lower than other published experiences in patients with SCD and CO19 infection. Figure 1 Figure 1. Disclosures Master: Blue Bird Bio: Current holder of individual stocks in a privately-held company.

Fetal Hemoglobin In Sickle Cell Anemia: A Glass Half Full?

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4691-4691
Author(s):  
Martin H. Steinberg ◽  
David H.K. Chui ◽  
George J. Dover ◽  
Paola Sebastiani ◽  
Abdulrahman Alsultan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Conflicts Of Interest ◽  
Globin Gene ◽  
Single Agent ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Sickle Erythrocytes ◽  
F Cells

HbF modulates the phenotype of sickle cell anemia by inhibiting deoxyHbS polymerization. HbF is confined to erythrocytes called F-cells that can be detected by FACS when these cells contain sufficient HbF. Measuring the amount of HbF/F-cell is difficult and not clinically available. African-Americans with sickle cell anemia have 2-80% F-cells with an average HbF/F-cell of 6.4±1.6 pg. The distribution of HbF/F-cell is highly individual regardless of HbF level. People with HbS-gene deletion hereditary persistence of HbF (HPFH) have a mean HbF of 30%, and HbF is evenly distributed among their erythrocytes. Polymer is not present in these cells either experimentally or after calculating the HbS polymer fraction at 70% O2 saturation. Therefore, each cell contains about 10 pg. of HbF. DeoxyHbS polymerization is prevented at physiologic venous and capillary O2 saturations of 40-70% when HbF/F-cell is 9-12 pgs. We call this the “protective” level of HbF. F-cells need not contain “protective” levels of HbF. Some β-globin gene cluster haplotypes are associated with high HbF. Carriers of these haplotypes can have milder disease. Nevertheless, even patients with high HbF can have frequent painful episodes, acute chest syndrome and osteonecrosis. Patients with HbS-δβ thalassemia have 15 to 25% HbF but are anemic and have vasoocclusive complications, albeit less often than in sickle cell anemia. Hydroxyurea reduces the morbidity and mortality of sickle cell anemia, an effect likely to be mediated by its induction of HbF. Patients treated with hydroxyurea are better and probably live longer, but adults are anemic and rarely asymptomatic. In all these patient groups, HbF is unevenly distributed among erythrocytes. In contrast, people with HbS-HPFH are nearly asymptomatic and not anemic. The failure of HbF to modulate uniformly all complications of sickle cell disease might be related to the heterogeneous concentration of HbF in sickle erythrocytes. HbF is associated with protection from the development of certain disease subphenotypes but has limited prognostic value in individuals. In many cross-sectional studies, high HbF was associated with a reduced rate of acute painful episodes, fewer leg ulcers, less osteonecrosis, less frequent acute chest syndromes and reduced disease severity. HbF had a weak or no clear association with priapism, urine albumin excretion, stroke and silent cerebral infarction, systemic blood pressure and tricuspid regurgitant velocity. Perhaps this is because intravascular hemolysis of cells with little or no HbF causes nitric oxide scavenging, or because these complications are less dependent on HbS polymerization. No study provides information on the concentration of HbF/F-cell other than providing the relatively meaningless calculated mean value. Rather than the total number of F-cells or the concentration of HbF in the hemolysate, HbF/F-cell and the proportion of F-cells that have “protective” HbF is the most critical predictor of the likelihood of some disease subphenotypes. Hypothetical distributions of HbF-cells with different levels of HbF/F-cell can be plotted for different concentrations of HbF. With mean HbF levels of 5%, 10% and 20%, and HbF content per cell of 1.5, 3 and 6 pg., assuming a fixed mean, the variance was changed to show how the distribution of HbF per cell can greatly vary, even if the mean is constant. For example, with 20% HbF, as few as 1% and as many as 24% of cells have “protective” HbF. When HbF is lower, few or no “protected” cells can be present. Due to the heterogeneous concentrations of HbF, HbS can polymerize in some F-cells that have sub-polymer inhibiting concentrations of HbF. Inducing high levels of HbF is one approach to treating sickle cell disease. Inactivating BCL11A, a repressor of γ-globin gene expression, abrogates sickle cell disease in transgenic sickle mice. Their HbF was distributed homogeneously, and their phenotype mimicked HbS-HPFH. If it becomes possible in humans to target BCL11A or its pathway with agents that affect gene transcription, will it result in pancellular HbF? Broadening the distribution of HbF amongst sickle erythrocytes with drugs like hydroxyurea that effect the kinetics of erythropoiesis, coupled with an agent whose primary mechanism of action is to increase the transcription of the γ-globin genes, might be the most fruitful approach to HbF induction therapy and more efficacious than single agent treatment. Disclosures: No relevant conflicts of interest to declare.

Sickle Cell Disease

2015 ◽  
Author(s):  
Caroline Freiermuth ◽  
Idan Cudykier
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Genetic Basis ◽  
Globin Gene ◽  
The United States ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
The Past ◽  
Pain Medications

Sickle cell disease affects between 70,000 and 90,000 individuals in the United States, the majority of whom are of African-American descent. The genetic basis of the disease is an abnormality in the β-globin gene, which causes the red blood cells to change to a “sickle” shape due to low oxygenation. The life span of patients with this disease has improved over the past few decades, although morbidity remains high. This review covers the pathophysiology of sickle cell disease and the stabilization and assessment, diagnosis and treatment, maintenance and preventive therapies, and cure of patients with sickle cell disease. Figures show hemoglobin electrophoresis; age at death for individuals with sickle cell disease in the years 1979, 1989, 1999, and 2006; sickled cells blocking blood flow; acute chest syndrome; dactylitis; and avascular necrosis. Tables list important trials, topics in need of further research, common complications, most common intravenous pain medications, and indications for transfusion. This review contains 6 highly rendered figures, 5 tables, 97 references, and a list of educational resources.

scholarly journals Vaso-Occlusive Events Precipitated By Intraarticular Steroid Injections in Patients with Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 2-3
Author(s):  
Landan Banks ◽  
Michel Gowhari ◽  
Robert Molokie
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Joint Pain ◽  
Clinical Decision Making ◽  
Steroid Injection ◽  
Globin Gene ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Steroid Injections ◽  
Corticosteroid Injections

Introduction: Sickle cell disease (SCD) is a group of inherited hemoglobin diseases (Hb), caused by a point mutation in the b-globin gene, that affects more than 100,00 people in the US and millions worldwide. No organ system is left unaffected by SCD, including the musculoskeletal system; complications which can greatly decrease quality of life and contribute to significant burden for these patients. Orthopedic complications include bone pain, dactylitis, growth retardation and atypical skeletal development, osteomyelitis and septic arthritis, bone demineralization, motor/mobility impairment, osteonecrosis and other causes of arthritis and rheumatologic disease. While surgical repair by arthroplasty is often a definitive solution to joint pain, conservative, non-operative treatments are first-line, including physical therapy and intraarticular corticosteroid injections. Corticosteroid injections can be used as a convenient outpatient solution to reduce inflammation and relieve pain without undergoing surgical measures. The use of steroids for non-orthopedic complications of SCD is not benign for many patients, and may be associated with the development of rebound vaso-occlusive pain events, requiring hospitalization, as well as hemorrhagic stroke. A review of the literature revealed two cases of patients with SCD who received intraarticular steroid injections for rheumatoid arthritis in whom the injections precipitated an acute vaso-occlusive episode that required admission. In view of these findings, we are interested in determining if any adults in our adult SCD clinic who had an intraarticular injection of a steroid developed worsening of pain. Methods: After approval by the UIC IRB, a retrospective chart review was conducted for subjects enrolled in the UIC Adult Sickle Cell Center treated with an intraarticular steroid injection. Data collected included: type of medication used, injection location, indication, pain complication and timeline of symptoms, admission, age, sex, genotype and BMI. Vaso-occlusive events described were unusually severe compared to the pre-corticosteroid clinical course and not reported with other triggers for SCD vaso-occlusive events. Results: Four patients were identified. Characteristics of these patients and injections are listed in table 1. Patients received one or more injections of knees, hips, wrists and fingers before recognizing the associated pain with the injection. Indications for injections included primary osteoarthritis, osteoarthritis secondary to avascular necrosis, tendonitis and tenosynovitis. All injections were a combination of local anesthetic and glucocorticoid. The majority of injections were 2 cc lidocaine 1%, 1 cc triamcinolone acetonide-40mg. Other local anesthetics used were xylocaine and bupivacaine. Discussion: With improvements in medical care, more people with SCD are surviving into adulthood and developing orthopedic complications of SCD, as well as aging, such as osteoarthritis. While the development of acute pain after treatment of the acute chest syndrome with steroids is known, there were only 2 reported cases of SCD patients who developed severe pain associated with intraarticular injections. We report here four additional cases of adults with SCD who received an injection and developed worsening of their pain, often requiring admission. While these numbers are small, we believe that this may be a much more common complication of steroid injections and providers should discuss this with their patients. The observations that are made in this study raise into question the idea that while temporary pain relief is the intended outcome, this is not the result in all patients- the fact of which should be a consideration made by all clinicians when offering steroid injections as opposed to non-steroid joint injections to patients with SCD. The pathophysiology behind this effect of steroid injections is unclear. In the event that a patient with SCD presents with pain symptoms, either worsening pain or pain uncharacteristic of their usual symptoms, a consideration for potential causes should be recent steroid injection for joint pain. These considerations may help guide clinical decision making. Disclosures No relevant conflicts of interest to declare.

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