scholarly journals Optimizing management of sickle cell disease in patients undergoing surgery

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 405-410
Author(s):  
Charity I. Oyedeji ◽  
Ian J. Welsby
Keyword(s):  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Surgical Complications ◽  
Acute Chest Syndrome ◽  
Surgical Team ◽  
Cell Disease ◽  
History Of ◽  
Baseline Hemoglobin

Abstract Individuals with sickle cell disease (SCD) are likely to be referred for surgery at some point in their lifetime due to a high incidence of musculoskeletal and intrabdominal complications such as avascular necrosis and gallbladder disease. Preoperative optimization is a multidisciplinary process that involves a hematologist with SCD expertise, an anesthesiologist, and the surgical team. The type and risk classification of the surgery, disease severity, medications, baseline hemoglobin, transfusion history, and history of prior surgical complications are often documented. Clinicians should consider perioperative risk assessment that includes determining the patient's functional status and cardiovascular risk and screening for obstructive sleep apnea. Many patients will require preoperative transfusion to reduce the risk of postoperative complications such as acute chest syndrome and vaso-occlusive pain crises. The hematologist should consider the patient's preoperative transfusion requirements and ensure that the surgical team has an appropriate plan for postoperative observation and management. This often includes follow-up laboratory studies, a postoperative pain management plan, and venous thromboembolism prophylaxis. The transfusion plan should be patient-specific and take into account the SCD genotype, baseline hemoglobin, disease severity, risk classification of the surgery, and history of prior surgical complications. In the intraoperative and postoperative period, dehydration, hypothermia, hypotension, hypoxia, and acidosis should be avoided, and incentive spirometry should be utilized to minimize complications such as acute chest syndrome. In this review we discuss preoperative, intraoperative, and postoperative strategies to optimize patients with SCD undergoing surgery.

scholarly journals To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

2021 ◽  
pp. 1-5
Author(s):  
Justin E. Juskewitch ◽  
Craig D. Tauscher ◽  
Sheila K. Moldenhauer ◽  
Jennifer E. Schieber ◽  
Eapen K. Jacob ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Termination ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Childbearing Age ◽  
Procedural Complications ◽  
History Of ◽  
Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

Prevalence of Pulmonary Hypertension (PHTN) in Sickle Cell Disease (SCD) Adolescents with Pulmonary Complications.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3759-3759
Author(s):  
Onyinye C. Onyekwere ◽  
Andrew Campbell ◽  
James Williams ◽  
Peter Gaskin ◽  
Sohail Rana ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Sickle Cell ◽  
Pulmonary Complications ◽  
Acute Chest Syndrome ◽  
University Of Michigan ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Howard University ◽  
History Of

Abstract Despite the high prevalence of PHTN in adults with SCD, the prevalence in the pediatric population with SCD is not known. We hypothesized that elevated pulmonary artery pressures may be found in SCD adolescents with history of pulmonary complications, such as acute chest syndrome (ACS), obstructive sleep apnea (OSA), asthma, and reactive airway disease. Thirty such sickle cell disease adolescents were screened at Howard University or University of Michigan for PHTN with Doppler echocardiography. We defined PHTN as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/sec (corresponding to a pulmonary artery systolic pressure greater than 35 mm Hg). PHTN was found in 16 SCD patients (53.3%) and 5 (16.7%) had TRV > 3.0 m/sec. Clinical findings according to the presences or absence of PHTN are shown in the table. Potential factors contributing to PHTN in patients with SCD include chronic hemolysis and chronic hypoxia. Our results suggest that PHTN is common among SCD adolescents with a history of pulmonary complications. Consideration should be given to screening such patients for PHTN and exploring treatment options. Further studies are urgently needed to clarify the prevalence and mechanisms of PHTN in adolescents with SCD. Clinical and demographic data of 30 SCD adolescents with pulmonary findings who underwent echocardiography at Howard University Hospital or University of Michigan PHTN (N = 16) No PHTN (N = 14) P Age in years (mean +/− SD) 15.9 +/− 3.2 17.4 +/− 2.3 0.17 Females (no. and %) 5 (31.3) 7 (50) 0.5 Hemoglobin SS Phenotype (no and %) 14 (87.5 11 (78.6) 0.5 Hemoglobin concentration (mean +/− SD) 8.0 +/− 2.1 9.3 +/−1.9 0.11 White blood cells (mean +/− SD) 10.9 +/− 2.9 9.7 +/− 3.7 0.4 Platelet (mean +/− SD) 475 +/− 172 364 +/− 240 0.17 Hemoglobin F percent (mean +/− SD) 5.1 +/− 3.5 6.4 +/− 5.5 0.6 Lactate dehydrogenase (mean +/− SD) 505 +/− 162 264 +/− 50 0.002 Total bilirubin (mean +/− SD) 4.1 +/− 2.6 3.4 +/− 2.6 0.5 Creatinine concentration (mean +/− SD) 0.6 +/− 0.2 0.7 +/− 0.2 0.18 Aspartate transaminase (mean +/− SD) 48 +/− 27 36 +/− 16 0.18 Alanine transaminase (mean +/− SD) 51 +/− 37 39 +/− 20 0.3

Severity of Sickle Cell Disease: Modeling Interrelationships among Hemolysis, Pulmonary Hypertension and Risk of Death.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 786-786
Author(s):  
Paola Sebastiani ◽  
Vikki G. Nolan ◽  
Clinton T. Baldwin ◽  
Maria M. Abad-Grau ◽  
Ling Wang ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Clinical Severity ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Risk Of Death

Abstract A single point mutation in the β hemoglobin gene causes sickle cell disease (SCD), but patients have extremely variable phenotypes. Hemolysis-related complications include pulmonary hypertension (PHT), priapism, stroke and leg ulceration; blood viscosity and sickle vasoocclusion are associated with painful episodes, acute chest syndrome and osteonecrosis. Predicting who is at highest risk of death would be useful therapeutically and prognostically. Applying Bayesian network modeling that describes complex interactions among many variables by factorizing their joint probability distribution into modules, to data from 3380 SCD patients, we constructed a disease severity score (DSS: 0, least severe; 1, most severe), defining severity as risk of death within 5 years. A network of 24 variables described complex associations among clinical and laboratory complications of SCD. The analysis was validated in 140 patients whose SCD severity was assessed by expert clinicians and 210 adults where severity was also assessed by the echocardiographic diagnosis of PHT and death. Information about PHT allowed a comparison of the DSS with the tricuspid regurgitant jet velocity (TRJV), an objective marker of PHT and an independent risk factor for death. DSS and three indices of clinical severity (severity ranking of individuals by expert clinicians; objective measurement of the presence and severity of PHT; risk of prospective death) were correlated. Among living subjects, the median score was 0.57 in 135 patients without PHT, 0.64 in 40 patients with mild PHT and 0.86 in 15 patients with severe PHT. The difference in average score between living patients with and without PHT is significant. The same increasing trend was noticeable in the subjects who died during follow-up: 0.60 in subjects without PHT; 0.68 in subjects with mild PHT; 0.79 in subjects with severe PHT. The utility of the DSS is also supported by the ability to assign a score to subjects for whom the TRJV cannot be measured. Surprisingly, besides known risk factors like renal insufficiency and leukocytosis, we identified the intensity of hemolytic anemia and clinical events associated with hemolytic anemia as contributing to risk for death. Priapism, an excellent reflection of the hemolytic anemia-related complications of SCD, is associated with PHT and its association with death was unexpected. Laboratory variables predictive of disease severity included LDH and reticulocytes that reflect the intensity of hemolytic anemia. Elevated systolic blood pressure increased the odds of death by 3.4, consistent with hypertension as a marker of early death in SCD. Subjects with sickle cell anemia are at greatest risk compared with subjects with sickle cell anemia-α thalassemia and with subjects with HbSC disease. Our model suggests that the intensity of hemolytic anemia, estimated by LDH, reticulocyte count and AST, and shown previously to be associated with PHT, priapism, leg ulceration and possibly stroke, is an important contributor to death. This model can be used to compute a personalized measure of disease severity that might be useful for guiding therapeutic decisions and designing clinical trials.

Corrected QT Interval Is Related to Markers of Hemolysis and Tricuspid Regurgitant Jet Velocity in a Young Cohort with Sickle Cell Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2258-2258
Author(s):  
Robert I. Liem ◽  
Luciana T. Young ◽  
Alexis A. Thompson
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Qt Interval ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Qtc Prolongation ◽  
Corrected Qt Interval ◽  
Conduction Abnormalities ◽  
History Of ◽  
Tricuspid Regurgitant Jet Velocity

Abstract Recent evidence suggests that prolongation in QT interval may be a frequent finding in patients with sickle cell disease (SCD). Few studies, however, have examined the relationship between conduction abnormalities and other cardiac complications, such as left ventricular hypertrophy (LVH) and tricuspid regurgitant jet velocity (TRJV) elevation, in this population. Moreover, long QT may be a marker of increased mortality in conditions, other than SCD, associated with LVH. We therefore sought to evaluate QT interval and its relationship to echocardiographic findings, laboratory parameters and disease severity in a cohort of children and young adults with SCD. Methods We prospectively evaluated the corrected QT interval (QTc) on standard 12-lead ECG in a cross-sectional, convenience sample of 73 subjects (41 males, mean age 14.2±3 years, range 10 to 24) with Hb SS, SC and S-β0 thalassemia undergoing screening for TRJV elevation. Subjects on chronic transfusions were excluded and all studies were performed at baseline on the same day. A review of available medical records was also performed. Results In our cohort, QTc (mean 436±24 ms, range 387 to 531) was prolonged > 440 ms in 30/73 (41%) of subjects at steady state. We also found TRJV elevation ≥ 2.5 m/s in 24/73 (33%) and LVH by ECG or echocardiographic criteria in 32/73 (44%) subjects. Using Pearson’s correlation coefficient, we observed significant correlations between QTc and TRJV (r=0.38, p=0.002), WBC (r=0.37, p=0.001) and several markers of hemolysis, including LDH (r=0.46, p=0001), Hb (r=-0.32, p=0.005), retic (r=0.29, p=0.013), plasma Hb (r=0.27, p=0.03) and AST (r=0.38, p=0.001). Using Student’s t-test for independent samples, only TRJV (2.55±0.33 vs. 2.34±0.26 m/s, p=0.006), LDH (450±166 vs. 329±143 U/L, p=0.001), WBC (10.6±4.7 vs. 8.6±3.3×109/L, p=0.048), retic (14.4±9.2 vs. 10.6±6.1%, p=0.039) and AST (50±22 vs. 38±15 U/L, p=0.009) were significantly higher and Hb (9.1±1.3 vs. 9.9±1.7 g/dL, p=0.04) lower in subjects with QTc > 440 ms compared to those with QTc ≤ 440 ms. We found no significant relationship between QTc and age, LV mass, platelet count or fetal Hb. By χ2 analysis, a larger proportion of subjects with QTc > 440 ms also had a history of acute chest syndrome (p=0.007), gallstones (p=0.047), exchange transfusion (p=0.04) and to a less significant degree, TRJV elevation (p=0.112). Prolonged QTc was not affected by sex, hydroxyurea use or a history of LVH, frequent pain, asthma, splenectomy, priapism and tonsilloadenoidectomy. Given sample size limitations and data reduction methods, we found by logistic regression analysis that the combination of TRJV and history of acute chest syndrome best predicted QTc prolongation, correctly identifying 80% of cases and resulting in positive and negative predictive values of 76% and 81%, respectively. Conclusions We conclude that QTc prolongation is common in a prospectively screened cohort of young sickle cell patients at baseline and is associated with evidence of hemolysis and to a lesser degree, TRJV elevation. Our results contrast with findings in other conditions that link QTc prolongation primarily to LVH. Future studies will be critical to further define QTc variability, pathophysiologic determinants as well as the clinical consequences of conduction abnormalities, which may or may not relate to TRJV elevation, in the sickle cell population.

Combining Fetal Hemoglobin and Reticulocytosis to Index Clinical Severity of Sickle Cell Disease in Children.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2556-2556
Author(s):  
Emily Riehm Meier ◽  
Colleen Byrnes ◽  
Maxine Weissman ◽  
Pierre Noel ◽  
Naomi L.C. Luban ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Supportive Care ◽  
Disease Severity ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Treatment Decisions ◽  
Clinical Severity ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
F Cells

Abstract Abstract 2556 Poster Board II-533 Predictors of disease severity during infancy or childhood in patients with sickle cell disease (SCD) are needed to guide treatment decisions with therapies that have known toxicities [transfusion, hydroxyurea (HU), bone marrow transplant]. Erythrocyte fetal hemoglobin (HbF) expression levels above 20% reduce sickle hemoglobin (HbS) polymerization and decrease hemolysis. As a result of the decreased hemolysis, the survival of erythrocytes is prolonged, and the overall level of erythropoiesis is reduced. To determine if clinical markers of increased HbF production and decreased erythropoiesis may be combined to score disease severity, we developed a Fetal Hemoglobin-Reticulocytosis Index (FRI) defined as: [HbF (%) × non-transfused F-cells (%)] / [Absolute Reticulocyte Count (K/uL)]. For these studies, red cell lysates were analyzed by high power liquid chromatography (HPLC) to estimate HbA, HbS, and HbF fractions. F-cells were analyzed by flow cytometry using antibodies directed against HbF, while transfused cells were labeled with antibodies directed against HbA. Dual staining with both antibodies provided a method for accurately distinguishing transfused and non-transfused F-cells (NT F-cells). A minimum of 10,000 cells was analyzed in all samples. Absolute reticulocyte counts (ARC) were determined using a Sysmex XE 2100 hematology analyzer (Sysmex America, Mundelein, IL). Preliminary studies revealed FRI values near 100 at one month of age followed by a rapid drop before the age of 4 years. Blood from children between the ages of 4 and 21 years was also studied to determine if FRI correlates with therapeutic regimen. FRI values for three groups were compared: those treated with chronic transfusion (n=19, mean FRI=0.72±1.04), HU (n=19, mean FRI=5.61±6.24), versus supportive care alone that did not include recent transfusions (n=42, mean FRI=2.70 ±4.85). When the FRI values from each of these groups were placed in rank order, the slope of the line increased sharply from a linear to an exponential shape near the FRI value of 2. To determine if the FRI=2 inflection may be indicative of reduced disease severity, the number of SCD events were determined in the 42 study subjects treated with supportive care. Overall, twenty-eight (66.7%) patients had an FRI<2, and fourteen (33.3%) patients had an FRI≥2. Among those patients, SCD events were tallied (listed in descending order according to number of events): painful crises requiring hospitalization (FRI<2, n=128; FRI≥2, n=25), pneumonia /acute chest syndrome (FRI<2, n=74; FRI≥2, n=18), splenic sequestration (FRI<2, n=14; FRI≥2, n=0), conditional transcranial Doppler [(TCD), FRI<2, n=13; FRI≥2, n=1), silent stroke (FRI<2, n=4; FRI≥2, n=2), bacteremia (FRI<2, n=2; FRI≥2, n=1), cholecystectomy (FRI<2, n=3; FRI≥2, n=0), and nephropathy (FRI<2, n=1; FRI≥2, n=0). None of the supportive care group had an overt stroke, abnormal TCD, sickle cell retinopathy, or priapism. Age adjusted analysis showed that the FRI≥2 group had significantly fewer total events per year [events/year: FRI<2 (0.70±0.52) vs. FRI≥2 (0.38 ± 0.36), p=0.02]. These data suggest that combining the clinical parameters of fetal hemoglobin production and reticulocytosis provides a simple index for SCD severity. Based upon this retrospective data, prospective studies are underway to determine if the FRI decline during infancy or FRI levels in childhood are useful to predict clinical severity and treatment decisions in SCD patients. Disclosures: No relevant conflicts of interest to declare.

A North American Experience Of Hemoglobin SC Disease, Its Complications, and Management

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2221-2221 ◽  
Author(s):  
Veronique Naessens ◽  
Richard Ward ◽  
Kevin H.M. Kuo
Keyword(s):  
Sickle Cell Disease ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Care Center ◽  
Comprehensive Care ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Baseline Hemoglobin ◽  
Hemoglobin Sc Disease

Background The phenotype of hemoglobin SC (HbSC) disease is distinct from sickle cell anemia (SCA) (HbSS and S/b0) but management of adults is mostly derived from studies of the latter group. Longitudinal observational studies on the complications and outcomes of hemoglobin SC disease are largely confined to centers outside North America. The unique ethnic composition of our cohort, consisting of mostly Western Africans and West Indians, permits further characterization of the HbSC phenotype. Objective to describe the baseline characteristics and long-term complications of a cohort of adult HbSC patients followed in a Canadian sickle cell comprehensive care center. Methods A retrospective observational cohort study was conducted on all adult patients with HbSC disease attending a sickle cell comprehensive care center in Toronto, Canada from 1994 to 2013. Baseline demographics, acute and chronic complications attributable to sickle cell disease, and laboratory data were collected. Medians were used to describe continuous variables, while percentages or ratios for categorical variables. Logistic regression was used to examine factors influencing the main clinical complications. Results 104 patients were included in the analysis, comprising of 38.5% males and 61.5% females. Median length of follow-up was 3.5 years (1 - 19) and median age at last recorded visit was 35 years (18 - 68). Median baseline hemoglobin was 119 g/L (82 - 153 g/L), hematocrit 0.340 (0.250 - 0.440), and fetal hemoglobin (HbF) fraction 1% (0 - 7.7%). Most frequent complications encountered were retinopathy (55.8%) and symptomatic avascular necrosis (27.9%), followed by painful vaso-occlusive crises requiring emergency room visit (23.1%). Presence of retinopathy was associated with higher baseline hemoglobin (OR 2.72 for every 10 g/L of hemoglobin, p = 0.037) and older age (OR 2.72 for every decade, p < 0.001). Acute chest syndrome (7.7%), priapism (7.5% of men), and renal involvement (8.2%), were less common than reported in the literature, while the rates of venous thromboembolism (8.7%), symptomatic infarctive or hemorrhagic stroke (2.9%) were slightly more common. Median right ventricular systolic pressure on 2D-transthoracic echocardiogram was 29 mmHg (17 – 43 mmHg). No patient underwent a right heart catheterization. Two patients died from septic shock, both at the age of 29. Disease-modifying therapy most often consisted of hydroxyurea (28.8%), followed by exchange transfusion (6.7%) and phlebotomies (5.8%). Hydroxyurea significantly increased the median HbF fraction (from 1% to 2.75%, p = 0.004 by related-samples Wilcoxon signed rank test). Conclusion In this large North American cohort of adult patients, we have again shown that HbSC disease is not as benign as traditionally thought. As such, patients with HbSC disease warrant similar follow-up to that provided to SCA. Retinopathy, avascular necrosis and painful vaso-occlusive crises were the most common complications in our study, albeit lower than in other reported cohorts. The frequent use of hydroxyurea in this cohort is quite unique compared to other sickle cell comprehensive care centers reported in the literature. However, therapeutic phlebotomy is less often used compared to the European experience. We also observed a lower frequency of retinopathy, avascular necrosis, painful vaso-occlusive crises, priapism and acute chest syndrome. Whether this observation is due to hydroxyurea use or to other genetic or environmental factors remains to be determined. Further studies are also required to develop a more evidence-based therapeutic strategy for this genotype of Sickle Cell Disease. Disclosures: No relevant conflicts of interest to declare.

Five years of experience with hydroxyurea in children and young adults with sickle cell disease

Blood ◽  
2001 ◽  
Vol 97 (11) ◽  
pp. 3628-3632 ◽  
Author(s):  
Alina Ferster ◽  
Parvine Tahriri ◽  
Christiane Vermylen ◽  
Geneviève Sturbois ◽  
Francis Corazza ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Laboratory Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference ◽  
History Of ◽  
Ischemic Attack

The short-term beneficial effect of hydroxyurea (HU) in sickle cell disease (SCD) has been proven by randomized studies in children and adults. The Belgian registry of HU-treated SCD patients was created to evaluate its long-term efficacy and toxicity. The median follow-up of the 93 patients registered is 3.5 years; clinical and laboratory data have been obtained for 82 patients at 1 year, 61 at 2 years, 44 at 3 years, 33 at 4 years, and 22 after 5 years. On HU, the number of hospitalizations and days hospitalized dropped significantly. Analysis of the 22 patients with a minimum of 5 years of follow-up confirm a significant difference in the number of hospitalizations (P = .0002) and days in the hospital (P &lt; .01), throughout the treatment when compared to prior to HU therapy. The probabilities of not experiencing any event or any vaso-occlusive crisis requiring hospitalization during the 5 years of treatment were, respectively, 47% and 55%. On HU, the rate per 100 patient-years of severe events was estimated to be 3.5% for acute chest syndrome, 1.2% for aplastic crisis, 0.4% for splenic sequestration; it was 0% for the 9 patients with a history of stroke or transient ischemic attack followed for an average of 4 years. No important adverse effect occurred. Long-term chronic treatment with HU for patients with SCD appears feasible, effective, and devoid of any major toxicity; in patients with a history of stroke, HU may be a valid alternative to chronic transfusion support.

scholarly journals Hematological Changes from Baseline in Children with Sickle Cell Disease Admitted for Acute Chest Syndrome Compared to Acute Pain Crisis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4909-4909
Author(s):  
Timothy Klouda ◽  
Nataly Apollonsky ◽  
Deepti Raybagkar ◽  
Bruce Bernstein
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Neutrophil Count ◽  
Laboratory Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
History Of ◽  
Pain Crisis ◽  
Hematological Changes

Abstract Title: Hematological Changes from Baseline in Children with Sickle Cell Disease Admitted for Acute Chest Syndrome Compared to Acute Pain Crisis Authors: Timothy Klouda1, Deepti. Raybagkar2, Bruce Bernstein1, Nataly Apollonsky2, Institutes:1Pediatrics, St Christopher's Hospital for Children, Philadelphia, PA, United States, 2Hematology, St Christopher's Hospital for Children, Philadelphia, PA, United States, Introduction: Children with Sickle Cell Disease suffer from multiple complications including acute pain crisis (VOC) and acute chest syndrome (ACS). Nearly 30% of children with SCD have had one episode of ACS, with the incidence higher in early childhood. The proposed pathophysiology of ACS is thought to be multi-factorial, with pulmonary fat embolism or infectious etiology being identified in a large number of patients. Increased sickling due to hypoxemia or pain has been shown to place patients at risk for ACS development., Studies have shown an increase in inflammatory markers including leukocytes and neutrophils, along with a decreased hemoglobin in SCD children who developed ACS, but no studies to date have compared laboratory changes during the acute illness to their baseline values. We hypothesized that children with SCD who are admitted for ACS will have a larger decrease in hemoglobin from baseline and a higher increase in white blood cell count from baseline when compared to those admitted for an acute pain crisis. Methods: Through retrospective chart review of patients with SCD admitted to St.Christopher's Hospital for Children we identified 45 patients with ACS. Laboratory data collected on admission from chart review included SCD genotype, age, BMI, hemoglobin, white blood cell count, absolute neutrophil count, absolute eosinophil count, platelets, reticulocyte count, hemoglobin F, vital signs and medication history. All 45 children had laboratory data collected from an acute pain crisis that occurred during a different admission for comparison. Collected data was compared to baseline laboratory data, collected during routine visit at sickle cell clinic within 1 year of admission. Changes in laboratory data from baseline during admission for ACS were compared to changes during admission for uncomplicated VOC. Results: Children with SCD who were admitted or developed ACS during admission had a larger increase in leukocyte count (6.99 vs 4.18, p=0.027) and neutrophil count (6.3 vs 3.74, p=0.04) from baseline compared to those admitted for VOC alone. Patients with ACS development also had a larger decrease in platelets (-124.74 vs -56.21, p=.047) from baseline when compared to VOC admissions. There was no statistically significant change from baseline labs when comparing hemoglobin (p=0.10), eosinophil count (p=.382), reticulocyte count (p=0.754), AST (p=0.061) and ALT (p=0.082) in the ACS and VOC groups. Children with a history of 2 or more lifetime ACS were more likely to have OSA (p=0.021), 3 or more VOCs in the past year (p=0.002), and a history of splenectomy, but it was not found to be statistically significant (p=0.155) Conclusion: Children with SCD who developed or were admitted with ACS had a significant increase in leukocyte and neutrophil count from baseline, and a decrease in platelets when compared to VOC admissions. There was no significant change from baseline in hemoglobin, reticulocyte and eosinophils detected. Future larger and multi-center prospective studies need to be performed to confirm the various changes identified in hematological markers seen in ACS vs VOC. Disclosures No relevant conflicts of interest to declare.

scholarly journals Successful Management of Sickle Cell Disease Patient with Covid-19 Infection in a Low Resource Setting: Case Study

2021 ◽  
pp. 110-114
Author(s):  
Akaba Kingsley ◽  
Edu C. Betta ◽  
Akaba Edakabasi ◽  
Essien Ofonime ◽  
Bibia Glory Philemon
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Disease Patient ◽  
Sickle Cell Disease Patient ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Management Options ◽  
Adequate Hydration ◽  
History Of ◽  
The Impact

Background: Sickle cell disease (SCD) patients have greater susceptibility to infections, they are reckoned to be vulnerable patients during the current COVID-19 pandemic. SCD patients are commonly affected by pulmonary complications such as acute chest syndrome (ACS), pulmonary embolism (PE) and pneumonia that contribute significantly to mortality risks. Aim: The study was aimed at showing the impact of SARS-COV viral pandemic on SCD patients. Presentation of Case: A 42-year-old male known sickle cell disease patient, who presented with a 5 days’ history of chest pain and difficulty in breathing with a pain score of 8/10. Pain was said to be localized and, subside on the ingestion of analgesics (Tab DF118/60mg and PCM 1000mg) with no known aggravating factor, but there was associated history of difficulty in breathing. The patient was being managed as a case of vaso-occlusive crises R/O acute chest syndrome, and was commenced on adequate hydration, oxygen saturation was between 95-85%. On examination, respiratory rate was 20 cycles per minute, pulse rate – 96 beats/minute, BP and chest examination were essentially normal. CBC showed the Packed Cell Volume of 31%, White Blood Cells 15.04 x 109/L, Neutrophils 7.51x103/µL Lymphocyte 6.50 x103/µL, Monocyte 0.76 x103/µL Eosinophils 0.20x103/µL, Basophils 0.05x103/µL, Platelet 358. The electrolytes (Na-135 mmol/L, K 3.5mmol/L, HCO3-20), urea -10 mmol/L and creatinine (88mmol/L) were normal, the chest x-ray showed cardiomegaly but the lung fields were clear. The patient was administered ceftriaxone (prophylactic antibiotics – 1 g daily).  The patient tested positive to COVID-19 and was immediately transferred to the isolation centre for proper management. He was commenced on oral medication, azithromycin, dexamethasone, ivermectine, amoxicillin/clavulanic acid, vitamin C, clexane and the analgesic was changed to paracetamol and dihydrocodeine to alternate 3 hourly with accordance to the national guidelines. In addition, he was administered subcutaneous enoxaparin due to the hypercoagulability state of SCD. The patient’s health status improved within 24hours of commencement of the above medications and remained stable all through the period of isolation and a repeat covid-19 test was done 15 days of admission using and reverse transcriptase PCR and was discharged home according to the National protocol. Conclusion: Studies and clinical trials are essential to evaluate effective diagnostic and management options for SCD patients and other high-risk conditions like diabetes hypertension, cancer patients and so on that are associated with fatal complications when infected with COVID-19 and similar diseases.

scholarly journals Misdiagnosis: Acute Chest Syndrome That Evolved into Acute Respiratory Distress Syndrome in a Patient without a Documented History of Hemoglobinopathy

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Christoph Sossou ◽  
Ogechukwu Chika-Nwosuh ◽  
Christopher Nnaoma ◽  
Jose Bustillo ◽  
Asad Chohan ◽  
...  
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Sickle Cell Disease ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Sickle Cell ◽  
Distress Syndrome ◽  
Delayed Diagnosis ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
History Of

Acute chest syndrome (ACS) is a feared complication of sickle cell disease. Here is a case of a patient who presented with symptoms suggestive of acute chest syndrome yet had a delayed diagnosis presumably due to the lack of documented history of sickle cell disease of the patient, consequently evolving into acute respiratory distress syndrome (ARDS). He was subsequently diagnosed with heterozygous sickle cell SC disease on hemoglobin electrophoresis. After appropriate management with mechanical ventilator, broad-spectrum empiric intravenous antibiotics, exchange transfusion, and intravenous fluid resuscitation, the patient was medically optimized and safely discharged home, with significant improvement noted on successive follow-up visits.

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