scholarly journals Incentive Spirometry to Prevent Acute Chest Syndrome in Adults with Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3660-3660
Author(s):  
Bart J. Biemond ◽  
Charlotte F.J. Van Tuijn ◽  
Aafke E. Gaartman ◽  
Erfan Nur ◽  
A. W. Rijneveld
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Risk Score ◽  
Predictive Value ◽  
Plasma Levels ◽  
Adult Patients ◽  
Acute Chest Syndrome ◽  
Control Group ◽  
Cell Disease ◽  
Incentive Spirometry

Abstract Introduction: Amongst patients with sickle cell disease (SCD) the leading cause of death is the acute chest syndrome (ACS). This pneumonia-like complication frequently occurs during or shortly after a vaso-occlusive crisis (VOC). In pediatric patients hospitalized for VOC, incentive spirometry has demonstrated to prevent the development of ACS. This study was designed to determine if a comparable effect of incentive spirometry can be demonstrated in adult patients with SCD. Furthermore, we aimed to validate the ability of the Bartolucci score to identify patients at risk of ACS and assessed the value of procalcitonin as a potential biomarker for ACS . In addition, clinical characteristics and laboratory results were determined to identify potential risk factors. Methods: In this multicenter prospective randomized trial, we included consecutive adult patients (≥18 yr) admitted for VOC presenting with chest or back pain above the diaphragm. Patients were randomly assigned to spirometry or control group. Patients presenting with ACS were excluded. A chest radiograph was performed 5 days after admission, or sooner when clinically indicated, in order to diagnose pulmonary abnormalities. ACS was defined as a new infiltrate/atelectasis combined with pulmonary symptoms. At presentation, procalcitonin plasma levels were assessed and the Bartolucci risk score was calculated to determine to the risk of developing ACS for each patient. In addition, clinical and laboratory parameters were compared between patients with and without ACS during admission. Results: In total 66 episodes of hospitalization for VOC in 48 patients were included. Median age was 26 years and 46 of the hospitalizations concerned patients with a severe genotype (HbSS/HbSβ0 thalassemia) versus 20 hospitalization with a mild genotype (HbSC/HbSβ+thalassemia). The overall incidence of ACS in this study cohort was 19.7%. In the spirometry group, ACS was diagnosed in 5/34 (14.7%) hospitalizations compared to 8/32 (25%) hospitalizations in the control group (OR 0.5 [0.15-1.8]; P=.293). Twelve of the 13 ACS episodes occurred in patients with a severe genotype. The Bartolucci risk score could be calculated for 50 hospitalizations. The scores area under the curve (AUC) was 0.747 (P=.013), with a negative predictive value (NPV) of 94% and a positive predictive value (PPV) of 31%. No difference in procalcitonin plasma levels were found between patients with and patients without ACS (0.52 ± 1.56 μg/ml versus 0.56 ± 1.44 μg/ml, respectively). At baseline, hemoglobin levels were significantly lower while LDH plasma levels, leukocyte and platelet counts were significantly higher in ACS hospitalizations as compared to non-ACS hospitalizations. Patients who developed ACS showed significantly more documented fever during admission (61.5% vs 17.0%) and a longer length of hospital stay (median 10.0 days vs 4.5 days). Conclusion: Incentive spirometry did not significantly reduce the development of ACS in this prospective study in adult patients with SCD admitted with VOC and pain above the diaphragm. Procalcitonin plasma levels and the Bartolucci score could not accurately identify patients that at risk to develop ACS, but a low score appeared to be a reliable tool to identify patients with a low risk of ACS. Disclosures No relevant conflicts of interest to declare.

scholarly journals A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease

2017 ◽  
Vol 179 (4) ◽  
pp. 627-634 ◽  
Author(s):  
Anaïs Winchenne ◽  
Jérôme Cecchini ◽  
Jean-François Deux ◽  
Nicolas De Prost ◽  
Keyvan Razazi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Sickle Cell ◽  
Risk Score ◽  
Adult Patients ◽  
Acute Chest Syndrome ◽  
Clinical Risk Score ◽  
Cell Disease ◽  
Clinical Risk ◽  
Artery Thrombosis

Refining Th Predictive Value of Secretory Phospholipase A2 In Sickle Cell Disease Patients with Acute Chest Syndrome

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 846-846 ◽  
Author(s):  
Lori Styles ◽  
Carrie Greene Wager ◽  
Richard J. Labotka ◽  
Kim Smith-Whitley ◽  
Alexis A. Thompson ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Phospholipase A2 ◽  
Sickle Cell ◽  
Predictive Value ◽  
Laboratory Data ◽  
Research Network ◽  
Acute Chest Syndrome ◽  
Secretory Phospholipase A2 ◽  
Cell Disease ◽  
Secretory Phospholipase

Abstract Abstract 846 Elevation of the serum level of secretory phospholipase A2 (sPLA2) has previously been reported to predict impending acute chest syndrome (ACS) in sickle cell disease (SCD) patients admitted with pain. The Sickle Cell Disease Clinical Research Network initiated the PROACTIVE Feasibility Study to 1) assess the feasibility of screening, randomizing and treating with simple transfusion eligible patients and 2) assess the predictive utility of sPLA2 in screening for imminent ACS, characterize the distribution of sPLA2 values in sickle cell patients hospitalized with pain, and define a cut-off point for predicting ACS to optimize its reliability, sensitivity and specificity in predicting ACS. All SCD patients (hemoglobin SS, SC and SBeta-thalassemia) admitted for pain at a participating center were to be screened for eligibility; patients with a diagnosis of ACS at entry were not eligible. sPLA2 levels were drawn daily on consenting patients for up to three days during the admission. All patients had at least one CXR (mandated at 72 hr if not done for clinical indications) during the course of their hospital stay to determine if they had developed a new infiltrate, considered diagnostic for ACS. Patients who developed T>38°C, a sPLA2 level > 100ng/ml and had a normal CXR were eligible for randomization to observation or simple transfusion; the remaining patients were followed in an Observation arm. Of 421 patients potentially eligible for screening, 238 were enrolled in the study with 10 of these patients randomized (4 to transfusion 6 to standard care). Of these, 203 patients had from one to three sPLA2 levels drawn prior to an ACS diagnosis or did not develop ACS; if transfused, they received blood only after a diagnosis of ACS was made. Twenty-two of these patients (11%) developed ACS. Analysis of the maximum sPLA2 levels prior to ACS diagnosis revealed that a threshold of 45 ng/ml was the most accurate level to predict ACS (accuracy: 73%, sensitivity: 73%, specificity: 73%). The positive predictive value (PPV: 25%) was low due to the relative infrequency of ACS events. When sPLA2 levels were analyzed in children versus adults, sPLA2 was more accurate for adults in predicting ACS with a level of 65ng/ml having accuracy: 88%, sensitivity: 44%, specificity: 95%, PPV: 57%. The addition of a requirement for fever did not improve the accuracy of sPLA2 in either adults or children. Further analysis of sPLA2 levels with various clinical and laboratory data is ongoing to improve the PPV of sPLA2. These results indicate that sPLA2 can predict ACS with good sensitivity, but may require additional parameters to be useful in clinical management of SCD patients admitted with pain and at risk for ACS. Supported by the NHLBI. Disclosures: Labotka: HemaQuest Pharmaceuticals, Inc: Research Funding.

Implementation of a European Cohort to Follow Sickle Cell Children and Adults Treated with Hydroxycarbamide

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 564-564
Author(s):  
Mariane De Montalembert ◽  
Frédéric Galacteros ◽  
Jean Antoine Ribeil ◽  
Uwe Kordes ◽  
Jean Benoit Arlet ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Safety Profile ◽  
Conflicts Of Interest ◽  
Subcutaneous Tissue ◽  
Acute Chest Syndrome ◽  
Control Group ◽  
Cell Disease ◽  
Skin Reactions ◽  
Number Of Patients

Abstract Hydroxycarbamide (HU) is a myelosuppressive drug marketed since 1968 for the treatment of hematological cancer, and authorized since 2007 in Europe as orphan medicinal product for the prevention of recurrent vaso-occlusive crises including acute chest syndrome in adults and children older than 2 years with sickle cell disease (SCD). ESCORT-HU (European Sickle Cell Disease Cohort – Hydroxyurea) is a multicenter prospective non interventional study implemented in Europe to collect more information about the safety profile of HU and morbi-mortality in SCD patients treated with HU. The study responds to EMA (European Medicines Agency) request and has been approved by the Ethical of Necker Enfants Malades Hospital (Paris, France).The ongoing study involves the largest number so far of patients with SCD treated with HU. Primary endpoints of ESCORT HU are to determine frequency of adverse events, and possible consequent changes of HU treatment. Secondary endpoints are to evaluate morbi-mortality of the disease although in the absence of control group. From June 2008 to June 2014, 483 patients (255 females; 228 males) were enrolled from 3 European countries, Greece (24%), Germany (19%), and France (56%). 67% patients were adults, median aged 37.35 yrs (17-83.5) and 33% were children, median aged 11.06 yrs (2.6-16.9). genotypes were HbS/HbS in 71.4% cases, and compound heterozygotous HbS/β-thalassemia in 22.8 % (Table 1). 137 (28.4%) patients experienced 421 events (Table 2). 132 (32.2%) of these events may be attributed to HU. The safety profile is roughly similar in children and adults. As expected the most frequent side effects were firstly blood disorders (n=86 events, 42.4%) such as neutropenia or thrombocytopenia. In all cases, these cytopenias were rapidly resolved with the transitory stop of HU. 71 events related to skin and subcutaneous tissue disorders were observed, mostly cutaneous dryness, skin reactions, alopecia and nails or skin pigmentation; 4 patients had a leg ulcer (34.8%). Most of these events are ongoing or stabilized despit the decrease of HU. No secondary cancer has been reported until now. Even if HU is an old drug with a relatively well-known safety profile, some uncertainties remain in terms of long-term safety as well as tolerance in the youngest people. The main interest of ESCORT HU is to offer the possibility of safety surveillance of hydroxycarbamide in European sickle cell patients. Table 1 Demographic data Adults Children < 17 years old Total Number of patients 322 (67%) 161 (33%) 483 Females/Males 183/139 72/89 255/228 Median age (yrs) (range) 37.35 (17-83.5) 11.06 (2.6-16.9) 28.58 Genotype SS 206 (64%) 139 (86.3%) 345 (71.4%) SC 1 (0.3%) 3 (1.86%) 4 (0.8%) Sβ0 51 (15.8%) 11 (6.8%) 62 (12.8%) Sβ+ 46 (14.2%) 2 (1.2%) 48 (9.9%) Other 18 (5.5%) 6 (3.7%) 24 (4.9%) Treatment with HU before enrollment in ESCORT HU No of pts 232 83 315 (65%) Median duration (range) of HU treatment before ESCORT HU 8.2 yrs (0.5 ans-24 yrs) 3. 1 yrs ( 71 days – 8.9 yrs) 6.85 (71 days-24 years)] HU ESCORT Daily mean dose (mg/kg/d) 16.11 ± 4.79 19.63 ± 4.69 17.32 ± 4.94 Abstract 564. Table 2 The most frequent events of hydroxycarbamide in the two populations of SCD patients ADULTS CHILDREN No ofGerman(%) No of adults No ofEpisodes(%) No of children Total(% /411) Events Related to HU treatment (Siklos®)(%**) Blood and lymphatic system disorders (%) 32 (17.7) 22 54 (31.03) 28 86 (20.9) 56 (65.1) Skin and subcutaneous tissue disorders (%) 42 (23.2) 28 29 (16.7) 19 71 (17.3) 46 (64.8) Nervous system disorders Headache (24), Dizziness/vertigo (14), 32 (17.7) 23 12(6.9) 10 44 (10.7) 11 (25) Gastrointestinal disorders Nausea (14), diarrhea (8), other (14) 20 (11) 17 23 (13.2) 16 43 (10.4) 7 (16.3) Metabolic and nutrition disorders: vit D deficiency (17), weight gain (5) 13 (7) 11 18 (18.3) 18 36 (8.75) 4 (11.1) Fever 11 (6) 10 12(6.9) 7 23 (5.6) 1 (4.3) Cardiac disorders (hypertension, bradycardia, chest pain, cardiomegaly) 4 4 2 2 6 1 (16.6) General disorders : fatigue 5 5 0 0 5 0 Hepatobiliary disorders 2 2 0 0 2 0 Neoplasms benign, malignant and unspecified (incl. cysts and polyps) Harmatoma, benign vulvar sebaceous cyst 2 2 0 0 2 0 Renal & urinary disorders 2 2 0 0 2 0 Reproductive system and breast disorders 3 3 0 0 3 0 Other 13 13 21 14 34 6 (17.1%) 181 80 /181(24.8%) 174 57 / 174(35.4%) 411 132/411 (32.2%) ** compared to the total number of “system organ class” events Disclosures No relevant conflicts of interest to declare.

Leukocyte Apoptosis and Inflammation in Iron-Overloaded Patients with Sickle Cell Disease or β-Thalassemia: A Mechanism for Increased Stroke and Disease Severity in Sickle Cell Disease.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1233-1233 ◽  
Author(s):  
Patrick B. Walter ◽  
David W. Killilea ◽  
Ellen B. Fung ◽  
Annie Lui ◽  
Jacqueline Madden ◽  
...  
Keyword(s):  
Cell Death ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Strong Predictor ◽  
Acute Chest Syndrome ◽  
Control Group ◽  
Cell Disease ◽  
Delayed Cell Death ◽  
Leukocyte Number

Abstract Sickle cell disease (SCD) is a hemoglobinopathy characterized by micro-vascular hypoxia-reperfusion, inflammation and leukocytosis. Studies in SCD have shown that leukocytosis is a strong predictor of stroke and disease severity. It is known that leukocytosis and inflammation contribute to increased leukocyte-endothelial adhesion and vasoocclusive events. Leukocytosis or increased leukocyte number is determined by the balance between cell death programs (apoptosis) and proliferation. In this study, we examine markers of apoptosis and proliferation in SCD as compared to thalassemia (a hemoglobinopathy that is vasculitis negative) and a control group. Methods: Markers of leukocyte turnover, inflammation and free iron (NTBI, non-transferrin bound iron) were compared in 11 patients with SCD (7M, 13 ± 4 yrs), 18 with thalassemia (7M, 24 ± 9 yrs) and 10 disease-free controls (5M, 27 ± 12 yrs). All SCD and thalassemia patients were healthy and event free in the previous 4 months. Blood was obtained fasting and prior to RBC transfusion; and plasma, serum and cells were separated by centrifugation. The pro-apoptotic markers nucleosome protein (DNA laddering) and Bax (initiator of mitochondrial permeability) and inflammatory marker, high-sensitivity C Reactive Protein (hsCRP) were determined by ELISA. NTBI (DNA damage inducing, pro-apoptotic) was measured by HPLC. Plasma levels of cytokines (anti-apoptotic, proliferation stimulating) were determined by using a multiplex bead-based immunoassay. Plasma lactate dehydrogenase (LDH), a marker of hemolysis, was also measured because hemolysis products have been shown to inhibit apoptosis. Results: Leukocyte number and absolute neutrophil count were 1.8 and 2.2 fold higher in SCD compared to thalassemia (P&lt;0.01). Evidence for decreased apoptosis in SCD included reduced nuleosome protein as compared to thalassemia and reduced Bax as compared to controls. In addition, LDH was significantly increased 1.9 fold in SCD relative to thalassemia (p=0.012). In contrast, NTBI was 2 fold higher in thalassemia than SCD and correlated with nucleosome protein (R=0.45; p=0.013). Finally, high levels of the cytokines IL-6 and IL-10 in SCD relative to thalassemia may be stimulating leukocyte proliferation and survival. These cytokines and hsCRP are also positively correlated with leukocyte number. Conclusions: These preliminary findings demonstrate that the balance of apoptosis vs. proliferation favors delayed cell death and enhanced cell proliferation in SCD, leading to increased circulating leukocytes. These changes may be related to hemolysis, increased levels of inflammatory cytokines, and lower amounts of NTBI in SCD. This leukocytosis increases the potential for vasoocclusive events that contribute to stroke, acute chest syndrome or renal tubule damage.

Is Incentive Spirometry Effective in Preventing Post Surgical Acute Chest Syndrome in Sickle Cell Disease Patients?

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4807-4807
Author(s):  
Adlette Inati ◽  
Fouad Ziade ◽  
Suzan Koussa ◽  
Ali Taher
Keyword(s):  
Postoperative Complications ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Perioperative Care ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Clinical Events ◽  
Number Of Patients ◽  
Incentive Spirometry

Abstract Persons with sickle cell disease (SCD) are more likely to undergo cholecystectomy and splenectomy than are the general population. Even with meticulous care, surgical complications are seen in approximately 25% to 30% of patients. A chart review of SCD patients who had splenectomies and cholecystectomies and who were treated in 3 centers between1987–2007 was undergone. Data pertaining to clinical events, surgery, perioperative care and outcome were collected and analyzed. Since 1995, laparoscopic surgery was the modality used. Management consisted of preoperative transfusions in 83% of patients, general anesthesia, adequate hydration, temperature conservation, non sedating analgesia and supervised incentive spirometry exercises (after 1995) in all patients. The total number of patients was 120: 81 underwent splenectomy, 59 cholecystectomy and 20 both procedures. Of patients who underwent splenectomy, 43.2% were females and 56.8% males; 64.2% had sickle cell anemia (SCA) and 35.8% sickle-beta thalassemia (ST). For those undergoing cholecystectomy, 39.0% were females and 61.0% males; 79.7% had SCA and 20.3% ST. Patients undergoing splenectomy were significantly younger (mean age 9.28 years) than those undergoing cholecystectomy (mean age 15.28 years) (p=0.037). Median operative time was 50 minutes, and median hospitalization duration was 2 1/2 days. No major intra operative complications or fatalities were noted. Postoperative complications included acute chest syndrome (ACS) in 5 patients (4% of surgeries). The mean time to onset of symptoms of ACS was 36 hrs after surgery (range, 24–96 hr). All patients who developed this complication did not receive incentive spirometry or were noncompliant with this therapy. As for the relation between clinical events and splenectomy/cholecystectomy, bivariate analysis showed a significant association between splenectomy and regular blood transfusion (p=0.005). Borderline significance was found for the association of cholecystectomy with ACS, joint necrosis and stroke (0.05&lt;p&lt;0.10). Predictors of splenectomy were regular blood transfusion (OR=2.38, [1.36–4.17] 95%CI], ACS (OR= 3.42, [1.64–7.15] 95%CI) and ST as compared to SS (OR=1.89, [1.10–3.27] 95%CI). Predictors of cholecystectomy were regular blood transfusion (OR=2.02, [1.10–3.71] 95%CI and sepsis (OR=2.59, [1.04–6.45] 95%CI). Sensitive predictors of postoperative complications could not be studied due to the small number of complications. This survey shows that splenectomy and cholecystectomy can be performed in SCD patients with minimal morbidity and no mortality. The current rate (4%) of post surgical ACS is lower than previously reported and is probably due to strict adherence to meticulous perioperative care. The absence of post surgical ACS in patients treated with supervised incentive spirometry is suggestive of a beneficial effect of this therapy in preventing this serious complication. The small number of patients with post surgical ACS prevents us, however, from drawing definite conclusions about the preventive role of this treatment. Nevertheless, early use of this simple, available and inexpensive tool most likely minimizes surgical morbidity and mortality from ACS, decreases hospitalization time, cuts down expenses and can be helpful particularly in countries with limited resources. Prospective controlled studies are, however, warranted to evaluate the efficacy of incentive spirometry and other perioperative interventions in preventing complications in SCD patients undergoing surgery.

Fever In Hospitalized Adult Patients with Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2652-2652
Author(s):  
Deepa Jeyakumar ◽  
Matthew Zibelman ◽  
Ryan Hurth ◽  
Lani Krauz ◽  
Santosh Saraf ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Hospital Setting ◽  
Adult Patients ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Indwelling Catheters ◽  
Upper Limit ◽  
Pain Crisis

Abstract Abstract 2652 Background: Sickle cell disease is complicated by veno-occlusive crises leading to pain crises, chronic end-organ damage and early mortality. With recent advances in the management of sickle cell disease in childhood, sickle cell patients are living longer. However, our understanding of the clinical course of adult sickle cell disease remains limited and is based largely on extrapolation of knowledge from children with sickle cell disease. Unfortunately, adult patients remain at an elevated risk of infections due to encapsulated organisms in the setting of functional asplenia. This risk is exacerbated by possible indwelling catheters and exposure to the health care environment. Fever in these patients can herald a serious infection. Alternatively, brisk hemolysis can be associated with fever. Wierenga, et. al. (2001) described that fevers to 39F in children were associated with acute chest syndrome (21%), painful vaso-occlusive crisis (27%), and bacteremia in 6%. To our knowledge, no such review of fever in hospitalized adult sickle cell patients has been published in the medical literature. Therefore, the clinician is placed in a diagnostic dilemma regarding the management of fever in adult patients with sickle cell disease. Objective: To determine the etiologies of fevers in hospitalized adult patients with sickle cell disease in an urban tertiary hospital setting. Methods: We performed an IRB-approved retrospective analysis of 143 admissions between 1995–2008 with sickle cell pain crisis and had a fever greater than 38.5C during the admission. The aim was to determine the prevalence of fevers due to infectious versus hemolysis-related causes in the population of interest. Elevated white blood cell count (defined as greater than 1.5x upper limit of normal), radiologic and/or culture data were used to classify a fever as due to infection. Elevated LDH and total bilirubin (defined as greater than 2x upper limit of normal) were used to classify a fever as due to hemolysis. The risks of infection in patients on hydroxyurea as well as indwelling catheters (including central lines and foley catheters) were assessed. We also evaluated the risk of hemolysis in patients on hydroxyurea. Finally, the use of antibiotics and duration of the fever in patients with hemolysis and infection were also evaluated. Results: Among patients admitted with sickle cell pain crisis and had a fever during their hospitalization, we found evidence of infection in 65% and hemolysis in 58%. Interestingly, 35% had evidence of both infection and hemolysis. Approximately, 11.8% had no significant evidence of infection or hemolysis. Antibiotics were used in 66% of all patients with pain crisis and fever. Among the patients who received antibiotics, 81% had evidence of infection and 19% had no evidence of infection. Approximately 1/5 patients with fevers received antibiotics despite the absence of evidence of infection. Infections were not increased among hydroxyurea users (61.5% with fever) over non-hydroxyurea users (67.9% with fever), p = 0.4. Fevers due to documented infections were found in 78% of patients with indwelling catheters compared with 62% of patients without catheters, p≤0.05. The risk of fever due to hemolysis was not significantly different between hydroxyurea and non-hydroxyurea users at 58% versus 57% respectively, p=0.9. Of patients with fevers for more than one day, infection was found in 69% of patients compared with 31% of patients who had no evidence of infection p=0.5. Whereas, of patients with fevers for more than one day, hemolysis was found in 57% of patients compared with 42% of patients who did not have evidence of hemolysis with p=0.9. Conclusions: Among adult sickle cell patients hospitalized with pain crisis and fever, hemolysis accounted for 58% of cases while infections accounted for 65% with 35% evidence of both. Infections were not increased among hydroxyurea users. Indwelling catheters did increase the risk of fevers due to infection. The risk of fever due to hemolysis was not significantly increased among patients on hydroxyurea. Finally, in patients with fevers for more than one day, hemolysis accounted for 57% of cases and infection accounted for 45%. These findings provide initial investigation of the etiologies of fevers in adult hospitalized sickle cell patients and further studies are necessary to confirm these findings. Disclosures: No relevant conflicts of interest to declare. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Complications in Pregnancy of Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 32-33
Author(s):  
Avani Singh ◽  
Conner Olsen ◽  
Xu Zhang ◽  
Robert E. Molokie
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Mode Of Delivery ◽  
Acute Chest Syndrome ◽  
Control Group ◽  
Thromboembolic Events ◽  
Cell Disease ◽  
Fetal Outcomes ◽  
Systemic Complications

Sickle cell disease (SCD) has many systemic complications including vaso-occlusive crises (VOC), stroke and increased mortality. However, as a result of advances in the care of children with SCD, over 90% of those born with SCD in the US live until they are at least 20 years of age. As survival improves, more women with SCD are able to have children. Through improvements in the management of pregnancy, the majority of women with SCD can have an uncomplicated birth though they do have a high incidence of medical and pregnancy complications compared to those women with normal phenotype. While current literature regarding pregnancy in SCD often compares sickle cell patients to normal hemoglobin phenotype patients, more research is needed comparing individuals with SCD. This study seeks to examine the number of vaso-occlusive crises, acute chest syndrome (ACS) episodes and thromboembolic events in pregnant patients with SCD, comparing during pregnancy, the 12 months before and the 12 months after pregnancy. A 10-year retrospective study was conducted at University of Illinois Health System (UIH) and included women over the age of 18 with sickle cell disease and pregnancy. These patients followed primarily at UIH for both their sickle cell and obstetric care. The query included 208 pregnancies and 177 patients. The primary endpoint was to compare the number of VOC, ACS, and thromboembolic events in patients with sickle cell disease during 9 months of pregnancy, the 12 months prior to their pregnancy and the 12 months after their pregnancy. Secondary endpoints include prophylactic, simple, and exchange transfusions throughout the pregnancy, mode of delivery, obstetric complications (preeclampsia, eclampsia, mortality), and fetal complications (IUGR, Apgar scores, mortality). Of 208 pregnancies from the initial query, 121 met inclusion criteria for the study. 87 of the 208 pregnancies met exclusion criteria due to other hemoglobin genotypes, delivery outside the study timeframe, delivery at outside hospital, or encounters for ectopic pregnancies, spontaneous or elective abortions. Paired comparisons between pregnancy, 12 months before the pregnancy, and 12 months after the pregnancy were carried out using non-parametric method (Wilcoxon's test) or generalized mixed effects models. Results demonstrated a statistically significant increase in VOC and ACS during pregnancy, compared to the 12 months prior and 12 months after pregnancy, as demonstrated in Table 2. Thromboembolic events had a slight trend of increase during pregnancy compared to the period before or after. These results were adjusted for age at delivery and restricted to SS genotype. VOC and severe genotype were found to associate with maternal and fetal outcomes such as C-section (p=0.04), transfusion requirement at delivery (p=0.007), low birth weight (p=0.008), and preterm delivery (p=0.02). Our findings demonstrate a definitive and statistically significant increase in VOC and ACS during pregnancy compared to the 12 months before or after pregnancy among women with SCD. This unique study compares patients with SCD to themselves longitudinally through time, utilizing a superior control group. Although this increase in VOC and ACS has been observed previously, prior studies compared pregnant patients with SCD to other patients with SCD, or to controls with normal hemoglobin. As hypothesized, thromboembolic events had a slight trend towards increasing during pregnancy, however this comparison was not statistically significant. Although prior data has shown prophylactic transfusions to decrease VOC in pregnant women with SCD, prophylactic transfusions were rarely utilized in this study group. These results demonstrate a definitive increase in VOC in pregnant individuals with SCD, and additionally affecting maternal and neonatal outcomes. Based on this study, decreasing rates of VOC during pregnancy may improve maternal and fetal outcomes. This may be achieved with prophylactic transfusions; however additional studies must be conducted to improve care in this patient population. This study will also allow us to further investigate additional characteristics that change during this timeline during pregnancy. This offers new opportunities to institute individualized preventive strategies, expanding the role of personalized medicine in the care of sickle cell patients. Disclosures No relevant conflicts of interest to declare.

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