Complications of Implantable Venous Access Devices In Patients with Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1649-1649
Author(s):  
Nirmish Shah ◽  
Daniel Landi ◽  
Radhika Shah ◽  
Jennifer Rothman ◽  
Courtney Thornburg
Keyword(s):  
Sickle Cell Disease ◽  
Bloodstream Infection ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Conflicts Of Interest ◽  
Bloodstream Infections ◽  
Venous Access ◽  
Categorical Variables ◽  
Cell Disease ◽  
Venous Access Devices

Abstract Abstract 1649 INTRODUCTION: Implantable venous access devices (VADs) are used in sickle cell disease (SCD) for patients with poor venous access to facilitate chronic blood transfusions and management of acute complications. Children and adults with chronic illnesses have high rates of VAD-related complications including bloodstream infection and thrombosis. Patients with SCD may be at higher risk given the presence of functional asplenia and evidence of a hypercoaguable state. The objective of this study was to define the frequency of VAD related bloodstream infections and thrombosis in adults and children with SCD. PATIENTS AND METHODS: We performed a single institution retrospective review of VAD placement in patients with SCD. Subjects were identified through the sickle cell clinic database and the Hospital Information System. Subjects were included if they had SCD, VAD placement between December 1, 1998 to December 1, 2009 and had completed at least 12 months of follow-up. VAD-related bloodstream infection was defined by positive blood culture and VAD-related thrombosis (deep vein thrombosis, superior vena cava syndrome, and pulmonary embolism without lower extremity thrombosis) was defined by imaging. Comparisons were made between pediatric and adult sickle cell patients using Student's t-test for continuous variables and Fisher's exact test was used to compare categorical variables; p<0.05 was considered significant. RESULTS: Of the greater than 800 sickle cell patients followed at our Comprehensive Sickle Cell Center, 32 subjects were eligible for inclusion (median age 20 years, range 1–59). There were 81 VAD placed (median 2.6 VAD per patient, range 1–7) with a total of 49268 catheter days (median 608, range 323–3999). The mean catheter lifespan in adults (1798 days ± 266) was significantly higher than pediatric patients (971 ± 328, p=0.039). There were a total of 66 VAD-related bloodstream infections (1.34 infections per 1000 catheter days) occurring in 17 of 32 (53%) subjects. Although not statistically significant, children had fewer VAD-related bloodstream infections (3 of 10; 30%) compared to adults (14 of 22; 64%, p=0.08). There were 24 catheter-related thromboses (0.49 thromboses per 1000 catheter days) occurring in 10 of 32 (41%) of subjects. Children also had fewer VAD-related thrombosis (1 of 10; 10%) compared to adults (9 of 22; 40%, p=0.08). The overall rates of infection and thrombosis per 1000 catheter days were not significantly different between adult and pediatric patients. CONCLUSION: In summary, we report a long lifespan and low rate of infection in the subjects who had VADs during the study period. Most concerning was a high proportion of adults with catheter-related thrombosis, which adds the burden of anticoagulation to patient management and put patients at risk for post-thrombotic syndrome. Potential lifespan of VADs, risk of bloodstream infection and thrombosis as well as its long-term consequences should be discussed with patients and families considering VAD placement. Disclosures: No relevant conflicts of interest to declare.

Infectious Complications of Implantable Venous Access Devices in Patients with Sickle Cell Disease

2004 ◽  
Vol 15 (4) ◽  
pp. 375-378 ◽  
Author(s):  
Steven C. Wagner ◽  
David J. Eschelman ◽  
Carin F. Gonsalves ◽  
Joseph Bonn ◽  
Kevin L. Sullivan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Venous Access ◽  
Infectious Complications ◽  
Cell Disease ◽  
Venous Access Devices

Complications of Central Venous Access Devices in Patients With Sickle Cell Disease and Thalassemia Major

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Javier Ordóñez ◽  
Agustín del Cañizo ◽  
Cristina Beléndez ◽  
Marina García-Morín ◽  
Laura Pérez-Egido ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Central Venous Access ◽  
Thalassemia Major ◽  
Venous Access ◽  
Cell Disease ◽  
Central Venous ◽  
Venous Access Devices

Adding Hydroxyurea to Chronic Transfusion Therapy for Stroke in Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4620-4620
Author(s):  
Susan Claster ◽  
Keith C. Quirolo
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Reticulocyte Count ◽  
Conflicts Of Interest ◽  
Hematological Parameters ◽  
Fetal Hemoglobin ◽  
Venous Access ◽  
Time Frame ◽  
Cell Disease ◽  
Transfusion Therapy

Abstract Abstract 4620 Introduction Transfusions have clearly been shown to prevent stroke in patients with Sickle Cell Disease(SCD). The usual goal of transfusion therapy is to keep the percent sickle Hgb (%S) less than 30. %S greater than 30 has been associated with stroke reoccurance. Inability to transfuse adequately may result from poor vascular access or high hemolytic rate. Hydroxyurea, a drug which requires active erythropoeisis, is usually not given to patients who are chronically transfused. However, the presence of an elevated reticulocyte count in a transfused patient indicates ongoing sickle hematopoiesis. We added Hydroxyurea therapy to two patients who were unable to achieve %S levels less than 30 and who had elevated reticulocyte counts to assess whether this drug could improve their hematological parameters. Results We treated two post stroke patients, one receiving pheresis therapy, and the other on straight transfusion therapy who were unable to achieve 30% Hgb S despite regular transfusions. Patient 1, a 33 y/o female with Hgb SS had been on transfusions since childhood for multiple CVA's and resulting moya-moya. Her last stroke had occurred while she was being chronically transfused and had a % S of 40. Due to venous access issues the patient was receiving straight transfusion only and was unable to adequately suppress her marrow. In 8/07 her %S was greater than 40 and her reticulocyte count was 11.3 %. Hydroxyurea was started at 1500 mg daily(22 mg/kg). Over the next 24 months her Bilirubin dropped from 8.7mg/dl to 1.9 mg/dl, her reticulocyte count dropped to 4%(figure below) and her MCV increased to 104.6 from 90. Her Hgb F rose to 30%.Her %S has not changed. She has remained clinically stable with no new neurological findings. The second patient, a 17 y/o male had been on chronic transfusions since childhood for a stroke which resulted in a dense left hemiparesis. He has been on a pheresis program for 13 years. Despite this his % S remained greater than 40% and had been as high as 60% prepheresis. Hydroxyurea was added in 5/08 at a dose of 25mg/kg. Within 6 months his pretransfusion %S had dropped to 22-27%. No increase in fetal Hgb was detected, possibly due to the exchange transfusion process. His bilirubin, which had been running between 12-16 mg/dl dropped to 7.6-8.9 mg/dl and his reticulocyte count went from an average of 19.6% to 11.2% in the same time frame. He has also remained stable. Discussion These two patients demonstrate that the addition of Hydroxyurea to patients on chronic transfusion can be useful in decreasing hemolytic rate and improving the efficacy of transfusions by decreasing sickle erythropoiesis and increasing fetal hemoglobin synthesis. Further studies of the combination of these two modalities may be warranted in those patients who cannot achieve adequate suppression of sickle erythropoeisis. Disclosures: No relevant conflicts of interest to declare.

scholarly journals A Retrospective Chart Review to Assess Indications, Risk Factors and Complications Associated with the Use of Indwelling Catheters in Pediatric Patients with Sickle Cell Disease - a Single Institution Experience

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4856-4856
Author(s):  
Chibuzo Ilonze ◽  
Michael P Anderson ◽  
Alexander Stubblefield ◽  
Janna M. Journeycake ◽  
Arpan Sinha
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Subclavian Vein ◽  
Chart Review ◽  
Pediatric Patients ◽  
Retrospective Chart Review ◽  
Venous Access ◽  
Cell Disease ◽  
Port Placement

Background: Sickle cell disease (SCD) and its complications are associated with frequent hospital visits and treatment often requires venous access for administration of fluids, medications and blood transfusions. Due to frequent use, peripheral venous access can become difficult over time because of venous scarring. Moreover, certain interventions such as chronic simple or exchange transfusions require reliable venous access for prolonged periods of time. Implantable central venous devices such as ports offer definitive access and mitigate the need for frequent peripheral access attempts. However, existing knowledge on the use of these devices in pediatric patients with SCD is limited. Due to possible increased risks of thrombosis and mechanical occlusion from hypercoagulable state, risk of infectious complications and need for surgical placement, ports are often not used routinely. We review the indications and complications associated with placement of ports in the pediatric cohort of patients with SCD - to better define the scope of port placement in this group. Methods: We performed an IRB-approved, retrospective chart review to assess the indications, complications and risk factors associated with port placement in children and adolescents with SCD at the University of Oklahoma Health Sciences Center (OUHSC). The study period analyzed was 17.5 years from January 1st, 2000 to July 30th, 2018 and included patients from birth to 21 years of age, diagnosed with SCD who had homozygous sickle cell disease (HbSS) or compound heterozygous SCD - either sickle cell-β0-thalassemia, sickle cell-β+-thalassemia or sickle cell-hemoglobin C (HbSC) disease, and had ports in place for more than 7 days. Patients were identified systematically by querying the OUHSC Clinical Data Warehouse using diagnostic codes followed by chart review. Results: Thirty-two patients with SCD and ports were identified during the study period, out of which 31 patients had HbSS and one patient had HbSC disease. The median age at first port insertion was 8 years (range 1-20 years). A total of 63 ports were placed for a total of 99,272 port days with a median port life span of 1340 days. The two main indications for port placement were - either chronic transfusions for which 48 ports were placed for a total of 80,238 port days, or poor venous access (PVA) for which 15 ports were placed for a total of 19,034 port days. Out of the 48 ports placed for chronic transfusions, 6 ports were for transfusions for primary stroke prophylaxis, 22 ports were for transfusions for secondary stroke prophylaxis, 17 ports were for transfusions for recurrent vaso-occlusive episodes (VOE) and 3 ports were for transfusions after multi-organ failure. A total of 54 port complications occurred (malfunction=29, infection=20, thrombosis=3, difficult port access=1, and intractable pain over port site=1). From the data available, more ports were placed in the left subclavian vein (LSV=20) than right subclavian vein (RSV=4) and right internal jugular (RIJ=2), however rate of overall complications were similar between LSV and RSV - 0.57 complications/1,000 catheter days in LSV and 0.58 complications/1,000 catheter days in RSV. The rate of port associated infection, defined as a positive blood culture drawn from the port, was 0.2 per 1,000 port days. A total of 20 infections identified mostly gram-positive organisms (n=15) predominantly Staphylococcus, compared to gram-negatives (n=3), fungus with Candida albicans (n=1) and a rare acid-fast bacilli infection with Mycobacterium mucogenicum (n=1). The rate of thrombosis, identified radiologically using vascular doppler ultrasound, was 0.03 per 1,000 port days. The rate of premature port removal arising from complications was 0.36 per 1,000 port days. Ports placed for chronic transfusions had a lower rate of removal (0.31 per 1,000 port days) compared to ports placed for poor venous access (0.58 per 1,000 port days) with a ratio of 0.54 which approached statistical significance (p=0.09; CI 0.26-1.21). Conclusion: Ports in pediatric patients with SCD are associated with low rates of thrombosis, infection and malfunction. Ports may be a reasonable alternative for vascular access in patients with SCD - especially in patients who require chronic simple or exchange transfusions and have difficult access. Larger prospective studies will be needed to further assess the scope of use of ports in this population. Disclosures No relevant conflicts of interest to declare.

The Effect Of Microalbuminuria On Duration Of Hospitalization In Adult Sickle Cell Disease Patients With Pain Crisis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4694-4694
Author(s):  
Mohammed Shaik ◽  
Borys Hrinczenko
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Categorical Variables ◽  
Cell Disease ◽  
Exact Test ◽  
Blood Disorder ◽  
Significant Difference ◽  
Pain Crisis

Introduction Sickle cell disease (SCD) is a genetic blood disorder of hemoglobin resulting in severe morbidity and early mortality. The prevalence of microalbuminuria and/or proteinuria in children with SCD varies from 18 to 28% and increases with age. However, the exact prevalence in adults in not known. In the general population, the presence of albuminuria has been shown to be associated with all cause mortality. The urine microalbumin to creatinine ratio (MA) is considered to be an early sign of impending sickle cell nephropathy. We sought to investigate the association of MA with various SCD genotypes (HbSS, HbSC, HbS/β-thalassemia) in our clinic and also the length of stay (LOS) in hospitalized SCD patients with acute pain crisis. Methods Twenty-eight consecutive SCD patients diagnosed in our clinic by hemoglobin electrophoresis were included in our study. The patients (pts) age, gender, hemoglobin electrophoresis, and baseline MA were obtained. Based on their MA level they were divided into two groups, abnormal MA (MA ≥ 30 mg/g creatinine) or normal MA (< 30 mg/g creatinine). Eleven of these 28 patients were eventually hospitalized for a sickle cell related pain crisis. Their MA level was obtained within 24-hours of hospital admission and their hospitalization length of stay (LOS) was also recorded. We analyzed the association between the two groups of patients, those with normal or abnormal MA, with the different genetic variants of SCD in both our clinic and hospitalized pts. Furthermore, for hospitalized pts we also assessed an association of MA with their mean LOS. The Chi-square test/Fisher’s exact test was used for categorical variables and the T-test/Mann-Whitney test was used for numerical variables. Results All twenty-eight patients were African American without significant renal impairment, with 11, 10, and 7 pts with SS, SC and S β-thalassemia (Sβ), respectively. Fifteen of these pts had abnormal MA, 12 pts were female. The median age was 35.5 yrs (range, 19 - 59), median LOS was 3.5 days (range, 2-8). The SS pts had higher abnormal MA levels followed by SC and then Sβ (p=0.03) (Table 1). There was no significant difference in gender between the two groups (p=0.2). SCD pts admitted to the hospital for pain crisis with an abnormal MA within 24-hours of admission had a significantly higher mean LOS when compared to pts with normal MA (p=0.0089) (Table 1). Conclusion Microalbuminuria is more prevalent in the severe genotypes of SCD disease such as SS and SC vs. Sβ pts. Thereby, MA might be a useful biomarker of generalized SCD vasculopathy, in addition to a known marker of progressive nephropathy. Furthermore, MA has a significant impact on length of hospitalization. An abnormal MA obtained within the first 24-hrs of hospitalization of a SCD pt in pain crisis was predictive of prolonged hospital duration. Early and more aggressive supportive care symptom management for those patients might be a reasonable option but requires more study. Further large prospective clinical trials are needed to validate our findings. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Complications of implantable venous access devices in patients with sickle cell disease

2011 ◽  
Vol 87 (2) ◽  
pp. 224-226 ◽  
Author(s):  
Nirmish Shah ◽  
Daniel Landi ◽  
Radhika Shah ◽  
Jennifer Rothman ◽  
Laura M. De Castro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Venous Access ◽  
Cell Disease ◽  
Venous Access Devices

Effect of Sickle Cell Disease Type, Age and Gender On the Prevalence of Chronic Organ Damage in Adults with Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4607-4607
Author(s):  
Raymond U. Osarogiagbon ◽  
Syed N Haider ◽  
Jun Tang
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Brain Mri ◽  
Organ Damage ◽  
Categorical Variables ◽  
Acute Chest Syndrome ◽  
Continuous Variables ◽  
Cell Disease ◽  
End Organ Damage

Abstract Abstract 4607 Introduction The high mortality risk that sickle cell disease (SCD) patients experience from infancy is cumulative through adulthood, largely because of the effect of cumulative end organ damage, which is a more powerful predictor of early mortality than frequency of painful episodes. The latter, though, gets more attention from patients and caregivers. Any vascular territory is susceptible to damage. The most common target organs are the brain, lungs, kidneys, retina and joints. We examined the prevalence of the full spectrum of end organ damage in a cohort upon entry into our adult SCD program and compared the clinical and laboratory characteristics of patients based on age, gender and SCD type. Patients and Methods Retrospective review of prospectively collected data on 118 adults upon entry into our program between February 2005 and October 2008. All patients underwent a standardized battery of tests to evaluate hematological and biochemical parameters at entry. Historical presence of episodes of acute chest syndrome, pneumonia, stroke, avascular necrosis, osteomyelitis, leg ulcers, priapism, and cholecystectomy and hydroxurea therapy was quantified. Pulmonary hypertension (PHT) was defined as a tricuspid regurgitant jet velocity (TRJV) ≥2.5 m/s on Doppler echocardiography; sickle cell nephropathy (SCN) as glomerular filtration rate, (GFR) < 90ml/min. and/or 24-hour protein>300mg and/or urine protein/creatinine ratio>0.3); cerebrovascular disease (CVD) as evidence of previous ischemic and/or hemorrhagic infarct and/or aneurysm formation on brain MRI/MR angiography; and sickle cell retinopathy (SCR) as background to proliferative retinopathy) on fluorescent retinal angiography. Characteristics of patients were evaluated with t-test for continuous variables and chi-square test for the categorical variables. Results The relevant statistically significant correlative variables in these 3 comparisons are shown in the following tables. Conclusions Our study highlights the various differences in the prevalence of sickle cell disease-related end organ damage and morbidity among different age, gender and two major sickle cell disease categories. It shows the significant progression of organ damage with advancing age and the more severe nature of SS/Sβ0 phenotype. Further expansion of this assessment may help identify specific high risk groups that can be targeted as candidates for more intensive preventive interventions. Disclosures: No relevant conflicts of interest to declare.

Demographic and Socio-Economic Features of a Cohort of Adult Sickle Cell Disease Patients.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4616-4616 ◽  
Author(s):  
Syed N Haider ◽  
Jun Tang ◽  
Raymond U. Osarogiagbon
Keyword(s):  
Sickle Cell Disease ◽  
Marital Status ◽  
Sickle Cell ◽  
Health Care Providers ◽  
Catchment Area ◽  
Conflicts Of Interest ◽  
Categorical Variables ◽  
Continuous Variables ◽  
Care Providers ◽  
Cell Disease

Abstract Abstract 4616 Introduction Despite improvements in diagnosis, preventive care and treatment, care of sickle cell disease (SCD) patients remains difficult. The majority are from lower socio-economic strata, poorly educated and from single parent households. The racial divide between patients (who tend to be almost all black) and their health care providers (who tend to be predominantly non black) can adversely affect the quality of care when stereotypes are assumed. We examined the demographic and socio-economic features of patients in our adult sickle cell disease program. Patients and Methods We retrospectively reviewed data on 118 patients that entered our program between February 2005 and October 2008. Our catchment area included parts of rural Mississippi, Tennessee and Arkansas as well as a major urban community. We reviewed the following demographic characteristics: age, gender, patient's marital status, parent's marital status, employment status, highest level of education, health insurance status and referral source. We then contrasted the characteristics of those with SC (which tends to be milder) with SS/Sβ0 sickle cell disease (which is phenotypically more severe). Continuous variables were evaluated using t-test. Chi-square test was used to assess categorical variables. Results The results are depicted in the table. Conclusions We describe the demographic and socio-economic features of a cohort of 118 patients seen in an academic center with a diverse catchment area. In spite of adverse family circumstances, relatively high proportion of the cohort was college educated (32%) or graduated from high school/GED (55%). 61% of the patients are either currently employed or current students. These results are quite contrasting to the general perception about the adult sickle cell disease population. Disclosures: No relevant conflicts of interest to declare.

Prevention of Infection in Adults Receiving Intravenous Antibiotic Treatment via Indwelling Central Venous Access Devices

2019 ◽  
Vol 14 (1) ◽  
pp. 47-49
Author(s):  
Basant K. Puri ◽  
Anne Derham ◽  
Jean A. Monro
Keyword(s):  
Bloodstream Infection ◽  
Antibiotic Treatment ◽  
Central Venous Access ◽  
Case Series ◽  
Bloodstream Infections ◽  
Venous Access ◽  
Host Cells ◽  
Central Venous ◽  
Intravenous Antibiotic ◽  
Venous Access Devices

Background: The use of indwelling Central Venous Access Devices (CVADs) is associated with the development of bloodstream infections. When CVADs are used to administer systemic antibiotics, particularly second- or higher-generation cephalosporins, there is a particular risk of developing Clostridium difficile infection. The overall bloodstream infection rate is estimated to be around 1.74 per 1000 Central Venous Catheter (CVC)-days. Objective: We hypothesised that daily oral administration of the anion-binding resin colestyramine (cholestyramine) would help prevent infections in those receiving intravenous antibiotic treatment via CVADs. Method: A small case series is described of adult patients who received regular intravenous antibiotic treatment (ceftriaxone, daptomycin or vancomycin) for up to 40 weeks via indwelling CVADs; this represented a total of 357 CVC-days. In addition to following well-established strategies to prevent C. difficile infection, during the course of the intravenous antibiotic treatment the patients also received daily oral supplementation with 4 g colestyramine. Results: There were no untoward infectious events. In particular, none of the patients developed any symptoms or signs of C. difficile infection, whereas approximately one case of a bloodstream infection would have been expected. Conclusion: It is suggested that oral colestyramine supplementation may help prevent such infection through its ability to bind C. difficile toxin A (TcdA) and C. difficile toxin B (TcdB); these toxins are able to gain entry into host cells through receptor-mediated endocytosis, while anti-toxin antibody responses to TcdA and TcdB have been shown to induce protection against C. difficile infection sequelae.

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