Care Practices in Gaucher Disease: Results of a French Study.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4441-4441
Author(s):  
Belmatoug Nadia ◽  
Nathalie Guffon ◽  
Jérôme Stirnemann ◽  
Christine Serratrice ◽  
Alain Robert ◽  
...  
Keyword(s):  
Gaucher Disease ◽  
Central Venous Access ◽  
Venous Access ◽  
Infusion Time ◽  
Mineral Density ◽  
Central Venous ◽  
Significant Difference ◽  
The Mean ◽  
Care Practices ◽  
At Home

Abstract Abstract 4441 Introduction Gaucher Disease (GD) management is well defined but little is known about the management of patients in routine practice. To describe it, this multicentre retrospective survey was proposed to all French Hospital Departments in whom Gaucher patients are monitored. Demographic and clinical data were collected, and therapeutic regimens were detailed. Results In 2008, 118 patients were enrolled in 54 centres, including 111 type 1 (GD1) and 7 type 3 (GD3). 90% of patients were adults at inclusion. At time of diagnosis 40% of GPS patients were less than 16 years. Glucocerebrosidase activity assay and bone marrow aspiration were part of the diagnosis in 80% and 51% of patients, respectively. A family survey has been performed in all children and overall in 85 % of patients, with positive results in 44% of cases. 54% of patients are managed by the physician who diagnosed the GD and the mean annual number of visits at hospital is 10 and 4 for children and adults, respectively. In patients diagnosed after the age of 16 years, 20.7% were splenectomised at time of inclusion. On average, hemogram and chitotriosidase are assessed twice a year, as bone mineral density and skeletal MRI are performed every 2 years. Ninety-six patients are treated with imiglucerase, 3 patients with miglustat and 2 patients with a combination of both. Adult patients were treated on average for 87.6 months versus 53 months for children. The mean Imiglucerase dose is 54 U/kg by infusion for GD1 with no significant difference regarding infusions frequencies, and 96 U/kg for GD3 and The treatment is infused every two weeks in 74% of the patients, and every 3 weeks in 18% of them, using a gravity infusion in 57 % of the cases. Only 7.8% of the GPS population is infused using a central venous access with similar rates in adults and children. Infusions are administered at hospital in 60% of cases and at patient's home in 40% of cases. A central venous access is used in 11% of patients who are treated at home. Infusion time is less than 2 hours in 55% of patients and is slightly longer at home compared to hospital. Observance is good or excellent in more than 95% of cases. Conclusion This is the first large national survey about Care Practices in GD patients. The clinical and biological follow-up is globally in line with French Guidelines. Treatment with imiglucerase is administered on average at the recommended dose and frequency, but with an infusion time often longer than that recommended. Patients should be more frequently treated at home, and thus quality of life studies and encouragement of home infusions organisation should be performed in France. Disclosures: Off Label Use: Cerezyme (imiglucerase) : enzymatic replacement therapy for Gaucher disease patients. The aim of imiglucerase is to replace glucocerebrosidase deficiency; The dose of treatment initiation is 60u/kg/2 weeks.. Bruno:Genzyme: Employment.

scholarly journals Central Venous Access Device (CVAD) Use and Complications in Sickle Cell Disease Patients from Medicaid and Commercially-Insured U.S. Populations

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2057-2057
Author(s):  
Nancy Maserejian ◽  
Cortney Hayflinger ◽  
Susan Eaton ◽  
Catherine Madigan ◽  
William E. Hobbs
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Central Venous Access ◽  
Venous Access ◽  
Treatment Burden ◽  
Cell Disease ◽  
Central Venous ◽  
Employment Equity ◽  
Equity Ownership ◽  
The Mean

Abstract Central venous access devices (CVADs), such as tunneled central venous catheters (tCVADs) and peripherally inserted central catheters (PICCs), help provide essential care for some patients with sickle cell disease (SCD). CVADs facilitate administration of multiple intravenous (IV) medications and blood products, as well as blood draws for laboratory analysis. Understanding CVAD use and complications is particularly relevant for SCD patients because of their high risk of having insufficient peripheral IV access. Prior studies describing CVAD use and complications in SCD patients were limited by small sample sizes, typically including 15-20 SCD patients in a single treatment center. The resulting estimates for CVAD use and complications in SCD patients vary widely, are insufficient for development of evidence-based guidelines, and may not be representative of the treatment burden in the broader SCD population. The purpose of this study was to describe the frequency of CVAD use and CVAD-associated complications among SCD patients in a large US population sample. We used data from two large U.S. insurance claims databases from Truven Health MarketScan® Research to examine both Medicaid-insured and commercially-insured SCD patients. From January 2009 through December 2013, these databases encompassed over 14 million Medicaid-insured and over 116 million commercially-insured patients. SCD patients were defined as patients with at least two International Classification of Disease-9 (ICD-9) diagnosis codes for SCD (282.41-42, 282.6x) on separate dates in excess of sickle cell trait codes, or one ICD-9 code for an inpatient (emergency department or hospitalization) visit with sickle cell crisis. CVAD insertions were identified using relevant procedure codes for tCVADs or PICCs. We conducted two sets of analyses for each database: (1) per patient among those with at least one CVAD insertion, and (2) per CVAD insertion. We conducted descriptive analyses on the frequency of CVAD-related procedures (e.g., repair, replacement, removal of obstructive material, repositioning), complications, and infections, thromboses, or phlebitis. A total 17,119 Medicaid-insured SCD patients and 21,342 commercially-insured SCD patients were observed for an average of 3-4 years, during which 1,945 (11.4%) and 1,316 (6.2%) patients, respectively, had at least one CVAD insertion. Most SCD patients (80%) were aged >18 y at time of first CVAD insertion; 18% of adult Medicaid SCD patients had at least one CVAD inserted. The mean number of CVAD insertions per patient was 3.1 (Medicaid) and 2.4 (commercially-insured). In the per CVAD analysis, complication claims were frequent, including infection (31-37%), thrombosis (4-5%), and phlebitis/thrombophlebitis (12-15%). The mean time to removal of CVADs (duration) was 31-34 days (PICC lines) and 102 days (tCVADs). In the per patient analysis, 54.3% had infection claims, 24.3% had thrombosis claims, and 10.2% had phlebitis/thrombophlebitis claims in Medicaid (see Table and Figure; additional results to be provided, also stratified by age). Both tCVADs and PICCs were commonly used in SCD patients, particularly adults, with high occurrence of infection, thrombosis and phlebitis/thrombophlebitis, as well as repeated CVAD insertions. Determinants of CVAD use and complications warrant further investigation to inform practice standards. These findings from a large observational study indicate that device-related risks of administering IV treatments are limitations of IV treatment options and may add to SCD treatment burden. Table 1. Medicaid-Insured Commercially-Insured Any CVAD (Overall) PICC Only tCVAD Only Both PICC and tCVAD Any CVAD (Overall) PICC Only tCVAD Only Both PICC and tCVAD N (%) 1,945 681 (35.0%) 893 (45.9%) 371 (19.1%) 1,316 450 (34.2%) 664 (50.5%) 202 (15.3%) Age, mean (sd) y 30.0 (16.3) 34.2 (14.9) 26.9 (17.5) 30.1 (13.6) 32.6 (17.1) 34.1 (15.8) 31.3 (18.3) 33.2 (15.9) CVAD insertions per patient, mean (sd) 3.1 (3.9) 3.1 (3.9) 1.8 (1.3) 6.2 (5.8) 2.4 (2.3) 2.5 (2.6) 1.6 (1.0) 4.5 (2.9) CVAD insertions total 6,107 3,651 2,456 n/a 3,082 1,636 1,446 n/a Duration per CVAD, median days 44 31 102 n/a 53 34 102 n/a CVAD complications, % patients with CVAD Complication, general 28.8% 13.2% 31.8% 50.1% 22.3% 9.1% 25.9% 39.6% Removal of obstructive material 11.2% 3.8% 15.3% 14.6% 9.0% 2.4% 11.4% 15.3% Replacement 8.3% 3.7% 10.2% 12.4% 6.5% 3.8% 6.5% 12.9% Figure 1. Figure 1. Disclosures Maserejian: Biogen: Employment, Equity Ownership. Hayflinger:Biogen: Consultancy, Employment. Eaton:Biogen: Employment, Equity Ownership. Madigan:Biogen: Employment, Equity Ownership. Hobbs:Biogen: Employment, Equity Ownership.

scholarly journals Risk factors for unsuccessful removal of central venous access ports implanted in the forearm of adult oncologic patients

2021 ◽  
Author(s):  
Mitsuhiro Kinoshita ◽  
Shoichiro Takao ◽  
Junichiro Hiraoka ◽  
Katsuya Takechi ◽  
Yoko Akagawa ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Risk Factor ◽  
Independent Risk Factor ◽  
Central Venous Access ◽  
Univariate Analysis ◽  
Venous Access ◽  
Central Venous ◽  
Significant Difference ◽  
Oncologic Patients

Abstract Purpose To evaluate the risk factors for unsuccessful removal of a central venous access port (CV port) implanted in the forearm of adult oncologic patients. Materials and methods This study included 97 adult oncologic patients (51 males, 46 females; age range, 30–88 years; mean age, 63.7 years) in whom removal of a CV port implanted in the forearm was attempted at our hospital between January 2015 and May 2021. Gender, age at removal, body mass index, and diagnosis were examined as patient characteristics; and indwelling period, indwelling side, and indication for removal were examined as factors associated with removal of a CV port. These variables were compared between successful and unsuccessful cases using univariate analysis. Then, multivariate analysis was performed to identify independent risk factors for unsuccessful removal of a CV port using variables with a significant difference in the univariate analysis. A receiver-operating characteristics (ROC) curve was drawn for significant risk factors in the multivariate analysis and the Youden index was used to determine the optimum cut-off value for predicting unsuccessful removal of a CV port. Results Removal of CV ports was successful in 79 cases (81.4%), but unsuccessful in 18 cases (18.6%) due to fixation of the catheter to the vessel wall. Multivariate logistic regression analysis showed that the indwelling period (odds ratio 1.048; 95% confidence interval 1.026–1.070; P < 0.0001) was a significant independent risk factor for unsuccessful removal of a CV port. ROC analysis showed that the cut-off value for successful removal was 41 months, and 54% of cases with an indwelling period > 60 months had unsuccessful removal. Conclusion The indwelling period is an independent risk factor for unsuccessful removal of a CV port implanted in the forearm of adult oncologic patients, with a cut-off of 41 months.

scholarly journals Supraclavicular versus infraclavicular approach in inserting totally implantable central venous access for cancer therapy: A comparative retrospective study

PLoS ONE ◽  
2020 ◽  
Vol 15 (11) ◽  
pp. e0242727
Author(s):  
Amine Souadka ◽  
Hajar Essangri ◽  
Imad Boualaoui ◽  
Abdelilah Ghannam ◽  
Amine Benkabbou ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Intraoperative Complications ◽  
Central Venous Access ◽  
Venous Access ◽  
Demographic Characteristics ◽  
Success Rates ◽  
Central Venous ◽  
Chemotherapy Administration ◽  
Significant Difference ◽  
Ultrasonographic Guidance

Introduction The insertion of an implantable central venous access is performed according to a variety of approaches which allow the access to the subclavian vein, yet the supraclavicular technique has been underused and never compared to the other methods. The aim of this study was to testify on the efficacy and safety of the subclavian puncture without ultrasound guidance « Yoffa » in comparison with the classical infraclavicular approach (ICA). Material and methods This is a retrospective study with prospective data collection on patients followed at the national oncology institute for cancer, in the period extending from May 1st 2017 to August 31st 2017. All patients had a totally implantable central venous access device inserted by the same surgeon AS for chemotherapy administration and demographic characteristics, as well as procedure details were examined. The primary outcomes were the intraoperative complications, while the secondary outcomes represented immediate postoperative and mid-term complications (at 15 months of follow up). Outcomes were compared between techniques by means of non parametric tests and the Fischer test. Results Our study included 135 patients with 70 patients undergoing the subclavian technique, while 65 were subject to the infraclavicular approach. Both groups had no statistically significant demographic characteristics. The number of vein puncture attempts exceeding once, the accidental artery puncture and operative time were more significant in the ICA group; (39,6 vs 17,6 p = 0,01) (9.2% vs 0; p = 0,01) and (27± 13 vs 23± 8min, p = 0.045) respectively. There was no statistically significant difference in the immediate and midterm complication rate between the two methods 1(1,4) vs 2 (3) p = 0.5. Conclusion In case of unavailability of ultrasonographic guidance, the use of the supra-clavicular landmarks approach is linked to higher success rates and less arterial punctures, thereby proving to be a safe and reliable approach.

Risk of Thrombosis with Different Approaches to Central Venous Access During Acquired Asparaginase Related Antithrombin Deficiency in Children with Leukemia.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2241-2241
Author(s):  
Susanna Ranta ◽  
Mats Heyman ◽  
Kirsi Jahnukainen ◽  
Mervi Taskinen ◽  
Ulla M. Saarinen-Pihkala ◽  
...  
Keyword(s):  
Central Venous Access ◽  
Thrombotic Event ◽  
Intervention Group ◽  
Venous Access ◽  
Control Group ◽  
Central Venous ◽  
Children's Hospital ◽  
On Demand ◽  
Significant Difference ◽  
Children’S Hospital

Abstract Abstract 2241 Background Children with acute lymphoblastic leukemia (ALL) have several risk factors for thrombosis including asparaginase related acquired antithrombin (AT) deficiency and central venous catheters (CVCs). Both external tunneled and internal CVCs (tunneled, fully implantable devices known as ports) are used to provide reliable venous access. After introduction of a new ALL protocol with prolonged continuous asparaginase treatment aiming at approximately 30 weeks depletion of asparagine more symptomatic thromboses than expected were observed at the Children's Hospital, Helsinki University Central Hospital, Finland. To ensure asparaginase treatment and prevent thromboses an on-demand AT substitution regimen was adopted. Previous studies have described AT substitution during ALL induction; this is the first description and evaluation of on-demand AT substitution over a longer period of time in children. Aim The aim of this study is to describe the prolonged on-demand AT substitution practice in Helsinki during depletion of asparagine from the blood with PEG-ASP and to compare experiences from two Nordic pediatric oncology centers (Children's Hospital in Helsinki and the Astrid Lindgren Children's Hospital Karolinska University Hospital, in Stockholm, Sweden) with the same leukemia protocol but different approaches to central venous access. Procedure All Finnish children with ALL and external tunneled CVCs diagnosed at Children's Hospital in Helsinki between May 2008 and November 2011 (n=38) were compared with Swedish children with ALL and internal CVCs treated concurrently with the same ALL protocol (n=39). Children treated in Helsinki according to the standard or intermediate risk NOPHO-ALL 2008 protocol after initiation of the AT substitution regiment received AT concentrate when their AT level decreased below 55% (the intervention group, n=25). The thrombosis rate and laboratory parameters were compared with children treated earlier in the same unit (the control group, n=10). Results In Helsinki, 2/10 (20%) children in the control group had 3 symptomatic thromboses and 2/25 (8%) children in the intervention group with on-demand AT substitution had one symptomatic thrombotic event each. Both thromboses in the intervention group were associated with concomitant steroid treatment. In the intervention group 14/25 (56%) received AT concentrate (median number of infusions 3, range 1–17). Altogether, 4/38 Finnish patients with external CVCs and 0/39 Swedish patients with internal CVCs had thromboses (Mann Whitney U test, p=0.039) in spite of similar exposure to asparaginase and low antithrombin. A significant difference was also observed in the use of tissue plasminogen activator (tPA): 28/38 Swedish patients had received tPA at least once to restore dysfunctional CVCs (usually loss of the ability to withdraw blood) compared to 2/38 Finnish patients (Mann Whitney U test, p≤0.001). Conclusions Most children are exposed to low AT levels during ASP treatment predisposing to thrombosis especially with concomitant steroids. Larger studies are needed to evaluate the benefit of prophylactic antithrombin treatment. Internal CVCs and tPA may be beneficial in preventing symptomatic thrombosis during leukemia treatment. Disclosures: No relevant conflicts of interest to declare.

Activated Partial Thromboplastin Times From Venipuncture Versus Central Venous Catheter Specimens in Adults Receiving Continuous Heparin Infusions

2014 ◽  
Vol 34 (5) ◽  
pp. 27-41 ◽  
Author(s):  
Mary Sue Dailey ◽  
Barbara Berger ◽  
Fortunata Dabu
Keyword(s):  
Central Venous Access ◽  
Venous Catheter ◽  
Venous Access ◽  
Central Venous ◽  
Central Venous Access Device ◽  
Heparin Infusion ◽  
Specimen Collection ◽  
The Mean ◽  
The Difference ◽  
Venous Access Device

BackgroundIn patients receiving heparin infusions, variations in specimen collection technique may contribute to inaccurate measurements of activated partial thromboplastin time (aPTT).ObjectivesTo determine if there is a difference in aPTT results between specimens collected from a central venous access device (CVAD) compared with venipuncture in patients receiving heparin infusions.MethodsSimultaneous blood samples (CVAD vs venipuncture) from 66 patients receiving continuous heparin infusions were compared.ResultsThe mean aPTT difference (peripheral aPTT minus CVAD aPTT) was −7.3 seconds (P=.07). Neither length of time heparin was turned off (P=.18) nor waste volume (P=.32) was significantly associated with the difference in aPTT. The median aPTT difference when the CVAD specimen was obtained from the heparin infusion port was −20.5 seconds, compared with −0.1, −3.0, and −0.2 seconds for specimens from a port proximal to, distal to, or coterminal with the heparin infusion, respectively (P=.008).ConclusionsUse of this protocol resulted in similar aPTTs when the CVAD specimen was not obtained from the heparin infusion port. However, obtaining the specimen from the heparin infusion port resulted in significantly higher aPTT values.

scholarly journals Performance of 3 mL versus 5 mL Discarded Volume for Blood Sampling from Central Venous Access Device

2021 ◽  
Author(s):  
HT Lalthanthuami ◽  
MJ Kumari ◽  
R Venkateswaran ◽  
PR Lakshmi ◽  
Lakshmi Ramamoorthy
Keyword(s):  
Critical Care ◽  
Blood Loss ◽  
Central Venous Access ◽  
Intraclass Correlation ◽  
Venous Access ◽  
Blood Sampling ◽  
Central Venous ◽  
Blood Samples ◽  
Serum Parameters ◽  
Significant Difference

Abstract Background Central venous access devices (CVAD) are an essential part of safe practices in critical care, which enable effective venous access and help in avoiding repeated venipuncture. Discard method is widely practiced for blood sampling. A single occasion of blood sampling may cause minimal blood loss; however, the cumulative volume sequential sampling may become clinically significant. The study aims to reduce diagnostic blood loss, ensuring that the subsequent blood sample is not diluted or contaminated by residual intraluminal fluid. Patients and Methods Within-subjects comparative design was adopted for 64 adult patients in the medical intensive care unit of a tertiary hospital. Two blood samples, using 3 mL and 5 mL discarded volume methods, were collected from each patient. Six serum parameters were measured on each of the paired samples and compared. Statistical Analysis Used Paired t-test and Wilcoxon signed rank test were used for comparing the two methods. Bland–Altman plot analysis and intraclass correlation were used for clinically meaningful analysis. Results When tested for fixed bias, there is no statistically significant difference between the methods. Potassium and creatinine levels showed significant proportional bias. The agreement limits of sodium, potassium, creatinine, and direct bilirubin were outside the clinically accepted interval, but the proportion of samples outside these intervals was less than 10%. All serum parameters showed excellent reliability, except for sodium which demonstrated good reliability. Conclusions The practice of discarding 3 mL of blood for discard method is suggested, instead of the standard 5 mL to reduce iatrogenic blood loss. Thus, nurses in critical care are uniquely positioned to limit the diagnostic blood loss while obtaining blood samples.

Successful Long-Term Central Venous Access

1998 ◽  
Vol 2 (1) ◽  
pp. 38-40
Author(s):  
Franco Tesio ◽  
Hamurabi De Baz ◽  
Giacomo Panarello
Keyword(s):  
Central Venous Access ◽  
Venous Access ◽  
Central Venous
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