Sickle cell disease: Experience of a tertiary care center in a nonendemic area

2008 ◽  
Vol 1 (1) ◽  
pp. 1 ◽  
Author(s):  
KC Aggarwal ◽  
PC Goyal ◽  
MS Prasad ◽  
S Saluja ◽  
M Sharma ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Cell Disease ◽  
Disease Experience

Sickle cell disease in pregnancy: 5 years' experience at a tertiary care center

2003 ◽  
Vol 189 (6) ◽  
pp. S150
Author(s):  
Thomas Ivester ◽  
Jessica Ruffin ◽  
Nazinan Kelishadi ◽  
Michael Schneider
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Cell Disease ◽  
In Pregnancy

Experience of an Interdisciplinary Pediatric Pain Service

PEDIATRICS ◽  
1991 ◽  
Vol 88 (6) ◽  
pp. 1226-1232
Author(s):  
Barbara S. Shapiro ◽  
David E. Cohen ◽  
Kenneth W. Covelman ◽  
Carol J. Howe ◽  
Sam M. Scott
Keyword(s):  
Sickle Cell Disease ◽  
Patient Care ◽  
Sickle Cell ◽  
Health Care Professionals ◽  
Tertiary Care ◽  
Direct Patient Care ◽  
Team Approach ◽  
Cell Disease ◽  
Number Of Patients ◽  
Physician Time

This article is a report of our experience with an interdisciplinary pain service in a large tertiary care pediatric hospital. During the first 2 years of operation, we received 869 consultations and referrals from more than 19 hospital divisions. Postoperative pain was the most frequent reason for consultation (56% of patients). Patients with pain related to cancer and sickle cell disease comprised 25% of the consultations. The remaining patients had a wide variety of primary diagnoses and causes of pain. We calculated the time spent by pain service physicians in direct patient care. The majority (63%) of physician time was spent with a small number of patients (17%). Most of these patients had pain that was unrelated to surgery, cancer, or sickle cell disease, and many posed dilemmas in diagnosis and treatment. Physician time was correlated directly to the use of psychologic and physical therapies for the pain, involving multiple team members. This experience supports the demand for an interdisciplinary pain service in a tertiary care children's hospital. A significant amount of physician time is necessary to provide patient care and to maintain a team approach, however, and pediatricians and other health care professionals who aim to implement such services should be cognizant of the time required.

Improvement In Acute Management of Vaso-Occlusive Pain In Pediatric Sickle Cell Disease with Use of a Clinical Pathway

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2660-2660
Author(s):  
Katherine Ender ◽  
Jennifer Freed ◽  
John Babineau ◽  
Mary Tresgallo ◽  
William Schechter ◽  
...  
Keyword(s):  
Pain Management ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Pathway ◽  
Clinical Pathways ◽  
Capture Rate ◽  
Tertiary Care ◽  
P Value ◽  
Time Interval ◽  
Cell Disease

Abstract Abstract 2660 Background: Sickle cell disease causes considerable morbidity and mortality. Three guidelines exist for the management of vaso-occlusive pain, although studies have shown that these guidelines are not well followed and that there is considerable variation in care. Investigations of clinical pathways for pediatric sickle cell pain have not been published to our knowledge, but research in other pediatric conditions has shown that the implementation of clinical pathways can improve medical care. We initiated an investigation of the effects of a pain management pathway for sickle cell vaso-occlusive crisis in the emergency department (ED) with the hypothesis that the introduction of a clinical pathway would improve the efficiency and efficacy of acute pain management. Methods: We conducted a prospective, cohort study from February 2009 to March 2010 in an urban, tertiary care ED. We collected data from patients aged 3–18 years old with sickle cell disease who presented to the ED with vaso-occlusive crisis (VOC) pain. Baseline data was collected for five months prior to the introduction of the clinical pathway, followed by a four-week time interval in which the ED physicians and nurses were in-serviced on the pathway, followed by six months in which data was collected with the clinical pathway in place. Our pathway, which is in checklist format with instructions for triage, monitoring, medication administration, and timing of assessments and interventions, was developed by representatives from the divisions of Pediatric Hematology, Pediatric Emergency Medicine, and Pediatric Pain Medicine, Symptom Management and Palliative Care and based upon current standard-of-care guidelines. Results: Over the eleven month study period, 68 patients were enrolled. Random chart audits revealed a 75% capture rate. Significant improvement was demonstrated in time interval to first analgesic from 74 minutes to 42 minutes (p value 0.02), time interval to first opioid from 94 minutes to 46 minutes (p value < 0.01), and time interval to subsequent assessment of pain score from 110 minutes to 72 minutes (p value 0.02). The percentage of patients who received ketorolac also increased from 57% to 82% (p value 0.03). Change in pain scores was not significantly different, nor was admission rate. Conclusions: Implementation of a pain management pathway for sickle cell VOC led to an improvement in the time interval to administration of first analgesic and time interval to pain re-assessment, bringing these aspects of patient management closer to accepted guidelines. Whether improvement can be made in admission rates, change in pain ratings, and patient satisfaction will require further study. Disclosures: No relevant conflicts of interest to declare.

Effectiveness Of An Analgesia Protocol For The Treatment Of Painful Vaso-Occlusive Crisis Of Sickle Cell Disease Patients In Emergency Room: A Retrospective Cohort Study

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2222-2222
Author(s):  
Veronique Naessens ◽  
Richard Ward ◽  
Kevin H.M. Kuo
Keyword(s):  
Sickle Cell Disease ◽  
Emergency Room ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Care Center ◽  
Median Number ◽  
Cell Disease ◽  
Dose Time ◽  
Chi Squared ◽  
Opiate Administration

Background Painful vaso-occlusive crisis (VOC) is the most frequent complication of sickle cell disease (SCD) and the main reason for interacting with the emergency room (ER). Guidelines highlight the need for rapid opiate delivery (< 30 min.) via specific analgesia protocol in order to achieve rapid pain control and reduce length of stay (LOS). Objectives To determine the rate of utilization of an analgesia protocol for SCD patients presenting with painful VOC to ER. Secondary objectives are to examine the relationship between protocol use, LOS, discharge from ER, time to first opiate delivery, total amount of opiates, and time to readmission. Methods An analgesia protocol, developed according to the BCSH guideline, was deployed in the ER of a major SCD comprehensive care center in Canada since 2009. A retrospective observational study was conducted of all SCD patients who presented to the ER with painful VOC between August 2009 and September 2012. Frequent ER visitor was defined as >3 visits/year. Factors influencing protocol use and discharge from ER were examined via Chi-squared test and multivariable logistic regression. Mann-Whitney U test and multiple regression were used to examine factors related to time to first opiate administration, LOS in ER, total quantity of opiates used, and as well as time to readmission. Results 602 ER visits were included in the analysis, comprising of 116 patients (61 males, 55 females) with 82% HbSS or S/β0. The median number of visits in the observation period was 2 (1 to 90), with 12 patients responsible for 57% of visits. The protocol was used in only 51% of visits, frequent ER attenders were less likely to be treated by the protocol (OR 0.989 per additional visit, p=0.007). Higher pain score on presentation was significantly associated with protocol use (OR 1.194 per 1 point increment, p=0.001), which in turn reduced the time to first opiate administration (69 vs. 108 min., p<0.001). Visits treated by protocol were longer (457 min vs. 385 min. in ER, p<0.001), used more opiates (95 vs. 50 mg PO morphine equi-analgesic dose, p<0.001) and patients were less likely to be discharged home (OR 0.78, p=0.003), though it prolonged the time to next ER admission (22 vs. 15 days, p=0.008). The length of inpatient admission was not affected by prior use of the Protocol in ER. Conclusion In this, the largest single-center report of ER sickle cell care, the rate of protocol use was moderate and although it resulted in faster delivery of first opiate dose, time to delivery did not reach the recommended target of 30 minutes. Protocol use did not positively influence any other outcomes. The protocol was preferentially used in patients who attends the ER infrequently. Further refinement of the protocol may lead to improvements in utilization by ER staff, leading to shorter time to first analgesia administration. This in turn may result in the protocol having a greater impact on patient outcomes in ER. 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.

Anemia and Celiac Disease-Experience At Tertiary Care Center of Northern India

2016 ◽  
Vol 2 (1) ◽  
pp. 3-8 ◽  
Author(s):  
Parveen Malhotra ◽  
◽  
Naveen Malhotra ◽  
Vani Malhotra ◽  
Pansi Pansi ◽  
...  
Keyword(s):  
Celiac Disease ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Northern India ◽  
Disease Experience

scholarly journals Should Women with Sickle Cell Disease be Administered Prophylactic Transfusions to Avoid Complications during Pregnancy?

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3679-3679
Author(s):  
Patricia Palcu ◽  
Kevin H. M. Kuo ◽  
Richard Ward ◽  
Nusrat Zaffar ◽  
Ann Kinga Malinowski ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Outcomes ◽  
Tertiary Care ◽  
Reproductive Age ◽  
Cell Disease ◽  
Fetal Outcomes ◽  
Hemoglobin F ◽  
Increased Risk ◽  
Rbc Transfusion

Abstract BACKGROUND: Increasing numbers of women with sickle cell disease (SCD) are reaching reproductive age. There is an array of possible pregnancy outcomes that women with SCD may experience. Many have described adverse maternal and fetal outcomes in pregnant patients with SCD including increased risk of pre-eclampsia, infections, painful vaso-occlusive crises as well as increased risk of intrauterine growth restriction, pre-term delivery and pregnancy loss. Not all women with SCD have complicated pregnancies, however. Limited data are available to categorize the proportion of patients who have uncomplicated pregnancies and protective characteristics for good pregnancy outcomes. Identifying these features potentially informs practice and reduces exposure to blood products as red blood cell (RBC) transfusion is associated with an increased risk of alloimmunization, particularly in pregnancy. OBJECTIVE: To determine the proportion and characteristics of pregnant patients with SCD who have uncomplicated pregnancies. METHODS: We retrospectively reviewed medical records of pregnant women with SCD from 1980 to 2018 at Mount Sinai Hospital, a university affiliated tertiary care centre in Toronto, Canada. We defined a pregnancy to be uncomplicated if the pregnancy was not associated with a hospitalization, RBC transfusion was not administered peripartum and/or the delivery was at term (37 or more weeks gestation). RESULTS: A total of 179 pregnancies were included (Figure 1). Preliminary results show that 57% of pregnancies had hemoglobin SS disease. The median maternal age was 27 years (range 16-43 years) (n=179). Median hemoglobin concentration, platelet count and leukocyte count were 91g/L (n=141), 304 x 10(9)/L (n=140) and 11 x 10(9)/L (n=140), respectively. Hospital admission was required in 61% of pregnancies (n=171) and RBC transfusion in 49% of pregnancies (n=173). The median hemoglobin F level was 0.05 (range 0.01-0.87). Nineteen percent had coexisting alpha gene deletion (s). The median birth weight was 2863 grams (n=155). Analysis is ongoing to determine characteristics associated with the pre-defined pregnancy outcomes. CONCLUSIONS: Women with SCD frequently have adverse maternal and fetal outcomes with pregnancy. However, 39% of pregnancies with SCD in this study did not require hospitalization and 51% did not require RBC transfusion. Additional analysis will attempt to identify protective features so as not to expose all patients to RBC transfusion unless it is required. These initial results suggest that risk scores should be developed for pregnant patients with sickle cell disease to identify who may benefit from RBC transfusion. Disclosures No relevant conflicts of interest to declare.

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