Transfusion practices for patients with sickle cell disease at a major academic medical center

Background: Case management (CM) and community health workers (CHWs) are two evidence-based health management strategies that can help reduce health risks, reduce readmission rates, and improve patient-provider relationships, all of which may be suboptimal in adults with sickle cell disease (SCD). In Fiscal Year (FY) 2016, the actual average vs. expected average length of stay (ALOS) among SCD adults at Virginia Commonwealth University Medical Center (VCU), an urban academic medical center, was 6.7 days vs 4.2 days. The 30-day readmission rate was 33.7%. We have previously presented results of a year-long pilot CM and CHW intervention for five of VCU's highest adult SCD utilizers, measured by calendar year (CY) 2015 VCU charges (Sop, et al., Blood 2017). Comparing CYs 2015 and 2016, there were numeric improvements in inpatient discharges, 30-day readmissions, 3-Day Emergency Department (ED) returns, ED discharges, outpatient visits, inpatient days, and total VCU charges. Herein, we show results of an extension of the pilot to evaluate responsivity to change over time: whether these improvements were lost among these same patients in the subsequent year (CY 2017) with diminished CM due to loss of funding, and whether they recurred in CY 2018, when CM was more robust as a result of new, more stable funding. Methods: Using the VCU charge and utilization database, we compared the above utilization variables for the five highest CY 2015 utilizers in CYs 2018, 2017, 2016 and 2015. The five original patients included 2 males and 3 females aged between 25-31 years old. One of the females passed away in 2016. Results: There was a trend toward numerically lower utilization during the two CM and CHW years (2016 and 2018), compared to the two diminished CM years (2015 and 2017). Inpatient discharges, inpatient days, and total charges see-sawed according to the intensity of intervention, going down during the year of the initial intervention, rising during the year after intervention, and going down again with resumption of intervention. However, there were notable exceptions: 30-day re-admissions were the lowest in 2017 a diminished intervention year; 3-day ED returns were flat throughout; ED discharges declined throughout, and; outpatient visits rose throughout. Conclusions: These pilot results must be interpreted with caution, since the sample was very small, and interventions were not standardized or randomized, so that secondary trends could be excluded. We found that, after diminution in 2017 of an initial CM and CHW intervention in 2016, several of the initial 2015-to-2016 improvements in academic medical center utilization for five high-utilizing adult SCD patients deteriorated. Subsequently, these improvements recurred when CM and CHWs were reapplied in 2018. We also found that other utilization improvements were sustained despite diminution or resumption of the intervention. This pilot result lays the groundwork for larger, more rigorous CM and CHW Table Disclosures Smith: Novartis: Consultancy, Honoraria. Lipato:Novartis: Honoraria.

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Diagnosis patterns of sickle cell disease in Ghana: a secondary analysis

BMC Public Health ◽

10.1186/s12889-021-11794-6 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Alexandra M. Sims ◽

Kwaku Osei Bonsu ◽

Rebekah Urbonya ◽

Fatimah Farooq ◽

Fitz Tavernier ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Screening Program ◽

Medical Center ◽

Academic Medical Center ◽

Sub Saharan Africa ◽

Cell Disease ◽

Mortality And Morbidity ◽

Pain Crisis ◽

First Pain

Abstract Background Despite having the highest prevalence of sickle cell disease (SCD) in the world, no country in Sub-Saharan Africa has a universal screening program for the disease. We sought to capture the diagnosis patterns of SCD (age at SCD diagnosis, method of SCD diagnosis, and age of first pain crisis) in Accra, Ghana. Methods We administered an in-person, voluntary survey to parents of offspring with SCD between 2009 and 2013 in Accra as a part of a larger study and conducted a secondary data analysis to determine diagnosis patterns. This was conducted at a single site: a large academic medical center in the region. Univariate analyses were performed on diagnosis patterns; bivariate analyses were conducted to determine whether patterns differed by participant’s age (children: those < 18 years old whose parents completed a survey about them, compared to adults: those > = 18 years old whose parents completed a survey about them), or their disease severity based on SCD genotype. Pearson’s chi-squared were calculated. Results Data was collected on 354 unique participants from parents. Few were diagnosed via SCD testing in the newborn period. Only 44% were diagnosed with SCD by age four; 46% had experienced a pain crisis by the same age. Most (66%) were diagnosed during pain crisis, either in acute (49%) or primary care (17%) settings. Children were diagnosed with SCD at an earlier age (74% by four years old); among the adults, parents reflected that 30% were diagnosed by four years old (p < 0.001). Half with severe forms of SCD were diagnosed by age four, compared to 31% with mild forms of the disease (p = 0.009). Conclusions The lack of a robust newborn screening program for SCD in Accra, Ghana, leaves children at risk for disease complications and death. People in our sample were diagnosed with SCD in the acute care setting, and in their toddler or school-age years or thereafter, meaning they are likely being excluded from important preventive care. Understanding current SCD diagnosis patterns in the region can inform efforts to improve the timeliness of SCD diagnosis, and improve the mortality and morbidity caused by the disease in this high prevalence population.

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Implementation of a Community Hospital-Based Fast Track Pathway for the Treatment of Acute Pain Episodes in Adults with Sickle Cell Disease

Blood ◽

10.1182/blood.v124.21.4854.4854 ◽

2014 ◽

Vol 124 (21) ◽

pp. 4854-4854 ◽

Cited By ~ 1

Author(s):

Richard Lottenberg ◽

Robert Krywicki ◽

Gurinder Doad ◽

Witemba Kabange ◽

Monisola Modupe ◽

...

Keyword(s):

Sickle Cell ◽

Fast Track ◽

Community Hospital ◽

Medical Center ◽

Academic Medical Center ◽

Cell Disease ◽

Observation Unit ◽

Academic Medical ◽

The Mean

Abstract Background: A growing number of adult patients with sickle cell disease (SCD) receive care in a community setting and often lack access to physicians with sickle cell expertise. To address this healthcare disparity we are testing a co-management medical home model with Hematology/Oncology (H/O) and Family Medicine (FM) physicians to facilitate evidence-based acute and chronic care. An Emergency Department (ED) Observation Unit based pathway for treatment of sickle cell pain developed at an academic medical center with a Comprehensive Sickle Cell Center (CSCC) has been adopted and modified to fit the needs of a community multi-specialty hospital with an unopposed FM residency program. The hospital serves a large sickle cell population in a predominantly rural setting with the closest CSCC 180 miles away. Methods: Pathway development was facilitated by having a formal meeting for the community hospital physicians and staff at the academic medical center and sickle cell experts providing ongoing on site consultation at the community hospital. Protocols for the community hospital were produced with input from physicians, nurses, advanced practice providers, and support services at multiple meetings. Adult patients with SCD presenting to the ED with pain are triaged at Emergency Severity Index Level 2 for evaluation by the ED physician. The ED protocol uses specific criteria to identify patients with uncomplicated pain. Patients presenting with abnormal vital signs (other than mild tachycardia), fever, pregnancy, or apparent other sickle cell-related complications are excluded. Patients qualifying for the pathway are directly admitted to the SCD unit (a hospital room with 4 infusion chairs on the H/O floor exclusively designated for care of sickle cell patients). Following intake evaluation by the nurse, a clinician is notified to evaluate the patient and provide orders for intravenous fluids and opioid patient controlled analgesia (PCA) which is administered according to hospital guidelines. PCA by the subcutaneous route is used if intravenous access is not readily available. A CBC is obtained whereas other laboratory testing and imaging studies are ordered based on clinical indications. H/O physicians and nurse practitioners cover the unit weekdays 8:00am-5:00pm and FM residents cover nights and weekends with back up by the on call H/O physician. Patients can be treated in the SCD unit up to 23 hours. For patients discharged home a follow up phone call by an H/O nurse will be placed within 3 days and an outpatient clinic appointment is scheduled to be within 7 days. Monthly quality assurance meetings are attended by H/O, FM, and ED physicians as well as nursing, pharmacy and administrative staff from the ED and H/O inpatient service to review process issues and patient outcomes. Consultation is provided by academic physicians with sickle cell expertise (H/O and ED) who attend each meeting in person or by conference call. Results: From March 5-June 30, 2014, 67 patients accounted for 271 visits to the SCD unit. The mean time in the unit was 13.6 hours. The mean pain score on admission was 8.7/10 and reduced to 4.9 upon discharge. PCA drug, pump setting, and dosage are recorded to be used for future visits. Over the 4 months 91.1% of the patients were discharged home from the unit. Six patients accounted for 31% (84) of the visits with only 4 hospital admissions. Conclusions: A fast track pathway for the treatment of acute sickle cell pain coordinated between ED, H/O, and FM physicians has been implemented at a community hospital using an Observation Unit based treatment program. During the entire initial experience the majority of patients have been discharged home with adequate pain relief. In the future the impact of the program will be evaluated including effect on frequency of hospitalizations, outpatient follow up, patient satisfaction, and cost effectiveness. The pathway can be adapted to other community hospital settings where sickle cell expertise is not locally available. Disclosures Kutlar: NIH/NIMHD: Research Funding.

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Clinical Transformation in Care for Patients With Sickle Cell Disease at an Urban Academic Medical Center

American Journal of Medical Quality ◽

10.1177/1062860619873402 ◽

2019 ◽

Vol 35 (3) ◽

pp. 236-241

Author(s):

Sanaa Rizk ◽

David Axelrod ◽

Gaye Riddick-Burden ◽

Elisabeth Congdon-Martin ◽

Steven McKenzie ◽

...

Keyword(s):

Sickle Cell Disease ◽

Case Studies ◽

Sickle Cell ◽

Academic Medical Center ◽

Thomas Jefferson ◽

Cell Disease ◽

Observation Unit ◽

Quantitative Analyses ◽

Clinical Transformation ◽

The Impact

This article demonstrates effects on utilization of a clinical transformation: changing locus of care from a dedicated sickle cell day unit to an approach that “fast-tracks” patients through the emergency department (ED) into an observation unit with 24/7 access. Retrospective quantitative analyses of claims and Epic electronic medical record data for patients with sickle cell disease treated at Thomas Jefferson University (inpatient and ED) assessed effects of the clinical transformation. Additionally, case studies were conducted to confirm and deepen the quantitative analyses. This study was approved by the Thomas Jefferson University Institutional Review Board. The quantitative analyses show significant decreases in ED and inpatient utilization following the transformation. These effects likely were facilitated by increased observation stays. This study demonstrated the impact on utilization of transformation in care (from dedicated day unit to an approach that fast-tracks patients into an observation unit). Additional case studies support the quantitative findings.

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Timing of the Initiation of Hydroxyurea and Hematologic Outcomes in Patients with Sickle Cell Disease (SCD)

Blood ◽

10.1182/blood.v120.21.1004.1004 ◽

2012 ◽

Vol 120 (21) ◽

pp. 1004-1004

Author(s):

Shaina Willen ◽

Nirmish Shah ◽

Courtney Thornburg ◽

Jennifer Rothman

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Significant Relationship ◽

Medical Center ◽

Fetal Hemoglobin ◽

Clinical Severity ◽

Cell Disease ◽

Younger Age ◽

The Mean

Abstract Abstract 1004 Hydroxyurea (HU) is approved for use in adults with Sickle Cell Disease (SCD) and increases the production of fetal hemoglobin (HbF). Increased HbF is associated with decreased clinical severity in adults and children with SCD, such as decreased numbers of vaso-occlusive events, transfusions, and hospitalizations. Higher HbF at initiation of HU is predictive of HbF response, but association between age of hydroxyurea initiation and HbF response has not been investigated. We hypothesize that starting hydroxyurea at an early age may improve hematological and clinical response. In order to determine if younger age at hydroxyurea initiation affects the percentage of HbF achieved with hydroxyurea, we conducted a retrospective cohort study. We identified subjects enrolled in the Duke University Medical Center Comprehensive Sickle Cell program who initiated hydroxyurea when they were less than 17.99 years of age and were prescribed hydroxyurea for at least six months. The following data were abstracted from the medical record between December 1996 and April 2011: age, hemoglobin, percentage HbF, and mean corpuscular volume (MCV) at start of HU and at maximum tolerated dose (MTD) of HU therapy. The correlation coefficient and p-values for various parameters were calculated. Seventy-three patients (41 males and 32 females) were included in the analysis. The mean age at hydroxyurea initiation was 5.5 years (1.2–14.1). The mean hydroxyurea dose at MTD was 28.6 ± 3.2 mg/kg/day. At initiation, the mean hemoglobin was 8.2 ± 1.2 g/dL, the mean MCV was 83±7.4 fl and mean HbF was 10 ± 5.7%. At MTD, the mean hemoglobin was 9.4 ± 1.1 g/dL, the mean MCV was 99 ± 11.1 fl, and the mean HbF was 21.7 ± 9.4%. As expected, at MTD, an elevated MCV was correlated with elevated fetal hemoglobin (r2= 0.19, p= 0.0001) [Table 1]. There was a statistically significant relationship between the age at HU initiation and the HbF at MTD (r2= 0.08, p= 0.015) [Figure 1] as well as the age at HU initiation and the hemoglobin at MTD (r2= 0.19, p= 0.016). The relationship between the age at starting HU and the overall change in HbF (DHbF) was not statistically significant (r2= 0.01, p= 0.41). There was not a statistically significant relationship between age at HU initiation and the MTD of HU (r2= 0.003, p= 0.61). The 6 patients started on HU at age less than 2 years (mean 1.5 ± 0.3 years) maintained a mean elevated HbF of 19.1 ± 5% at last documented follow-up with follow-up ranging from 1.4–13 year of uninterrupted hydroxyurea use. Starting hydroxyurea therapy at a younger age appears to improve HbF response as measured at MTD, although there is variability in the level of fetal hemoglobin attained. There is not an association seen with the DHbF or dose at MTD and age at hydroxyurea initiation. In summary, starting hydroxyurea at a younger age, when HbF is >20%, leads to persistence of HbF production and overall improvement in hematological efficacy. This was not simply the result of achieving MTD at a younger age before physiologic decline of HbF. Disclosures: Off Label Use: Hydroxyurea for complications of sickle cell disease in pediatrics. Shah:Eisai: Research Funding; Adventrx: Consultancy.

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Measuring Transition Readiness in Adolescents and Young Adults with Sickle Cell Disease Using the Transition Readiness Assessment Questionnaire

10.22541/au.162138591.15170944/v1 ◽

2021 ◽

Author(s):

Anjali Oberoi ◽

Alyssa Patterson ◽

Amy Sobota

Keyword(s):

Young Adults ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Center ◽

Self Report ◽

Adolescents And Young Adults ◽

Cell Disease ◽

Readiness Assessment ◽

Transition Readiness ◽

Assessment Questionnaire

Background/Objectives: Adolescents and young adults (AYA) with sickle cell disease (SCD) face challenges related to the disease and its treatment. The Transition Readiness Assessment Questionnaire (TRAQ) is a self-report tool for assessing transition readiness for youth with special health care needs (YSHCN), including SCD. This study uses the TRAQ to understand transition readiness in patients with SCD treated at the Boston Medical Center, evaluates associations between TRAQ scores and transition outcomes (e.g., EDr, EDu), and compares TRAQ scores in this population with other YSHCN. Methods: We reviewed electronic medical records of AYA with SCD who completed the TRAQ in the pediatric hematology clinic between January 1, 2019, and March 1, 2020, and categorized healthcare encounters to calculate EDu and EDr. We used t-tests and ANOVA models to analyze mean TRAQ scores, sex, age, genotype, EDu, and EDr. Results: The sample was 45 AYA patients with SCD between 13 and 22 years old. The mean TRAQ score for the overall patient sample was 3.67. Mean TRAQ scores did not significantly vary by sex or genotype but did significantly increase with age. TRAQ scores were lower in the SCD population than in other YSHCN. TRAQ scores did not correlate to EDu or EDr. Conclusions: AYA patients with SCD have lower transition readiness than other populations of YSHCN. The age of 18 may not be the most reliable attribute of readiness, though older patients do have higher readiness. The relationship between TRAQ scores, EDr, and EDu is not clear and requires further evaluation.

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Impact of the COVID19 Pandemic on Admissions and Emergency Department Visits of Adults with Sickle Cell Disease

Blood ◽

10.1182/blood-2020-142803 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 37-38

Author(s):

Alice J. Cohen

Keyword(s):

Emergency Department ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Center ◽

Cell Disease ◽

Face To Face ◽

Outpatient Visits ◽

Pain Medications ◽

Time Period ◽

Ed Visits

Background: The most common complication of sickle cell disease (SCD) in adults is vaso-occlusive crisis that is characterized by severe pain. These events can often be managed at home with oral analgesics, but if the pain is not controlled or the patient develops other associated problems, they seek care in an emergency department (ED). In the ED, they receive initial treatment with pain medications and are assessed for other complications such as infection and acute chest syndrome. If an individual's pain is not controlled in a short period of time, the majority of these patients are admitted to the hospital for inpatient management or placed in an observation unit (OBs) for 6-47 hours. The COVID-19 pandemic affected the Greater Newark community starting in mid March with the majority of all inpatient admissions (Ads) being COVID related through the end of May. It has been observed both at our medical center and nationally that during this time period and even afterwards, the number of ED visits and Ads had significantly fallen. The reasons for this finding may include fear of contracting COVID infection at the hospital, regular telemedicine (TM) calls to facilitate outpatient management, and an increase in the number of prescriptions of home pain medications. The purpose of this analysis was to examine patterns of ED visits, Ads, outpatient visits, prescription renewals and nurse (RN) and social worker (MSW) calls in order to determine the impact of COVID-19 infection on the local SCD community. Methodology: A retrospective review was undertaken of billing data and the EMR of all patients with SCD treated at Newark Beth Israel Medical Center (a 450 bed community-based academic tertiary care medical center) between January 2020 and June 2020. Data collected included the number of and reason for ED and OBs, Ads, the number of TM and outpatient visits, and MSW and RN telephone contacts. All patients 18 years of age and older were included. Overall, 100 adults with SCD received care between January and June. Results: Peak hospital COVID Ads, ED and OBs for all patients (SCD and non-SCD) occurred during the weeks between March 25 and May 24, 2020 with a daily inpatient census over 200 between April 7 and 24. SCD Ads at peak COVID (April-May) were significantly lower at 26±2/month compared to 64±11/month pre-COVID (January-February) (p= 0.04). ED and OBs were unchanged. During the peak of COVID, 10/93 (11%) SCD Ads (1 death) were COVID related with 80/96 (86%) for uncomplicated pain crises. MSW and RN called all patients proactively to offer support at onset of COVID pandemic. During this same time period, the number of MSW telephone contacts increased from 138±37/month pre-COVID to 372±21/month during COVID (p=0.02). RN contacts with SCD patients were stable and mostly were for pain prescription renewals. TM was initiated in March 2020 and an increase in these visits correlated with a fall in face to face physician visits: 83.5±11/month pre-COVID to 39.5±8/month peak COVID (p= 0.04), and TM 0/month pre-COVID and 31±4/month peak COVID (0.01). Conclusion: The outbreak of COVID-19 in the community reduced the number of Ads for patients with SCD without an increase in ED and OBs visits. MD face-to-face encounters were reduced but outpatient care continued with the initiation of TM, regular RN contact with maintenance of pain medication prescriptions and a greater numbers of MSW calls for psychosocial support. Further investigation and understanding of the use of Ads for SCD care, and the reduction during COVID, may have implications for current SCD management. Disclosures Cohen: GBT: Speakers Bureau.

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Bacterial Sepsis in Children Who Have Sickle Cell Disease

Pediatrics in Review ◽

10.1542/pir.15.4.136 ◽

1994 ◽

Vol 15 (4) ◽

pp. 136-162

Keyword(s):

Sickle Cell Disease ◽

Mortality Rate ◽

Sickle Cell ◽

Medical Center ◽

Albert Einstein College ◽

Albert Einstein ◽

Febrile Child ◽

Cell Disease ◽

Pneumococcal Sepsis ◽

Montefiore Medical

Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY Infection is the principal cause of death in patients who have sickle cell disease. Because the peak incidence of death is between 1 and 3 years, during which period the mortality rate approaches 3%, it is extremely important for the pediatrician to evaluate and treat promptly the febrile child who has sickle cell disease. Under the age of 5 years, as many as 15% of children who have sickle cell disease develop sepsis or meningitis, with a 30% mortality rate. Children in this age group develop pneumococcal sepsis with 400 times the frequency of the general population. Haemophilus influenzae infection is 2 to 4 times as frequent as in other children.

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An Elevated TRV Is Associated with Venous Thromboembolism in Sickle Cell Disease

Blood ◽

10.1182/blood.v120.21.3222.3222 ◽

2012 ◽

Vol 120 (21) ◽

pp. 3222-3222

Author(s):

Rachel M Adams ◽

Lianne S Kopel ◽

Demedrick Anton Bland ◽

Elizabeth S Klings

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Conflicts Of Interest ◽

Medical Center ◽

Heart Catheterization ◽

Cell Disease ◽

Complete Evaluation ◽

Ct Angiogram ◽

Significant Difference ◽

History Of

Abstract Abstract 3222 Objective: An elevated tricuspid regurgitant jet velocity (TRV) by echocardiography, used to screen for pulmonary hypertension (PH) and mortality in sickle cell disease (SCD), likely has a multi-factorial etiology. SCD is a hyper-coagulable state, yet, the contribution of venous thromboembolic disease (VTE) to an elevated TRV is unknown. Based on the known association between VTE and PH, we hypothesized that SCD patients with an elevated TRV will have an increased prevalence of VTE. Methods: We reviewed data collected prospectively as part of the PH in SCD study conducted at Boston University/Boston Medical Center from 2004–2010. Subjects were included if they underwent both echocardiography and testing for VTE. An elevated TRV was defined as > 2.5 m/sec or the presence of PH on right heart catheterization. A history of VTE was defined by a positive CT/angiogram, ventilation/perfusion scan or Duplex ultrasound of an extremity (not catheter-related). Data were analyzed using a Chi-Square test and an odds ratio for VTE was calculated. Results: We reviewed the records of 162 patients enrolled in the PH of SCD study; 97 underwent both echocardiography and an evaluation for VTE.5/53 patients (9.4%) with normal echocardiography had a history of VTE, compared with 13/44 (29.5%) in the elevated TRV group. SCD patients with an elevated TRV were four times more likely to have a history of VTE compared to those with a normal echocardiogram (OR: 4.03, 95% CI 1.31–12.41, p=0.011). There was no significant difference in age, gender, history of asthma, hemoglobin genotype, hematologic profiles or renal function between patient groups. Conclusion: An elevated TRV and PH are associated with a history of VTE in SCD patients. This suggests a role for thrombosis in disease modulation and underscores the need for a complete evaluation for VTE in SCD patients with an elevated TRV. Disclosures: No relevant conflicts of interest to declare.

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Pain Management in Adults With Sickle Cell Disease in a Medical Center Emergency Department

Journal of the National Medical Association ◽

10.1016/s0027-9684(15)30729-x ◽

2010 ◽

Vol 102 (11) ◽

pp. 1025-1032 ◽

Cited By ~ 23

Author(s):

Lawrence R. Solomon

Keyword(s):

Emergency Department ◽

Pain Management ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Center ◽

Cell Disease

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