DEVELOPMENT OF ALGORITHMS TO PREDICT PAIN IN HOSPITALIZED PATIENTS WITH SICKLE CELL DISEASE USING OBJECTIVE DATA FROM MEDICAL RECORDS

Author(s):  
Nirmish Shah
Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3521-3521
Author(s):  
Rebekah Shaw ◽  
Sarah Kappa ◽  
Robert Sheppard Nickel

Abstract Background: Hydroxyurea is daily oral medication proven to decrease complications of sickle cell disease (SCD). While concerns have been raised about the safety of hydroxyurea, it is now generally viewed as a well-tolerated medication for SCD. The primary toxicity of hydroxyurea that requires holding of treatment is reversible cytopenia. Due to its classification as a chemotherapeutic agent and safety concerns regarding inappropriate chemotherapy ordering, hydroxyurea can only be ordered by "chemotherapy-certified" providers at some hospitals. At our hospital system, pediatric resident physicians were restricted from ordering hydroxyurea. Instead of being a part of a resident's hospital admission orders, hydroxyurea for inpatients had to be ordered separately by a hematology fellow or attending physician. In June 2016 our hospital changed its policy to allow residents to order hydroxyurea for patients with SCD admitted to the hospital who were already on hydroxyurea at home. We hypothesized that this change in policy to allow residents to order hydroxyurea would increase the proportion of patients with SCD appropriately receiving their home hydroxyurea by hospital day 1. We also hypothesized that this policy change would not result in an increase in the proportion of patients inappropriately receiving hydroxyurea when it should have been held based on the admission complete blood count (CBC). Methods: We conducted a retrospective review of the medical records of a random sample of patients admitted to the hematology service the year before (2015) and the year after (2017) the policy change in 2016. Patients were eligible for study if they were admitted to the hematology service and were taking hydroxyurea as documented by a clinic note within the last three months. Patients were excluded if they were admitted to the intensive care unit or for surgery. Patients were also excluded if discharged on hospital day 0 or 1. Institutional guidelines advise holding hydroxyurea if any of the following: absolute neutrophil count <1250/µL; platelet count <80K/µL; reticulocyte count <100K/µL, unless hemoglobin >8.0 gm/dL. Hydroxyurea was classified as "inappropriately given" if a patient received hydroxyurea despite having an admission CBC value below a hold parameter. Hydroxyurea was classified as "appropriately not given" if a patient did not receive hydroxyurea when having a CBC value below a hold parameter. Patients who were on hydroxyurea who never received hydroxyurea inpatient with CBC values above the hold parameters were classified as "inappropriately not given." Patients admitted in 2015 (before resident ordering) were compared with patients admitted in 2017 (after resident ordering) using a chi-square test or Fisher exact test. Results: In total, 217 hospitalizations of eligible patients were reviewed: 91 before the policy change and 126 after the policy change. Based on the admission CBC, hydroxyurea should have been held for 8 patients. Excluding these patients who should not have received hydroxyurea, patients after the policy change were significantly more likely to have received their home hydroxyurea by hospital day 1: before 62/90 (69%) vs. after 105/119 (88%), p=0.0005. The proportion of patients who inappropriately received hydroxyurea was very low in both groups: before 1/91 (1%) vs. after 3/126 (2%), p=0.64. No serious adverse clinical events occurred from this "inappropriate" administration of hydroxyurea. The figure graphically displays the proportion of patients in the two groups who: appropriately received on hospital day 0/1/2+, inappropriately did not receive, appropriately did not receive, and inappropriately received hydroxyurea. Conclusion: Resident ordering of home hydroxyurea for hospitalized patients with SCD appears to be safe. Policies that permit residents to order hydroxyurea as part of a patient's admission orders can help increase the proportion of patients who receive this important medication while inpatient. Figure. Figure. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3090-3090
Author(s):  
Stephanie Guarino ◽  
Sophie M. Lanzkron

Abstract Introduction Sickle cell disease (SCD) is the most common inherited hemoglobinopathy and is estimated to affect more than 100,000 Americans. Current Centers for Disease Control statistics indicate that Black Americans die from COVID-19 at a disproportionately high rate, 13.6% of 449, 373 deaths but only account for 12.54% of the United States Population (CDC COVID Data Tracker accessed 5/5/2021). A voluntary clinical reported registry of COVID-19 infections in patients with SCD reported both high hospitalization rates (69%) and case fatality rates (7%) (Panepinto, 2020), but only reported data from March 20-May 21, 2020. A retrospective cohort review from England demonstrated a 4-fold increased risk of hospitalization and 2.6-fold increased risk of death due to COVID-19 for those patients with sickle cell disease compared to the general population (Hippisley-Cox, 2021). The unique constellation of SCD manifestations complicate both the diagnosis and management of COVID-19, particularly related to anticoagulation and transfusion practices. Understanding the impact of early exchange and anticoagulation would guide development of appropriate treatment guidelines and future understanding of pathophysiology. We report on the outcomes for all hospitalized inpatients with SARS-CoV-2 and SCD at institutions using Cerner electronic health record (EHR). Methods Exempted retrospective review approved by ChristianaCare IRB. We obtained deidentified data from the Cerner COVID Data Lab which includes information on all hospitalized inpatients with SARS-CoV-2 and sickle cell disease as documented by ICD 10 D57.XX from 12/10/2019-10/15/2020 at institutions that use the Cerner EHR. Those with sickle cell trait excluded. The data included 209 patients; 1 patient had separate 2 visits, only the first visit in the data. Evaluated anticoagulation use, not dose. Descriptive statistics are given: percentages for categorical variables and mean (standard deviation) for continuous variables. Comparisons between patients who died or went to hospice versus patients who recovered were done using unadjusted chi-squared tests or t-tests. Results: Admission: 124 (74.3%) were afebrile (temp &lt;100) 52 (28.4%) were tachypneic (RR &gt;22) 10 (6.2%) were hypoxic (&lt;92%) 7 (3.3%) were intubated during hospitalization Many patients had comorbidities including diabetes, hypertension, and congestive heart failure, but it was not clear if patients had multiple co-morbidities. Treatment: 16 (7.7%) got transfused RBC between 1.77 and 589.18 hours into admission 5 (2.4%) got Remdesivir, none of these patients died/went to hospice. No exchange transfusions, but maybe wasn't captured in coding data 149 (71.3%) received anticoagulant, dosing unable to be obtained so not known if this was prophylactic or treatment dosing. Outcomes: 158 (80.2%) discharged home 18 (9.1%) discharged facility 8 (4.1%) died 2 (1.0%) discharged hospice 11 (5.6%) left against medical advice 12 (5.7%) missing disposition data Those who died/went to hospice had longer hospital stays, were more likely to be hypoxic and initially tachypneic. None of these patients received remdesivir. Study included small numbers but those who died more likely to have hypertension, diabetes w/ and w/out complications, CHF. Discussion This data only included hospitalized patients, doesn't account for patients who remained outpatient so case fatality rate likely lower. 10/209 patients died- 4.8% fatality rate, 12 patients missing final discharge disposition. 18 went to facility, may have died as outpatient. Only a small number of patients received remdesivir and there were overall low rates of anticoagulation, concerning given SCD is a hypercoagulable state. Study limitations include only hospitalized patients in the dataset and only draws from Cerner EMR institutions, a 26% market share. Based on our own SCD population, only a small percentage of patients with SCD and COVID-19 hospitalized. Additionally, there is likely significant variability between institutions' treatment protocols, particularly in the early months of the data set. Finally, we could not adequately gauge severity of COVID-19 disease given notable variations in institutional resources. TABLES (in process): TABLE 1- Demographics- split by death/hospice vs. other dispositions TABLE 2- Admission Characteristics- Death/hospice vs. others TABLE 3- Treatments- Death/Hospice vs. others Disclosures Lanzkron: Shire: Research Funding; Novartis: Research Funding; Bluebird Bio: Consultancy; CSL Behring: Research Funding; Imara: Research Funding; GBT: Research Funding; Pfizer: Current holder of individual stocks in a privately-held company; Teva: Current holder of individual stocks in a privately-held company; Novo Nordisk: Consultancy.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2039-2039
Author(s):  
Stephanie O Ibemere ◽  
Laura Niederer ◽  
Charity I Oyedeji ◽  
Ebony Burns ◽  
John Myers ◽  
...  

Abstract Introduction Emerging literature suggests body mass index (BMI) may be increasing in individuals with sickle cell disease (SCD), a condition historically associated with underweight status. However, the current prevalence of overweight and obese adults with SCD remains unclear in a country where high BMI is prevalent in the general population. We present an epidemiological analysis of the prevalence of overweight and obese individuals in a large representative sample of adult SCD patients, identifying associations between BMI, sociodemographic and clinical characteristics. Methods Using the Sickle Cell Disease Consortium (SCDIC) registry, we compiled a detailed clinical and demographical from a non-random sample of adults, aged 20-45 years. The SCDIC collects data from eight academic centers providing comprehensive care throughout the U.S. Adult participants were excluded from the analysis if they were under 20 years of age, pregnant at the time of enrollment, or were without medical records or patient enrollment surveys. Sociodemographic information and patient-reported outcomes, including pain frequency and severity, SCD complications, and hydroxyurea use, were collected at the time of enrollment. Non-SCD medical conditions and anthropometric measurements were abstracted from medical records. We stratified BMI, measured in kilograms per meters squared (kg/m2) following CDC criteria: underweight (&lt;18.5 kg/m2), normal weight (18.5-24.9 kg/m2), overweight (25.0-29.9 kg/m2), and obese (&gt;30 kg/m2). An epidemiological analysis was performed of BMI in those with SCD. Bivariate analyses were conducted using Chi-square test, t-test, and ANOVA; non-parametric tests were used when appropriate. Data were analyzed using SAS 9.4 (SAS Institute; Cary, NC). Results In total, 1,664 adults met the inclusion criteria for this cross-sectional analysis of SCDIC registry data. The median BMI for the entire sample was 23.9 kg/m² (IQR: 21.1-28). The majority of participants were African American (99.1%), female (56.6%), and had an HbSS genotype (69.6%). The prevalence of an obese BMI (17.5%) was greater than underweight BMI (6.4%) among the entire cohort (Table 1). When compared to under/normal weight participants, those who were overweight/obese were older (median age 31.2 versus 29.3 years; p&lt;0.0001) and had a higher prevalence of hypertension (45.1% versus 28.3%; p&lt;0.0001). Most participants in the overweight/obese BMI categories had genotype HbSS (59.0%), however, genotype HbSS accounted for 77% of the under/normal weight category. Most participants with an overweight/obese BMI also had some college or vocational training (39.9%) and had Medicare, Medicaid, or military insurance (71.9%). Median BMI did not differ on reported use or non-use of hydroxyurea (23.9 [IQR: 21.1-27.6] vs 23.9 [IQR: 21.0-28.4] p=0.1), mean number of patient-reported SCD complications (2.6 versus 2.6; p=0.6), nor mean pain frequency (49.6 versus 50.6; p=0.06). However, overweight/obese participants reported significantly higher mean pain intensity than their under/normal weight counterparts (51.8 versus 50.8; p=0.03). Conclusion To date, this is the largest analysis of adult BMI among individuals with SCD in the U.S. Among the eight SCDIC sites spanning from the Northeast to Southwest U.S., the prevalence of underweight BMI was less than that of overweight or obese BMI status, challenging previous understandings of weight status and further aligning with the growing rates of overweight and obesity in the general U.S. population. Significant associations between high BMI and hypertension, age, and pain intensity highlight an opportunity for further research to understand the impact of increasing BMI on SCD outcomes and non-SCD comorbidities. Figure 1 Figure 1. Disclosures Ibemere: bluebird bio Insights Council: Consultancy, Honoraria; Ugali Youth: Consultancy, Membership on an entity's Board of Directors or advisory committees. King: Health Resources and Services Administration: Research Funding; National Cancer Institute: Research Funding; Global Blood Therapeutics: Research Funding; National Heart, Lung, and Blood Institute: Research Funding. Hankins: Global Blood Therapeutics: Consultancy; UpToDate: Consultancy; Bluebird Bio: Consultancy; Vindico Medical Education: Consultancy. Tanabe: CSL Behring: Consultancy. Shah: Emmaus: Consultancy; GLG: Consultancy; Alexion: Speakers Bureau; Guidepoint Global: Consultancy; Bluebird Bio: Consultancy; CSL Behring: Consultancy; Novartis: Research Funding, Speakers Bureau; GBT: Consultancy, Research Funding, Speakers Bureau.


Rev Rene ◽  
2015 ◽  
Vol 16 (3) ◽  
Author(s):  
Júlia Lamese Amaral ◽  
Nívea Aparecida Almeida ◽  
Paula Silveira Santos ◽  
Patrícia Peres de Oliveira ◽  
Fernanda Moura Lanza

Objective: to describe socio-demographic and economic characteristics, lifestyle, clinical manifestations, use of medicationsand monitoring of adults with sickle-cell disease. Methods: a descriptive study with quantitative approach, made with 20adults, registered in a Hematology and Hemotherapy Center, using, for data collection, interviews in the participant’s homeand medical records. In order to have the database, the EpiDatae analysis software through statistical program was used.Results: most of the population consisted of women, married, with complete high school, which used exclusively the UnifiedHealth System. The average age was 30.6 years, and 90.0% (95% CI 68.3-98.8) were black. All of them reported painful crisesand fatigue. They used folic acid daily 35.0% (95% CI 15.4-59.2). Conclusion: the implications of sickle-cell disease could bemitigated through primary, secondary and tertiary health care, according to the needs of those adults.


2018 ◽  
Vol 198 (4) ◽  
pp. 534-537 ◽  
Author(s):  
Manyoo A. Agarwal ◽  
Mahek Shah ◽  
Brijesh Patel ◽  
Vikki G. Nolan ◽  
Guy L. Reed ◽  
...  

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Narcisse Elenga ◽  
Loic Niel

This study in French Guiana assessed the frequency of alloimmunization to red cell antigens in sickle cell disease patients over 1995–2011 and identified the most common antibodies. A retrospective analysis of the transfusion history and medical records of 302 patients showed that 29/178 transfused patients had developed alloantibodies (16%). The most frequent alloantibodies were anti-LE1, anti-MNS1, anti-LE2, and anti-FY1 and were developed after transfusion of standard red cell units. The frequency of the clinically significant antibodies in this population of SCD patients was 11% (19/178). The antibodies found on those patients who had delayed hemolytic transfusion reaction were anti-K1, anti-FY1, and anti-MNS3. The strategies used to decrease alloimmunization in French Guiana are discussed.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2969-2969
Author(s):  
Arrey-Takor Ayuk-Arrey ◽  
Isha Darbari ◽  
Allistair Abraham ◽  
Robert Sheppard Nickel

Abstract Background: Hematopoietic stem cell transplant (HSCT) using an HLA-identical sibling donor is a well-established cure for sickle cell disease (SCD). Historically, HSCT was only offered to patients with SCD who had suffered severe complications; however, given improved HSCT outcomes, it is now reasonable to consider HSCT for most patients with SCD who have an HLA-identical sibling. Thus, HLA typing of all full siblings of patients with SCD should be a clinical priority to ensure patients are aware of and have access to this therapeutic option. The primary objectives of this study are to describe the baseline prevalence of HLA typing among a cohort of hospitalized pediatric patients with SCD and to evaluate whether having had HLA typing is associated with certain characteristics. Secondarily, the study describes the acceptability of HLA typing among patients with a healthy (non-SCD) full sibling who had not already had HLA typing at baseline after dedicated outreach to these families. Methods: Between January 1, 2020 and December 31, 2020 a REDCap database of all hospitalized patients with SCD was prospectively maintained. Patient demographic and clinical information was abstracted via retrospective chart review. To evaluate socioeconomic status, a neighborhood area deprivation index (ADI) was determined for each patient using their home address and the Neighborhood Atlas website (https://www.neighborhoodatlas.medicine.wisc.edu/). ADI is a validated ranking (0-100) of Census Block Groups, considering income, education, employment, and housing quality. A higher ADI represents greater socioeconomic disadvantage. As part of a clinical outreach initiative, patients' families who had not already had typing at the time of their hospitalization were contacted to determine if the patient has a healthy full sibling and, if applicable, offer sibling HLA typing. This outreach was originally planned to occur in person at the time of hospitalization or clinic follow-up, but due the COVID-19 pandemic it was paused and when resumed conducted mostly via telephone. Results: During the 52-week study period, 291 patients with SCD were hospitalized at the study pediatric institution. Seventy-one patients (24%) had already completed HLA typing at the time of their first hospitalization during the study period. These patients with HLA typing at baseline were significantly more likely to have a diagnosis of sickle cell anemia (HbSS/HbSβ 0 genotype) and be on disease-modifying therapy (hydroxyurea or chronic transfusion) compared to patients without typing at baseline (Table). Age and sex were not significantly different between patients with and without typing (Table). The group of patients who did not have HLA typing at baseline had a significantly greater ADI (mean 29.7 vs. 24.0, p=.008) and proportion of patients with a high disadvantage ADI score ≥40 (23% vs. 10%, p=.02), Figure. Of the 220 patients with no history of HLA typing, the sibling status of 187 patients was determined via outreach to these families as of July 2021. Among these 187 patients, 81 (43%) reported having a healthy full sibling. Among these 81 patients with siblings, after being offered family HLA typing, 42 (52%) were interested and referred for HLA typing, 29 (36%) were undecided, and 10 (12%) declined typing. Conclusion: Hospitalized pediatric patients with SCD who had already been HLA typed were more likely to have a severe SCD genotype and be on disease-modifying therapy as expected. Patients who had not had HLA typing were more likely to live in a socioeconomically disadvantaged neighborhood. This finding suggests that dedicated outreach to all families regarding HLA typing is needed. Our clinical initiative to offer typing to all hospitalized patients with healthy full siblings was feasible, with a majority of families interested in pursuing HLA typing. Continued work is needed to ensure patients with SCD have equal access to curative therapy regardless of socioeconomic status. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.


2006 ◽  
Vol 119 (10) ◽  
pp. 897.e7-897.e11 ◽  
Author(s):  
Paul D. Stein ◽  
Afzal Beemath ◽  
Frederick A. Meyers ◽  
Elias Skaf ◽  
Ronald E. Olson

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