scholarly journals Improved Utilization Amongst Adult Sickle Cell Disease Patients: A Multi-Disciplinary Medical Home Approach

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 22-23
Author(s):  
Daniel Sop ◽  
Shirley Johnson ◽  
Benjamin Jaworowski ◽  
Wally R Smith
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Medical Home ◽  
Research Funding ◽  
Readmission Rate ◽  
Cost Savings ◽  
Multidisciplinary Teams ◽  
Cell Disease ◽  
Number Of Patients

Background Worldwide, Sickle Cell Disease (SCD) is the most common monogenetic disorder. SCD is costly to the American health care system: $460,151 per patient for the 100,000 affected US patients. Biological and behavioral health comorbidities with limited hospital resources contributed to a cost increase in SCD readmissions at the Virginia Commonwealth University Health System (VCUHS) from 2012-2016, after a long history of decline. With increasing census, in FY16 the 30-day SCD readmission rate was 33.7%, up from 22.5% in FY14. The average SCD length of stay was 6.7 days, compared with an expected length of 4.2 days. The number of ED visits for SCD patients in FY17 was projected to be double that of FY14. The percent of ED returns in 3 days was projected to be triple that of FY14. Method The VCU Adult SCD Medical Home (ASCMH) was funded and launched to address this issue, based on the success of a pilot that showed cost savings of $333,000 for 5 patients in 12 months. The SCD ASCMH officially began January 1,2018 with a two-year pilot funding period. The ASCMH was structured and designed using quality improvement (QI) principles and consisted of multidisciplinary teams to coordinate inpatient care, emergency care, and ambulatory care respectively. Evaluation compared utilization during CY 2017 (pre-intervention) versus CY 2019 (2 years post intervention). For all patients we compared the average 30-day readmission rate, the average length of stay (ALOS), the average 3-Day ED return rate, the number of ED discharges, the numbers of inpatient days, inpatient discharges, and outpatient visits, the number of patients who used the ED, and total VCU charges. Results Among 541 (2017) and 592 (2019) SCD adults, including 40.6% males and 59.4% females, comparing pre-intervention to intervention, average utilization and VCU charges were either numerically or statistically significantly reduced. Mean 30-day readmission rates were reduced (31.60% vs 20.40%) ALOS was reduced (5.6 days vs 4.3 days), inpatient days were reduced (5313 days vs 3340 days), and total charges were significantly reduced ($15,664,895 vs $13,939,842). Conclusion At VCU, a multi-disciplinary ASCMH that featured intensive case management (CM) reduced annual utilization and cost savings for adult SCD patients. CM program elements that were most effective should be studied in the future and next steps should include a randomized controlled trial that can demonstrate evidence of their efficacy in strengthening and improving utilization Table Disclosures Smith: Emmaeus Pharmaceuticals, Inc.: Consultancy; GlycoMimetics, Inc.: Consultancy; Novartis, Inc.: Consultancy, Other: Investigator, Research Funding; Shire, Inc.: Other: Investigator, Research Funding; Imara: Research Funding; Novo Nordisk: Consultancy; Ironwood: Consultancy; Pfizer: Consultancy; Incyte: Other: Investigator; Health Resources and Services Administration: Other: Investigator, Research Funding; NHLBI: Research Funding; Patient-Centered Outcomes Research Institute: Other: Investigator, Research Funding; Global Blood Therapeutics, Inc.: Consultancy, Research Funding; Shire: Research Funding.

scholarly journals Implementation of a Standard Order Set at a Sickle Cell Acute Care Observation Unit

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3396-3396
Author(s):  
Renee Cheng ◽  
Rishi Patel ◽  
Sandra Kang ◽  
Frances Tian ◽  
Xu Zhang ◽  
...  
Keyword(s):  
Pain Management ◽  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Acute Care ◽  
Sickle Cell ◽  
Research Funding ◽  
Mixed Effects ◽  
Data Sets ◽  
Cell Disease ◽  
Data Set

I NTRODUCTION: Acute painful vaso-occlusive crises (VOC) in sickle cell disease (SCD) are the leading cause of emergency department (ED) encounters and frequent hospital admissions. For patients presenting with an uncomplicated VOC, acute care observation units (ACOU) have previously been shown to reduce admission rates and length of stay. We wished to evaluate if implementing a standardized acute care order set (ACOS) at the University of Illinois Hospital Sickle Cell ACOU would decrease the time to first dose of analgesic medication, inpatient hospital stays, and subsequent admissions to the ACOU. METHODS: The ACOS includes standard orders for laboratory tests to monitor severity of sickle cell hemolysis, intravenous fluids, and analgesic medications including opioids. We conducted a retrospective analysis to evaluate if the ACOS enhanced workflow and improved the timeliness of treatment in patients experiencing a VOC. The ACOS was created in April 2017 and we compiled data from the three months before (January-March 2017) and after (May-July 2017) ACOS creation. We collected data on the time it took to administer the first opioid dose, admission rate, length of stay, number of acute care visits, ED visits, and inpatient hospitalizations in a three-month span, and demographics including variations in age, gender, and sickle cell disease genotype. Patient data was collected from a pharmacy-generated list of patients who received narcotics in the ACOU during the aforementioned time period. We analyzed the effect of the ACOS on the aforementioned variables. A mixed effects linear model was used to compare time to first dose of opioids and length of stay between data sets. A mixed effects logistic model was used for binary outcomes. Covariates of age (years), gender, and severity of sickle cell hemoglobin genotype (severe: HbSS, HbS beta0 thalassemia; mild: HbSC, HbS beta+ thalassemia) were included in the models. Statistical analyses were carried out in R version 3.4.3. DISCUSSION: The pre-ACOS data set contains 291 patient encounters for 76 patients with a median age of 37 years (interquartile range [IQR], 30-47 years), 66% female, and 71% with severe genotypes. The post-ACOS data set contained 289 patient encounters for 80 patients with a median age of 32 years (IQR, 27-45 years), 80% female, and 73% with severe genotypes. Implementation of an ACOS was associated with decreased time to pain management by 3.7 minutes (p=0.077) in patients presenting with uncomplicated VOC and with fewer repeat visits to the ACOU in the studied 3-month period [OR 0.35 (95% CI 0.13-1.00), p=0.049]. The median number of opiate doses received by patients in both data sets was 3. Using 3 as a cutoff, the implementation of the ACOS was also associated with more patients receiving >3 doses of opiates [OR=1.84 (95% CI 1.05-3.19), p = 0.033]. We demonstrate that in SCD patients experiencing VOC, a standardized ACOS was associated with a trend to reducing time to receiving pain management, with increased total opioid doses during the ACOU admission (suggesting better pain control), and subsequently with a statistically significant reduction in the number of repeat ACOU visits in the studied 3-month period. We have shown that a standardized ACOS that streamlines workflow in an ACOU may play an important role in delivering timely and quality care to patients with SCD. Disclosures Gordeuk: Pfizer: Research Funding; Novartis: Consultancy, Honoraria, Research Funding; Global Blood Therapeutics: Consultancy, Honoraria, Research Funding; Modus Therapeutics: Consultancy, Honoraria; Ironwood: Research Funding; CSL Behring: Consultancy, Honoraria, Research Funding; Imara: Research Funding; Inctye: Research Funding; Inctye: Research Funding; Pfizer: Research Funding; Emmaus: Consultancy, Honoraria; CSL Behring: Consultancy, Honoraria, Research Funding; Ironwood: Research Funding; Novartis: Consultancy, Honoraria, Research Funding; Imara: Research Funding; Modus Therapeutics: Consultancy, Honoraria.

scholarly journals Using Lean Six Sigma to Develop a Patient Centered Medical Home for Adults with Sickle Cell Disease

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3408-3408
Author(s):  
Shirley Johnson ◽  
Sarah Hartigan ◽  
Emily Holt ◽  
Daniel Sop ◽  
Chantal McHenry ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Six Sigma ◽  
Medical Home ◽  
Lean Six Sigma ◽  
Patient Centered Medical Home ◽  
Target Area ◽  
Cell Disease ◽  
Patient Centered

Background: Adults with sickle cell disease (SCD) suffer poor quality, disparities, higher utilization and mortality, and unmet medical need. Thus, in 2017 Virginia Commonwealth University Health (VCU) used pilot results and axioms of the Patient-Centered Medical Home (PCMH) and Lean Six Sigma quality improvement to build an Adult SCD Medical Home. Methods and Results: VCU funded the program in October 2017. We implemented it over the next fifteen months. We first used a Six Sigma QI cycle called DMAIC (Define, Measure, Analyze, Improve and Control). Define: we defined three improvement targets in year one: inpatient, emergency department (ED), and ambulatory care. Behavioral health, program evaluation, ambulatory clinical policies, and pediatric to adult transition were added in year two. We identified ourselves or recruited hospital partners as champions/leaders for each care target area. Each formed a multidisciplinary implementation team. The inpatient care team actually preceded Medical Home funding. Where feasible, each team conducted PDSA cycles to test and improve best practices or metrics. Measure: we ranked 567 adult SCD patients based on their CY 2017 30-day readmissions, length of stay, 3-day ED returns, ED discharges, inpatient days and discharges, outpatient visits, ED use, and charges. Each team also developed metrics. Analyze: The top 50 highest utilizers by charges were targeted for intervention. Improve: We aimed for six 12-month intervention patient results: 1) reduce readmissions by 15%; 2) reduce average length of stay by 1.5 days; 3) reduce charges by 15%; 4) improve compliance with SCD inpatient guidelines; 5) improve quality, safety, and financial reporting; 6) improve patient experience. Leaders also used Six Sigma tools weekly to oversee teams, identify resources, plan, and hire staff: for example, we ranked the urgency of steps using "Now, Later, Latest" process charts, and documented progress using "Quick Wins" communication logs. Control: Processes were continuously amended. Stakeholders met and supported us monthly to quarterly. We distributed an annual report. Metric results are presented in a separate abstract. Conclusion: Lean Six Sigma QI principles were effective for developing and implementing an adult SCD medical home. We believe the above processes could be replicated elsewhere. Figure Disclosures Lipato: Novartis: Honoraria. Smith:Novartis: Consultancy, Honoraria.

scholarly journals Tiered Oral Therapy Protocol for Sickle Cell Vaso-Occlusive Crisis

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3446-3446
Author(s):  
Margaret Guy ◽  
Lauren Magee ◽  
Jessica Keiser ◽  
Nicole Carter ◽  
DaleMarie Vaughan ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Readmission Rate ◽  
Oral Therapy ◽  
Historical Control ◽  
Hospital Charges ◽  
Cell Disease ◽  
Readmission Rates ◽  
Therapy Protocol

Background: Adults with sickle cell disease (SCD) often present with extremely painful acute vaso-occlusive crises (VOC). VOC guidelines quote studies supporting early initiation of opioids, but are lacking in a standardized approach to achieve adequate analgesia and subsequent de-escalation. Various SCD studies comparing continuous basal versus demand bolus patient-controlled analgesia (PCA) show conflicting outcomes. Thus the objective of our pilot was to develop a standardized approach to pain management utilizing PCA and a Tiered Oral Therapy Protocol (TOTP) in an effort to affect healthcare utilization by decreasing length of stay without increasing readmission rates. Methods: We studied admissions of 31 SCD (any genotype) patients (8 males, 23 females) ≥ 18 years old, all African-American, admitted for VOC, and treated by hospitalist providers using TOTP on one medical unit at Virginia Commonwealth University Health System from July 2018 to June 2019. Inpatient admissions for one calendar year prior to the initial TOTP admission served as the patient's historical control. TOTP is guided by the patient's individualized treatment plan and starts with continuation of their home basal dosing, demand dose only PCA, and immediate-release oral opioids for additional breakthrough pain. TOTP goals are aggressive up-titration of opioids until adequate analgesia. After a period of stability the next phase encourages timely, aggressive down-titration of opioids to speed discharge. We primarily compared length of stay (LOS), hospital charges, and 30-day readmission rates for TOTP vs historical controls. Secondarily, we compared adverse safety events: opioid-related rapid responses, naloxone administration, or falls. Results: Utilization of TOTP resulted in reductions in LOS (21%, 4.7 days controls vs. 3.7 days TOTP, p<0.014), and hospital charges ($1,627,497.73 controls vs. $944,528.96 TOTP, p=0.1403), and an insignificant increase in the 30-day readmission rate (35.6% controls vs. 42.7% TOTP, p=0.5027). Hospital charge data is difficult to interpret as a full year of post TOTP encounters are not available for all patients. There were no TOTP vs. historical control differences in opioid-related safety events. Conclusion: Standardization of therapy using the TOTP improved length of stay and reduced charges for patients with sickle cell disease admitted for acute VOC without increasing adverse safety events or significantly impacting the 30 day readmission rate. We have enlarged this pilot to include other VCU medical units, to test whether we can reproduce our initial one-unit success. Further, we recommend TOTP implementation and testing in other hospitals to exclude secondary trends, since TOTP was but one among several interventions in a VCU Adult SCD Medical Home. Figure Disclosures Smith: Novartis: Consultancy, Honoraria.

scholarly journals Hydroxyurea Prescription Fills and Adherence, Among Pediatric and Adult Medicaid Eligible Patients with Sickle Cell Disease in North Carolina

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3391-3391
Author(s):  
Nirmish Shah ◽  
Christian Douglas ◽  
Nancy Crego ◽  
Emily Bonnabeau ◽  
Marian Earls ◽  
...  
Keyword(s):  
North Carolina ◽  
Sickle Cell Disease ◽  
Old Age ◽  
Sickle Cell ◽  
Research Funding ◽  
Acute Chest Syndrome ◽  
Age Group ◽  
Cell Disease ◽  
Good Adherence ◽  
Number Of Patients

Introduction: Sickle cell disease (SCD) is a complex disease for which pain is the hallmark. Hydroxyurea (HU) is the standard of care for treatment for most patients with SCD and reduces the frequency of pain episodes, acute chest syndrome, need for red blood cell transfusions, hospitalizations and has been shown to improve mortality. Despite National Heart Lung and Blood Institute (NHLBI) recommendations for the use of HU beginning at 9 months of age, adherence has been historically low. We aimed to: 1) describe HU prescription fills and adherence for persons with SCD enrolled in Medicaid during a 12-month period in North Carolina (NC); and 2) determine factors that may predict good adherence. Methods: Medicaid claims were examined from data obtained from Community Care of North Carolina (CCNC) for patients with a diagnosis of SCD (ICD 9 CM codes: 282.6x, ICD 10 CM codes: D57.0x, D57.1, D57.2x, D57.4x, D57.8x) between March 1, 2016 and February 28, 2017. HU claims were identified using the drug name. Only those enrolled in Medicaid for 12 months were included in this analysis. The number of HU prescriptions filled per enrollee by age group was determined by summing the number of filled HU prescriptions over the study period for each eligible enrollee. The number of HU days supplied is the sum of the days of supply on the prescription (e.g. 30-day supply) in a 12-month period per person. The duration of HU treatment days was measured as the number of days between the date of the first HU prescription filled and the last day of the study period. The number of days between breaks in treatment is the sum of days with no HU supplied, divided by the number of gaps (missing next HU prescription fill) per person. HU adherence was categorized into one of the followings: 1) Good - if number of days supplied is ≥80% of duration of HU treatment; 2) Fair or Moderate - if number of days supplied is 60-79% of duration of HU treatment; 3) Poor - if number of days supplied is < 60% of duration of HU treatment. Logistic regression was used to evaluate HU treatment adherence (good versus fair or poor). The model was conditioned on age, gender, participant residence (metro, non-metro adjacent to metro and non-metro non-adjacent to metro), co-management (at least one PCP and one hematologist visit/patient during the study period) and months enrolled in CCNC. Results: A total of 2,790 patients with Medicaid claims data were reviewed, with 649 patients meeting inclusion criteria (at least one HU prescription and 12 months enrollment in Medicaid). The participants in the sample were majority female (51.77%), lived in metropolitan areas (78.12%) and had a mean age of 16.49 years old (SD=11.49) A third of the sample (32.20%) had at least 1 HU prescription during the study period (Table 1). Those who were 1-9 years old had the highest median number of days supplied (221; range 21-750), the least median days between breaks in HU treatment (14.20; range 0-318), and the longest duration of HU treatment days (median 340; range 0-364). Those who were 18-30 years old had the lowest number of median days supplied (110; range 4-366) and the most median days between treatment (49.3; range 0-337). The 1-9 year olds also had the highest number of patients classified as good HU adherence (47.50%) and conversely the lowest classified as poor HU adherence (37.50%). In contrast, the 18-30 year old age group had the lowest good HU adherence (18.03%) and the highest poor HU adherence (69.40%) in the sample. The 31-45 year old age groups had the next lowest good HU adherence (20.93%) and next highest poor HU adherence (60.47%). Good HU adherence was most influenced by participant age. Prediction by co-management was minimal (Figure 1). Gender, residency and number of months enrolled in CCNC had little influence on HU adherence. Conclusions: Although recommended for most patients, HU was prescribed for less than one third of all patients with Medicaid in NC. Pediatric patients had the highest rate of HU prescription (40-46%) and patients over the age of 30 had the lowest (11-12%). In addition, of those prescribed HU, most patients were not classified as having good adherence. Importantly, poor HU adherence was most prevalent in the transition age group (18-30 year old), supporting the need for increased focus during the move from pediatric to adult care. Efforts should continue to explore methods that improve adherence including provider education and innovative patient strategies such as mHealth. Disclosures Shah: Alexion: Speakers Bureau; GBT: Research Funding; Novartis: Consultancy, Research Funding, Speakers Bureau. Tanabe:NIH: Research Funding; AHRQ: Research Funding.

scholarly journals Clinical Characteristics of Severe Acute Chest Syndrome in Children with Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4175-4175
Author(s):  
Shaniqua C. Johnson ◽  
Venee N. Tubman ◽  
Titilope Fasipe ◽  
Julie P. Katkin ◽  
Nicholas Ettinger ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Research Funding ◽  
Viral Infections ◽  
Clinical Findings ◽  
Categorical Variables ◽  
Acute Chest Syndrome ◽  
Pleural Effusions ◽  
Cell Disease

Abstract Background: Acute chest syndrome (ACS) is a form of acute lung injury that is a leading cause of morbidity and mortality in children and adults with sickle cell disease (SCD). Despite advances in health maintenance and disease-modifying therapies for SCD, ACS remains a common and often severe complication. There is still limited understanding of why some patients suffer more severe ACS episodes. Few studies have specifically investigated clinical parameters associated with severe ACS in the pediatric population. The purpose of this study is to determine the association between hematologic and radiologic clinical findings and severe ACS in children and adolescents with SCD. Methods: We conducted a single-institution retrospective chart review of 120 pediatric patients with HbSS or HbSβ 0 thalassemia and at least one documented episode of ACS, and then further classified patients by their most severe ACS event. We extracted laboratory and radiologic data to compare clinical findings. Infections reported in patient encounters were verified by documented viral PCR and blood cultures. Continuous variables were analyzed using Welch's t-test; categorical variables were analyzed using Fisher's exact test. Results: Eighty patients in the moderate ACS group were excluded for this analysis. Twenty patients (median age 5.6 years, SD 4.20) had mild ACS defined by no supplemental oxygen requirement, only one segmental or lobar infiltrate on chest radiography, and transfusion of less than 3 units (or 15 cc/kg) of red blood cells. Twenty patients (median age 7.6 years, SD 4.01) had severe ACS characterized by acute respiratory failure requiring mechanical (invasive or non-invasive) ventilation and/or exchange transfusion. Median length of stay for patients with severe ACS was longer than for the mild cohort (mean: 8 days vs 2 days, p &lt;0.001). The median absolute neutrophil count (ANC) was significantly higher in the severe ACS cohort (19.8 x 10 3µL vs. 9.4 x 10 3 µL, p = 0.004). The median platelet count nadir was significantly lower in the severe ACS group compared to the mild group (165 x 10 3 µL vs. 309 x 10 3 µL, p &lt;0.001). Review of radiologic data showed that the median number of lobes affected in the severe ACS group were 3, compared to 1 lobe in the mild group (p &lt;0.001). Approximately 65% of patients with mild ACS had left-sided disease (p = 0.18), while 85% of patients with severe ACS had bilateral disease (p = 0.002). The risk of pleural effusions was also significantly increased with severe ACS [OR 76.00 (95% CI 9.235-825.6), p &lt;0.001]. When we analyzed infection at time of ACS, the proportion of laboratory-confirmed viral infections were twice as high in the mild ACS cohort (41.2%), compared to the severe ACS cohort (20.0%, p = 0.279). There were no bloodstream bacterial infections found in either cohort. Conclusion: Severe ACS in children is a clinically distinct phenotype associated with longer length of stay, higher ANC and lower platelet nadir counts during active ACS, increased involvement of 3 or more lung lobes, and presence of pleural effusions. A lower proportion of confirmed viral infections were found in the severe ACS cohort compared to mild ACS patients, although did not reach statistical significance. Future prospective studies investigating the utility of these specific laboratory and radiographic parameters as components of an ACS severity prediction score are warranted. Disclosures Tubman: Forma Pharmaceuticals: Consultancy; Novartis Pharmaceuticals: Honoraria, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Perkin Elmer: Honoraria. Fasipe: Emmaus: Consultancy; Bluebird Bio: Consultancy; Global Blood Therapeutics: Consultancy, Other: Grant Review Committee ; Novartis: Consultancy; Pfizer: Research Funding.

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.

scholarly journals Sickle cell disease pain management and the medical home

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 433-438 ◽  
Author(s):  
Jean L. Raphael ◽  
Suzette O. Oyeku
Keyword(s):  
Pain Management ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Medical Home ◽  
Cost Effective ◽  
Cell Disease ◽  
Patient Centered ◽  
Centered Care ◽  
The Individual ◽  
Effective Models

Pain is the most common cause for hospitalization and acute morbidity in sickle cell disease (SCD). The consequences of SCD-related pain are substantial, affecting both the individual and the health care system. The emergence of the patient-centered medical home (PCMH) provides new opportunities to align efforts to improve SCD management with innovative and potentially cost-effective models of patient-centered care. The Department of Health and Human Services has designated SCD as a priority area with emphasis on creating PCMHs for affected patients. The question for patients, clinicians, scientists, and policy-makers is how the PCMH can be designed to address pain, the hallmark feature of SCD. This article provides a framework of pain management within the PCMH model. We present an overview of pain and pain management in SCD, gaps in pain management, and current care models used by patients and discuss core PCMH concepts and multidisciplinary team–based PCMH care strategies for SCD pain management.

scholarly journals COVID Symptoms and COVID Anxiety in Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 16-17
Author(s):  
Wally R Smith ◽  
Benjamin Jaworowski ◽  
Shirley Johnson ◽  
Thokozeni Lipato ◽  
Daniel M Sop
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Telephone Survey ◽  
Cell Disease ◽  
Weighted Mean ◽  
The Us ◽  
Associated Symptoms ◽  
At Home

Background Even before the US upswing of the current COVID pandemic, the number of sickle cell disease (SCD) patients coming to hospitals and EDs appeared to fall drastically. This happened despite SCD patients having often been heavy utilizers of the ED and hospital for their iconic vaso-occlusive crises (VOC). Though ambulatory SCD clinics quick converted largely to telehealth in order to comply with stay-at-home orders designed to suppress person-to-person transmission, some SCD patients appeared to avoid care, delay care, or refuse doctors' invitations for care. Presumably patients did so out of COVID fears, but this has not been confirmed in the literature. Further, whether these patients had COVID symptoms but stayed at home has not been studied. As part of quality improvement (QI) to conduct COVID surveillance in an adult sickle cell program, we sought to explain and predict SCD health care utilization patterns we were observing, as well as to determine urgent physical and mental health needs of patients who appeared to be avoiding care. Methods Fifteen staff in the Adult Sickle Cell Medical Home at Virginia Commonwealth University, a large urban academic medical center, conducted a telephone survey ("wellness check"was used when we talked to patients) of all known adults with SCD over 19 days in 2020. A staff member confirmed the patient had SCD, asked permission to proceed, then asked about symptoms consistent with COVID-19. At the end of the telephone survey, respondents wer invited to complete an email survey of sickle cell and COVID-19 utilization attitudes (19-33 items, depending on the response pattern, either drawn from the National Health Interview Survey, from the Adult Sickle Cell Quality of Life Measurement quality of care survey, or drafted by the authors), the Sickle Cell Stress Survey-Adult (SCSS-A, a 10-item previously validated survey), and anxiety and depression (PHQ9 of the PRIME-MD). Results Of 622 adults approached by phone call, 353 responded to the following yes/no screening questions regarding the prior 14 days: fever over 100 F 0/353 (0.00%); cough 3/353(0.01%); difficulty breathing 0/353(0.00%); unexplained shortness of breath 2/353(0.01%); sore throat 2/353 (0.01%); unexplained muscle soreness 2/353(0.01%);contact with anyone who tested positive for COVID-19 2/353(0.01%); testing for COVID 19 6/353(0.02%). For QI purposes, we set a threshold of three or more COVID-associated symptoms or the presence of fever as criteria requiring intense telephone or in-person staff monitoring for the following week. Only three patients met criteria. A total of 219/353 had email surveys sent. Of 63 patients (28.8%) who returned email surveys by June 10, 2020, 35.9% had already managed a "pain attack" at home 4 or more times in the prior 12 months, and 45.5% of these said their bad ER experiences were very or somewhat important in that decision. In the prior 14 days, although 30/64 reported a crisis for at least one day, only 4/64 had visited the Emergency Department for pain. On a 0-10 scale, 21/61 patients endorsed "0" for worry that they would be COVID-infected by going for medical care (weighted mean 3.9), but 18/59 endorsed "10" for worry they were more at risk of COVID because of SCD (weighted mean 6.31), and 22/60 endorsed "10" for worry they would fare worse than others if COVID infected (weighted mean 6.97). Many patients forwent "needed" care (16/62) or delayed "needed" care by at least a day (36/61). Eleven patients met criteria for moderately severe to severe depression on the PHQ-9, and 28/63 somewhat or strongly agreed with the statement "death is always on the back of my mind" on the SCSS-A. Conclusions In adolescents and adults with SCD, many were already reticent to come to the ED for pain, but a significant portion reported delays or avoidance of needed care during the early stages of the US COVID pandemic, and few reported using the ED despite over half reporting at least one crisis day in 14. Patients nonetheless reported very few COVID-associated symptoms. Fears of COVID infection/susceptibility may limit visits for needed sickle cell care among adults. Acknowledgements: Mica Ferlis RN, FNP, Caitlin McManus, RN, FNP, Emily Sushko, RN, FNP, Justin West, RN, Kate Osborne, RN, Stefani Vaughan-Sams, Marla Brannon, BS, Nakeiya Williams, BS Disclosures Smith: GlycoMimetics, Inc.: Consultancy; Emmaeus Pharmaceuticals, Inc.: Consultancy; Novartis, Inc.: Consultancy, Other: Investigator, Research Funding; Global Blood Therapeutics, Inc.: Consultancy, Research Funding; Shire, Inc.: Other: Investigator, Research Funding; NHLBI: Research Funding; Patient-Centered Outcomes Research Institute: Other: Investigator, Research Funding; Health Resources and Services Administration: Other: Investigator, Research Funding; Incyte: Other: Investigator; Pfizer: Consultancy; Ironwood: Consultancy; Novo Nordisk: Consultancy; Imara: Research Funding; Shire: Research Funding.

scholarly journals Clinical and Biomarker Predictors for Avascular Necrosis in Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3091-3091
Author(s):  
Michael Rabaza ◽  
Maria Armila Ruiz ◽  
Liana Posch ◽  
Faiz Ahmed Hussain ◽  
Franklin Njoku ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Research Funding ◽  
Medical Attention ◽  
Cell Disease ◽  
Advisory Committees ◽  
Early Management

Abstract Introduction Sickle cell disease (SCD) affects 1 in 365 African Americans and approximately 25 million people world-wide. A common skeletal system complication is avascular necrosis (AVN), which can cause substantial pain and a reduced quality of life. While early management of AVN is focused on increasing range of motion with physical therapy and pain relief, there are no clear predictors for who is more likely to develop AVN and earlier institution of these preventive measure could help decrease disease progression. Vascular endothelial growth factor (VEGF) is a biomarker of endothelial injury and may indicate reduced vascular supply to the femoral or humeral head. Here we describe potential risk factors and biologic pathways for AVN in SCD, as understanding these may lead to improvements in future monitoring, early detection, and early intervention practices. Methods We investigated clinical and laboratory risk factors associated with AVN in a cohort of 435 SCD patients from our center. Blood samples, clinical, and laboratory data were collected at the time of enrollment during a clinic visit. Genotyping for alpha thalassemia was performed by PCR and the serum concentration of VEGF was measured by ELISA. AVN status was confirmed by review of the medical record and available imaging. We conducted a cross-sectional analysis comparing categorical and linear variables by AVN status using the chi-square and Kruskal-Wallis test, respectively. The independent association of the clinical and laboratory variables with AVN status was determined by logistic regression analysis. The initial model included variables with a P-value &lt; 0.1 on univariate analysis and the final model was ascertained by stepwise forward and backward selection. Median values and interquartile range (IQR) are provided. Results The median age of the cohort was 32 (IQR, 24 - 43) years, 57% (250/435) were female, and 46% (198/435) were on hydroxyurea. AVN was observed in 34% (149/435) of SCD patients. SCD patients with AVN were older, had more frequent vaso-occlusive crises requiring medical attention, and had a higher body mass index (Table I) (P ≤ 0.002). We measured VEGF in 241 of the SCD patients with serum samples available at the time of enrolment. Serum VEGF concentrations trended higher in SCD patients with versus without AVN (420 vs. 359 pg/mL, respectively; P = 0.078). In the multivariate analysis model, AVN was independently associated with increased number of vaso-occlusive crises (OR 1.1, 95% CI: 1.0 - 1.14; P = 0.02), AST concentration (natural log OR 0.5, 95% CI: 0.2 - 0.9; P = 0.03), VEGF concentration (natural log OR 1.4, 95% CI: 1.0 - 1.9; P = 0.047), and tobacco use (OR 1.9, 95% CI: 0.9 - 3.7; P = 0.078). Discussion In conclusion, we demonstrate a high prevalence of AVN in an adult cohort of SCD patients. The presence of AVN was independently associated with a greater frequency of vaso-occlusive pain episodes, which may demonstrate a shared pathophysiology between AVN and vaso-occlusion that merits further investigation. We demonstrate that serum VEGF concentrations are higher in SCD patients with AVN and may be a clinical tool to identify those at high-risk and for earlier intervention for this complication. Figure 1 Figure 1. Disclosures Gordeuk: Modus Therapeutics: Consultancy; Novartis: Research Funding; Incyte: Research Funding; Emmaus: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; CSL Behring: Consultancy. Saraf: Pfizer: Research Funding; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding.

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