Healthcare Utilization Among Children and Adolescents with Sickle Cell Disease during the COVID-19 Pandemic

Background Early experience with the COVID-19 pandemic showed disproportionately high morbidity and mortality among individuals with certain chronic medical conditions. Individuals with sickle cell disease (SCD) are at high risk for pulmonary and other complications including acute chest syndrome (ACS) and have high rates of hospitalization from other viral respiratory infections, raising concern that COVID-19 would be associated with higher morbidity, mortality and health care utilization among those with SCD. Public health interventions such as social distancing, avoidance of large group activities, and widespread use of masks have been shown to reduce the transmission of COVID-19 in the general population but have been inconsistently implemented. In Georgia, COVID-19 restrictions, including school closures, were implemented in mid-March, and on-site school instruction was replaced by virtual instruction for the remainder of the school year. At our institution, most routine, non-urgent outpatient clinic visits were cancelled or postponed from mid-March through May in order to minimize COVID-19 exposure risk. Efforts to initiate the use of telemedicine as an alternative to in-person office visits were rapidly instituted. We hypothesized that adherence to public health restrictions, especially sheltering in place, would be high among patients and families with SCD, and sought to measure the impact of COVID pandemic on healthcare utilization in children and adolescents with SCD in the Atlanta area. Methods The SCD Program at Children's Healthcare of Atlanta (CHOA) provides comprehensive outpatient, emergency and inpatient services at 3 locations in metropolitan Atlanta. CHOA's Sickle Cell Clinical Database (SCCD) contains prospectively collected demographic, diagnostic, treatment and other clinical information on all patients with SCD beginning in 2010, including all outpatient clinic, emergency department (ED) and inpatient hospital utilization. To assess the impact of COVID-19 on healthcare utilization, we tracked clinic, ED and inpatient utilization for the 4-month period (March through June) 2020 compared with the same 4-month period in 2018 and 2019. Results The figure shows utilization patterns for each four-month period from 2018-2020. As expected, face to face outpatient clinic visits fell dramatically from February to April 2020 (-25% in March, -64% in April) and then returned to pre-COVID levels by June. The addition of telemedicine visits raised total outpatient visits in June 2020 to above pre-COVID levels. Total utilization during the 4-month period in 2020 were compared to the mean for the same periods in 2018 and 2019. Face to face clinic visits decreased from 2971.5 to 2023 (-32%), ED visits from 1,217 to 687 (-44%), and total inpatient admissions from 699 to 410 (-41%). Admissions with a primary discharge diagnosis of pain decreased from a mean of 407 in 2018-2019 to 173 (-57%), fever/infection from 67.5 to 40 (-41%), and ACS from 101 to 75 (-26%). Patients with chronic pain and/or history of high utilization (>5 admissions in a given year) showed decreases in utilization similar to all other patients. Summary These data describe the significant changes in utilization among pediatric patients with SCD during the COVID-19 pandemic. Face to face outpatient clinic visits decreased during March and April but returned to pre-COVID levels in June. Unexpectedly, ED and inpatient hospital utilization for acute illness decreased dramatically through April and remained low through June. In March there was a significant decrease in the clinic setting due to a large number of cancelled or rescheduled outpatient visits, despite many being rescheduled as telemedicine visits. However, the largest unexpected decrease was seen in emergency department visits and hospitalizations for acute events, specifically fever and pain events. It is also important to note the decreased utilization of patients with chronic pain who are typically high utilizers. During clinic encounters, families mentioned that less stress from school, reduced respiratory infections, and better medication adherence with parents at home, were possible contributors to reduced sickle cell symptoms while sheltering in place. These observations will guide the development of a patient survey with the goal of obtaining qualitative data to explain the reasons for decreased utilization during the pandemic. Figure Disclosures Lane: FORMA Therapeutics: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees.

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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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Frailty status changes are associated with healthcare utilization and subsequent mortality in the elderly population

BMC Public Health ◽

10.1186/s12889-021-10688-x ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Chia-Ming Li ◽

Chih-Hsueh Lin ◽

Chia-Ing Li ◽

Chiu-Shong Liu ◽

Wen-Yuan Lin ◽

...

Keyword(s):

Healthcare Utilization ◽

Outpatient Clinic ◽

Reference Group ◽

National Database ◽

Care Utilization ◽

Frailty Phenotype ◽

Frailty Status ◽

Outpatient Visits ◽

Clinic Visits

Abstract Background This study determined (1) whether a change in frailty status after a 1 year follow up is associated with healthcare utilization and evaluated (2) whether a change in frailty status after a 1 year follow up and health care utilization are associated with all-cause mortality in a sample of Taiwan population. Methods This work is a population-based prospective cohort study involving residents aged ≥65 years in 2009. A total of 548 elderly patients who received follow-ups in the subsequent year were included in the current data analysis. Fried frailty phenotype was measured at baseline and 1 year. Information on the outpatient visits of each specialty doctor, emergency care utilization, and hospital admission during the 2 month period before the second interview was collected through standardized questionnaires administered by an interviewer. Deaths were verified by indexing to the national database of deaths. Results At the subsequent 1 year follow-up, 73 (13.3%), 356 (64.9%), and 119 (21.7%) elderly participants exhibited deterioration, no change in status, and improvement in frailty states, respectively. Multivariate logistic analysis showed the high risk of any type of outpatient use (odds ratios [OR] 1.94, 95% confidence interval [CI] 1.02–3.71) among older adults with worse frailty status compared with those who were robust at baseline and had unchanged frailty status after 1 year. After multivariate adjustment, participants with high outpatient clinic utilization had significantly higher mortality than those with low outpatient clinic visits among unchanged pre-frail or frail (hazard ratios [HR] 2.79, 95% CI: 1.46–5.33) and frail to pre-frail/robust group (HR 9.32, 95% CI: 3.82–22.73) if the unchanged robustness and low outpatient clinic visits group was used as the reference group. Conclusions The conditions associated with frailty status, either after 1 year or at baseline, significantly affected the outpatient visits and may have increased medical expenditures. Combined change in frailty status and number of outpatient visits is related to increased mortality.

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Adherence with Hydroxyurea in Children with Sickle Cell Disease

Blood ◽

10.1182/blood.v112.11.167.167 ◽

2008 ◽

Vol 112 (11) ◽

pp. 167-167 ◽

Cited By ~ 1

Author(s):

Courtney D. Thornburg ◽

Agustin Calatroni ◽

Brittany Herzberg ◽

Marilyn J. Telen ◽

Alex Kemper

Keyword(s):

Sickle Cell Disease ◽

Treatment Adherence ◽

Sickle Cell ◽

Poor Response ◽

Cell Disease ◽

Good Adherence ◽

Hematologic Response ◽

The Impact ◽

Morisky Scale ◽

Clinic Visits

Abstract Hydroxyurea is the only safe and efficacious medication to prevent complications of sickle cell disease (SCD) in children. Unfortunately, hydroxyurea requires daily dosing and frequent drug monitoring. Our objectives were to assess adherence to hydroxyurea, the impact of adherence on hematologic response, and whether we could identify those children at greatest risk for non-adherence for a future intervention. Between 2006 and 2008 children with SCD who had been taking hydroxyurea for ≥5 months were enrolled in an IRB-approved study of treatment adherence. Because adherence is difficult to measure, we used multiple estimates: attendance at clinic visits (visit within 0.5 month of recommended time frame indicating good adherence); caregiver-report with a previously validated instrument (the Modified Morisky Scale; range 0–4; ≤1 indicating good adherence) and with a visual analog scale (VAS) developed for this project (>75% of doses within the last 3–4 weeks indicating good adherence); provider estimates of adherence completed by the physician or physician extender seeing the patient in clinic (“often” or “always” adherent indicating good adherence); and pharmacy prescription refills (≥5 month-supply refills in the six months prior to enrollment indicating good adherence). Caregivers also completed a written survey regarding hydroxyurea utilization. Eighty-three children with SCD enrolled in the study (46 males and 37 females, ages 3.5–17.8 years). The average duration of hydroxyurea therapy at study entry was 4.6 years (range 0.4–11.3). The mean dose at the time of the study was 24.2 mg/kg/day (range 16.6–31.6). Between hydroxyurea initiation and enrollment in this study hemoglobin increased by 1.3 g/dL (95% CI: 1.1–1.5; p<0.0001); mean corpuscular volume increased by 14 fL (95% CI: 10–17; p<0.0001); and %HbF increased by 7.7% (95% CI: 6.0–9.4; p<0.0001). Eighty-nine percent of caregivers rated hydroxyurea as very valuable. More than 90% of parents agreed that hydroxyurea prevents pain and helps their child be more active, lead a more normal life and go to school. Good adherence was estimated at 82% by VAS; 85% by Morisky score; 84% by medical provider report; 77% by clinic visits; and 46% based on pharmacy refills. Increase in hemoglobin was significantly associated with good adherence as measured by the parent/proxy Morisky score (r= −.26; p=0.02) and prescription refills (r =0.30; p<0.01) when adjusting for dose, age at enrollment and length of treatment. The number of pharmacy refills and the Morisky score explained approximately 29% of the variation in hemoglobin response. In summary, families value hydroxyurea and believe that hydroxyurea provides significant benefits. The estimate of adherence was >75% by 4 out of 5 measures. However, the estimate based on pharmacy refills was <50%. Pharmacy refills may underestimate adherence due to extra stock of drug at home or drug dispensed during hospitalization. Alternatively, patients may not fill prescriptions even though they attend clinic visits and report adherence. Although there was an overall significant hematologic response to hydroxyurea, the response was variable. Given that only 29% of the hematologic response was explained by adherence, there are likely multiple pharmacodynamic factors which contribute to response variability. Pharmacy refills and Modified Morisky Scale may be used to identify: children at high risk for poor response due to non-adherence and children with good adherence with poor response due to individual pharmacodynamics. Future hydroxyurea effectiveness research should include improvements in disease specific adherence measures, clinical measures of effectiveness and methods to improve treatment adherence.

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Clinical and Biomarker Predictors for Avascular Necrosis in Sickle Cell Disease

Blood ◽

10.1182/blood-2021-147829 ◽

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 < 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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Sickle Cell Disease Model Mice Lacking 2,3-Dpg Show Reduced RBC Sickling and Improvements in Markers of Hemolytic Anemia

Blood ◽

10.1182/blood-2020-142052 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 27-28

Author(s):

Kelly M. Knee ◽

Amey Barakat ◽

Lindsay Tomlinson ◽

Lila Ramaiah ◽

Zane Wenzel ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Hemolytic Anemia ◽

Sickle Cell ◽

Red Blood Cell ◽

Organ Damage ◽

Complete Elimination ◽

Cell Disease ◽

The Impact ◽

2,3 Dpg

Sickle cell disease (SCD) is a severe genetic disorder caused by a mutation in hemoglobin (b6Glu-Val), which allows the mutant hemoglobin to assemble into long polymers when deoxygenated. Over time, these polymers build up and deform red blood cells, leading to hemolytic anemia, vaso-occlusion, and end organ damage. A number of recent therapies for SCD have focused on modulating the mutant hemoglobin directly, however, reduction or elimination of 2,3-DPG to reduce Hb S polymerization and RBC sickling has recently been proposed as a therapeutic strategy for SCD. Current clinical studies focus on activation of pyruvate kinase to reduce 2,3-DPG, however, direct targeting of the enzyme which produces 2,3-DPG; Bisphosphoglycerate Mutase (BPGM) may also be possible. In this study we evaluate the impact of elimination of 2,3-DPG on SCD pathology by complete knockout of BPGM in Townes model mice. Animals with complete knockout of BPGM (BPGM -/-) have no detectable 2,3-DPG, while animals that are heterozygous for BPGM (BPGM -/+) have 2,3-DPG levels comparable to Townes mice. Western Blot analysis confirms that BPGM -/- animals completely lack BPGM, while BPGM -/+ animals have BPGM levels that are nearly equivalent to Townes mice. As expected from the lack of 2,3-DPG, BPGM -/- animals have increased oxygen affinity, observed as a 39% decrease in p50 relative to Townes mice. Complete elimination of 2,3-DPG has significant effects on markers of hemolytic anemia in BPGM -/- mice. Mice lacking 2,3-DPG have a 60% increase in hemoglobin (3.7 g/dL), a 53% increase in red blood cell count, and a 29% increase in hematocrit relative to Townes mice. The BPGM -/- mice also have a 57% decrease in reticulocytes, and a 61% decrease in spleen weight relative to Townes animals, consistent with decreased extramedullary hematopoiesis. Consistent with the reduction in hemolysis, BPGM -/- animals had a 59% reduction in red blood cell sickling under robust hypoxic conditions. BPGM -/+ animals had hemoglobin, RBC, and hematocrit levels that were similar to Townes animals, and a similar degree of RBC sickling to Townes mice. Liver phenotype was similar across all variants, with areas of random necrosis observed in BPGM -/-, BPGM -/+ and Townes mice. Higher percentages of microcytic and/or hyperchromic RBCs were observed in BPGM -/- animals relative to BPGM -/+ or Townes animals. These results suggest that modulation of 2,3-DPG has a positive effect on RBC sickling and hemolytic anemia, which may have therapeutic benefits for SCD patients. However, the lack of improvement in organ damage suggests that modulation of 2,3-DPG alone may not be sufficient for complete elimination of SCD phenotypes, and further investigation of this therapeutic avenue may be necessary. Disclosures No relevant conflicts of interest to declare.

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10-Year Mortality in Patients with Sickle Cell Disease from a Large Population-Based Cohort

Blood ◽

10.1182/blood-2020-141613 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 56-56

Author(s):

Kristina Lai ◽

Marianne McPherson Yee ◽

Beatrice Gee ◽

Maa-Ohui Quarmyne ◽

Ross M. Fasano ◽

...

Keyword(s):

Mortality Rate ◽

Board Of Directors ◽

Sickle Cell ◽

Bacterial Infections ◽

Mortality Rates ◽

Population Based ◽

Cell Disease ◽

Adult Care ◽

Advisory Committees ◽

Hb S

Background Mortality rates and causes of death in children with sickle cell disease (SCD) have changed significantly over the past several decades. With ongoing improvements in standards of care, modern mortality estimates must be updated. Children's Healthcare of Atlanta (CHOA) houses one of the largest pediatric SCD programs in the country. This analysis reviews mortality in children with SCD who were treated at CHOA between June 2010-June 2020. Methods We reviewed the CHOA's Sickle Cell Clinical Database (SCCD) for deceased patients. Demographics, SCD genotype, date and age at death, healthcare utilization, hydroxyurea (HU) use, chronic transfusion therapy (CTT), and bone marrow transplant (BMT) were obtained from the SCCD. Cause of death and history of significant comorbidities were abstracted from the medical record. Mortality rates were calculated using person-time for all CHOA SCD patients and population-based mortality was obtained from CDC WONDER Online Database. Results A total of 3,698 patients with SCD were seen at CHOA from June 2010-June 2020 and accounted for 19,998 person-years. Of the 46 patients who died during that time, all but 1 patient had been seen in the CHOA Sickle Cell Outpatient Clinic at least once. That patient received SCD care at a different institution, died from complications of a non-SCD related disease following transfer to CHOA, and was excluded from analysis. Of the 45 remaining patients (178 person-years), the majority were sickle cell anemia genotypes (n=37, 82%; Hb SS or Hb S β0 thalassemia), followed by Hb SC (n=5, 11.1%) and Hb S β+ thalassemia (n=3, 6.7%); 53% (n=24) were female. The average age at death was 12.8 years (1.0-22.8 years). Twenty-one (46.7%) patients had ever been treated with HU and 11 (24.4%) were currently on HU at the time of last contact (either death or last CHOA encounter). Eleven (24.4%) patients had ever been treated with CTT, and 3 (8.9%) were being treated with CTT at time of last contact. In comparison, during the same time period an average of 58% and 12% of the overall CHOA SCD population were being treated with HU and CTT, respectively. Forty-one patients had at least 1 SCD-related encounter at CHOA within 12 months prior to death. For the 4 patients who had not been seen at CHOA within 12 months, the average time since last contact was 1.72 years (1.03 - 2.7 years). Eighty percent (n=8) of the 10 patients who died at age >19 had either recently transitioned to adult care or had documentation of a transition plan. During this 10-year period, the crude death rate was 2.3 per 1,000 person-years. The majority of deaths (62%, n=28) were attributable SCD-related causes and corresponded to a cause-specific mortality rate of 1.40 per 1,000 person-years. The remaining 38% (n=17) of deaths were caused by complex non-SCD comorbidities or accidental trauma and corresponded to a non-SCD related mortality rate of 0.85 per 1,000 person-years. In comparison, the mortality rate for African American persons age <22 in Georgia from 2009-2018 was 0.9 per 1,000 person-years. Of the 28 patients who died due to an SCD-related cause: 12 (27%) experienced an acute illness related to SCD, 4 (9%) succumbed to acute bacterial infections, 3 (7%) died from complications of SCD treatment (1 procedure-related, 2 drug-induced hemolytic anemia), and 1 (2%) died from complications of BMT. Nine (20%) had either recently transitioned to adult care or were lost to follow-up and had no significant non-SCD comorbidities, therefore cause of death was unknown. Of the 17 non-SCD related deaths, 16 (36%) were due to complex non-SCD comorbidities (including cancers, autoimmune disorders, and congenital heart disease) and 1 (2%) from an accidental trauma. Conclusions To our knowledge, this is the largest and most recent study of mortality in patients with SCD from a single institution. Overall, the demographics of deceased patients were similar to those of the CHOA SCD population as a whole. This cohort indicates that trends in mortality of children with SCD have largely shifted away from bacterial infections to other complications of SCD, subsequent treatments, or comorbidities. Our data confirm that patients nearing transition to adult care are at high risk of mortality. This study is limited to deaths that are known to the CHOA system, so future analyses will expand this cohort to include data from death certificates and CDC's National Death Index. Disclosures Lane: FORMA Therapeutics: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees.

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Social Work Perspective

PEDIATRICS ◽

10.1542/peds.83.5.903 ◽

1989 ◽

Vol 83 (5) ◽

pp. 903-905

Author(s):

Sandra Hernandez

Keyword(s):

New York ◽

Chronic Illness ◽

Sickle Cell Disease ◽

Newborn Screening ◽

Sickle Cell ◽

New York State ◽

Early Years ◽

Cell Disease ◽

York State ◽

The Impact

The ultimate objective of newborn screening for sickle cell disease should be twofold. The first essential step is the identification of the infants at risk. This has been effectively done in New York state as of 1975 through the New York State Newborn Screening Program. However, identifying these children is not enough. Second is the much more complicated task of providing comprehensive follow-up care for families whose children are affected by the disease, including the much needed psychosocial services. This area continues to be sorely neglected. The increased risk of death due to overwhelming infection in the first 3 years of life for children with sickle cell disease has been noted in the literature. When there is no specialized care, 15% to 20% do not survive. Therefore, it is essential for knowledgeable staff to make contact and begin to develop a trusting relationship as soon as possible with parents of infants born with sickle cell disease. Prophylactic penicillin and pneumococcal vaccination can reduce mortality during the early years. Family involvement with a consistent, available team of health care providers is pivotal in understanding this chronic illness and coping effectively with this extraordinary stress. Our staff is available by telephone for consultations with patients or other medical staff during clinic and emergency room visits and hospitalizations. One element that is clear in our experience at the St Luke's-Roosevelt Hospital Sickle Cell Center in New York City is that adjustment to this chronic illness is a lifelong process. One or two counseling sessions at the time of diagnosis are not sufficient to enable families to fully understand the information given or to realize the impact of having a child with a chronic illness.

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Global geographic differences in healthcare utilization for sickle cell disease pain crises in the CASiRe cohort

Blood Cells Molecules and Diseases ◽

10.1016/j.bcmd.2021.102612 ◽

2021 ◽

pp. 102612

Author(s):

Crawford Strunk ◽

Immacolata Tartaglione ◽

Connie M. Piccone ◽

Raffaella Colombatti ◽

Biree Andemariam ◽

...

Keyword(s):

Sickle Cell Disease ◽

Healthcare Utilization ◽

Sickle Cell ◽

Cell Disease ◽

Geographic Differences

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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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Hydroxyurea can be used in children with sickle cell disease and cerebral vasculopathy for the prevention of chronic complications? A meta-analysis

Journal of Child Health Care ◽

10.1177/1367493518814922 ◽

2019 ◽

Vol 24 (1) ◽

pp. 64-77

Author(s):

Ramiro Manzano Núñez ◽

Carlos Andrés Portilla Figueroa ◽

Herney Andrés García-Perdomo

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Lower Risk ◽

Meta Analysis ◽

Risk Difference ◽

Cell Disease ◽

High Quality Research ◽

Abnormal Hemoglobin ◽

High Concentrations ◽

The Impact

We conducted a systematic review for evaluating the impact of hydroxyurea and chronic blood transfusion in children with sickle cell disease (SCD). A search was done in four databases from inception to 2017. Trials enrolling pediatric patients with SCD and cerebral vasculopathy with or without previous episode of stroke and that reported outcomes of occurrence of stroke and other events were included. Trained reviewers determined eligibility, risk of bias, and abstracted data. Random-effects meta-analysis was conducted. We found that the primary outcome was the occurrence of stroke. We found two trials that recruited 254 patients. No difference was found for confirmed stroke occurrence (risk difference 0.04 [95% CI: −0.03 to 0.03]) and for new-onset neurological deficit (risk difference 0.11 [95% CI: −0.00 to 0.21]). Transfusions provided a significant lower risk of vaso-occlussive crisis (risk difference 0.10 [95% CI: 0.001 to 0.20]). Finally, transfusions provided a lower risk of having high concentrations of abnormal hemoglobin S (mean difference 37.94 [95% CI: 27.55 to 48.32]). As a conclusion, transfusions plus chelation therapy might be used instead of hydroxyurea in children with SCD. There is a lack of high-quality research in the care of children with SCD, and therefore a call for action is needed.

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