Emergency Department Visits Made by Patients with Sickle Cell Disease: A Descriptive Study, 1999–2007

doi:10.1016/j.amepre.2010.01.001

American Journal of Preventive Medicine

Volume 38, Issue 4, Supplement, April 2010, Pages S536-S541

https://doi.org/10.1016/j.amepre.2010.01.001 Get rights and content

Background

Patients with sickle cell disease (SCD) often use emergency department services to obtain medical care. Limited information is available about emergency department use among patients with SCD.

Purpose

This study assessed characteristics of emergency department visits made nationally by patients with SCD.

Methods

Data from the National Hospital Ambulatory Medical Care Survey (NHAMCS) for the years 1999–2007 were analyzed. The NHAMCS is a survey of hospital emergency department and outpatient visits. Emergency department visits by patients with SCD were identified using ICD-9-CM codes, and nationally weighted estimates were calculated.

Results

On average, approximately 197,333 emergency department visits were estimated to have occurred each year between 1999 and 2007 with SCD as one of the diagnoses listed. The expected source of payment was private insurance for 14%, Medicaid/State Children's Health Insurance Program for 58%, Medicare for 14%, and other/unknown for 15%. Approximately 29% of visits resulted in hospital admission; this was 37% among patients aged 0–19 years, and 26% among patients aged ≥20 years. The episode of care was indicated as a follow-up visit for 23% of the visits. Patient-cited reasons for the emergency department visit included chest pain (11%); other pain or unspecified pain (67%); fever/infection (6%); and shortness of breath/breathing problem/cough (5%), among other reasons.

Conclusions

Substantial numbers of emergency department visits occur among people with SCD. The most common reason for the emergency department visits is pain symptoms. The findings of this study can help to improve health services delivery and utilization among patients with SCD.

Introduction

Sickle cell disease (SCD) is an inherited blood disorder characterized by the production of an altered type of hemoglobin by those affected by the disease. When sickle cell hemoglobin deoxygenates while passing through blood vessels, it polymerizes and becomes fibrous, causing the red blood cell to become rigid and change shape so that it appears sickle-shaped. The altered red blood cells can occlude blood vessels, resulting in lack of oxygen to tissues, and pain. Repeated, unpredictable episodes of acute pain are a complication of SCD that affects a substantial number of patients with the disease.1, 2 Other complications of SCD are increased susceptibility to life-threatening infections, especially among children; stroke; pulmonary complications; skin ulceration; and other deleterious health events. Vaso-occlusive episodes result in tissue and end-organ injury, which could have damaging effects on the health of people with SCD. Because of SCD-related complications, many people with SCD may make more- frequent medical care visits than others among the general population. Comparatively higher levels of hospitalization and emergency department visits among SCD patients than other selected groups have been previously reported.3, 4, 5 A study conducted using data from Tennessee's state-funded managed care program found that black people with SCD made two to six times more emergency department visits than blacks without SCD.³

Limited information is available related to characteristics of emergency department visits among SCD patients. Information related to emergency department use by patients with SCD can help to develop services and strategies for best meeting the healthcare needs of patients with SCD. Various factors may affect healthcare access and utilization patterns among patients, such as patients' perception, insurance, availability of transportation, and disease-related factors. For example, Logan et al.⁶ found that factors related to healthcare utilization among adolescents included parental knowledge about SCD and disease severity. The objective of the current study was to describe emergency department utilization by patients with SCD nationally. The number of such visits and associated patient and other characteristics were assessed.

Section snippets

Methods

Data were from the National Hospital Ambulatory Care Surveys (NHAMCS) from 1999 to 2007. This survey is conducted by the CDC, National Center for Health Statistics. The survey collects patient visit–related information from a national probability sample of emergency department and outpatient visits to short-stay hospitals. A multistage cluster sample design is used to select hospitals from the states and the District of Columbia. Federal, military, and Veterans Administration hospitals,

Results

From 1999 to 2007, the NHAMCS had a combined sample of 300,394 emergency department visits. This number represents a weighted estimate of approximately 1 billion (95% CI=933,758,000, 1,073,966,000) emergency department visits occurring nationally during those years. The total numbers of emergency department visits in the sample with a primary diagnosis of SCD and any diagnosis of SCD were 415 and 502, respectively. These numbers represent weighted estimates of approximately 1,475,000 (95%

Discussion

Information on emergency department utilization among patients with SCD can be helpful in developing strategies for improving health services and health outcomes, such as through their use in the planning of services and improving effectiveness. The findings in this study indicated that a substantial number of emergency department visits are made annually by patients with SCD. These findings are consistent with previous studies that have reported heavy use of emergency departments among SCD

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Cited by (163)

Self-reported pain levels for emergency department visits associated with sickle cell disease in the United States
2024, Blood Advances
Characteristics of Emergency Department Visits Made by Individuals With Sickle Cell Disease in the U.S., 1999–2020
2024, AJPM Focus
Individuals living with sickle cell disease experience high levels of morbidity that result in frequent utilization of the emergency department. The objective of this study was to provide updated national estimates of emergency department utilization associated with sickle cell disease in the U.S.
Data from the National Hospital Ambulatory Medical Care Survey for the years 1999–2020 were analyzed. Complex survey analysis was utilized to produce national estimates overall and by patient age groups.
On average, approximately 222,612 emergency department visits occurred annually among individuals with sickle cell disease, a nearly 13% increase from prior estimates. The annual volume of emergency department visits steadily increased over time, and pain remains the most common patient-cited reason for visiting the emergency department. Patient-reported pain levels for individuals with sickle cell disease were high, with 64% of visits associated with severe pain and 21% associated with moderate pain. Public insurance sources continue to cover most visits, with Medicaid paying for 60% of visits and Medicare paying for 12% of visits. The average time spent in the emergency department increased from previous estimates by about an hour, rising to approximately 6 hours. The average wait time to see a provider was 53 minutes.
Utilization of the emergency department by individuals living with sickle cell disease remains high, especially for pain. With more than half of patients with sickle cell disease reporting severe pain levels, emergency department staff should be prepared to assess and treat sickle cell disease–related pain following evidence-based guidelines and recommendations. The findings of this study can help improve care in this population.
Society for Maternal-Fetal Medicine Consult Series #68: Sickle cell disease in pregnancy
2024, American Journal of Obstetrics and Gynecology
Pregnant individuals with sickle cell disease have an increased risk of maternal and perinatal morbidity and mortality. However, prepregnancy counseling and multidisciplinary care can lead to favorable maternal and neonatal outcomes. In this consult series, we summarize what is known about sickle cell disease and provide guidance for sickle cell disease management during pregnancy. The following are Society for Maternal-Fetal Medicine recommendations:
- 1.
  We recommend that patients with sickle cell disease be managed by a multidisciplinary team that includes maternal-fetal medicine, hematology (ideally a hematologist specializing in sickle cell disease), genetics, pain management, and social work or behavioral health (as appropriate) starting in early pregnancy (best practice).
- 2.
  We recommend that pregnant patients with sickle cell disease receive all routinely recommended antenatal vaccinations in addition to meningococcal and pneumococcal vaccinations if due (GRADE 1A).
- 3.
  We recommend prenatal vitamins without iron unless iron deficiency is confirmed and initiation of 4 mg of folic acid for pregnant patients with sickle cell disease (GRADE 1B).
- 4.
  We recommend fetal growth surveillance every 4 weeks beginning in the 28th week of gestation (GRADE 1C).
- 5.
  For patients with uncomplicated sickle cell disease and a normally grown fetus, we suggest weekly or twice-weekly antenatal testing beginning at 32 to 34 weeks of gestation. For patients with complicated sickle cell disease (i.e., maternal hypertension, vaso-occlusive crises, fetal growth restriction, or other coexisting complication), we suggest initiating individualized antenatal testing at diagnosis or at a gestational age when delivery would be considered if results are abnormal (GRADE 2B).
- 6.
  We recommend following evidence-based guidelines for the management of chronic and acute pain during pregnancy (best practice).
- 7.
  We suggest the use of prophylactic transfusions be individualized for high-risk patients with sickle cell disease in accordance with American Society of Hematology guidelines and directed by a hematologist and maternal-fetal medicine subspecialist in shared decision-making with the patient (GRADE 2B).
- 8.
  We recommend shared decision-making occur regarding the use of hydroxyurea in pregnancy, in conjunction with a sickle cell disease specialist and maternal-fetal medicine subspecialist, accounting for the timing of use and individual disease severity (GRADE 1C).
- 9.
  We recommend that reliable contraception be offered to patients with sickle cell disease to decrease the risk of an unintended pregnancy and associated maternal and perinatal risks (GRADE 1B).
COVID-19 outcomes in patients with sickle cell disease and sickle cell trait compared with individuals without sickle cell disease or trait: a systematic review and meta-analysis
2023, eClinicalMedicine
Clinical manifestations and severity of SARS-CoV-2 infection in individuals with sickle cell disease (SCD) and sickle cell trait (SCT) are not well understood yet.
We performed a systematic review and meta-analysis to assess COVID-19 outcomes in individuals with SCD or SCT compared to individuals without sickle cell disease or trait. An electronic search on PubMed, Embase, and Cochrane Library was performed on August 3, 2023. Two authors (IFM and ISP) independently screened (IFM and ISP) and extracted data (IFM and ILC) from included studies. Main exclusion criterion was the absence of the non-SCD/SCT group. Exposure effects for binary endpoints were compared using pooled odds ratio (OR) with 95% confidence intervals (CI). I² statistics was used to assess the heterogeneity and DerSimonian and Laird random-effects models were applied for all analyses to minimize the impact of differences in methods and outcomes definitions between studies. The overall quality of evidence was assessed using the GRADE system. Review Manager 5.4 and R software (v4.2.2) were used for statistical analyses. Registered with PROSPERO, CRD42022366015.
Overall, 22 studies were included, with a total of 1892 individuals with SCD, 8677 individuals with SCT, and 1,653,369 individuals without SCD/SCT. No difference in all-cause mortality was seen between SCD/SCT and non-SCD/SCT (OR 1.18; 95% CI 0.78–1.77; p = 0.429; I² = 82%). When considering only studies adjusted for confounders (8 studies), patients with SCD/SCT were shown to be at increased risk of death (OR 1.86; 95% CI 1.30–2.66; p = 0.0007; I² = 34%). No significant difference was seen between individuals with SCD and SCT (p = 0.863). The adjusted for confounders analysis for hospitalisation revealed higher rates for the SCD (OR 5.44; 95% CI 1.55–19.13; p = 0.008; I² = 97%) and the SCT groups (OR 1.31; 95% CI 1.10–1.55; p = 0.002; I² = 0) compared to the non-SCD/SCT population. Moreover, it was significantly higher for the SCD group (test for subgroup difference; p = 0.028).
Our findings suggest that patients with SCD or SCT may present with a higher mortality and hospitalisation rates due to COVID-19 infection.
None.
Critical Revisits Among Children After Emergency Department Discharge
2023, Annals of Emergency Medicine
Identifying higher risk groups could reveal ways to prevent critical emergency department (ED) revisits. The study objectives were to determine the rate of critical ED revisits among children discharged from the ED and to identify factors associated with critical revisits.
We performed a retrospective study using the Healthcare Cost and Utilization Project State ED Databases (SEDD) and the State Inpatient Databases (SID). We included data from 6 states from 2014 through 2017. Critical ED revisit was defined as either ICU admission or death within 3 days of the initial ED discharge. We included all patients younger than 21 years. The main outcome was the rate of critical ED revisit. We also determined the relative risk (RR) of a critical ED revisit for the most common index ED visit diagnoses. We used negative binomial regression to calculate incidence rate ratios (IRR) of a critical ED visit by pediatric volume and complex chronic conditions.
A total of 16.3 million children were discharged from an ED over the 4-year study period. There were 18,704 (0.1%) critical ED revisits, 180 (0.00001%) of whom died. Asthma (RR 2.24, 95% confidence interval [CI] [2.11 to 2.38) had the highest relative risk of a critical revisit among all ED diagnoses. Adjusting for hospital volume and patient age, patients with complex chronic conditions were also more likely to have a critical ED revisit (IRR 11.03, 95% CI, 7.76 to 15.67).
Critical revisits after ED discharge were uncommon among children in our study sample, with revisits resulting in patient death within 3 days of an ED discharge being rare. Given the short time interval between ED discharges, however, future research should focus on understanding higher risk patients among those with asthma and a history of complex chronic conditions.
A feasibility randomized controlled trial of an mHealth app vs booklets for patient-facing guidelines in adults with SCD
2023, Blood Advances
Despite the increased number of evidence-based guidelines for sickle cell disease (SCD), dissemination of evidence-based guidelines in lay language for individuals or families with SCD has not been evaluated. We conducted a feasibility randomized controlled trial to determine the acceptability of a mobile health (mHealth) app with patient-facing guidelines to improve the knowledge of individuals with SCD about SCD-specific knowledge and reduce hospitalizations. Primary outcome measures include recruitment, retention, and adherence rates. Adults with SCD were enrolled at 2 sickle cell centers between 2018 and 2022. Participants were randomized to receive either an mHealth app + booklet with patient-facing guidelines or a booklet with the guidelines alone. Participants completed surveys at baseline and a final 6-month visit. Approximately 67 of 74 (91%) agreed to participate and were randomized, with 50 of 67 (75%) completing all the study components. All participants who completed the study in the treatment arm used the app. Our results demonstrated high recruitment, retention, and adherence rate for the first randomized trial for an mHealth app with patient-facing guidelines in adults with SCD. This clinical trial was registered at https://www.clinicaltrials.gov/ as #NCT03629678.

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HemoglobinopathyEmergency Department Visits Made by Patients with Sickle Cell Disease: A Descriptive Study, 1999–2007

Background

Purpose

Methods

Results

Conclusions

Introduction

Section snippets

Methods

Results

Discussion

J Emerg Med

Blood

J Pediatr

J Emerg Nurs

Sickle cell disease

Pediatr Clin N Am

Sickle hemoglobin (Hb S) allele and sickle cell disease: a HuGE review

Am J Epidemiol

Medical care utilization and mortality in sickle cell disease: a population-based study

Am J Hematol

Comparison of costs to the health sector of comprehensive and episodic health care for sickle cell disease patients

Public Health Rep

Health care utilization and expenditures for low income children with sickle cell disease

Pediatr Blood Cancer

Parent factors and adolescent sickle cell disease, associations with patterns of health service use

J Pediatr Psychol

Ambulatory health care data

Models of comprehensive, multidisciplinary care for individuals in the U.S. with genetic disorders

Pediatrics

Hemoglobinopathy
Emergency Department Visits Made by Patients with Sickle Cell Disease: A Descriptive Study, 1999–2007