Sickle cell disease (SCD) affects approximately 100,000 people in the United States (US) and accounts for 75,000 hospitalizations and $475 million in hospitalization costs per year. There is little information about which physician specialists provide the majority of outpatient care and their prescribing practices for SCD. Many suspect much of adult SCD outpatient care is provided by emergency medicine physicians because of lack of access to high quality care from hematologists and primary care. Here, we evaluate a facet of care in SCD by evaluating the outpatient medications frequently prescribed to SCD patients and the physician specialties most involved in writing those prescriptions.

Methods: We used the National Disease and Therapeutic Index (NDTI), a nationally representative audit of outpatient visits in the continental US conducted by IMS Health. The survey includes approximately 4100 sampled physicians each calendar quarter who provide information about every clinical encounter during 2 consecutive workdays. Physicians are selected by random-stratified sampling by specialty and geographic region from the master lists of the American Medical Association and the American Osteopathic Association. The complex sampling frame allows extrapolation to national estimates for outpatient visits and associated prescriptions. Data for each visit include patient diagnoses based on codes from the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM), and medications prescribed during these visits. The complex sampling frame allows extrapolation to national estimates for office visits and associated prescriptions. We analyzed medications prescribed for the years 2012 through 2015 for patients with SCD. Our analyses included all visits by patients with a recorded SCD diagnosis (International Classification of Diseases, Ninth Revision [ICD-9] codes 282.41-282.42, 282.60-282.64, 282.68, or 282.69). We used descriptive statistics to examine the counts of prescriptions. We aggregated quarterly data from 2012 to 2015 to present annual trends. With linear regression analysis, we assessed the rate of change of prescriptions each year.

Results: Outpatient visits by patients with SCD increased steadily from 279K (thousand) in 2012 to 433K in 2015 with an average percent increase of 16.0% per year. Hematologists, both pediatric and adult provided, on average, 157K outpatient visits annually and had the highest number of patient visits for SCD compared to all other specialties (Table 1). Of all emergency medicine visits, 87.4% occurred in patients over 20 years of age, while 65.0% of all hematology visits were from patients under 20. Over the study period, the most frequently prescribed drugs out of all drugs prescribed to patients with SCD were opioids (26.4%), folic acid (15.7%), hydroxyurea (12.7%), and antibiotics (11.0%). Opioid prescriptions increased by 55K prescriptions each year (p = 0.016), from 100K in 2012 to 260K in 2015. Adult and pediatric hematologists (37.2% of total opioid prescriptions), emergency medicine (26.6%), adult primary care medicine (20.8%), and pediatrics (8.2%) prescribed opioids the most frequently of all clinicians. The proportion of visits with opioid prescriptions was highest in emergency medicine visits compared to all other specialties (Table 2). Hydroxyurea prescriptions increased by 26K per year (p=0.04) and grew from 39K in 2012 to 154K in 2015. The yearly increase of hydroxyurea prescriptions was statistically significant for patients between the ages of 3 to 9 years (15K per year; p = 0.04), but was not statistically significant for other age groups.

Conclusion: Opioids were the most frequently prescribed medication during visits for people with SCD and one-quarter of these were prescribed by an emergency medicine provider. That more adults receive care in the emergency department and that more children receive care at hematology visits reinforces concerns about access to care for the adult population with SCD. Future studies are needed to understand the reasons patients are receiving a large number of opioid prescriptions in the emergency department and whether improved access to outpatient subspecialty care could decrease this practice.


Lanzkron: Prolong: Research Funding; Bayer: Research Funding; HRSA: Research Funding; Pfizer: Research Funding; Global Blood Therapeutics: Research Funding; PCORI: Research Funding. Segal: PCORI: Research Funding.

Author notes


Asterisk with author names denotes non-ASH members.

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