Challenges in SCD management

Healthcare professionals face a lack of support in treating and managing sickle cell disease (SCD)—the example of the emergency department (ED)

Overcrowding in the ED and lack of resources can lead to frustration for ED providers treating patients with SCD

When patients with SCD experience severe pain, they often have no option other than to seek treatment in an ED. However, EDs are designed for medical emergencies and function to prioritize access to care for those who need it most immediately. Therefore, patients with SCD who do not present overt external signs of a life threatening illness may face many challenges in the ED, including deprioritization, overcrowding in the ED, and a lack of adequate resources.1,2

In a survey of people living with SCD, regarding ED providers across several Sickle Cell Disease Implementation Consortium (SCDIC) clinic centers, more than half reported that they did not receive ED care quickly enough, with wait times exceeding more than 1 hour. Additionally, 46% and 35% also reported feeling emergency physicians and ED nurses, respectively, did not care about them.

EDs are chaotic, and providers often have partial or incomplete information regarding the treatment of SCD. The same survey showed that only 56% of ED providers confirmed a standard protocol for SCD in place at their ED. SCD education is variable across all EDs and there may be training and education gaps between nurses, physicians, and other healthcare professionals.2,3

In an attempt to provide guidance to SCD healthcare professionals, the National Heart, Lung, and Blood Institute (NHLBI) published treatment guidelines in 2014.1
Statistics showing some of the challenges of sickle cell disease management
of ED providers reported having any awareness of NHLBI’s guidelines for treating vaso-occlusive crises.
Statistics showing some of the challenges of sickle cell disease management
Statistics showing some of the challenges of sickle cell disease management

Data are from a survey-based needs assessment at several SCDIC centers. The assessment surveyed 516 adolescents and adults with SCD and 243 ED providers from 7 and 5 regions of the US, respectively.

Improving patient experience in the ED

Addressing the challenges of treating patients with SCD in the ED can start with a few considerations1,4,5:
  • Training for all ED providers on the unique clinical needs of patients with SCD
  • Education on implicit bias and how it affects care for patients with SCD
  • Education on medical mistrust and how it affects the willingness for patients to seek treatment when they need it
  • Improving access to healthcare professionals and hematologists for routine patient care
  • Encouraging patients to seek regular outpatient or telemedicine services
  • Ensuring patients receive comprehensive care that includes preventive services and counseling
Additionally, clinically accepted guidelines provide recommendations to help healthcare professionals manage the spectrum of complications related to SCD.

NHLBI Guidelines

Healthcare professionals may feel restricted in making treatment decisions for patients with SCD due to the opioid epidemic

The current opioid epidemic in the US has had a significant impact on the treatment of SCD and on healthcare professionals who treat patients with SCD. Among 243 ED providers surveyed, almost two-thirds (62%) reported that the opioid epidemic is a significant barrier to treatment. And according to a separate qualitative study consisting of 15 in-depth interviews with people living with SCD, the epidemic has added to the challenge of receiving adequate care due to restrictive opioid prescriptions and provider preoccupation with opioid monitoring over pain management.1,3,6

Pain is the leading cause of hospital visits for those living with SCD, and opioids are a common treatment option for SCD pain management. However, patients with SCD who need opioids to manage pain are often labeled as “drug-seekers” or “frequent fliers” and do not receive or have access to medication due to reluctance by providers. A review of multiple studies examining the opioid epidemic's impact on the SCD community reported that about 60% of nurses believed that people living with SCD were opioid abusers and 30% of nurses were concerned about giving high doses of opioid to people living with SCD.1,7 This stigmatization of people living with SCD leads to1:
  • Delays in treatment delivery
  • Suboptimal dosing of opioids
  • Poor SCD pain management
Despite fears around drug abuse and addiction, research on opioid use among people living with SCD shows the addiction and mortality rate for the SCD community has remained unchanged while addiction and deaths related to opioid abuse are on the rise for the general population.7

To mitigate stigma associated with patients with SCD, including the stigma around the use of opioids for pain management, healthcare professionals suggest that improved knowledge and empathy can help. Potential solutions include training on1,8:
  • CULTURAL COMPETENCY – the ability of individuals to establish effective interpersonal and working relationships by recognizing the importance of social and cultural influences on patients
  • COMMUNICATION BETWEEN PATIENT AND HEALTHCARE PROFESSIONALS – ensuring there is understanding of the patient perspective among healthcare professionals and vice versa
  • USE OF SELF-ASSESSMENTS AND PATIENT RATING INSTRUMENTS – patient-reported evaluation methods to improve provider knowledge and mitigate stigma towards patients with SCD
Currently there is guidance regarding the use of opioids for acute complications of SCD. The NHLBI 2014 guidelines recommend a more proactive approach.9 Training on these guidelines, as well as in sickle cell care in general, may help improve treatment of pain in patients with SCD.

The transition from pediatric to adult SCD care is often troubled for healthcare professionals and patients

One study shows, the mortality rate
more than doubles between the ages of







As patients with SCD grow into adulthood, new challenges arise. Now that children with SCD are living into adulthood due to better screening and preventative treatment, the transition to adult care is an essential step for people living with SCD, their caregivers, and their healthcare professionals. However, the transition from pediatric to adult care carries several issues that often result in interruption or discontinuation of the quality of care the patients received in childhood.11,12

The transition period can be a difficult time for patients, families, and healthcare professionals. The availability of healthcare professionals with SCD experience has not been able to meet the needs of the population of adults with this disease. In addition, healthcare professionals face the challenge of increases in patient mortality during this period—the mortality rate from SCD in patients aged 20 to 24 years is twice that of patients who are 15 to 19 years old.10,11,13
This rise in rate of morbidity, mortality, and acute care utilization may be due to a combination of factors that include11:
  • Limited number of adult-oriented practitioners to provide adequate and ongoing care
  • Increase in comorbidities (eg, renal and cardiac disease, iron overload, silent cerebral infarcts) as patients age
  • Behavioral and cognitive challenges that influence medication adherence
Often, patients with SCD may not immediately seek treatment after their last pediatric appointment, resulting in an interruption or even a discontinuation of care.12

Currently, there is no established metric for successful healthcare transition; however, care transition guidelines are available to help healthcare professionals transition from pediatric to adult care.

ASH Transition Toolkit

Improving awareness of SCD guidelines in order to optimize hospital resources and maintain continuity of care  

Through the use of SCD guidelines and healthcare resources (eg, individual pain protocols and electronic health records [EHRs]), there is an opportunity to improve the treatment of SCD. Sharing knowledge and treatment expectations with counterpart physicians can help to provide a smooth transition and ensure continuity of care for patients.
Abena O. Appiah-Kubi, MD

There aren't that many adult hematologists that you can easily transition young adults living with sickle cell disease over to. It is a challenging moment in life for that individual; however, healthcare institutions began to recognize this a few years ago and started to work on some more ways to help young adults living with sickle cell disease receive the care they need.”

Abena O. Appiah-Kubi, MD, MPH

Improving knowledge of updated guidelines and resources specifically for SCD can provide needed support for healthcare professionals Improving knowledge of updated guidelines and resources specifically for SCD can provide needed support for healthcare professionals

Our partnership is our strength

Let’s change the way we view SCD and work
together for better outcomes in this disease
Let’s change the way we
view SCD and work together for
better outcomes in this disease


1. Masese RV, Bulgin D, Douglas C, Shah N, Tanabe P. Barriers and facilitators to care for individuals with sickle cell disease in central North Carolina: the emergency department providers’ perspective. PLoS ONE. 14(5):e0216414. 2. Glassberg JA. Improving emergency department–based care of sickle cell pain. Hematology Am Soc Hematol Educ Program. 2017;2017(1):412-417. 3. Linton EA, Goodin DA, Hankins JS, et al. A survey-based needs assessment of barriers to optimal sickle cell disease care in the emergency department. Ann Emerg Med. 2020;76(3S):S64-S72. 4. Yusuf HR, Atrash HK, Grosse SD, Parker CS, Grant AM. Emergency department visits made by patients with sickle cell disease: a descriptive study, 1999-2007. Am J Prev Med. 2010;38(4 Suppl):S536-S541. 5. Alsan M, Wanamaker M. Tuskegee and the health of black men. Q J Econ. 2018;133(1):407-455. 6. Sinha CB, Bakshi N, Ross D, Krishnamurti L. Management of chronic pain in adults living with sickle cell disease in the era of the opioid epidemic: a qualitative study. JAMA Netw Open. 2019;2(5):e194410. 7. Nadia SR, Ballas SK. The opioid drug epidemic and sickle cell disease: guilt by association. Pain Med. 2016;17: 1793-1798. 8. Beach MC, Price EG, Gary TL, et al. Cultural competence: a systematic review of health care provider educational interventions. Med Care. 2005;43(4):356-373 9. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312(10):1033-1048. 10. Kayle M, Docherty SL, Sloane R, et al. Transition to adult care in sickle cell disease: a longitudinal study of clinical characteristics and disease severity. Pediatr Blood Cancer. 2019;66(1):e27463. 11. Inusa BPD, Stewart CE, Mathurin-Charles S, et al. Paediatric to adult transition care for patients with sickle cell disease: a global perspective. Lancet Haematol. 2020;7(4):e329-e341. 12. McLaughlin JF, Ballas SK. High mortality among children with sickle cell anemia and overt stroke who discontinue blood transfusion after transition to an adult program. Transfusion. 2016;56(5):1014-1021. 13. Kanter J, Gibson R, Lawrence RH, et al. Perceptions of US adolescents and adults with sickle cell disease on their quality of care. JAMA Netw Open. 2020;3(5):e206016.

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