Level up your care of life-threatening sickle cell complications in the ER

08/31/2023

Emergency providers are among the most resilient and nimble in the healthcare community. They’re routinely expected to assess and treat nearly every imaginable injury and condition including the acute symptoms of sickle cell disease (SCD).¹

SCD is an inherited disorder affecting a patient’s hemoglobin. With SCD, the abnormal hemoglobin becomes rigid, which causes red blood cells to take on an irregular crescent shape and lose flexibility.²

Cells can burst due to their lack of flexibility as they travel through the blood vessels. The misshapen cells don’t last as long as normal red blood cells—and the body may not be able to make enough new ones, which can lead to anemia. Finally, cells may stick to vessel walls, block blood flow, and deprive tissue of oxygen.² This can cause vaso-occlusive crisis (VOC), a sudden and severe type of pain.^1,2

These and other acute complications have led SCD patients to rely on the expertise of emergency healthcare staff. For these emergency providers, learning more about common acute presentations and pushing back on negative stereotypes can have a huge impact in helping reduce future complications—that may have life-altering consequences.

Below, Brandon Hardesty, MD, a board-certified adult hematologist with the Indiana Hemophilia & Thrombosis Center (IHTC) in Indianapolis, shares his unique perspective on issues relating to emergency sickle cell care and commonly shared experiences among patients with SCD.

Q&A with hematologist and SCD expert Dr. Brandon Hardesty

Question: How common is SCD in the state of Indiana?

Dr. Hardesty: Nearly 1,700 Indiana residents live with SCD. Of these, 87 percent are Black or African American, and 83 percent are non-Hispanic. In 2019, 1 in every 2,608 infants born in Indiana was born with SCD. Within the Black or African American population exclusively, this number was 1 in 446.³

Question: Are there areas of Indiana with a higher rate of SCD among residents than others?

Dr. Hardesty: Yes. According to 2020 figures, while individuals living with SCD live all over the state, the majority reside in Marion and Lake Counties (63 percent). Additionally, Allen County is home to 5.8 percent of Indiana’s SCD population.³

Question: What is the life expectancy for someone with SCD?

Dr. Hardesty:  SCD was once considered a childhood disease. In fact, in the 1970s, the average life expectancy for Hoosiers living with SCD was less than 20 years. Today, 95 percent of Americans living with SCD reach 18 years.⁴

Unfortunately, the life expectancy for adults has not significantly improved over the last few decades. Persons with severe forms of sickle cell disease have a median survival into their 50s, while individuals with less severe disease typically live into their 60s. There is a mortality spike in the 20s around the time pediatric patients are transitioning to adult medicine.⁵ Some of this is thought to be due to a lack of adult hematologists caring for SCD, but other factors contribute as well.

Continued advocacy and support for Indiana SCD care, resources and programs is critical. Young sickle cell patients are living longer, but continued access to care and treatment adherence are important keys to increasing their life expectancy.

Question: What is the most common cause of SCD-related ER visits and hospitalization?

Dr. Hardesty: Vaso-occlusive crisis (VOC) is typically sudden, severe pain and is the most common complication in this population. Due to the severe nature of VOC in the chest, back and/or extremities, emergency care is necessary. Moreover, adults with SCD who require more than 3 hospitalizations for VOC in a year are at an increased risk of premature death.

Question: What should providers know when encountering a patient with suspected VOC in the ER?

Dr. Hardesty: First, ER providers should keep in mind that VOC is typically sudden and commonly experienced in the extremities, chest and back. Due to the acuity and severity of the pain, treatment with opioids (typically given IV) is indicated. It’s also important to remember that VOC can occur in the presence of other complications.

As such, the provider should initially treat pain while performing a workup and consider an evaluation for other causes of pain such as pulmonary embolus, acute chest syndrome, or other entities. Pain should be treated with opioids using individual protocols; NSAIDs can also be used, but opioids remain the primary treatment.

Question: What is the most common cause of SCD-related death?

Dr. Hardesty: Acute chest syndrome is the most common cause of SCD-related death and is the second leading reason for hospitalization in children and adults with SCD. A patient with SCD experiencing acute chest syndrome might present with a cough and shortness of breath. Necessary treatment for these patients includes antibiotics that cover pneumococcus and atypical bacteria, as well as blood transfusions and supplemental oxygen.

Question: What are other presentations of SCD complications in the ER?

Dr. Hardesty: In addition to VOC and acute chest syndrome, the below are presentations of complications ER providers might encounter:

• Fever (must be treated with antibiotics to cover pneumococcus due to functional asplenia)
• Acute kidney injury
• Acute worsening of anemia
• Hemorrhagic stroke
• Ischemic stroke
• Multisystem organ failure
• Priapism

Question: What is the most common misconception about SCD patients that can impact their care?

Dr. Hardesty: Pain is invisible, and every patient’s SCD-related pain crisis is different. When a patient with SCD visits the ER for VOC, they are often anxious about whether healthcare staff will believe their level of pain and need for pain treatment.

Opioids should be used to aggressively treat VOC pain, but there has been significant concern in the healthcare community regarding opioid treatment. A common fallacy is that there is a high rate of opioid misuse within the SCD community when—in fact—the rate of opiate use disorder among this population is low.

Between 1999 and 2013, an astounding 174,959 patients without SCD died in the US due to opioid prescription pain relievers (OPR), compared to just 95 patients with SCD. Less than 1 percent of deaths among patients with SCD due to all causes was due to OPR in the same period.⁶

Question: What can ER providers do to help improve the care of patients with SCD?

Dr. Hardesty: Some ways ER providers can continue to improve outcomes for patients with SCD:

• Truly listen to patients; they have been dealing with their SCD-related pain for decades and know their bodies well. Parents of adolescents and young adult patients can be instrumental in understanding complications, as these patients are still learning.
• Inspire positivity. Encouragement in trying times can go a long way, and positive comments can help just as much as negative comments can hurt (e.g., “You don’t look sick”).
• Be understanding when a patient with SCD says they don’t feel well. Some patients have reported healthcare staff not believing they are sick due to eating when, in fact, eating helps stave off opiate-related nausea. Additionally, many patients will use their phones to help distract them from the severe pain they are experiencing. Engaging with electronics does not indicate a lack of pain.
• Treat each patient with SCD as an individual case. Patients living with SCD are often fighting an uphill battle of disease-related complications, in addition to racial stereotypes. Providers should be aware of their own unconscious biases that may come from prior interactions. Each case should be treated as a new and unique patient.
• Encourage ongoing and timely outpatient follow-up of chronic SCD issues to help reduce the need for ER visits.

Question: Where do Indiana patients with SCD go for care?

Dr. Hardesty: There are only six SCD care clinics in the state—located in Marion, Allen, and St. Joseph counties, and two outreach clinics in Lake and Vanderburgh counties. The remaining patients are seen by local emergency departments and hematologists if they are lucky.

Clinical excellence meets personalized care in the treatment of sickle cell disease

Serving Indiana patients as one of the state’s only SCD care clinics, the Indiana Hemophilia & Thrombosis Center (IHTC) is recognized as both a leader in managing patients with SCD and a nationally and internationally known center of excellence in the overall treatment of blood disorders. Home to a highly skilled team with proven expertise, their exceptional contribution in patient care, education, and research has made IHTC the premier choice for blood disorder treatment throughout Indiana.

Give your patients an opportunity to receive the highest quality SCD care, conveniently located in the Indianapolis area. Call (317) 871-0000 for more information or to refer a patient today.