Sickle Cell Awareness Month: Part II: For Patients in Pain, Being Believed Can Be Part of the Treatment

September is Sickle Cell Awareness Month, an effort to educate the public about an inherited blood disorder that disproportionately affects Black Americans and remains, for most patients, a lifelong illness without a widely accessible cure.

For people living with sickle cell disease, a trip to the emergency room during a pain crisis can become a medical emergency within a medical emergency.

The first emergency is the pain itself.

The second can be convincing someone that the pain is real.

As September marks Sickle Cell Awareness Month, patients, physicians and advocates are calling attention not only to advances in treating the inherited blood disorder, but also to a persistent obstacle that can interfere with care: the stigma surrounding opioid pain medication and the perception that some patients are “drug-seeking.”

That perception can have serious consequences for people experiencing a vaso-occlusive crisis, one of the most painful and common complications of sickle cell disease.

Understanding the pain

Sickle cell disease causes red blood cells to become hard, sticky and shaped differently from healthy round blood cells. Those abnormal cells can obstruct blood flow, depriving tissues of oxygen and triggering sudden episodes of severe pain.

The pain can occur in the chest, back, arms, legs, abdomen and other parts of the body. A crisis may last hours or days and can become severe enough to require emergency treatment or hospitalization.

For patients experiencing severe acute pain, opioids can be an appropriate part of treatment. The need for those medications, however, can place sickle cell patients at the intersection of two significant health care issues: chronic pain and the national concern over opioid misuse.

That can result in suspicion replacing compassion.

Research presented at the American Society of Hematology's 2025 annual meeting examined 398,895 emergency department visits for vaso-occlusive crises between 2019 and 2024 in which patients received at least one opioid medication. Researchers found the first opioid dose was administered within 60 minutes in only 32.5% of visits. Patients younger than 19 received timely treatment in 52% of visits, compared with just 30% among older patients.

The findings are particularly significant because sickle cell pain can intensify rapidly. Delaying adequate pain relief can prolong suffering and complicate an already frightening medical experience.

When a label follows the patient

Another study raises questions about what happens before medication is even administered — how health care professionals perceive and describe sickle cell patients.

A University of Chicago study published April 13 in JAMA Network Open examined 39,871 clinician notes involving 18,326 patients. Researchers used natural language processing and machine learning to identify negative descriptions such as “aggressive,” “angry,” “nonadherent,” “noncompliant,” “noncooperative” and “refuse.”

The researchers found patients with sickle cell disease had 2.46 times the adjusted odds of having a negative descriptor in their medical notes compared with Black patients without sickle cell disease. They also had higher odds than patients with chronic pain who did not have sickle cell disease.

The study suggests that the problem cannot be explained by race alone. Researchers concluded that racial bias can be compounded by disease-related stigma surrounding chronic pain and opioid treatment.

That distinction matters.

A patient requesting the medication that has previously controlled a sickle cell crisis should not automatically be viewed as someone seeking drugs for nonmedical purposes.

A patient who knows which medication and dosage worked during previous crises may simply know their disease.

A patient crying, pacing, becoming frustrated or repeatedly asking when medication is coming may be responding to extraordinary pain.

The challenge for health care professionals is to distinguish substance use disorder from legitimate pain management needs without allowing assumptions or stigma to determine care.

Treatment goes beyond the emergency room

Modern sickle cell treatment is not limited to controlling pain during a crisis. Long-term care can involve medications intended to reduce complications, preventive care, blood transfusions for some patients and coordinated treatment by hematologists and other specialists.

Hydroxyurea remains an important disease-modifying therapy for many patients with sickle cell disease, and researchers continue to study ways to improve long-term adherence to the medication.

The larger goal is to reduce the frequency and severity of complications so patients are not forced to depend on emergency departments as their primary source of care.

That requires continuity.

Ideally, patients should have access to physicians and treatment teams familiar with sickle cell disease, individualized pain-management plans and medical records that clearly communicate what has worked during previous crises.

Emergency departments also can benefit from standardized protocols that reduce the possibility that treatment will depend on an individual provider's perception of whether a patient “looks” as though they are in enough pain.

Pain is subjective. Sickle cell pain is no exception.

Georgia takes action

Georgia lawmakers have also acknowledged the need for greater attention to sickle cell treatment.

House Bill 334, known as the Sickle Cell Disease Protection Act, requires the Georgia Department of Community Health to conduct an annual review of medications and treatments for sickle cell disease available to Medicaid recipients, provide opportunities for public input and issue an annual report.

Such efforts are particularly important because managing sickle cell disease requires more than treating individual episodes. Patients may need primary care physicians, hematologists, emergency physicians, pharmacists, nurses, mental health professionals and other specialists working together.

Changing the culture of care

Medical treatment alone cannot solve every problem surrounding sickle cell disease.

The culture surrounding treatment must change as well.

Clinicians should be aware that language entered into an electronic health record can influence how the next doctor, nurse or other health professional views a patient. Calling someone “noncompliant” without explaining why a treatment was not followed can create a lasting impression that extends well beyond a single hospital visit.

The University of Chicago researchers said clinicians should seek to understand why patients are having difficulty following treatment plans rather than perpetuating negative labels. They also called for interventions addressing both race-based and disease-related biases.

For patients, individualized care plans can help. Patients and families can work with their hematologists or sickle cell specialists to document medications, previous complications and effective pain-management strategies before the next emergency occurs.

For health systems, training should include more than understanding the biology of sickle cell disease. It should address implicit bias, opioid stigma, communication and the unique experience of people who may have spent a lifetime repeatedly entering emergency rooms in severe pain.

And for the public, Sickle Cell Awareness Month provides an opportunity to understand something patients have been saying for generations:

The pain is real.

The disease is real.

And asking for relief from excruciating pain should not automatically make someone a suspect.

A person experiencing a sickle cell crisis should not have to prove their character before receiving treatment.

They should be treated as a patient.