Sickle Cell Disease

Researcher Ononogbu Improving Patient Outcomes through Awareness, Compassionate Care

By Kristin Mitchener

When the birth of her son revealed she was a carrier of the trait for sickle cell disease (SCD), Onye Ononogbu, Pharm.D., MPH, BCOP, was admittedly shocked, as she had gone through life believing she did not have the trait.

“Because I didn’t have sickle cell symptoms as a child, my mother mistakenly thought I didn’t have the trait,” Ononogbu said. “So, I never got screened.”

In SCD, due to a gene mutation that affects the hemoglobin molecule, red blood cells are abnormally shaped, hard, and sticky, and resemble the C-shaped farm tool called a sickle. When red blood cells sickle, they do not bend or move easily and can block blood flow to the rest of the body.

According to Ononogbu, SCD affects primarily Black Americans and Hispanics, especially in the U.S.; however, it’s prevalent in Spanish-speaking regions in the Western Hemisphere (South America, Caribbean, Central America), Saudi Arabia, India and Asia, as well as Mediterranean countries such as Turkey, Greece and Italy.

The complications of living with SCD – which include severe pain episodes, or “crises,” chronic anemia, increased risk of infection, and stroke – are exacerbated due to health care inequities and discrimination faced by SCD patients, including lack of cultural humility among providers and stereotypes about SCD patients being “drug seekers.”

Given the complexity of SCD, collaboration between hematologists, primary care providers, pharmacists, social workers and mental health professionals is essential. This ensures that all aspects of the patient's health are addressed. But holistic, comprehensive care is not always available, which is but one disparity facing SCD patients.

Lacking in literature

Federal agencies give an estimate of 100,000 people currently affected by sickle cell disease, but there is a glaring problem with this number.

“This has been the estimate for over two decades now,” said Ononogbu, research assistant professor in the college’s Department of Pharmacy Practice and Translational Research. “Through state newborn screening programs to identify life-threating diseases – one being SCD – all newborns are screened, but for those living with SCD, we really have no idea.”

There are many reasons why the sickle cell disease burden is widely unknown today, including systemic racism and health care bias. In addition, Black Americans generally have been mistreated through multiple instances of unethical medical research conducted in their communities, leading to mistrust of the health care system.

“The first drug intentionally created for sickle cell disease came out in 2017,” Ononogbu said. “Sickle cell disease has been around since 1910 and the first intentional drug was approved in 2017 – that is startling to me.”

Mental health hurdles

Ononogbu is researching mental health in SCD patients, providing insight into another largely unknown aspect of this vulnerable patient population. According to Ononogbu, studies show that patients with chronic conditions that involve pain, such as osteoporosis or arthritis, are likely to experience depression.

“With SCD patients, they are continually experiencing pain followed by discrimination when they go to the ER, e.g., being labeled as ‘drug seekers,’” Ononogbu said. “As an ‘invisible’ disability that requires accommodation, especially during pain crises, patients often experience discrimination at school and work because they don’t ‘look sick.’ Also, sickle cell patients deal with strokes at a young age, which can cause cognitive decline and issues with the neural pathways, so that can lead to some depression as well.”

Notably, Ononogbu is also researching the microbiome of SCD patients. She is exploring how the microbiome is connected to inflammatory markers that lead to pain in sickle cell patients and if there is a difference between those who have pain crises frequently versus those who don’t have pain crises frequently.

Hope in heme

As a Board-Certified Hematology/Oncology Clinical Pharmacy Specialist at UT Physicians Comprehensive Sickle Cell Center, Ononogbu provides specialized care and evidence-based treatment for complex patient cases. Her clinical practice also provides abundant opportunities for patient-centered research in the understudied disease. While a sickle cell diagnosis can be devastating, Ononogbu remains positive.

“The hope is that there are people who do understand sickle cell,” Ononogbu said. “There are people who care, and patients with sickle cell disease are living longer now because of the available treatment options.”