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A Reckoning With Pain

Sickle cell patient and community advocate, Kyle Smith, describes the physical and emotional impact of a disease too long in the shadows.

A young Kyle Smith smiling and bending in a side pose
Kyle Smith, now 31, recently gave CSL Behring scientists a candid look at the daily struggles of people who live with sickle cell disease.

Kyle Smith was just a kid going to the movies on an 80-degree day. He brought a jacket knowing the air-conditioned theater would feel cool, but still the drop in temperature constricted his blood vessels and triggered a pain crisis related to sickle cell disease.

“Half an hour later, I was experiencing – at the time – the worst pain of my life,” Smith, now 31, told more than 100 CSL Behring scientists during a recent virtual meeting.

portrait of Kyle Smith, a sickle cell patient and advocate 

Note that he qualified his statement by saying “at the time.” The pain crisis caused by a chilly theater led to complications, hospitalization and time in the intensive care unit. He was ultimately cleared to go home, where his pain returned and worsened. It was due to withdrawal from the high doses of opioid pain medications that were required to control his severe pain during his lengthy hospitalization, Smith said. He recalls being “balled up in a fetal position” unable to sleep or eat.

It motivated him to do anything he could to avoid future sickle cell complications and the hospital. In the meantime, he was getting older and growing up. That’s good news for most kids, but if you are Black and have sickle cell disease, becoming an adult can create a new obstacle to getting health care, Smith said. His medical care experience as a cute kid left him unprepared for quite different treatment as a teenager and adult.

As an adult, trips to the ER bring dread because waits are hours-long and medical staff at times misconstrue sickle cell patients as drug addicts desperate for pain medication, Smith said. And he has already felt the stinging loss of a close friend who died young due to a brain bleed caused by sickle cell disease.

“Fighting for your life while simultaneously fighting racism is exhausting,” he said.

Sickle cell disease, so-called because of the way it curves red blood cells into sickles or crescent shapes, makes patients “old before their time,” said CSL Behring’s Dr. Greg Kato.  He aided CSL Senior Scientist and conference organizer Lisa Lindqvist in arranging the May 19 conversation about health disparities in sickle cell disease with Smith and fellow sickle cell expert, Dr. Kim Smith-Whitley.

Kato joined CSL Behring in 2020 to lead the company’s research efforts into sickle cell disease following a 30-year career, including stints at the U.S. National Institutes of Health (NIH), Johns Hopkins University and the University of Pittsburgh.

Smith-Whitley, who until recently directed the Comprehensive Sickle Cell Center at the Children’s Hospital of Philadelphia, treated Smith as a patient and said he represents one of millions globally who have the inherited blood disorder. She praised him for his work as Co-Founder and CEO of Crescent Foundation, a nonprofit community-based organization focused on helping people navigate the condition.

Smith and the Crescent Foundation have done important work in the community to educate patients about clinical trials for potential new treatments and about the COVID-19 vaccine, she said. The sickle cell patient population is particularly at risk of the novel coronavirus. 

Smith is one of an estimated 100,000 in the United States who have sickle cell disease, considered a rare disease in the U.S. The disease, which can cause strokes and organ damage, affects so many in Africa that it is not a rare disease there, Smith-Whitley said. In sub-Saharan Africa, as many as 50-90% of children with sickle cell disease die by age 5, she said.

And though it was identified 100 years ago, sickle cell disease has been inadequately researched and underfunded everywhere, including the U.S., despite the global toll the disease takes, Smith-Whitley said. Research and attention have ramped up in recent years, and a few new treatments have been approved. Much remains to be done.

Kato, who recently edited a new medical text about the disease, thanked Smith for sharing his story with so many scientists working behind the scenes. Dr. Andrew Nash, CSL’s Chief Scientific Officer, called the session “hugely valuable to us.”

“Thank you for helping to inspire everybody on this call to do more in this battle,” Kato said.