For most couples, there’s marriage, and then there’s what you do with the rest of your life. For Drs. Peter Sims and Therese Wiedmer, though, the two worlds are one and the same.
Sims and Wiedmer came to Oklahoma and OMRF together from Duke University in the 1980s. The two scientists shared more than a partnership in life; they also both studied how healthy red blood cells resisted damage from the complement system, the precise part of the immune system that does the damage in PNH.
As is often the case in science, one way to figure out how something works is to study a model that’s broken—and try to understand what went wrong. “That was where my interest in PNH began,” says Sims. “It had been known for a number of years that there was something very unusual about the blood cells produced by PNH patients.”
In particular, researchers knew that PNH patients had a higher degree of platelet activation, a process that plays a key role in initiating blood clotting. Clotting is an important function in the human body, repairing tears and breaks and preventing hemorrhaging. But when it occurs in the wrong place, as it does with PNH patients, it can form deadly blockages that can lead to organ failure or stroke.
So Sims and Wiedmer put their heads together with Drs. Chuck Esmon and Fletcher Taylor, two world leaders in the study of blood clotting, who also happened to be at OMRF. Working with Esmon and Taylor, Sims and Wiedmer developed a hypothesis. “Our suspicion was that a crossover from the complement system triggered the activation of a clotting mechanism in PNH patients,” Sims says. “We thought the PNH cells held a secret. Whatever that unknown thing was”—a sort of shield that protected red blood cells from the complement system—“the PNH blood cell was missing.”