It’s a condition that most of us have never heard of, yet it can cause a person’s system to shut down and is fatal for many patients. It’s called APS and a more severe form is known as CAPS. For years there have been very few treatments, but a new therapy may be saving lives.
Janice Bielot and Terence Jackson have been inseparable since meeting at a friend’s party seven years ago.
Janice says, “he’s just amazing.”
Janice is not only Terence’s girlfriend; she’s his organ donor, giving up a kidney in a living donor chain.
Terence began having kidney problems at age 30, followed by trouble with a heart valve.
Doctors diagnosed Terence with CAPS, a condition that was causing multi-organ failure.
“That your body could turn on itself to that degree was shocking information,” Terence says.
While doctors aren’t exactly sure what causes CAPS or its more common form, APS, it’s an autoimmune disease that is fatal 50 percent of the time.
Robert Montgomery, MD, PhD, Chief of Transplantation at Johns Hopkins Medicine in Baltimore, Maryland explains, “the very small blood vessels, the capillaries and blood vessels that size become gummed up with debris and platelets and they start to clot.”
Dr. Montgomery says at one time blood thinners were the only option, but now, a drug that is ]approved to treat a serious kidney disease help CAPS patients. It’s a protein inhibitor called Eculizumab.
“It prevents that protein from binding to the antibody and killing the cells on the inside of the blood vessel,” Dr. Montgomery explained.
Terence says the drug and persistence saved his life. “Push, because you may be that exception or you can lead to an exception,” he says.
Terence Jackson and other CAPS patients treated with Eculizumab will need to be on the drug for the rest of their lives. He says the CAPS or APS will never go away, but he believes the drug will make the condition manageable.
Dr. Montgomery also says Jackson was the second CAPS patient worldwide to have a successful transplant while on the drug.
