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Body Under Attack: Treatment Plan for Rare Disorder

Wednesday, March 14, 2018 8:28 AM

Richard Boren initially thought it was a bad case of the flu, one he just could not shake. Only after prodding from his wife to seek medical attention did Boren finally go to the Emergency Department at NorthShore Highland Park Hospital. He had no idea the trip would turn into a fight for his life.

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The 55-year-old Wadsworth man did not have the flu, but rather an extremely rare autoimmune disorder yet to be unraveled. Within hours of being admitted on Dec. 5, 2016, he stopped breathing. If not for the expertise and vigilance of his NorthShore physicians and broader care team in Lake County, Boren recalled, he wouldn’t be alive today. “These doctors were amazing. They saved my life!”

Springing to Action

Shortly after his arrival, the Highland Park Hospital’s emergency team recognized the seriousness of his condition and moved him to the Intensive Care Unit (ICU). “That’s when I stopped breathing,” Boren recalled. “I was a healthy male, who had not had a sick day from work in a decade. And suddenly, I stopped breathing.”

Boren remembers nothing of what happened next, but learned from his family of the “fast and furious response” to save his life, including intubation, or insertion of a breathing tube to maintain an open airway. Once stabilized, the care team began the daunting task of finding out the cause.

As the Highland Park ICU worked feverishly to keep him alive, Kellogg Cancer Center Medical Oncologist and Hematologist Britt Hanson, DO, was trying to figure out the source of the attack. Boren and his family credit Infectious Disease Specialist Daniel Zimmerman, MD, and Kidney Specialist Sandeep Mehta, MD, for keeping their defenseless patient stabilized. They also praised the entire ICU team, particularly nurse Maureen Walsh, who had ironically cared for Boren’s ailing father just a few months earlier.

Seeking Answers
Dr. Hanson suspected the culprit was hemophagocytic lymphohistiocytosis, or HLH. In the uncommon and life-threatening disorder, immune cells become overactive and begin attacking healthy cells. HLH is particularly challenging to diagnose because the initial symptoms mimic common, less dangerous infections. Dr. Hanson ordered bloodwork and a bone marrow biopsy, which confirmed the rare syndrome was present.

“Ordinarily, immune cells should destroy infected, damaged cells of the body. In HLH, the immune system begins to damage the patient’s own tissues and organs, including the liver, brain and bone marrow where blood is made,” explained Dr. Hanson, who holds an academic appointment at the University of Chicago Pritzker School of Medicine. “Sometimes doctors can actually see these angry immune cells ‘eating’ other cells using a microscope. This process is called hemophagocytosis.”

Mayo Clinic Collaboration
While it is uncertain what sets off HLH, the challenge for Boren’s care team was bringing it under control. Dr. Hanson recommended a course of steroids and chemotherapy. Due to the highly complex nature of the disease, she also took advantage of NorthShore’s ongoing collaboration with Mayo Clinic for a second opinion. Dr. Hanson reviewed the case with Mayo Clinic Hematologist Ronald Go, MD, who concurred with both her diagnosis and treatment plan.

“This isn’t an easy diagnosis and tough to treat,” noted Dr. Go. “Having a second opinion on a case like this may be the difference between life and death. With HLH, the body’s immune system is so revved up, it’s like a car running at 100 miles per hour and you take away the brakes. So the challenge is how do you put the brakes back on?”

Road to Recovery
The treatment protocol designed by Dr. Hanson did exactly that. It “put the brakes” on Boren’s out-of-control immune system. He steadily began to improve and was released from the hospital, able to return to his home and grateful family.

“This was a nightmare for my family and I have to give them credit for sticking with me the whole way,” Boren emphasized, mentioning his wife, their two children and his mother. As his recuperation continues, there have been challenges along the way. But Boren is now back at work and back on deck at a side job that brings him a lot of joy: running Renegade Charters, a fishing charter boat service on Lake Michigan. Grateful to be on the mend, he is quick to praise his NorthShore care team.

“They’ve been incredible. They not only provided the expertise to ensure that the treatments were working and that my body was responding in a positive manner, I know they truly cared about me and my family,” he said. “Without them I would not be here today to tell this story.”