Renal autotransplant

Sisters Emily and Sophia Johnston receive renal autotransplants to ease their pain

Two women, Sophia and Emily Johnston, standing next to each other smiling.
Emily and Sophia Johnston

The only fortunate thing about Emily Johnston being diagnosed with an extremely rare pain syndrome was that her younger sister, Sophia, had recently received the exact same diagnosis.

As a result, Emily didn’t have to go through nearly as much uncertainty and frustration as her sister did while she was trying to figure out what was wrong. And, as it turned out, Emily was able to undergo a life-changing surgery at UW Health in Madison, Wisconsin, even before her sister had her procedure.

Of course, that’s all water under the bridge now, because the procedure worked for both sisters, and now they’re both looking forward to next steps. “We were just so happy because it worked,” said Emily. “I wasn’t going to be in pain every day.”

A long wait for answers

Sophia, who is now 21, first began experiencing pain in her right flank and back area when she was 14. “The doctors I saw just brushed me off and said it was stress,” she said. “But I was throwing up all the time because the pain was unbearable. I missed a lot of school and wound up having to attend online during my senior year of high school.”

After graduating from high school, Sophia stumbled across an article about loin pain hematuria syndrome (LPHS), a rare condition that is characterized by severe flank pain and blood in the urine. “I thought, that really sounds like me,” said Sophia.

Both Sophia and Emily live in Ridgway, Pennsylvania, and after a long search, Sophia finally found a doctor in Pennsylvania who specialized in treating LPHS. Unfortunately, that doctor only saw patients from her own county, so she referred Sophia to the Renal Autotransplant program at UW Health.

“Everyone at UW Health was so amazing from the beginning,” Sophia said. “They were really kind to me, and they believed me. With my pain, I always felt like I had to prove myself with doctors, but I didn’t have that experience at UW Health at all.”

Doctors at UW Health felt they could help Sophia by performing a renal autotransplant—a procedure that involves removing the kidney and ureter and replacing it lower in the body. Such a surgery would relieve the pressure that was causing Sophia so much pain, they told her. But before she underwent the procedure, she would need to lose some weight first.

Family connection

In the meantime, just two months before Sophia received her LPHS diagnosis, Emily, now 26, started having her own excruciating pain. She’s a K-12 art teacher, and because she only gets 10 sick days per year, she had to grit her teeth and suffer through the pain in her classroom. She eventually received intermittent disability leave.

Emily was hospitalized in February 2023, and while she was there, her mother mentioned to the doctor that Emily’s sister had LPHS. Doctors there performed some tests on Emily and diagnosed her, too, with the same condition. The team at UW Health agreed to take Emily on as a patient, as well.

“It was only because Sophia had suffered so long that I was able to get help as quickly as I did,” said Emily.

Emily underwent a renal autotransplant of her left kidney on Sept. 30, 2023, and Sophia had an autotransplant of her right kidney on Oct. 17. Both sisters noticed right away after the surgery that the pain on that side of their body was gone, and as they have healed from their surgeries, they were able to slowly return to their lives.

Their journey isn’t over, however. Recently, Emily has started feeling minor pain in right side, and Sophia has pain in her left side. Some patients need to have autotransplants of both kidneys, and the sisters both suspect they will eventually undergo another procedure.

For now, though, they are enjoying the escape from debilitating pain. “I got to go Christmas shopping with my family and bake and decorate cookies,” said Sophia. “I feel better than I’ve felt in a long time.”