Jacob Cole is 15. The high school freshman likes Harry Potter, the Buffalo Bills, concerts, fast food and fancy cars, as in Ferraris and Lamborghinis. Though functionally blind from birth and small for his age, Jacob at one time was a middle school wrestler.
A local TV station in the Southern Tier - where Jake lives - profiled him in 7th grade. Jake had made a name for himself when he won his first wrestling match despite his blindness, while actively participating in middle school using his braille typewriter and sharp wit. The reporter called him an inspiration.
But by the end of the following summer, Jake started feeling sick. “I just kept getting weaker and weaker. I didn’t want to eat anything, and I started throwing up a lot,” he says.
Jake’s mom, Kathleen Teeter, noticed his diminished energy and the flu-like symptoms and took him to the doctor. A few days later, she noticed his legs wobbled as he was getting into the car. He didn’t seem to get better. She took Jake to the Emergency Room at a local hospital. As he was attached to IVs and blood drawn from his arm for tests, Kathleen noticed that Jake was turning blue.
It was hard for Kathleen to understand what was happening. She is deaf and communicating in a crisis can be a challenge. Her father, Robert Cole, M.D., joined her at the hospital. A retired surgeon, Dr. Cole explained to Kathleen that blood tests showed Jake’s kidneys were shutting down and he would be transferred to Rochester for treatment.
A Desperate Condition
When they arrived at Strong Hospital’s Emergency Department, a team of physicians, nurses and sign language interpreters met the family at the door. It was then that Kathleen learned just how serious Jake’s situation was. “He was dying in the ambulance,” Kathleen says now, “The doctors told me he had Stage 5 kidney failure.”
“Jake’s creatinine level was about 20 times what it should have been,” says Maggie Doody, the Pediatric Nephrology Nurse Practitioner at Golisano Children’s Hospital who was part of the team treating Jake in GCH’s Pediatric Intensive Care Unit from the start. “Kidneys are a vital organ, and when things go south, they can go south quickly. He needed immediate hemodialysis treatment.”
“Jake basically had no kidney function when we first saw him,” explains Jake’s doctor, William Varade, M.D., pediatric nephrologist at GCH.
Doctors inserted a catheter to begin hemodialysis, replacing the natural functioning of his kidneys in filtering waste products from his blood stream. “At first, hemodialysis has to be done slowly to avoid possible seizures,” says Varade, “It took a couple of days for Jake’s condition to stabilize.”
“I was out of it, but started getting the idea that it was pretty bad,” Jake recalls.
As Jake started to feel better, his family began to understand that Jake would need on-going dialysis. But to get him out of the hospital and back home, Kathleen needed to learn how to do Jake’s dialysis at home.
For weeks, Kathleen and Jake stayed at the Ronald McDonald House in Rochester. Kathleen went to school, and, with the help of an interpreter and online courses, she learned how to hook Jake up to a peritoneal dialysis machine, keep his port sterile, and administer his many medications. Jake, meanwhile, went to the hospital for regular dialysis treatment and consistent monitoring.
Finally, Jake and Kathleen could return home. With Kathleen’s help, Jake got his dialysis treatment overnight as he slept. Special monitoring equipment was installed so Kathleen would be alerted to a problem not by the usual audible alarm, but by her bed shaking.
“Dialysis is a life-saving treatment,” says Doody, “but the goal for any patient with end stage kidney disease is a transplant. Transplants are a treatment, not a cure, but provide a much better quality of life, particularly with someone so young.”
“I felt like ‘Robot Jacob.’ I didn’t want to live life attached to a machine,” says Jake. In July 2020, he was put on a transplant list.
Young, healthy kidneys from deceased donors are rare. Rather than wait, Kathleen and Jake began looking for a potential living donor. They didn’t have to look far.
Jake’s older half-brother, Shawan Teeter, 23, offered to get tested to see if he was a match for Jake. He was. The transplant team at Strong put Shawan through a variety of additional tests to make sure he himself was healthy enough to donate one of his kidneys.
All systems were go. But the pandemic made scheduling surgery difficult. Finally, on January 12, 2021, a year and four months after Jake’s kidneys failed, he and Shawan came back to Rochester for the transplant surgeries.
Transplant surgeon Randeep Kashyap, M.D., MPH and his team worked in adjoining operating rooms to remove the kidney from Shawan, and immediately transplant it into Jake. “It happens so quickly that the kidney, once it’s attached, it begins working almost immediately,” says Dr. Varade.
“I don’t remember much,” says Jake, “I hurt afterwards for a week. Shawan said he hurt for two weeks.”
After the transplant, “It was amazing to watch Jake’s lab results normalize, and his creatinine level continues to decrease,” says pediatric nephrologist Hannah Brummer, M.D. As Jake’s health improves, the pediatric nephrology team tried to figure out why Jake got so sick. Genetic tests revealed that Jake has Senior-Loken Syndrome, a congenital disorder that mainly affects the eyes and kidneys beginning in early childhood, and gets progressively worse into adolescence.
“Jake’s kidney disease may have been progressing silently for a while, but the symptoms wouldn’t necessarily be obvious,” explains Brummer. “Unfortunately, kids in Jake’s situation present at the hospital in an advanced stage.”
A Teenager Again
Jake comes back to GCH regularly for check-ups and additional testing. He is working toward managing his many medications on his own. There are still challenges: GCH’s nephrology social work team are helping with everything from transportation to appointments to badgering drug companies and pharmacies to provide his medications with instructions in braille.
“Jake’s condition is very stable now,” says Doody, “He feels well and looks good. And he is growing!”
He is meeting soon with Alex Levin, M.D., M.H.Sc., chief of UR Medicine’s Flaum Eye Institute and an optho-geneticist, to see if there is any way to improve his eyesight.
Like so many kids his age this year, COVID has kept Jake home, where he logs in to his online classes and tries to keep up with his homework. As a transplant recipient, he takes medication to suppress immune responses to his new kidney, complicating a return to in-person school.
This time next year, Jake hopes to be back in school or maybe on a beach vacation with his family, to cheer on the Bills in person or dance at a concert.
“Shawan knows he saved his brother’s life and they have become so close,” says Kathleen, “It’s all a miracle.”