Sitting on the living room floor of his Union home building a toy car out of Legos, Bradley Scott looks like an average 10-year-old, but to doctors treating him for the rare kidney disease FSGS, he’s something of a medical mystery.
“He’s been rewriting the medical books since he was a baby,” Bradley’s mom, Amanda Scott, remarked. “I think every specialist that we’ve seen says he’s rewriting the medical books.”
FSGS, which stands for Focal Segmental Glomerulosclerosis, literally means some sections of kidney filters are scarred.
It is a rare disease — just five in 100,000 children are diagnosed each year — that attacks the kidney’s filtering units (glomeruli) causing serious scarring which leads to permanent kidney damage and even failure, the NephCure Kidney International website explains.
“Each kidney is made up of approximately 1 million tiny filters called ‘glomeruli,’ ” the site reads. “Much as a coffee filter keeps coffee grounds in, glomeruli filter the blood, taking out the water-like part which becomes urine and leaving the protein in the blood. When glomeruli become damaged or scarred (sclerosis), proteins begin leaking into the urine.”
Because Bradley’s kidneys are not working correctly, all of his organs are affected, said Scott.
The function of kidneys is to clean toxins and waste product from the blood, with the waste being expelled from the body through urine.
“Healthy kidneys help regulate blood pressure, remove waste and water, signal your body to make red blood cells, and help regulate growth in children,” NephCure.org explains.
It was just a few years ago that Bradley began exhibiting symptoms that something wasn’t right, but before that he was always one of those kids who seemed to get sick easier than most.
“He was always one of those kids who would catch everything, like his immune system was just weaker,” said Scott.
One oddity of Bradley’s childhood was that when he was an infant, he had episodes where he would turn blue from head to toe. It was terrifying for his parents to watch, but doctors couldn’t find any physical cause for the color change. Bradley’s health appeared to be fine.
“It wasn’t his heart or his oxygen level,” said Scott. “They said it was his autoimmune system. That was all we could get out of it.
“It looked like he wasn’t getting oxygen, but he was breathing fine,” she said.
The change would start in Bradley’s toes and fingers, and you could actually watch his body turn blue.
“Doctors called him the Smurf baby,” Scott recalled.
Sometimes the color change would last for just a couple of minutes, she noted. Sometimes it would last for hours.
Doctors expected Bradley would outgrow these episodes, and he did. By the time he was in kindergarten, it only happened if he had a bad cold or the flu or his body was run down, said Scott.
Then a couple of years later, on Christmas Day 2011, Bradley was in the emergency room with a serious infection that required surgery. He was just shy of his 8th birthday.
After surgery, Bradley developed pneumonia, and from that point forward he seemed to be sick constantly, either with pneumonia or strep throat, said Scott. At one time he developed a weird eye infection.
All of the infections and illnesses he had were never minor, always severe, said Scott.
It got to the point that Bradley’s pediatrician, Dr. Timothy Warren, at Mercy in Washington, said Bradley should be seen by an immunologist.
A urine test ordered by the immunologist found large amounts of protein in the urine (referred to as spilling protein), which meant something was wrong with Bradley’s kidneys. Some of Bradley’s blood tests also came back abnormal.
“His immune system was hit hard,” said Scott. “The antibody that helps the body fight different viruses, his IgG, was really, really low. After more tests, we found out it’s all because of his kidneys.”
The immunologist sent the family to a nephrologist, or kidney doctor, at Cardinal Glennon Children’s Hospital in St. Louis. Bradley has been seeing him since October 2012.
The following month he was diagnosed with Nephrotic Syndrome, which is not a disease itself but when a person has signs and symptoms of kidney damage. There are different strains of the syndrome.
Bradley has had two kidney biopsies. The first, in November 2012, identified him as having Minimal Change Disease, a type of Nephrotic Syndrome most common in children. It simply means that there has been little or no change to the glomeruli or the surrounding kidney tissue. There was no scarring in the kidneys.
By January 2014, things had changed. A second biopsy found more damage to Bradley’s kidneys and he was diagnosed with FSGS.
“Basically it means he’s starting to get more scarring in his kidneys, and if we can’t get his kidneys in remission, eventually he will need a transplant,” said Scott.
Unfortunately that won’t stop the disease.
“There have been some people who have had seven or eight transplants, and they are still living with the disease,” said Scott. “It’s just that the kidneys eventually develop so much scarring that they stop functioning all together.”
Last October, the Scotts visited the Mayo Clinic in Rochester, Minn., at the urging of Bradley’s nephrologist, just to make sure they were on the right path with his care and doing all they could.
“We found out a lot more things we need to keep up on,” said Scott. “The nephrologist we saw there said we have a long road ahead of us because it is incurable.
“A lot of kids, or people who have this, get put on different medications and some go into remission. Their kidneys stop spilling protein, but Bradley hasn’t stopped spilling protein since he’s been diagnosed.”
Last year was the hardest year yet for Bradley and his family in dealing with his illness. It seemed hard to get Bradley to a point where he wasn’t feeling sick every day.
“Sometimes he gets these muscle pains to where he can’t even walk,” said Scott. “He gets sick easier than most people because his kidneys aren’t filtering correctly, so he gets run down, plus the medication that he takes for his kidneys are also immunosuppressants.”
Just a couple of months after Bradley was diagnosed with Nephrotic Syndrome, he was starting to do a somersault on a mattress and ended up fracturing his back.
It turned out Bradley was extremely low on Vitamin D, which helps bones stay strong, also as a result of his kidney disease.
He experiences headaches, stomachaches, nausea and overall body pain. When he complained of a serious pain in his eye, a trip to the eye doctor found swelling on his optic nerve. The pediatrician changed some medications around, but after a few days nothing was better, so Scott took Bradley to the emergency room at Cardinal Glennon.
Doctors found Bradley had fluid around his brain, and there was serious concern that a tumor could be the cause.
Bradley had a spinal tap, which led to a spinal headache and him catching a virus. He ended up in the hospital for four or five days.
In the last several months, Bradley has been feeling better, said Scott, even though his kidney function hasn’t changed at all.
Scott had wondered if some of Bradley’s headaches and stomachaches were side effects from him taking so many medications and vitamins — he was taking seven pills a day, she said — so over the summer she had him stop taking the Vitamin D and calcium pills.
He continued to take the medicines he needed for his kidneys.
“They had him on so much, and I wanted to give his body a break,” said Scott.
“We didn’t know if the nausea was because he was on so much medication, the headaches could have been from different pills . . . I wanted to give his body a clean out and a rest.”
Currently, Bradley only takes three pills a day — medications to prevent him from getting the fluid around the brain again and for his kidneys.
His counts haven’t gotten any better, but they haven’t gotten any worse either, Scott noted. And physically Bradley has started feeling better.
In addition to his medications, Bradley also has regular physical therapy sessions to keep his body strong.
This year Bradley is a fifth-grader at Clark-Vitt Elementary in Union, and so far this year he hasn’t missed much school.
In the past, he wasn’t so lucky, but through it all, Scott said his teachers have been supportive of his needs. They have met with him after school or at the public library to help him with his schoolwork and make sure he wasn’t falling behind.
Team Bradley’s Battle
The Scotts will be participating in the NephCure Walk being held Saturday, Oct. 4, at 10 a.m. in Kirkwood Park at West Adams and Geyer Road.
Their team name is Bradley’s Battle.
They are participating as a means to raise funds for the NephCure Foundation, which conducts research to find answers and ultimately a cure for Nephrotic Syndrome.
But for the Scotts, participating also means finally being able to meet other families living with the same things they are.
Amanda Scott said she has found some support groups on Facebook, and people there have shared good information, especially recipes for a low-sodium diet, which is something Bradley needs as part of his condition.
The Scotts haven’t found anyone in Franklin County, much less a child, who is living with Nephrotic Syndrome. The closest person Amanda Scott has found in Bradley’s age group is a 9-year-old boy in Kansas City.
Bradley too said he’s excited to meet others with his same issues. He often feels like the only one in the world who is going through this, said Scott. He asks, “Why me?” a lot.
Scott said as much as she would love to raise money for the NephCure Foundation, she is happy just to raise awareness of the need. And she would love it if Bradley had a large team rooting for him at the walk.
“I think it would be awesome to have a great big team for him,” said Scott. “Right now it’s just family and a couple of friends.”
It doesn’t cost anything to sign up or be on the team, she noted.
To learn more about the NephCure Walk, people can visit www.TheNephCureWalk.org or call 1-866-NEPHCURE.
People can get in touch with Amanda Scott through email at email@example.com.
‘I Have Searched and Searched’
Scott has been searching for answers to why Bradley developed Nephrotic Syndrome, but so far she and doctors have found nothing.
“We just had genetic testing in June to see if we can get any answers from that,” she said. “It takes six months to get results back, so we don’t have anything yet.
“I have searched and searched and searched for answers,” said Scott.
She’s hopeful that one day Bradley will go in to remission, meaning his kidneys will stop “spilling protein,” but she knows even that will likely only be a temporary condition.
“No one knows where it comes from, and when people go into remission, even the smallest thing can make them start spilling again, like a mosquito bite or if they catch a cold,” said Scott.
So for now, Bradley finds joy in the days he feels well enough to act his age. He likes sports and he is playing soccer this fall. He also likes baseball and hopes next season he’s strong enough to play.
Bradley also likes to make and build things with Legos or clay, something he’s had a lot of time to do on those days when he’s either recovering from a surgery or just not feeling at full strength.
When he grows up Bradley said he wants to be a mechanic. He already helps his dad, Grant Scott, work on cars.
Bradley has two brothers, Brandon, 13, and Gavin, 3.