ABOUT THE CONDITION
Denys-Drash syndrome is characterized by abnormal kidney function, a cancerous tumor of the kidney called Wilms tumor and disorders of sexual development in affected males. DDS is a genetic disorder caused by mutations in the Wilms tumor suppressor gene, WT1.
Source: National Organization for Rare Disorders
HOW TO HELP
For more information about Shafer Daves or to donate to his medical fund visit www.cotaforshaferd.com.
The phone call came at 6:30 p.m. the Friday before Labor Day. We need to see your son at Vanderbilt tonight, the doctor told them.
Before the phone rang, it had been a normal day of laughter, school art projects, lunch and meetings, plans for a long weekend and family visits, with pauses long enough to give 2-year-old Shafer doses of medicine and water in his feeding tube.
But when they got the call, the laughter turned to quiet, and Kevin and Jada Daves did what they have done so many times before. Phoned someone to care for their other four children and packed clothes for at least a week. Sent out a quick email and a text message, telling family and friends that Shafer was headed back to the hospital, asking them to pray.
The creatinine level in Shafer's blood was too high, which meant his transplanted kidney wasn't functioning properly. No one knew exactly what it meant, but it wasn't good.
That Friday was like so many others in the Daves family, where every day is a balancing act between four healthy children and one with a transplanted kidney. Figuring out how to pay the hospital bills when your household income is cut in half because one parent is caring for a sick child. How to keep your children in school when you make trips to Monroe Carell Jr. Children's Hospital at Vanderbilt in Nashville at least once a month.
How to live when the specter of death is always a possibility.
"Every day is a crazy good day, and every day is a crazy bad day," Jada said. "People want your life all packaged with a nice bow on top. That isn't our life. If there is one thing we have learned, it is to live in the moment."
Those are the lessons the Daves family has learned since Shafer was diagnosed with a kidney disease so rare only 200 cases have been reported worldwide.
On the surface, they could be so many families in the tri-state area. Kevin is a computer systems programmer who works from home. Jada was a professional speaker before she took time off to deal with Shafer's illness. Their five children -- Shayli, Sharayah, Shanel, Shaw and Shafer -- are blond stairsteps from 2 to 10. They dance to Wii games, ride horses and play fetch with their dog on the front lawn of their Ooltewah home. The whole family loves basketball, with the parents coaching and the children playing.
Shafer -- who has the boundless energy of his age, clear blue eyes, chubby cheeks and a passion for dancing dinosaurs and Mickey Mouse -- seems the picture of perfect health.
Until he lifts his shirt to proudly show off the surgery scars on his stomach. One to repair his congenital hernia of the diaphragm. Two -- one on either side -- to remove his kidneys. One long line down the center where his mom's kidney went into his abdomen.
Tell-tale signs of countless surgeries and a life half lived in ICU.
Shafer was born on Oct. 15, 2009, after an emergency C-section. His parents took him home to join his three sisters and one brother, ready for the typical sleepless nights, burp cloths and his first smile.
But when he was 10 days old, he was admitted to the hospital with labored breathing. His first stay in ICU. Doctors diagnosed the hernia and did the surgery to repair it.
After the surgery, they came to Kevin and Jada. Shafer's kidneys were not normal, they said. They needed to do more tests.
And for the first time, the couple heard the words Denys-Drash Syndrome -- a rare genetic mutation, with only about 200 cases reported in the last five decades although there are likely more that have not been reported.
Shafer's kidneys would eventually fail, doctors said. He would likely develop kidney cancer.
There is no cure.
"The bottom line was our son was dying," Jada said. "They advised we put him in hospice."
Patients can wait years for kidneys
Jada Daves never hesitated when her infant son needed a kidney. She gladly volunteered hers.
And when it comes to living kidney donors, by far the most of them are parents, siblings, spouses or other relatives.
Since 1988, when the Organ Procurement and Transplantation Network first began tracking organ donations, out of more than 300,000 kidney donors, only about 1,000 living donors have given kidneys anonymously and 17,000 other living donors have been unrelated to the donor.
At the same time, nearly 100,000 people are on the waiting list for a kidney transplant.
Daves would like to see more awareness of the need for kidney donors, especially living donors.
"That has been part of the journey for me -- helping spread the word," Daves said.
Dr. Dan Fisher, surgical director for the UT Erlanger Kidney Transplant Center, said the list of people waiting on kidneys grows every year while the number of available kidneys and the number of transplants done every year have changed little recently.
"If you do the math, the average person is waiting about five years," Fisher said.
And every year nearly 5,000 people die while waiting for a transplant, according to the Organ Procurement and Transplantation Network.
People first become eligible for a donation when their kidneys function at only 10 percent of normal capacity.
A case like Shafer's, where failure is inevitable and the danger of developing cancer is a factor, does not change the criteria. If the transplant network didn't use strict criteria, the waiting list would become even longer, Fisher said.
"There's got to be a threshold," he said. "In theory, the transplant ought to help the sickest. No system is perfect, but I'd say it works pretty well."
Fisher said kidney transplants haven't changed that much since he first opened Chattanooga's transplant center in 1988. The Chattanooga center does about 15-20 transplants a year, he said. It performs only adult transplants, which is why Shafer Daves had to receive his transplant in Nashville.
What has changed is medication used to keep people from rejecting kidneys. Doctors now have a wide range of medications to use, which can ensure patients are not as likely to reject the transplanted kidney.
The first problem after transplant is dealing with acute rejection, but the longer the kidney stays in the body, the less likely rejection becomes. To see how well the kidney is functioning, doctors track creatinine -- or waste -- levels in the blood. The higher the level, the more likely the kidney is being rejected.
"Whenever the creatinine level is too high, you need to jump on that as fast as possible to turn it around," Fisher said.
Since Shafer has made it through more than a year after transplant, the chances of acute rejection have dropped significantly, Fisher said. His case is also helped by the fact that Jada's kidney matched Shafer's so closely.
"The kidney basically looks just like his," he said.
But no matter how well a kidney matches, the body will eventually reject it, a process known as chronic rejection. Over the years, antibodies slowly destroy the kidney.
"They know deep down the kidney does not belong; it's a slow, smoldering process," Fisher said.
BY THE NUMBERS
Waiting list for kidney transplants August 2012
Kidney transplants performed in 2011:
U.S.: 16,058 756
Alabama: 227 22
Georgia: 427 35
Tennessee: 430 13
Source: Organ Procurement and Transplantation Network
TO BE A DONOR
To learn more about being a living kidney donor, contact the National Kidney Foundation at www.kidney.org or call 800-622-9010.
To sign up to be an organ donor
In Tennessee: www.donatelifetn.org
In Georgia: www.donatelifegeorgia.org
That was nearly three years ago.
Faced with the fact that their son was seriously, even terminally, ill, Kevin and Jada refused to consider any alternative other than treatment.
"We needed to go down fighting," Jada said.
At T.C. Thompson Children's Hospital at Erlanger, they began the long journey to keep their son alive, to keep their family together and to enjoy the moments in between.
First came the waiting game. Shafer needed a new kidney, but his diseased ones had to fail before doctors could give him a new one. (See sidebar on A9.)
And the kidneys needed to fail before they developed cancer, which they almost certainly would. Cancer would mean treatment, remission and years of waiting before doctors would clear him for a transplant.
Along the way loomed medical pitfalls, hospital stays and the knowledge each illness could be his last.
"It sounds crazy, but we were praying for his kidneys to fail," Jada said.
The prayer was answered on Shafer's first birthday.
Once Shafer's kidneys failed, doctors began the process of getting him ready for transplant and finding a compatible donor.
Shafer was put on dialysis, his tiny body hooked up to a machine for 12 hours every night after he was allowed to go home from the hospital. His parents held him, sleeping in a recliner to keep him from pulling out the tubes.
Both Kevin and Jada volunteered to give Shafer one of their kidneys; one kidney that would keep his body functioning. Even as small as he was, a 6-inch adult kidney -- about three times the size of his own -- could fit into his abdomen.
Jada's kidney was a perfect match -- six out of six genetic markers -- a case so rare for a parent that doctors told them it was statistically improbable.
The process of testing for a match, getting Shafer ready for transplant, working through the protocol checklist and scheduling the major surgery took months.
But finally on June 22, 2011, after Shafer had been on dialysis for eight months, Vanderbilt doctors completed the transplant. Within hours, Jada was visiting her son in ICU.
The Daves family tells the story of Shafer's diagnosis and kidney transplant dozens of times to friends and family, to strangers in restaurants and people they meet on the beach. Shafer even tells it now, proudly pulling up his shirt to show his battle scars.
Miracle after miracle, they call it.
They take turns filling in the pieces -- remembering the first wet diaper after transplant that showed his kidney was working, the months spent in Nashville during recovery, Shafer's second birthday, a cowboy-themed celebration. They tell how their faith got them through the darkest days and how countless community members gave them a helping hand.
The story that is harder to tell is living after the transplant.
They are still figuring out that one every day. Their new normal, they call it.
More than a year after Shafer got his new kidney, they feel they can draw a deep breath.
Jada has returned to her public speaking career part-time. She and Kevin decided to home school the children this year, giving the family more flexibility.
The older children are learning how to cook, contributing to the family's tradition of brunch every morning. Shayli experiments with baking pony cupcakes for Shafer's third birthday in October.
Shaw and Shafer dash around the kitchen island on their scooters or play a game of fetch with curly-haired black Shadow, the family dog.
Kevin works in his front office, taking short breaks to mediate squabbles or answer the phone.
The family routine also includes doses of immunosuppressant and other medicines, some by mouth and some going into the feeding tube. Shafer takes a total of about 20 doses a day of a combination of seven or eight meds.
And every two hours, they pour water into Shafer's feeding tube to flush out his adult-sized kidney. "Belly juice," Shafer calls it, as he clips the tube going into his stomach.
At night, he sleeps in a recliner next to his parents' bed that has been fitted to keep him from rolling around. A feeding pump attached to his stomach tube drips in additional fluids and nutrition for about nine hours a night.
Kevin says he has learned to sleep with one ear open, quickly reaching for Shafer as soon as he moves to make sure he doesn't pull out the tube.
From the beginning, they have included the entire family in Shafer's illness, explaining his treatment and never shying away from what it means for the whole family. Balancing time with their four healthy children while realizing Shafer will take a lion's share of their care has been one of the hardest parts, the Daves said.
"We knew we couldn't save Shafer and lose the other four; we try to give them touch points of normalcy," Kevin said. "But there have been some cracks in the concrete."
The illness has changed all of them. Ten-year-old Shayli is the responsible oldest child and the worrier, remembering the days when she thought Shafer was going to die.
Shanel, 6, wants to be a doctor and practices putting a blood pressure cuff on Shafer and dosing him with "medicine."
Shafer's monthly check-ups in Nashville usually mean a trip for the entire family. In August, the family had four appointments in one day -- a doctor's visit and renal ultrasound for Jada and the same for Shafer.
First stop is the food court, where Shafer demands his favorite -- pizza. He talked excitedly, describing the horses the family saw on the drive to Nashville.
"One, two, three, four, five," he counted, lifting a finger for each horse in the trailer. They were black, green and blue, he insisted.
Shafer had almost continuous urinary tract infections in August, with back-to-back antibiotic treatments. One day he was fine, and the next day his fever spiked to 104 degrees. Jada worries about the damage being done to his fragile kidney.
Doctors aren't sure what is causing the problem and scheduled a procedure to look at his bladder in mid-September.
By the time Shafer headed into his second appointment, he was becoming impatient. He wanted to go see the train downstairs, the fish in the pond outside the hospital. Instead, Kevin took him to draw blood for lab tests.
The four children and Jada stayed in the waiting room.
"I can't do this one today," Jada said.
When Shafer saw the needle in the nurse's hand, he began screaming. Kevin and another nurse held him down, clamping his legs and arms as tightly as they could. The first needle stick was unsuccessful and they moved to the next arm.
Shafer's screams rose in crescendo. Kevin began singing softly, "Jesus loves me, this I know ..." The nurses joined in and, for a second, Shafer's screams lessened. But not for long. His sobs continued until the tiny tube was filled with blood and they pulled out the needle.
"This place changes you," said Jada, rejoining Kevin and Shafer as they headed down the hall to another exam room, this time for blood pressure and temperature.
No more blood, they promised Shafer.
The visit to Dr. Kathy Jabs, who has been Shafer's doctor since before the transplant, was less tear-filled.
Shafer is doing well, Jabs told the Daves. They just need to figure out what is causing his infections. She put him back on a low-grade antibiotic, hoping to ward off future infections.
Jabs has treated a handful of children with Denys-Drash Syndrome and even more with renal failure. In a world where sick children are the norm, Shafer is one of the lucky ones, Jabs said.
With his own diseased kidneys out of his body, the challenge is making sure he doesn't reject his mom's kidney or get sick because of his lowered resistance. If his kidney fails, he will need to go back on dialysis until he can get another transplant.
But that doesn't mean he won't be able to keep up with his brother and sisters or do everything a growing child should.
"The goal is to lead a normal life," Jabs said.
Fundraiser for Shafer medical expenses
Shafer Daves loves music. All he needs is a prompt from his parents or siblings to break into some of his favorite songs, singing "Jesus Loves Me" or dancing to music on an iPod.
And from the beginning, some of his favorite music videos have been the Gaither Vocal Band.
The "Gaifers" he calls them, and he'll watch and sing along to them for hours, ever since he was a toddler.
So when Jada Daves began organizing a fundraiser to help pay for Shafer's medical bills, her first thought was to call one of the men behind the music.
Gordon Mote, the Gaithers' pianist for six years, will perform at the fundraiser Nov. 8 at the Tivoli Theatre in Chattanooga. Other performers include Squire Rushnell, a former ABC executive and the author of the inspirational "God Wink" book series, and his wife, Louis DuArt, a Broadway actress and celebrity comedic impressionist.
All the money raised will be used to pay for Shafer's continuing medical expenses. Jada Daves, who is working with T.C. Thompson Children's Hospital at Erlanger, the YMCA and the city of Chattanooga to organize the fundraiser, said she hopes they will be able to make it an annual event with some of the money going to help other families with sick children.
"This is so much bigger than Shafer," she said. "We've just been chosen to care for him, and we want to give back the tremendous support we've been given."
Mote, who was born blind, said he was thrilled when Jada Daves asked him to participate in the fundraiser. The Daves first met him at a concert he performed and later visited his studio with their family.
"We had so much fun and I'm excited to be in Chattanooga," Mote said during a recent phone interview. "I love children like Shafer, but I also feel a real attachment to the parents who are trying to give them as normal a childhood as possible."
IF YOU GO
• What: An Evening of Inspiration, Music and Comedy
• Where: Tivoli Theatre, 709 Broad St., Chattanooga, TN 37402, 423-757-5050
• When: 6:30-9:30 p.m. Nov. 8
• Cost: $50 for adults and $25 for ages 12 and under; all proceeds go to support Shafer Daves' medical account with the Children's Organ Transplant Association.
Purchase tickets online at www.ChattanoogaOnStage.com, charge tickets by phone by calling 423-757-5050 or purchase at Memorial Auditorium Box Office Monday-Friday from 10:00 a.m. to 6:30 p.m. The Tivoli box office will open on day of show at 10:00 a.m.
Sitting in Shafer and Shaw's bedroom, Jada recalled some of the worst days of Shafer's illness.
He was in the pediatric unit at Children's Hospital before he was a month old, a feeding tube, a ventilator and other tubes attached all over his body. Jada sat in a rocker several feet from his tiny bed, tears running down her face. Kevin leaned over Shafer's bed, singing the hymn "It Is Well With My Soul."
The hospital staff let them stay around the clock, spending every minute they could with their son.
"When sorrows like sea billows roll; whatever my lot, thou hast taught me to say, It is well, it is well with my soul," Kevin sang.
He asked Jada to come join him at Shafer's bedside.
She shook her head.
"I just couldn't fake it. My mother's heart was breaking, and I couldn't say those words," Jada remembered.
Today, she has reached that point of peace with Shafer and with their family. She sees the way Shafer connects with everyone they meet, a "community baby" whose story has touched so many lives.
"When life changes, it doesn't mean life is worse," she said. "You just know every day is a gift."
They try not to dwell on the future, Kevin said.
Transplanted kidneys have a lifespan of 10 or more years, especially from a living donor that matches as well as Jada's did. That means Shafer may make it into his teens before his mom's kidney fails.
By then, one of their four other children may be able to donate a kidney.
"If we choose to worry about it, we're going to miss where we are now," Kevin said. "You know there will be issues that come up, but just pray it's not today,"
He is the one who asks most of the questions during the doctor's visit, the calming voice that talks Shafer through his tears.
Jada took the call from Vanderbilt that Friday night. After a second round of blood work, Shafer's creatinine level had gone from a normal of .3 to .7, the highest it had been since the transplant.
We need you up here tonight, the doctors told Kevin and Jada. It is the phone call they always have to be ready for, the reminder that a split second can change their lives.
"We were so happy five minutes ago" is a phrase Shayli frequently uses to describe those moments when everything changes.
For nearly 24 hours at Vanderbilt, Shafer was on an IV as they pumped medication and fluids into his body. Through the night and the next day, Jada and Kevin took turns sitting next to his bed, holding Shafer's hand and singing to him.
They responded to dozens of texts, emails and messages from their friends and family all around the world who were praying.
And Shafer's creatinine levels began to drop steadily. By Saturday evening they were back within normal range, and doctors released Shafer.
Like they have so many times before, Kevin, Jada and Shafer walked past the colorful animals on the walls, past the theater where they met country music group Rascal Flatts in a private concert, through the glass doors and out into the parking lot.
They were euphoric, Jada said.
But they also know they will be back.
"We've been doing this dance for 21/2 years now," Jada said. "It's scary -- you don't ever exhale. But we'll deal with tomorrow, tomorrow."
Contact staff writer Mariann Martin at email@example.com or 423-757-6324.