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Toddler with Rare Kidney Disorder Beats Odds After Transplant Fails

Vadim Krisyan(NEW YORK) -- Ayana Richards said she will never forget waking up in the hospital to find out that the kidney she donated to her 3-year-old daughter had failed.

The doctor came in two hours after initially telling Richards the transplant was a success to say that a blood clot had destroyed the new organ beyond repair.

“I remember him having tears in his eyes,” said Richards, who lives in New York City. “I have to take pause because that moment, I think, is one of the lowest points in my life I ever had.”

But the doctors at Mount Sinai weren’t willing to give up so easily.

Anaya -- which is Ayana Richards’s first name spelled backwards -- was born with a rare kidney disorder called Finnish type congenital nephrotic syndrome, an inherited disorder that caused her kidneys to “leak” vital proteins.

Normal kidneys take in blood and sift out waste, which goes to the bladder and leaves the body as urine. Anaya’s kidneys were causing her to expel important proteins through her urine in addition to the waste. Since proteins are needed for everything from keeping blood from clotting inside the body to making the immune system function, Anaya’s health was in jeopardy.

“In the past, if you didn’t do anything, there’s no doubt she would have died as an infant,” said Dr. Jeffrey Saland, who cared for Mount Sinai’s Kravis Children’s Hospital, where Anaya would eventually get her transplant.

But neither Richards nor Anaya’s pediatrician knew Anaya had this disorder when she was born. After all, it’s common among people of Finnish descent, and Anaya was black and Hispanic.

“As a first-time mother, I didn’t know what to expect, just what she looked like,” said Richards, who was the first in her family to move to the United States from Trinidad and initially didn't have her mother around for guidance. “But you know that instinct. I just knew that something was wrong.”

Anaya cried a lot during her first two months of life, Richards said. She also threw up a lot and wasn’t as aware as Richards thought she should be.

But one day, the crying changed. It was piercing.

“I remember running her on foot to Harlem Hospital,” Richards said. “I knew the ambulance wouldn’t have been quick enough.”

That night, they relocated to New York Presbyterian Hospital, where doctors did a spinal tap and discovered that Anaya had bacterial meningitis. After a series of tests, they found out Anaya got meningitis because of the effects of her kidney disorder.

One doctor told Richards the disorder was so rare that she should be able to go outside blindfolded, play the lottery and win.

“He was trying to up my spirits, but I didn’t want to be lucky that way,” she said.

They sent Anaya home with a central line in her chest so Richards could give her regular infusions and injections of the proteins her kidneys removed. But the tube was prone to infection and clots because of Anaya’s condition.

“She needed a central line to live, but the central line could also cause something that can kill her,” Richards said, explaining that Anaya came down with several infections.

After Anaya got an infection that caused her head to swell to the size of a basketball, Richards started thinking about a kidney transplant to allow Anaya to live without the central line and its risks.

Richards took Anaya to Mount Sinai’s Kravis Children’s Hospital, where Saland was on the team that cared for her.

“We said the only way to prevent these issues is not to be in this situation anymore,” Saland said.

Once Richards learned she was a donor match, they transplanted her kidney into Anaya and removed Anaya’s two bad kidneys. Although they initially told Richards the transplant was a success, it failed two hours later when a blood clot did irreparable damage to the new kidney after it was transplanted.

And things would get worse before they got better.

The swelling from Anaya’s protein deficiencies and infections were made worse by the anesthesia, causing Anaya’s trachea to collapse when doctors tried to remove her breathing tube. When Richards went to visit her in a wheelchair, she said she remembers hearing the beeping of the machines and feeling as if she was in some horrible movie when the beeping stopped and Anaya went into respiratory arrest.

“I turned and I looked at Anaya. They were still pumping her. 'You are going to go through this and you will fight,'" Richards recalled telling her daughter.

Anaya pulled through with a tracheostomy and escaped with no neurological damage, but it would be a few months before a new kidney would become available. Since Anaya was so young, she was moved to the top of the kidney transplant waiting list, which has different sets of rules than other organ transplant waiting lists because kidneys from adults can be placed into small children.

Since Anaya was on dialysis for the two months leading up to her transplant, her protein levels returned to almost normal, preventing her blood from abnormally clotting like it did during the first transplant.

Now, Ayana is almost 4 years old. She is learning Mandarin Chinese at her preschool and was voted “most likely to become president” in her class.

“Each visit is like a treat because we get to see her develop in a way her kidney function is not a factor –- it’s a non-issue for her development,” Saland said. “[We] get to see her blooming.”

Copyright 2014 ABC News Radio

ABC News Radio