From Indiana University Purdue University Indianapolis
National Kidney Month: IUPUI biologist develops treatments for polycystic kidney disease
A biologist in the School of Science at Indiana University-Purdue University Indianapolis is developing treatments for polycystic kidney disease, which affects between one in 400 and one in 1,000 people worldwide.
Bonnie Blazer-Yost, professor of biology, said the disease equally affects people across gender, race and ethnicity. It can dramatically change the kidneys' appearance and function.
"Healthy kidneys normally are the size of a fist, but a kidney with cysts can grow to the size of a football," she said. "All patients experience pain as the cysts grow, and some develop them in the liver. Fifty percent of people affected by the disease will progress to complete kidney failure. They will need either dialysis or a transplant to resume the kidneys' function, which is to remove waste products from the blood."
One part of Blazer-Yost's research examines the effects of a commonly used diabetes treatment on polycystic kidney disease.
"This treatment has been used for over 15 years, and its side effects and long-term safety profiles are well-known," she said. "My colleagues and I have shown that in preclinical animal models, the drug could be used at a concentration level that is approximately tenfold lower than the lowest dose to treat diabetes. This low dosage is important because it is envisioned as a lifelong medication that will be started when polycystic kidney disease is diagnosed."
Blazer-Yost is also researching a natural chemical called LPA, or lysophosphatidic acid, that is found at high concentrations in fluid inside the cysts.
"Late in the disease, when the cysts become very large, they leak fluid or burst. This fluid bathes the outside of the remaining cysts, and LPA stimulates additional cyst growth," she said. "My colleagues and I have found the most pertinent LPA receptor on the cysts, which makes it a promising drug target to control growth in the disease's later stages." [Read more]
Family Kidneys
From Philly.com, by Jane M. Von Bergen, Inquirer Staff Writer
Turning to family for love - and a kidney
Like most of us, Kathleen Barrowclough, 70, was born with only two kidneys.
So, she couldn't save both her brother and her sister, who each needed a kidney transplant to stay alive.
"My brother said, 'I won't take your kidney, because Nancy is going to need it,' " Barrowclough recalled, and although the story is 20 years old, the retired nurse from Hockessin, Del., can't say it without a catch in the throat.
"He refused," she said, and then bravely made a stab at a joke about needing Kleenex.
In 1997, Nancy Pyle, now 75, received her sister's kidney. By that time, their brother was dead of an unrelated cancer.
"My sister I consider my angel, because she has given me life and I can never repay her," Pyle said.
Mountains of emotion complicate the landscape of organ transplants, and Barrowclough's family has experienced all of them. Hope, fear, sorrow, but most of all, a family loyalty remarkable even to seasoned medical professionals.
Six members of the family, along with a separate mother-and-son team, came to Jefferson Transplant Institute's day of sharing and learning Sunday to talk about how they decided to give and receive a kidney from a family member.
In all, about 40 attended the session, including staffers.
On average, 22 people die daily waiting for an organ transplant, according to UNOS, the United Network for Organ Sharing, a Virginia nonprofit that promotes organ donation. An estimated 122,000 patients need organs. Of them, 100,000 are seeking kidneys.
Survival chances improve if a friend or relative will donate. But, how do you ask? Do you ask? What if your child offers? Do you say yes? Do you turn them away?
Pyle, of Norman, Okla., stayed on dialysis for years, waiting for a donor, hoping to avoid turning to her sister, afraid that it would come back to haunt her if she and her sister fought, as sisters do.
"I was afraid to let her donate," Pyle said. "I was afraid it wouldn't work. What if I took her kidney and something happened? I'd be blamed for it."
Since then, donating kidneys has become almost a family ritual, complete with donors and recipients wearing matching bracelets and exulting when the transplanted kidney works so well that they pee on the operating room table.
Generations ago, the sisters' grandparents, Ruby and Stephen Dashiell, married a little too close on the family tree for optimum genetic health.
Related somewhat as cousins, both had polycystic kidney disease, a genetic condition in which cysts form on the kidneys and ultimately block their function, leading to death.
Because both had the condition, they passed it to their seven children and all died of it, including Barrowclough and Pyle's father. If only one spouse has it, there's a 50 percent chance that each offspring will be spared.
When they learned of the family scourge, Pyle decided not to research if she had it "because you couldn't get health insurance if you had a pre-existing condition [then]."
Eventually, she learned she had it and had passed it on to one of her two daughters, Melanie Darrow. Her other daughter, Stephanie Chissoe, 48, of Washington State, donated a kidney to her sister.
"I always knew this is what I wanted to do. I felt honored that I could do my part," Chissoe said. "It takes a village and we can all do this to help each other.
"We never felt any pressure. We had to force our kidneys on my sister," she said, laughing.
In the family of three, Barrowclough was the only sibling to escape the condition, which meant her children did, as well.
Her children, daughters Lou Montgomery and Lori Hoffman, each with two healthy kidneys, donated one kidney to a cousin.
Montgomery, 46, of Newark, Del., donated hers to Darrow, when the kidney from Chissoe failed.
Hoffman, 45, of New Castle, Del., donated hers to Debra Warner, 55, of Newark, in a complicated switch.
Warner's and Hoffman's blood types weren't compatible, so Hoffman donated her kidney to a woman and her husband donated his kidney to Warner.
As Warner got sicker and sicker, she hid her condition from her family, even knowing her cousin Lori would be willing to help. "I didn't want to put that on her," Warner said.
Warner is the daughter of Barrowclough's brother - the one who died.
"I felt some satisfaction that my daughter could help Deb, because I couldn't help my brother," Barrowclough said, her voice again catching. "But if I weren't able to give Nan my kidney, she wouldn't be here today."
Their family has had decades to work it out, so much so that organ donation is an expectation, as regular as lamb at Easter.
But kidney disease smacked the Thompson family in the face 11 years ago when their 17-year-old son, Tim, ballooned 20 pounds in 24 hours as his kidneys went into failure.
Parents Tim and Karen Thompson, of Cherry Hill, both had health issues, but they shopped hospitals until they found one, Jefferson, willing to accept Karen Thompson's kidney. The transplant occurred in September, and Thompson said she has never felt better.
"I came in here fighting for his life," she said, "and no one was going to tell me 'No.' If I had six kidneys, I'd give them all."
She's not a hero, she said, just a mother, but in giving her kidney to him, she took him off the waiting list and let someone else move ahead.
In 2015, 30,973 organ transplants were performed, and of them, about 5,000 came from a living donor. [Read more]
Paul Bentley (middle) and his sons, Nate (from left), Mike, Alex and Nick Bentley are facing some difficult choices. Paul needs a kidney. Nick and Nate would be excellent donors, but the family had already planned for them to donate kidneys to their two brothers. Credit: Family photo
The Bentley family had it all figured out.
Mom Judy donated a kidney to dad Paul nine years ago.
And two of their four sons, Nick and Nate, who dodged the disease passed down from their dad, would someday each give a kidney to their brothers, Alex and Mike, who have the disease.
"We thought we had a completely closed system where everybody was taken care of," Judy said. "The family joke is that you're either going to be a recipient or you're going to be a donor. But you're not going to be nothing."
Then the kidney Paul received from Judy began to fail and now has, in his words, flamed out. He went on dialysis three times a week beginning in February and desperately needs another kidney.
Tough decisions may lie just ahead, particularly for the brothers with healthy kidneys. A flier distributed by the family to find a suitable donor puts it this way: "If we don't find a donor for Paul soon, one of them is going to have to choose between saving his dad and saving his brother."
Some call this kind of agonizing decision a Sophie's choice.
"It weighs super heavily on me all the time," said the oldest son, Nick, 38, a board game designer in Madison.
He has known for years that he does not have the genetic defect, called polycystic kidney disease and characterized by multiple cysts and enlargement of the organ. There is no cure. Like his brothers, his chance of getting it or not getting it was 50/50.
"For all that time I've thought of myself as a vessel for the preservation of a kidney for one of my brothers. I thought my dad was taken care of. Now that's turning out not to be the case," Nick said.
Paul is 70, which is on the high end of patients considered for kidney transplants. He and Judy raised their sons in Whitefish Bay and now live in Oostburg. Paul is retired after 30 years working in marketing at Cramer-Krasselt.
His disease was not diagnosed until he was in his 40s. He lived with the weakness, nausea and other symptoms before finally getting the kidney from his wife in 2007, and at the same time having his own broken kidneys removed. The couple's hope was that this would buy him 20 or more years, but the kidney came with a virus that was benign for Judy but problematic for Paul.
He is on the waiting list at Froedtert Hospital for a kidney from a deceased donor, and is about to get on the list at University of Wisconsin Hospital in Madison.
But with time drawing short, his real hope is to find a live donor. A couple family members, friends and a former co-worker already have stepped forward. But it's not that simple.
"Most people think you just put your hand up and you're the live donor," Judy said. "But there's a huge qualification process, and there's a big wipeout rate of people who want to donate. We've had two people who went to Froedtert who have already been rejected." [Read more]
