Kidney Donor Families; PKD Treatment Research

PKD Treatment Research

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

MICHAEL BRYANT / STAFF PHOTOGRAPHERWith kidney disease prevalent in their family, (from left) Lori Hoffman, Lou Montgomery, Stephanie Chissoe, Debra Warner, Kathleen Barrowclough, and Nancy Pyle all have something else in common: Each now has one 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]

From Milwaukee Journal Sentinal, by Jim Stingl

Great need, limited kidneys leave family with tough options

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]

PKD Disesae Mechanisms; Professor with PKD, I'll teach until I collapse; Desensitization: Waiting List Relief; Elderly and Dialysis

PKD Research

From Eureka Alert, MEDICAL UNIVERSITY OF SOUTH CAROLINA

Signed, sealed, undelivered: Mechanisms underlying polycystic kidney disease

Polycystic kidney disease develops when the message to generate cilia is 'lost in the mail'

IMAGE: KNOCKDOWN OF DYNAMIN-BINDING PROTEIN OR TUBA RESULTS IN ABNORMAL KIDNEY DEVELOPMENT AND OTHER ABERRANT PHENOTYPES IN ZEBRAFISH MODELS (MIDDLE AND RIGHT PANELS) COMPARED WITH CONTROLS (LEFT PANEL). view more

CREDIT: IMAGE COURTESY OF JOSHUA LIPSCHUTZ, M.D., OF THE MEDICAL UNIVERSITY OF SOUTH CAROLINA

In an article published online ahead of print on Feb. 19, 2015 in the Journal of Biological Chemistry (JBC), investigators at the Medical University of South Carolina (MUSC) and the Ralph H. Johnson VA Medical Center report findings from in vitro and in vivo studies that elucidate the mechanisms underlying the impaired ciliogenesis and abnormal kidney development characteristic of polycystic kidney disease (PKD). Depletion of dynamin-binding protein or Tuba, a guanine nucleotide exchange factor, disrupted renal ciliogenesis in cell culture and led to abnormal kidney morphology in a Tuba knockdown zebrafish model of PKD.

Currently, no drug has been approved by the U.S. Food and Drug Administration to treat autosomal dominant PKD, which affects a half million Americans and more than 12 million people worldwide. The disease is characterized by the development of fluid-filled cysts in both kidneys, leading to end-stage renal disease, usually around age 50 to 60. In PKD, it is speculated that dysfunctional cilia are unable to detect the presence of urine flow, triggering reactivation of developmental pathways, which lead to the uncontrolled production of cysts that eventually destroy the kidney.

Cilia, the finger-like protrusions on most epithelial cells, were not so long ago thought to be as irrelevant to cell biology as the appendix is to physiology, a vestigial remnant of a long ago evolutionary past. Today, they are recognized as essential chemo-mechanical sensors that monitor and regulate what crosses into and out of a cell.

Dysfunctional cilia are now known to be implicated in not only PKD but a wide range of diseases affecting the eyes, ears, heart, and other organs. Understanding how cilia become dysfunctional in these diseases could provide insight into how to better treat or prevent them.

"How are cilia made? If you know that, you can figure out what goes wrong in ciliopathies, including polycystic kidney disease," says nephrologist Joshua H. Lipschutz, M.D., the senior author on the article, who holds a dual appointment at MUSC and the Ralph H. Johnson VA Medical Center.

Much must go right for ciliogenesis to occur. Proteins necessary for ciliogenesis are manufactured in the endoplasmic reticulum before traveling to the trans-Golgi network to be sorted into "zip-coded packages" or vesicles for transport to the cilia. Lipschutz and others previously showed that the exocyst, a protein targeting complex, plays a crucial role in receiving these "zip-coded packages" containing ciliary proteins. The GTPase Cdc42 regulates the exocyst, which is the mailbox where these "packages" are received in the kidney. Renal ciliogenesis occurs only when the packaged proteins are delivered to the Cdc42-activated exocyst complex. Depleting either the exocyst or Cdc42 disrupts renal ciliogenesis.

In the JBC article, Lipschutz and his MUSC coauthors go a step further -- showing in cell culture and a zebrafish model that depletion of Tuba, a guanine nucleotide exchange factor required for Cdc42 activation, also disrupts renal ciliogenesis. Tuba is thought to ensure that the Cdc42/exocyst mailbox is in place at the base of the cilia and ready to receive the packaged proteins. Without Tuba, Cdc42 is not appropriately activated, and the exocyst is mislocalized, so the undelivered packages continue to pile up, perhaps playing a role in the uncontrolled production of renal cysts in PKD.

When grown in a collagen gel, Madin-Darby canine kidney (MDCK) cells form into cysts, and the orientation of proteins, called polarity, are abnormal following Tuba knockdown. Specifically, apical proteins that would normally face the urinary space are mislocalized throughout the cell.

In zebrafish, injection of low doses of both Tuba and Cdc42 antisense morpholinos, which had no effect when administered separately, led to severe phenotypes similar to those seen following knockdown of other ciliary proteins. This is called genetic synergy and provides further evidence that both Tuba and Cdc42 are part of the same pathway. Because knockdown of Tuba in zebrafish affects cilia in a number of organs, including the brain, a variety of aberrant phenotypes were seen in the Tuba knockdown zebrafish model.

Lipschutz, who directs the zebrafish core at MUSC along with co-author Seok-Hyung Kim, Ph.D., is well aware of the advantages of the zebrafish for research -- its genome is well characterized, it can be bred rapidly and inexpensively, and its transparent body enables easy visualization of aberrations under microscopy. However, the next step in this line of research will be to study the effects of Tuba depletion in the kidneys of mice, since murine kidneys are more like human kidneys than those of zebrafish.

Once the pathways underlying impaired ciliogenesis in PKD are more fully understood, therapeutic interventions can be designed to disrupt those pathways. As Lipschutz notes, "We do this research to help our patients. Further elucidating the pathways that underlie impaired ciliogenesis is an essential step in beginning to develop treatment options for PKD and other ciliopathies."

Living with PKD

From Daily Mail, United Kingdom, By CHLOE LYME FOR MAILONLINE

'I'll teach until I collapse!' Dedicated professor who is too ill to stand keeps giving lessons on his KNEES

An encouraging story of a sick teacher from China who continues to work despite his painful treatment, has touched the hearts of millions across the country.

Yu Gongmao from Wuhan city was diagnosed with polycystic kidney disease at the age of 30, over the years his health has deteriorated and he developed uraemia, reports the People's Daily Online.

Despite having to undergo intense treatment three times a week, he continues to teach sitting on his knees as he is too weak to stand. The 41-year-old was pictured giving classes at Wuhan University on March 11.

In 2012 professor Yu passed out in class and was immediately rushed to hospital where doctors found a tumour in is brain. He was operated on which was successful, but his health continued to worsen.

Unfortunately he was left with bad eyesight in his left eye, a result of the operation.

After relapsing in 2014 he was told his kidneys were failing, he is now awaiting a kidney transplant.

He receives dialysis three times a week, both of his kidneys have problems leading to uraemia.

Speaking to the People's Daily, professor Yu said he will 'keep teaching until I collapse in class'.

Since he became ill, professor Yu - who works at the Department of English at the university - has suffered from severe weight loss, dropping to around 120 pounds.

In a week, professor Yu teaches more than ten classes on his knees as he is too weak to stand. He even works as a translator and a tour guide when he is not teaching.

He said he loves to interact with his students, but the disease makes it difficult to give them the physical support they need. [Read more]

From U.S. News and World Report, By Robert Preidt, HealthDay Reporter

Kidney Dialysis Might Not Extend Survival of Elderly

Dialysis does not significantly improve survival for elderly kidney failure patients, a new study indicates.

The findings suggest that conservative care may be a reasonable option for some kidney failure patients over 80.

The researchers don't say that dialysis treatment should not be given to anybody older than 80 or with severe co-occurring conditions. "But we show that the treatment is on average of little advantage regarding survival," said study co-leader Dr. Wouter Verberne of St. Antonius Hospital in Nieuwegein, the Netherlands.

The findings were published online March 17 in the Clinical Journal of the American Society of Nephrology.

"Our next task is to predict who benefits and who does not," Verberne said in a journal news release. "Until we are able to give a better prediction of the results of dialysis treatment at high age, we can merely suggest that conservative management is an option which should honestly be discussed when [kidney failure] is approaching."

Conservative therapy includes controlling fluid and electrolyte balance, treating anemia, and offering suitable comfort and end-of-life care.

In this study, researchers examined outcomes among older kidney failure patients in the Netherlands who received either dialysis (204 patients) or conservative care (107 patients). Among those over age 80, the investigators found no statistically significant difference in survival between dialysis and conservative care.

Overall, patients with other health problems died sooner than those without additional medical issues, the study authors reported.

Further research is needed to assess how different treatment options affect patients in other ways, such as quality of life and severity of symptoms, Verberne concluded in the news release.

Kidney Transplant

From Fast Coexist, by CHARLIE SORREL

A New Kidney Transplant Procedure Makes All Donors Compatible

Desensitization is a new technique that prevents rejection of transplanted kidneys, which means that donors and patients can be much more easily matched. A new study of 1,000 patients shows its promise at changing how organ transplants work.

Incompatibility between donor and recipient is the biggest barrier to successful transplants. If the recipient’s immune system attacks the organ, then the transplant fails. Finding a successful match between non-related people is a one in 100,000 chance, according to Popular Science.

Desensitization works by filtering antibodies from the patient’s blood, which suppresses the immune response against the foreign kidney. During the eight-year study from Johns Hopkins University School of Medicine, patients undergoing desensitization treatments were pitted against folks who received kidneys from compatible donors, and those who remained on dialysis. The results were telling: About 76% of desensitization patients were still alive after eight years compared to 44% of those who stayed on dialysis. The desensitization process even beat out compatible donations—only 63% of those receiving a compatible organ survived.

This is significant because at any given time there are around 20,000 people waiting for kidneys who are "highly sensitized and for whom finding a compatible donor is nearly impossible," says the study’s lead author Dorry Segev. "That’s a lot of people who could have a better chance at surviving if they are allowed to move forward with incompatible live donor transplantation."

The process has been in experimental use for 15 years, but this is the first nationwide study to assess the benefit to patients. The idea is to push the process into regular use. One barrier to this might be cost. Desensitization, as an ongoing process, is expensive. But not, crucially, as expensive as dialysis. "Incompatible transplantation is essentially one-tenth of the price of dialysis for a patient stuck on the wait list," Segev says in a news release.

Also, by essentially making incompatible organs compatible, waiting times can be cut. "During the wait for a compatible organ, the patient gets sicker and sicker. This can become costly—physically, emotionally and financially—for the patient and his or her loved ones," says Segev.

What the study shows is that transplant recipients are twice as likely to survive using desensitization than with their next best option. Those are convincing numbers, and because they are gathered from multiple facilities across the country, they make a good case for more widespread use of the technique.

Desensitization: Organs from Incompatable Donors, Diet Recommendations, Hard to Identify, ADPKD Progress, Support Group

New Techniques

From Tech Times, By Katrina Pascual

New Technique Allows Kidney Transplant Patients To Receive Organ From Incompatible Donors

A large national study offers a ray of hope for tens of thousands on kidney transplant waiting lists who may never find a compatible donor.

Through a method called desensitization, doctors successfully altered the immune system of patients to enable them to accept kidneys from incompatible donors. Substantially more patients remained alive after eight years compared to those who stayed on waiting lists or received their kidney from a dead donor.

“[This] has the potential to save many lives,” reports Dr. Jeffrey Berns, kidney expert at the University of Pennsylvania and president of the National Kidney Foundation.

Clint Smith, a 56-year-old New Orleans lawyer, would no longer spend the rest of his life on kidney dialysis after undergoing the procedure, which he believed changed his life.

Desensitization filters the antibodies out of the patient’s blood, and then offers him an infusion of other antibodies for protective action, while the immune system is regenerating its own antibodies. It is unclear how, but the reproduced antibodies prove less likely to attack the transplanted organ.

If the regenerated antibodies remain a concern, drugs are provided to destroy any white blood cell that would prompt the antibodies to attack the kidney.

"We used to say if you had a compatible donor, you could do a transplant. Now you can say, if you have an incompatible donor, we still can make that transplant happen," says Dr. Dorry Segev of John Hopkins University and study's senior author.

The eight-year survival rates were nearly 77 percent among 1,025 patients who received an incompatible kidney from a live donor, and 63 percent for more than 5,100 matched patients remaining on a waiting list or received a new organ from a deceased donor. It was 44 percent, on the other hand, for more than 5,100 who stayed on the waiting list without getting a kidney.

The research was conducted at 22 medical centers, projected to help about 32,000 individuals in the United States who have anti-HLA antibodies, or those whose immune systems were technically unfit to the donated kidney.

Kidney exchange has risen in recent years as a viable option for those with incompatible living donors, where they could swap donors with someone else. The exchanges have often led to compatible organ pairs. Still, the patients’ antibodies would get in the way, rejecting almost every donated organ.

“[D]esensitization may be the only realistic option for receiving a transplant [in this case],” says Dr. Krista Lentine of the Saint Louis Center for Abdominal Transplantation, who was not involved in the research.

The procedure is expensive, costing up to $30,000 and using drugs that are not approved for the said purpose. The transplant could cost $100,000. Still, specialists argued that desensitization could be cheaper in the long term compared to dialysis, where one could incur $70,000 a year in expenses for the rest of his life.

The findings were published in the New England Journal of Medicine.

From The Washington Post, By Emma Brown

These teen scientists, vying for $1 million in scholarships, will give you faith in the future


One of them has studied ways to make plastic decompose more quickly.

Another has been working on using skin cells to grow kidney tissue, taking steps toward a new treatment for kidney disease.

And a third invented a device to diagnose lung diseases in developing countries. It’s made out of $35 worth of materials — patent pending.

They are among 40 U.S. teenagers who have been named finalists in the prestigious Intel Science Talent Search, which recognizes rising stars who have done notable original research. All 40 will be in Washington this weekend competing for $1 million in scholarship awards, including three top prizes of $150,000 each.

It can be deeply humbling to scroll through their project descriptions and biographies. And it also can inspire hope: These young people are looking for answers to some of the world’s most intractable problems, and they’re making important progress. They also have interests outside of science, from dancing to skiing to soccer and music.

Demetri Maxim, 18, has created a method to bioengineer kidney tissue from a patient’s own cells, work that he hopes eventually will mean that scientists will be able to grow new kidneys for people suffering from chronic kidney disease, so they won’t have to wait for an organ donor or risk their bodies rejecting a donor’s kidney.

“I was essentially growing kidneys in a cell, that’s how my friends say it,” said Maxim, a senior at Gould Academy in Bethel, Maine.

The project is personal for him. He has polycystic kidney disease, a genetic condition that leads to kidney failure. His grandfather and his great-grandfather also had the same condition, and his mother nearly died from it; a kidney transplant saved her life.

“I have the same disease that my mom does, so someday I’m going to need a kidney,” Maxim said.

Maxim said he had to teach himself a lot of basic research skills by reading papers and taking online tutorials. But last summer, after sending out countless emails to researchers, he found a Harvard professor who was willing to serve as a mentor and give him access to a real lab. The professor, it turned out, had years earlier diagnosed Maxim’s mother with polycystic kidney disease.

Maxim used skin cells to grow stem cells using a lab protocol developed by Nobel Prize-winning scientists. He used those stem cells to grow kidney cells. And then, to create tissue, he transferred those kidney cells onto a three-dimensional structure. That 3-D structure was composed of de-cellulized mouse tissue, or mouse tissue stripped of all its cells.

Maxim then tested the kidney cells by transplanting them into a live mouse with a functioning kidney. He found that the cells survived and became integrated into the mouse’s organ.“And that’s never been done before,” Maxim said. “It’s one thing to have cells in a dish, but it’s a totally different story to have functional tissue.”

There’s still a lot of work to be done before human patients can receive organ transplants grown from their own cells. But Maxim plans to stick with it when he goes to college next year.

Meantime, he’s been helping his high school create a research program for other aspiring young scientists. He applied for grants and solicited donations that allowed him to build a lab at the school, and he’s been teaching freshmen and sophomores how to do basic lab work.

Maxim said it’s difficult to find other students his age who are interested not just in skiing and soccer and music, as he is, but also deeply interested in science. He said he was shocked to be an Intel finalist, and is excited to spend time with the other 39 finalists. “You’re with your real friends for a week,” he said. “That’s what I’m most looking forward to.” [Read more]

From The Irish Times, Dublin, Ireland, by Dick Ahlstrom
Irish engineer wins major prize for kidney dialysis design

Irish engineer Vincent Garvey, who has won a major international prize for his design for a low-cost kidney dialysis machine.

An Irish engineer has won a major international prize after designing a low-cost kidney dialysis machine.

Smaller than a suitcase and geared for use in the developing world, the machine has the potential to save millions of lives in countries where conventional kidney treatments are either too expensive or difficult to access.

The engineer, Vincent Garvey, is originally from Donaghpatrick, Co Meath, but has spent the past 15 years in China, where he runs engineering consultancy firm MD Devices.

The goal of the Affordable Dialysis Prize 2016 was to design a portable dialysis machine costing about €900 for use in places where there was no assured electricity supply or clean water.

Conventional dialysis machines typically cost between €18,000 and €27,000.

Mr Garvey took up the challenge with a design that could be used anywhere, with solar power used to produce the sterile water needed to help clear the body of life-threatening toxins.

He scooped the prize, worth €91,000, after a unanimous decision by the international panel of judges.

His win was announced on Thursday, which was World Kidney Day.

Mr Garvey said it was “incredible” to win the prize.

There were millions of people worldwide who don’t have access to dialysis “and currently suffer pretty awful deaths”, he said.

“Vincent has been inventing things his whole life,” said his sister Maria, who lives in Dublin.

“The family is very proud and delighted for him.”

Mr Garvey had just retired from full-time work and was visiting family inAustralia when he heard about the dialysis prize.

“I worked on the design last year in Australia. It was much more fun than trying to be a retiree,” he said.

“It looked like it would make a huge contribution if it worked.”

He is now developing the system to bring the design into use.

Prototypes

A Melbourne company is making and testing prototypes and he is looking for a firm to manufacture the machine.

It may be possible to test the system on animals within 12 months and on humans in about three years, he said.

A study published by The Lancet found more than nine million people needed access to dialysis for terminal kidney failure worldwide, but only 2.6 million received this life-saving treatment.



Living with PKD

From Hungry Forever

Here Are Some Diet Recommendations for Individuals With Chronic Kidney Disease

Causes, Symptoms and Diagnosing CKD

Individuals with hypertension and diabetes are more at a risk of developing CKD. Other causes include genetics, infections, polycystic kidney disease as well as hereditary factors; if an individual has family members with CKD, they are more likely to develop it themselves.

The symptoms of CKD are mild in its early stages but tend to become more obvious as it progresses. Common symptoms include dizziness, breathlessness, fatigue, swelling, rashes, and nausea.

If evidence points to the possibility of CKD a doctor can carry out a variety of tests to diagnose the condition including blood tests, urine tests and a kidney sonography.

Treatment and Management

While CKD is a lifelong disease, there are various ways to slow it down or halt it from progressing to the fifth stage of the condition, known as ‘End Stage Kidney Disease’. These include managing diabetes and blood pressure to ensure that it does not get out of hand and aggravate the CKD.

Other management techniques include diet control and, in the latter stages of CKD, dialysis, in which kidneys are manually cleansed of toxins. Here are dietary factors that a patient with CKD should know about:

Low Versus High Protein Diet

Many medical practitioners recommend a low protein diet for patients not on dialysis. This is because when the body digests protein, urea – a waste product – is produced. Normally, the kidneys dispose of the urea, but if not functioning properly the urea can build up in the blood, causing fatigue.

Therefore, cut down on your intake of protein daily, trying to limit yourself to 1 gram per kilogram of your body weight a day.

However, once a patient starts dialysis, they should switch to a high protein diet to replace the tissues that are lost in the process. Protein rich foods include fish, poultry, meat and dairy – depending on the stage of CKD limit or increase their intake as necessary. Vegans can try breads, cereals, beans or pasta.

Limit Foods High In Phosphorus

Phosphorus, a mineral found in bones typically helps maintain strong bones. However, excess levels of phosphorus can leech calcium from the bones making them brittle and more liable to breaking. Functioning kidneys work to remove excess levels of phosphorus, but an individual with CKD needs to cut down on phosphorus rich foods as the kidneys can no longer carry out this task.

High phosphorus foods include cheese, custard, milk, yogurt, ice cream, colas, carp, chicken liver and other offal, lentils and beans, seeds and nuts. As you can see, some of these foods are the same an individual on a high protein diet would need to take in, so consult with a dietician for alternative protein supplements you can take if you need to limit your intake of phosphorus.

Increase Your Intake Of Carbohydrates

Many experts recommend increasing your intake of carbohydrates if you are put on a low protein diet as they can help make up for the calories lost from not eating a lot of protein. However, ensure that the carbohydrates you eat do not contain high phosphorus levels or – if you’re a diabetic -a large amount of processed sugars.

Fluids

As your CKD progresses, a doctor will recommend you reduce your intake of fluids as a fluid build-up can cause breathing problems. Monitor your intake of fluids as per doctor advice and cut down on eating water rich foods like fruits, ice cream, soups and tomatoes.

Limit Sodium Intake

As well as helping you manage high blood pressure, which is one of the causes of CKD, a low sodium diet will ensure you feel thirsty less often. Having less sodium in your body will allow it to release extra fluid more easily.

Try not to add too much salt to your daily meals, snack on unsalted munchies and study the sodium content of processed food packaging before eating it.

While these are some general tips that an individual with CKD can follow to improve their quality of life, it’s important to realise that according to the stage and causes of the condition, dietary advice can change. So, use this information as a supplement to doctor recommendations and consult with a dietician to reach a diet plan that will allow you to reach the highest quality of life possible with the condition.

From News24, Cape Town, South Africa, by Tladi.Moloi

Kidney disease tough to identify

MANY people who have chronic kidney disease do not know it, because the early signs can be very subtle.

It can take many years to go from chronic kidney disease (CKD) to kidney failure. Most people with CKD live out their lives without ever reaching kidney failure. People with stage 3 CKD have about an 80% chance of never having their kidneys fail.

At any stage of kidney disease, knowledge is power. Knowing the symptoms of kidney disease can help you get the treatment you need to feel your best.

If you or someone you know have one or more of the following symptoms of CKD, or you worry about kidney problems, see a doctor for blood and urine tests.

Remember, many of the symptoms can be caused by other health problems. The only way to know the cause of your symptoms is to see your doctor.

Changes in urination

Kidneys make urine, so when the kidneys are failing, changes in urine and urination may occur. Examples are:
You may have to get up at night to urinate.
Urine may be foamy or bubbly.
You may urinate more often, or in greater amounts than usual, with pale urine.
You may urinate less often, or in smaller amounts than usual, with dark-coloured urine.
Your urine may contain blood.
You may feel pressure or have difficulty urinating.

Swelling

Failing kidneys do not remove extra fluid, which builds up in your body causing swelling in the legs, ankles, feet, face or hands.

Fatigue

Healthy kidneys make a hormone called erythropoietin, also known as EPO, that tells your body to make oxygen-carrying red blood cells.

As the kidneys fail, they make less EPO. With fewer red blood cells to carry oxygen, your muscles and brain tire very quickly. This is anemia, and it can be treated.

Skin rash or itching

Kidneys remove wastes from the bloodstream. When the kidneys fail, the build-up of waste in your blood can cause severe itching.

Metallic taste in mouth or ammonia breath

A build-up of wastes in the blood (called uremia) can make food taste different and cause bad breath. You may also notice that you stop wanting to eat meat, or that you are losing weight because you just do not feel like eating.

Nausea and vomiting

Uremia can also cause nausea and vomiting. Loss of appetite can lead to weight loss.

Shortness of breath

Trouble catching your breath can be related to the kidneys in two ways. Firstly, extra fluid in the body can build up in the lungs. Secondly, anemia can leave your body oxygen-starved and short of breath.

Feeling cold

Anemia can make you feel cold all the time, even in a warm room.

Dizziness and trouble concentrating

Anemia related to kidney failure means that your brain is not getting enough oxygen. This can lead to memory problems, trouble with concentration and dizziness.

Leg or flank pain

The most common causes of CKD do not cause any pain. Also, much of the pain that is near the kidneys is not caused by a kidney problem. However, some people with CKD experience pain.

Some people with kidney problems may have pain in the back (where the kidneys are) or on the same side as the affected kidney. Causes include:
Polycystic kidney disease (PKD), which causes large, fluid-filled cysts on the kidneys and sometimes the liver, can cause pain.
Kidney infections and kidney stones can cause severe pain, often in spasms.
Bladder infections can cause burning when you urinate.
People with medullary sponge kidney say it is painful. – Lifeoptions.com

Knowing the symptoms of ­kidney disease can help you get the treatment you need to feel your best.

From News-Medical, United Kingdom

Pioneering progress on ADPKD: an interview with Tess Harris

Can you give me a brief overview of autosomal dominant polycystic kidney disease (ADPKD), its prevalence and how it affects patients?

ADPKD is a chronic, progressive and inherited kidney disease characterized by fluid-filled cysts that develop in the kidneys and other organs. It is the most common inherited kidney disease, and affects millions worldwide. There are no epidemiological registries that can give precise numbers affected.

Prevalence figures vary between 3 to 4 in 10,000 diagnosed patients, so well over 200,000 in the EU. ADPKD is an important cause of chronic kidney disease (CKD) and kidney failure, accounting for one in 10 patients needing kidney dialysis or transplantation.

The direct EU cost of treating ADPKD patients on dialysis or transplant is approx. 1.5 billion Euros/year and this will double to €3 billion when one adds costs of other medication and care resulting from symptoms.

Outside of nephrology, ADPKD is little known, even though its sufferers require complex, multidisciplinary care with a comprehensive assessment of their manifestations, complications and prognosis - all of which vary dramatically between individuals owing to the genotype variability even within families.

Coupled to this complex care management, ADPKD is associated with high healthcare costs since many individuals progress to kidney failure requiring dialysis and/or transplantation at an earlier age than other kidney patients - e.g. in the UK, the age of kidney failure is 55, compared with 65 for non-ADPKD patients.

Normal Kidney (left) and Abnormal Kidney (renal failure) showing scarring (blue)

Aside from the clinical and economical manifestations, ADPKD has detrimental effects on various other aspects of an individual’s life. Individuals with ADPKD have commented that the condition has a negative impact on their employment, obtaining health and/or life insurance and increases anxiety and depression due to the concerns of dialysis and transplantation, and the impact of a dominantly inherited condition on themselves and their families.

Why is there no standardized treatment and knowledge base surrounding the disease? What factors have limited the progress towards standardization?

The optimization and standardization of ADPKD care in Europe is hampered by the lack of evidence-based consensus guidelines and standardized care pathways. Although recommendations have been developed in certain countries (e.g. Spain) and for specific medication they are limited by the lack of good-quality data on many aspects of care.

The EAF Report cites data from the ERA-EDTA registry showing that the prevalence of dialysis or transplantation use for ADPKD varies between European countries. This variation thought to be a result of international differences in policies governing access to, and reimbursement of, dialysis and transplantation.

Currently, kidney transplantation is the treatment of choice for ADPKD. However, financial pressures, a shortage of donors and limitations in medical, surgical and nursing expertise are preventing more widespread use of kidney transplantation.


What is the ‘Brussels Declaration on ADPKD’, what does it signify and what changes does it hope to make to EU policy?

The declaration is a ‘Call to Action’ to policy makers, hospital managers, patient groups and those interested in ADPKD care. The declaration:
Summarizes unmet needs in ADPKD
Presents six policy-focused recommendations

These recommendations are broadly grouped into the following themes:
Collaboration: Increase platform for continued dialogue and collaboration to improve the quality and longevity of lives of individuals with ADPKD
Improved care: Develop model for ADPKD ‘tiered care’
Research: Increased research collaboration
Empower: Increase influence of and role of patient groups and their role in health technology assessments (i.e. contributing to the assessment of medications)

The primary goals of the Declaration are:
Raise awareness of ADPKD and all its implications for patients, health services and economies in Europe
Recommend strategies to improve ADPKD care at EU & national levels
Encourage and facilitate collaboration between the individuals and groups involved in the management of ADPKD. [Read more]

From Longview News-Journal, Texas

Polycystic Kidney Disease support group

The East Texas Chapter of the PKD Foundation will have a support and encouragement meeting for families affected by Polycystic Kidney Disease from 1 to 3 p.m. March 19 at the Hope Fellowship Church, 300 Hollybrook Drive in Longview.

This meeting is for PKD patients and their families, caregivers and friends. Everyone is invited to come to the free event to learn more about this hereditary disease. Snacks and drinks will be provided. For information, call Teresa at (903) 759-3141 or at eastexaschapter@pkdcure.org.

Gift of Life

From The Daily Star, Oneanto, NY, By Jessica Reynolds

A Delaware County man whose life was saved by a kidney transplant is celebrating National Kidney Month by encouraging others to become organ donors.

More than 26 million Americans have chronic kidney disease and, because it can develop for years without noticeable symptoms, most of them don't know it, according to the National Kidney Foundation. If left untreated, chronic kidney disease can lead to kidney failure, requiring dialysis or a transplant for survival. There are more than 95,000 people waiting for kidney transplants, according to the foundation.

National Kidney Month is about raising awareness, but it's also about hope, highlighting medical advances and celebrating success stories, according to the foundation. Shayne Moshier, a 53-year-old Andes native, is one of these success stories.

Moshier was diagnosed at age 16 with polycystic kidney disease, a hereditary disorder in which clusters of cysts develop in the kidneys, he said Monday.

“My dad had it,” said Moshier, who owns a fabrication and welding shop in Andes. “I was able to maintain it quite well until my mid-40s, and that's when everything went to hell.”

For a year, Moshier — who was a member of the Andes Fire Department and on the town board — traveled three times a week to a Bassett Healthcare Network clinic in Oneonta to receive dialysis, he said, which is a process that helps filter toxins from the body when the kidneys can no longer perform these functions.

Soon, Bassett offered Moshier a game-changer: the option of home hemodialysis, which he did for five years, he said, all while serving on the town board and operating his business alongside his wife, Tina.

“The dialysis was time-consuming," Moshier said. "It took us about five hours. But for someone who's active, it's the way to go.”

After six years of dialysis, Moshier faced kidney failure and needed a transplant in order to live, he recalled. He was referred to Strong Memorial Hospital in Rochester. Nine months later, on Dec. 12 2013, he finally received a kidney transplant.

“I was in the hospital for five days,” Moshier said. “I was told that the donor was a 56-year-old male who had been hit by a car in Rochester. He donated many of his organs.” [Read more]

From WCMH, Channel 4 NBC Affiliate, Columbus, OH, By Carolyn Yaussy

Columbus woman receives kidney during National Kidney Month

Lori Coulter suffers from polycystic kidney disease, a condition that ends in kidney failure and causes a host of other complications.

Coulter spent two years on dialysis after her first transplanted kidney failed in April of 2014. Faced with a three- to five- year wait for a new kidney on the National Registry, Coulter turned to Facebook for help. She set up a page called Find a kidney for Lori, and to her surprise a complete stranger came to her rescue.

“Angela Allen is my angel donor. I don’t know her story as to what [or] why she decided to help but I’m so thankful and blessed that she did,” said Coulter.

Although many people from around the county were tested, Allen was Coulter’s match.

March is National Kidney Month, and fittingly both women will undergo surgery at the Ohio State University Wexner Medical Center on Tuesday.

Coulter feels blessed that Allen chose to get tested, saying, “It amazes me that there are people in the world who are willing to give an organ to a total stranger.”

Sisters match, Improving Home Dialysis; Biking to Paris for PKD

Gift of Life

From The Salem News, MA, BY ETHAN FORMAN
Danvers sisters a perfect match in kidney transplant

Susan Kelly Vitale, 56, of Danvers, has been battling polycystic kidney disease since she was 19, her older sister says.

The disease is hereditary, and it leads to cysts in the kidneys, causing symptoms such as high blood pressure, chronic pain and kidney stones.

Her sister, Danvers resident Judy Procopio, 59, said Vitale, a hairdresser at Noggins Hair Salon in Middleton, has lived for many years with PKD, using blood pressure and other medications to control symptoms. But when her kidney function began to fail, and the cysts attacked her liver and pancreas, she needed a transplant.

Turns out, Procopio, who has been tested and does not have the gene for PKD, was a perfect match for her older sister when it came to donating a kidney.

"It's almost like I'm giving her [her own] kidney because it's such a perfect match for her," said Procopio, who works in airport operations for JetBlue in Boston.

On Feb. 16, both sisters underwent five hours of surgery at Tufts Medical Center in Boston; both are now home recuperating.

"After my kidney was successfully removed, [Susan] was in the adjoining room waiting to receive it," Procopio said in an email. "The transplant team is second to none in the country, and we thank them for their expertise in their field."

Procopio said the result of the transplant surgery is her sister will be able to avoid dialysis treatments, and she will be able to lead a normal, productive life.

Procopio said she and her sister are close. In all, there are five siblings, including two older sisters and a brother.

The sisters grew up in Danvers with three other siblings — two older sisters and a brother — but then their father, who worked for General Electric, relocated the family to Rutland, Vermont, where Susan and Judy attended high school. They eventually moved back to Danvers and they live about two miles apart now.

Procopio said the sisters are also grateful for financial help they received through a Gofundme.com page Vitale set up to help with medical and other bills not covered by insurance. Any remaining funds after expenses are paid will be donated to the National Kidney Association, Procopio said.

Dialysis News

From The Hill, Congressional Blog, By former Rep. Karen L. Thurman

Congress can improve access to home dialysis

The day the truck arrived to deliver my husband John’s home dialysis supplies, our quality of life changed for the better. As family, we had been completely overwhelmed by his kidney failure; he had to make three, 100-mile trips to a dialysis center each week, to be hooked up to a machine for up to six hours. After visiting the center, he was drained, lethargic, and worn out. With home dialysis, our family could be a family again.

My husband was diagnosed with hereditary polycystic kidney disease (PKD) and knew he eventually would go into kidney failure. Most people are unaware that their kidneys have failed until they wind up in the hospital – which means they lose out on a comprehensive discussion of their treatment options. Patients, my husband being one, are often encouraged to do their dialysis at a center. I strongly believe that patients should receive accurate information about their option to dialyze in the comfort of their home. Research shows 40 to 50 percent of patients will choose home dialysis when educated about this option.

According to a report released this week by Matrix Global Advisors, patient education is just one of the obstacles to home dialysis – many of which are tied to federal policies – that can be easily overcome. Patients who understand their options and prefer to dialyze at home are often deterred by the requirement of a care partner for home hemodialysis. For low-income single patients in particular, the cost of hiring a care partner often prevents the use of home dialysis.

Medicare provides some assistance to dialysis patients, but gaps in coverage prevent greater use of home dialysis. Today, Medicare limits home hemodialysis sessions to three sessions per week, despite evidence that more frequent sessions would be beneficial. In addition, Medicare does not cover the full cost of training a patient to dialyze at home and requires patients to visit their physicians in-person once a month, even when the patient is in good health.

This Congress, policymakers have the chance to improve access to home dialysis.

In 2015, the House introduced a comprehensive telemedicine bill – H.R. 2948, the Medicare Telehealth Parity Act – would make it easier for home dialysis patients to use telemedicine connections and remote patient monitoring to connect with their physicians.

The Senate has made similar progress through the Senate Finance Chronic Care Working Group, which released some options to improve the care and treatment of Medicare beneficiaries, such as those with kidney failure. The options included expanding access to home dialysis treatment through the use of telemedicine. Hundreds of patient groups responded to these options; the responses even listed other ways to improve access to safe dialysis treatment, including education on dialysis options, care partner issues, and training. The Committee’s proposal is a start. It may be one of the few bipartisan measures on which Congress could act that would make significant improvements to the care received by dialysis patients when it is finalized later this year.

As a former member of Congress and caregiver to a dialysis patient, I urge both the House and the Senate to support policies to increase access to home treatments. I am glad John was fortunate enough to have this option. It significantly improved his and our family’s quality of life.

PKD Fundraising

From the Loughborough Echo, United Kingdom, BY ZOE HIGGINS

Epic ride as Dan gears up for transplant op - to gain mum's kidney

A MAN who is due to receive a kidney from his mother this summer has created a comic strip of his story ahead of a charity cycle ride to Paris.

Dan Norcott, 41, who lives with wife Ali and son Arthur in Wymeswold, has Polycystic Kidney Disease, the same condition that unfortunately led to the death of his father Malcolm in 2004.

Since his diagnosis at the age of 21, Dan's kidney function has coasted slowly downwards so later this year his mother Jenny will donate one of her kidneys.

But before the operation, cycling enthusiast Dan has decided to pedal 388 miles from his home in Wymeswold to Paris in 10 days to raise money for The Leicester Kidney Care Appeal.

Dan's longtime friends Rich Moss and Andy Stafford will be joining him on the trip in May, and their online JustGiving page has already raised more than £4,200.

And to raise awareness of their fund-raising efforts, Dan has created a comic strip with the help of Terry Wiley which tells his story.

"Dad had two transplants, and many years of dialysis, which he found incredibly difficult," said Dan.

"Mum had to learn how to operate the machine housed in a portable building in our back garden, and for many years acted as his nurse as well as his wife.

"Still, he was determined to enjoy life, and we escaped to the countryside whenever we could.

"I was moved to tears recently when a picture of him halfway up a mountain, post-transplant, was shown as inspiration during a presentation at a recent chronic kidney disease information day. He set a fine example that I intend to follow.

"Various complications with the second transplant led to an early death at the age of 59.

"His was an unpleasant death but he died with incredible grace and fortitude.

"In short, I'm very lucky. I'm ill but a treatment is available, and I have someone willing to give me a gift that could extend my healthy life by a decade or more.

"Many people at this stage experience great pain, but I have very little, and my symptoms are mostly just a drowsiness and general lack of pep.

"When I was young, my dad told me about a long bike ride he???d been on with his friend Frank, where they tried to get all the way round the coast of England.

"I've always wanted to go on such an extended trip, but never thought I would have the time.

"When I recently learned that my transplant was so close, I decided that I should grasp the opportunity while I still had it, and use it to try and do some good."

To donate, please visit pedallingtoparis.com