Reducing Transplant Waiting; All in the Family; Youth and PKD; Boston Walk for PKD; Somatostatin analogues show promise

Reducing Transplant Waiting

From Lincolnshire Echo, United Kingdom

Lincoln woman says revolutionary kidney treatment "changed her life"

A Lincoln woman says a pioneering kidney transplant operation has "changed her life".

Deborah Bakewell was one of the first patients in the world to receive the treatment which uses a donated organ flushed with oxygenated blood after being kept in cold storage.

Deborah had the operation, which is thought could cut transplant waiting lists by more than 10 per cent, at Leicester General Hospital.

Following Deborah's treatment, which was funded by Kidney Research UK, around 40 people have successfully had the operation.

Speaking to the BBC Deborah said: "It was the difference between changing my life or continuing on the treadmill of dialysis.

"I have got more energy and I can consider going on holiday before I had the treatment I could go on holiday.

"It has really changed my life.

"I just thought 'well this is an opportunity I must take' it was only a split second when I thought 'great I am going to be a guinea pig here'."

Deborah was diagnosed with polycystic kidney disease, which gradually leads to kidney failure, when she was 23.

Before the operation, Deborah endured nine hours of dialysis a day.

The technique, known as normothermic perfusion, boosts the effectiveness of damaged organs which previously would have been rejected as unsuitable for transplant.

The treatment improves the function of damaged kidneys from the elderly or people with high blood pressure and diabetes, and cuts the risk of the organ being rejected.

Living with PKD

From Augusta Chronicle, Augusta, Georgia, By Tom Corwin
Transplant patient finds kidney problems run in family

W. Frank Walker lost his kidneys to polycystic kidney disease and received a transplant from his stepdaughter. The disease has moved to his liver.

It wasn’t until kidney problems surfaced for W. Frank Wal­ker, 65, that he really began exploring his father’s side of the family and turned up a terrible history of kidney disease leading to transplants and in some cases death.

Walker underwent a “miraculous” kidney transplant but is now struck by a rare form of his disease that also attacks the liver, putting him back on the transplant list. He has one big lesson he wants to share with others: “It’s important to know your family history.”

That wasn’t possible for him early on. His parents divorced when he was young and he spent most of his life in Florida and didn’t really know his father’s side of the family well.

After moving back to the area to take care of ailing grandparents, he developed kidney stones and was diagnosed in 1986 with polycystic kidney disease, where fluid-filled sacs form on the kidneys and can eventually shut them down. He was told it was a hereditary disease, which led him to look at his father’s side of the family. Though he was told his father died from a stroke at 46, Walker discovered he actually had kidney disease, which led to more digging.

What he found was that his kidney disease “runs rampant through my father’s side of the family,” Walker said. “When I found out about it, I started contacting my family members on my father’s side, letting them know about this insidious disease because people weren’t aware of it.”

Some older relatives might have known about the history of kidney disease but kept quiet for fear it might lead to denying them insurance, back when pre-existing conditions could do that, he said.
Walker said that after being referred to the pre-transplant program at Geor­gia Regents Medical Cen­ter, the care he got there helped him go 20 years before the kidneys failed and he needed a transplant. Remarkably, he matched perfectly with his stepdaughter, Maya Brown, who is not a blood relative.

“I was blessed to have a stepdaughter because none of my siblings would have been eligible for transplant,” Walker said. “And none of my children would have been eligible to be a donor.”

But just a year after the transplant, Walker had to have part of his liver removed because it too had begun to sprout cysts. It caused his liver to enlarge to the point where it was crushing the rest of his digestive system. He eventually was put on a transplant list for a new liver at Piedmont Atlanta Hospital.
Because polycystic liver disease usually doesn’t cause liver failure, Walker at first was rejected because it wasn’t seen as life-threatening. But after problems with eating and digestion caused him to lose 80 pounds in a year, he qualified for a transplant last year.

Now, he is working with the Georgia Transplant Foun­da­tion to raise money to help him afford the medication he will need after the surgery, on top of the anti-rejection medication he already takes for his kidney.

Walker’s efforts to reach out and inform his family have revealed the terrible toll it is taking on them. Since his transplant, three cousins have had kidney transplants, three others are on dialysis and waiting for a transplant and two cousins have died waiting for kidneys.

Walker is probably not the only one in the Augusta area waiting on a new liver. [Read more]

From The Hans India, By:V Naveen Kumar
Hyd youth seen falling prey to kidney diseases

Hyderabad: Increasing number of youth falling prey to kidney failures is causing concern among doctors. Though there has been sudden increase in kidney failures all over the world, particularly in developing countries like India, the youth getting afflicted by the disease in increasing numbers in Hyderabad should be a wake-up call for the government, it is felt.

At least 70 per cent of patients of kidney disease are in the age group of 20-40, according to the Head of the Department of Nephrology, Nizam Institute of Medical Sciences, Dr D Sree Bhushan Raju. Another 20 to 30 per cent patients were affected due to acquired conditions and some of them genetic conditions like polycystic kidney disease.

Explaining the background to the sudden explosion of kidney failures, Dr Raju said youth in the age group of 20-40 too were diagnosed with suffering from hypertension, which is called “high BP” in common man’s parlance. Those, affected by the hypertension, would generally feel that they were affected it because of stress. But there was no relation between tension and hypertension, Dr Raju explained.

The delay in diagnosing the hypertension and failure on the part of patients to take medication was also the cause for damage of kidneys between 30 and 40 per cent. Patients with hypertension would visit doctor very late and it would result in detecting it. Dr Raju, while cautioning the youth to be careful, said now there was no need for panic over the kidney disease.

Wonderful treatment for the disease in the form of dialysis and also transplantation were available. But, unfortunately most of the patients, succumb to disease before they visit the doctor. He urged people to visit doctor as soon as disease has manifested itself.

Walk for PKD

From WCVB ABC Affiliate Boston

Sunday September 20, 2015: Boston Walk for PKD

BOSTON —This year, 4-year-old Jamie Rice and his uncle Mike of Boston will join thousands nationwide to come together and walk in support of those affected by polycystic kidney disease (PKD). PKD is one of the most common, life-threatening genetic kidney diseases. It affects thousands in America and millions worldwide, who are in urgent need of treatments and a cure. It is the fourth leading cause of kidney failure.

The 2015 Boston Walk for PKD is planned for Sunday, Sept. 20 at DCR’S Artesani Park. PKD patients, their families, friends and members of the PKD medical community will be fundraising and walking together to unite to fight and end PKD. Registration is at 9 a.m., with the Walk beginning at 10:30. Activities for children will take place at 10 a.m.

Jamie has been participating in the Walk for PKD with his family since 2013. Each year, Jamie’s goal is “to help Uncle Mike because he has a really bad disease that tells his kidneys to make bubbles. Then his kidneys can’t do their job.”

When Jamie first learned his uncle had PKD, he insisted on giving Mike extra kisses to help him feel better. He also had plenty of questions: what is a kidney? Does Uncle Mike hurt? Will he get better? How does a transplant work?

Jamie commented, “A new kidney has to come from someone else like me, but I’m too little. I can get money to people to help Uncle Mike, and those people try to find a way to make people feel better while they have PKD or to make it stop.”

Jamie has a piggy bank, which he named his “Help Uncle Mike PKD Pig.” Each year, he fills it with coins that he’s found or change his parents have given him and donates the money at the Walk for PKD. Last year, Jamie helped his team raise more than $20,000.

The annual Walk for PKD is the signature fundraising and public awareness event for the PKD Foundation and is the largest gathering of PKD patients, family, friends and supporters; more than 11,000 strong. The Walk for PKD is hosted in more than 50 cities nationwide. Since 2000, the Walk for PKD has raised nearly $24 million. This money raised supports the PKD Foundation’s efforts to fund research to find treatments and a cure, and to provide information and support to people affected by PKD.

Register for the Boston Walk for PKD sponsored by WCVB at walkforpkd.org/boston.

PKD Research

From Healio

Somatostatin analogues found to improve HRQL in patients with polycystic liver disease

In an analysis of placebo-controlled clinical trials, somatostatin analogues improved health-related quality of life among patients with polycystic liver disease, according to study data.

Researchers analyzed pooled data from two studies that evaluated health-related quality of life (HRQL) in 96 patients with polycystic liver disease (PLD) after treatment with somatostatin analogues Sandostatin (octreotide, Novartis Pharmaceuticals; n = 24), Somatuline Depot injection (lanreotide, Ipsen Pharma; n = 27) or placebo (n = 36) for 6 to 12 months. The tool Short-Form 36 was used for the evaluation to determine whether somatostatin analogues improved overall HRQL and to identify factors associated with change in HRQL in PLD.

“We used random effect models to delineate the effect of somatostatin analogues on HRQL,” the researchers wrote. “We determined the effect of demographics, height-adjusted liver volume, change in liver volume, [and] somatostatin analogue-associated side effects with change in HRQL. In patients with autosomal dominant polycystic kidney disease, we estimated the effect of height-adjusted kidney volume and change in kidney volume in relation to HRQL.”

Analyses showed that somatostatin analogues improved physical component scores (PCS), “but remained unchanged with placebo,” (3.41 ± 1.29 vs. − 0.71 ± 1.54; P = .044) according to researchers. However, somatostatin analogue treatment had no impact on the mental component score.

Age, gender, diagnosis and change in liver volume were not associated with change in PCS. A larger liver volume at baseline was found to be associated with a greater decrease in PCS and HRQL over the course of follow-up (−4.04 ± 2.02 points per logarithm liver volume; P = .049).

In a subgroup of patients with autosomal dominant polycystic kidney disease (ADPKD), treatment with the somatostatin analogues also improved PCS compared with patients who received placebo (P = .01). In addition, patients with ADPKD with large liver and kidney volumes had a greater decrease in HRQL (5.36 ± 2.54 points per logarithm liver volume; P = .040 and − 4.00 ± 1.88 per logarithm kidney volume; P = .039), respectively.

“Somatostatin analogues improve HRQL in symptomatic polycystic liver disease,” the researchers concluded. “Halting the progressive nature of polycystic liver disease is necessary to prevent further decline of HRQL in severe hepatomegaly.” – by Melinda Stevens

Organ Donor Heros; Time to Take Them Out; PKD Fundraising; Gift of Life

Organ Donors Save Lives

From The Whig Standard, Kingston, Ontario, Canada, By Anisa Rawhani,

Frank Wallwork received kidney from deceased donor

A deceased stranger saved Frank Wallwork from the same disease that took his mother.

Wallwork, and many of his family members, have polycystic kidney disease, an inherited disorder that takes over the kidneys, often leading to a decline in the organs' function.

He knew years ahead the disease was coming, and in 2010 his kidneys failed.

He soon went on dialysis. For four years, three times a week, he'd be hooked up to a machine that would take over the kidneys' job of filtering, four-hour sessions at a time.

Three years into the process, he and his wife decided Wallwork should go on the waiting list for a transplant.

Wallwork was one of seven people to receive a kidney transplant performed at Kingston General Hospital in the 2014-15 year.

In the past year, deceased and living organ and tissue donors saved a record number of lives in Ontario. Among them were 12 donors from Kingston.

Since 2011, 5% more Kingston residents have become registered donors, which puts the city 16% higher than the Ontario average.

But while Ontario is a leader in tissue and organ donation, there are still 1,572 Ontarians waiting for a life-saving transplant, and every three days one of them dies.

Although more than 85% of Ontarians are in favour of organ donation, millions of eligible donors haven't registered.

Wallwork waited two years before a kidney from an anonymous deceased donor became available.

"I've always wanted to thank somebody," he said, but, as the donor is deceased, the process isn't as straightforward. "It's hard to get the right words to thank somebody for that."

"(Donors') families should consider them heroes."

One organ donor can save eight lives. The registration process takes two minutes at www.BeADonor.ca and can also be done at ServiceOntario centres when renewing a driver's licence or health card.

Re

Taking Action

From The Times of India, Coimbatore, by Pratiksha Ramkumar

City hosp removes man's kidney with massive cysts

COIMBATORE: For almost a decade, Vinod, 46, lived knowing he had cysts in his Kidneys which were growing in size every year.

But on June 26, when Vinod, who works for an oil refinery in Kuwait, began bleeding while urinating, he knew he could no longer procrastinate treatment.

Vinod had been long diagnosed with a hereditary condition, known as polycystic kidneys, after scans revealed cysts in the kidneys. But due to financial constrains, Vinod postponed his treatment for almost a decade until he knew he could no longer do so after the incident on June 26.

The disease, which affects one in several thousands of people across the globe, causes clusters of cysts to develop within the kidneys. Cysts are non-cancerous round sacs containing water like fluid, vary in size and can grow very large as they accumulate fluid.

Vinod's mother, who had been also diagnosed with the same condition, had been operated over a decade ago.

In the case of this disorder, over a period of time, say a decade or two, the cysts proliferate in number and size replacing the entire kidney which leads to a complete kidney failure.

"Ten years ago, around the same time his mother underwent surgery for a a kidney transplant, he complained of a severe headache. Doctors, who did his prognosis, informed him that his kidneys were beginning to malfunction too," said Dr Devdas Madhavan, consultant neurologist and transplant surgeon at Kovai Medical Centre and Hospital (KMCH). "Despite knowing that he too was suffering from the same condition, he decided to wait."

A decade later, after quickly resigning from his job in Kuwait, Vinod walked back into KMCH on June 28, with an unusually bloated stomach and a hemoglobin level that stood at five compared to the normal 12 to 13. "The cysts swelled up in both kidneys weighing 11.8kg," said Dr Madhavan. "The cysts had completely replaced the kidneys and this led to kidney failure" he said.

The patient was immediately put on dialysis and his haemoglobin levels were brought back up to normal. The doctor and his team comprising nephrologist Dr Vivek Pathak and anaesthetists Dr N Selvarajan and Dr Shantini performed a three-hour surgery on Monday to remove the cysts. "The surgery was a challenge as the cyst needed to be removed without damaging any of the blood vessels and finding these vessels can be tricky," said Dr Madhavan. "Damaging the vessels would lead to excessive bleeding," he said.

Vinod is currently recovering and has been put on dialysis. After another two months, doctors plan on a kidney transplant.

PKD Fundraising

From The Daily Observer, Pembroke, Ontario, Canada, By Ryan Paulsen

Kidney Walk slo-pitch tournament raises $5,000

Ryan Paulsen / Daily Observer Colleen McMillan and Dan Munro throw out the ceremonial first pitch at the 3rd Annual Mixed Slo-Pitch Tournament, held in support of the Pembroke Kidney Walk at Stafford Park on Saturday. In October, 2014, Munro, this year's Kidney Walk ambassador, received a kidney donation from McMillan, a good friend of his wife.

For the third year running, the Stafford Ball Diamond was the place to be this weekend to take in some excellent slo-pitch action for an excellent cause, as the Sutherland family (Don, Cathy, Colleen, Meghan and Heather) hosted their annual tournament in support of the Pembroke Kidney Walk and the Canadian Kidney Foundation.

“This year’s tournament was a huge success," says Heather Sutherland, "thanks to all the amazing support we get from the community. Between donations for baskets, people giving their time and services during the event and volunteers, we receive so much help and the whole Sutherland family is grateful for this.”

The Sutherlands have been mainstays in the local fundraising scene to the benefit of the Kidney Foundation since Don was diagnosed with polycystic kidney disease and received a transplant after a live donation from his wife in 2008.

This year's tournament title sponsor was The Sutherland Team, made up of Lifelong Financial Solutions and Meghan Sutherland Mortgage Team.

Twelve teams converged on the field for a full day of play, with the ceremonial first pitch thrown out by Dan Munro, this year's Kidney Walk ambassador, and Colleen McMillan. Last year, McMillan, a long-time friend of Munro's wife, donated a kidney to him, freeing him from the restrictive routine of dialysis.

"It's totally changed my life," says Munro. "I'm free. I can finally do the things I want, like travel."

At the end of the tournament, Almost Heroes emerged victorious, clutching the A-division championship title, while the Alice Outlaws scooped up the top spot in the B-division.

Off the field attractions included car washes provided by CodyMobile, free Kangen Water by Second Chances 4U, massages available for donations by The Lavallee Centre, a basket raffle, bat raffle, barbecue and 50/50 draw.

The real winner, however, was the Kidney Foundation itself, which will see the benefits of the more than $5,000 raised over the course of the day.

Another feather in the cap for the tournament was the presence of Kim Sheldrick, an Ottawa volunteer with the A4 Lions and the Gift of Life Network. Sheldrick was promoting the organ donation cause, and even got seven more people to sign up as registered organ donors.

“The ability to have people sign up as organ donors on site was as great addition this year," says Heather. "It’s so wonderful to see people recognize the importance this plays in saving people’s lives.”

This year's Kidney Walk is slated for Saturday, Sep. 26 at the Pembroke Waterfront, with all proceeds going to support the work of the Kidney Foundation of Canada.

Gift of Life

From Tri-City Herald, Voice of Mid-Columbia, Washington State, BY SARA SCHILLING

Pasco man recovering after kidney transplant

Vaughn Jensen, left, donated a kidney to his friend Tanaka Modac.

A 45-year-old Pasco man who was awaiting a new kidney is recovering after a successful transplant surgery.

Tanaka Modoc had the transplant on June 30 at Virginia Mason hospital in Seattle.

He’s been discharged from the hospital, but will stay in Seattle for a while longer to be near his doctors as he recovers.

He could be cleared to head home at the end of the month, said his wife, Darla, in a phone interview from Seattle.

“Everything is going really well,” she said.

Modoc’s donor — a friend from the Tri-Cities — also successfully came through the transplant operation.

Vaughn Jensen, 46, of Benton City, was cleared to return to the Tri-Cities about a week after the surgery.

He offered to be tested as a possible kidney donor for Modoc not long after the two friends met a couple years ago.

Becoming eligible for testing required effort and commitment. Jensen had already quit drinking, but he started exercising and eating better and shed about 30 pounds.

Jensen couldn’t be reached Friday. But he told the Herald last month that, “I believe God puts us in this world to do things. I believe this is one of the reasons I was born — to give this kidney to him.”

Modoc, who spent 20-plus years as a pipe fitter before medically retiring, experienced kidney failure three years ago because of polycystic kidney disease, a genetic condition in which cysts develop within the kidneys. In February of this year, Modoc’s kidneys were removed altogether.

Before the transplant, Modoc talked with the Herald about his excitement. He’d been tied to dialysis since his kidneys failed, and “Vaughn is letting me out of jail. He’s letting me have freedom. I’ll be free again,” he said. [Read more]

Prom Miracle; Kidney Donors Needed; Fierce Drug Delivery Against Cysts; Giving Kidney at age 77

Living with PKD

From Plymouth Herald, United Kingdom, By ALEX SHIPMAN

Biker escort takes 'miracle' teenager Beth to her prom

A "MIRACLE" teenager who wasn't expected to live past the age of two was escorted to her school prom by more than 100 bikers who turned out to surprise her.

"Shocked and emotional" Bethany Crocker was taken to her prom in a Ferrari surrounded by dozens of motorbikes after her dad appealed on Facebook for volunteers to help make the 16-year-old's day special.

Bethany's spectacular entrance at the Copthorne Hotel drew cheers from spectators on the street after kind strangers came forward when her dad Derek's social media post was shared more than 700 times.

Bethany was born with polycystic kidney disease (PKD) and her parents were advised to terminate the pregnancy.


At birth, she was moved to a specialist children's hospital in Bristol because of the rare condition, which affected only four other people in the world at that time. It means Bethany's kidneys only function at 30 per cent capacity so she has to be tested regularly at Derriford Hospital.

Her father, Derek, said: "Bethany is a free spirit and I think because of her long-term illness she lives day by day. She is a miracle."

The convoy met in Honicknowle before the All Saints Academy prom – when they pulled away, people on the street were filming and clapping.

"When we got to the prom, we pulled up at the main doors and there wasn't enough room to get all the bikes in so they waited on the road," said Derek.

"We went round to the red carpet at the back of the building, Bethany got out and everyone revved the hell out of their engines.

"It was the best entrance this town has ever seen – for total strangers to come together in that number for a girl who has been seriously ill is absolutely heart-warming.

"I was hoping for four at the front of the car and four at the back, but the response was unreal." The Ferrari was lent to the family by owner of Jem Scaffolding, Darren Toms.

Derek recalled: "I told my girlfriend's brother about the situation and he spoke to Darren and Darren got back to us within a couple of hours to say we could have the car.

"The crazy thing is that it was Darren's son's prom on the same night, but he gave us the Ferrari instead!

"I have never met Darren or spoke to the gentleman before, but this is an absolutely beautiful act of kindness by him."

Darren also made a donation to the children's wards at Derriford Hospital so that they could purchase some new toys on behalf of Bethany.

The teenager's parents have separated and she now lives with her mother, Tina, in Weston Mill.

The family regularly attend church on a Sunday and Derek has said that "everybody prays for her".

If her kidneys fail, which is likely, Bethany would go on to dialysis before needing a kidney transplant. [Read more]

From The Indian Express

At 77, retired manager gives wife new life, gifts his kidney

Two years after transplant for polycystic kidney disease, Moti Lalwani and Padma look forward to celebrating their 50th wedding anniversary

At 77, Moti Lalwani did not think twice before gifting a kidney to his ailing 75-year-old wife, Padma. Two years after a successful kidney transplant, the couple look forward to celebrating 50 years of married life. “While it is not uncommon, a transplant at that age is rare,” say nephrologists.

“I feel absolutely normal. After all, if you can see with one eye, then you can function with one kidney as well,” says Lalwani.

Padma says she had polycystic kidney disease – an inherited kidney disorder. Her condition worsened and nephrologists soon recommended dialysis. “That’s when my sons decided that a transplant would be better,” Padma recalled.

“Padma suffered quite a bit. She had to undergo dialysis which is painful and had to get used to a rigorous diet. Plus, we were anxious that she might contract any other infection and hence decided on a transplant. Easier said than done. We had to rule out her ageing brothers, and my sons also did not fit the medical criteria,” says Lalwani, adding, “My blood group was O+ve and while doctors refused initially considering my age, it was the lack of any medical ailments or related problems like blood pressure and diabetes that they gave the green signal to the transplant. The transplant was done in September 2013,” recalls Lalwani, who retired as an operations manager at Tata Power Company. 

“The medical examination showed that my kidneys even at 77 years were perfectly normal,” Lawani said adding that the operation was conducted at Mumbai’s Breach Candy Hospital by Dr Bhependra Gandhi. In Pune, Dr Avinash Ignatius, who was treating them at Noble hospital and is a senior consulting nephrologist at DaVita health care, said the Lalwani couple perhaps was the oldest donor-recipient pair. He adds that the couple’s successful transplant operation shows that age in some cases may not matter. 

“However, it is essential to do a proper medical check. Every month, we examine at least 80 new patients with chronic kidney disease. There are at least 10 who need dialysis every month and there is a long wait list for kidney transplants,” says Dr Ignatius, who is also a member of the Pune Zonal Transplant Coordination Committee (ZTCC) [Read more]

From Mohave Valley Daily News, Bullhead City, Arizona, by DK McDONALD

Search for kidney donor continues

Friends of a local woman are reaching out to the community to find a living kidney donor.

Stephanie Hendrix, a long-time resident of Bullhead City and Fort Mohave, has polycystic kidney disease, which causes cysts to grow in the kidneys. She is in desperate need of a living donor for a kidney transplant.


“Since March, between 20 and 25 people, most from the Bullhead City area, have come forward to be tested for compatibility,” said Nancy Mongeau, a friend of the Hendrix family. “Five went on to the second level of testing, but so far, no one has matched.”

Hendrix has no kidney function and is currently undergoing dialysis three hours a day, three times per week. She has A-positive blood type and the donor’s blood type can be A-positive, A-negative, O-positive, or O-negative.

Living donors must be between the ages of 18 and 60.

Mongeau and other friends have banded together to help locate a living donor and to fundraise to support that donor through the transplant process.

“The donor’s medical expenses are covered 100 percent,” Mongeau said. “There is financial help available to the donor to cover any uncovered expenses and lost income.”

A frequent question from potential donors is what happens if they donate a kidney and then they or their immediate family needs a kidney.

“A donor in need of a kidney goes to the top of the kidney donor recipient list,” Mongeau said.

For answers to other questions, or to be tested for compatibility, call Winnie of Banner Good Samaritan’s Live Donor Team at 602-839-0210.

“There are steps to become a donor,” Mongeau said. “The first is to contact Winnie at the Living Donor Network. Winnie will schedule or conduct a telephone interview with people who call. If the interview determines a possible match, the potential donor will have their blood type verified at Sonora Quest Lab in Bullhead City.

“If a blood type match is made, the donor will be interviewed for a complete physical and psychological evaluation. The final step is for the donor to meet with the Banner Good Samaritan Committee who determines suitability.

“For people who really want to know what it is like to become a donor, I recommend the Erma Bombeck Project website; it covers everything.”

The Bombeck Project site can be found at https://azkidney.org/erma-bombeck-project.

PKD is a genetic disorder, inherited from a parent. About 600,000 people in the U.S. have PKD. It is the fourth leading cause of kidney failure.

Hendrix, the wife of Dr. Richard “Buck” Hendrix, is a Mohave High School graduate, life-time member of River Valley High School Booster Club, and a founding member of The River Fund, Inc., a local nonprofit which provides direct emergency and crisis services for individuals and families in Bullhead City, Fort Mohave, Mohave Valley and Laughlin.

“I am asking the community to please help Stephanie,” Mongeau said. [Read more]

From KDKA, CBS affiliate, Pittsburgh, PA, by Brenda Waters

Salvation Army Co-Workers Reunite After Kidney Transplant, Both Doing Well

It has been nearly a month since a Salvation Army employee gave a co-worker a second chance at life.

One gave the other a kidney, and now both are doing great.

Mike Riemer, 55, is moved by the many cards he has received, some from strangers.

His kidney donor, 28-year-old Kate Esker, is happy to know that others now want to become living donors.

“I hope to inspire people, two people said they are going to look into getting checked to see if they can donate… been pretty inspiring,” said Esker.

Let’s go back to June 9.

Riemer knew for more than 30 years that his kidneys were failing; he has polycystic kidney disease. His mother, grandmother and great-grandmother all died from PKD.

After going public with his need for a donor, 17 people volunteered. Esker, Riemer’s Salvation Army co-worker, was a perfect match.

It is something Riemer says he thinks of often.

“I reflect on this multiple times every day,” he said. “I’m so grateful to the Salvation Army, to Kate Esker and her husband, and to God for making this happen.”

The two families got together Tuesday for the first time since the surgery in June.

“The joke was that we found the fountain of youth the hard way. A 28-year-old kidney with 28-year-old hormones is in my body now. I can’t remember when I felt this good,” said Riemer.

And Esker says, “I felt confident all the way through, and to hear all the good things from Mike.”

Esker was back at work two weeks after the surgery. Riemer hopes to return in another month.

PKD Research

From Fierce Drug Delivery

UCSB team tackles aggressive kidney disease by hitching a ride into cysts

Renal cyst fluid contains growth factors that cause the excessive proliferation and cyst growth associated with polycystic kidney disease.--Courtesy of UCSD

Researchers from UC Santa Barbara have developed a method by which to deliver antibodies to the kidney and curb a specific type of cyst growth that gives rise to polycystic kidney disease, which is inherited and has no known cure.

UCSB's Thomas Weimbs has deployed the antibody immunoglobin-G, which binds to specific proteins and growth factors in the kidney to inhibit cyst growth. But the problem with this treatment in the past is that the antibody can't penetrate the wall of a cyst that is already present, and it can't inhibit the proteins therein. To get past the cyst wall, Weimbs' team used immunoglobin-A, which can cross a cell layer by bonding to polymeric immunoglobin receptors, work that Weimbs had done in the past while conducting postdoctoral research at UC San Francisco.

"I put one and one together," Weimbs said in a statement. "I thought if STAT6 is highly active in polycystic kidneys, maybe it also expresses a lot of pIgR--and that turned out to be the case. So we tested this in mouse models and in human polycystic kidney tissues, and, in both cases, high levels of pIgR were expressed in kidney cysts."

In mice, the immunoglobin-A remained inside the cyst at a rate of 7% while getting a ride from the receptors. Once in there, the cysts are thought to hold them because they "don't have an exit," according to Weimbs.

The team published its results in the Journal of Biological Chemistry.

"Our strategy allows for the repurposing of thousands of existing monoclonal antibodies that have already been developed, which opens up a whole new class of therapeutics not previously used for PKD therapy," Weimbs concluded. "This paper is proof of concept that we can use IgA to target polycystic kidneys. The next hurdle will be proof of therapeutic efficacy where we actually take an IgA antibody targeted to a specific protein or growth factor and see if it can inhibit cyst growth."

From The American Journal of Physiology 

The cpk model of recessive PKD shows glutamine-dependence associated with the production of the oncometabolite 2-hydroxyglutarate
Abstract

Since polycystic kidney disease (PKD) was first noted over 30 years ago to have neoplastic parallels, there has been a resurgent interest in elucidating neoplasia-relevant pathways in PKD. Taking a non-targeted metabolomics approach in the B6(Cg)-Cys1cpk/J (cpk) mouse model of recessive PKD, we have now characterized metabolic reprogramming in these tissues leading to a glutamine-dependent TCA cycle shunt towards total 2-hydroxyglutarate (2-HG) production in cpk as compared to B6 wild type kidney tissue. After confirmation of increased 2-HG expression in immortalized collecting duct cpk cells as well as in human ARPKD tissue using targeted analysis, we show that the increase in 2-HG is likely due to glutamine-sourced α-ketoglutarate. In addition, cpk cells require exogenous glutamine for growth such that inhibition of glutaminase-1 decreases cell viability as well as proliferation. This study is a demonstration of the striking parallels between recessive PKD and cancer metabolism. Our data, once confirmed in other PKD models, suggest that future therapeutic approaches targeting this pathway, such as using glutaminase inhibitors, have the potential to open novel treatment options for renal cystic disease.

Transplant Crisis on BBC; Travel to Transplant; Rachel's Kids Donations; Donate Life Challenge; 30 years with a donated kidney

Gift of Life

From Scottish Sunday Express, By PAULA MURRAY

Donate the greatest gift to save a life

A FAMILY struck by genetic kidney condition have spoken out to highlight the crisis in Scotland’s organ transplant system.

Gary and Susan Robertson and their two sons feature in a heartbreaking new BBC documentary as they embark on the emotional rollercoaster ride of finding a suitable donor.

Gary is one of around 70,000 people in the UK with polycystic kidney disease, a life threatening, inherited illness which also affected his mother and grandfather.

Although he has known about the condition since he was 18 it wasn’t until around his 40th birthday five years ago that his health started to deteriorate.

However, neither he nor wife Susan, 42, had expected him to fall ill so young when the previous two generations had reached the age of 61 before needing a transplant.

The father-of-two was so desperately unwell the family’s life was literally put on hold.

Susan said she signed up as a living donor without his knowledge but after seven months of tests was told they were not compatible.

However, they singed up for a transplant chain and found matches involving two other couples and three hospitals in total.

She told the Sunday Express: “I was losing him. He became so quiet, he just was not the same man anymore.

“We knew the day would come when he needed a transplant but we had always believed he’d be more like his mother and grandfather and in his 60s before that happened.

“But that’s not how it worked for us.

“He went downhill rapidly.”

The couple, from Currie, near Edinburgh, kept their children, 13-year-old Murray and Sean, 10, in the picture from the start.

With hospital trips to dialysis sessions scheduled three times a week the boys became a huge part of the process.

Although they both have a 50 per cent chance of having the same condition as their father Susan and Gary will not have them tested as "there is nothing that can be done about it".

Gary's journey to better health has not been easy. He needed a pre-transplant operation after the surgeon found swelling in his kidneys caused by bursting cysts.

The, just 48 hours before the main surgery was due to go ahead, Susan developed a cold and the procedure was put on hold.

However, eventually three people with life-threatening illnesses were given a new lease of life last year.

Looking back at the "emotional rollercoaster ride" Susan said it had actually been a relief for Gary that she was not the perfect match for him.

The mother-of-two recalled: “Gary admitted he had been concerned that he might reject the kidney. He said he would have felt as if he was rejecting me and did not know how he could have coped with it.”

Last year 425 people in Scotland received an organ while around 550 remain on the transplant waiting list. [Read more]

From Forbes, by Peter Ubel

Your New Liver Is Only A Learjet Away: Part 2 Of 3

Irena Bucci was receiving follow-up care after delivering her second baby when the obstetrician discovered a problem with her kidneys. “My creatinine was rising,” creatinine being a waste product normally cleared out of the bloodstream by healthy kidneys, “and my doctor didn’t know why. I didn’t have high blood pressure or diabetes,” two diseases that are common causes of kidney failure. Bucci met with a number of kidney specialists, in hopes of uncovering the cause of her kidney failure. “But the tests didn’t discover one. And without a diagnosis, they couldn’t figure out how to treat my illness. They told me it was just a matter of time before my kidneys failed.”

In the absence of a transplant, kidney failure is usually treated by dialysis. With this treatment, patients no longer face imminent death. Prior to the emergence of dialysis, people with irreversible kidney failure usually died in a matter of weeks. On dialysis, many patients live for years. But they do not necessarily live in great health. The medical literature estimates that patients on dialysis face annual mortality rates close to 20%. Bucci desperately wanted to avoid that fate. “My doctor urged me to find a living donor, and get transplanted before I needed dialysis. A few even told me that since I am from Russia, I should go abroad and find someone willing to ‘donate’ a kidney.”

But Bucci was not comfortable receiving a kidney from a stranger—that struck her as both unethical and as medically dangerous: “Who knows what kind of organ you would get that way.” She also didn’t have any relatives who could serve as living donors. So she settled in for the long wait to receive a transplant from a deceased donor. At Georgetown University Medical Center, near where she lived, less than a quarter of their kidney transplant candidates receive a transplant in a typical year. Bucci knew that each one of those years she spent waiting for a transplant could be her last.

And then she read Walter Isaacson’s biography of Steve Jobs, and learned that he had flown to Tennessee to receive a liver transplant, and thought to herself: “Why don’t I do something like that!” She decided to get herself listed at as many transplant centers as possible, and travel to which ever one could find her a suitable organ.

Bucci began undergoing transplant evaluations at hospitals located relatively close to Washington D.C., but that operated in different transplant areas than Georgetown. In the process of undergoing those evaluations, she received some bad news that made it even more important for her to get listed at multiple centers – she had a PRA of 90. The PRA, or Panel Reactive Antibody test, estimates the percent of potential donor kidneys that a person’s immune system will reject. Probably as a result of her pregnancies, Bucci’s immune system was highly reactive; her body was choosy about what kind of foreign antigens it would tolerate. Whatever the reason for her high PRA, Bucci would be unable to accept 90% of eligible donors. As an IT expert with a Master’s degree in mathematics, it was not difficult for Bucci to figure out that her long wait for a kidney had just gotten longer.

That’s when Bucci began spending her free time getting herself onto as many transplant waitlists as possible. She researched hospitals in the Northeastern United States and made appointments at transplant centers with relatively short wait times. She stretched beyond the Northeast, even getting herself listed at two programs in Florida. And not surprisingly, she connected with Sridhar Tayur, the CEO of OrganJet, a company that helps patients receive distant transplants to reduce their waiting time for an organ. (See Part 1 for the origins of this company.) Tayur convinced her to add the University of Pittsburgh to her list of centers. She travelled to all those programs outside of work hours, because she did not want her employer to realize she had kidney failure. She was already noticing that her illness affected her concentration, and she did not want to give her boss a reason to relieve her of her job: “I didn’t want to hit them over the head with my illness,” she told me. “They weren’t going to learn about my kidney problems until I left work to get a transplant.”

From Seaway News, Cornwall, Ontario, By Adam Brazeau

Rachel's Kids' biggest 'Small Moments of Joy' donation yet

CORNWALL, Ontario - His battle with Polycystic kidney disease is no fairy tale story.

So before six-year-old George Jacobs and his family tour Disneyland thanks to a Cornwall-based charity, they're making a pit stop to meet his favourite silver screen star: Emmet from the Lego Movie.

For over two years, the youngster needed daily care at CHEO in Ottawa while on dialysis as he waited for a transplant. PKD, the inherited condition that affects kidney function, has no known cure.

In 2013, George’s long wait was over: a donor surfaced.

"He's been a lot better since then," said Mitchell.

During the long hospital visits he found strength in dreaming that one day he would meet the fictional character and visit the popular California attraction.

Enter Rachel's Kids' 'Small Moments of Joy' program.

The charity founded by Dr. Rachel Navaneelan has coordinated with an American foundation to help send the Akwesasne family of four to Disneyland, and several other large amusement parks along the way, including Universal Studios Hollywood, SeaWorld, Legoland Hotel, and Busch Gardens.

Tickets, travel expenses, food, and accommodations are all taken care of.

George (III) will be making the two-day trek with George (Junior) Jacobs (dad), Sabrina Mitchell (mom), and Kayden Jacobs, 11, (big sis'). They leave on Thursday (July 2) and plan to return on July 13.

“The kids are getting really excited,” said Mitchell. “I just want to say thanks for everything Rachel’s Kids has done.”

As a patient of Dr. Navaneelan's, she would discuss George's condition. When his immune system strengthened, allowing him to travel, the Rachel's Kids board of directors decided to "make his wish come true."

George's father noted that this is the family's most extravagant adventure to date.

Watching her brother fight back from having 20 per cent kidney function to his current state of a rambunctious boy has been “very emotional. [Read more]

From The Langley Times, British Columbia, Canada, by Gary Ahuja

Healthy McBeth readies for GranFondo

Roy McBeth (above, right) was the recipient of a new kidney from his boss, Kevin Wright (left). McBeth won the Prospera Valley GranFondo Fortius Changing Gears contest and has spent the past few months (photo below) preparing for the 160-km GranFondo ride. The fourth annual ride begins and ends in Fort Langley on July 19.

While cycling 160 kilometres in less than five hours is an above-average time, Roy McBeth has his mind set on bettering his 2014 mark.

Competing in the Prospera Valley GranFondo for the first time in 2014, McBeth finished the grueling ride in 4:53:38, which was good for 43rd in the men's 35 to 44-year-old age group.

It was also 43 minutes better than the average time (5:36.58) of all 502 participants for that distance.

"I was not feeling very good, but I had made it," McBeth said.

"I don't think I was slow by any means, but I would like to improve on that."

What makes his 2014 time even more impressive was the fact McBeth did so with a kidney functioning in the 15 to 17 per cent range, by his estimation.

McBeth, who is now 45, has cycled for the past 20 or so years, competing in mountain biking, road racing, cyclocross, triathlons and Xterra, which is off-road triathlon events.

But he always did so while struggling with a hereditary genetic condition called polycystic kidney disease (KPD).

It is a disorder that causes multiple cysts to form in the kidneys.

McBeth's grandmother died of the condition in her late 30s while his dad passed away in 2010 at the age of 63.

McBeth was diagnosed as a teenager.

"There is not a lot you can do for the disease," he explained.

"The best options are to live a healthy lifestyle, watch your diet and don't drink excess alcohol, that sort of stuff.

"I knew it was coming. It was more trying to get out in front of what the end game was from what I had seen from other family members and knew that I didn't want to go that route with the years of dialysis, kind of wasting away in that manner."

"I didn't start to notice any decline in my health and my physical performance and competing until my mid 30s; that's when the kidney decline really started to accelerate (and) by the time I was in my 40s, things were really started to become problematic," he added.

Things really began to go downhill about two years ago.

McBeth began to suffer high blood pressure, fatigue and lethargy. Cysts also began to grow on his kidneys, becoming so enlarged that it became harder to exercise because the cysts would push on his diaphragm, making it harder to take deep breaths. He also suffered nausea, headaches and just generally not feeling well.

"Things sort of snowballed and once things go bad, they pick up and get bad quite fast."

He was facing either dialysis — a process that removes waste and excess water from the blood and must be performed four or five hours every second day — or a transplant. [Read more]

From Journal Pioneer, Summerside, Canada, by Eric McCarthy

Recipient reflects on 30 years with sister’s kidney

Sisters, Yvonne Baglole, left and Tish Lidstone, enjoy a chat. This Saturday the pair mark a special milestone, Yvonne’s 30 years with the kidney Tish donated.

Yvonne Baglole was just a week into her three-times-a-week trips to Halifax for kidney dialysis when her doctor advised dialysis wasn’t going to work.

Her only hope was a kidney transplant.

Her brother and sister had already been tested, just in case, and her sister, Tish Lidstone, was identified as a perfect match.

But Baglole was reluctant. “I didn’t want her to jeopardize her own health for mine,” she reflects.

‘You don’t have any more choice. You either die or you get the transplant done,’ her husband, Jimmy recalls the advice from her kidney specialist.

“I knew I was going to do it for her and I never gave it a second thought,” Tish said of her on-the-spot decision to give Yvonne a kidney.

“She’s my sister. She’s my pride and joy.

I’m 11 years older than her and I looked after her.”

Tish was 61 and Yvonne was almost 50 when the transplant was performed on July 4, 1985. This Saturday they mark the 30-year milestone of that life-saving transplant.

Tish, now 91, said she’s never had any ill-health because of her decision.

As for Yvonne, “it’s made my life,” she said. Doctors could tell the kidney was functioning within five minutes of the transplant.

“That’s 30 years of life,” Tish acknowledges.

When the sisters were asked whether the kidney transplant created a special bond between them over the past 30 years, Tish responds, “Yeah, for the last 79 (years).”

“When you’re older like that and you get a baby coming into the household, it’s quite a prize,” Tish recalls. “We thought she was the most precious thing.”

“She’s been my protector for all my years,” Yvonne acknowledges.

Yvonne was born with Polycystic Kidney Disease, but only learned of it following the death of her brother Orville Betts. An autopsy determined he had PKD. Family members were subsequently tested. The other two members of her Betts family didn’t have the disease. Through time her kidneys wasted away to the point that dialysis or a transplant became necessary. [Read more]