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.