PKD Voice, To Paris, Walking, Cruis'n, Delay Progression

PKD Voice

From PKD Foundation

Bring Your Voice to Capitol Hill

The PKD Foundation partners with other organizations to participate in annual legislative and public policy conferences in Washington, D.C. After participating in advocacy training, we spend a day on Capitol Hill meeting with our members of Congress, to both raise awareness for PKD and discuss our legislative priorities for the year.

We invite you to visit our recently updated Advocacy Action Center where we have tools for you to advocate from home year-round. If you would like to receive emails about PKD advocacy efforts, please visit Advocacy Alerts to sign up. Thanks for your support!

Rare Disease Week
Feb. 29 - March 3

The PKD Foundation is joining Rare Disease Legislative Advocates (RDLA) to bring ARPKD families and rare disease community members to Washington, D.C. from across the country to learn about federal legislative issues, meet other advocates and share their unique stories with legislators.

The PKD Foundation will reimburse up to $600 for travel expenses including airfare and hotel for advocates attending the legislative conference and meetings on Capitol Hill. Please note: to receive reimbursement, you must contact us for registration information at pkdadvocacy@pkdcure.org.

For additional information about the event, visit Rare Disease Legislative Advocate's website.
Schedule of Events
Monday, Feb. 29, 7:30 a.m. – 3:30 p.m.: Rare Disease Day at the National Institutes of Health (NIH)
Monday, Feb. 29, 5:30 – 9:30 p.m.: Cocktail Reception and Rare Disease Documentary Screening featuring Dusty’s Trail: Summit of Borneo
Tuesday, March 1, 8:30 a.m. – 5 p.m.: Legislative Conference - Required for reimbursement
Wednesday, March 2, 7:30 - 9 a.m.: Lobby Day Breakfast
Wednesday, March 2, 9 a.m. – 5 p.m.: Scheduled Meetings with Members of the House and Senate - Required for reimbursement
Thursday, March 3, 12 – 1 p.m., Congressional Rare Disease Caucus Briefing
Thursday, March 3, 5 – 7 p.m.: Rare Artist Reception



PKD Fundraising

From PR.com

Polycystic Kidney Disease Fundraiser Aims to Top Record

The Hamilton Chapter of the PKD Foundation of Canada’s first annual dinner-dance benefiting Canadian research on polycystic kidney disease (PKD) raised more than $6,000 – the highest amount by any chapter’s debut fundraiser for the charity. This spring, the Hamilton Chapter will be stepping up again to try and top $7,000 at its HOPe FOR A CURE in March – National Kidney Month.

Hamilton, Canada, February 24, 2016 --(PR.com)-- As fundraisers go, this one has proven to be uniquely successful, thanks in large part to the lively music, which appeals to people of all ages.

“The plan is to hold an affordable family-friendly, fun night out; it’s easy to have a great time knowing you’re helping a good cause,” says Joy Pekar, Event Organizer.

The PKD Foundation of Canada is the only national organization exclusively devoted to providing support for polycystic kidney disease patients and raising funds for scientific study of the inherited disease that affects about 1 in 500 people worldwide.

An Italian themed dinner generously sponsored by Gordon Food Service, and swing era music performed by the Jumpin’ Jive Band will keep people hopping between 6pm and 11:30pm on March 5 at Germania Club, 863King Street East in Hamilton, Ontario.

“HOPe FOR A CURE 2016 promises to be bigger and better, with the amazing items that have been rolling in for the silent auction as well,” said Shiona Mackenzie-Morrison, Chapter Coordinator.

Tickets, $25 in advance or $30 at the door, are available online through Eventbrite, or by calling the PKD Foundation of Canada.

Contact

Hamilton Chapter, PKD Foundation of Canada
S Mackenzie-Morrison, Hamilton Chapter Coordinator
1-877-410-1741
Contact
www.endpkd.ca/index.asp
Jeff Robertson
endpkd@endpkd.ca

From Melton Times, United Kingdom

Kidney patient gears up for 388-mile charity cycle ride to Paris before life-changing transplant

A Wymeswold man due to receive a kidney from his mum this summer is planning to cycle all the way to Paris before that to raise funds for charity.

Dan Norcott and his two friends, Rich Moss and Andy Stafford, will be taking on their 388-mile cycle challenge from Wymeswold to Paris between May 4 and 15 to raise money for the Kidney Care Appeal, a joint venture between Leicester’s hospitals and the University of Leicester.

Dan (41) has polycystic kidney disease (PKD), a genetically transmitted illness where cysts develop in the kidneys and gradually increase in size, disrupting the function and almost inevitably leading to them failing.

Dan’s father, who died in 2004, also had the condition.

Dan said: “Since I was diagnosed at the age of 21, my kidney function has coasted gently downwards. I’ve just entered the last phase of the illness. My mum is going to give me a kidney and, sometime this summer, I’ll be ill enough to need it.

“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.

“I find it hurts to stand upright for more than a few minutes, but fortunately walking and cycling are largely free from pain, if a little slower than normal.”

Dan, who is married to 2012 Man Booker prize-shortlisted author Alison Moore, is planning to complete his epic bike ride in three sections over 10 days, covering about 45 miles a day. The first leg (132 miles) will take Dan and his friends from Wymeswold to London, the second leg onto Newhaven (107 miles) and the final leg from Dieppe to Paris (149 miles).

The trio set an initial target to raise £5,000 but hope to beat that. You can sponsor them by visiting https://www.justgiving.com/Pedalling-to-Paris-with-PKD/5

For more information about their challenge visit http://pedallingtoparis.com/

From Michigan City LaPorte, Indiana

Cruis'n For a Cure for PKD

Sunday, May 15, 2016
12:00PM - 2:30PM

Cruis'n For a Cure for PKD Car show and Car cruise.

Car Show and 40-mile car cruise, $10 per vehicle, all wheels welcome. Rain or Shine. Concessions, trophies, prizes, LaPorte County Fairgrounds

Walk for PKD

Saturday, September 17, 2016
9:00AM - 11:30AM

Walk for PKD.

Northern Indiana Walk for PKD – 2-Mile Walk Sat, Sept 17, 2016. Check in 9am; Walk 10am at Creek Ridge County Park – Michigan City, IN. For more information, please contact Walk Coordinator,northernindianawalk@pkdcure.org. Polycystic Kidney Disease (PKD) affects thousands of Americans and 12.5 million children and adults, worldwide. There is no treatment or cure, but there is hope. Walk, form a team, help with the committee, volunteer and more! Register online and take advantage of a host of effective and Free online team features like emailing members, tracking team progress and fundraising tools. Contact the PKD Foundation for more information on how you can help.www.pkdcure.org. Come join us.www.walkforpkd.org/northernindiana.

Gift of Life

From State Journal-Register, Springfield, IL, By Dave Bakke

Dave Bakke: Former high school sweetheart pays debt with kidney donation

Corey Having and Lindsey Leonhard dated for four years in high school and went to four proms together; two of his at A-C Central and two of hers at Virginia High School. But that is not the only reason Corey holds a special place in Lindsey's life. She went through a lot of stress in those days.

"When Corey and I met," says Lindsey, "I was having lots of family turmoil. I was 13 and had depression. He was my best friend. He was there when I needed somebody. I always hold myself personally in debt to him. I don't know what would have become of my life without him."

After high school, there came a time in their relationship when Lindsey was ready for the city while Corey preferred the small-town life. So they went their separate ways.
That was about 10 years ago. We come to present times and it is Corey who is desperately in need of someone.

"I have PKD," he says. "It's a kidney disease that starts with a cyst that grows until it shuts down your kidneys."

Polycystic kidney disease runs in Corey's family. His father died from complications related to the disease. The Mayo Clinic estimates that nearly half the people who have PKD suffer kidney failure before age 60.
Corey, 28, was first diagnosed after he broke his back at age 14. His kidney cysts have grown to the point where he requires dialysis every other day. Last year, his doctor told him he had to have a kidney transplant. He was put on the national list for people in need of donors.

"They say it's five years before you even get a shot at it," he says, "unless you have a live donor."
The word went out through social media. Corey has sisters, but they were ruled out as donors because of the family history of PKD. So it looked bleak until someone from the past stepped up — his old high school sweetheart, Lindsey.

She's 25 now and had stayed in touch with Corey and knew what was happening with his health. Last fall, Lindsey volunteered to be tested at Memorial Medical Center as a possible kidney donor. She was found to be a match for Corey, but he tried to talk her out of being a donor.

"I didn't even want her to be tested," he says. "There's always a fear that something will happen to the one kidney the donor has left, and I didn't want that for her. I even went to her mom and tried to get her to talk her out of it."

That was a non-starter. Lindsey's mother, Dennise Minor, not only didn't try to discourage Lindsey, Dennise has become her daughter's biggest supporter in this.

From Green Bay Press Gazette, by Samantha Hernandez, USA TODAY NETWORK-Wisconsin

Town of Union woman with no kidneys needs donor

A Union woman is looking for a kidney donor after a possible donor was unable to take part in the procedure.

Misty Scudder, 26, was diagnosed with polycystic kidney disease (PKD) when she was in eighth grade. Scudder explained that the disease is genetic and causes cysts to form in the kidneys. If the cysts burst it can cause more problems. Scudder’s birth father also has the disease.

“The more they rupture, the bigger they get,” Scudder said.

About four months after the birth of her son, Isaiah Jr., her condition began to rapidly deteriorate. Doctors found that two large blood clots in her brain had ruptured. She underwent an operation to stop the bleeding in November 2014. Afterward she was in the hospital for five weeks.

“After that they told me I was going to be in kidney failure because the dye from the surgery … my kidneys could not filter it because of the PKD,” she said.

She went from having stage III kidney disease to stage V.

She has been on dialysis since July 2015. At first she was having the procedure done three times a week and now does peritoneal dialysis in her home each night.

The hardest part of Scudder’s journey is she had someone ready to donate. Unfortunately, in December doctors found protein in the donor’s urine. The protein is an indicator of possible kidney problems.

While Scudder, who is O positive, can live on dialysis, she is hoping someone will step forward and volunteer to be a donor.

"I don’t feel like a normal person,” she said.

PKD Research

From American Journal of Physiology

Concomitant use of rapamycin and rosiglitazone delays the progression of polycystic kidney disease in Han:SPRD rats: A study of the mechanism of action

BACKGROUND AND PURPOSE: Attributing to their anti-proliferative effect, both rapamycin and PPARγ could halt the progression of ADPKD. Whether combined use can enhance effect is unknown. EXPERIMENTAL APPROACH: The present study used rapamycin and the PPARγ agonist rosiglitazone concomitantly to observe their effects on the proliferation of ADPKD cyst-lining epithelial cells and the progression of ADPKD in Han:SPRD rats. KEY RESULTS: Concomitant use of the two drugs inhibited the proliferation of WT9-12 cells significantly through a superimposition effect. Rosiglitazone inhibited the phosphorylation of mTOR target p70S6K. Concomitant use of rosiglitazone and rapamycin further down-regulated the p-p70S6K level. Rosiglitazone also inhibited the phosphorylation of Akt and antagonize the activation of Akt induced by rapamycin. Concomitant use of rosiglitazone and rapamycin significantly retarded the deterioration of renal function, decreased cyst cell proliferation and interstitial fibrosis in Han: SPRD rats. Rapamycin significantly increased cholesterol levels in the blood, whereas rosiglitazone mitigated rapamycin-induced hyperlipidemia. CONCLUSION AND IMPLICATIONS: These results indicate that the effects of concomitant use of rosiglitazone and rapamycin in inhibiting the proliferation of WT9-12 cells and delaying the progression of ADPKD in Han:SPRD rats are stronger than those of either drug alone. The present study may provide a new strategy for the long-term treatment of ADPKD.

Artifical Kidneys with Nanofilters to repalce dialysis; Broadway Sings for PKD, Finding a perfect match; It's Cricket

PKD Research

From MedGadget reprint from Vanderbilt University, watch video

Artificial Kidney Made of Nanofilters and Living Cells to Replace Dialysis

Image credit: Vanderbilt University

At Vanderbilt University scientists are building an artificial kidney that they envision will one day will be a standard of care over dialysis. The device consists of a silicon nanotechnology filter chip and embedded living kidney cells that would work together to mimic the functionality of a healthy kidney. The end result is expected to be about the size of a natural kidney, small enough to be implantable and powered by the body’s own blood flow.

The filter component has tiny pores that can be individually shaped to perform a specific task. These filters would sit in a series, each one performing a different filtration step. Between the filter slices there would be living kidney cells that perform tasks that the man made components are not very good at, including re-absorption of nutrients and getting rid of accumulated waste.

Dr. William Fissell is an Associate Professor of Medicine at Vanderbilt University. 

“There are only about 20,000 patients a year who receive a kidney transplant,” Fissell said.

He’s dedicated his life’s work to this project and he said it’s taken decades of research.

“In the 1980s is when I first met a dialysis patient when I was a paramedic in Boston,” Fissell said. “And the incredible courage and humanity of the patients that I cared for every day then, 30 years ago, and that I care for every day today drives this work forward."

Fissell said they use a microchip technology to dissect fluids inside the artificial kidney.

"These are probably things like the insides of your video camera, or the gyroscope in your iPhone. We've taken that technology and used it to make a miniature membrane that's small enough that it can fit inside a patient's body and efficient enough that it doesn't require big bulky pumps to circulate fluid around,” he added.

He said the challenge will be connecting the membrane to the patient’s blood vessels.

“We use the same engineering tools that, for example, people use to design jet liners,” Fissell said. “The computational fluid dynamics that allows you to predict exactly how much force of fluid will experience as it's moved from your aorta another artery through this artificial device."

PKD Fundraising

From Broadway World

Wesley Taylor, Jessica Phillips & More Set for BROADWAY SINGS FOR PKD at Laurie Beechman

The fifth annual Broadway Sings for PKD, a concert benefiting the Polycystic Kidney Disease Foundation, will be offered Thursday, February 25 at 7:00 pm at The Laurie BeechmanTheatre.

The concert will feature new musical theatre songs by emerging writers, including Scotty Arnold, Ben Bonnema, Christina Capatides, Seth Christenfeld, Amy Engelhardt, Benny Gammerman, Sean Havrilla, Doug Katsaros, Rachel Kunstadt, Jane Lee, Natalie Lovejoy, Brandon Michael Lowden, Rachel Peters, Paulo K. Tirol, and Amanda Yesnowitz

Performers will include Abby Burke, Hannah Corneau, Emma Davis, Angel Desai (Company), Sam Heldt, Jillian Louis (It Shoulda Been You), Jessica Phillips (Next to Normal, "Law & Order SVU"), Ariella Serur, and Wesley Taylor (The Addams Family, Rock of Ages). Ben Cameron hosts.

Broadway Sings for PKD is produced by Rachel Kunstadt with musical direction by Benjie Dia.

Proceeds from the evening will go to the Polycystic Kidney Disease Foundation, which "promotes programs of research, advocacy, education, support and awareness in order to discover treatments and a cure for Polycystic Kidney Disease and improve the lives of all it affects."

Tickets are $15, and there is a $20 food or drink minimum. Tickets are available at www.westbankcafe.com.

For more information on the Polycystic Kidney Disease Foundation, please visit www.pkdcure.org.



Living with PKD

From Stuff, Canterbury, New Zealand, by BRENDON EGAN

Canterbury cricket umpire Eugene Sanders wins national award

Christchurch's Eugene Sanders, second left, turned to cricket umpiring after a kidney disease and won national umpiring award. He is pictured talking with International umpires, from left, Richard Illingworth, Ranmore Martinesz and Richard Kettleborough.

A kidney disease forced Eugene Sanders to give up playing cricket seven years ago.

Rather than being lost to the sport, the Christchurch man turned his attention to umpiring.

Sanders' commitment and dedication has paid off, winning a national award, run in conjunction by New Zealand Cricket (NZC) and optical company, Specsavers.

In 2009, Sanders was playing in a suburban club game for Heathcote and realised something was wrong when he got home and experienced abdominal pain and discovered blood in his urine.

He visited the doctor and was diagnosed with polycystic kidney disease, which came as a major shock.

Sanders was told if he kept playing cricket he would be putting his life at risk.

"I'd been a bowler all my life. The motion going over your side, probably wouldn't be a good idea with the cysts," he said.

"You're prone to a lot of infections with the cysts bursting."

Sanders, 43, freely admits he is a more talented umpire than he was cricketer.

Switching to umpiring was an easy call.

"I thought why not get into the hot seat, the best seat in the house, and I decided to do that."

He was nominated for the national award, which recognises the unsung heroes of community cricket, by his brother, Charne Christensen, who wrote a supporting letter.

Sanders prevailed over five regional winners.

"It's very humbling. It's the last thing I expected. You don't expect these awards, especially when you just do it for the love of the game.

"It came as a very welcome surprise." [Read more]

NEJM Journal Watch

How Does Autosomal Recessive Polycystic Kidney Disease Progress to Kidney Failure?

Decline in glomerular filtration rate in children with ARPKD was comparable with declines in other pediatric chronic kidney diseases.

Autosomal recessive polycystic kidney disease (ARPKD) can cause death in the neonatal period; however, the majority of children with this condition now survive infancy. This improvement in survival necessitates a better understanding of the natural history of ARPKD to guide care of survivors.

In a prospective study, researchers compared glomerular filtration rate (GFR), blood pressure, left ventricular hypertrophy, and proteinuria in 22 children with ARPKD surviving infancy and two cohorts of children with other renal diseases, including 44 children with aplasia/hypoplasia/dysplasia and 44 with obstructive uropathy. All children were diagnosed during the neonatal period. Results were as follows:

Children with ARPKD had an annual decline in GFR of –1.4 mL/min/1.73m2 (representing a 6% decrement), which was similar to declines in the other groups.

In all groups, annual decline in renal function was greater with baseline GFR level <45 ml/min/1.73m2 versus ≥45.

Children with ARPKD aged ≥10 years had more than double the rate of annual GFR decline compared with those aged <10 years.

Hypertension and left ventricular hypertrophy rates were similar between all groups; however, antihypertensive therapy use was higher among patients with ARPKD.

Children with ARPKD had less proteinuria compared with the two comparison groups.

From Wellsville Reporter, Wellsville, NY, By Brian Quinn

Wellsville couple a perfect match

Kelly Case said she chose to donate a kidney to her husband, Jesse, not long after hearing that the two were a match. PHOTO PROVIDED

It took a long time to get to this point, Jesse and Kelly Case said recently, but the two of them were a match.
On one level, the two Wellsville residents have known that for a long time — they have been married since 1992. What they found out much more recently is that Kelly Case was a match for her husband when Jesse, who has suffered from polycystic kidney disease (PKD) needed a new kidney (for more information on PKD, see the accompanying box).

“It’s called PKD. I have the adult version,” said Jesse Case, 56, who works at Dresser-Rand in Wellsville. “Basically, cysts grow on your kidneys until they can’t function anymore. It’s something you’re born with, but the diagnosis of it was 16 years ago. I went into kidney failure slightly over a year ago, in December 2014. I’ve been on dialysis since then.”

The short version of this story is the couple found out last year that Kelly was a donor match, which led to the surgery that was performed five days ago at Strong Memorial Hospital in Rochester. The surgeries on Kelly and Jesse went well, Kelly’s sister, Tammy Christman, said Friday. Kelly came home Friday and Jesse was coming home Saturday, she said.

The longer version involved testing and visits to doctors in Hornell and Rochester and discussion between the husband and wife, the Cases said in the days before they made the trip to Rochester for Tuesday’s operations.

“The Dialysis Center in Hornell is who I do everything through. They made the referral up to Strong for us to get on the donor list,” Jesse Case said. “It can take several years to find a donor.”
Kelly recalled asking last May, when Jesse went for an evaluation, whether she could be tested to see if she was a match.

“We did know enough to know it goes by blood type and that’s how I got involved. They said living organ donation is the best outcome. I knew we were the same blood type (Type O) and they tested us and we were a match,” she said.

The Cases both went through a two-day testing period at some point.

“They did a lot of tests and you meet with the entire team and they fully explain the process to you, step by step — ‘This is what’s going to happen. This is what is expected. This is how things will work,’” he said.
The way doctors worked with her as a potential donor was very private, Kelly remembered.

Pre-emptive Kidney Transplant; Kidney Transplant Success Greater Prior to Dialysis, Tolvaptan European Recommendations

Living with PKD

From Science Codex

National underutilization of preemptive and early kidney transplants

ROCHESTER, Minn. -- A kidney transplant is a life-changing and life-saving procedure. Yet, a new study conducted by Mayo Clinic and the University of Michigan shows that only one-third of patients who ultimately receive a living donor kidney transplant receive it pre-emptively (i.e., before starting dialysis). Less than two-thirds receive a transplant either pre-emptively or within a year of starting dialysis.

Existing research suggests that less time spent on dialysis before transplant can improve patient outcomes and survival after transplant. However, this new research shows there has been no increase in the utilization of what is known as timely living donor kidney transplants, which includes pre-emptive and early transplants, since 2006. The study "Under-utilization of timely kidney transplants in those with living donors," was published recently in the American Journal of Transplantation.

"Early referral to transplant evaluation and access to information about living donor kidney transplantation is key to a successful timely transplant and to improved long-term outcomes," says Mark Stegall, M.D., a professor of surgery at Mayo Clinic and senior author of the manuscript. "One important area where people lack education is on how to communicate with family and friends about their need for a kidney transplant and the fact that live donors offer, on average, a better outcome than deceased donor transplantation."

"Past studies and patient data have shown that we can improve a person's quality of life and chance of surviving end-stage renal disease if we can avoid or minimize the amount of time they spend on dialysis," says Ankit Sakhuja, M.B.B.S., a graduate of Mayo Clinic Renal Transplant Fellowship. He also currently is an assistant professor and director of the kidney paired donation program in the Division of Nephrology at the University of Michigan.

Mayo Clinic and University of Michigan researchers examined data from the United Network for Organ Sharing to evaluate the use of timely kidney transplants from 2000 to 2012 for 68,128 patients who received living donor transplants. Although data showed an improvement in the use of pre-emptive and early living donor transplants between 2000 and 2006, there has been no improvement since.

"Patients who are receiving transplants from compatible living donors should, theoretically, not need to go on dialysis at all," says Dr. Sakhuja. "In comparison, patients with no living donors may wait for a deceased donor organ for a long time. But in reality, we see a wide variation in the timing of transplants and no improvement in the use of timely transplants from living donors, despite the potential availability and known benefits."

Factors that influence a person's chance of receiving a timely living kidney transplant are thought to include lack of available living donors, decreased number of living donors, lack of insurance, lack of education or knowledge, delayed diagnosis and delayed referral.

Paired donations and direct donation represent two viable and available options for timely living kidney transplant. Yet both can be influenced by early referral and by a patient's understanding of their situation and the transplant evaluation process. The use of timely kidney transplant can vary greatly from one transplant center to another, based on factors including the patient population, the transplant team and its comfort in evaluating living donors, and providing prompt transplant evaluations for patients. But according to Dr. Stegall, approximately 80 percent of kidney transplants at Mayo Clinic in the past 15 years have been from living donors, with 40 percent of those being pre-emptive living donor transplants.

From Home Town Station, Santa Clarita, CA, by: Chris McCrory

Santa Clarita Man In Need Of Kidney After Battle With Polycystic Kidney Disease

James Zimmerman and his oldest daughter, Jordyn, who also suffers from PKD.

A Santa Clarita man is in need of a kidney after three potential donors were turned away during the strict medical screening process.

The family of James Zimmerman has been impacted severely by polycystic kidney disease, or PKD, with no rescue in sight. His grandmother, mother and sister died of the disease while he, his uncle and his daughter are all suffering under PKD today.

“He has gone into stage four renal failure,” said Vicci Zimmerman, his wife of 25 years. “Our only hope is a living donor.”

An average adult kidney is about the size of a clenched fist, but Zimmerman’s kidneys are much larger, each one estimated to weigh about 22 pounds. This is because PKD produces large cysts on a kidney, filled with toxic fluid that makes it impossible to drain them or remove the excess.

“His kidneys are unable to process out the toxins from his blood,” Vicci Zimmerman said. “Water gives him heartburn at this point.”

James desperately needs a functioning kidney, but due to the strict screening process and his own O blood-type, no donor has been able to give him one.

Donating a kidney does not impact a donor’s life, said Vicci Zimmerman. And the costs are all taken care of by insurance.

She added that for most people, they can return to work the week after donating a kidney.

However, if no kidney is found for James Zimmerman in the next few months, he must undergo dialysis to continue cleaning his blood.

He is currently on schedule to start dialysis in the next few months, his wife said, sometime in April.

However, if Zimmerman does go on dialysis, his chances for a successful kidney transplant are reduced significantly.

“You have a much better chance of not rejecting a kidney without dialysis,” said Vicci Zimmerman. “But I only know to be positive.”

Those interested in seeing if they are able to donate can do so by email.

From Renal and Urology News

Polycystic Kidney Disease Raises Atrial Fibrillation Risk

Patients with polycystic kidney disease (PKD) are at elevated risk of new-onset atrial fibrillation (AF), according to researchers.

In a population-based cohort study using inpatient claims data from Taiwan's National Health Insurance Research Database (NHIRD), investigators found that PKD patients had a significant 31% increased risk of AF compared with individuals who did not have PKD, after adjusting for age, sex, and comorbidities. The risk was higher in patients aged 50 to 64 years and those without any comorbidities, “suggesting that the development of AF in patients with PKD is highly associated with the disease itself,” the investigators reported in Medicine (2016;95:e2623).

The study by Tung-Min Yu, MD, of the China Medical University in Taiwan, and colleagues compared 7,203 PKD patients with 28,739 randomly selected controls frequency matched according to age, sex, and baseline comorbidities. The risk of AF in PKD patients increased as the number of risk factors increased. Compared with patients with no risk factors, those with 1 risk factor had a significant 59% increased risk of AF. Patients with 3 risk factors had a significant 67% increased risk. Patients with 4 and 5 or more risk factors had a 2.2 and 3.6 times increased risk, respectively.

PKD patients with congestive heart failure (CHF) and chronic kidney disease (CKD) had the highest risk of AF, followed by PKD patients with hypertension and CHF, and PKD patients with hypertension, CHF, and CKD, according to the researchers. In addition, the odds of dying from AF was 69% higher in the PKD patients than in the comparison cohort. [Read more]

From Nursing in Practice, BY HELEN ROSE , JACQUELINE ANNAND

Managing chronic kidney disease in primary care

Key learning points:

– Chronic kidney disease is a common condition that is frequently encountered in primary care

– Primary care nurses are in a prime position to identify those at risk of chronic kidney disease and its complications

– It is often associated with otherlifestyle related long-term conditions and a significant opportunity exists to provide education and monitoring to prevent progression to end stage renal failure

Chronic kidney disease (CKD) is defined as an abnormality of kidney function or structure present for more than three months,1 and has been increasingly recognised as a significant global public health problem.2,3,4

Since estimated glomerular filtration rate (eGFR) recording was implemented on all urea and electrolyte blood samples in the UK in 2006, the scale of the problems has become clearer.5 Attention has been increasingly focused on early stage disease management in an attempt to slow or prevent progression to renal failure and modify associated cardiovascular risk.1,6

UK prevalence rates are estimated to be around 6-8%, and increase with age, diabetes mellitus and cardiovascular disease.3,4 Only around 4% of individuals with early CKD will progress to requiring dialysis treatment. However, it is estimated that a much larger proportion, around 46% will suffer mortality over a five year period due to related cardiovascular events.1

Causes

Underlying disease processes in CKD are various and wide ranging, the disease is often found alongside a number of other long-term conditions such as diabetes mellitus and cardiovascular disease, conditions that both have the potential to lead to renal damage.

There are a number of primary renal diseases and multisystem diseases, such as Immunoglobulin A (IgA) nephropathy, lupus and vasculitis that can cause inflammation and damage to the glomerular tissue in the kidney.

Underlying urological conditions can also lead to CKD obstructions caused by congenital malformations of the urinary tract, prostatic enlargement and renal calculi can all be indicated in the development of CKD. In addition, there are a number of hereditary conditions such as polycystic kidney disease, alports syndrome that can lead to CKD.7

Signs and symptoms

Table 1 outlines the stages of CKD and the symptoms and complications associated with each stage. It is important to remember that mild to moderate CKD often produces very little in the way of symptoms. It’s usually identified by the presence of protein in the urine or by elevated blood creatinine levels. Therefore it is important that those at increased risk of CKD development such as individuals with diabetes and hypertension are screened vigilantly for signs of CKD development. This includes monitoring of urea and electrolytes (Us and Es) for signs of creatinine rise or fall in eGFR, and urinalysis and urinary albumin creatinine ratio (ACR) for signs of microproteinuria development.1,8

In stage 4-5 the individual may start to complain of symptoms of nausea, extreme fatigue, loss of appetite, itching and shortness of breath. Oedema may be noted.

[Read more]

From Ripon Gazette, United Kingdom, by Dan Windham

Horrific kidney disease blights generations of Wetherby woman's family

For Boston Spa mother-of-two, Jayne Dryden, her entire life has been spent watching on helplessly as her loved-ones battle a horrific and hereditary kidney disease.

Polycystic kidney disease (PKD) has defined Jayne’s life, from watching her mother battle the inherited condition to finding out at just 21-years-old that she had also developed the disorder.

Now 52, Jayne spends three days of her week on dialysis, for four hours a time to treat the disease, but she is not alone in her struggles.

Five of her mother’s ten brothers and sisters have undergone kidney transplants and only one of Jayne’s three siblings has been fortunate enough to avoid inheriting the condition.

And, just like her mother did, Jayne will have her son and daughter succumb to the disease after tests revealed they had not managed to escape the powerful gene.

Despite the disease, Jayne is still managing to keep up her full-time job but admits she is finding it ‘harder and harder’ to live a normal life as her condition deteriorates.

She said: “My mum started dialysis when she was 51 but after seven years my dad was able to give her one of his kidneys. Unfortunately it only lasted for about seven years before it gave up.

“She’s back on dialysis now and in October I was forced to start as well. My kidney function was at 11 per cent, then down to nine but when it got to six my doctor told me it was time.

“The process is gruesome and there are points when you think ‘why is this happening to us?’. It’s very upsetting and it really has defined our family.”

However, Jayne explained that having the condition never made her doubt her decision to try for children but was fully aware the odds were stacked against them.

Polycystic Kidney Disease is the world’s most common inherited kidney disease which affected between one in 400 and one in 1000 people worldwide. [Read more]

PKD Treatment

From Renal and Urology News

Recommendations Issued for Tolvaptan Use in ADPKD

In May 2015, the European Medicines Agency approved the use of tolvaptan, a vasopressin V2 receptor antagonist, for slowing progression of cyst development and renal insufficiency in adult ADPKD patients with CKD stages 1–3 at initiation of treatment and evidence of rapidly progressing disease. This makes tolvaptan the first pharmaceutical agent approved for slowing disease progression in ADPDK. Tolvaptan was originally approved for the treatment of hyponatremia, and in the United States, it is only approved for that use. The FDA in 2014 asked for additional efficacy and safety data.

The recommendations are based on the consensus of ADPKD experts and methodologists in the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. In the consensus document, which was published online in Nephrology Dialysis Transplantation (NDT), the ERA-EDTA suggests that tolvaptan can be prescribed to adult ADPKD patients younger than 50 years with CKD stages 1–3a (an estimated glomerular filtration rate [eGFR] greater than 45 mL/min/1.73 m2) who have demonstrated or who are likely to have rapidly progressing disease.

ERA-EDTA recommends not starting tolvaptan in patients aged 30–40 years with CKD stage 1 (eGFR greater than 90 mL/min/1.73 m2) or patients aged 40–50 years with CKD stages 1 or 2 (eGFR greater than 60 mL/min/1.73 m2).

The organization recommends that rapid disease progression be defined as a confirmed annual eGFR decline of at least 5 mL/min/1.73 m2 in 1 year, and/or at least 2.5 mL/min/1.73 m2 per year over a period of 5 years. It can also be defined as a greater than 5% increase in total kidney volume per year by repeated measurements (preferably 3 or more, each at least 6 months apart and by magnetic resonance imaging).

With regard to dosing, ERA-EDTA suggests that tolvaptan be started with a dose of 45 mg in the morning and 15 mg in the evening, uptitrating the dose to 50/30 and 90/30 when tolerated, and discontinuing tolvaptan when patients approach end-stage renal disease.

PKD Clinical Trials Review; Statin Use: Tolvaptan Now Recommended in Scotland

PKD Treatrment

From Renal and Urology News, by Jody A. Charnow, Editor
Tolvaptan Lowers ADPKD Albuminuria

Tolvaptan decreases albuminuria over time in patients with autosomal dominant polycystic kidney disease (ADPKD), independent of blood pressure (BP), according to a post-hoc analysis of the TEMPO 3:4 Trial. The finding contrasts with the initial trial report, which concluded that tolvaptan had no effect on albuminuria.

The trial was a 3-year prospective, randomized, placebo-controlled study that included 1,375 ADPKD patients randomized to receive the V2 receptor antagonist tolvaptan or placebo. Researchers measured albuminuria in a spot morning urine sample prior to tolvaptan dosing and expressed as albumin-to-creatinine ratio (ACR). The investigators who conducted the post-hoc analysis noted that the initial TEMPO 3:4 Trial looked at categorical “albuminuria events,” which may have resulted in a loss of sensitivity to detect changes. The post-hoc analysis, led by Ron T. Gansevoort, MD, of University Medical Center Groningen in The Netherlands, examined the effect of tolvaptan on albuminuria as a continuous variable.

At baseline, the patients had a median ACR of 3.2 mg/mmol, and 47.9%, 48.7%, and 3.4% of patients had normal, moderately increased, and severely increased ACR, respectively, Dr. Gansevoort and his colleagues reported online in Nephrology Dialysis Transplantation. Patients with a higher baseline ACR had higher BP and total kidney volume (TKV) and lower estimated glomerular filtration rate (eGFR).

Over 3 years, ACR rose in the placebo arm and decreased in the tolvaptan arm (+23 vs. −0.40 mg/mmol). The difference in ACR increased over time, reaching a maximum of 24% at month 36. At that time, the researchers observed only a minor difference in blood pressure (BP). The decrease in ACR was similar in all subgroups and remained after withdrawal of tolvaptan. During follow-up, higher baseline ACR was associated with more rapid eGFR loss, but not TKV growth rate. Compared with placebo, tolvaptan was associated with a significant beneficial effect on TKV growth in all ACR subgroups, but was stronger in patients with a higher ACR, Dr. Gansevoort's team reported.

“In our opinion, the gradual onset of the ACR lowering response that remains after drug withdrawal indicates that the reduction in albuminuria with V2 receptor antagonists is a reflection of structural benefits that have obtained, such as less growth in TKV during tolvaptan treatment,” the authors wrote.

ADPKD is a relatively low albuminuria disease, so the effect of tolvaptan on albuminuria per se is of limited clinical relevance, the authors pointed out. The new findings are particularly important because they indicate that, in ADPKD patients, tolvaptan induces structural renal benefits and assessing albuminuria status in this disease may help to identify ADPKD patients who may benefit more from tolvaptan treatment, according to the researchers.

From Charity Today, United Kingdom

Tolvaptan now recommended for the treatment of ADPKD

TOLVAPTAN (marketed as Jinarc) has been approved for use in adults with autosomal dominant polycystic kidney disease (ADPKD) by the Scottish Medicines Consortium (SMC). This follows the National Institute for Care and Clinical Excellence’s (NICE) similar decision in October 2015 for patients in England and Wales. Northern Ireland has since adopted NICE’s recommendation, meaning patients across the UK are now eligible to receive the drug if they meet criteria outlined by their associated body. There was previously no licensed drug available to treat ADPKD in the UK.

Tolvaptan slows the progression of cyst development and helps protect renal function. In most cases this is likely to significantly delay the need for dialysis or a kidney transplant for ADPKD patients.

Kidney Research UK participated in the tolvaptan consultation processes with both regulatory bodies and ensured a rounded set of evidence was on offer by sharing the real life experiences of patients affected by ADPKD. [Read more]



PKD Research

From Med Gadget

Polycystic Kidney Disease – Pipeline Review, Market Growth, Segment, Trends and Forecast 2016 – 2020

“Polycystic Kidney Disease Global 2015 Clinical Trials Review, H2” provides an detailed overview of Polycystic Kidney Disease scenario. Report includes top line data relating on Polycystic Kidney Disease Global clinical trials scenario. This report on Polycystic Kidney Disease also includes an review of trial numbers as well as their ( Polycystic Kidney Disease ) average enrollment in uppermost/top countries which are conducted worldwide. Polycystic Kidney Disease report also covers disease clinical trials by country (G7 & E7), sponsor type, region, trial status as well as end points status. Report Polycystic Kidney Disease also includes prominent drugs for in-progress trials (Note: based on number of ongoing trials).

Request for report sample of Polycystic Kidney Disease Market, here: http://www.marketresearchstore.com/report/polycystic-kidney-disease-pipeline-review-h2-2015-46109#RequestSample

Scope of Polycystic Kidney Disease Report:
This report includes a snapshot of worldwide clinical trials landscape on Polycystic Kidney Disease scenario.

Report on Polycystic Kidney Disease also provides top level data related to the Global clinical trials by country (G7 & E7), sponsor type, region, trial status as well as end points status on Polycystic Kidney Disease scenario

Report reviews top companies involved in Polycystic Kidney Disease as well as provides enlists all trials (Trial title, Phase, and Status) pertaining to the company on Polycystic Kidney Disease scenario. [Read more]

From Renal and Urology News

Statins Reduce Proteinuria, Mortality in CKD Patients

Statins improve survival and other outcomes in patients with chronic kidney disease (CKD), but they do not appear to slow progression to end-stage renal disease (ESRD), a new meta-analysis finds.

Since previous research yielded inconsistent results, Zhenhong Zhang, MD, Pinsheng Wu, MD, and colleagues at Southern Medical University in China, pooled data from 23 randomized controlled trials (1995–2014) including 39,419 participants for an updated meta-analysis. The causes of CKD included diabetes,hypertension, glomerulonephritis, autosomal dominant polycystic kidney disease, idiopathic membranous nephropathy, and metabolic syndrome. Eight types of statins were examined.

According to results published in Pharmacological Research, statins improved proteinuria, reducing urinary protein excretion to 682.68 mg daily. Statin treatment also reduced all-cause mortality by 22%.

“Statins unquestionably reduce the risk of death in populations with, or at low risk of cardiovascular disease,” the researchers stated. “Our study further indicated that statins had the same effect on non-end stage CKD.” Statins lower lipid levels, protect the vascular endothelium, stabilize plaques, and suppress inflammation.

PKD Fundraising

From Sudbury Star, Canada, By Ben Leeson, Sudbury Star

VIDEO: Campaign featuring local boy raises nearly $49M

John Lappa/Sudbury Star Taylum Lamoureux and his mom, Desiree, play at their home in Greater Sudbury on Oct. 14 as a film crew captured a day in the boy's life. Taylum, a patient of Sick Kids Hospital in Toronto, was born with a rare kidney disease and underwent a kidney transplant in May.

SickKids' Foundation's holiday campaign featuring Taylum Lamoureux, the local toddler who received a kidney transplant last year after being born with polycystic kidney disease, raised nearly $49 million during November and December.

SickKids – Unpause contributed the best holiday campaign in the foundation's history, the foundation said, with a six per pent increase over the previous year. [Read more]