Sunday, August 28, 2016

PKD and Cancer Risks, Iran: Unique Kidney Donor System, PKD Fundraising: Saddlebrook, Pembroke, Maidenhead

PKD Cancer Risks

From New England Journal of Medicine, By Amy Orciari Herman

Polycystic Kidney Disease Tied to Increased Risk for Kidney, Gastric Cancers

Adults with polycystic kidneys may have increased risk for multiple cancers, regardless of kidney function, suggests a Lancet Oncology study.

Using a Taiwanese health insurance database, researchers matched over 4300 adults with polycystic kidney disease to 4300 controls without it. Adults with histories of cancer, chronic kidney disease, or end-stage renal disease were excluded.

During roughly 4–5 years' follow-up, the incidence of any cancer was 20 per 1000 person-years among those with polycystic kidneys versus 11 per 1000 among controls. After full multivariable adjustment, risks were significantly increased for cancers of the kidneys (hazard ratio, 2.5), colon (HR, 1.6), and liver (HR, 1.5).

Commentators say that “knowledge of the potential that there might be an excess cancer risk in early polycystic kidney disease should be widely known.” However, “there is insufficient evidence ... to embark on kidney, liver, and bowel screening programmes for patients with polycystic kidney disease, other than those already used in the general population.” [Read more]



Kidney Donation Debate

From MedicalXpress

In Iran, unique system allows payments for kidney donors

In Iran, unique system allows payments for kidney donors

In this July 2, 2016 photo, doctors perform a kidney transplant at Modarres Hospital in Tehran, Iran. In Iran, a unique system allows those in need of a transplant to buy a kidney. The program, which has seen Iran's waitlist for kidneys …more


The whirling hum of a dialysis machine could have been the soundtrack to the rest of Zahra Hajikarimi's life but for an unusual program in Iran that allows people to buy a kidney from a living donor.

Iran's kidney program stands apart from other organ donation systems around the world by openly allowing payments, typically of several thousand dollars. It has helped effectively eliminate the country's kidney transplant waiting list since 1999, the government says, in contrast to Western nations like the United States, where tens of thousands hope for an organ and thousands die waiting each year.

Critics warn the system can prey on the poor in Iran's long-sanctioned economy, with ads promising cash for kidneys. The World Health Organization and other groups oppose "commercializing" organ transplants. Some argue such a paid system in the U.S. or elsewhere could put those who cannot afford to pay at a disadvantage in securing a kidney if they need one.

But as black-market organ sales continue in countries like India, the Philippines and Pakistan and many die each year waiting for kidneys, some doctors and other experts have urged America and other nations to consider adopting aspects of Iran's system to save lives.

"Some donors have financial motivations. We can't say they don't. If (those donors) didn't have financial motives, they wouldn't ... donate a kidney," Hashem Ghasemi, the head of the patient-run Dialysis and Transplant Patients Association of Iran, told The Associated Press. "And some people just have charitable motivations."

The AP gained rare access to Iran's program, visiting patients on dialysis waiting for an organ, speaking to a man preparing to sell one of his kidneys and watching surgeons in Tehran perform a transplant. All of those interviewed stressed the altruistic nature of the program—even as graffiti scrawled on walls and trees near hospitals in Iran's capital advertised people offering to sell a kidney for cash.

As far as organ donations go, kidneys are unique. While people are born with two, most can live a full, healthy life with just one filtering waste from their blood. And although a donor and recipient must have a compatible blood type, transplants from unrelated donors are as successful as those from a close relative. In addition, kidneys from a living donor have a significantly better long-term survival rate than those from a deceased donor.

Iran started kidney transplants in 1967 but surgeries slowed after the 1979 Islamic Revolution and the storming of the U.S. Embassy in Tehran, in part due to sanctions. Iran allowed patients to travel abroad through much of the 1980s for transplants—including to America. But high costs, an ever-growing waiting list of patients and Iran's grinding eight-year war with Iraq forced the country to abandon the travel-abroad program.

In 1988, Iran created the program it has today. A person needing a kidney is referred to the Dialysis and Transplant Patients Association, which matches those needing a kidney with a potential healthy adult donor. The government pays for the surgeries, while the donor gets health coverage for at least a year and reduced rates on health insurance for years after that from government hospitals.

Those who broker the connection receive no payment. They help negotiate whatever financial compensation the donor receives, usually the equivalent of $4,500. They also help determine when Iranian charities or wealthy individuals cover the costs for those who cannot afford to pay for a kidney.

Today, more than 1,480 people receive a kidney transplant from a living donor in Iran each year, about 55 percent of the total of 2,700 transplants annually, according to government figures. Some 25,000 people undergo dialysis each year, but most don't seek transplants because they suffer other major health problems or are too old.

Some 8 to 10 percent of those who do apply are rejected due to poor health and other concerns. The average survival rate of those receiving a new kidney is between seven to 10 years, though some live longer, according to Iranian reports.

In the United States, about a third of kidney donations come from living donors. The average kidney from a deceased donor lasts 10 years, while one from a living donor averages about 15 years, according to Dr. David Klassen of the United Network for Organ Sharing, or UNOS, which oversees the U.S. transplant system. Recipients of living-donor kidneys in the U.S. fare better in part because they haven't been on dialysis as long before their transplant. [Read more]




PKD Fundraising

From Saddlebrook- Elmwood Park Daily Voice, NJ, by Anthony Locicero

Grandmother's Struggle Inspires Saddle Brook Man To Raise Money, Awareness

TJ Sullivan raised $2,000 for the PKD Foundation and Theresa LaBarck Donation Fund last year.

TJ Sullivan of Saddle Brook watched his grandma deal with her illness and decided to conduct some research.

Twenty-five years ago, Theresa LaBarck – or "mom-mom" as Sullivan calls her – was diagnosed with Polycystic Kidney Disease

"PKD is one of the world's most common inherited disorders in which clusters of cysts develop primarily on or around the kidneys," Sullivan said. "This can lead to other medical issues, such as aneurysms, high blood pressure and kidney failure. Mom-mom is one of the strongest women I know, and continuously battles PKD with a determination to overcome it."

Sullivan started researching the disease two years ago and had a fundraiser in Ridgefield Park to benefit both the PKD Foundation and Theresa LaBarck Donation Fund .

"Giving back means the world to me," said Sullivan, who donated $1,000 to both organizations. "Feeling like you are making a difference with whatever charity you are passionate about creates a lot of positive energy inside. I've been so incredibly passionate about the PKD Foundation because its mission to find treatments and/or a cure for this incurable disease hits so close to home."

Sullivan is planning a second even, " Winter Wonderland , " from 5-10 p.m. Dec. 10 at Ridgefield Park's Knights of Columbus.

"The goal is to raise even more awareness for Polycystic Kidney Disease and the PKD Foundation," said Sullivan, who works part-time at his family's Ridgefield Park deli. "Money isn't always everything; sometimes word of mouth does more good. As I get deeper into my fundraising efforts, I meet even more incredible people that are somehow affected by PKD."

Ridgefield Park Mayor George Fosdick will serve as one of the keynote speakers.

Joe Kenney, Marty Rotella and the Sandy Stones Trio will perform.

A spaghetti and meatball dinner will be served.

Sullivan, a 2014 Saddle Brook High School graduate, previously volunteered at St. Jude Children's Research Hospital events and raised money for St. Philip The Apostle Church .



From The Daily Observer, Pembroke, Canada

Fighting kidney disease one step at a time

Heather Sutherland, left, helps lead the 2015 edition of the annual Kidney Walk in Pembroke out of the marina towards Pembroke's downtown core.


The Kidney Foundation of Canada is urging Ontarians to lace up their sneakers and help move research forward for Canadians living with kidney disease.

The annual Kidney Walk event brings thousands of participants out in support of people living with kidney disease and their families. Nearly 10,000 Ontarians depend on life-sustaining dialysis treatments several times a week, over 1,000 are waiting for a kidney transplant, and countless others live with reduced kidney function that may have gone undetected. Across the country, millions are at risk of developing kidney disease.

Forty communities across Ontario will hold walks this fall in support of the 1 in 10 Canadians with kidney disease and The Kidney Foundation of Canada. One of those walks takes place here in Pembroke. For 2016, the walk is scheduled to take place on Sunday, Sept. 17 based out of the Pembroke marina. Registration will take place at 10 a.m. with the walk actually starting at 11 a.m.

The Sutherland family of Pembroke (Don, Cathy, Colleen, Meghan and Heather), has been hosting fundraisers for the Kidney Foundation since Don was diagnosed with polycystic kidney disease and received a transplant after a live donation from his wife, Cathy, in 2008. In addition to organizing the annual walk, the family also hosts an early summer ball tournament which is traditionally the first event of the year in support of the Pembroke Kidney Walk.

Every step you take on your Kidney Walk journey will provide hope for those living with kidney disease when they need it most. A diagnosis of kidney disease is life changing and impacts the entire family. For some, it means losing hours of the week to dialysis, impacting their opportunities to work, spend time together and travel.

That's why teams of friends and families will join together to walk to support their loved ones.

Create a team and join together at a Kidney Walk near you to help make a difference. It only requires a little time and effort, but each step will provide hope to many. Registration is easy online at www.kidneywalk.ca.

The Kidney Foundation of Canada works to improve the lives of all people affected by kidney disease through research, government advocacy, peer support, education and programs.

Join the kidney community at Kidney Walk and help create a future without kidney disease.

For more information, please visit www.kidneywalk.ca or call Bruce Hill at 613-724-9953 / 1-800-724-9953 ext.4560 email at bhill@kidney.on.ca.



From Maidenhead Advertiser, England, by Grace Witherden

Lido swimmer takes on mammoth challenge for charity

Lido swimmer takes on mammoth challenge for charity


A 36-year-old who took on a mammoth swimming challenge has managed to raise more than £2,300 for the Polycystic Kidney Disease charity.

Jenny Max, who grew up in Holyport, decided to swim in ten lidos across London in one day to raise awareness for polycystic kidney disease (PKD).

She swam in all ten lidos on Saturday, July 23 and is now planning her next challenge.

The cause is close to her heart as Jenny, along with her mother, brother and sister all suffer from the genetically inherited disease.

The disease causes cysts to grow in the kidneys which reduce their function and can lead to kidney failure.

The former Winbury School pupil said: “Outdoor swimming is my passion and I wanted to do something for a good cause.

“It’s amazing how much has been raised. I had a friend who transported me around London on a motorbike all day and ended up joining in as well.”

Visit www.justgiving.com/fundraising/jenny-max to donate.

Sunday, August 21, 2016

PKD Symptoms & Causes, "You Get Back Up', Gift of Life Chain: Pittsburgh, PKD Care Crowd Funding

Gift of Life

From WPXI, Pittsburgh, PA

Pittsburgh man's decision to donate kidney helps save 6 lives

Two strangers are forever linked after a decision to donate a kidney ended up saving six lives.

Leanne Ermert was close to being on daily dialysis in December as polycystic kidney disease left her in need of a new kidney.

Channel 11 News has followed Ermert’s story, which caught the attention of Willie Rhodes.

“I thought about it for a little bit and I just said, ‘What the heck, I've done nothing special in my life. I've got no kids. Maybe this is why God's been waking me up in the mornings,’” Rhodes said.

He decided to reach out to Ermert, and the two immediately bonded.

“She is from South Park, so hey, it's one Pittsburgher helping out another,” Rhodes said.

But Rhodes’ kidney was a match for more than just Ermert.

“My kidney went to a man in Wisconsin, 40 years old, who they said had an 84 percent chance of not getting a match,” he said.

Rhodes’ donation set off a six-person chain of kidney donations that ended with one from California going to Ermert. Confidentiality rules prevent them from knowing who the other people are, but they do know that so far everyone is healthy and healing -- including Ermert.

“I got what I wanted when I went to go see her on Friday. She was, there was so much light in her face,” Rhodes said.


Acquired cystic kidney disease is a form of kidney disease that develops in later stages of chronic kidney disease and most commonly after a patient has started to undergo dialysis to clean the blood.

Symptoms

Acquired cystic kidney disease differs from other forms of kidney diseases with cyst development in that it is fairly asymptomatic. The patient rarely feels much from the presence of the cysts in their kidneys. The kidney tends to remain the same size rather than expand due to the fluid filled sacs. Thus unlike polycystic kidney disease where the gradually expanding kidneys can be uncomfortable for the patient, an individual with the acquired cystic kidney disease can be oblivious to the structures developing inside them.

The presence of the cysts may become apparent should a cyst burst. This can cause the patient to feel pain or possibly develop a fever. Further investigation would then potentially reveal the cause. Signs of the disease can also include blood in the urine, a lump in the stomach, reduced appetite, pain at the side of the body, anemia and weight loss.

Another potential complication is that a cyst may become cancerous and cause renal cell carcinoma. This occurs in about 7% of cases, and the cancer is located in the tubules of the kidney. These are the structures that help to filter the blood from the kidney and create the urine that we excrete from our bodies.

The longer a person has dialysis, the higher the risk is of them developing renal cell cancer as a result of acquired cystic kidney disease as this disease becomes more likely with the length of time that a person experiences end stage renal disease.

Causes

The initial kidney failure, also known as end stage renal disease, that triggers the development of acquired cystic kidney disease can be caused by a number of different triggers such as diabetes, lupus, heart problems and urinary tract conditions. These diseases can result in permanent damage to the kidneys. Genetic diseases can also be implicated in the deterioration of the kidneys; however, acquired kidney disease is not by itself a hereditary disease.

Many researchers hypothesize that nephron loss of any etiology results in compensatory tubular cell hyperplasia in the intact nephrons. This response usually starts by activation of proto-oncogenes and release of various growth factors, which in turn accumulate in patients with chronic kidney insufficiency. Over a prolonged period of time, these factors then lead to tubular hyperplasia and subsequent cyst formation.

Regardless of such hypotheses, the causes of the cysts in acquired cystic kidney disease are not yet fully understood. The cysts only develop in the kidneys and nowhere else in the patient so are considered to occur as a result of activity in this area but this has not been proved. It has been suggested that they are a result of the build-up of waste products that the kidneys, with male hormones also playing a role. The lack of knowledge in the field means that the disease cannot currently be cured.




From Kilgore News Herald, Kilgore TX, by James Drapper

'You get back up'

In renal failure due to polycystic kidney disease, Edward Jones Financial Advisor Wilbur Yates is on the lookout for a donor after his intended match fell-through suddenly. NEWS HERALD photo by JAMES DRAPER

In renal failure due to polycystic kidney disease, Edward Jones Financial Advisor Wilbur Yates is on the lookout for a donor after his intended match fell-through suddenly. NEWS HERALD photo by JAMES DRAPER

Coke’s one of the things Wilbur Yates misses, “that carbonated water taste,” but there’s another absence he’s feeling more keenly at the moment – a good night’s sleep. He’s counting sheep as he counts the hours and days ahead until a new kidney donor is matched.

The Glomerular Filstration Rate measures how effectively the kidneys are working: 20 is a milestone on a downward trend, 15 marks renal failure and Yates is hovering around a GFR of 6. He’s on dialysis nine long hours each night, and what he thought would be a temporary setup has become, at the moment, an indefinite trial.

Poised to receive a kidney from a ready, willing and able donor, a little more than a week ago Yates learned the bad news: there were complications. Though a direct match, an eleventh hour update disqualified the donor.

On paper, the procedure was scheduled for Aug. 31. That date’s gone now.

“It was hard. It was fairly devastating,” Yates said. “When you’ve got two weeks and you’ve got the operating room reserved, that was hard, that was not what we were expecting. We were surprised.”

After a year of paperwork and preparation, the Edward Jones financial advisor made the Baylor Transplant Services list in January. He was added to East Texas Medical Center’s transplant program a few months later.

Awaiting a match, “I got pretty rundown. Really rundown,” he said Wednesday. “Actually to the point both doctors said I would need to go on dialysis, ‘to get cleaned up.’”

Yates opted for peritoneal dialysis.

“That allows him to still be able to come in and work,” his executive assistant, Donna Beets, explained. “Regular dialysis would interfere with work … very cumbersome, and it’s very time consuming.”

Though a bother at night, peritoneal dialysis is an easier procedure Yates said, slower and less stressful on the body.

“My whole purpose is, I’m going to continue to work,” he insisted. “I enjoy working and being with and enjoying my clients. [Read more]




From YouCare

We must help Darell Drew's symtoms of PKD and dialyisis!

My good friend of 42 years! was diagnosed with PKD in 1985. PKD,Polycystic Kidney Disease, is an incurable genetic disorder that is characterized by a life of cysts plaguing the kidneys and disrupting their normal functions. He has been bravely battling the disease for more than 30 years, 10 of which he has relied on kidney dialysis to perform the functions of normal, healthy kidneys. Darrell was placed on the kidney donor list back in 2006 and is anxiously awaiting good news. Please help make Darrell's journal as comfortable as possible by donating towards our goal of $6,000. to provide him with necessary vitamins, minerals and medicine needed to make him feel better and stay healthy enough to stay on the donor list.




Sunday, August 14, 2016

Brothers Living with PKD: Indy; PKD Gift of Life: Longview, WA, La Crosse, WI; PKD Fundraiser

Living with PKD

From WRTV, ABC Affiliate Channel 6, Indianapolis, IN, by Amanda Starrantino


Two Whitestown brothers are taking a special journey together – sharing a passion for racecars, and also a rare disease with no cure.

Isaac and Eli Fox were born just three years apart, and they both share the need for speed. But every day off the track, the brothers have a different race to worry about.

"We have ARPKD," the brothers explained. "My kidneys have cysts on them. My liver is shutting down, or eventually will, and so is my spleen. They are both enlarged."

The boys have autosomal recessive polycystic kidney disease – a rare genetic disorder found in one-in-20,000 children. The Fox family has two of the 12 children in Indiana with the disease.

For now, with no cure, the Fox brothers go day-by-day with the knowledge that their clock is ticking faster than most.

"The way we let them live their lives with racing and being in athletics as much as they are able to," said the boys' father Jamie. "Because we always know that the day is coming that they will have to have transplants, that they are not necessarily going to take a turn for the worst, but it will get much harder before they get better."

The brothers are both on the two-year track for transplants for their kidneys and livers. Their spleens will have to be removed too.

This weekend, the Fox family will be participating in the Indianapolis Walk for PKD. Their team has so fair raised nearly $1,000.

Click here to learn more about the Indianapolis Walk for PKD , orhere to learn more about the disease itself.



Gift of Life

From The Daily News, Longview, WA

Cowlitz 2 firefighter to donate kidney to local woman

A Cowlitz 2 firefighter has found another way to serve his community.

Brad Yoder, a Cowlitz 2 Fire & Rescue Firefighter/EMT, will donate his right kidney to Dana Clayton, a local woman who is lifelong friends with Yoder’s wife, Laura, according to a Cowlitz 2 press release.

The surgery will take place Monday at Legacy Good Samaritan Hospital in Portland.

Clayton suffers from Polycystic kidney disease, a genetic disorder characterized by the growth of numerous cysts in the kidneys. Yoder’s kidney is an exact match.

According to the press release, PKD cysts can greatly enlarge the kidneys and replace their normal structure, which can result in kidney disease. Kidney function declines over time and can lead to kidney failure. Clayton is in the end stages of the disease, according to the press release.

“Most candidates go through a four to six-month testing process before finding a match,” Yoder said, according to the press release. “Ours took only six weeks; it was just meant to be. For me, this is the ultimate in servanthood. I’m donating so that she can live, period.”

In order for Yoder to take enough time off from work to undergo and recover from the operation, multiple employees donated sick time to him.

“My brothers and sisters from Local 3828 and all the employees at Cowlitz 2 have been super supportive,” he said. “This brotherhood and department breathes and selflessly displays servanthood. ... I am blessed to be a part of this organization.”

Yoder and his two brothers grew up in Kalona, Iowa. After high school, Yoder enlisted in the U.S. Marine Corps and achieved the rank of Lance Corporal. He later moved to Washington and began riding bulls in rodeo circuits. In 2001 he signed up to serve as a volunteer firefighter for Cowlitz 2 Fire & Rescue and fought wildland fires working for the U.S. Forest Service.

Cowlitz 2 hired Yoder full time in 2007. In addition to responding to emergencies, Yoder has instructed the agency’s high school cadet program for 10 years. He and his students also created a memorial, featuring an actual piece of steel from the World Trade Center, honoring those lost in terrorist attacks on Sept. 11, 2001.




From WKBT-TV Channel 8, La Crosse, WI, by Brittany Schmidt


Every 10 minutes someone is added to the national transplant waiting list, according to the Organ Procurement and Transplantation Network.

But as the number of people in need of a lifesaving organ transplant continues to grow, the number of donors does not. However, one local woman is doing her part to help by becoming a living donor.

"I am going to do this, as long as I am healthy. I am going to give my kidney,” said 27-year-old Savannah Allds.

Savannah said it's been a goal of hers all her life, and her mother can attest to that ambition.

“She is pretty determined -- stubborn sometimes-- and she certainly is set in her beliefs,” said Cynthia Allds, Savannah’s mother.

“I have known for a very long time I wanted to be a donor, but didn't get very serious with it until my 20s when my mom’s health declined,” said Savannah.

“My kidney function right now is 18 to 19 percent,” said Cynthia.

In her early 20s, Cynthia was diagnosed with polycystic kidney disease, which is a disease where cysts form on the kidneys and destroy their function.

At first, Cynthia didn't really worry about it, but over the past several months the disease has started to take its toll.

“You may look at me and say you look healthy, but it's really how you feel. With that low of a function, it's very tiring. I can get up and two hours later I am exhausted,” said Cynthia.

“I saw what happens to the body and the progression of the disease throughout the years,” said Savannah.

“This year I was told I have been teetering on preparing for dialysis. When I was told that, I started crying because reality is here,” said Cynthia.

Cynthia needs a kidney transplant. Doctors typically look at family members first because they are more likely to be a match, but polycystic kidney disease is hereditary, which means Savannah is at risk of having it too. However, Savannah isn't going to stop her from trying to donate her kidney to her mom.

“I fought with my mom’s doctors. They didn't want me to do it until I am 30 but my mom doesn’t' have until then,” said Savannah.

“She literally sat there and argued with him that she wanted genetic testing,” said Cynthia.

After countless tests, scans and months of waiting, Savannah was cleared to donate her kidney to her mother. On June 24, Savannah and her mother checked into Mayo Clinic Health System in Rochester, Minnesota.

“If I can give my mom 5 to 10 years with a healthy kidney, I would be grateful for that,” said Savannah.

“Words can't even explain it, really. It's just kind of like an odd sense of my daughter is giving me a gift,” said Cynthia. “I gave her life and she is giving me life back basically and it's just, I am glowing inside but I don't have words to describe it."

The transplant was successful and after about eight weeks of taking it easy Savannah will be back to normal, but her mother's life will be changed forever and she has her daughter to thank for that.

“I love you (Savannah),” said Cynthia.

Because Savannah was being tested to give her kidney to her mother, throughout the entire process she had her own team -- a team that knew nothing about her mother's situation so that they could keep Savannah's best interest in the forefront when making the final decision to allow her to donate her kidney.

If Savannah had not been able to give her kidney, Cynthia said she had a friend who was willing to donate a kidney to her as well so she said she has been very lucky.

Allds is one of about 7,700 people who have donated an organ this year so far. Her hope is that by sharing her story, others will consider being a living donor or sign up to be a donor after death. You can find more information atdonorregistry.wisconsin.gov.




PKD Fundraising

From Big Sky Business Journal, San Diego, CA

PKD Foundation Golf Fundraiser

The annual Callan Taylor Fund for Kidney Disease Charity Golf Tournament will hold its fifth event in Billings this year on Sunday, September 11 at Pryor Creek Golf Course. Previous tournaments have been held in San Diego, CA, where the fundraiser was first launched by the family of the little boy, who is its namesake. 

Since Callan Taylor had family members in both San Diego and Billings, and since many people in Billings have been involved and have supported the fundraiser, organizers decided to hold it in Billings this year. Funds raised benefit the PKD Foundation. Callan died due to Polsycistic Kidney Disease.

Sponsors so far include Callaway Golf, Aero Club of San Diego, Brazen BBQ of San Diego, the High Horse of Billings, Big Sky Collision of Billings and the Big Sky Business Journal of Billings. Additional sponsors are being sought, as are teams to participate in the tournament. Contributions are also welcome.

For more information call Reid Pyburn, 406-690-1135

Sunday, August 7, 2016

What is PKD? Gift of Life: North Andover; Kidney Voucher, Waiting: Australia

What is PKD?

From News-Medical.net, By Dr Liji Thomas, MD


Polycystic kidney disease. Normal and polycystic kidneys - Image Copyright: Designua / Shutterstock


Polycystic kidney disease (PKD) is a hereditary condition in which the kidneys develop multiple cysts. This leads to renal enlargement, distortion of the normal structure of the kidneys and functional impairment.

The result may be chronic kidney disease, progressing to end-stage kidney disease (ESRD) requiring dialysis or kidney transplantation.

It affects both genders and all ethnic groups but leads to chronic kidney disease in males and in women who had three or more pregnancies and who have high blood pressure.
Symptoms

PKD often presents with pain in the back and the sides of the ribcage and headaches, while hematuria is sometimes present. In autosomal recessive PKD, enlarged kidneys, respiratory distress and growth failure in infants may be the presenting symptoms.

Incidence of PKD

PKD may be autosomal dominant or recessive. Autosomal dominant PKD occurs in up to one in 400 people but the autosomal recessive type occurs in one in 20 000 – 40 000 people.

Complications of PKD
Pain in the back or sides
Chronic renal disease
Frequent urinary tract infections
Renal failure
High blood pressure
Liver and pancreatic cysts
Abnormal heart valves
Aneurysms in the brain and heart
Intestinal diverticula
Hematuria
Kidney stones
Respiratory arrest, growth failure and hepatic dysfunction in childhood, in the autosomal recessive type

Why does PKD occur?

PKD is the result of a gene defect, which is usually present in the parent and is passed on to the offspring. At least three mutations have been found to be associated with PKD, of which two are found in the autosomal dominant type.

The most common form of PKD is the autosomal dominant type. It usually starts between 30 and 50 years, but symptoms start only when the cysts have reached a size of 0.5 cm or more. In the recessive form, cysts form in infancy or even in utero.

Diagnosis and treatment

Genetic testing is carried out on saliva or blood and looks for the presence of specific gene mutations. This helps with diagnosis as well as predicting the chances of passing on the disease. Prenatal genetic testing can identify the presence of the gene in fetuses in utero. However, genetic testing fails to predict the time of onset of symptoms or their severity.PKD is diagnosed on the basis of imaging tests and genetic testing. Imaging the kidneys involves ultrasound, CT scans or MRI scans. A scan which shows two or more cysts before the age of 30, for instance, combined with a family history of PKD, may lead to a diagnosis of the condition.

PKD has no cure and treatment is directed towards symptom control and achieving a longer life. This may involve cyst removal to relieve pain, bringing down the blood pressure, lifestyle changes and monitoring for heart valve complications or brain aneurysms. For instance, diet is important in preventing some complications, mostly due to high blood pressure and so a low-sodium diet with high intake of vegetables is often recommended, with more poultry, fish or dairy products for protein.

In the autosomal recessive form of PKD, the kidneys may be so large that they prevent normal breathing. In such cases, nephrectomy is unavoidable and such children must receive a kidney transplant or dialysis. Proper growth is ensured by dietary guidelines, sometimes in conjunction with growth hormone treatment for severe growth arrest.

Respiratory arrest is always a possibility in infants with the severe form of the disease and ventilator support is often required, as well as cardiopulmonary resuscitation in emergency situations. Kidney function is replaced by dialysis, usually peritoneal, but also hemodialysis in some cases. Control of urinary infections, hypertension and measures to slow the progression of liver disease is required. Combined liver and kidney transplantation is necessary in some cases.



PKD, Gift of Life

From Eagle Tribune, North Andover, MA, By Zoe Mathews

Kiki found a kidney

Kiki found a kidney



The sign out front of Common Grounds Cafe that once asked for a kidney donor has had its message changed from plea to praise. That's because RuthEllen "Kiki" Bernard found what she was looking for -- a matched kidney donor.

Kiki, who suffers from polycystic kidney disease, found a donor just weeks after the sign went up at the hands of her husband, Wayne. The couple said finding a donor wouldn't have happened without the sign, which brought curious passersby into the cafe to ask questions, and garnered more conversation and awareness in the community about organ donation.

The couple learned Kiki's kidneys were functioning at about 20 percent at the beginning of the year, and that she would need a kidney donor or face dialysis treatment. They kept things under wraps, not reaching out to family or friends. They faced the daunting and lengthy kidney donor waiting list alone.


"It's not something we'd talk about over dinner," said Wayne. "In a way, we didn't have to go through all of this if we had just been more open about what was going on."

But then in June, months after Kiki found out about her failing kidneys, Wayne put up the initial sign out front of his cafe on Route 97.

The sign, and the increased word of mouth, forced the couple to start talking about what was going on, and that communication extended into the Bernard family.

Lisa Twombly, Wayne's niece, had her blood drawn for compatibility testing as soon as she heard about Kiki's situation. Meanwhile, the couple said hundreds of people came through Common Grounds' doors inquiring. Not all filled out the donor applications the couple had on hand, but the couple estimates more than a dozen did.

"I said even before I knew my blood type that it was going to be me," said Twombly.

Twombly got her results last week and found out she was a near perfect match for Kiki, who scored a nine out of 100 on an antibody test that compares a transplant's recipients blood with the general population.


"I have a low antibody count, so based on 100, I have a score of nine, which means that if there were 100 donors, only nine wouldn't be a match," said Kiki.

And Twombly said her test only showed one antibody against Kiki's, meaning that the likelihood Kiki's body will accept Twombly's kidney is fairly high.

The next step for Twombly is a rigorous set of tests to ensure she is mentally and physically healthy enough to donate an organ, slated for mid-August.


When Twombly told her sons what she might be doing for her aunt, they were initially afraid for her.

"My son, Taylor, was scared about the surgery," she said of her 10 year old. "But I told him how he had surgery too, he has cochlear implants, and later he came up to me and said how he thought I was a hero, and was brave for doing this. So that feels amazing."

Just one day after the updated sign went up thanking people, Wayne said more than 20 people have come into the cafe to offer congratulations.

"There was a woman who dropped off green pins for kidney transplant recipients, like the breast cancer pins. And one daily customer, we found out got a kidney transplant 30 years ago," he said. "It's everywhere, and apparently it's just something nobody talks about."

This experience motivated Wayne to consider donating one of his own kidneys. He said he has learned so much about people suffering from kidney disease, that he's thinking about donating his kidney to start a chain -- his kidney would be transplanted into a recipient who had a donor willing to give a kidney but wasn't a match, and the incompatible donor gives a kidney to someone else.

Kiki was initially skeptical of Wayne putting the sign up; he had done it without her knowledge. But after the outpouring of support, she felt she had to let people know how grateful she was, so she put up the new sign that reads "THANK YOU - THANK YOU - WE HAVE A DONOR".




From Wall Street Journal, By JEFFREY VEALE

Give a Kidney, Get a Kidney


The numbers are staggering. Because of a lack of donor kidneys, an average of 13 people die every day while waiting for a transplant. There are more than 100,000 names on the kidney waiting list in the U.S. and another 30 million people with chronic kidney disease who are at risk of joining them. More than 85% of those on the waiting list in 2015 are still waiting.

The good news is that there were 17,878 kidney transplants in the U.S. last year, the most in a single year, according to the United Network for Organ Sharing. And the numbers may keep growing thanks to an innovative voucher program that started in 2014 at Ronald Reagan UCLA Medical Center and is spreading across the country.

Here’s how it works: If you donate a kidney now, you will receive a voucher that a loved one could use to secure a kidney in the future. The Advanced Donation program is coordinated through the National Kidney Registry, which uses a national database to quickly and efficiently match donors and recipients.

The idea was approved by the Ethics Committee of the American Society of Transplant Surgeons in June, and has been sent to that group’s executive committee for formal approval. Ten hospitals across the country have so far joined UCLA to honor the voucher program. Donors currently need to go to one of these hospitals to receive a voucher but many other centers are expected to join.

The voucher idea is the brainchild of retired judge Howard Broadman. His grandson Quinn was born with a single kidney that wasn’t fully functioning. The retiree knew that eventually the boy would need a lifesaving transplant. Yet Mr. Broadman, who was 60 years old when Quinn was born, knew that by the time the boy needed a kidney transplant, his grandfather would be too old to donate.

Mr. Broadman approached Ronald Reagan UCLA Medical Center and proposed a simple but brilliant idea. He offered to donate a kidney immediately to a stranger on the waiting list, if in return he could secure a kidney for his grandson when the time comes. Quinn would be prioritized for a kidney when he needed a transplant.

Nothing like this had ever been done, but the UCLA Living Donor Committee and the National Kidney Registry Medical Board agreed to do it. In December 2014, Mr. Broadman donated a kidney, and 4-year-old Quinn now has a voucher to redeem when he needs it, perhaps avoiding dialysis.

If only one-half of 1% of adults in the U.S. agreed to become living donors, we could rapidly clear the waiting list for kidneys. As dramatic as this sounds, it is important that potential donors be fully informed of the risks of donating a kidney. There is a 3 in 10,000 death rate and approximately a 5 in 1,000 chance that donors will develop end-stage renal disease.

Living-donor kidneys are ideal because they generally function twice as long as kidneys from deceased donors—indirectly reducing the wait list. Nearly 700,000 people are now on kidney dialysis in the U.S. Treating end-stage renal disease costs more than $30 billion each year—and takes an enormous physical and emotional toll on patients and their families. Imagine if doctors could address kidney disease long before that was even an issue.

With more kidneys to choose from, doctors could also more easily manage donor chains. Many people volunteer to donate a kidney to a friend or loved one in need, only to find out that they are not a compatible match. With this program, they could still donate, and the National Kidney Registry computer software would find a matching donor. One donor in the voucher program could be used to trigger a chain of transplants, creating the added benefit of freeing people from dialysis now, and their loved ones from dialysis in the future.

Potential donors often state that they are interested in donating a kidney but are hesitant because a spouse or a child could need one in the future. With the voucher program, that issue is no longer a concern.

As a transplant surgeon, I’m invigorated by the prospect of what this voucher program could mean. This could be our only realistic shot at eliminating the waiting list for kidney transplants.

Dr. Veale, a transplant surgeon at Ronald Reagan UCLA Medical Center, is director of the UCLA Kidney Transplantation Exchange Program.




Waiting

From The Ballarat Courier, Australia, by Melanie Whelan

Wait for a life-changer


Mr Barnes’ regime is strict. The process takes him four and a half hours, it is physically demanding and it is keeping him alive.

Three years ago, Mr Barnes’ kidneys failed. He was put on an active waiting list for organ donation that would change his life.

“(Organ transplant) is something I always think about...it would certainly make a big difference in my life,” Mr Barnes said. “For me to receive a kidney, it would be from a deceased person and it is hard, but you still hope to get the call every day and it’s got to be a match.”


This year’s DonateLife Week, which started on Sunday, is themed ‘end the wait' and urges Victorians to join the Australian Organ Donor Register online.


Mr Barnes was diagnosed with polycystic kidney disease when he was 28 years old. Growing cysts would enlarge his kidney and gradually damaged the kidney tissue to the point when they failed.


Now aged 45, Mr Barnes said his life was not all doom and gloom. He maintains a healthy lifestyle and diet and has to carefully watch his fluid intake, which can often mean a fine line between dehydration and too much fluid.

As director of construction company SJ Weir, Mr Barnes works smarter by doing some paperwork on his iPad on dialysis and scheduling his dialysis in afternoons to get work done early then be fresher the next day.

Travel on short notice is tougher. Mr Barnes has to be assured he can have treatment at set times when away.


“It probably took me 12 months to get used to the way life changed with dialysis,” Mr Barnes said. “Before I was on it, I took my life for granted a bit. Dialysis really changes your life but is now part of my weekly routine.”

One third of Australians have registered their donation decision on the national donor register, despite almost 70 per cent of people indicating they would be willing to become a donor. DonateLife encourages people to talk about their decision with loved ones.