Sunday, April 17, 2016

PKD Support; Research into PKD Suppression; A Teacher's True Gift

PKD Support

From Ottumwa Courier, Iowa, by Neal Querio

Community comes together to support local principal

Fundraiser



Watching a loved one suffer is a heavy burden for anyone to bear, but Cindy Green had no intention of standing by while her husband Jay suffered.

On Saturday afternoon, the community members of Ottumwa and Ottumwa Community School District gathered together to raise money for James Elementary Principal Jay Green.

Back in 2005, Jay was diagnosed with Polycystic Kidney Disease. This disease can vary in severity, but it usually causes noncancerous cysts to form on a person’s kidneys and can eventually spread to other organs. Jay tried to stay positive after the diagnosis, but over the years his kidneys have slowly shut down, eventually moving him into stage 5 liver failure. Jay was put on the list for a transplant, but Cindy began looking into donating one of her own kidneys.

“Cindy keeps saying it’s not that big of a deal; I’m sure anyone would do this for their spouse,” said Jay. “But that is really a testament to how caring she is because she doesn’t have to do this.”

Initially doctors told the couple that Cindy was not a match so she was unable to donate. However, they went to get a second opinion at the Mayo Clinic in Rochester, Minnesota, where they finally got some good news. Cindy was indeed a match and they could move forward with the transplant as soon as possible.

“People keep telling me that it is such a big deal to be doing what I’m doing, but Jay would do anything for anyone and I wanted to do the same,” said Cindy.

Wanting to help out with the financial burden the family was facing, the Ottumwa School District community pulled together to host a fundraiser for Jay and Cindy. There were people from all over Ottumwa and even members of the Cardinal Community School District, where Cindy works as the curriculum director.

There was also an Iowa Donor Network booth set up by Heather Butterfield, a former student of Jay’s. She said when Cindy asked her to be a part of the event, Butterfield knew right away the answer was yes. Butterfield wanted to support Jay and encourage people to become registered donors.

“The wait-list for kidneys is one of the longest ones,” said Butterfield. “Even if we just reach one person and get them registered, that can make a significant impact.”

Evans Middle School teacher Jessica Short played a major role in getting the fundraiser organized for the couple. Short worked with Jay when she first started teaching 15 years ago and she wanted to do what she could to help out someone who she said was one of the most compassionate people she knows.

“We wanted to do this because he’s Jay. He’s amazing and he is always willing to help everyone else,” said Short.



Gift of Life

From Fox News

Surgery set for teacher who volunteered to donate kidney to student

Jodi Schmidt is donating a kidney to her student, Natasha Fuller.

This WONDERFUL lady named Jodi Schmidt is a teacher at Natashas school...and she gave the best ever present to Tasha.Jodi is a match for Tasha to get a kidney.........so very very thankful to Jodi and her family....Tasha will get a NEW kidney from this wonderful lady......GOD BLESS!!!!!!

A Wisconsin teacher who surprised the family of one of her student’s by volunteering to donate a kidney will soon make good on her promise, as the surgery has been set for April 27. Jodi Schmidt, a first-grade teacher at Oakfield Elementary School, surprised Natasha Fuller’s grandmother with the news in March.

In a recorded video Schmidt hands 8-year-old Fuller’s grandmother, Chris Burleton,a pink box to open.

"I just wanted to give you a gift just for how hard you work just to keep her here because we really appreciate everything you do," Schmidt told Burleton.

As the grandmother opens the box she sees a message informing her that Schmidt was found to be a match for Fuller and immediately bursts into tears

"Here I thought I was coming to school because she was naughty," Bulerton said in the video.

Fuller, an identical twin, was diagnosed with polycystic kidney disease and prune belly syndrome before she was born. Her mother and eight siblings live in Oklahoma while Fuller lives in Wisconsin so she can receive dialysis three days per week at Milwaukee Children’s Hospital.

"It's been really hard," her mother, Kerri Cox, told KFDX.com. "We try to Skype as much as possible. Her and her twin, they send letters constantly and Skype."




From Lompoc Record, California, by Gina Kim

Flag-raising ceremony celebrates organ donors nationwide

041216 Donate Life 01.jpg


Marian Regional Medical Center recognized National Donate Life Month by participating in the national "Flags Across America" event and raising the OneLegacy Donate Life flag in a special ceremony to honor donors and staff Tuesday afternoon.

Partnering with OneLegacy, the nonprofit, federally designated organ, eye and tissue recovery organization, Marian raised the flag at the front of the hospital to help spread the message that organ and tissue donations save and improve lives.

The flag raising was followed by a commemoration speech by Kerin Mase, chief executive officer and president of Marian Regional Medical Center.

"We've been celebrating this important occasion here at Marian since 2003," Mase said. "Promoting organ tissue donation is a cause close to our hearts here, as we have saved so many lives through donation."

She continued: "Approximately 200 people in Santa Barbara County are waiting for a life-saving organ, and hundreds more are awaiting cornea and tissue transplants.

"We're partnering today with OneLegacy and are proud to raise our OneLegacy Donate Life flag to honor our staff who help facilitate these lives in the gift of donation," she continued.

"We're flying our flag today, letting people know that we're here to support the community, the patients we serve and life," she said.

The recognition of National Donate Life Month commemorates those who have received or continue to wait for a life-saving organ transplant or life-enhancing tissue transplant.

Currently, more than 21,000 California residents are waiting to receive life-saving organs.

The wait for organ transplants in the Santa Barbara County area is about seven to 10 years. In the last three years, Marian Regional Medical Center saved 20 lives through organ donation.

Lisa Marciano, donation development coordinator at OneLegacy, shared her personal story as a patient who waited for a kidney for two years after a life-altering automobile accident when she was 25 years old.

"My mother had polycystic kidney disease, and I did, too, but growing up I was a sports addict, skied, played tennis and swam," Marciano said.




PKD Research

From Uro Today

Inpp5e suppresses polycystic kidney disease via inhibition of PI3K/Akt-dependent mTORC1 signaling.


Polycystic kidney disease (PKD) is a common cause of renal failure with few effective treatments. INPP5E is an inositol polyphosphate 5-phosphatase that dephosphorylates phosphoinositide 3-kinase (PI3K)-generated PI(3,4,5)P3and is mutated in ciliopathy syndromes.

GermlineInpp5edeletion is embryonically lethal, attributed to cilia stability defects, and is associated with polycystic kidneys. However, the molecular mechanisms responsible for PKD development uponInpp5eloss remain unknown. Here, we show conditional inactivation ofInpp5ein mouse kidney epithelium results in severe PKD and renal failure, associated with a partial reduction in cilia number and hyperactivation of PI3K/Akt and downstream mTORC1 signaling. Treatment with an mTORC1 inhibitor improved kidney morphology and function, but did not affect cilia number or length. Therefore, we identify Inpp5e as an essential inhibitor of the PI3K/Akt/mTORC1 signaling axis in renal epithelial cells, and demonstrate a critical role for Inpp5e-dependent mTORC1 regulation in PKD suppression.

Human molecular genetics. 2016 Apr 07 [Epub ahead of print]

Sandra Hakim, Jennifer M Dyson, Sandra J Feeney, Elizabeth M Davies, Absorn Sriratana, Monica N Koenig, Olga V Plotnikova, Ian M Smyth, Sharon D Ricardo, Robin M Hobbs, Christina A Mitchell

1. Cancer Program, Monash Biomedicine Discovery Institute, Department of Biochemistry and Molecular Biology, Monash University, Clayton Victoria 3800, Australia.,

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