The Invincible Valen Keefer: PKD and Organ Transplant Advocate

In Features, Magazineby RI Magazine

A polycystic kidney disease survivor, Valen Keefer’s life has been a long series of complicated surgeries—including two transplants—and countless setbacks and near-misses. So why is she still smiling?

BY MATT TUTHILL

She’s been defying the odds from the very beginning. Long before she knew she had polycystic kidney disease. Long before that disease caused both kidneys to blow up to the size of footballs and require a transplant at the age of 18. And long before her liver, with malformed bile ducts that caused sepsis, gave out and required another transplant.

To get the full scope of Valen Keefer’s against-all-odds story, you have to go back to before she was born, with her mother Pam Cover sitting in the parking lot of York Hospital in Pennsylvania in 1982, tissues in her hands, her husband Bill beside her, as she grappled with the most important decision of her life. Pam, early on in her pregnancy with Valen, was scheduled to have an abortion that day. Pam’s doctor had said the procedure would be wise on account of the fact that Pam suffered from polycystic kidney disease (PKD) and stood a 50 percent chance of passing it on to her child.

Pam and her husband Bill already had one child, but had learned more about the difficulties of PKD in the intervening years. And on that fateful day, they were only six months removed from losing Pam’s mother Pauline to the disease. To spare another soul from the agony of PKD, an abortion felt like mercy.

PKD, which afflicts over half a million people in the U.S. and 12.5 million people worldwide, riddles the kidneys with fluid-filled cysts, greatly reducing their ability to carry out their crucial function of filtering waste products from the body. In turn, this can lead to high blood pressure, kidney and liver cysts, cyst bleeds, aneurysms, diverticulosis, and heart problems. In some cases—like Valen’s—the cysts cause the kidneys to exponentially increase in size. There is no cure.

That grim reality of PKD swirled in Pam and Bill’s minds as they sat in the car, staring around at the other cars in the parking lot. But for as much as they couldn’t bear the thought of sharing this disease with another person, Pam had another image she couldn’t get out of her head. Some days prior, she went for a walk and witnessed a mother pushing her child in a stroller. She made eye contact with the child, and later told author Dennis McCloskey in his biography of Valen, My Favorite American, that the child gave her “a penetrating look… I knew immediately those beautiful eyes were telling me to have this baby inside me.”
Pam didn’t tell Valen about the near-abortion until 2007, when her daughter was 25 years old.

But by that point, Valen was already deep into a medical odyssey that would see her receive not just kidney and liver transplants, but spend seven months on dialysis, six months on a feeding tube when she came down with pancreatitis, receive over 70 blood transfusions, and undergo dozens of other procedures and surgeries to treat everything from scoliosis to skin cancer. Her medical history is a morbid, but clichéd punchline. Indeed, if she didn’t have bad luck, it seems she’d have no luck at all.

Left, Valen’s husband Noah greets her as she awakens from liver transplant surgery. At right, still recovering several days after surgery.

And yet, despite life pummeling her at nearly every turn, you would be hard-pressed to find anyone more in love with life itself. Valen’s gratitude is boundless, and extends to everyone from her doctors to random people who drop by to comment when she posts on Instagram, where she flashes her movie-star smile for hundreds of pictures, all of which contain a callback to her overwhelming sense of gratitude, to PKD awareness, or an upcoming fundraiser. Valen’s “thank you” comes from the heart, and she says it constantly. Suffice it to say she held no ill will toward her mother, and instead thanked her for being brave enough to go against her doctor’s recommendation.

Valen’s deepest, most heartfelt gratitude, however, seems reserved for her donors, both her living donor, Sally Robertson, who gave up a kidney for Valen back in 2002, and her deceased liver donor, whose family she hopes to meet some day. (Valen wrote a letter to the family of her deceased donor; that family then has the option to reply or not on their own timeline.)

“This gift is… miraculous and mind-blowing and surreal,” Valen says, “and still hard for me to comprehend seventeen years later… It’s something that I can’t even explain looking at Sally, just thinking of how selfless she is.”

Valen and Sally connected via Sally’s daughter, Emily, who met when they were in eighth grade. Sally had been vaguely aware of Valen’s health complications over the years, but didn’t get the full picture until Emily and Valen graduated high school and roomed together at Millersville University in Pennsylvania. That’s when Valen’s kidney condition, which had worsened throughout her senior year of high school, bloomed into a critical emergency.

Valen spent the next year in the hospital as doctors tried a number of procedures and medications to drain the cysts and stop the bleeding, but nothing was effective. All the while, Valen’s kidneys continued to grow to such unfathomable proportions that strangers, thinking she was well into a pregnancy, didn’t hesitate to ask her for her due date. It was disheartening, but the least of Valen’s worries. The kidneys were so huge they pushed up against her lungs, making it difficult to breathe.

“And it was just constant looking in the toilet,” Valen says. “I remember my mom and I standing at Hershey Medical Center and we just wanted to see yellow pee. And we just were so disappointed every time because it would be red. I was losing so much blood that they were giving me transfusion after transfusion to replenish it. And then they got to where they looked at my parents and said, ‘There’s nothing more we can do.’”

Valen’s parents wouldn’t hear of it, and transferred Valen to Johns Hopkins in Baltimore. Specialists at the hospital decided to remove both kidneys and place Valen on dialysis until a donor could be found. When Sally heard of the situation, she immediately volunteered.

Sally, a deeply religious and spiritual woman, says she made a leap of faith that felt like true destiny.
“I could feel the Lord saying to me, ‘You’re going to do this. This is what I have planned for you,’” Sally recalls. “And I really had a peace about it. In my heart, I knew it was going to work.”

The transplant wasn’t just successful, it marked a major turning point in Valen’s life. When she was back on her feet, she dove into her advocacy work for PKD awareness and, more generally, for organ donation. In 2008, she became the subject of My Favorite American. She was energetic and productive, but a new normal had taken root: twenty pills a day and monthly labs to ensure her body wasn’t rejecting the new kidney.

A short time after that she met Noah Keefer through mutual friends. Valen, a woman who wears her heart on her sleeve, was drawn to the same in Noah.

“People always say things about me smiling all the time,” Valen says, “and you don’t necessarily see men like that. I was like, ‘Oh my gosh. He looks so happy. That’s adorable.’”

Noah offered to buy her a drink and Valen declined, blurting out that she couldn’t because of her kidney transplant. Subsequent attempts at small talk yielded more extraordinary details.

“He called me a couple days later to just talk and see if I wanted to hang out,” Valen recalls. “I said, ‘Well, I can’t. I’m in Washington, D.C. about to speak to the congressional kidney caucus about polycystic kidney disease.’ He was like, ‘Oh my gosh. OK.’”

When they finally met up for a date, they ordered the same thing—chicken Caesar salad and water.
“We just kind of looked at each other and realized, ‘Yeah. This is gonna work.’”

They married in 2011, but unbeknownst to Valen, her health complications were far from over.

Seemingly random illnesses in 2014 and 2015 gave way to a life-threatening bout of sepsis in 2016. Being a transplant patient, Valen is on a lifetime regimen of immuno-suppressant drugs; her immune system will always identify her transplanted kidney as a foreign body and could reject it at any time. And since she’s never fighting with the full strength of her immune system, any time she gets sick she has to rush to the emergency room as even a minor infection could quickly get out of hand.

Sally and Valen celebrate the anniversary of Sally’s donation. This summer marks seventeen years since the kidney transplant.

Doctors treated the sepsis with antibiotics and IV fluids and sent Valen home without a firm diagnosis. After repeat bouts, however, they believed she had primary sclerosing cholangitis, or PSC. But Valen found it hard to believe that she could suffer from both PKD and an unrelated liver disease. Doing her own research, she found that some PKD patients suffer from Caroli’s disease, which adversely affects the bile ducts of the liver and can cause sepsis.

Whatever the case, time was short; by 2018 Valen was in dire need of a liver transplant. Facing a long wait list in California—where she’s lived with Noah since 2010—she also wait-listed in St. Louis. She and Noah were forced to relocate temporarily while she waited. Once doctors informed her that she was at the top of the list for her blood group in St. Louis, she and Noah temporarily relocated there. After a week, she got the call. After eight hours on the operating table at Barnes-Jewish Hospital, she emerged yet again as a survivor. Six weeks later she was back home.

Pathology of her original liver revealed that she did not have primary sclerosing cholangitis (PSC) as doctors suspected, nor textbook Caroli’s disease, but bile duct malformations as a direct result of her PKD.
Genetically, no one in Valen’s family has had as harrowing of an ordeal. Her older brother has suffered some complications, and so has her mother Pam, though she is healthy today at the age of 63.

Once Valen was back on her feet, she went right back to her advocacy efforts: public speaking, cutting videos for Barnes-Jewish Hospital, working on the organizing committee for the Corks for a Cure event, and appearing at the Dreamwalk Fashion Show—a body inclusive fashion show—in NYC on September 15.

These are just a few more notches in an already stacked resume; Valen has spoken at over 80 events across North America, written over 250 inspirational blog posts for non-profit organizations, helped the PKD Foundation raise over $1,000,000 to help find a cure, and in 2017 received the Bounce Back Give Back Award—given to extraordinary organ recipients from the Chris Klug Foundation.

It’s easy to see why Valen, with her boundless energy, passion and gratitude for life—and yes, that smile—is the ideal spokesperson for PKD awareness and organ donation. She proves there is not just life with severe PKD, but potentially a great one. With enough exposure to Valen’s particular brand of optimism, potential donors start to get a picture that they wouldn’t just be giving a few more years so a sick person could barely chug along—but maybe deliver a ripple effect like Valen’s where you don’t just save a life, but positively affect every life the recipient’s touches.

That’s been Sally’s experience, and watching Valen make the most out of her life has been its own reward.
“I tell her, ‘I just love watching you grow older,’” Sally says. “I love seeing her age because I know she’s still here because she wouldn’t have been if she didn’t have the transplant.

“And now it’s the same thing with her liver. It’s just been really humbling. Humbling for me to have been the one God choose to help her. And that’s how I feel. I love her dearly, and I just want her life to be full of joy. I truly do… None of us know how long we’re here. We don’t. But I think we’re to make the best of the time that we have. We’re to love other people in the very best way that we can while we’re here.”

This is, of course, the driving force behind Valen’s tireless work ethic, as she toes the line every day between survival and advocacy.

“I’m technically balancing three different immune systems,” Valen explains. “My immune system, the liver, and the kidney, because they’re all different. So, we have to balance the meds and make all of us happy and, well, and it’s a constant struggle… Realistically, the organs won’t last forever, and with being transplanted so young I’ll need another kidney, I’ll probably need another liver, and thinking about that…”

It’s one of the few times Valen seems lost for words, but she soon finds positive footing.

“This is when I focus on my extraordinary caregivers and the appreciation I have for my parents and Noah,” Valen says. “I never imagined to find a husband so dedicated, supportive, patient, caring and loving as Noah. We approach this transplant journey as a team and I can’t imagine doing it without him. He gives me something to look forward to and something to fight for. My parents were amazing throughout my childhood and are still extremely supportive and dedicated.

“I was not blessed with great health but I sure am rich in love.”

“I have a mission to use this gift the best way possible,” Valen continues. “I never feel like, ‘I’m done. I’ve thanked her.’ I’ll never feel that way. It’s a continuous mission. So, I thank her—and my liver donor—by action. I thank them every day by taking really good care of myself and by showing others how amazing the gift of life is. I want people to know, ‘If I’m an organ donor, this is how recipients are grateful. This is what they do with my gift.’ I want to set an example for that.”

Wherever else Valen’s story might lead, it doesn’t feel premature to say: mission accomplished.

Valen has continued her advocacy work after recovering from liver transplant surgery. Read Valen’s inspirational blog at PKDwillnotbeatme.com. Watch one of Valen’s many TV interviews HERE. If you’re interested in collaborating with Valen in her advocacy efforts, e-mail her at: pkdwillnotbeatme@yahoo.com

Interested in learning more about being an organ donor? Visit DONATELIFE.net.

Follow Valen on FACEBOOK, INSTAGRAM, and TWITTER.

Valen, appreciating every ray of sunshine and every breath of fresh air.

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