Polycystic Kidney Disease and Bile Duct Malformations
Since the day she was born, Valen Keefer, has faced a challenging medical heritage. Everyone on her mother’s side has suffered from polycystic kidney disease (PKD). The oldest to live in her family is her mom at 62-years-old. Everyone else passed away from PKD complications before the age of 53. Her 42-year-old brother is currently on dialysis and her mom still has her original kidneys and her health is well with not needing to endure dialysis or transplant yet. Although Valen was told that she would follow the same path as the rest of her family and not be affected until her late 40’s to early 50’s, her path with PKD wound up evolving the complete opposite than the rest of her family and she is the youngest in her family to endure such severe side effects from the disease.
Valen was diagnosed with PKD at the age of 10 when she went to her pediatrician for a check-up and her blood pressure was 160/140. She was sent for an immediate ultrasound and cysts polluted her kidneys. From age five, she battled through seizures, scoliosis surgery and countless cyst bleeds. She spent a year in the hospital when she was 18 where she had a bilateral nephrectomy, pancreatitis, endured daily dialysis, a congenital anomaly (bleeding vessel in her stomach), and more than 70 blood transfusions because of all of the cyst bleeds. Her kidneys were working 60%, but had to be removed because they could not stop the cyst bleeds. Valen was too sick to be placed on the waiting list but needed a kidney to survive. A close family friend was a match and a surgeon at Johns Hopkins took the risk of doing the transplant and at 19 years old on August 13, 2002, she became the first in her family to receive a life-saving kidney transplant. Over sixteen years later, that same kidney is doing great!
If you had asked Valen about her PKD journey a couple years ago, she would have told you that she climbed that mountain and conquered it when she received her kidney transplant. She never fathomed this devastating disease would come back into her life, full force, many years later.
Valen knew PKD could affect other parts of her body, causing aneurysms, diverticulosis and polycystic liver disease. Her scans for these potential issues were always clear and she was grateful her liver remained unaffected by PKD.
In Fall 2016, Valen started to get sporadic bouts of sepsis. As a transplant patient with a suppressed immune system, she rushed to the emergency room every time, with tremors, a fever, abdominal pain and nausea. She had a high white blood cell count and bacteria in her bloodstream, but all other lab tests were fine. She was given fluid and IV antibiotics for a few days, and sent home never knowing the cause.
Over time these mysterious infections worsened. She worked closely with her doctors, doing every test possible to find the source of infection. After several more sepsis episodes, a PET scan was approved and her liver lit up leading them to the source of infection. In the meantime, she kept getting sick with sepsis and was enduring daily IV and oral antibiotics and needed a life-saving liver transplant immediately.
Valen’s doctors did not think she would survive the wait time for a liver in her home state of California, so they suggested she get dual listed. Her and her husband temporarily relocated to St. Louis, MO and on August 4, 2018 she received a liver transplant at Barnes Jewish Hospital.
Upon pathology of her liver, it was determined that she did not have primary sclerosing cholangitis (PSC) as she was originally diagnosed with, but rather her polycystic kidney disease (PKD) had caused bile duct plate malformations leading to the liver infections, which caused the sepsis episodes and the need for a new liver. Her native liver did not have one cyst on it!
Today as a dual transplant recipient, Valen is a 36-year-old woman who is more passionate than ever to share her PKD journey and educate and inspire. In her 15 years of being active in the PKD community, she has never met another person that needed a kidney transplant because their cysts would not stop bleeding or a person who needed a liver transplant because of the issues that she had with her bile ducts. Valen had genetic testing done and is confirmed that she has ADPKD, PKD1 gene. To think that all of these unique manifestations occurred in the most common form of PKD shows that Valen can’t be the only one experiencing these life-threatening complications and underscores the vital need for her story to be shared for patients and the medical community to learn from.
Her threefold mission is to increase the public’s understanding of the importance of research into kidney disease, to help spread awareness of PKD, and to promote organ donation. Because so many members of her family grapple with PKD, or have died from it, she has developed a strong connection with those who endure the pain and suffering caused by PKD. Valen’s story has really resonated with the PKD community and they find hope from all that she has endured, how she shares her story and is the voice for the PKD community and how she lives every day positively demonstrating that one can live a fulfilling life post-transplant and with PKD.
Valen has been a tireless advocate for PKD since 2004 when she founded the South Central Pennsylvania chapter of the PKD Foundation, and organized numerous PKD fundraising walks and educational seminars. She is an experienced public speaker, having shared her story at more than 80 events across North America. Valen has used her first-hand experiences with the disease and shared them in her blog – PKD Will Not Beat Me – where she has written over 230 inspiring blogs that have been published on the PKD Foundation website. Her story also inspired a Canadian author to write her biography. The award-winning biography is titled, My Favorite American, by Dennis McCloskey.
Valen has earned many accolades and awards, including:
- Corks for a Cure committee member, helping to organize the annual PKD fundraiser raising over $150,000 – 2016-2019
- Recipient of the Bounce Back Give Back award from the Chris Klug Foundation, 2017
- Being selected as one of the 12 Inspiring Women of “20 Million in 2012” by Donate Life America, 2012
- Appointed a national spokesperson for University Kidney Research Organization (UKRO), 2011
- Selected as winner of nationwide 2011 Astellas “Ride of a Lifetime” Rose Parade Contest, 2010
- Recipient of the Susan P. Byrnes Health Education Center “Celebration of Life Award,” 2009
- Selected as the “Smart Woman” for Smart Magazine, 2009
- Recipient of the first-ever “Helene Kenigsberg Award for Excellence in Public Awareness of Polycystic Kidney Disease” from the PKD Foundation of Canada. This award was established in 2009 and is the only award of its kind, honoring individuals who have made an outstanding contribution in creating public awareness of PKD on a national level.
- Subject of award-winning biography, My Favorite American, by Dennis McCloskey, 2008
- Recipient of the following Dale Carnegie Course Awards: Human Relations, Human Relations Champion, Outstanding Performance, and Highest Award for Achievement, 2008
- Campaign Chair for the PKD Foundation’s “Campaign for a Cure,” raising over $1,000,000, 2005–2006
- Nominated for, received the most votes in a nationwide contest, and named the first-ever DaVita “Kidney Idol,” 2005
- Chapter and Walk Coordinator for the South Central Pennsylvania Chapter of the PKD Foundation, raising more than $100,000, 2004–2007
- Founder of the South Central Pennsylvania Chapter of the PKD Foundation, 2004
Valen’s blog – www.pkdwillnotbeatme.com
Recent speech given on March 9, 2019 – https://vimeo.com/324120906
Recent TV interview for Donate Life Month on March 31, 2019 – https://www.kcra.com/article/norcal-woman-saved-by-organ-donors-gives-back/26999430?fbclid=IwAR3nXn9RCGLpIoZLKlXNq_5zD3s17PNvqer2N8MItYaiRagbTr5jk7U0dMM
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