Challenge Accepted

When diagnosed with polycystic kidney disease (PKD), facing and overcoming challenges are part of the disease. This month, the PKD Foundation is hosting a month-long challenge. One that if accepted, will help others and ourselves. Are you up for the 31 Days of PKD Challenges?


In honor and celebration of March’s National Kidney Month, the PKD Foundation has selected simple, daily challenges that can help raise awareness of PKD. I hope you will join me in participating in the fourth annual 31 Days of PKD Challenges! Please follow along on Facebook, Twitter and Instagram for daily updates on our progress. Share your progress too, using #PKDChallenge, #kidneymonth and #endPKD.

So far this month, a PKD educational video has been shared, special Kidney Month graphics were posted on social media, people were encouraged to share their PKD stories, to stay connected by joining the Foundation’s email list and to drink lots of water. It is important to give a voice to our disease. I accepted Tuesday’s challenge by doing an interview with James Goodnow, founder of Kidney Challenge. The mission of his organization is to ‘call on people to speak up on kidney disease.’ He shared our 10-minute Skype interview on his social media page, which has more than 14,000 likes, to help raise awareness of PKD. I encourage you to watch the interview by visiting:

It is encouraging to know that we are not alone. There are so many of us fighting the good fight to END PKD, including patients, family members, loved ones, PKD Foundation staff and scientists. Scientists take on a big challenge every day to find treatments and cure for PKD. A huge announcement was made on Feb. 26. A treatment for ADPKD was approved in Canada!

Dr. WatnickI spoke to Dr. Terry Watnick, who was my nephrologist at Johns Hopkins in 2002 when my kidneys were removed and I received a kidney transplant. Dr. Watnick was the Principal Investigator for the TEMPO 3:4 Site at Johns Hopkins and is now the Principal Investigator for the REPRISE Site at the University of Maryland. That is the new tolvaptan study that is recruiting participants with more advanced PKD. She is also the Chair of the Scientific Advisory Committee for the PKD Foundation. Dr. Watnick is extremely passionate and determined to help all of us with PKD. She shared the information (below) on tolvaptan being announced as a treatment for ADPKD in Canada, in hopes to answer some questions you may have.

“Tolvaptan is a drug that has been approved in Japan and Canada to treat polycystic kidney disease. Tolvaptan slows cyst growth by interfering with the actions of vasopressin. Vasopressin is a hormone that we all produce, which maintains water balance by acting on the kidney to increase water reabsorption. Vasopressin increases the levels of cAMP, which are high in many forms of polycystic kidney disease and are thought to contribute to cyst growth.

The FDA in the United States has approved tolvaptan for the treatment of conditions where the levels of sodium in the blood can be low (such as congestive heart failure or cirrhosis).

Based on data generated in the laboratory, the TEMPO 3:4 trial was initiated to study tolvaptan for the treatment of ADPKD.  TEMPO 3:4 was conducted in 1,445 adults with ADPKD and showed promising effects of tolvaptan in slowing cyst growth.  This three-year study included participants with large kidneys and relatively preserved kidney function at the start of the study. Over three years, those individuals treated with tolvaptan showed a significant reduction in the rate of kidney cyst growth. There was also slowing in the rate of loss of kidney function by about 30 percent in individuals treated with tolvaptan compared with those that received placebo (a sugar pill). The most common side effects of tolvaptan include thirst, polyuria and nocturia. Tolvaptan was also associated with an increase in the incidence of elevated liver enzymes, which can indicate liver damage. Because of this, all patients who take tolvaptan must be monitored very closely for elevations in liver enzymes. In the U.S., the FDA has requested additional study of tolvaptan and there is a new study underway that will test tolvaptan in more advanced PKD.”

There was nothing like this going on 13 years ago when I was gravely ill and in need of a transplant. It is miraculous to see how much has been discovered over the past decade. We are all looking for hope and this is indeed hopeful news. Each of us plays an important role:

  • the strong patients who step up and share their story, take the best care of themselves and if possible, participate in trials
  • the PKD Foundation staff that work tirelessly on advocacy, supporting patients and funding research
  • the dedicated scientists, who put the pieces of the puzzle together to improve PKD patient’s quality and longevity of life.

Dr. Watnick 2I believe if we all join together and accept and face the challenges that are associated with PKD, that more treatments will unfold and hopefully a cure will be found.

“You’ll never find a better sparring partner than adversity.” – Golda Meir

Do you have any advice on how you have overcome PKD challenges? Are you participating in the 31 Days of PKD Challenges, and which ones have you enjoyed and/or are you looking forward to?


  1. Cheryl C Hebert

    I am third generation with PKD. GFR is around 15 but not on dialysis. On a shot that has helped with the cysts and follow a strict diet as well which both factors combined have worked well so far. I discovered I had this when I was testing to donate my kidney for my father who ended up passing away after 14 years of dialysis. Now one of my two children has been tested and does have it as well. I continue to work a full time job but energy level is low and try to drink plenty of Water and rest. Prayer, positive attitude, and a wonderful dr. who has been treating patients with this for many years has been a blessing.

    • Roger Salls

      Hi Cheryl. Would be interested to know more about your diet and the shot you received. I’m at 20% gfr and not on dialysis. Feel pretty good with decent energy. I get seen at the Denver V A Hospital. I’ve just recently committed to drinking at least 3 liters of water daily, which has made a difference. Have been put on sodium bicarbonate pills and told they help in slowing down cyst growth. Have you heard anything about this? Has your Dr. said anything about Tolvapton? I’m confused about weather it’s being used here in the U S for PKD. Roger

      • Adriana

        Roger Salls, I am also at 20% eGFR. I entered stage in 2014. I evaluated for a transplant with a living donor and now just waiting to feel it’s that time. I don’t feel bad enough to want it yet and I also don’t want to lose my chance. I have been drinking 3 liters of water for about 10yrs and eat very low sodium (a bit too low it seems at time and low potassium so I was asked to cut back on my water in my case.) I’ve known since age 15 and have had mostly an organic plant based/low protein diet with ‘treats’ here and there. Moderation. Not sure I have ever heard about ‘a shot’ mentioned by Cheryl.

    • Valen Keefer

      Hi Cheryl,

      Thank you for sharing your history and advice. I never heard of a shot to help with cysts and hope you can share more information on that. I wish you and your family all the best!

  2. Istvan Fabian

    Hi Cheryl,

    I also have PKD so had my mom and my daughter. I wonder if you could tell me about that shot you mentioned?

    Thank you,
    Wish you well!

  3. Laurie

    Valen, you’re an inspiration! Thanks for sharing and supporting and raising awareness for PKD.

    As much as drinking a lot of water helps, having a positive attitude truly makes a difference. Even though I’m aware of my diagnosis and my kidney function continues to decline steadily, all in all, I’m thankful every day for the health I have and for the solutions – both existing and potential.

    • Valen Keefer

      Hi Laurie,

      Thank you very much! It is my pleasure and honor.
      I agree with you 100%. A positive attitude is so important. Thank you for sharing your positive and hopeful spirit. I wish you the best on your path with PKD!


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