My Path with PKD
Almost everyone on my mother’s side of the family has suffered from polycystic kidney disease (PKD). The oldest to live in my family is my mom at 63-years-old. Everyone else passed away from PKD complications before the age of 53. My 42-year-old brother is on dialysis and my mom still has her original kidneys and her health is well with not needing to endure dialysis or transplant yet. Although I was told that I would follow the same path as the rest of my family and not be affected until my late 40’s to early 50’s, my journey wound up evolving the complete opposite than the rest of my family as I am the youngest in my family to endure such severe side effects from the disease.
I was diagnosed with PKD at the age of 10 when I went to my pediatrician for a check-up and my blood pressure was sky-high at 160/140. I was sent for an immediate ultrasound and cysts polluted both of my kidneys. From age five, I battled through seizures, scoliosis surgery and countless cyst bleeds. I spent a year in the hospital when I was 18 where I had a bilateral nephrectomy (removal of both kidneys), pancreatitis (fed by a picc line and unable to eat or drink anything for six months), endured daily dialysis, a congenital anomaly (bleeding vessel in my stomach), and received more than 70 blood transfusions because of all of the cyst bleeds. My kidneys were working 60%, but had to be removed because they could not stop the cyst bleeds. I was too sick to be placed on the transplant waiting list but needed a kidney to survive. A close family friend, Sally Robertson, was a match and world-renowned transplant surgeon, Dr. Robert Montgomery, took the risk of performing the transplant and at 19 years old on August 13, 2002, I became the first in my family to receive a life-saving kidney transplant at Johns Hopkins. Seventeen years later, that same kidney is doing great!
If you had asked me about my PKD journey several years ago, I would have told you that I climbed that mountain and conquered it when I received my kidney transplant. I never fathomed this devastating disease would come back into my life, full force, many years later.
I knew PKD could affect other parts of my body, causing aneurysms, diverticulosis and polycystic liver disease. However, my scans for these potential issues were always clear and I was grateful my liver remained unaffected by PKD.
In Fall 2016, I started to get sporadic bouts of sepsis. As a transplant patient with a suppressed immune system, I rushed to the emergency room every time, with tremors, a fever, abdominal pain and nausea. I had a high white blood cell count and bacteria in my bloodstream, but all other lab tests were fine. I was given fluid and IV antibiotics for a few days, and sent home never knowing the cause.
Over time these mysterious infections worsened. I worked closely with my doctors, doing every test possible to find the source of infection. After several more sepsis episodes, a PET scan was approved and my liver lit up leading doctors to the source of infection. In the meantime, I kept getting sick with sepsis and was enduring daily IV and oral antibiotics and by 2018 needed a life-saving liver transplant.
My doctors did not think I would survive the wait time for a liver in my home state of California, so my hepatologist suggested I get dual listed. My husband, Noah, and I temporarily relocated to St. Louis, MO and on August 4, 2018 I received a liver transplant at Barnes-Jewish Hospital.
Upon pathology of my liver, it was determined that I did not have primary sclerosing cholangitis (PSC) as I was originally diagnosed with, but rather polycystic kidney disease (PKD) had caused bile duct plate malformations with von meyenburg complexes leading to the liver infections, which caused the sepsis episodes and the need for a new liver. My native liver did not have one cyst on it!
Now as a 36-year-old dual transplant recipient, I am more passionate than ever to share my PKD and transplant journey, with the hopes of educating and inspiring others and letting them know that they are not alone. In my 15 years of being active in the PKD community, I have never met another person who had their kidneys removed when they were working 60% and needed a kidney transplant because their cysts would not stop bleeding or a person who needed a liver transplant because of the issues that I had with my bile ducts. I had genetic testing done and it is confirmed that I have ADPKD, PKD1 gene. To think that all of these unique manifestations occurred in the most common form of PKD shows that I can’t be the only one experiencing these life-threatening complications and underscores the vital need for my story to be shared for patients and the medical community to learn from.
My 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 my family grapple with PKD, or have died from it, I have developed a strong connection with those who endure the pain and suffering caused by PKD. I hope by learning more about my PKD journey that you will be reassured that you are not alone and that you can get through this. I believe in you!
Want to learn more about PKD? PKD Foundation has a wealth of information available through their site. Start here to find out the basics of the disease.