Virginia’s mother, Rose
The story begins probably generations ago but what we know is that my Mom’s father died from complications of kidney failure about 85 years ago. PKD was not mentioned, but later was assumed to be the cause. The disease passed on to three of his four children – my mother, Rose, and her two sisters, Rita and Gloria. My mother began having issues with the disease in her late 40’s and was told she may only have 6 months to live. She lived for 7 years, to the age of 52, but the devastating news and hemodialysis treatments sent her into a deep depression which lasted the rest of her life.
Unfortunately, she was not a candidate for a transplant. My Aunt Rita died at age 60 from a lung disease but an autopsy showed she also had PKD. My Aunt Gloria’s kidney function didn’t decline until she was in her late 60’s when she was put on hemodialysis and then peritoneal dialysis for about 10 years until she died from the disease. A widow with no children, she lived with and then near my husband, David, and I for the last few years of her life so that we could help her manage her illness. During this time, my husband had successful quadruple heart bypass surgery.
My mother passed the disease down to me and to one of my three brothers. In 1968 my brother Michael was a senior in high school and on his school football team. During football practice, he was tackled and doubled over in excruciating pain. We did not know he had enlarged kidneys due to PKD and should never have played such a contact sport. We did not even know at that time that PKD was hereditary. It was an agonizing, long night, waiting to hear if Michael would survive the operation for a ruptured kidney, which was removed, but he recovered well. Michael’s remaining kidney though began to fail in his early 30’s, at which time he had a successful transplant, receiving a kidney from his brother Joe. About 20 years later, complications from the disease and transplant related medications began to take a toll on Michael. He had a heart attack in 2003 and had a heart transplant in 2008. A couple years after his heart transplant, skin cancer that he had battled for years eventually spread throughout his body and he succumbed to multiple tumors in 2011 at the age of 60.
My youngest brother Bill did not inherit PKD, but his story is one of profound heartbreak. In 2006, at the age of 50, he was diagnosed with Stage 4 Non-Hodgkin’s Lymphoma. Shortly thereafter, his 22 year-old son Joel was tragically killed in a car accident. Bill suffered through three years of treatments, trials, 2 bone marrow transplants, and the devastating death of his son, before succumbing to the disease in 2009 at the age of 53. He left behind a loving wife and young daughter.
Then there’s me and my 5 children, 4 of whom inherited PKD. In January 1992 my doctor said I could no longer hold off from starting peritoneal dialysis which I was dreading. I chose this option because I wanted to continue my job as a teacher and also have time to spend with my very young grandchildren. Nights were challenging at times, but my days were free.
In June of 1992 I had an emergency hysterectomy and had several blood transfusions. l also had to do hemodialysis for a few weeks until I healed from the surgery. While in the hospital, I received a call from Albany Medical Center offering me a kidney from a cadaver, but because I had lost so much blood my nephrologist said it was too dangerous for me to undergo the transplant. My next offer for a kidney came in January of 1993 and I had a successful transplant. I only had one setback a couple of months later which put me back in the hospital again in Albany. I was running extremely high temperatures. This was terrifying for me as I was afraid I would lose my new kidney. Thank God I did recover and have had no issues with my kidney since. This month I will celebrate my 27th anniversary of the kidney transplant!
Early in 1999 I was stunned to find out I had developed portal hypertension with esophageal varices. I knew I had cysts on my liver as well as on my kidneys but also knew the cysts don’t affect the functioning of the liver like they do the kidney. I was told I could bleed out if I didn’t have a liver transplant ASAP. I was moved up the transplant list pretty quickly and had banding done 3 times while waiting for the fateful call. I retired from teaching that July and in November 1999 I received a liver at UPMC Pittsburgh, sadly from a 13-year-old girl who died while waiting for a heart transplant. My surgery lasted 12 hours as my liver had grown very large and was complex to remove. All has gone very well with my liver transplant and I just celebrated my 20 year anniversary. I attribute my remarkable success to God’s grace and to my determination to be positive and remain active, walking almost daily, attending yoga classes several times a week, and playing tennis on a regular basis.
Three of my four children with PKD have already had successful living donor kidney transplants. In 2006, my oldest daughter Katherine gave a kidney to her sister Margaret (age 40 at the time). In 2007, my son Brent (age 34 at the time) received a kidney from a dear friend. In 2017, my son Frank (age 50 at the time) received a kidney from his ex-brother-in-law! All three are doing very well so far.
But in the summer of 2017, my family endured its greatest heartbreak related to PKD. My daughter Linda, who has PKD, but whose kidneys were still functioning well at the age of 49, suffered a ruptured brain aneurysm. She was alone when it occurred and was found nearly lifeless, several hours later, by her 18 year-old son. After emergency surgery to save her life, she suffered multiple strokes, killing large swaths of her brain tissue. Knowing that aneurysms are a risk with PKD, Linda had an MRA 10 years prior which showed no sign of aneurysm. She was told by her doctor at the time that it was not necessary to retest, leading to an unfortunate false sense of security. My son Brent has a small brain aneurysm that has been monitored and fortunately has been unchanged in size for about 15 years now.
Linda was a vibrant, active woman, worked to help others, as a counselor and social worker, and raised two amazing children. Though she has made progress following 50 days in a coma, the Linda we knew is largely gone as she has been left with severe physical and mental impairments. Fortunately, she does remember her family and friends and can communicate with us, but seeing my daughter’s mind and body impacted in this way has been the most heart-wrenching experience of my life. My life is now devoted to giving her the best care that we can. It’s amazing what a single heredity disease can do and all the implications and effects it can have on a family’s history. My family has experienced many blessings and good fortune, but also profound heartbreak. I continue to thank God every day for our blessings and pray for a miracle for Linda. lf you or your loved ones have PKD, and have not been screened for a brain aneurysm, please take my advice and consider doing so.
Saving lives together
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