Voices of PKD: Wendy Marshall-Fagan, Moncton NB
PKD was not spoken of when I was a child. My father inherited the disease from his father (my grandfather) who died before I was born, and no one else in the family had it.
I have been in touch, in recent years, with extended family on my father’s side, and they say that my grandmother mentioned in letters that my dad had the “Marshall Disease”.
Apparently, family members on my father’s side have been doling out advice for generations to “drink lots of water to keep the ‘Marshall Disease’ at bay.” They had no idea the Marshall Disease was polycystic kidney disease.
My dad knew he had it though, as my mother’s father was a physician, and insisted on having my dad tested before he would allow my mother to marry him, so that she could make an informed decision on marriage and future family. Obviously, it didn’t stop them.
My dad was 22 years old when he married my mom in 1963, so he knew he had it early on. I knew growing up that my dad was on several medications, including medication for high blood pressure, but I don’t remember exactly when I found out that my dad had PKD.
He reached end-stage renal failure and started dialysis when I was in grade 12, and at that point I became aware that there was a possibility that I may have inherited the disease from him.
I went and had myself diagnosed, against my fathers wishes, at the age of 18. I’m now 57.
He forbade me from getting myself tested, as he was concerned about the impact on my future - specifically he was worried I would not qualify for life insurance. The first chance I had, I went and had myself tested. I had to know.
In keeping with that rebellious streak, I also had 3 children against my nephrologist’s advice – at ages 29, 30, and 32 (the last was a surprise).
I’ve never kept my PKD a secret from my kids. I’ve always been as honest and open with them about it as possible. Like my dad though, I advised my children to delay testing for as long as possible - or at least until they get their adult lives in order - but to keep an eye on their blood pressure.
One of my three has had themselves tested (I’m not mad about it) and at this time, it looks like she is clear. My other two are content not knowing for now. I had children because I really wanted to be a mom, more than anything in the world. And I would do it again.
The pregnancies did not seem to have a huge impact on my kidney function, and I never regretted having my children. Since then, it’s been a slow but steady decline over the years.
My predominant symptom, from childhood onwards, has been persistent nausea, particularly in the mornings - much like morning sickness (which I had 24/7 for all 9 months of all 3 pregnancies).
I had one bout of kidney stones in my 30s, and three gout afflictions before I started preventative prescription treatment last year.
Apart from being diagnosed with high blood pressure in my 20s, I feel I’ve been quite lucky to escape many of the ailments from this disease that affect so many.
I try to hide all of my symptoms from my co-workers, friends, and family, and even myself, for a multitude of reasons.
I don’t want my children and family to worry about me. I don’t want to be perceived as the “weakest link” at work. And I want to delay dialysis and transplant for as long as possible.
For me, this is definitely an invisible disability. I’m still working full-time. I still look relatively healthy on the outside. I’m still active, and I still travel. People are shocked when I tell them my current kidney function is 12%.
When I was first diagnosed with PKD, I absolutely struggled with it mentally on a daily basis. But I was 18. I struggled with a lot of heavy things at that age, and through my 20s.
I wish I had something inspirational to say about how I got therapy, had a lightbulb moment, and got past it all, but I don’t. Life just happened.
I didn’t get therapy. I didn’t follow a renal diet, or take any precautions at all really. I just put one foot in front of the other, got married, had babies, stopped focusing on the disease, and started to focus on the living.
I’ve been on Jinarc (Tolvaptan) for several years now (I was patient #1 for this drug in New Brunswick!), and I am sure it has helped to slow the progression of the disease.
I’ve had no issues at all with Jinarc. To be honest, for the first couple of years that I was on the drug, I frequently forgot to take my evening dose. More often than not, in fact.
Eventually I was prescribed another medication (for a different problem) that had to be taken in the evening as well. Once I paired the two together and incorporated it into my routine, I never forgot again. But it took a few years.
I’ve never contemplated stopping Jinarc. The only side effect I’ve had from it is having to get up several times in the night to use the bathroom, but the frequency of those nighttime trips seems to be diminishing with my kidney function. Five years ago, I was getting up 3-4 times per night to void. Now it’s always once, and sometimes twice.
Unfortunately, I am now solidly in Stage 5 renal failure, with a current kidney function of 12%. I am waiting for surgery to have my peritoneal catheter placed, and I expect to be on dialysis by Spring 2024. Until then I am working – and will continue to work – full-time as an Education Assistant in a public elementary school in Moncton, NB.
The only reason it is important to me to keep working right now is to pay the bills. I’ve been a single mother since 2002, when my kids were 3, 5, & 6. They are all adults now, but I haven’t had the luxury of a dual income in more than 20 years. I have no savings.
I love my job, but if I could afford to stop working now, I absolutely would. I work at a very physically demanding job. I am sick every single morning of my life, and I am chronically fatigued. I wish I had the choice to stop working. I would retire tomorrow.
I have outlived every known relative who has had this disease before me (my dad passed at 49, my grandfather at 54, and my great grandmother at 40) so I feel absolutely blessed for each and every day that I remain on this earth.
I wish I could say that I am optimistic about my future, but I watched my dad struggle through two transplants which both failed, and ultimately witnessed his premature death in 1990. And while I know that there have been improvements in science and medicine over the past 33 years, I just have this feeling… So I am delaying dialysis as long as my nephrology team will let me, and I’m working on getting my life in order now, while I’m still pretty mobile.
I just try to live one day at a time. I try to live in the moment, and do as much as I can while I can.
I love to travel, and I’ve done a bit over the years. My best friend and I went on an Alaskan cruise this past summer – my “last blast.” When I’m not working, and I have enough energy, I’m slowly going through my house which I’ve owned since 1997, trying to downsize so that my children don’t have so much to deal with when I’m gone. And I got my will done.
It’s important for me to fundraise, specifically for the PKD Foundation of Canada, because this disease has affected my family for generations. If I were to win the lottery, after I took care of my friends and family, my first charity of choice would be PKD Foundation of Canada, of course!
I believe it is especially important for those affected by the disease to do what they can to work together to find better treatments and, ultimately, a cure. I do it for myself, my children, my grand children-to-be, and on down the line.
I remember my dad used to sell peanuts for the Kidney Foundation of Canada back in the 1980’s. I, myself, have fundraised and participated in the Moncton Kidney Walk in 2017 & 2018, the Montreal Walk to END PKD in 2019, and the Halifax Walk to END PKD in 2022 & 2023.
Thank you again for taking the time to listen to my story.
I would love to meet others in my area with the same disease - it would be nice to have a local community of support outside of my nephrologist’s office. As you can imagine, my diet is now quite restricted. I would love a place to share recipe ideas and make new friends who can relate to my journey.