Voices of PKD: Wilma Haas, Edmonton AB
I was diagnosed with PKD in 1989 when pregnant with my first child (the disease was diagnosed on my first pre-natal ultrasound). My only prior symptom was slightly elevated blood pressure. I had never heard of the disease, and on learning that it is a hereditary disease, I researched its’ prevalence within my family. I come from a large family, as did my father, so I assumed that I would find other PKD incidences within my family: I was wrong. No one in my immediate family or the families of either of my parents have/had a history or diagnosis of PKD. My nephrologist's conclusion is that my PKD was the result of mutation.
After my diagnosis I was monitored regularly by an Edmonton nephrologist (who practices as part of the Renal Clinic at the University of Alberta Hospital). My kidney function remained 'reasonable' for many, many years, and other than being advised to remain on a low sodium diet and agree to take medication to control my blood pressure, no other intervention was advised.
In approximately 2018, my kidney function/eGFR began to seriously decline. I was referred to the Chronic Kidney Clinic (also at the UofA Hospital) and attended a number of information sessions to discuss options for the onset of renal failure. Dialysis was discussed (and the need for preparatory surgeries) as well as transplant and comfort care options. In discussions with my family, I opted for living donor transplant. There are some advantages to coming from a large family!
My husband volunteered to by my living donor. His assessment process began in late 2018 (when my eGFR was approximately 13). My assessment process took eight months. My husband's took longer (about 10 months). My husband was found to be a strong match. The transplant was done pre-emptively in January 2020.
My husband recovered relatively well from surgery. My path was bumpier. I was diagnosed as being in 'early rejection' approximately two months after transplant. This required high dosage IV steroid injections, which unfortunately coincided with (caused?) the onset of glaucoma. I also developed a large lymphocele (fluid collection) beside my new kidney, which raised concerns about potential further rejection episodes. The lymphocele was drained in the hospital twice, yet recurred a third time. My transplant team (transplant surgeon and transplant nephrologist) recommended I undergo a second surgery to correct the cause of the fluid build¬up. That surgery was completed four months after the transplant - in May 2020.
I have recently celebrated my two year "kidney-versary". My kidney function is currently stable and I hope to celebrate many, many more such anniversaries.
My Hope for my Family and the Future of PKD
My sincerest hope for my family is that they be PKD free or if my children should have inherited the disease, that they, like me, not experience significant kidney failure until later in life. I also hope that I will have many, many more years of good health and that I will continue to be a meaningful presence in the lives of my children.
My hope for PKD is, of course, that scientists and researchers identify successful 'cures' for this wretched disease, rather than having to rely on mere treatments to prevent kidney failure, early death and the myriad difficulties associated with having PKD. Failing the discovery of a cure in the near future, my hope is for further improvements in the treatment of PKD. By 'improvements’ I mean less invasive and time-consuming treatments, medication (e.g., immunosuppression drugs, Tolvaptan) regimes with fewer undesirable side effects and new disease risks (e.g., diabetes, virally-related cancers, eye disorders, etc.). Having a sufficient supply of willing (and healthy) organ donors, transplant surgeons and nephrologists are also items on my 'wish list'.