Self-education is vital

Tess Harris, a British citizen and CEO of Polycystic Kidney Disease Charity, was diagnosed with ADPKD (autosomal dominant polycystic kidney disease) over 40 years ago. She recounts her battle with the disease and how self-education helped her overcome the challenges she faced.

What was your diagnosis, and can you briefly describe the treatment you have received?

I was diagnosed with ADPKD (autosomal dominant polycystic kidney disease) over 40 years ago. I inherited ADPKD along with three siblings from my father, who inherited it from his mother. ADPKD is the world’s most common inherited kidney disease; it is lifelong and incurable. My father and one of my sisters have since died from ADPKD complications. I was treated for symptoms for many years: initially blood pressure medication for hypertension, and in 1994 I had my first serious complication, a ruptured infected kidney cyst. My kidney function deteriorated, and in 2019 I started peritoneal dialysis. I was fortunate to receive a transplant from a deceased organ donor in 2020

What have been the most challenging aspects of being diagnosed and living with kidney disease?

On diagnosis, I went to a medical bookshop (this was pre-internet) and read that ADPKD was incurable, and the average age of death was 57. I decided that I would get on and enjoy life because it was likely to be shorter than expected. I had a very varied and stimulating business career and volunteered for the PKD Charity in 2005. I live in the UK and have free access to treatments and care (other than small charges for prescriptions). I worked mostly for myself or under contract for companies, so I never had an issue with taking time off ‘work’ for hospital appointments. The biggest challenge came when I had to start dialysis. I chose CAPD [Editor’s note: Continuous Ambulatory Peritoneal Dialysis] because I wanted more freedom to travel, and it suited me because I work mostly from home.

How do/did you manage or cope with these challenges?

I practice Stoicism which enables me to focus on the things I can influence and ignore those I can’t. I’ve grown to accept, even embrace my fate. Life is short, and I intend to live every day the best I can. I enjoy my work, especially supporting others with PKD and being involved with research.

What are the most important learnings in your journey with Fabry disease?

Self-education is vital. I’ve sought information from reliable sources over the years, and I am extremely knowledgeable.
It gives me confidence in making decisions. I continue to read, attend conferences and learn daily about ADPKD.

Over the years, well-meaning friends and others have offered ‘advice’ which I’ve always accepted politely and then dismissed. My go-to sources for evidence are always PubMed and official guidelines. I have a wide social and professional network but am equally happy in my own company. I have a strong interest in politics and advocate where I can for greater equality in society.

What do you think are the more important things that can help patients to live well with kidney disease?

Always ask questions, e.g., What are my treatment options? Benefits and harms? How can I get more information to help me decide? Rely on evidence-based information, not ‘Dr. Google’. Eat healthily, cut out processed foods, read the food labels, reduce salt (everyone eats too much of it), exercise, don’t smoke, take your medications. Try to live the best life you can.

What sorts of things would you suggest helping patients become better empowered and be in more control of their health/life?

Healthcare Professionals (HPCs) should give or signpost patients to reliable information sources and local support groups. Patients get huge benefits from talking with others with similar experiences. Check the sources of information that you come across.

What are your hopes and fears for the future concerning your disease and your life in general? Are you hopeful that you will be okay?

Developing treatments to slow or prevent ADPKD progression is frustratingly slow. There is only one licensed therapy, and there have been three failed clinical trials in the past ten years. I am beyond those types of treatments, and I am now focused on hanging onto my new kidney, by being the best, compliant patient I can be. I know that the odds are that my life will be shorter than ‘healthy’ people, but that doesn’t bother me. On a positive note, there are many more researchers and doctors studying ADPKD than when I was diagnosed, and I’m sure that – one day – innovative treatments will be available. It is good news for future generations.

What is something you wish people knew about living with kidney disease?

The general public is grossly ignorant about their kidneys and doesn’t know the risks of chronic kidney disease. Kidney disease can be silent to the outsider. Like many people with CKD, I’ve never looked ‘unwell’. Owing to my lifelong focus on keeping as healthy as possible and my Stoical disposition, I know that I look and feel younger than many of my age. But not looking unwell, can disguise the struggles that many patients experience daily. Moreover, many people believe that dialysis and transplant are a ‘cure’. They don’t understand the burden of treatment, drugs/side effects, impact on heart, bones, etc.

How did the recent COVID-pandemic change your experience with Fabry disease? 

I was suspended from the transplant list for six months, but otherwise, it hasn’t changed my experience greatly. Whilst on PD during COVID, I was shielding at home and felt safe. I didn’t miss going out or traveling because we were all in the same situation across the world. I’ve had excellent, safe care during my transplant and after.