In this post, Xan Nowakowski shares and reflects on a new article they’ve published concerning their experiences navigating cystic fibrosis (CF) and complications related to kidney disease and healthcare delivery. 

People with the same chronic disease often have very different experiences. When you live with a condition that affects your whole body, you can be different from other people with your condition in a huge variety of ways. A disease that impacts a particular organ or system badly in one person may leave it relatively untouched in another. As a result, it can be hard for people without that condition—and sometimes even people who live with it themselves—to understand the full scope of experiences that are possible for patients.

Yet this understanding is critical to developing and improving health care resources, both in clinical settings and in the general community, for people with chronic conditions. My own experiences as a medical educator and health care advocate have shown me this important truth almost constantly over the years. Likewise, my own journey with cystic fibrosis (CF) has demonstrated where a lot of the gaps in health care lie—and how they could be improved by amplifying the voices of patients with diverse experiences.

CF can be a tricky disease to describe because it affects almost every part of the body in some way. The basic gist of this condition is that instead of making a thin liquid that lubricates your tissue, your body instead makes something more like rubber cement. This happens because you either can’t make, can’t transport, or can’t use a specific protein that helps electrolytes move into and out of your cells. Because so many different tissues in the body require mucus to stay healthy, CF can do a lot of damage to different organs over time.

Treatment for CF has improved a lot in recent decades. As a result, many people with the disease are now living long lives. However, as of right now there is no cure and no reasonable promise of one, meaning the disease still does a lot of damage as people grow older. Health care providers are thus seeing many more CF patients surviving to develop complications like kidney disease, which used to occur in only a small fraction of people with the condition because respiratory failure ended their lives first.

As someone who has lived with CF related kidney disease since my earliest adult years, I have learned a lot about what clinicians do and don’t know concerning my health care needs. I’ve also met other people with CF who didn’t know the disease could impact the kidneys! Like any other adult with CF, I have a unique cluster of complications that don’t match up exactly with those of most other patients. Unlike some adults with the disease, I don’t have clinical diabetes or liver problems. On the other hand, I have kidney disease and heart issues.

Working with clinicians and advocates who really understand the diversity of the CF population has opened my eyes to important opportunities for improving care for people with complex health conditions. When I got connected with a specialized CF team after moving to Orlando, I quickly learned a lot about how to keep my kidneys healthy and improve some of the symptoms I was experiencing. Over time, I began to feel much better physically and more empowered cognitively about managing my kidney issues. Because of this, I also changed my thinking about whether or not I would be willing to consider a kidney transplant in the future.

I wrote about the lessons I learned from this journey in a manuscript called “Original Parts: Aging and Reckoning with Cystic Fibrosis Related Kidney Disease”. Last week, this paper was published in Patient Experience Journal. It’s a good example of how “writing where it hurts” can enrich academic literature as surely as it can expand general knowledge about improving care for people with health challenges. Likewise, open access journals like PXJ play an important role in making new information about patient experiences available far beyond university campuses!

I feel excited about amplifying the voices of other people aging with CF using some of these new tools for writing, sharing, and learning. If you’d like to read the manuscript, it’s available here free of charge. As usual, let us know if you have any problems accessing the text—we will happily send you a PDF copy instead.