When Should Dialysis Be Started in Children and Young Adults with Kidney Failure?

As a rare kidney disease patient, parent, and advocate, I appreciate the analysis of data in the article "Timing of Kidney Replacement Therapy among Children and Young Adults," published in this issue of CJASN.1 It is valuable to analyze the timing of treatment and its relationship with outcomes for patients who experience this medical trauma so early in their lives. Alport syndrome, a rare genetic kidney disease, is often most cruel during teen and young adult years. For fast progressors such as men with X-linked genetic variants or men and women with autosomal recessive variants, the average age of kidney failure is 22 years without early angiotensin-converting enzyme/angiotensin II receptor blockers intervention or tolerance.2–4 When I reflect on my son's experience, who went through kidney failure, dialysis, and transplant at age 19 years, and so many other young patients I have come to know in my role as Executive Director of Alport Syndrome Foundation, two words come to mind: life interrupted. Just when these bodies and minds are preparing for independent life, they become tethered to dialysis machines. Lives, dreams, and plans are put on hold. Facing mortality in a dialysis chair at this age has both short- and long-term effects. I applaud Larkins et al. for intentionally expanding the age range of their research to include patients up to age 25 years. The expansion likely allowed for the study to include patients with Alport syndrome and acknowledges KRT as unusual but significantly life altering as a young adult. It is not surprising that the authors found eGFR alone, at the time of KRT, to be a poor predictor of treatment success. These findings illustrate the true patient experience. There is no single magic number that should be a deciding factor to initiate KRT. My son's eGFR never officially dipped below 25 ml/min per 1.73 m2. However, when his body showed signs of failing to thrive, such as no sense of hunger, significant brain fog, muscle cramps, and extreme fatigue, I am grateful his nephrologist instructed him it was no longer safe to avoid dialysis. While my child was experiencing kidney failure, I was in touch with other parents whose teens or young adult children with Alport syndrome were facing the same journey. The reported eGFR of these patients varied significantly. One 21-year-old man, still attending college courses, sustained an eGFR of lower than 10 for several months and was able to avoid dialysis before receiving a kidney from his living donor father. In the article, the most significant source of variability in initiating KRT was the specific center where the patient received treatment. This reflects the disturbing true experience of patients. Patients are guided and treated differently by site. This was highlighted in a recent LA Times article in which patients were denied a place on the US organ transplant waiting list at one site, sometimes for years, but then approved quickly on the list at another site.5 In far too many cases, patients die while waiting. I question the classification of the four etiologies used in the study for this article. I view them as overly broad and, therefore, not insightful about the variety of ways that young kidneys fail and the relative success of different treatment methods. For example, a 5-year-old White male patient with hereditary C3 in an urban setting is going to experience a different kidney failure pattern and treatment options than a 19-year-old White male with Alport syndrome in the same setting. In this article, both are considered one etiology for analysis. I also believe the context of geographic region should be more prominently featured. An improved title might include the specificity of Australia and New Zealand. Both have nationalized health care programs and therefore access to and easier adherence with life-saving immunosuppressant medications that play a role in outcomes. In addition, the geographic region studied reports patients receiving KRT at slightly higher eGFRs as time passes: median of 7 eGFR in 1998 up to median of 8 eGFR in 2018. Although the authors do show statistical significance, from a lay perspective, these differences seem minor. The focus on pediatric KRT is valuable. However, one of the most important considerations for initiating treatment is extremely individual: the patient's goals. I encourage physicians to ask patients what their goals are before initiating life-changing KRT. It could be to finish a year of college, learn to drive, attend a dance, or take a trip. Motivation for life is key at any age. If dialysis can be avoided or delayed, or personal goals accommodated, let that be an important factor in guiding treatment. My son's goal was to experience his first semester of college before starting dialysis, and it was this taste of college life that helped motivate his recovery. It was everything.