Dr. Rheault, MD, is a pediatric nephrologist and pediatric dialysis medical director at the University of Minnesota Masonic Children’s Hospital. She is also a #NephJC workgroup member and clinical researcher with interests in Alport syndrome and nephrotic syndrome. Follow her @rheault_m
“But, tell me doctor, what is this going to MEAN for their life?” asks the parent of a child about to start dialysis for end stage renal disease (ESRD).
For the parent of an infant, they want to know how dialysis will affect their baby’s ability to walk and talk and be successful in school. For the parent of a teenager, they worry that the already difficult teen years will suddenly get more terrifying as their adolescent adjusts to the rigors of dialysis and transplant.
Which of our contenders in the Dialysis Region has the edge in this matchup?
I’m going to give it away: No one’s a winner when children have ESRD.
Pediatric nephrologists have come a long way in the past 30 years in improving outcomes for our youngest dialysis patients. In a recent ERA/EDTA publication, the risk of death for children starting dialysis at <1 year of age was 10% at 1 year and 16.1% at 5 years with similar findings in a US cohort. But what effect does chronic renal failure have on the developing brain?
In children, we talk about delays in meeting expected developmental milestones instead of decline in neurocognition. Delays in all domains of development have been observed in children with chronic kidney disease (CKD) and ESRD including fine motor, gross motor, and cognitive function, although delays are by no means universal. In the Chronic Kidney Disease in Children (CKiD) study of somewhat older children with mild to moderate CKD, 21% to 40% of participants scored at least one SD below the mean on measures of intelligence quotient, academic achievement, attention regulation, or executive functioning although neurocognitive functioning was within the average range for the entire group. Neurodevelopmental sequelae of CKD are likely improving over the past 30 years with early aggressive medical intervention with nutrition, physical and occupational therapy, and early transplantation. The good news is that transplantation leads to improvements in neurocognitive measures in children with ESRD.
How can we maximize normal cognitive and motor development in children with CKD and ESRD? The first goal is to recognize that developmental delays are a risk and to refer children for formal assessments. Once a baseline status is known, deficits can be addressed through referral to the local birth to three program, development of an individualized education plan, referral for physical or occupational therapies, or other appropriate interventions. And of course, transplantation as soon as possible is also vital.
Patient-Reported Outcomes and quality of life in children with CKD and ESRD
Imagine you’re 15 years old and you are on chronic dialysis with a central venous catheter. You get invited to your best friend’s birthday party at the local trampoline park where the planned menu includes pizza and soda. Do you skip the party because the food doesn’t fit into your renal diet and you can’t jump on the trampolines anyway with your catheter in place? Do you go and watch everyone have fun without you? Is there anything your dialysis team could have done to help you with this? Did they talk to you about what your goals are for living on dialysis? Why do they care so much about PTH? You’ve never even heard of that term before.
The effect of ESRD on quality of life has been well described in adults requiring dialysis. The quality of life areas that are most often adversely impacted in adults with ESRD include cognitive dysfunction, depression, anxiety, pain, sleep disturbance, reduced physical functioning, sexual dysfunction, and reduced social interaction.
Children with ESRD requiring dialysis also demonstrate lower quality of life scores in all domains that improve after transplantation. A number of disease- and treatment-specific factors may contribute to lower quality of life scores, including dietary and fluid restrictions, medications, and missed school days because of medical appointments. Lower emotional functioning scores have been found in children on dialysis for longer periods of time and in females. Interestingly, parental ratings of quality of life for children on dialysis are significantly worse than patient reports. Children with ESRD report similar quality of life scores as children with newly-diagnosed cancer receiving chemotherapy and radiation therapy, reinforcing the significant effect of ESRD on everyday functioning.
The effect of milder degrees of kidney dysfunction on childhood quality of life is less well studied. The CKiD study was one of the first studies to examine quality of life in children with mild to moderate chronic kidney disease of all causes. Children with CKD had lower scores in all domains compared to healthy youth. Longer disease duration and older age were associated with higher PedsQL scores, one reproducible measure of quality of life in children. Interestingly, GFR did not correlate with PedsQL scores; however, this study did not include children requiring renal replacement therapy.
How can we improve the quality of life for children with CKD and ESRD?
We can start by really talking to our patients and families and understanding their goals. For some children and adolescents, it may be going to dialysis in the afternoons so they can make it to their favorite technology class scheduled in the mornings. For others, it may mean being able to “cheat” on their renal diet on occasion when they have large family or cultural celebrations that center around food. For still others, it may mean improving their stamina so they don’t have to use a wheelchair at school. If we don’t ask, we don’t know what’s important to them.
As in adults, the SONG-Kids (standardized outcomes in nephrology) initiative was formed to identify a standard set of outcomes in clinical trials of CKD and ESRD that are relevant and meaningful to children and families as well as providers. This is a welcome step toward improving the care of children with kidney disease with their wishes firmly in mind.
Which team has the edge for this year’s NephMadness? For children, it’s really a toss-up. Both are vitally important and have significant room for improvements in our current practice.
– Guest Post written by Michelle Rheault (@rheault_m)
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