Electrolyte Management After Kidney Transplant in Cystinosis

Nephropathic cystinosis is a rare inherited lysosomal storage disorder caused by mutations in the CTNS gene, resulting in the accumulation of cystine within lysosomes and progressive damage to multiple organ systems, particularly the kidneys. Renal Fanconi syndrome develops early in life, leading to significant urinary losses of electrolytes and nutrients, impaired growth, and eventual progression to end-stage renal disease. While kidney transplantation restores renal function, it does not cure the underlying disease, requiring lifelong nutrition considerations with shifting clinical priorities.

This case study presents the nutrition management of a 15-year-old female with nephropathic cystinosis after a living donor kidney transplant. Before transplant, nutrition interventions focused on electrolyte replacement, hydration, and support of growth and development. Before transplant, nutrition interventions focused on replacing electrolytes, maintaining hydration, and supporting growth. After transplant, the priorities shifted toward protecting the transplanted kidney, managing medication side effects, and helping the patient adapt to a completely different way of eating. Laboratory values improved, but the biggest challenge wasn’t correcting electrolytes, it was helping the patient navigate new food restrictions without making eating feel overwhelming. Using phosphate binders, involving the patient in decision-making, and preserving as much normalcy as possible created a plan that felt realistic and easier to sustain over time.

Materials Available

• Poster

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Project Type(s): MNT Concentration Clinical Poster

Author(s): Michaela Skloven

Program(s): Master of Science, RDN Training

Year: 2026

Adviser(s):

• Michelle Averill