Shared decision-making and goals of care setting in a child with progressive renal failure caused by primary hyperoxaluria
Abstract
We present the case of a patient born in the 29th week of pregnancy with primary hyperoxaluria type 1, a rare hereditary metabolic disease, in which the absence or insufficient function of the liver enzyme alanine-glyoxylate aminotransferase (AGT) leads to the accumulation of calcium oxalate in the kidneys and, in advanced decline in kidney function, in other tissues. In the first months of life, the girl developed terminal kidney failure even before the administration of lumasiran, which reduces the formation of oxalates based on the principle of RNA interference.
The rapidity of the progression of renal failure led the primary pediatric team to decide to start peritoneal dialysis and, after achieving optimal weight, to plan a kidney transplant with simultaneous long-term treatment with lumasiran, or a dual liver and kidney transplant, as very risky but potentially curative treatment methods. The child's parents expressed disagreement with the proposed procedure, and the primary pediatric team indicated the intervention of the support and palliative team with the aim of: 1. clarifying the parents' understanding and prognostic awareness, 2. verifying the consistency of the parents' priorities and values, on the basis of which they expressed their disagreement, 3. supporting shared decision-making about a reasonable goal of care, taking into account both treatment alternatives, including their benefits and risks, as well as the values and preferences of the child's parents.
The case report provides basic information about the disease and treatment alternatives and focuses on the description of the shared decision-making process, which results in the agreement of the treatment team with the parents on a meaningful and realistic goal of care, and a plan for its fulfillment.