More than a decade ago, a multidisciplinary team at BC Children's Hospital (BCCH) set out to create a novel clinical pathway to support the care of children with nephrotic syndrome across the province. The pathway's success has led to local and international recognition, as well as inspired the development of additional pathways to support the care of children with other pediatric nephrology conditions.
Nephrotic syndrome is a condition where excessive protein (albumin) leaks into the urine leading to symptoms such as significant weight gain and swelling (edema) of the legs, hands and face. While the exact cause is unknown, the condition is treatable with corticosteroids such as prednisone. Many children experience relapses throughout childhood, requiring repeated courses of steroids and other immunosuppressive medications with potential side effects. In BC, about 200 affected children are dispersed across the province, which is challenging as the only pediatric nephrology centre is in Vancouver.
Dr. Cherry Mammen is a nephrologist at BCCH who helped develop the nephrotic syndrome pathway. He notes that while BCCH is the primary location where these patients are treated, they are still diagnosed and receive care for some of their complications at different locations across the province, for example from pediatricians. It therefore made sense to create a clinical pathway to support pediatricians and other care providers, facilitating more consistent and high-quality care for these children.
He says this first involved a deep dive into medical records spanning nearly two decades, to understand how pediatric nephrotic syndrome was being treated across the province. “We were actually shocked in terms of how much variation there was in treatment, for example with initial prednisone doses that were being used," says Mammen.
Much of the pathway development was supported by clinical research coordinator Marisa Catapang. She, Mammen, and other members of the nephrotic syndrome pathway working group ended up meeting weekly for years, poring over literature, developing and refining their recommendations, and engaging with pediatricians and other interest-holders to promote the pathway.
In addition to developing a pathway for clinicians to follow, they created a patient-facing toolkit to support families of children with nephrotic syndrome managing the disease at home. Families are responsible for monitoring urine for protein levels and checking weight daily for swelling, reducing salt and fluid intake, and ensuring daily doses of prednisone during the child's illness. “It's a lot of responsibility, so we wanted to empower them to be able to care for and monitor their child at home comfortably and consistently, with tools developed from the clinical pathway team," explains Mammen.
In 2024, the research group published a study in the Canadian Journal of Kidney Health and Disease showing how the clinical pathway has helped standardize care of children with nephrotic syndrome across BC, regardless of location. The pathway has been highlighted in numerous additional papers and presentations, and has gained the interest of clinicians outside of Canada. In 2018, the team won the BC Patient Safety & Quality Council's (BCPSQC) Quality Award.
While the pathway initially focused on the straightforward aspects of treating nephrotic syndrome, like initial prednisone doses, Catapang says they are now adding evidence to the pathway on how to treat more complex occurrences with the disease, for example frequently relapsing cases and cases more resistant to therapy.
Notably, the nephrotic syndrome pathway has inspired other groups at BCCH to develop pathways for additional conditions, such as childhood hypertension and congenital anomalies of the kidney and urinary tract.
“The lessons that we learned from building the original nephrotic syndrome pathway are now influencing how we're developing the other ones," Catapang says, noting that this also means heavier involvement of patient partners in developing and updating the pathways. “Building on the experiences that we had with this first time around, we're hoping to make the next subsequent pathways and future iterations of this one richer, more inclusive, more relevant for the users."
Access the Nephrotic Clinical Pathway here: https://www.bcchildrens.ca/clinics-services/nephrology
Study: Outcomes Using a Standardized Provincial Childhood Nephrotic Syndrome Clinical Pathway
