In 2009, a study on renal involvement in cystic fibrosis (CF) patients found that “although rare, clinically significant renal disease may arise in young adult patients with CF.”
Since that time, the median life expectancy for CF patients nearly tripled — from 26 years in 2008 to 66 years today. As more CF patients live into their 30s and beyond, more are developing renal disease.
In order to improve not just the quantity but also the quality of CF patients’ lives, renal conditions need to be caught early and treated preemptively.
Agnieszka (Agnes) Swiatecka-Urban, MD, is a pediatric nephrologist physician-scientist at UVA Health Children’s. Part of the Neonatal Kidney Collaborative, her research focuses on improving outcomes for patients with chronic conditions that can lead to kidney disease or failure — like patients facing cystic fibrosis. This is accomplished by early testing, before symptoms develop. It also involves thinking critically about what's causing renal conditions to develop.
Detecting Kidney Disease Sooner
With cystic fibrosis, early on, pulmonary health requires the greatest share of attention. That makes it easy to miss things like early warning signs of kidney disease. “It’s often recognized only after advanced damage has occurred, and the causes are poorly understood,” Urban says.
Urban’s multi-primary investigator research is working to find better ways to detect kidney disease earlier in patients with cystic fibrosis. Within UVA Health, the team is a collaborative interdisciplinary panel of experts bringing a mix of specialties only found in large academic medical centers. Not only can adult and pediatric CF specialists offer insight, but also biostatistics experts.
“Our investigations use cutting-edge tools and the leading animal models to identify targetable mechanisms of CF kidney injury and develop focused protective strategies,” Urban explains.
Intervening Before Symptoms Occur
Urban’s study includes the novel hypothesis that CF-causing mutations also affect kidneys. These changes could “reduce their ability to respond to stress caused by systemic inflammation associated with CF, medications, dehydration, and environmental causes.”
In addition to being genetically predisposed, CF patients are treated with medications that can also damage the kidneys. And yet for many patients, their kidney disease isn’t detected until it’s advanced.
But by doing urinalysis before signs occur, those at risk can be identified. “The urine provides a ‘liquid biopsy’ window into the kidney. By analyzing the urinary protein profile, immune cell activation, and microscopic vesicles the kidneys shed, we will delineate the pathways predicting progression to chronic kidney disease,” Urban explains.
Once detected, treatment can keep kidney damage from progressing.
Life Quality & Quantity
“Until recently, respiratory failure was the primary cause of death,” Urban notes. But medical advancements mean that now, “long-term life-limiting manifestations in other organs, such as kidney disease, are more frequently recognized and merit further attention.”
Untreated kidney disease significantly impacts quality of life. But it also carries a significant risk for premature death from related heart complications. With earlier recognition and treatment, these risks can be avoided. By recognizing the disease before there are any symptoms, patient outcomes can be improved.