Is There a Light at the End of the Dark Tunnel of Kidney Disease in Children?

This month at Black Health Matters, we are focusing on kidney disease and kidney health. As part of our Black Health Matters Fall Summit 2020, we welcomed Dr. Rasheed Gbadegesin, professor of pediatrics, Division of Nephrology at Duke University.  

Dr. Gbadegesin (pronounced bah-deh-GEH-shin) is the co-director of the Duke CTSA KL2 program and the program director of the Duke Pediatric Research Scholar program. He was elected into the American Society for Clinical Investigation in 2016. Dr. Gbadegesin is also an investigator at the Duke Molecular Physiology Institute speaking about kidney disease, specifically FSGS or focal segmental glomerulosclerosis.  

In the last 10 years, Dr. Gbadegesin and his colleagues have identified at least five genetic causes of steroid resistant FSGS and other kidney diseases. He chatted with Black Health Matters about this disease in children.

BHM: What exactly is FSGS?

Dr. Gbadegesin: Focal segmental glomerulosclerosis or FSGS is a kidney condition that causes progressive scarring of the kidney filters, leading to permanent kidney damage requiring dialysis and kidney transplantation. FSGS manifests as leaking of protein in the urine, low blood protein (known as albumin), body swelling, elevated cholesterol and progressive kidney damage.

BHM: Is it linked to a higher rate of childhood mortality or adult mortality? Or any long-lasting physical effects?

Dr. Gbadegesin: It is a chronic and debilitating condition that has significant effects on the health of affected individuals. It is particularly severe in children because it can have long-term impact on their growth and development. It is associated with higher mortality in both children and adults, and it is a leading cause of kidney failure among people of African ancestry.

BHM: Are there any treatments? What are they, and what are any possible side effects?

Dr. Gbadegesin: There is currently no approved treatment. We use a combination of immune suppression medications, such as steroids and medications that lower protein in the urine. A combination of these medications will be effective in only 30 percent of affected individuals. None of these medications are specifically designed to treat FSGS, and they are associated with major side effects.

BHM: How will this affect (if at all) a child’s physical, intellectual or social development?

Dr. Gbadegesin: This is a progressive disease; therefore, it has a long-lasting effect on the physical and psychosocial development of a child.

BHM: Will this place any restrictions on their activities?

Dr. Gbadegesin: Yes, if they have uncontrolled body swelling. Also, with progressive kidney damage, they get tired easily.

BHM: Should a parent alter their family’s diet to help treat or manage this? 

Dr. Gbadegesin: With progressive kidney damage, the body cannot handle water, salt, high protein load and other minerals effectively, so you will need to discuss with your child’s kidney doctor, who will work collaboratively with a kidney nutritionist to come up with plans. Also, when the child is swollen, you may have to restrict water and salt intake.

BHM: What are some resources you might have to help a family manage this diagnosis, including lists of suggested foods, activities or dietary supplements?

Dr. Gbadegesin: There are many resources available, but your best source of information is your child’s kidney doctor and the members of the multidisciplinary team, including dietitians, social workers, psychologists, child life specialists and nurse practitioners. 

BHM: Are there any organizations that would help a child meet peers who are also suffering from this or that might help put families in touch with other parents with a child who has this—or a similar—diagnosis?

Dr. Gbadegesin: Yes, there are multiple organizations out there. A leading organization that has been in the forefront of raising awareness and finding a cure for this condition is Nephcure Kidney International.

BHM: What is the long-term prognosis for children who are diagnosed with this? Can a child with FSGS still be able to live a long, healthy, “normal” life? 

Dr. Gbadegesin: The most important prognostic factor is response to all the medications we currently use in the treatment of the disease. If your child responds to this medication and enters complete remission, the long-term outlook for normal kidney function is very good. 

Unfortunately, only about 30 percent of children will be responsive to therapy. In those that are unresponsive, a majority will develop permanent progressive kidney scarring leading to dialysis and kidney transplantation within five to 10 years of diagnosis. 

Even after kidney transplantation, about 40 percent of children may have recurrence of the disease in their new kidney. The most important goal in therapy is to form a close therapeutic alliance with your child’s provider to work towards finding an effective treatment that will slow the rate of progression of the disease.  Also, there are multiple ongoing research studies trying to understand the disease better and find new therapies. So, there is light at the end of the dark tunnel.