Spina bifida and kidney disease

Spina bifida (SB), which translates to "split spine," is a birth defect that occurs when the neural tube that later forms your spine and brain fails to close completely during fetal development. There are about 1,500 babies born with SB in the U.S. each year, and those who have it are permanently disabled.

The effects of SB on the bladder frequently result in damage to the kidneys.

Because SB affects the spine and brain, most individuals with SB have what is known as a neurogenic bladder. This condition causes bladder control problems because the brain is unable to receive signals to urinate through the spinal cord from nerves around the bladder, so the bladder is not able to store or empty urine effectively. A neurogenic bladder is a major risk factor for progressive kidney damage.

A neurogenic bladder typically results in frequent urinary tract infections and can, in more extreme cases, become a hostile bladder, which means the bladder does not work with the bladder neck to release urine.

A neurogenic bladder may result in high bladder pressure, bladder-sphincter dysfunction, and backflow of urine into the kidneys. These conditions may result in hydronephrosis (a swollen kidney due to poor urine drainage), pyelonephritis (a urinary tract infection that travels to the kidneys) and kidney scarring. Lifelong bladder management is necessary for people with SB.

Babies born with SB usually have normal kidney function at birth, but 26% of adults with SB have some kidney damage, and some later progress to kidney failure and require dialysis or a kidney transplant to survive. While diabetes is the leading cause of kidney failure in people without SB, kidney failure in people with SB is most often caused by urological (urinary tract) issues. Individuals with SB who develop kidney failure are more often younger, female, non-Hispanic white and Hispanic.

People with SB who progress to kidney failure typically have the same life expectancy on dialysis as kidney failure patients without SB, and their transplanted kidneys also last about the same amount of time. Like all people living with a kidney transplant, people with SB are able to keep their transplanted kidney healthy with a kidney-friendly eating plan specific to post-transplant living. SB patients with kidney failure, though, are less likely to receive a kidney transplant in the first place, possibly because donated organs are in such high demand and the disability status of patients with SB means they are sometimes not considered the best candidates for transplant.

Just as in adults with SB who are living with a kidney transplant, one of the most important and effective ways to protect and maintain kidney function in children with SB is to follow a kidney-friendly eating plan. Children with SB are likely to have issues with obesity, bowel health or constipation, and pressure sores due to a lack of minerals and vitamins. Following a kidney-friendly eating plan could help protect children with SB from these issues and maintain their kidney health for the long run.