Standing Up to Hereditary Kidney Disease with Home Dialysis: Rapa’s Story

Rapa, now in his thirties, began his kidney disease journey when he was born with Alport Syndrome (AS). AS is a rare, inherited, progressive disease that damages blood vessels in the glomeruli of the kidneys. The condition, that his mother also had, can lead to kidney disease and eventual end-stage kidney disease (ESKD; also known as end-stage renal disease, or ESRD), along with hearing loss and eye abnormalities. Rapa, who lives with his wife Hadassah in the Los Angeles area, is affected by all of these complications and wears contact lenses and hearing aids. He has had a challenging journey with his health, having experienced all types of dialysis, vascular access complications, serious infections and three failed kidney transplants. Six months ago, Rapa’s life took a much more positive turn when he, and his wife as his care partner, began managing his own home dialysis treatment, with the Tablo ^® Hemodialysis System. The couple hopes that by sharing their story they can raise awareness among the kidney disease community of the flexibility, autonomy and other benefits of at-home treatment.

A Brave Journey Started in Childhood

A renal biopsy at age nine confirmed his AS diagnosis, and Rapa was no longer able to play sports or do other physical activities he enjoyed. At 13, his kidneys failed and he had an abdominal catheter placed for peritoneal dialysis (PD).

“I took my kidney failure diagnosis very hard, as my mother went through PD and hemodialysis. But she encouraged me to manage my own home dialysis, and those experiences as a child really prepared me to take care of my own dialysis now,” he says.

He then qualified for and received his first kidney transplant, but the organ only lasted about a year. When it failed, he ended up in a brief coma in the hospital. He still remembers being shocked at the catheter in his chest, and seeing his blood flow through it during his hemodialysis.

Around his 15th birthday, he had an arteriovenous (AV) fistula surgically placed on his left wrist, but it didn’t mature and he had one placed on his upper left arm. That access was also problematic, so he had an AV graft placed in the same arm, that luckily worked well. He was receiving hemodialysis three days a week at a center. He remembers the center as a cold, unpleasant experience. “I was just a kid, I couldn’t do the things I wanted to do and felt like there was no way out,” he says.

Then in 2005, when he was 17, Rapa’s mother passed away from a brain aneurysm. He went through a difficult time after this, and as he managed his own health challenges. Being very self-reliant, he wanted to prove that he could succeed and have a high-quality life despite being an AS and dialysis patient.

When he was 18, Rapa had a second kidney transplant, but his body rejected the organ after only a few months. He went back in-center, and at age 19, had another graft placed in his arm. Later, a third graft developed a life-threatening staph infection.

At that point, he reluctantly had a graft placed in his right thigh. This was a defeating moment for Rapa as his mother had also been on dialysis and had a graft in her thigh, and it was a milestone he swore he would never reach. Despite this setback, he was determined to push forward as he had done in the face of many other challenges in his young life.

His third and final kidney transplant, in December 2019, soon after he and his wife were married, was hard-fought by his nephrologist due to the failure of his previous two transplants. He was ultimately approved for surgery, but doctors discovered that he had anti−glomerular basement membrane (anti-GBM) glomerulonephritis, a rare immunological complication of AS that occurs in 5% or fewer of kidney transplant recipients. Despite extensive plasmapheresis, intravenous immunoglobulin treatment and other graft-saving measures, the implanted organ failed.

A Turning Point

The third transplant failure was a milestone for Rapa, as he realized that he would be on dialysis for the rest of his life. He dedicated himself to taking care of his health, and to helping others. He became involved in the Alport Syndrome Foundation, Dialysis Patient Citizens and the National Kidney Foundation, where he met patients going through similar struggles as his own. He became and still is an active patient advocate.