Ryleigh’s Kidney Journey
Our beautiful daughter Ryleigh came into this world on December 30, 2014. Ryleigh's kidney journey began like many other nephrotic children. Around one year old we began noticing a pattern of swelling in her eyes and belly (edema). At 18 months old, after being misdiagnosed as having allergies, her edema eventually became so severe that we did our own research and demanded a test to check for protein levels in her urine. This is the marker for Nephrotic Syndrome, a condition where the filters in the kidneys leak protein and other nutrients into the urine in mass quantities. On October 31st of 2016 our suspicions were confirmed and we packed our bags and headed to Birmingham Children’s hospital. Initially diagnosed with the autoimmune disease called Minimal Change Disease, we found comfort that it was possible to gain “remission” with steroids and she would likely grow out of it. We had a difficult few months, spending 19 nights on and off in the children’s hospital for severe edema and infections. In December of 2016 after no response to steroid treatment, they performed a kidney biopsy and revealed that she had a much more serious form of the disease called Focal Segmented Glomerular Sclerosis. This form of the disease progressively scars the kidney filters, leading to declined function over time. We were devastated, as now we realized that we would likely deal with this for the rest of her life.
We had a good run from January of 2017 to August 2017 with no complications or illnesses, and we had her on a diuretic that seemed to keep her from getting the edema, and an immunosuppressant drug called Tacrolimus to hopefully get her body to stop spilling protein. Unfortunately, this same drug compromises the immune system and she contracted a severe infection that led her back to Birmingham with significant kidney damage. While she was in the hospital we decided to give her an experimental drug called Rituximab, essentially a type of chemo drug that we hoped would push her into remission. We also decided to run genetic testing to see if we could pinpoint whether or not her form of the disease was immune-related or from a genetic mutation. Fast forward several months and 2 more hospitalizations for infections, we were again hit with devastating news that her condition was in fact a result from an NPHS1 genetic mutation. Shayla and I are recessive carriers of the disease and we were told that we had a 1 in 4 chance of any future children also having the same condition. The diagnosis meant almost certain end stage kidney failure in her future. The only silver lining was that it meant post-transplant she has an almost non-existent chance of recurrence, which is common with FSGS.
We had another good run from January of 2018 until this past April when routine lab work showed that she had dropped in kidney function from 68% to 21%, seemingly overnight with no warning or symptoms. We began preparing for the worst and scheduled a transplant evaluation at Children's Hospital of Atlanta at Egleston. Unfortunately the week before her appointment Ryleigh contracted strep throat and a UTI that caused fever and dehydration, which is detrimental to kidney function to someone already as fragile as she is. We went to the ER at Memorial hospital here in Savannah, but they were not comfortable caring for her, so at 4am that Saturday morning we were transported by ambulance to Wolfson's Children's Hospital in Jacksonville and admitted to the ICU.
She was calculated at only 10% function and her hemoglobin was at 5 (normal is around 12). They put her on antibiotics and gave her a pint of blood, pumped her full of fluids and agreed that if stable enough it was imperative that we attend her scheduled transplant evaluation. They released her to us that Monday afternoon and we drove from Jacksonville to Atlanta and took a 5 hour nap before beginning the 7 hour long transplant clinic on Tuesday morning, meeting the massive collaboration of experts that will save her life in the next few months and trying to make sense of the aggressive plan detailing how they plan on doing it.
Now at a baseline of only 13.9% function, we are in stage 5 end stage renal failure. Before transplant she will have surgery to place a PD dialysis port, allowing us to perform dialysis daily at home while she sleeps. Shortly after we determine that dialysis is working, she will have a double nephrectomy (both kidneys removed), so that she will stop spilling the protein that will be crucial in ensuring the blood supply doesn't clot to her new kidney. Finally, God willing after she heals from that, myself (if I’m a match) or another living donor will give her a kidney for transplant.
We are lucky in that she has been stable and the dialysis and transplant have been postponed until her kidney function falls to 10% or below. We thank God for every day that she is stable and happy and can live like a normal little girl. We know that we have a long road ahead and many scary and serious surgeries for a 4 ½ year old to deal with. We are lucky that Ryleigh is a brave little girl and already has been through so much that she is tough as nails. Her huge personality and love of life has never wavered and we know she is resilient and will come out of this feeling better and healthier than ever. She is truly an inspiration to everyone who knows her.
Transplant is NOT a cure, but merely a treatment, as she will still deal with this for the rest of her life. The nephrotic syndrome might go away, but transplant complications are always a palatable risk with infection and rejection. We ask for all the prayer warriors out there to pray for strength for her little body but also for mommy and daddy as we trust GOD, and place her in the care of these surgeons that will make sure we bring our sweet baby home a new and improved version of her physical self.....
The Children's Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.