Raith is a sweet, kind and fun-loving 13-year-old boy. He loves playing baseball, snowboarding and playing video games.
Raith’s latest medical journey began nearly two years ago with sporadic yet severe bouts of joint pain. His ankles, knees or hips would inexplicably hurt so badly that he could barely walk. The next day however, he would be fine again. Several office visits with local doctors over the following months yielded no significant explanations other than potential growing pains. During his spring break last year Raith became quite sick, and his joint pain became so severe that he was unable to walk, much less return to school following break. Another trip to a local doctor had a variety of labs drawn. One of the labs uncovered a problem with Raith’s kidney function. The doctor recommended he visit the ER at Children’s Primary Hospital in Salt Lake City, about a four hour drive away; he was admitted there that night.
A bevy of tests over the following few days yielded a diagnosis: Granulomatosis with Polyangiitis (GPA), formerly known as Weggoner’s Disease. GPA is a rare autoimmune disorder that usually manifests in adults. It is a form of Vasculitis that inflames smaller blood vessels throughout the body like the ones found in joints, the kidneys, the heart and lungs as well as in the sinuses. Raith was prescribed high doses of steroids to reduce the inflammation of these blood vessels. During his two week stay at Children’s Primary Hospital he received intravenous infusions of Rituxan/Rituximab, a drug discovered in recent years to effectively treat GPA. Raith also received two blood transfusions and several rounds of Plasmapheresis, a process whereby much of his own plasma is removed and replaced with donated plasma, a veritable human oil change.
He was then released to head home in hopes that as the blood vessels in his kidneys became less and less inflamed, little or no permanent damage had been done. Raith spent that summer visiting the local hospital in Driggs twice weekly for blood draws and follow-up labs.
Unfortunately, permanent damage had in fact occurred in Raith’s kidneys. In August he would be readmitted to Children’s Primary Hospital where he would be diagnosed with with Stage 5 Renal Failure and yet another even rarer disease: Atypical Hemolytic Uremia Syndrome (AHUS). HUS destroys red blood cells and platelets in the body faster than they can be produced. To treat this, Raith began receiving intravenous infusions of Soliris/Eculizimab, an extremely expensive drug that effectively treats HUS; the only other treatment involves constant blood transfusions. Raith received these infusions every other week initially at Children’s Primary in Salt Lake and later at St. John’s Hospital in Jackson, Wyoming. As for his kidney failure, two catheters were surgically implanted into his torso. Raith began hemodialysis using his chest catheter, while his stomach catheter matured. Raith and his mother lived at the Ronald McDonald House in Salt Lake over the following two months while he received four-hour-long hemodialysis treatments three times each week. The close of September saw Raith released to begin in-home peritoneal dialysis using his stomach catheter. Raith underwent 10-hour-long dialysis treatments nightly, while he slept. Unfortunately Raith’s body did not tolerate very well this form of dialysis. Despite attempts by his doctors at Children’s Primary to change up his prescription for the way the peritoneal dialysis was administered, Raith endured extreme pain and became intensely nauseous as a result. Raith developed anxiety toward his treatments and to all things medically related; he also was becoming depressed. After three months on peritoneal dialysis, the pain simply became too great for him to bear. The close of 2018 saw Raith readmitted yet again to Children’s Primary. Raith reigned in the new year in a wheelchair with his sister and father inside an enclosed hospital sky bridge overlooking the fireworks shows of Salt Lake.
Another chest catheter was surgically implanted. Raith returned to hemodialysis treatments, and his stomach catheter was removed. Raith’s demeanor and energy levels improved rather immediately. During this stay at Children’s Primary, Raith also received his last infusion of Soliris/Eculizimab as it would counteract his scheduled maintenance dose infusion of Rituxin/Rituximab. He has not needed to return to the infusions of Soliris/Eculizimab since. Following a much shorter two-week stay at the Ronald Mcdonald House in Salt Lake, again with his mom, Raith was released to begin receiving his four-hour long thrice weekly hemodialysis treatments at an adult clinic in Idaho Falls. This is the closest available dialysis clinic to Raith’s home (about an hour long drive each way). The mostly elderly patients, as well as all of the medical staff, seem to appreciate Raith’s youthful verve and playfulness. Despite stricter dietary restrictions (2,000mg of sodium and only 32 ounces of fluid per day) and the three-times-per-week drives and treatments in Idaho Falls, Raith continues to return to his old self on hemodialysis. He is much happier, far less anxious and much more energetic and active than he ever was on peritoneal dialysis. For instance, he now again enjoys riding his bike around town, an activity he simply could not do some months ago.
Just this month, May of 2019, Raith’s kidney doctors and others at Children’ Primary reviewed his case and have released him to receive a kidney transplant. Both his mother and father are in the process of becoming a donor for him. One of the many previously unrealized truths of this medical journey for Raith is that a donated kidney does not last a lifetime. In fact, donated kidneys last on average only 8-15 years. This means that Raith will likely need multiple kidney transplants over the course of his lifetime. He will also remain on a strict and lifelong regiment of anti-rejection medications.
We always knew that our son was far stronger than he ever realized. His medical journey has only reinforced that knowledge; it has shown us that he is indeed far stronger than even we realized. His medical journey has demonstrated to us Raith’s amazing resilience, and we mean this beyond physical and emotional resilience. Despite the negative aspects of this latest journey for him, Raith remains the kindest and most generous soul we have ever come into contact with.
Raith is hoping for a new kidney in the near future. While a new kidney is not a cure, it is definitely a start to a new journey, one we are very much looking forward to.
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.