According to a story from the Tennessean, learning that both her husband and daughter had the rare and dangerous disease atypical hemolytic uremic syndrome (aHUS) was devastating for singer-songwriter Cameron James. However, over time, she has chosen to view the situation with gratitude and is learning to develop a new perspective.
Atypical hemolytic uremic syndrome is an extremely rare progressive disease that can be life-threatening. Although the exact cause is unclear, research suggests that their could be a genetic basis for the syndrome. Patients experience long-term uncontrolled activation of their body’s complement system, which is the portion of the immune system responsible for disposing of foreign particles. The greatest risk posed by atypical hemolytic uremic syndrome is that it can cause the formation of blood clots in small blood vessels. This can lead to potentially lethal medical emergencies such as kidney failure, stroke, and heart attack. Symptoms include fatigue, diarrhea, confusion, swelling, nausea/vomiting, and abdominal pain. Pancreatitis, seizures, liver necrosis, and coma may occur in severe relapses. The drug, Soliris, a complement system inhibitor, is the best treatment option. Patients with kidney problems often must resort to dialysis. To learn more about atypical hemolytic uremic syndrome, click here.
Elise, Cameron’s daughter, was first diagnosed with aHUS in 2014 when she was just three years old. She had already experienced brain damage from a fluke injury that happened early on in Cameron’s pregnancy, which was diagnosed after motor skill delay at age one. It was only a year ago, when, after a night of severe vomiting, Cameron’s husband, James Shadinger, was also diagnosed with aHUS. Although the news was devastating, they believe that Elise’s diagnosis may have saved her father’s life. Now, Elise and James must receive weekly infusions that keeps their illness at bay, which are shipped to them in a cooler.
There is only one treatment for aHUS, called Soliris. It’s one of the most expensive drugs in the world, at around $500,000 per year. Fortunately, the family’s insurance plan covers the immense cost, which they are very grateful for. They are currently advocating for a bill called “the access to marketplace insurance act,” which, if passed, would remove a current federal restriction that allows private insurance companies to reject third party patient financial assistance. Third parties include charities, civic unions, and churches. They have been to DC twice to lobby and tell their story.
Cameron says “We want to help protect the ability for rare disease patients to receive the financial help that people want to offer them.”
“Advocating on behalf of AHUS and this bill (H.R. 3976) has brought a deeper purpose to our story and purpose to the pain. God is getting all the glory as we are living testimony to the ‘goodness of God in the land of the living.'”