At age 5, Cameron was diagnosed with Hunter syndrome, a rare genetic disorder which strikes only males. People with Hunter syndrome lack the enzyme needed to break down long chains of sugar molecules. The molecules build up in the body and interfere with organ and cell function, leading to a number of serious issues. For Cameron, the major issues were an aortic and mitral valve leak and an enlarged heart on one side. But the syndrome also caused him to have an enlarged liver and spleen, carpal tunnel syndrome, hearing loss, low platelet counts, sleep apnea, multiple hernias, severe ear infections, eye issues, and a decrease in his walking ability.
Cameron was initially treated with IVIG, a blood product administered intravenously to boost immunity. Cameron shared that, “During this procedure, sometimes I would start to get cold, and then I would be shivering, shaking in my seat. Then a massive headache that felt as if bricks were falling on my head would come. The nurse would give me something, and I would be asleep for the rest of the day. I no longer have to have IVIG treatments due to the new medicine I’m taking.”
When Cameron was about 9 years old, he was one of 12 people worldwide selected to participate in a clinical trial for a promising new medication, Elaprase. Today Elaprase is the standard treatment for Hunter Syndrome, addressing the underlying cause through enzyme replacement. But then, it was still unproven and Cameron’s family had to fly every other week to Chapel Hill, NC, to participate in the clinical trial.
After only a month, Cameron started to see results. His liver and spleen started returning to normal. And his heart stabilized to the point that they no longer had to consider a valve replacement. Other things began to improve as well. This continued for four to five years until Cameron was able to have weekly treatments at Children’s Hospital at Erlanger. At first, the more frequent regimen made Cameron’s legs hurt, so they backed off to every other week until he could resume weekly treatments without pain. He has not had any other bad reactions to Elaprase.
Today Cameron comes to Children’s Hospital at Erlanger every Thursday for treatment as he has for the last 10 years. He is now able to receive the medicine through a port, which alleviates the need for constant injections. He is usually at the hospital from 9AM to 2:30PM for treatment, during which he can watch TV or sleep. Sometimes Cameron feels drained the following day, but he says that after a few days his energy comes back.
During his 18 years with Hunter syndrome, Cameron has had several surgeries. He has had tubes placed in his ears, multiple hernia procedures, two hand surgeries, a tooth extraction and the surgery to place his medication port. Surgery is high-risk for Cameron because of his difficult airway intubation. More recently, it has been harder for the team to wake him after surgery.
Cameron says the 14 years of Elaprase treatments have helped him in a lot of different areas. He says, “I am a lot better than I used to be.”