When Noah was three years old his parents, Elaine and Mark, rushed him to their local hospital when his eyes and abdomen became swollen. Doctors thought it might be kidney failure, but after three days, he was back to normal.
A couple of months later, his eyes swelled again, but this time Elaine and Mark took him to BC Children’s Hospital. There, Dr. Anne Junker realized Noah was showing signs of an extremely rare disorder called systemic capillary leak syndrome (SCLS). During SCLS attacks – which are life-threatening and can be rapidly precipitated by minor infections – fluid drains from the blood system into the surrounding tissues. As well, the blood itself, drained of fluid, becomes dangerously thick, impairing circulation.
In December 2011 Noah had another episode, but worse than the others. He was admitted to BC Children’s Pediatric Intensive Care Unit, where he underwent fasciotomies, a procedure that involves incisions along the insides of the arms and the legs, to relieve the intense pressure of the fluid that was trapped between his skin and his muscles.
Dr. Junker and Noah’s parents decided to try a treatment that was reported to work in other patients with SCLS. Noah was started on regular infusions of intravenous immunoglobulin (IVIG) and, over the past year, has had fewer and less severe attacks.
It’s been 14 months since that last episode, and Noah, who’s now six, is treated every four weeks. So far, so good, says his mom. He’s even okay with the large scars, which, Elaine tells him, are his “superhero scars.”