05/26/2026
We are exactly a week on from Sam's surgery. Last Monday was, without a doubt, the longest day of our lives.
Vanishing White Matter disease is a stress response disease. Sam's brain loses white matter and motor control in response to bodily stressors: fevers, head trauma, certain medications including anesthesia all can cause Sam's white matter to disappear. Obviously with a 12.5 hour long surgery we were hitting many of these triggers. The last child with an early onset form of VWM that had this surgery (less severe than Sam's) did not survive. We were counting on the clinical trial medication that Sam is on to keep him alive, and incredibly, it did.
We are so grateful to Sam's surgical team at CHOP for taking all the precautions necessary to make the surgery as safe as possible for him, and for being incredibly skilled at their jobs. A few hours into the surgery, we were told that the neuromonitoring was only able to see Sam's left arm, meaning they were blind to any impact on his legs and right hand during the surgery, risking paralyzation. He can barely use these limbs anyway so we agreed. After the surgery we noticed they were paying close attention to feeling in these limbs, and luckily he had feeling in all of them.
Sam is doing so well, we are managing the pain with a combo of va**um, tylenol and ibuprofen. We have noticed his speech is a little slower and is definitely struggling more with his left hand, undoubtably the result of the stress of the surgery and fevers he had after, but these are very minor prices to pay for successfully straightening his spine. He is no longer coughing all the time because the curve is compressing his lungs, his ribs are no longer touching his hips, he can sit in his wheelchair without falling over the side and his snoring has completely stopped. All in all, a better result than we could have dreamed of.
