Ignorance is bliss...

Cannon has been doing so terrific that sometimes it's easy - and wonderful - to forget he has HPP. But just when I think I can take a deep breath, the rare metabolic bone condition rears its ugly head to remind me of the severity of this terrible disease.

A few years Cannon had surgery to relieve a skull malformation called a chiari malformation. You can read more about it in my post from March of 2013. In a nutshell, Cannon's brain extends beyond the base of his skull into his spinal column. The pressure caused by this condition has caused a large pocket of fluid - called a syrinx - to form in his spinal column, preventing the spinal fluid from flowing easily up and down his spine. While he has no current symptoms aside from a headache here and there, neurologists fear that without surgical intervention, he could have other neurological symptoms (including loss of feeling or use of parts of his body).

The first surgery was a chiari decompression surgery where brain surgeons removed a piece of bone from the base of his skull with hopes that this would help relieve the pressure and improve the flow of spinal fluid. Unfortunately, because the pressure was so high, they had to be very careful and conservative with this procedure, as Cannon had some arteries and veins that were supposed to be the size of hairs that were the size of a sausage. We are very blessed to have skilled surgeons at CHOP who knew that this put him at risk and made this decision on the spot in the OR the last time around. I could tell by the look on the surgeon's face the last time that it was not an easy surgery for him.

Cannon has since had followup MRIs to monitor the syrinx, which unfortunately has actually not decreased, but possible even increased, in size. Because of the continued neurological risks, we are going back for a second round of surgery - this time likely more invasive. An anatomical MRI seems to indicate that the veins and arteries that were swollen the first time have shrunken in size, making an intradural decompression surgery more feasible. The "dura" is the sheath that encases the spinal column. This time, surgeons will cut through this sheath to hopefully relieve the pressure within the spinal column that is causing the syrinx. Of course, I'm explaining this in the most basic of terms, but that is what we are dealing with.

Cannon had his pre-op appointment today - he did will for his vital signs, bloodwork and testing. Now, we wait until Tuesday. Please keep Cannon in your prayers and pray for his surgeons who will be performing this very intense procedure. We are comforted knowing that these surgeons are so incredibly skilled and talented. The last time we met with Dr. Heuer, he left our appointment to perform spinal bifida repair on an unborn baby in utero. Just incredible and amazing! He will be joined with the Head of Neurosurgery for this procedure, Dr. Storm.

More to come! Thank you everyone. <3