He Can't Catch a Break

Matthew had his full spine MRI last Tuesday. His cervical spine shows a 9 mm herniation of the cerebral tonsils. His last MRI it was 7mm. It also showed that a part of his skull called the clivus is shorter than normal. From what I can gather, the part of his skull that is supposed to contain the cerebral tonsils is too small, which causing the brain to be pushed down and out of his skull.

          His thoracic spine images show a mild right curvature. Which means my 2 year old has scoliosis. It is extremely rare for a 2 year old to have scoliosis. My understanding is that this is directly caused by the Chiari malformation.  The only way to stop the progression of the curving of the spine is to decompress the Chiari malformation: i.e brain surgery.

          His lumbar spine MRI showed the lower part of his spinal cord, called the conus, at the L2 vertebrae. It depends on what part of the L2 vertebrae the neurosurgeon considers “normal” to know whether this is clinically significant. However, Matthew’s leg issues cannot be explained with the Chiari alone. Given all his symptoms and the sacral dimple, he may have what is called an occult tethered cord, which means hidden, not seen on MRI.

          Thankfully we will be seeing the neurosurgeon on Friday morning. From everything I have read in the past few weeks, I am fairly certain she will recommend the decompression of the Chiari given all of his symptoms and the scoliosis. With what urgency he will need it I don’t know. As for the tethered cord issue, of this I am much less sure. It seems to fit, but then again, so did cerebral palsy. My biggest concern with regards to the tethered cord, is if he does have one that is hidden, it is still causing irreversible damage that will only get worse if left untreated.

          The neurosurgeon comes highly recommended from a variety of sources, including parents of former patients and from the hospital nursing staff. After his MRI on Tuesday, the nurses could not stop raving about his neurosurgeon. I am also very happy with the fact that if and when we have to hand our 2 year old over to the neurosurgeon, she will be the only one operating. We won’t have to worry about a young resident practicing for the first time on our son’s brain; the hospital he will be at is not a teaching hospital.

          I am terrified for my son. He doesn’t seem to be able to catch break. September seems to be particularly bad for him. Last September he was diagnosed with a peanut allergy (to which he also added lentil and black beans). This September is the Chiari malformation. I worry about his immediate future and his prognosis before, during, and after brain surgery. But I also worry about his long term emotional health. The peanut allergy alone sets him up for a childhood full of bullying and exclusion. Adding the limitations the Chiari puts on him (with or without surgery) I am scared for when my 8 year old little boy comes to me in tears asking why he is different. Asking why he can’t do what the other kids can. I wish I could take it all away. But knowing that I can’t, makes me fight even harder. He is the strongest kid I know. He doesn’t take crap from anyone. I just hope that personality trait stays with him throughout his life.

Out of Left Field

Two weeks ago Matthew had a MRI of his head to rule out (or in) mild cerebral palsy as the cause of his gross motor delay as well as several other issues. Nobody was expecting what the images showed. It turns out Matthew has what is called a Chiari I malformation. Essentially his cerebellum extends below the base of his skull. Many people have this and are completely asymptomatic. Others however, have severe issues related to this condition. Unfortunately it looks like Matthew falls into the latter category.  His excessive drool, low tone in his mouth and face, and choking on thin liquids are all signs that his Chiari malformation is causing issues.  His gross motor delay and spasticity in his legs may be caused by a related condition called Syringomyelia (fluid filled cysts along the spine) and/or a tethered spinal cord. He will be having a complete spinal MRI in the coming weeks to check for those related conditions.

After spending some time researching this condition and finding a Facebook group (because there are Facebook groups for everything) I found a pediatric neurosurgeon in our area who specializes in this condition. Thankfully his pediatrician is able to write us a referral to this doctor, as she has worked with her before.  I have already emailed the neurosurgeon asking what additional images she would like to have completed prior to his initial appointment. She was extremely quick to respond so now it is a matter of getting the MRI scheduled.

So what does this mean for Matthew? It may be as simple as watch and wait while limiting certain activities. It may be as complex as brain and spinal surgery to prevent further neurologic damage. The truth is, at this point we just don’t know. Someone asked if he was in pain. Honestly, I don’t know. He could very well be living with chronic pain, but because it is normal for him, he doesn’t complain.

Before his MRI, my biggest fear was it would show an inoperable, malignant brain tumor. This was my second biggest fear. Something that may not kill him, but will make his life even more challenging. There is no cure for this. Surgery will only prevent further damage. It may not even alleviate current symptoms. And no amount of Google searching could find a connection between food allergies and a Chiari Malformation. Trust me. I tried.

I don’t know much yet. We need to wait until the MRI of his spine is completed and we meet with the pediatric neurosurgeon. I do know that my son is the strongest, most stubborn child I have ever met. He has already fought through several challenges in his short life, and he will fight through this one. I just wish he didn’t have to.