Yaraslovsky Terminal, Moscow; Our Story Begins

I never intended to take the slow train to Vladivostok from Moscow.  Unlike the Trans-Canadian Railway or the Orient Express, the Trans-Siberian Railway has always been primarily for moving cargo.  Much like the political exiles of the past, I was pretty much thrown onto the train without much in the way of preparation or supplies.  There have been numerous points at which I would have liked to depart the train and return to civilization as I formerly knew it.  But when you have a sick child, that just is not an option.  My child, a minor, with very little in the way of coping skills, is on this train, and so I have no choice but to accompany him, making the ride as smooth and enjoyable as possible—for him, for me, for my husband, and for my other two children.

When did I know we’d boarded the train to Siberia?  When did I realize it wasn’t, at least, the Express?  It probably took longer than it should have to make that realization.  

I didn’t know when my nearly nine year old, Christopher, first started getting intermittent headaches in the fall of 2015.  He’d just started a new swim team and was swimming more yardage at greater intensities than he had previously, so I assumed he was having low-blood sugar headaches and instituted a high-protein afternoon snack.

I didn’t know, in the late winter of 2016, when he started complaining of horrible stomachaches or hellacious headaches every morning, and begged to just do his reading assignments for the day on the couch, not anything that required typing or writing.  I was generally able to convince him to do most of his schoolwork, but it took three hours of wheedling and meltdowns, all from a kid who used to routinely finish his schoolwork for the day by breakfast.

I didn’t know, March 18th, 2016, when I took him to our pediatrician’s office and it was suggested that he was having abdominal migraines, likely in conjunction with inflammatory gut disease (it runs in my family), and that we ought to do a head CT just because he’d been having headaches for so long.  After all, he’d just passed a full neurological exam, so surely the head CT order was just CYA medicine.  For those of you thinking an inflammatory gut disease diagnosis would surely qualify for the Trans-Siberian Railway, remember, it runs in my family, so I know how to deal with G/I tract issues, especially after mothering two kids with food allergies.  

My first inkling that this wasn’t going to be an easy ride was when the pediatrician called with the results of the head CT.  Ironically, it was April Fool’s Day.  The one time that morning I wasn’t available to answer my phone was, of course, when she called and left an innocuous message for me to call her back.  When I did, the receptionist looked for her, found her busy with another patient, and—rather than having a nurse deliver the news—told me she’d have the doctor call me back.  At this point, blaring alarms started going off in my head.  Normal results, the nurse would have delivered.  Abnormal results in the realm of the everyday, the nurse also would have delivered.  The fact that the doctor felt that she, herself, had to give me the diagnosis could only mean that it was very, very bad.  And all of sudden, into the middle of my spring afternoon, crept every memory of a sick, colicky, failure-to-thrive baby Christopher.  Closely following those incidents were the memories of my kindergarten-aged child being sent to the pediatric endocrinologist to determine whether or not his growth plates had been adversely affected by all those early illnesses because he was still chronically underweight and much shorter than his older brother at the same age.  In the hours I waited for the pediatrician to call, I was certain my son had a pituitary tumor that had escaped the radar, and while I looked like I was calmly folding laundry on the outside, I was having a panic attack internally.

Thank goodness the boys were outside walking the dog when the call came.  My daughter was watching late afternoon cartoons.  I was therefore alone, and could just let the tears fall, when the pediatrician told me the head CT showed my child to have a congenital birth defect called a Chiari I malformation.  He was born with it, he’s had it all his life, but with the headaches he was now experiencing, I was told he needed to see a pediatric neurosurgeon as soon as possible.  I dutifully took notes, called the neurosurgery office, and left a message, since it was Friday and they were all gone for the weekend.

That delay served me well.  For while Christopher’s headaches had gotten much worse in the two weeks prior, now persisting around the clock rather than appearing at some point each day, I had time to talk to a few people and process the news that we were, indeed, bound for the first stop on the Trans-Siberian Railway.  I discovered from two physician friends that Chiari I malformations are often over diagnosed and that, if you’re going to have brain surgery, Chiari decompressions are the most routine of all possible brain surgeries.  I also had time to talk to my husband and a couple very dear friends, who agreed that this wasn’t where we’d intended to travel, but looking up the most basic information on Chiari malformations, it certainly wound a lot of the seemingly unrelated threads of Christopher’s early life together.

Also, it wasn’t childhood cancer.  We had to wait until we could get a brain MRI and a neurosurgery consult to confirm the diagnosis, but my worst fears of a pituitary cancer were unfounded.  Chiari I malformations occur because there isn’t quite enough room at the back of the skull (the posterior fossa) to contain all of the brain.  The brain stem and cerebellar tonsils therefore push down and out the hole at the bottom of the skull, called the foramen magnum.  This herniation can lead to headaches.  We told Christopher his brain is just too big for his head and the surgeons might need to make more room.

Figure 1--Diagram of the bottom of the human skull, labelling the pertinent parts.  Image Courtesy of the Mayfield Clinic.

Figure 2--In a normal brain, the brain is entirely above the black line drawn across the brainstem and cerebellar tonsils, but in a Chiari I Malformation, the tonsils typically descend at least 4mm below the foramen magnum.   Image Courtesy of the Mayfield Clinic.

Image 1--This is the head CT image that resulted in a diagnosis of Chiari I Malformation.  Not the clearest picture, but it does appear that brain tissue is extending significantly below the foramen magnum.