The Greatest Evil is Physical Pain

Yes, the distractor rods are out.

Yes, I am moving past the PTSD of this last surgery.

Yes, we are experiencing a lot of miracles and milestones as of late.

No, things are not okay.

Why?

Because my son is in massive amounts of pain.

Why?

We are not entirely sure.


It all started on December 17th.  Jayson had taken several falls that day.  His balance was off, and he was struggling.  One fall caused him to fall rear first into the corner of the couch, then he hit his cube chair, then he fell on his bottom on a pile of toys.  He cried for like 20 minutes.  Not fun times.  He calmed down and I decided to give him his oral feeds.  He had a VERY hard time in his high chair.  He was having a lot of jerky spasms with grunts and almost breath holding spells.  They happened over and over.  He does similar things with his seizure he gets while he eats.  But this time he wasn't neurologically impaired.  He seemed to be in pain.

It continued through the evening.  He stopped walking.  He laid down and didn't want to move.  He was super fidgety and squirmy and constantly brought his knees into his tummy.  He groaned and whined and tried dozens of positions trying to get comfortable.  He crossed his legs and put his hands between his legs.  Nothing seemed to help. It really seemed like a tummy pain, and it got worse while eating.  I wanted to take him into the ER right away, but realized it could possibly be a tummy pain or bug and I should give it time.  So we got him his bath, put him to bed and he slept.

I prayed that when he woke the pain would be gone.  It wasn't.  He slept well, woke up, took off his cpap mask, sat up and started grunting and jerking all over again.  It seemed worse.  He was starting to fuss and cry.  I knew I had to take him in so I started getting things ready.  I went to give him his meds, but they wouldn't go in his tube.  It seemed to be clogged.  WEIRD!!  I pushed and pulled with the syringe, and finally gave it a good amount of pressure--- his button in his tummy split open!!!!  I've never seen anything like it.  So that meant one of a couple of things-- there was a problem with the tube and that's why it didn't flush, and then split OR there was an obstruction in his belly that prevented it from flushing and then broke the tube, OR maybe the break in the tube somehow was causing pain in his belly.  I had to give him some time to see if it got better after I replaced the tube.

My older sister was in town and wanted to meet for lunch at a restaurant that was right by Primary's.  I thought that was perfect.  We could eat and visit, give J time to see if he felt better, and if not we would be right by Primary's.  Well, he didn't feel better.  My sisters saw some of his jerky spasms in response to almost a jolt of pain, and they saw his fidgetiness due to his discomfort.  We headed to Primary's.

It was a pointless visit, and I almost knew it would be.  He showed his jerky spasms to the nurse, but then she left early.  No one else saw much evidence of his pain.  They don't know Jayson.  They don't know that he doesn't cry; they don't know he doesn't complain; they don't know that he has one of the highest pain tolerances in the world.  They tried their best to listen, but they saw no reason to keep him since he looked uncomfortable to them, although in great pain to me.  His abdominal x-ray came up free of obstruction and showed very little gas.  No constipation, no real problems.  The urinalysis came back clean.  No bladder infection, no kidney stones.  We left without answers hoping the pain would resolve as quickly as it came.

It didn't.  We saw our pediatrician the next day.  Jayson was doing much better that day, but still was clearly uncomfortable.  The pediatrician did a full exam and couldn't really pinpoint it either.  The next day was rough.  Jayson hardly moved.  He whined.  He grunted.  He jerked and even started to cry.  I was curious if he was running a fever.  That might give us some clues.  Jayson prefers rectal temps over all other forms, and they give us the most accurate readings with his autonomic nervous system dysfunction.  So I laid him down and took his temperature.  Oh-my-hell----- I've never heard him scream like that.  It was awful!!!  But it was a clue-- rectal pain.  I hit my mommy groups online and asked for help in piecing the puzzle.  Crossing legs, grabbing in between legs, worse when sitting in high chair or car seat, prohibiting him from walking, not constipation, not a noticeable hemorrhoid or tear, not an obstruction.  Mommies put their heads together and one came up with a very plausible answer-- tail bone injury.  I totally forgot he had a bad fall that day!!  Ding ding ding!  I started treating it like an injury by putting many pillows and blankets down for J to sit on and he started to do better.  We treated with round the clock Motrin and Tylenol and he did a little better.  The pediatrician talked to me over the phone and confirmed that was likely the explanation.  He documented it in his file as a tailbone injury, like a break given the level of pain and duration.  We thought we had it figured out.  It sucked, but it was okay because I knew what to expect.

Christmas came and went.  Jayse was sad and miserable, which made the rest of us sad and miserable.  He just needed time, we thought.  It HAS to get better at some point, we thought.

It didn't.

Things got worse.  Jayson was more and more vocal about his pain and discomfort.  He'd have a better day and be completely in bed whining the next.  Suddenly, he stopped sleeping.  He was waking up at 6, then 5, then 3AM in bad bad pain.  He was jerking, thrashing, and grunting for hours.  Tylenol and Motrin weren't touching it.  I was at a complete loss.  How would a tailbone injury get worse?

It wouldn't.

I had this strong feeling during dinner one night that there was something more going on.  I felt like I needed to think critically about the signs.  The thing that really caught my attention was how much worse it made it to sit in a seat--- carseat, high chair, wheelchair, cube chair.  This made his jerky episodes out of control and resulted in legit tears, despite the blankets and cushions we placed on his chairs (carseat excluded, of course).  This is NOT the first time Jayson has had these types of episodes... while eating... in chairs.  We've called them seizures before, but there was often a neurological component to them, but not always.  These episodes weren't EXACTLY alike, but similar enough to strike me.  He's had those episodes off and on since August of 2013.  I've sent these videos to our neurologists and doctors of these episodes, wondering what they were.  The latest of these videos have been under scrutiny of a team of over 10 doctors trying to figure out what was happening-- is it pain? If so, where?  Reflux? Seizures? Neurological episode? Behavioral?  We didn't know.  All I know is that they nearly ALWAYS happen in a chair.  What could make him act like this in a chair?  Lack of support? Instability? Dislike for sitting? Or pain while sitting upright in a chair that forces you to put weight and pressure on your bottom.  That was it.  All of these chairs force him to put pressure on his lower spine.  He is strapped in or secured.  He doesn't do this when sitting on the floor, but when on the floor he is always leaning back or leaning forward.  He rarely sits upright.  That is part of why his therapists think he has low tone, even though his tone is pretty good.  He avoids sitting up on his tailbone/spine.

So if he's always done this type of jerky spasm in reaction to pain of sitting on his bottom, WHY was it so much worse suddenly?  What changed recently that could awaken a condition or injury?  Was it the fall? Maybe. But I don't think so.  He started walking.  Jayson started walking, and the pain shortly followed.

I started connecting some dots, and with each dot I got more and more sick to my stomach.  Severe pain, crossing legs, leaning back or on the side, pain while sitting, regression in walking, strange stepping patterns while walking, a tailbone that protrudes, sacral dimple, neurogenic bladder, pain while getting bum changed due to legs and buttocks in the air, chronic diarrhea and chronic constipation, autonomic nervous system dysfunction, sleeping in strange positions, Chiari Malformation. This wasn't a tail bone injury.  This wasn't an injury at all.  This wasn't going away.

At dinner that night I had a feeling in the pit of my stomach about what it was, but I didn't want to go there yet.  I told my husband that I thought something else was going on.  I shared all the signs, and he agreed.  We decided we'd go to bed and research it the next day and make some calls.  Jayson woke us up in excruciating pain at 4AM.  I gave him Tylenol and started my research.  I only had to type one thing into the Search bar, because I already knew what it was.

Tethered Cord.

Read about Tethered Cord here and here.

It couldn't be.  Our neurosurgeon told us he didn't have it.  I asked him in February if we had checked for Tethered Cord with Jayson's last MRI and if we should check for it with the next MRI.  He said we already did, and his MRI was clear.  I stopped myself mid-thought.  Our neurosurgeon had already made a humongous mistake by missing Jayson's intracranial pressure and craniosynostosis.  What made me think he wasn't capable of being wrong here too?  I looked in our records to see if Jayson had in fact had a lower lumbar MRI.  Nope.  He hadn't.  Just a C-spine.  We had been lied to.  Tethered Cord is a possible explanation of problems J's had for a very long time.

I just cried.  I sat and cried tears of exhaustion, tears of anger, tears of frustration, tears of sadness, tears of defeat.  We weren't even fully recovered from the last surgery; could we really be looking at yet ANOTHER major surgery??  I had a big feeling of comfort come over me with a voice whispering, "Three."  What did that mean?  Three?  Three big health conditions.  Three major surgeries.  Three big battles.  I felt comforted like I could do it.  One more big discovery.  One more big surgery, and then we would be okay... for a while.

I tried calling neurosurgery the next day.  Their office was closed for the entirety of the Holiday season.  When Jayson woke up the next morning at 3AM freaking out from the pain I called the neurosurgeon on-call at Primary's.  I described what had been going on, my observations and my thoughts.  She agreed.  Tethered Cord.  She said to get into the pediatrician ASAP and get some narcotics to help him sleep and an order for an MRI.  The pediatrician couldn't get us in the next day, so we saw a partner in the clinic on Saturday morning.  He thoroughly examined Jayson and agreed that Tethered Cord sounded likely.  He wrote us a script for Oxycodone and an order for a lower lumbar MRI.  Unfortunately, the radiology department was closed on Saturday so even though they had the order for the MRI, I couldn't call and schedule it.

I called today which resulted in a day filled with frustration after frustration.  The SOONEST radiology could get Jayson in was January 28th.  Three weeks away.  Jayson has already suffered for nearly 4 weeks with NOTHING being done because we thought it was his tailbone.  And now, he is supposed to wait another three weeks for an MRI???  No. Not okay.  I called Neurosurgery.  They wouldn't see him until AFTER the MRI.  February 2nd.  So we will not have answers NOR a plan until AFTER February 2nd.  Really not okay.  I left a message for the neurosurgery nurse.

The neurosurgery nurse practitioner called me back to tell me there was nothing she could do.  She tried to tell me the problem was that I had my pediatrician write the MRI order instead of neurosurgery, and THAT'S why it was scheduled later and THAT'S why Jayson couldn't be fast-tracked.  Hell to the no.  I lost it. I did EXACTLY what the neurosurgeon on-call told me to do.  The NS nurse practitioner said she didn't listen to my message too closely and wanted to know why I was so sure my son had Tethered Cord.  I told her I wasn't sure, it was just a possibility!  Something we should rule out.  I wanted to know HER opinion.  I listed my concerns, thoughts, observations.  She interrupted me and said, "It's sounding like tethered cord.  Let me call radiology.  Please hold."  She came back on the line to tell me that radiology wouldn't budge for her either.  I asked her if I could go anywhere else for the MRI in Utah.  No.  I asked about going out of state for the MRI.  No.  I asked if there was anything else we could test for or look for?  It's my fear that after we put all of our eggs in the tailbone basket, that we then put all of our eggs in the Tethered Cord basket only to find out another month of pain later that Tethered Cord wasn't the problem either.  I don't want to waste time.  If it could be something else, I want to look into that right now too while we're waiting.  She said that she feels fairly certain that it's either Tethered Cord or a Syrinx. She doesn't feel any other testing is necessary.  It felt good to have my instincts validated, but not like this.  I asked her, "So you're telling me as a professional that you feel comfortable telling me that my son gets to spend the next three weeks in pain, on narcotics just to sleep and function, and that there is NOTHING we can do about it."  She answered yes.  So that was it.  I hung up.

I have since talked to our pediatrician's office Nurse Care Manager, who we love.  She assures us that the radiology department at Primary's will be called three times a day, every day, searching for openings until we get Jayson in.  Other than that, I don't know what else to do.  The nurse practitioner said it might be possible to get him in sooner through the ER, but she didn't recommend it due to all of the illness.  One of those respiratory illnesses is LIFE THREATENING to my son.  It's sad, but if forced to choose, I would choose to keep my son alive and in pain over in the ER getting fast-tracked to an MRI.

So there we are.  This is where we stand.  A helluva place to be.  Clearly I'm not happy about it; clearly I'm struggling to cope.  Everything ends up being a fight, and I'm tired.

Just to inform you, Tethered Cord is not necessarily a life-threatening condition.  It is, however, life-altering.  It affects the neurological system.  The longer it goes untreated, the more likely there is for there to be permanent neurological damage.  It is congenital, you are born with it, but it often acts up when kids get old enough to walk and recognize pain.  Many adults are diagnosed after they've had it their whole lives and they've thought it was growing pains, then a joint or back issue only to find out it was Tethered Cord.  It can cause no pain, mild pain, or excruciating pain.  It occurs when the lower part of the spine becomes tethered so it doesn't freely move.  Then when the child moves, grows, walks, stretches, it pulls and causes awful pain and nerve reactions.  It can be constant or cause shooting pain.  I think it could potentially be a trigger for some of J's seizures, but that is my Mommy MD opinion.  It often occurs with Chiari Malformation, a brain condition Jayson has, and I've heard that Tethered Cord can cause or contribute the Chiari by pulling the brain stem downward.  I've also read that it should always be screened for when Chiari Malformation is suspected because it should be treated first, since it could be the force to pull the brain stem downward, causing herniation.  Clearly our neurosurgeon did not read the same neurosurgery text books I have.  Little cysts can also form at the bottom of the spine with the Tethered Cord, and that's called Syringomyelia.  A Syrinx could also potentially be a problem; a syrinx is a pocket of spinal fluid that collects within the spinal column.  Fortunately, these things are all operable.  For Tethered Cord, the lower spine needs to be untethered, unconnected so the spine and spinal fluid can freely move.  Syringomyelia can be removed, and a Syrinx can be drained.  All of these surgeries are high-risk procedures done by a neurosurgeon.  There are risks of permanent nerve damage and paralysis.  They are in the same category of riskiness as Jayson's cranio and Chiari surgeries.  Lovely.  I know, our motto is GO BIG OR GO HOME.

So that's where we are.  As usual, we could use your prayers.  Our little family unit has been through the ringer, and Little J cannot catch a break.  I know we have God on our side, and Little J has superhero powers, so I know we will get through.  Thank you all for your love, prayers, and support.  Your offers to supply hit men are also greatly appreciated, but are not needed at this time ;)

For an excellent, clear and SHORT explanation of Tethered Cord and how it is surgically treated, watch this youtube video here.