The News that Broke Me

I've been avoiding this post as long as I possibly could.  And even as I write this, I don't know that I can actually post it.  The past few weeks have been ruthless and beyond difficult for many reasons.  Unfortunately, the possibility that my son has intracranial pressure and could need another brain surgery is just one of them.  But there is so so much more.

I wrote a post about the anxiety I had before Jayson got his last brain MRI.  I was in agony and did not want the results.  I feared what they might be and the pain they might bring.  A couple of days before the MRI I had a vision, a dream while I was awake, and I saw myself grieving.  It was in response to the MRI we were about to have.  The news was not good.  I did not want to go forward.  I did not want to know whatever information was waiting for us. 

I was mentally prepared for the fact that we might learn that Jayson is having intracranial pressure.  I was mentally prepared that the results might bring hard things.  I was NOT prepared, however, for a completely new diagnosis.  I was not prepared to have absolutely everything we know about Jayson change in an instant, while nothing in his brain actually changed.  I was not prepared to be devastated, crushed and completely broken by the new diagnosis found on Jayson's MRI report.

I haven't allowed myself to fully process this new diagnosis, partly because our neurologist has not helped us in understanding what it is and what it means.  I haven't wanted to write about it because something about writing it down makes it feel too real.  I haven't wanted to share what we are dealing with because it seems that once I share this news I can't ever take it back or pretend it never happened.  Once it's out, I can't hide from this new knowledge anymore and honestly, I don't know how I will cope.  The only thing getting me through is convincing myself it isn't real and there must be some mistake.

There were several new findings on Jayson's MRI, which was not really something I was expecting.  One was the fluid in his optic nerves bilaterally.  Another was the position of his carotid arteries.  Lastly was the diagnosis of a condition called Rhomboencephalosynapsis.

Not familiar with it?  Neither were we.  Naturally, I visited the world wide web for some insight and there was not a lot there.  Why?  Because there have only been 40-50 diagnosed cases in the world since 1914, according to the research and literature.  I should be surprised, but the rarity of Jayson's condition(s) no longer surprises me.

What is it exactly?  It's a brain abnormality, a malformation that happened before birth.  Technically it is defined by a partial or complete missing vermis, a part in your brain that divides the two cerebellar hemispheres.  Therefore, the two cerebellar hemispheres are fused together.  This malformation is found in isolation or with other brain abnormalities and genetic conditions.  It is recently diagnosed often in utero or shortly after birth by MRI.  Due to its rare occurrence, many doctors and radiologists are not familiar with it, so it can go undiagnosed for many years, such as in our case.

What does it explain?  A LOT of Jayson's issues, actually.  I think this means more to us than Jayson's actual genetic syndrome that was recently diagnosed.  It is also seen with or can cause vertebral anomalies, abnormal skull shape, dysmorphic features, absent olfactory bulbs, ataxia (balance issues), abnormal eye movements, delayed motor development, mental retardation, other brain abnormalities including Chiari Malformation, seizures, involuntary movements, and hydrocephalus.  All of these things are conditions Jayson has, with the exception of hydrocephalus (which is now something doctors are considering given this diagnosis and the latest MRI findings).  Heart and kidney problems can also occur.

Isn't this a good thing?  Yes and No.  Yes this is good because it's an answer.  These are answers we have desperately been seeking since my son was first born.  But it's a lot more complicated than that.

First off, let me express my anger and frustration.  Once again a major condition my son has had since birth was overlooked and undiagnosed.  He was BORN with this condition.  Most patients are now diagnosed in utero.  My son was not.  Some are diagnosed later because they had no need for an MRI until later in their lives.  We had J's first MRI at 6 months of age.  It was not diagnosed then or during Jayson's other 7 or 8 brain MRI's.  In fact, the radiologist who diagnosed it in this MRI was Jayson's radiologist who read his very first MRI.  His brain was unchanged.  His vermis was absent then, and it is still absent now.  I cannot express in words the anger and frustration I feel knowing the answer for most of Jayson's complications and conditions was right in front of us the entire time.  How was this missed?  It is very easily detected!  How is this even possible??  I get tired of saying that; I really, really do.

Secondly, let's talk about what this really means.  A part of Jayson's brain is missing.  It's not there.  It can't grow; it can't appear; it can't be repaired.  No amount of gene therapy, medication, therapy, happy thoughts or even prayers will make a piece of his brain appear that was never formed.  There is NO HOPE.  None.  I can't let that even sink in.  I'm always so full of hope!  Even with a rare diagnosis of just 6 kids in the world, I can somehow visualize us finding more families, starting a foundation, funding research, discovering an amazing doctor who will create a treatment that could potentially heal my son.  There is a glimmer, although faint, of hope in that situation and in every other situation we have encountered.  But in this, there is no hope.  No surgery, medication or treatment will ever heal my son's brain and I am completely crushed by that realization.

Lastly, it's time to be painfully real.  If you haven't googled Rhomboencephalosynapsis yet, let me share with you what is found all over medical sites, foundation pages and research papers.

I don't think this needs an explanation.  I don't think I need to try and even express what I'm feeling.  Pure devastation.  Heartbreak.  Disbelief.  Numbness.  There honestly aren't words.  But I'm barely functioning most days; I'm pretending I live in a world where this information doesn't exist in order to function on the other days.  I hate this so much.  I hate all of it.  I don't want this to be my son's journey.  I don't want this to be our destiny.  I want another option.  I want there to be another way.

Because I can't live in a world where he doesn't exist.  I just can't.

We have never been told in the past that my son's conditions are terminal in childhood.  Up to this point we have been told that his conditions are life threatening, and given a healthy life with the support of his medical equipment, he could live to be in his 30's or 40's.  That was still hard to digest, but that seems so far off.  Somehow I feel like it would be easier then, although unlikely.  But we have never been told there is a good chance my son will not make it to adulthood.  This is new.  And it hurts like hell.  No, it breaks.  I'm literally in pieces.

I know a that there's a lot of not-so-great things that can come of Facebook, but it is my lifeline.  When I searched Rhomboencephalosynapsis on Facebook I found another family IN IDAHO that comes to Primary's for treatment!!!  Their daughter is younger than Jayson.  I messaged them and she informed me of a Facebook group to support families of children with this diagnosis.  Little did I know that a good friend of mine was also a member and her son has RES as well!! We've always known our children were similar.  I gained so much relief and peace from their posts, and maybe even a little bit of hope.  There are around 200 members of the group and it consists of many family members, including extended, as well as some medical professionals.  But there are many more than 40-50 people diagnosed with RES.  There are several adults who are members who post regularly!  There is a spectrum of cognition and abilities in the group.  Even so, it appears Jayson is among the most affected and the most medically complex.  Members claim the outlook is not always as grim as the research says, and that the research is 4+ years old and outdated.  They don't feel it is almost always terminal in childhood.  I know that's just the opinion of FB group members, but for now that is exactly what I'm holding on to.  I need that to be my truth.  I need to believe it with all of my heart.

I was very hesitant to research about RES or try to process that this could be a diagnosis until I spoke with our neurologist.  As I wrote in previous blog posts, he has not been in contact with us.  After I got our MRI report and saw this diagnosis and read one article about what it was and what it meant for Jayson, I sent our neurologist an email.  I asked him to please contact us as soon as possible to discuss the results.  I listed 3 or 4 main questions, some of which were about RES. I explained how important it was that we learn if Jayson really had this condition. I wanted to know how it was missed all of these years, and what it means for his future.  I explained what I had read in research and that I'm overly anxious reading that children with RES don't typically live to adulthood.  I told him my Mommy heart needed to know if my son really had this condition.  No response.  Still.  Nothing.  My heart is aching;  I am scared and terrified;  I cry myself to sleep each night worrying that this diagnosis might in fact be ours to own, yet he has still never called us to discuss Jayson's MRI results and RES.  I'm so confused.

I reached out to genetics via email.  We were due to set an appointment and I asked our genetic counselor if a diagnosis of RES changes anything?  Does RES come with Jayson's MN1 mutation?  Do other kids have it?  Is it something different?  Does it mean a different genetic mutation?  Does Jayson have two mutations?  Do we need to do more testing?  Genetics responded by scheduling an appointment with us... just two weeks later!!!  Anyone who knows anything about genetics knows that is an absolute MIRACLE.  We assumed it meant they had some news for us about MN1.

I plan to discuss our genetics appointment in more detail in my next post, but we got a chance to talk with Dr. V our geneticist about RES last week in our appointment.  His big question was the same as ours--- If it is congenital, formed before birth, how was this missed all of these years and with all of these MRI's?  I somehow hoped and prayed that it was a mistake.  That if we reviewed Jayson's other images they would clearly show a vermis and in these latter images we would see there was some mistake.  Dr. V said out loud, "I honestly wonder if it's not a fusion of hemispheres, but instead his hemispheres in his brain are so smooshed from pressure that it looks like the vermis is absent."  YES!! I like that answer.  Please God, let it be that.

Dr. V found Jayson's first MRI for us and found the right image to analyze the vermis.  He printed it out and handed it to us.  He pulled up his current MRI with the appropriate image to analyze Jayson's cerebellar hemispheres and printed out a copy of that too.  He pointed to where the vermis should be in J's current image.  No vermis.  No separation of hemispheres.  Could it just be smooshed?  He pulled up the first MRI image from when Jayson was 6 months old.  Absolutely the same.  Absolutely no vermis.  There were not two hemispheres, and in this image there was no evidence of crowding or pressure.

It was true.  Jayson has rhomboencephalosynapsis.

My heart sank clear into my stomach and I swore I might vomit right there in the clinic.

There was no denying it.  Jayson had RES.  And it went undiagnosed for six years.  And my baby is not likely going to live a long life.  And I have no idea how I will accept that.

There are different severities of RES and they are dependent on the level of vermis absent.  Many patients have a few pieces missing.  Others have a part of the vermis that formed, while the rest of it didn't.  Jayson, however, doesn't seem to have formed a vermis at all.  Patients with no vermis have the grimmest prognosis.  Even seeing it in writing doesn't help me accept it.  I'm in denial.  This isn't real.  It can't be.

Clearly, most doctors do not know about RES, what it looks like or how to diagnose it.  We were fortunate that our geneticist did have some familiarity.  However, I know he is not the expert.  I have learned in my support group that the expert on RES and other hindbrain and cerebellar conditions lives in Seattle.  I asked Dr. V if it would be worthwhile to pay him a visit?  He claimed he and this doctor are close friends and would be getting together later that week!! He expressed he would mention Jayson and his case to his doctor friend.  I took that as a sign that God is mindful of us.  God cannot replace the missing vermis in Jayson's brain.  He cannot take away my agonizing pain.  But He can stand with me every step of the way.  And I am grateful for that.

I am hopeful that we can visit this doctor in Seattle.  There is no treatment or cure for this condition, but there is much more knowledge to be had.  I need the info.  I have to know as much as possible in order to do as much as I can.  I am still holding on to a glimmer of hope that maybe this doctor in Seattle will look at Jayson's images and see a speck of a vermis, that maybe not ALL of it is missing.  Which could mean his case isn't as severe.  I need that hope.  I need to believe things are not as bad as they seem.  I need to know I'll have my boy with me by my side for many more years to come.  Honestly, I can't live without him.

I can't live in a world where he doesn't exist.

Please God, please don't make me live without my baby.

Please let there be a miracle.  Let there be a way he can spend many happy years with us spinning, eating pancakes and lounging with his feet up on the table.  I need to hear his giggle and see his smile.  I need to hold him forever.  Please God.  Let us make zillions of more memories with those we love.  Let there be time. 

I need time. 

I need him.  I NEED every bit of him. 

I need my Little J here with me on this earth.  No, not even eternity is not long enough.

Please God.  Please.

PLEASE.

Silence

Have you ever been told bad news that needed follow up and further explanation?  Maybe something like, "Your mom is being transferred to the hospital.  She was in an accident." or "This is the school.  Your child had a fall and needs to be picked up from school.  He/she may need to see a doctor."  or maybe "It doesn't appear there is enough money to keep your job position next year.  It depends on our finances."  It sucks to hear bad news and be in limbo wondering if things are going to be okay or fall apart. We had heard it appears Jayson has pressure in his brain that is causing his severe pain and that he needs a lumbar puncture to test pressures.  As a mom, I had a million follow up questions to this information:

How do we know he has pressure?
What evidence did they find?
If we confirm he has pressure, is it looking like he may need another cranial expansion surgery?
Or will he need a shunt?
Are we certain his Chiari is stable?
Why a lumbar puncture?
Why have we never done one before with his previous concerns for pressure?
Why has ICP bolt monitoring been suggested over a lumbar puncture in the past?
Would bolt monitoring give us more information?
Is it possible he may need both a lumbar puncture and bolt monitoring?
Is there a way to avoid doing both?
How can we keep Jayson calm during and after any of these procedures?
If the imaging suggests a build up of pressure over time, what have we missed in past imaging?
Who is heading this up?
Is it time to get an appointment with neurosurgery?
Is my son's life in danger with high icp?
What is our timeline?
How quickly will we move on this?
Our cranial surgeon took another job across the country.  Who should we see now?

None of these questions can be answered by a single person, besides neurology.  Our neurologist should have an answer to all of these, even if the answer is that we don't know at this time.  None of our other doctors could begin to tackle these.  We were at the mercy of our neurologist and I cannot even put into words the heaviness I felt in my heart knowing it was likely my son had pressure in his brain causing his headaches and there was nothing I could do but WAIT for someone to contact me.

We waited.  And waited.  And waited. After 3-4 days, I sent off another email to our neurologist.  After all, he STILL hadn't responded to my requests for him to call us to discuss Jayson's concerning MRI findings.  He sent in the order and said he would call with results.  That typically happens in 24-48 hours.  It had been weeks.  In the email I updated him on Jayson's pain and let him know that Jayson was still suffering all day every day.  I explained how hard it is to hold him while he cries and promise him Mommy is doing everything she can, when all I can do is wait for him to get back to me with information and a plan.  I said I was also still waiting to go over findings in Jayson's MRI including a new diagnosis.  I cc'd our Comprehensive Care doctor, the queen bee, the magician to makes magical things happen.  Then I waited some more, hoping and praying my phone would ring.

For WEEKS I had taken my phone everywhere with me.  To the bathroom, to the school bus to get Jayson on and off the bus, to his school, to work meetings, to other doctor appointments, to therapy sessions, to church, to social gatherings.  I have carried it in my pocket, arm pit, purse or bra absolutely EVERYWHERE as I hoped and prayed for a phone call from our neurologist.

It never came.

After another couple of days, I got an email response from him.  It didn't answer any of my questions and only made my head spin with more questions.  It was brief, only a couple of sentences and said that he was sorry to hear Jayson was still in pain and he believed Jayson needed a lumbar puncture and he would get one set up.  He said he had NOT yet been in contact with Dr. D our neuro ophthalmologist, despite the fact we had been told they were in contact making a plan together. In response to my question about J's MRI findings, he simply defined Jayson's new condition as a congenital malformation of the brain.  Signed, Dr. L.  That was it.  No answers.  No explanations.  In my opinion, no help whatsoever.  What evidences of pressure are there?  Why does he need the lumbar puncture?  Why a lumbar puncture (LP) over bolt monitoring? In our previous experience with pressure our doctors told us a LP told us very little and bolt monitoring would give us the information we needed about pressures.  And I need a whole lot more information about J's new diagnosis other than it's a brain malformation.  I got more information from Wikipedia!

I was FRUSTRATED.  Of course, I cried.  My whole entire world depended on this man.  My son's very life is in his hands.  Yet he can't call me.  He can't answer my questions.  He can't write more than two sentences.  He can't take the time to connect with me or Mike and let us know what is going on in our son's brain and what their plan is to address it.  We have worked with this man for SIX YEARS.  How can he let us down in our greatest time of need?  We know he's busy, but how can anything be more important than my sweet Jayson???

As I struggled to know what to do, I received an email from Dr. M in Comprehensive Care.  She told us to go ahead and contact neurosurgery.  It was just one sentence, but at least it was something.

So I called neurosurgery.  I had not prepared myself for the experience I was about to have.  I should have known better.  Neurosurgery and I have a LOT of history, and it's never been good.  But I reached out blindly and expected it to go well.  It didn't.

I left a message which was returned the next day.  Their not-so-lovely-at-times nurse called me back and our conversation went a little something like this:

K: Hi Mrs. West.  You're going to have to help me out here because there is a lot I'm not understanding.  You are wanting to schedule an appointment for Jayson, correct?

Me: Yes, our doctors are saying it's time to see NS based on recent MRI findings and evidence of icp.

K:  Okay, well you see, it appears Jayson has not been seen since the summer of 2016.

Me:  Yes, that is correct.  He has been stable until recently.

K:  Okay, well he was supposed to come in the summer of 2017 for a yearly follow up, but you neglected to make that appointment.  Since you neglected the care of your child then, I'm not understanding the urgency for an appointment now.

Me:  (My brain: oh my hell, did she really just say that??!!!)  {in tears}  Oh, well I don't think I knew we were supposed to come in for a follow up.  No one called me.  I didn't receive a post card.  And our care coordinator never brought it to my attention.  You see, I had just had a baby last summer and I was recovering from severe pre-eclampsia.  I was told to keep my summer free of unnecessary appointments and care for myself and my preemie baby.

K: I see, well since you neglected to make that appointment I'm not certain you can see Dr. B.  Besides, this sounds like a cranial issue to me so you should see your cranial doctor, not neurosurgery.

Me:  Well over the summer last year Jayson's headaches came back and we took him to see his cranial doc who only measured his head and said he was fine.  Now we have imaging that says differently and that he had pressure then and now.

K:  Oh, so he was having problems last summer and you still didn't make an appointment.

Me:  We saw his team who said we didn't need to see neurosurgery.  We increased one of his meds and it helped for a couple of months.

K:  Well, I'm not sure how we can help with all of this.  We will probably need another referral and if his pain is managed by medication now, then I don't think he needs to...

Me: (I interrupted her very angrily)  Will you just LISTEN?!  The med only helped for three months. His pain came back in December.

K:  Why didn't you call us then?

Me:  I'll explain if you just LISTEN!  His team thought it was his ears.  He had a bad double ear infection.  It took weeks to get surgery to switch out the clogged tubes.  Then we were told we had to give him time to heal.  The pain persisted so neuro ordered an MRI.  Now the MRI shows pressure.  We've heard conversations are happening behind the scenes but we haven't directly heard from our neuro.  But Comp Care wants us to see Dr. B in neurosurgery.

K:  Okay, well I don't think you can see Dr. B.  But you also see another neuro surgeon Dr. K with his cranial doc.  It sounds like it is his craniosynostosis.

Me:  We don't know that.  How could you know that?  I don't know that because no one has talked to me.

K:  It sounds like you don't really know what is going on.

Me:  No, actually I don't.  That is why we need to see neurosurgery!  And Dr. M said we should make an appointment.

K:  Well, I don't know about that.  I think it's best if I message your doctors and see if they can decide if Jayson needs to be seen and which of his two neurosurgeons he should see.  Does that sound good?

Me:  Yes, I hope they respond soon.

K:  Me too, have a nice day.

My blood was boiling.  But instead of yelling, what did I do?  I cried.  A lot. At work.... in front of a teacher.  Thank goodness we had a relationship and she knows what is going on with my son.  I was embarrassed but I couldn't keep it in.

How is this real life??  How is this okay???  How is this considered GOOD CARE?!  What about Primary's motto "The Child First and Always"?!!  How is this crap happening AGAIN?!?!?!

I was up in arms and felt so helpless.  Neuro wasn't responding.  Comp care was only deferring to neuro and telling us to get in to see Neurosurgery.  Neurosurgery was saying I missed an appointment and my child may not be able to be seen.  I didn't know what to do.  Whenever I have been in this desperate situation I have cried to Dr. M in Comp Care and she makes it okay.  She hasn't helped much yet, but if she knew all that was going on I knew she would help.  She always had.  So instead of writing her another desperate email, I decided to call.  I called and left her a message with her nurse who said she was in meetings all day and would get this message to her ASAP.  Hopefully she would call me soon.  In the meantime I may need to consider taking my son in to the ER to be admitted.  Things would definitely happen then.  I didn't want to expose him to a million of other things, so I just prayed that Dr. M in Comp Care would get back to me soon.

I wish I could end this post stating we got in touch with our neurologist, but we didn't.  I wish I could say that we reached out to neurosurgery and they got us in to see them and we now have answers.  But we didn't.  I wish I could say that once again our Comprehensive Care doctor came to our rescue and helped all involved parties communicate.  But she didn't.  I wish I could say that this experience with Primary's, our doctors, and a crisis was DIFFERENT than our previous experiences, but I can't.  But what I can say is that this frustration triggered so much PTSD it has made it hard to function.  It's like Groundhog Day and I'm reliving a nightmare I can never wake from.  I was stuck.  No where to go, no one to go to.  I knew my son was in constant pain and the cause was likely pressure, which is life threatening, and no one out of our NINETEEN doctors and specialists was stepping up to help us.  This is the kind of stuff you read about or watch on TV.  But I was living it.  AGAIN.  And my son was the one suffering.  It's not okay.  It's never been okay.  Our hospital and doctors can do so much better.  They need to do better.  We HAVE to do better, for Jayson.  He deserves better.

Don't Poke the Bear

I often write with a lot of positivity, hope and optimism.  But sometimes this journey isn't so positive, hope can be hard to hold on to, and optimism is unrealistic.  I really believe in keeping it real, and there have been a significant number of REAL moments lately.  The kind that frustrate, the kind that break you, the kind that make you question everything.  Consider this a REAL special needs mama post.

I've been told I have the patience of Job; I have a lot of patience with Jayson, this medical journey, incompetent people, etc etc.  To be honest, you almost have to have a ridiculous amount of patience in order to stay sane in this special needs life.  Doctors offices, medical billing offices and home health are some of my most frequent numbers called on my phone.  As anyone with experience with these entities knows, very little gets done in ONE phone call with a happy, perky tone of voice.  The same is true for doctor appointments.  I feel like sometimes little can get done until Mama Bear starts to growl.  Well, this mama's teeth are showing...

We had a WONDERFUL visit with our new neuro ophthalmologist two weeks ago.  She gave me hope and comfort that we were going to figure this out and get J's pain under control.  I'm so grateful for that, but the hope and optimism wears away quickly after another few days of seeing your child in pain all day every day.  Answers can't come soon enough.  I don't have much patience when it comes to J's suffering.  I was very glad that J's ophthalmology appointment was shortly after this neuro ophthalmology appointment since Dr. D (our neuro ophthalmologist) was waiting on Dr. J's (our ophthalmologist) examination and thoughts to decide exactly what to do.

The good news is that Dr. J is FABULOUS with Jayson.  He has a way of getting my very challenging child to cooperate with a very challenging examination.  He felt he got such a great look at his optic nerves in the appointment, and that we don't need to put Jayson under anesthesia, which I'm thrilled about!  What doesn't sit well with me is when a doctor questions me and my perceptions as J's mother.  What makes me growl is when a doctor questions my son and his ability to communicate his pain.  We have worked so damn hard for so damn long to get to this point where Jayson can communicate some things with us.  Among these things, the most important to me is J's ability to communicate his pain.  I don't take kindly to doctors discrediting that or questioning his ability to communicate.  NOT. AT. ALL.  I also don't take kindly to doctors questioning other doctors in front of me, without conversing with them.  J's examination showed no optic nerve swelling.  FABULOUS news.  This typically rules out intracranial pressure.  TYPICALLY.  But when has my child every been typical? When I asked Dr. J what would explain the fluid investing the optic nerves bilaterally, he responded that there is no way there is fluid behind in the nerves.  He said the radiologist must have over reached or misinterpreted the images to appease the mother who is voicing complaints of eye pain.

Oh no he didn't.
He did NOT just say that a radiologist overreached to appease ME and my complaint of eye pain.
Holy. freaking. crap.

This doctor has NOT read the MRI report.  This doctor has NOT reviewed the MRI images.  This doctor is NOT trained to interpret MRI images.  Yet, he had the audacity to state that this very skilled radiologist must have over reached or misinterpreted the findings... and to appease ME!  I informed him that the neurologist only listed head pain as the reason for imaging, not eye pain. The radiologist had no idea Jayson had been experiencing eye pain.  Dr. J stated that there must be some mistake because if there is fluid on the nerve there is almost always intracranial pressure, and if there is intracranial pressure there is almost always swollen optic nerves, and J's nerves were not swollen.  I informed him that Jayson is NOT typical, and he tends to be part of the small percentage that is the exception.  Dr. J is not typically our ophthalmologist.  This is the second time we have seen him since ours has been out on maternity leave and has a long wait list to be seen.  So I reminded him of Jayson's past, that he has had long-term severe intracranial pressure with completely normal eye exams without swollen optic nerves.  I reminded him my son had to have a cranial expansion surgery to relieve the pressure and that parts of his skull were completely worn away from the pressure inside his head YET his eyes looked fine.  You're not going to like what he said next.

"How do we KNOW he had pressure?  Did he have a lumbar puncture?  Did he have intracranial pressure monitoring?  No, he didn't. We don't know for certain that he had pressure since these tests were not done."

WOW.  Just wow.  Apparently imaging, symptoms, my son's skull being worn away and my doctors cutting open his skull and expanding it were not enough evidence for him.

I honestly could not believe this doctor appointment that was supposed to give us answers had taken such a bad turn.  I could not believe that a doctor who could work so well with J could struggle so much to listen to me, his mother, and trust in Jayson's other doctors.

I don't like it, but when I get mad I just start to cry.  I can't help it.  I want to yell.  I want to fight.  But instead I cry.  So I cried.

In tears I told him about J's pain.  I narrated a typical day for Jayson.  Not only does he rub his head a couple of times a minute like Dr. J had clearly observed in clinic, but almost daily he breaks down sobbing from the pain.  My son has had two brain surgeries and two cranial surgeries, without crying.  So he's experiencing pain that pushes him beyond his threshold.  That's noteworthy.  I told him about how J's school has called and had me come get him because he was in too much pain.  I told him about how fed up I was with doctors punting him to another specialist or blaming Jayson's behavior or sensory issues as the culpruit because they were too busy or unwilling to look beyond typical causes for his symptoms.  I told him that I can't continue holding my son, while he sobs and rubs his head, and promise him things are going to get better.  I depend on doctors to keep that promise, and they aren't willing to help ease his pain.  He gave the whole, "I'm a dad too, I can imagine how that might feel speech" and I just shut down.  This is also what I do.  I shut down as a coping mechanism and wait until I'm alone to completely fall apart, cry, scream, and cuss a lot.

Fortunately I made it to the car before I fell apart.  As I was getting Jayson in his car seat he grabbed my hand and put it on his head.  I know, baby.  I know.

I called my bestest friend in the whole wide world on the way home, my sister.  She is often my voice of reason.  She comforts me, toughens me up, encourages me, cries with me and knows me better than anyone.  I honestly didn't expect her to become ever angrier than I was about the whole experience.  But she works with radiologists and knows how highly competent and respected they are.  How dare this doctor discredit what a radiologist saw on J's imaging.  It wasn't opinion.  It wasn't interpretation.  It was something he saw on J's imaging. Fluid is clear on imaging.  It is not easily mistaken.  How dare this doctor criticize a professional radiologist and his findings, particularly when he had not even looked at the images himself.  How dare this doctor discredit our other professionals who have worked with Jayson for years.  My sister validated my frustration and said I needed to make a complaint.  This wasn't okay.  She was tired of this.  I was tired of this.  Why does absolutely everything with Jayson's care have to be a fight???!!!

I came home and wrote most of this blog post to cool down and collect my thoughts. I didn't finish it then, so I didn't post it.  I was interrupted by a phone call... by this doctor.

I was surprised to answer and have Dr. J on the line.  Hadn't he had enough?  I certainly had.  I heard him say, "Hello Mrs. West. It's Dr. J.  I just got off the phone with Dr. D (neuro ophthalmology) and I wanted to give you an update of what she says..."  I closed my eyes and took a deep breath.  I knew what was coming.  More of what he dished out in the clinic.  I was certain he was calling to boast and say Dr. D agreed with him and Jayson is fine, the pain is behavioral, he doesn't have pressure, and the images aren't accurate.  I prepared myself to hear this doctor pat himself on the back and tried to preemptively think how I would respond.

But I was wrong.  Nothing in the world prepared me for what I heard next.

"Dr. D met with a team of radiologists and neuro-radiologists last week.  She cares very much about your son and took the time to coordinate this meeting.  They all spent 30 minutes going over Jayson's imaging, both current and past, and they believe there is a lot of evidence to support that Jayson is experiencing intracranial pressure."

My heart sank.  I felt like it stopped beating, honestly.  And like I had been gut punched.  I wanted to say something, but I couldn't because I felt like that air was knocked out of me.

He continued, "There is a lot of evidence in the imaging over time to believe that Jayson has had pressure in his brain for some time, and it is building.  They think this is causing his pain.  And I was wrong.  They confirmed your statement that Jayson has never had swollen optic nerves even with intracranial pressure.  He is a complex case.  What I failed to relay to you is that swollen nerves is just like one out of maybe ten signs of intracranial pressure.  Jayson has many of the others.  I was wrong to imply that it was the only way to tell if he has pressure."

I knew I had to talk.  I had to find a way to respond.  The pressure in my throat and my chest started to move, but it triggered the tears.  Trying not to sob over the phone I managed to say, "Okay.  This is all information that no parent wants to hear.  But I appreciate the call very much. I appreciate the update and that we are starting to figure this out.  This is a much different outcome than I expected.  So what do we do now?  Is someone supposed to contact me?  Dr. D? Our neurologist?  What do we do now?"

He responded, "This is not my area of expertise.  I do want to do a more thorough exam when he is under anesthesia, which is sounds like should be soon since they plan to do a lumbar puncture to test Jayson's pressures in his brain (spinal tap).  I would suspect either Dr. D or your neurologist should be contacting you in the next day or two.  I'm sorry.  I wish Jayson well and will plan to examine him when he is under anesthesia.  If you don't hear from anyone soon, call me back and I will try and message your docs and facilitate some conversation."

My head was spinning.  I was so full of emotion that I couldn't process it all.  All I could do was sob.

The doctor was wrong, and he admitted it.
My son was not okay like he said.
A team of doctors sat down together to talk about my son.
Jayson likely had intracranial pressure.
Pressure in his brain is likely causing his symptoms.
Pressure usually means brain or cranial surgery.
I still haven't heard from our neurologist since the MRI.
When we he call??
What are our next steps?
How can Jayson possibly endure a spinal tap?
How can Jayson be expected to endure even MORE?

It was all too much.  I crumbled, collapsed, and just cried out every emotion until my body couldn't produce any more tears.  I knew I needed to be strong for Jayson, but not now.  In that moment, I was just broken.

Restored Hope: The Power of a Skilled Physician

I've been distant the past couple of weeks.  I haven't wanted to come to terms with our current situation and I'm still not fully aware of the significance of our testing results.  I'm not ready to share all of the details, but I do want to provide an update.

Jayson's imaging was completed a few weeks ago and we have access to the reports.  His CT scans revealed he does not have Superior Canal Dehiscence and has some problems with his facial nerves and his cochleae, neither of which can explain his pain and sensitivity to sound.

His MRI report was hard to digest.  The first thing I noticed and celebrated was that it was evident there were not signs of intracranial pressure, meaning his Chiari was stable and no need for another cranial expansion!!!  However, there were several new findings with conditions I had never heard of nor could ever pronounce without practice.  As I hit the world wide web for resources, I found little and discovered one of his new conditions was ultra rare.... 50-60 cases since the year 1914 rare.  And according to the research articles published, the outlook and prognosis are not good.  I clearly began to panic and started the balancing act of becoming informed enough to be able to have a good conversation with the doctors without becoming the crazed, emotional Google mom.  I reached out to our neurologist who said he would contact us with results, but despite several efforts we still have not heard from him.  Another troubling MRI finding indicated we needed to see neuro ophthalmology.  Coincidentally, we had already been referred again and had an appointment in just a couple of weeks.  It was not clear whether these other findings could explain Jayson's head, eye and ear pain and discomfort.  I was desperate to hear from our specialists, because in the meantime Jayson has continued to have daily, chronic pain.  His teacher called a week ago frantic saying she had never seen Jayson in so much pain before and we had to go get him.  We have observed Jayson rubbing his head, eye and/or ear from several times an hour to several times a minute while he tries to keep himself busy and distracted.  We have been awakened by his grunting, groaning or monitor alerting us he was in pain in the middle of the night.  We have watched and listened as he desperately tells us, "I hurt head eye," on nearly a daily basis.  We make promises to Jayson we may never be able to keep, letting him know Mommy and Daddy are working with doctors to find his pain and to fix it.  There is no feeling like it in the world to watch your child be in chronic pain and promise them you'll take it away, knowing you very well may never be able to.  As a parent, my thoughts have been consumed by my worry and fear.  My productivity at work and in my extracurricular activities has greatly been affected.  How do you continue to do life's daily tasks knowing that nearly every minute of every day your child is in pain?  It's impossible.

Jayson's sedated ABR hearing test resulted his hearing has remained the same, mild low frequency hearing loss in his right ear.  However, the audiologist said his ears looked terrible!  A lot of redness, inflammation, drainage and blood.  She said it looked recent, not from his tube surgery.  She encouraged us to contact our ENT as soon as possible and to get in with our pediatrician on Monday.  We did and sure enough, one of Jayson's ears was infected... again.  We began drops and then Jayson caught a cold, which always end up in double ear infections.  A couple of days later the drainage and severe pain began and we started oral antibiotics via G-tube again.  I was fed up.  This was literally one ear infection after another since December, even after replacing the clogged tubes.  We keep using the same antibiotics!!  What if it's not treating the infection?  Why haven't they cultured the infection?  I called ENT and they got us into an urgent appointment, which took place yesterday.

Yesterday's appointment was among our most discouraging appointments ever.  The ENT examined Jayson and told us his ears looked perfect.  No sign of infection.  No inflammation.  No issues.  Yes, he was still on antibiotics which was likely treating the pre-existing infection, but why was he still grabbing his ear all day long and hollering out in pain??  Why was he struggling to sleep, and participate in school and therapy?  I asked the ENT these questions.  Instead of thinking, hypothesizing or collaborating, he simply said it had nothing to do with his ears.  I mentioned the couple of abnormalities found on his CT scans and asked if these can cause pain.  He said no, and the findings were insignificant.  I asked what else it could be, pleading with him to throw around some ideas and brainstorm with me.  All I got was, "It's not his ears.  I can say that confidently.  It's a neuro issue."  When I informed him that neurology and other specialists have indicated them believe this is an ear issue, he stated his ears look perfect and were not even that bad when he replaced his tubes.  I asked about facial nerves, sinuses, any other explanation and he simply said this had nothing to do with his specialty and that Jayson is a complex kid.  Maybe it was sensory or behavioral??  When I asked how that would explain the pain in his sleep, he admitted it wouldn't. He told us to follow up with neurology.  That was it.  No ideas.  No questioning, pondering, wondering or thinking.  What his short answers and body language told me was, "Because Jayson can't tell us, his pain isn't real.  It's not worth my time."  My mommy heart sunk and I had a lump in my throat.  I threw out one last desperate question with tears in my eyes, "But what could possibly be causing his pain?" to which he responded, "I know it's hard.  Jayson is complex.   He is his own person.  Summer is almost here,  Perhaps that will be a better season for him."  His passive comments physically hurt and I felt isolated, alone and desperate.  This physician is one of Jayson's key team members.  If he wasn't willing to help figure this out, who will?

I left feeling sad, defeated, frustrated and angry.  How different would this encounter have been if Jayson could have talked?  What if Jayson could tell him, "No, I'm not making this up.  Off and on throughout the day my ears hurt really bad! I can't concentrate, play or sleep.  They are bothering me all day every day.  I don't cry often and I can handle a lot of pain and surgeries without pain, but sometimes my ears, eyes and head hurt me so bad I just cry."  I doubt the doctor could look him in the eye and tell him, "It's not your ears.  I can say that confidently.  It's a neuro issue.  Maybe it's sensory or behavioral.  Maybe summer will be better for you."  It is heartbreaking how differently our non-verbal children are treated because they cannot communicate in traditional ways, all because it takes effort and time to understand what they have to say.  I wish so much things were different and all doctors were willing to listen to Jayson, even when he cannot communicate with his words.

It was hard to feel so down after the appointment and realize that one of my biggest fears may become a reality.  We really might be told by Jayson's doctors that Jayson's chronic pain is his new normal and there is nothing we can do.  The thought made me physically ill, and greatly increased my anxiety about the following appointment.  The next morning (today) we would have an appointment with neuro ophthalmology.  We saw a doctor in this specialty when Jayson was just a few months old and it was NOT a positive experience.  We were insulted and my son's conditions were very quickly dismissed, and he was even more medically fragile at that time than he is now.  I worried we were putting ourselves in a situation to be treated this way again.  And after our ENT appointment, I feared it was even more likely.  I had been holding onto hope the past few weeks that maybe THIS was the doctor who could give us answers.  Maybe THIS doctor could connect the dots and offer solutions no one else had thought of.  Maybe we could learn more about Jayson and how his brain and eyes are affecting the rest of his body.  When it was just he night before the appointment, I didn't want the next morning to come!! I didn't want my hopes crushed nor did I want another doctor telling my son his pain isn't real or doesn't matter.  I feared if that were to happen I would snap.

I woke up this morning both extremely nervous and excited.  I chose to hold on to hope over fear and I said a long prayer this morning begging my Heavenly Father to help this doctor listen and truly see Jayson.  I pleaded for Heavenly Father to help her to offer us something positive such as an answer, an explanation, a connection, a treatment, anything.  As we were taken back to a room, we first visited with a nurse and then a resident doctor.  They were both very attentive, detail oriented and really seemed to care.  The resident had good questions and took careful notes.  He was already familiar with Jayson's extensive background and didn't ask me to relay his long history.  He knew exactly what to ask and what details would be important.  He left for a few minutes to converse with the doctor before she came in.  Dr. D and the resident had already taken some time to look up Jayson's recent MRI and review all of the images.  She asked for the resident to recount what he had learned about the patient and she listened intently and asked us clarifying questions.  She watched my videos demonstrating Jayson's behaviors indicating pain.  She immediately started throwing out ideas, all things I had never heard of before.  The resident took notes as she asked more questions to narrow down her ideas.  Some of my responses seemed crazy and I feared her reaction.  She asked, "How long are Jayson's episodes of pain?"  "It is weird, but they seem to last just a few seconds.  But they cluster," I responded.  She validated me by telling me that was very helpful information.  She mentioned a couple of conditions that come with sharp, quick pain to the head, eyes and ears.  She asked, "So how often do they happen?"  I answered, "All day every day right now.  Some days are worse than others.  On a bad day he can grab his head or eye a couple of times a minute when they cluster.  Otherwise several times an hour."  She responded, "So you could say hundreds of times a day?"  I got nervous.  That sounds crazy, right?  "Yes, I guess so... "  "That can happen," she said.  "This is very helpful."  I showed her videos demonstrating how some are just subtle rubbings of his head and eye during regular activity, while others he is crying, screaming and hand flapping while grabbing at this head, eye, neck and ear.  After seeing the video she turned to me and grabbed my arm and said, "You are a very good mother to notice this.  Some of these are subtle. It is wonderful you have paid close attention and caught this so soon.  Many would not have noticed."  I was shocked to hear such a compliment when some doctors have accused me of worrying too much and reading too much into Jayson's behaviors.  I said, "I have to be, he's non-verbal.  I have to observe him and be his voice." "Yes, but it is to be commended.  You know your son well."  My heart swelled.  How wonderful it felt for her to notice and appreciate my attentiveness.

She told me this was going to take collaboration.  She typically works with adults.  She told me she is not a pediatric doctor and would never pretend to be.  She told me she would not dare to jump in and start prescribing medicines or tests without talking with my son's specialists.  She asked for their names and told her resident to have the assistant begin looking them up, contacting them and getting them on the phone asap.  She said this would take a collaborative effort and she would be driving the conversation.  She also said she needed time to get with her team and go over all of Jayson's MRI and CT imaging, looking to see what might have been missed and compare previous images with current ones.  She mentioned a couple of specific things she was looking for that may or may not show in Jayson's imaging.  If it's not there, she may want to order more detailed imaging to ensure with confidence it isn't there.  She told me above all else, she needs to prove with certainty that this is not intracranial pressure and that will take time and more tests. 

Wait, is this really a doctor who didn't feel she knew everything?  A doctor who wants to collaborate with others despite the challenges?  A doctor who didn't want to dismiss Jayson due to his complexities and inability to speak, but rather would like to investigate some possible conditions and treatments?  A doctor who saw his pain, even the subtle evidences, and said it was not okay for him to suffer?  A doctor who listened when I said there was a change beginning in December and is diving into what could have caused that change?  A doctor who acknowledges that things can be missed in imaging and is willing to comb through it with a team?  A doctor who admits that intracranial pressure can exist and be missed even if an MRI report says it is not present and a standard eye exam does not discover optic nerve swelling?  Does a doctor like this seriously exist????  This woman is exactly who I had hoped and prayed for.  EXACTLY.

So she came up with a plan and she began acting on it immediately.  She wanted my neurologist on the phone, now.  She sent messages to my doctors right away.  She made a recommendation for a new medication for our neuro to review and hopefully prescribe immediately.  She suggested a couple of tests to run if they couldn't find what they were looking for in Jayson's current imaging.  She said she would personally contact Jayson's ophthalmologist who we see on Monday to let him know about our visit today and what she is looking for and recommending.  She told me she will see this through and communicate with our involved parties.  When I asked her when we needed to follow up or what my next steps would be to ensure everyone is following through she responded, "I will be coordinating this.  I must rely on your pediatric doctors because I am not a pediatric specialist, but I know what we are looking for.  My focus is on finding the source and managing your son's pain and I am confident we can do that.  That is my job and I will need the help of your specialists to make it happen, but we will get it done.  We will get his pain under control.  That I know we can do."  Tears filled my eyes.  I wanted to jump her and give her the biggest mama bear hug imaginable.  But I held myself back.  Instead I gave her an awkward arm rub and said she can never know how much those words mean to a mother of a child in pain.

And that was our visit.  Honestly, out of this world.  One of our largest blessings to date.  It was so very refreshing and inspiring to have such a wonderful doctor visit with a doctor and team who had familiarized themselves with Jayson's records, weren't intimidated by his complexity, brainstormed ideas and challenged themselves to think outside of the box, pieced together clues and information I gave them, listened like REALLY listened, came up with a plan with several options, communicated immediately with other team members, had a follow up plan and a solid goal to relieve my son of his daily head, eye and ear pain.  I guess today I'm grateful this apparently "is a neuro issue" because this is the right woman to be figuring it out (even though the irony is that one or her main suspicions is an ENT issue). 

After today, I am hopeful we will figure this out.  I am hopeful my son will know what it is like to have a pain free day again.  I am hopeful that we will get one or two steps closer to really understanding Jayson and his complex body.  And I am hopeful that my son is finally being heard and his {silent} voice matters.  As we got in the car, Jayson's daddy told him, "The doctor listened and cared Jayson.  She is going to help your head and eyes feel better." The big smile that spread across his face at that moment will stick in my mind and heart forever.  His response confirmed what we already know-- He knows what is happening.  He knows when he needs help.  He knows who is trying to help him.  And he knows Mommy and Daddy are doing everything they can to keep their promise.

And we won't stop until we do.

I am sharing this short video of a few clips demonstrating Jayson's pain in case others might have some ideas.  Please feel free to comment or message me if you have some suggestions or insight.  Thank you!

When Hope Seems Lost

I don’t how we got here. How did we get to this place where we are looking for answers again? Just a few months ago I was crying daily happy tears as I watched my son enjoy life, learn and thrive! I knew in my heart it wouldn’t last forever and I treasured every single moment I didn’t live in worry and fear. I soaked in every memory of my happy boy enjoying life with minimal pain and symptoms. But how did we get to this place of worry, concern and pain again so quickly? Like a light switch things went from enjoying the best in life to fearing the worst. Seriously, what happened? And can we ever get back to that amazing place of comfort and bliss? I don’t know... I really hope so.

Starting last summer J started showing signs of frequent headaches and migraines. We’ve seen this before and we typically make our rounds to the specialists, try some things that don’t work, and then one day things get better for a few months. So we made our rounds and the docs said to hold tight. We increased his med gabapentin and things got better... for about 2-3 months. Then his headaches and migraines came back with a vengeance in December. His head an ear pain was incapacitating. He couldn’t go to school, get through a session of therapy, or get through his day without crying in pain. My son has a ridiculously high pain tolerance, so we knew things were bad. He had one ear infection after another with tons of blood, pus and possibly a ruptured ear drum. We learned his tubes were clogged and not functioning so he went into surgery for new T-tubes to relieve the pressure and clear the infection. Slowly things got better, much better actually. We crossed our fingers and hoped that his headaches were brought on by ear infections. But  it seems they weren’t.

I was thrilled J was doing so much better! He could go to school and function again! He enjoyed therapy and could get through his daily sessions again. He was mostly happy. But every day he still had moments of rubbing his head and eye, whining, grunting or crying. This is what he had done in the past and docs have said we don’t know if for certain it’s his head. It could be referred pain or behavioral. Except now, we do. Since Jayson started communicating on his iPad in September he has been able to tell us when he is in pain and where. It’s been eye opening and life changing, but also heartbreaking. Nearly daily Jayson is communication to us, “I hurt. Head. Eye. Medicine.” It’s usually combined with the head rubbing and whining so we believe he is telling the truth. His head and his eye hurt. A lot.

Doctors have surprised me at how much they care about Jayson and how he is feeling. Now that he has a voice, he is being heard. I was prepared for a long battle of taking pictures and videos over months to convince  them my nonverbal son with a high pain tolerance is in fact in pain. But it wasn’t needed this time. In fact, their concern has thrown me for a loop. For perhaps the first time on this journey, they seem more concerned than I was. As I saw several of them over the last month and a half, I have described various symptoms and concerns, telling them it probably sounds crazy that he’s having so many things go on right now that seem unrelated. They have explained that they don’t think they are unrelated. They’ve explained the many functions of the brain stem and how essentially all of my concerns could be tied to brain stem compression, or chiari. No. Just no. I’ve convinced myself for my own sanity that J’s Chiari is stable, especially after cranial expansion surgery. I told myself this isn’t going to be an issue anymore. But what if it is?? It takes my breath away to think about.

Let me go back and explain our past few appointments and situations:

Back in December Jayson had ear infection after ear infection.  He was miserable, constantly crying and blood and pus were continuously draining out of his ears.  When this was happening, I repetitively told Mike, “Something is different this time.” We’ve dealt with dozens and dozens of ear infections. Nothing was like this time. None have ever seemed so painful. Usually we discover them when he’s acting like nothing is wrong! I wondered why things were so painful this time and planned to ask the doctor at our ENT appointment in February. As I prepared for the appointment, I remembered that my new friend, who is the mom of the other boy with J’s Syndrome, told me to ask about an ear condition. She said her son was diagnosed with it and it causes major balance issues as well as ear and head pain. I decided I better look it up to have some background info to ask my doc about it. Superior Canal Dehiscence. As I read through the description and symptoms, my eyes filled with tears. Everything fit. It would explain so much!! It could explain the ear and the head pain and symptoms. It would explain nearly all of the same things a brain stem compression would. It was a real possibility in my mind, so I prepared and collected medical records for hours so our ENT could see that this is a real possibility. The day of our appointment Jayson was sobbing in pain for over an hour, holding his ears and head. It was heartbreaking and so difficult to get him to the doctor in that state. But the doctor saw first hand what we were dealing with. We talked about ear tubes right away. I hesitantly mentioned Superior Canal Dehiscence wondering how I could reach my notes with an inconsolable, thrashing child in my lap. But I didn’t need them. The ENT was familiar with the condition and felt it was worthwhile to screen for it with a CT. I felt so relieved. With more answers, a diagnosis and more communication, things were becoming easier. Everything isn’t always a fight anymore. It’s so relieving.

So we had a plan to do a CT on each ear looking for Superior Canal Dehiscence the next time J was under anesthesia. That ended up being soon with his ear tubes, but we couldn’t fit the CT scans in then since J was an urgent add-on. I was worried about not having answers about his ears since our next procedures could be months or a year away!! But I tried to tell myself that it wasn’t urgent and we could just wait.

We were referred by ENT to audiology to check J’s ear tubes. Sure enough, they were both clogged. The audiologist confirmed what I had read— there is little more painful than having Superior Canal Dehiscence and no way to relieve pressure through open canals. She said that could explain why these rounds of ear infections were so much worse. His ear tubes were completely clogged. Maybe this was leading us to answers. She said he also failed his clinical hearing tests, which he often does. His behavior concerns and his inability to verbally respond and follow directions make it difficult to test him.  So the audiologist ordered a sedated ABR hearing test to get a better look at his hearing.

Jayson had a terrible fall at school that no one witnessed.  The teachers said he likely lost his balance while trying to stand (as he often does) and hit his face on the chair or table.  He split his lip open and lost a tooth.  We had to quickly take him to the hospital where we discovered he needed stitches and some teeth pulled since they were misplaced.  Fortunately, we had ear tube surgery scheduled the next morning.  They coordinated things so the ENT could stitch up his lip and a dentist could come in and assess his mouth and pull 3 of his teeth while we were doing the ear tube surgery.  It was quite the traumatic episode, but Jayson was brave as usual.  After J's ear tube surgery things got much better like I mentioned and he healed quickly from his accident.

A couple of weeks later I prepared myself for our neuro appointment. He had healed from his ear tube surgery and things were better, but he was still in daily pain.  I had videos and pictures cued and a list of concerns ready. I wanted to discuss another brain MRI, not knowing whether or not it would be necessary. After just beginning our conversation, he told me he and two other of J’s doctors feel an MRI is necessary. I didn’t have to ask. I didn’t need evidence. His symptoms were concerning enough they spoke for themselves and it scared me. He said he wanted the MRI sooner rather than later. That scared me even more.

Another one of J’s doctors candidly explained J fits the mold for a child with a recurring Chiari and brain stem compression. He said he’s glad our neurologist ordered and MRI. If he hadn’t, he would be asking about one. He said if the results are questionable and doctors are fearful of risks for a second decompression, he has seen so much benefit from decompression. It should be something J’s docs should really consider. How are we having a conversation about brain surgery??? How did we get here?! I left that appointment so grateful for the candid comments of a doctor we love, but with so much fear in my heart.

So here we are today at Primary Childrens getting a brain MRI, 2 ear CT scans and an ABR hearing test. We've had so many of these tests. Too many to count, honestly.  This is his 11th or 12th MRI, his 6th or 7th CT scan and his 4th or 5th ABR.  So why does it feel so different this time?  Why do I feel like an elephant is sitting on my chest?  Why can I not feel hope no matter how hard I try?  I've thought about it, and I think I understand why things are so different this time, why my anxiety is so high and why I can't find something to hope for.

There is no ideal situation here.

There is nothing specific to pray for and nothing I really want to hope for.

Every possible outcome to these procedures feels so very heavy, sad and hopeless.

In the past I have hoped and prayed for clear imaging, that nothing concerning will be found.  But it's different this time. If Jayson's MRI and CT scans show that his Chiari is fine, that there is no evidence of pressure and no change since his last MRI, and that his ears are typical then WE HAVE ABSOLUTELY NO EXPLANATION FOR HIS DAILY HEADACHES AND SUFFERING.  I personally know decompressed Chiari patients who are on disability and live a painful, miserable life with daily headaches.  I don't want that for my son.  I want his headaches to be fixable.  I want there to be an explanation.  I want to be able to tell him the pain won't last forever.  But if absolutely nothing shows up on his scans, I can't tell him that.  I fear and believe that if everything checks out okay, his doctors will just say he is prone to chronic headaches and migraines and nothing will change in his life.  I can't feel good about that.  I can't celebrate clean scans knowing it means there is nothing we can do to ever relieve his pain since we don't know the source.  That feels heavy, discouraging and hard.  

There have been times I've prayed for something to be found on imaging, so we would have an answer and treatment plan.  But that was before I knew how horrific and traumatic the answers, treatments and surgeries are.  Knowing how risky and painful Chiari decompression surgery is, I absolutely do not ever want to repeat it.  I've known kids and adults who have died during surgery.  I've known others whose surgeries resulted in brain damage or even more pain.  I don't want any of that for Jayson!!  We got lucky that his first surgery went as well as it did.  I now know and understand the risks.  I now know how terrifying the surgery is and how painful and hard recovery are.  I really don't want to live that with Jayson again... ever.  But I also know it's likely.  The research shows the likelihood of a child needing a second decompression surgery greatly increases if they had their first surgery early in life, if they have a craniofacial syndrome with craniosynostosis and if a stent was never put in the brain.  Unfortunately, all three factors apply for Jayson.  We know this is a real possibility but I want to avoid it as long as humanly possible.  We also know how traumatic Jayson's cranial expansion surgery was.  None of us will ever be the same because of that surgery.  Just thinking about the possibility of Jayson needing that surgery again is enough to make me start vomiting.  I can't think about it without having a panic attack.  Yet, we know it's likely he will need another one.  His doctor told us his brain will be full size between ages 8 and 10, and many children need at least one more surgery at or around that time.  I don't want this.  I never want this.  I don't want his scans to be clear and have no answers, but I definitely don't want anything like these answers.  I don't know how we would all get through this again.  My boy shouldn't ever have to endure any of that again.

So then, logically, I should be able to hope that he tests positive for the ear condition Superior Canal Dehiscence, right?  It could explain his pain and symptoms, but it isn't Chiari or intracranial pressure.  That should be what I pray for.  But no.  As I've learned more about this condition, it would be like receiving a life sentence of complete and utter misery.  Let me copy and paste some descriptions of symptoms with SSCD:

Vestibular symptoms and signs: The vestibular symptoms in SSCD can be debilitating and often provoke patients to seek medical attention. Patients may note that loud noises cause them to see things moving or that they experience a similar sensation when they cough, sneeze, or strain to lift something heavy. They may perceive that objects are moving in time with their pulse (pulsatile oscillopsia). Some individuals can bring on the sensation of motion—and cause their eyes to move—by pressing on their tragus (the area of skin and cartilage located just outside the ear canal). Patients may experience a feeling of constant disequilibrium and imbalance.

Auditory symptoms and signs: Patients with SSCD may also complain of symptoms such as hearing their eye movements, hearing their own voice too loudly in the affected ear (autophony), or having a distorted sensation of sound in the affected ear during activities such as running.

Jayson has both vestibular and auditory symptoms that lead us to believe this is a possible diagnosis.  His recent fall has finally helped his doctors realize his balance issues are in fact a big deal.  And we know his ears and hearing have been affected and sensitive.  As I read about this condition and watched videos of patients describing their lives, it broke my heart.  It is painful and very disabling.  Although this condition isn't life threatening, I really don't want this for my boy either!!!  There is a surgical treatment but it is highly invasive and much more extensive and risky than Jayson's previous surgeries so I don't think we'd ever consider it.  You can manage symptoms by avoiding triggers.  We would drastically have to change our lifestyle which we'd be more than willing to do.  Jayson would likely never be able to fly in an airplane and we may look into relocating if it would help symptoms.  This would be a very difficult condition to deal with and we really don't want this to be the explanation of his symptoms.

So really, it feels like there are not any ideal outcomes.  There is nothing that I am really hoping or praying for and any outcome brings with it an immense amount of anxiety and heartache.

What we really want is our boy to be happy and pain free.  We want him to enjoy life again, ALL of the time.  We want him to be able to participate in his favorite activities without rubbing his head or having to lie down.  We want him to be able to stay safe and school and not have to worry about him losing his balance and hurting himself.  We want him to be able to sleep without moaning and rubbing his head.  It's a lot to ask and not a lot at the same time.

I think many people take their health and comfort for granted.  That's all we want for our sweet son.

So now we wait... I should have access to the MRI and CT reports sometime this weekend and I will hear from the audiologist about his ABR right away.  I really didn't want today to come.  I don't want answers and I don't want to hear that we don't have any.  I just want peace and I can't think of a way that peace, comfort or hope can come from any of today's results.  I guess what I am hoping for is a miracle.  Something we haven't thought of.  A way for everything to be okay without a lot of suffering.  God is good and we know that.  He has helped and guided us all along Little J's Journey.  It is possible He has this under control and we are about to learn of His great plan for Jayson.

And my faith is about the only thing that brings me hope on these hard days.

So for now, I'm not praying for answers and I'm not praying for clean scans.  Today I'm praying for peace and I'm confident that is something my loving Father can give me.

Thanks for your love and prayers.