Khabarovsk Way-Station, Pediatric General Floor Recovery

Playing Color Code.  Notice Heather decided he needed a ginormous orca.

August 22, 2016, 10:00pm:  It's been a busy day.  And since I'm posting this update from the Peds General Floor post, not at the beginning of a new Rehab post, we are indeed still in Bloomberg 10 South.  Not sure exactly what happened, but somehow nobody notified Kennedy Krieger about Christopher or got insurance authorization Friday.  Nobody's claiming responsibility on that one, but I know the evaluation was done by Physical Medicine & Rehabilitation Therapy last Thursday and Neurosurgery had signed off on it by 8am Friday.  So I had to spend time today meeting with the Case Manager at Hopkins and the Director of KKI, which included a tour of the facility and its operations.  

Christopher still astounds everyone with his progress and had additional PT this morning.  That had him walk one lap around the entire ward.  He can walk a straight line with support fairly well, but loses his balance on turns.  In straight halls, that's not too bad, but in a room with furniture to negotiate, he struggles.  We attempted to play Jenga for his left hand fine motor skill practice today, but he kept toppling the tower, so we played pick-up blocks instead.

Behavioral Psych also came today.  They are involved to help him negotiate the emotional shock of waking up with hemiparesis, but also to help him regain emotional regulation and executive function as his front lobe heals from the edema of surgery. They had him work on breathing exercises with their biofeedback app.  He made the music play his first attempt, which is shocked them all.  So, Christopher has excellent breath/mind control, he just needs help applying it when stressed.  They'll work on that.

I'm not sure whether he actually ate anything today.  Certainly, everything he drank before 8pm came back up.  Lots of projectile vomiting today.  We have officially determined that oxycodone is not Christopher's friend.  I was able to dribble ~100mL Pedialyte into him just before bedtime.  So for now, he gets Tylenol only for regular pain control and Reglan/Benedryl for rescue.  

Child Life totally came through for us today, though, and got Christopher an appointment with Hollister, the golden retriever therapy dog visiting today.  There was a slight misstep when his owner asked what sports Christopher likes to play (we don't really do ball sports!), until Christopher said he's a swimmer.  Turns out, the handler is a triathlete and Iron Man, so they got to talk about swimming, cycling, and running.  We've now decided we need to make a bucket list of the Top 20 Things Christopher wants to try when he's an athlete again.

So.  Today hasn't been bloody awful, and it's been rather productive, it's just not been particularly enjoyable.  I've been incredibly emotional today.  It just shouldn't be so hard for to do simple things like go the bathroom or play Jenga.  He's cried a lot today, too.  Not sure whether or not we still believe in the stages of grief, but he's definitely done with anger and denial and moved on to tears.

11:00pm:  Christopher didn't have a single rage today.  Or anxiety attack.  When he's in pain, he gives us an honest answer.  He isn't in love with the idea of rehab, but he isn't arguing its necessity or refusing to go.  Once we were past the migraine, he got up and had a blast making Star Wars paper craft planes with Bryson.  Then we made total fools of ourselves launching them off the mezzanine along with paratroopers.  After Scott and the Pitsenbargers left for home, we did his PT homework assignments and then he READ House of Hades for 2 hours.  I enlarged the typeset a good bit for him, but still.

1:20pm:  Christopher's head CT is perfect.  Well, as perfect as a 4 day post-craniotomy/AVM resection with Chiari I malformation can be.  No brain bleed.  Just the migraine from the other side.  Christopher has a new IV in the right arm, but it's not his hand, so it isn't as aggravating or difficult to work around.  Into the IV went Zofran and fluids to stop the nausea.  That seems to have kicked in. He will be getting pain meds every 4 hours indefinitely because it is still too soon post-op for him to get actual migraine rescue drugs.  The hope is that while we know we aren't stopping the migraine, he just won't care about it.  He is also getting Reglan and Benadryl through the IV, which is close to being a migraine cocktail, it's just missing the NSAID (think Motrin).  Neurosurgery says that we definitely need to rethink his migraine prevention protocol, since we were noticing 2 weeks ago that he was getting breakthrough migraines, but didn't take him to his headache specialist because it was too close to surgery to change the protocol.  We are now too soon post-op to change things up.....grrrr.  

But we do have a plan.  

And as much as migraines suck, they are not a reason to keep him on the Peds floor, so he will still likely be transferred to Kennedy Krieger to begin rehab tomorrow.  They have a Pain Management team there, so they will definitely become part of his care team in order to make sure that migraines and Chiari headaches do not hamper resolution of his left side hemiparesis (official diagnosis).

Christopher's PT came again today, and insisted he get up and walk, migraine and all.  She also gave him exercises for homework.  But she also gave us some more information:

1) He's likely to be at Kennedy Krieger for at least 3 weeks.

2) Extreme anger is really common in AVM resection patients when it is near the frontal lobe.  The surgeons don't drain all the blood during surgery, and the tiny pool they left (and which was still in place in Friday's CT scan) can affect executive functioning until it resorbs.  In other words, he has no filter for anger or tact.  The Decadron he was receiving, while a truly excellent option for limiting the swelling in his brain post-op, is also notorious for triggering rages.  Not a great combination.

3) Pet therapy is often utilized at KKI, and since Christopher needs fuzzy animals like he needs oxygen, that will definitely be part of his plan.

4) KKI has a therapy pool, so they will try to incorporate water therapy as soon as his incision site is cleared since Christopher loves the water.

Now that the meds have taken effect, Christopher is pretty chipper again.  He just finished several rounds of Color Code with Bryson and Heather.  Then they had a discussion of fluffy animals and how "When I was little, I couldn't sleep without holding a stuffed animal, but now that I'm older, holding a vibrating cat is ten better than a stuffed animal."  Scott and Heather took that to very non-G rated places.  Now they're doing Mad Libs.  Which has led to the discovery that Christopher can read small print again!!!  

11:02am:  Head CT done.  He's vomiting now.  Still too close to surgery for a migraine cocktail.  IV line going back in.  Waiting to choose which IV drugs to administer based on whether they diagnose it as migraine or brain bleed.

August 21, 2016, 8:30am:  Another beautiful, restful night for Christopher.  He woke up at 7:00 to pee, change his pajamas, and then surgery-site headache/Chiari pain exploded into a hellacious migraine.  Oxycodone and Tylenol were administered right away, 90 minutes later, no effect.  Massive pressure in the right eye, vision blurry, doubles if he tries to focus.  Neurosurgeons have rounded and are checking with Ahn and Tamargo to see if they want a CT/MRI scan, and whether we can try an IV migraine cocktail.  They are definitely on it since he hasn't really complained much at all about headache pain prior to this, and never an hour-post meds.

Look, no more dressings!

August 20, 2016:  Christopher was right, PICU was stressing him out.  He slept great all night and nearly through his 4 hour vitals checks.  His blood pressure readings were all good, too.  Once he woke up, though, his mood was still pretty volatile.  I need to start a list of all the things doctors should warn you about before brain surgery:

1) 95% of patients get constipated from the anesthesia and painkillers, so you'll be one of them even if you've never been constipated before--seriously, why don't we do prophylactic meds for this?

2) As the nerves in the brain regenerate, pain and temperature will be ultra-sensitive even as decreased sensory perception persist--information I could have used before he started crying in pain from the left arm IV drips at the same time the right arm didn't care, or screaming every time the arm gets bumped, or shivering under 4 blankets because the left side was bitterly cold in a 75* room.

3) Decadron, the anti-inflammatory used to control brain swelling, brings on massive Roid Rage.

4) The number of needle marks in the body post-op is much greater than the number of lines put in.  The kid seriously looks like a heroin addict.
5) You need to be able to lift your child's weight in a dead lift without throwing out your back.

Every 9yo boy should have a view of the Life Flight helipad out their hospital window.

There are several other things the doctors forgot to mention, but that I found in my research.  The short term memory gaps, vision loss, and attention span deficit all fell into this category.

We should now be past Item 1, Item 2 is beginning to subside, Number 3 finished tonight, the bruising from the fourth item is turning pretty colors, and I was only mildly sore helping Christopher move because of Item the Fifth.  I am sure that he will still have a good deal of emotional volatility even after the steroid titrates out of his system over the next few days, but I'm hopeful the irrational rages will cease.  The attention span deficit is clearing.  Instead of the 3 minutes to follow a single thread he had on Day 1, the 10 minutes he had on Day 2, he can now watch several episodes of Avatar: The Last Airbender in a row.  We'll try listening to audiobooks again tomorrow, I hope.

Pain is better today than yesterday, but still requires careful round the clock dosing.  That, they told us to expect.

Physical therapy is still not his favorite activity, but he is making rapid improvement in his ability to place the left foot each step provided he has full support of his torso.  The effort of a 6-yard walk no longer makes him want to vomit and he can sit up straight for a few minutes.  The left arm is still very weak and he has very limited ability to complete left hand fine motor tasks (e.g. tearing wrapping paper was his PT assignment today).  The anger is actually serving him really well in that respect.  He's so motivated to go home that he keeps pushing himself to walk as much as possible instead of use the wheelchair.  I actually read an article last week about the benefits of a negative outlook, as luck would have it.  The anxiety he had before surgery led him to ask (and to search out answers) for nearly every aspect of his procedure, so he was very prepared going in.  And his neurosurgeons continue to be amazed at how quickly he is recovering from what was near total motor and sensory loss on the left side, so our sample size of one clearly validates this theory!

Working on kicking the leg way out in front with each step instead of shuffling.

Dr. Tamargo visited late afternoon today and told us he no longer needs to be on the Peds floor.  Unfortunately, the Kennedy Krieger Institute cannot take new patients on the weekend and he cannot be discharged home in his current condition.  So we'll continue PT/OT from 10 South tomorrow and await transfer to KKI Monday.  He has been given pretty free rein of the hospital, though, and since Heather and Bryson drove from WV to visit, we plan on folding Star Wars paper airplanes and launching them from the mezzanine into the main entrance of Bloomburg Tower tomorrow morning.  Today we explored the playroom and ate dinner in the courtyard abutting the original hospital building to escape the drudgery of a patient room.

Seeing kids has been huge for Christopher, first his brother yesterday and now Bryson this weekend.  These photos are of our "escape" from 10 South to the courtyard abutting the original hospital building for an early dinner/late nap.

Khabarovsk Way-Station; PICU Recovery

Friday, August 19, 2016; 9:00pm:  Today was an incredibly emotional day.  Christopher has some very minor blips in his short term memory.  So he was absolutely certain we hadn't done his CT and berated us sharply for leaving before it was done.  Later, I told him I was leaving to talk to one of his care team members.  He completely forgot, and a nurse came to get me because his pulse and bp had skyrocketed from his rage at my absence.  He slept between all his neuro exams until 10:30, and then spent the rest of the day jumping from one anxiety attack to the next.  Finally, Dr. Ahn rescued him by deciding PICU was preventing solid sleep and comfort free from tubes and cords and released his bp constrictions.  By dinner time, Christopher was ensconced in 10 South on the regular peds floor.

He is much stronger today, and his short walks to the bathroom are already much stronger than his first walk this morning.  But it is all just.so.very.hard.  For me as much as for him.  For tonight, we will try for a semi-solid night's sleep.

Friday, August 19, 2016; 1:00pm:  The train is stopped indefinitely.  Christopher was maintaining his MAP 75 level, but just as they were ready to turn off nicardipine, MAPs went to 85.  He is on a much lower dose than the max he's been on, but the expectation is that he has at last another night in the PICU.  The expectation is that he'll then go to the regular floor until Monday.  Christopher's vision is still blurry up close and doubles if he focuses to the left.  His visual tracking is slow, as is his ability to focus on a conversation.  Tamargo feels this is an artifact of brain swelling and it will resolve.  Rehab medicine anticipates 3-4 months for most of Christopher's gains in the left side recovery, but full recovery will take 6-12 months.  Because he only has one IV now, all non bp meds are being given by mouth.

Physical Therapy and Occupational Therapy have both come to work with Christopher today.  Walking 40 feet was the equivalent of running a marathon for him today, but he did do it.  He even got a bonus 12 feet using the restroom after his CT scan.  The physical therapist told him that both Physical Medicine & Rehabilitation Therapy and Neurosurgery have recommended him for inpatient rehab at the KKI facility on the Johns Hopkins campus.  To say he isn't happy about that is an understatement.  I do not yet have a timeline for how long they expect him to remain there before he can come home and continue physical therapy as an outpatient.  He will continue doing PT/OT here in Bloomburg Tower until he transfers to KKI.  Occupational Therapy prescribed a boot to help him flex the left foot for walking. They will also work with him to improve his vision and regain precise control of his left arm and leg.

Physical Therapy doing an initial assessment before transferring all his IVs and monitors to a portable stand to take his first walk.

Friday, August 19, 2016; 5:30am:  We topped off his fentanyl at 2am because he was thrashing and said he had a headache again.  All was well until 4am, when he tried to pull himself up and across the bed to pee on his own.  I awoke to him nearly slamming his crani site into the bed rail, and jumped up to help him onto the bedside commode.  We thought everything was going great and I was really pleased by the stability of his torso until I saw blood dripping from his hand onto the floor.  IV line pulled....who designs their length, anyway?!  Mild panic ensued as I called for help and pushed the nurse call button and several nurses and techs rushed in to help.  He's all cleaned up, and back asleep now, and down one IV line since it was probably coming out this morning and his MAPs maintained at 65 the whole time he was offline the esmalol and nicardipine.

So.  A whole beautiful night's sleep, increased ability to hold his head and torso up for a few minutes, and he got rid of one dreaded IV.  PT/OT never made it yesterday, but the rehab physician team did complete their eval and put in orders for inpatient PT/OT to begin today.

Overnight Update:  Sleep!!  So far he's logged 5 hours' sleep after level 10 pain at 9pm.  He was restless and was eye open/sleep talking midnight-1am, but he's been still for awhile.  Fingers crossed he stays asleep for the rest of the night.  MAPs stable, but not low enough to start titration off bp meds.

Thursday, August 18, 2016:  Christopher is having a really rough time in PICU.  Last night, the fentanyl kept him so chatty he only slept about 2 hours.  I suspect the Decadron steroid to control swelling is also to blame.  His nurse and I were pretty busy trying to keep him calm.  Today, he is really frustrated at the near total loss of use of his left leg.  Sensation has returned, and he has some limited ability to lift or bend the leg, but he still can't control the ankle, foot, or toes.  PT/OT should come today to start teaching him/us how to function with a listless leg.  The arm is still weak, but has full mobility and control.  No fentanyl because he says he isn't in pain, but still can't sleep for more than 45-60 minute stretches.  He has trouble focusing his eyes to read or watch a movie, and can't concentrate to listen to an audiobook, which has led to several anxiety attacks.  This is all very normal post-craniotomy, and we knew to expect it, but it's very hard on a 9 year old.  His MAPs are being maintained at 70, but it takes two different blood pressure meds to do it.  He is therefore definitely staying a second day in PICU before he can transfer to the regular floor.  Tomorrow they will do a CAT scan to monitor his brain.

The neurosurgeons are still confident that the left side deficits are temporary.  The timeline for full recovery is indeterminate, though.  It could be 72 hours, or it could be considerably longer.  The rehabilitation physician came and evaluated him this afternoon.  They are definitely recommending Physical Therapy/Occupational Therapy, but we don't yet know details.  As it stands, he cannot navigate daily life, even sitting up straight is more than he can handle just now.  Rehab is definitely going to have him begin PT/OT while he's still here at Johns Hopkins, hopefully as early as this afternoon.

Khabarovsk; Craniotomy and AVM Resection

8:29pm  When we first got to PICU around 4:150pm, all the physicians and nurses were delighted to tell us how smart and funny he is.  Christopher's speech was slurred, and he was telling everyone everything about everything.  But then his mean arterial pulse (MAP) got higher than they wanted, he kept going tachycardic, and he was really agitated.  A dose of pain meds calmed him down and he went to sleep, so Scott and I rushed to grab dinner in the food court.  Unfortunately, he was agitated again by the time we got back, and we found the nurses had just topped his fentanyl off with Tylenol. They're still titrating his nicardipine upwards, as his MAP is still hovering around 75.  As long as he's calm, he's no longer tachycardic, and watching Home seems to be a good distraction right now.  He was having intermittent double vision, but that might be clearing up.  

Initially, in Mr. Charming Phase, Christopher was ecstatic about all things and how amazing it was to be AVM free.  Now, he hates hospitals.  But where before he was really upset about his sore throat, now he's complaining about how much his head hurts.  He often has an anxiety attack several hours before a major spike in his headache, so I suspect that was the source of the earlier agitation.  A nurse has pretty much been in here the whole time adjusting his IV bags and meds and checking his stats.  Hopefully that will change soon.

As predicted, the left arm and leg sensation and motor control deteriorated.  His arm is pretty weak, but he can direct its movement and wiggle all his fingers.  His left leg went completely numb for awhile, but his foot sensation is back.  He cannot move his toes, but if he really concentrates, he can lift the leg a bit.  He is now aware that it is numb and unresponsive, and is definitely disturbed by that.  But since the leg has improved from its 4pm status, the neurosurgeons feel confident it is all temporary.  In general, they're quite pleased with his status, but did go ahead and prescribe Cepra as a prophylactic against seizures, which is fairly standard in neurosurgery patients.

The incision site is larger than we expected, and looks a bit like a tonsure, but so far, he has only mild swelling in his scalp and face.

3:29pm  He's out.  He's awake.  He's calm and responding well.  Drs. Tamargo and Ahn just came to visit us.  The AVM is gone.  The walls were so thin, they could see the blood flowing through the them.  So, it was about to rupture, definitely within the next few years, and it did begin leaking as they pulled out the nidus.  The left side is responding well, he can move his fingers and even his toes, but he definitely has weakness in the left leg.  They warned us that as swelling sets in, it will get worse before it gets better, but they are hopeful it will be temporary and he'll regain full use.  Christopher did need blood transfusions, but nothing "out of the ordinary".  PICU should allow us back to see him within 45 minutes.

Stroke Watch 2016 is over! Next phase, AVM recovery....because I need this kiddo back. The one that ran a 10k on the Great Wall of China last year. The one who said, "But Mommy, I can't just walk. I have to run."

1:17pm  Supposedly, the OR called Reception at 10:18am to say they'd started the craniotomy.  Reception says not.  No idea what happened there, but I've been a ball of nerves trying to figure out why a one hour angiogram had turned into four.  When we hadn't heard anything by noon, Reception called the OR for us, and then said they were still doing the angiogram.  Just now, Reception told us things were going well, but had no idea whether they'd started the craniotomy or not.  I started shaking, so she called the OR and let me talk to the nurse.  Craniotomy started at 10:18am, they think they are halfway through, and they currently have interventional neuroradiology in again to check the status of the AVM.

Official AVM Ejection Photo.  And modeling of the Johns Hopkins University Hospital Garage Charging Spots

10:00am  We made it to Surgery Day.  In theory, Stroke Watch 2016 is now behind us.  Christopher was amazing this morning.  Mr. Charming himself, the pre-op nurse was convinced a little man was hiding in his young body because he totally took charge of answering all the questions about his medicines, food/liquid intake, everything.  He had to explain what Stroopwafel is, since that was his 10pm snack last night.  Scott is here with us today, so we played Mad Scientist Mad Libs while we waited. He is definitely concerned about having not just one, but two IV lines, and also the arterial line.  Christopher told me last night he’s not scared of the surgery per se, just what happens after he wakes up.

Me, too. 

Mad Scientist Mad Libs

We met his anesthesiologist and anesthesiology fellow, his operating room nurse, and saw both Dr. Tamargo and Dr. Ahn before they took him back to the OR.  I pointed out the stereoscopes, the fellow took charge of the gas that put him to sleep, and then he was out.  No crying, no balking, very calm.

His OR nurse and anesthesiology fellow beginning the prep work after he was out.

It took them right at an hour to get him prepped for the pre-op angiogram, they should be nearly done with the imaging now.  And then the neurosurgeons will have the information they need to decide exactly where to place the hole in his head and the removal of his arteriovenous malformation will commence.

I expect a call soon letting us know that they are done with the angiogram and beginning the craniotomy.  At that point, we may look into donating blood, as Dr. Tamargo told us he's quite likely to need blood products today.  AVMs like to bleed.

For now, we wait.

Birobidzhan; We go to the ER at Children's National

We’ve now had a week to process everything that happened at Hopkins last Monday.  I understand where Dr. Groves was coming from, and I really do think we’ll all feel better once the AVM is gone and we aren’t on stroke-watch any more.  I really hate being on stroke watch for a child.  It’s not the natural order of life, and the symptoms are scary.  I just wish she had a better way of presenting that in clinic.  But I also know that as much as we dislike her bedside manner, her surgical skills are probably impeccable.

Tuesday we went to Christopher’s pediatric opthalmologist, Dr. Chaudri, to have his optic nerves re-evaluated.  Luckily, they’re in perfect condition, and he passed his color vision and strabismus (lazy eye) exams beautifully.  But she did discover that his right pupil is slightly larger than his left now.  She tested it several times, in light and dark, and during the transition, to be sure.  Then she asked for his lead surgeon’s number so she could make sure they’re aware.

Turns out, Groves’ PA saw it in clinic Monday, so they are.  I emailed his surgical team the next day just to make sure it got into his file, and Stephanie, the PA we usually work with, let us know.  I think this helps explains part of Groves’ demeanor in clinic.  My labrador, Sunshine, also decided a few days prior to the Hopkins visit that he really didn’t want to go on walks with the boys anymore—except that he’ll let my oldest, Alexander, take him without balking.  We’re pretty sure he can smell that something is different.  He exhibited similar behavior in April when the headaches were really scary.  The change in Christopher’s pupil is subtle, but along with the increase in his headaches and Sunshine’s behavior, we’re fairly certain the pressure inside Christopher’s head is higher than it should be.  A few days ago, he told me he wasn’t dizzy per se, but all his cells felt like they were spinning.  Two nights ago, his feet were numb.  Last night, he said his fingers felt like they were hot, except they were cool to the touch. 

I called Dr. Bernier, his headache specialist, yesterday, because the headache was so bad.  Because his gut has also been upset the last week and he had a low grade fever for several days prior to that, she was hopeful he has a gastro bug that’s about to run its course.  She reminded me that any illness will make his headaches worse.  But she gave me the main line phone number to Children’s National Medical Center and told me to ask for the on-call neurologist if I had any other questions at any time of day.  I didn’t think to tell her about the weird sensations he’s been having while I was on the phone with her, but when I was reviewing stroke symptoms online, “hallucinations” show up under “altered mental state”.  So I called Children’s National this morning.  The neurologist says they aren’t hallucinations, that the pressure inside his brain is too high, and the sensations he’s experiencing are indicative of blood flow being altered.  She wants him imaged, and asked us to bring him into the main campus in the Capitol.

We beat traffic driving into the District, but are now in the thick of it for the final few miles.

Update 8/12/2016:  The ER team did not mess around, they were in complete agreement that he needed a CAT scan.  Dr. Bernier had also put a note in the system for him to be direct-admitted for a scan if he showed up.  Luckily, it was clear, which gives them 90-95% confidence no bleeding has occurred.  The neurology team came downstairs to evaluate him and decide whether they wanted to rule out the other 5-10% with additional tests.  And then they went to pow-wow to see if they couldn't come up with some kind of AVM-safe pain control to attempt while they kept him overnight.  On the one hand, they decided additional tests weren't necessary.  But, as we're getting used to hearing, there's really nothing they can do for pain until the AVM is gone.  They do think he had a gastro bug.  And where a gastro bug takes most of us from a level 1 to a 3, any illness will take him from where he is living at a 7 up to a 9.  Hence the scary symptoms.  Although, it took them 5 hours to make all those decisions, and unfortunately he had a really painful IV this time around.  But he was discharged and sent home, with strict orders to come back if things escalate again.  Luckily, things have stayed pretty boring since then.

Chita; Final Hopkins Visit Prior to AVM Surgery

Today we were scheduled to meet with two of Christopher’s neurosurgeons.  First we met with Dr. Mari Groves, his pedi spinal doc and Chiari specialist.  Despite what we’ve been told by the vascular team, she isn’t ready to discuss treatment for his Chiari I malformation in any way.  She feels that his upcoming microsurgical dissection and craniotomy to remove his AVM is too huge and major a surgery to even contemplate the next steps.  When we pressed her, she told us that she wouldn’t even consider surgery for 6-12 months.  Months!!!

Christopher crumpled in his chair.  It was like he’d been sucker-punched in the gut.  

We explained how severe his headaches are, how attempting to play for a few days leaves him with hideous headaches for days, how his activity is so limited it goes against everything I’ve ever learned in 13 years of parenting, and all the science espoused by the American Academy of Pediatrics about the importance of 1-2 hours of daily exercise for all children.  We also explained that Dr. Ahn had told us his brain would, of course, need to fully revascularize before a second surgery, and Dr. Tamargo would have the final word, but that he anticipated 3 months between surgeries.  Dr. Groves relented a bit and said we could do a follow-up in 3-6 months to see where things stand.  But she reiterated that she doesn’t want us to think about the Chiari at all, but to focus on the AVM surgery because it could actually kill him if it ruptures, it’s extremely major surgery, and we just can’t think beyond it right now.

The last I spoke with our pediatrician, he told me that after AVM surgery, the options for pain control open up a lot more and we can try sending Christopher to an anesthesiologist pain specialist.  So I switched tactics and asked her what she thought of that course of action to get him through to the next surgery.  Her response was a vague sort of, well, we can try it, but nothing controls the pain of a Chiari headache.

I’m not sure I’ve ever been so upset in my life.  We’ve spent a lot of time the last 3 weeks trying to make this first surgery a positive experience.  We’ve talked about how AVMs often cause headaches and motion sickness, so it might not “just” be a pre-requisite to the Chiari decompression (because, let’s face it, the daily pain he experiences is more pressing to my 9 year old than the chance of the AVM rupturing inside his brain, although he’s fully aware of its repercussions).  We’re organizing a brain party for him and his friends the weekend before surgery so that we can celebrate this first step to his recovery.  As he’s asked questions about different aspects of surgery, I’ve tried to frame them as positively as possible:  Will they use staples?  Maybe.  But if they do, it’s because they’re stronger than stitches and they want you to have the smallest, straightest scar possible.  

I feel like she undid 3 weeks of pep talks, party planning, Q&A sessions, and meditation in 10 minutes.

Thank goodness, our appointment with Dr. Tamargo went much better.  He’s a portly, grandfatherly type, and instead of getting straight to business, he started at the beginning, with our new patient questionnaire.  Then he asked, “How did you get to here?  What got Christopher to this diagnosis?  What started this path?”  And he listened, while gave the 10 minute version of Christopher’s story.  Then he excused himself so that he could speak with Dr. Groves.  When Dr. Tamargo came back, he said that he believes the main source of Christopher’s pain is the Chiari, and he feels it’s 50/50 which gets treated first, so he had gone to ask Dr. Groves if she wouldn’t do that surgery first.  

She declined.

With that out of the way, he explained his 31 years of experience, how he came to be the guy at Johns Hopkins with the most experience at handling AVMs, and why he thinks Christopher makes a great surgical candidate.  Since we hadn’t actually seen the results of his angiogram, Dr. Tamargo was happy to guide us through the images.

Image 1--View of the AVM and its feeders in Christopher's angiogram.

Image 2--Close-up of the AVM.  It really is just a tangled web.

His Spetzler-Martin score is a 2, surgical excision is generally considered only if the grading is no higher than a 3 of the 5 possible points.  The AVM is larger than we had initially anticipated, 2.6cm on its longest axis, but still well below the 3cc cut-off to be considered small.  It has only superficial veinous drainage.  It doesn’t have an aneurism hiding inside it.  It is centered on his central sulcus, which makes it prime real estate, although the sensory strip is less critical than the motor strip, but Dr. Tamargo assures us he’s removed AVMs from just the motor strip before.  Dr. Ahn had told us that the nidus, or core of the AVM, is actually just behind the central sulcus in the sensory strip, with just a few vessels snaking into the motor strip region responsible for movement of his left leg.  While there is always a non-zero chance of death or permanent neurological deficit, in 31 years, he’s never had either.  There is a greater risk that Christopher will wake up with weakness in his left leg, or even his whole left side, but it will almost certainly be temporary.  Nearly all AVM patients come in with headaches because that’s usually what leads to brain MRI and the initial diagnosis.  Half of all AVM patients report their headaches ceasing after surgery, but there’s no way to know ahead of time which half Christopher will fall into.  So we’ll hope.

Figure 1--This image is known as the homunculus diagram of motor and sensory control in the brain.  Notice that small regions like the tongue and lips take up far greater brain space than the entire trunk due to the highly detailed and sophisticated muscle movement necessary for speech.  Image Courtesy of the Brain Book, p. 23.

The surgery itself is predicted to take 6 hours.  With an angiogram before to determine exactly where to open him up, an angiogram after to make sure the AVM is completely gone, and the time to prep him for surgery, we’re looking at 8+ hours.  The operating room has been booked for Dr. Ahn and Dr. Tamargo’s use for Christopher for the entire day.  Recovery is likely to be one night in Neurological Intensive Care, 2-3 days on the Peds floor before he comes home.  Dr. Tamargo wants us to be prepared for him to need 6 weeks before he can return to work/school.

After surgery, Dr. Tamargo says that if everything goes perfectly, he’s prepared to clear Christopher for Chiari surgery at the 6 week post-op appointment.  Again, I told him that while Dr. Ahn deferred to Tamargo as the expert, he was thinking it would take 3 months.  Dr. Tamargo smiled and said that was likely a more realistic scenario, but he really was prepared to clear him earlier and move up the normal MRI schedule if necessary to make that happen.

And then we went to Kona Grill for sushi.  And edamame because Christopher loves edamame.  And passion fruit creme brulee, which was to die for.

Now, he’s in a pretty good mood.  He likes Dr. Tamargo, he trusts Dr. Tamargo, he’s excited for the AVM surgery.  I am in complete agreement.  But I’m still a ball of nerves because of the earlier appointment.

Ulan Ude; Dealing with the Unimaginable

I recently purchased the Hamilton musical.  I've never been a fan of rap, but the music resonates with me strongly.  Bonus, the boys ask lots of questions about the American Revolution.  But then there's this:

"It's Quiet Uptown" is a song from the Broadway musical Hamilton performed by Renée Elise Goldsberry (Angelica), Lin-Manuel Miranda (Hamilton), Phillipa Soo (Eliza).

ANGELICA:

There are moments that the words don’t reach

There is suffering too terrible to name

You hold your child as tight as you can

And push away the unimaginable

The moments when you’re in so deep

It feels easier to just swim down

ANGELIC/ENSEMBLE:
The Hamiltons move uptown
And learn to live with the unimaginable

My child is not dead.

My child does not have cancer.

My child is not recovering from a catastrophic car accident.

I started crying when I first heard this song.  I am living the unimaginable.  Dr. Ahn had called to tell me the results of the weekly vascular neurosurgery conference.  And instead of giving me a timeline for gamma knife surgery followed by a Chiari decompression, he told me they felt it was better to operate and microsurgically dissect the AVM.  The team was concerned his headaches were getting worse, they felt he needed a Chiari decompression in order to alleviate the headaches, but they were worried opening the skull without first removing the AVM was too risky.  It could lead the AVM to rupture.  Gamma knife surgery takes 2-3 YEARS to resolve the abnormal blood vessels, it doubles the risk of rupture during the first year, and it carries some of the same complication risks for surrounding healthy brain tissue as old-fashioned surgery.  So, Dr. Rafael Tamargo, the resident AVM expert at Johns Hopkins and Director of Cerebrovascular Neurosurgery, will take the lead and dissect out Christopher's AVM.  Also of note:  his AVM is straddling the central sulcus line, with the nidus in the sensory cortex, not the motor strip.  So while there is some risk of temporary damage to his left leg and even his whole left side, it isn't the catastrophic damage they originally thought operating in that region could cause.

Image 1--The tangled ball of yarn at the top center is Christopher's arteriovenous malformation.  You can see that it has two main feeder arteries and that it clearly looks different than the branching vessels around it.

I wasn't thrilled the first time we were told Christopher might need skull base surgery, but I'd been mollified with the thought that it was "the most routine of all possible surgeries". And really, it just barely qualifies as brain surgery.  The idea is that the surgeon widens the skull and C1 where they pinch the brain, and then opens the dura, adding an extra large patch so that cerebrospinal fluid can flow freely again.  The brain itself usually isn't touched.  (Reference this post for more details.)  That made it seem like a reasonable approach, especially if amitriptyline couldn't control his headaches.  At least, it was better than asking a 9 year old to live the life of an invalid.

This is different.  This is bona fide, crack your skull open and extirpate brain tissue, sit in an OR waiting room all day while your child's brain is open on a table, brain surgery.  I've always been taught that the brain is a sealed system, something we shouldn't open.  And now they want to open it twice.

Figure 1--In an AVM resection, vascular neurosurgeons, working with a stereoscope,  use specialized instruments to first close off and detach the feeder arteries.  Image Courtesy of The Aneurysm and AVM Foundation.

Figure 2--In the next stage of surgery, the core of the AVM, the nidus, is removed, along with any brain tissue it encloses.  Then the draining veins are closed off in the final step.  Image Courtesy of The Aneurysm and AVM Foundation.

For more details on the procedure, visit The Aneurism and AVM Foundation.

But the alternative is to wait until the AVM ruptures.  At a 2.5% annual risk, 25% over a 15-year period, it will almost certainly rupture in his lifetime.  The rule of thumb is 

105 - age at diagnosis = 96% 

for Christopher.  One-third of rupture patients die before they reach the hospital.  The remainder vary between those with the worst headache of their life and permanent, debilitating loss of neurological function.  As much as I hate the idea of a craniotomy and microsurgery, I am rational enough to appreciate the superiority of a calculated preventative procedure to an emergent, all-hands-on-deck trauma. 

My unimaginable may not be as terrible as the worst case scenario, and two weeks later, I have learned to live with it, thanks largely to my incredible support network of friends and family.

So instead of focusing on the unimaginable, I try to focus on this rallying cry for Christopher instead, cheesy though he thinks it is:

“Fight Song” by Rachel Platten 

Like a small boat

On the ocean

Sending big waves

Into motion

Like how a single word

Can make a heart open

I might only have one match

But I can make an explosion

And all those things I didn't say

Wrecking balls inside my brain

I will scream them loud tonight

Can you hear my voice this time?

This is my fight song

Take back my life song

Prove I'm alright song

My power's turned on

Starting right now I'll be strong

I'll play my fight song

And I don't really care if nobody else believes

'Cause I've still got a lot of fight left in me

Taishet; the Cerebral Angiogram

As much as possible, we’ve been trying to make our Johns Hopkins trips family outings as much as they are trips to check on Christopher’s brain.  This usually means booking a hotel on Hotwire (Scott’s pretty awesome at getting great rooms for good deals) and staying overnight so we aren’t pressured by the Capital Beltway—I95—395 traffic gauntlet.  After our first visit with Dr. Groves, we all went to the Baltimore Science Center for the first time.  Since cerebral angiograms are officially “minimally invasive” outpatient procedures, we booked a hotel for two nights, this time as well.  The idea was that I’d take Christopher to Hopkins at o’ dawn-thirty while Scott took the other two children to the Science Center for another field trip.  The next day, we’d all go to the National Aquarium together.

Image 1--The Children's Tower at Johns Hopkins University

Christopher displayed no nerves whatsoever either in driving to Hopkins from our hotel or while in PACU awaiting his procedure.  In fact, the same incredibly charming demeanor he’d had at Inova Children’s in April was on display.  I asked him about it afterwards, commenting, “I thought you hated hospitals and needles?”

“Well, yeah, but hospitals actually do stuff.  Doctors’ appointments just ask lots of questions without fixing anything.”

Image 2--Smelling anesthesia flavors; he chose cherry.

Out of the mouths of babes.  He’s not necessarily wrong.  The anesthesiologists were quite concerned that his headaches were still getting worse even though Dr. Bernier had adjusted his dose to 20mg amitriptyline two weeks’ prior.  So they decided they’d try to give him some extra fentanyl during the angiogram to see if they couldn’t relieve the headache.  We also got a full description of the cerebral angiogram and how it works by the pediatric interventional neuroradiology resident.  Say that ten times fast.  He asked if we knew what it meant, Christopher said no.  I told him, “Interventional Neuroradiology” means they use radiation like X-rays and gamma to actually treat and fix the diseased tissue inside of you, not just image it.  Pediatric means they only treat kids.”  I got a gold star for the day.   And then it was time to wheel him back to the angiography suite, the anesthesiologists cracking horrible potty humor jokes the whole time.  They put on the mask, I gave him a kiss just before he lost consciousness, and then I went to the waiting room.  

Image 3--Ready to Go, calm as a cucumber; the anesthesiologist is pictured on the left.  I tried to remember his name, I've failed, but he was awesome.

Dr. Monica Pearl, the interventional neuroradiologist performing the procedure, walked me down the hall, since she’d been at the weekly neurovascular conference and hadn’t seen us earlier.  I felt, at that point, that everything was coming together.  When we’d seen Dr. Bernier for his headache follow-up, she had told us we should seek out a second opinion regardless of what Dr. Ahn told us regarding the AVM, just because it’s such a big thing.  When I asked who at Children’s National she recommended, I was told all the complicated cases are referred to Johns Hopkins, and Dr. Monica Pearl is the best. 

Waiting is hard.  Luckily, I was at the climax to The Maltese Falcon, but even then is was difficult to remain focused.  Also, that hospital is cold.  Even in a sweater.  Finally, I got a phone call from the nurse saying they were taking him back to PACU and everything had gone well.  Next I got a call from Dr. Pearl wanting to know where to find me so we could discuss his results.

As much as I was hoping for Option 1 or 2, as outlined by Dr. Ahn, I didn’t really expect it.  From the very, very little I’d allowed myself to research on AVMs, Christopher had enough symptoms it didn’t seem likely this was a nothing.  And yet, we were all still hoping he was undergoing a very expensive procedure for a nothing diagnosis.  Dr. Pearl, however, quietly told me that there is, indeed, a true AVM in Christopher’s brain.  It is small, just under 2 cubic centimeters in volume, it isn’t hiding an aneurysm, it has only superficial veinous drainage, all good things.  But it has to come out.  It’s in prime real estate, so if it were to rupture, the damage would be catastrophic.  She had already taken the images for the gamma knife surgery, because she, too, thought it was inoperable.  And so I cried.  I was expecting this result, but the reality still caught me off guard.

And then I put myself together and went in to see Christopher around the corner in PACU.  Honestly, he wasn’t in great shape.  The breathing tube had scratched his throat pretty badly, so I spent the next several hours offering him sips of popsicle slushy every few minutes.  The first two hours of laying flat didn’t bother him at all, he was too preoccupied by the sore throat and I had his iPad monopolizing his attention with Zootopia.  The second two hours at a 30 degree incline weren’t too bad, either, although sometimes he slipped and we had to make adjustments.  By then, he’d moved on to Home.  The fentanyl they’d given him for the headache worked to lower its intensity the first part of recovery, but his headache was worse than usual by discharge and they’d given him IV Zofran for nausea.  But he did his laps around the unit just fine, the catheter site had no hematoma, and he was able to walk on his own to where I’d parked that morning.  

Image 4--For the record, cherry slushies look like a slasher movie when spilled in PACU.

Surprisingly, Christopher didn’t ask in PACU about his diagnosis.  I had decided I wouldn’t offer until he asked.  On the drive home, he asked.  I was as gentle as I could in saying he definitely had an AVM and they definitely wanted to remove it.  He crumpled in the backseat and started crying.  He almost never cries in all of this.  It didn’t last long, but he was definitely in a very bad mood as we entered the hotel room.  The leg was also bothering him from the short walks to and from the vehicle, and he was supposed to have a quiet afternoon to initiate his 7 days of limited activity and no swimming while the catheter site healed.  So, we curled up in one of the hotel room beds, watching Shark Week with the rest of our family, and ordering take-out  for dinner (mediterranean kabobs  and rice, one of his favorite meals).

I was up most of the night, finally doing my research and looking at the longitudinal studies on AVMs and treatment outcomes via peer reviewed papers.  I was feeling very grateful for my training as a research scientist at that point.  As hard as the formal AVM diagnosis was on Christopher, I took it better.  I think because at this point, I’d opened up to the most important people in my life and just about everybody knew what we’re facing.  Secrets are hard.

The next day we still went to the Aquarium, although it was one of our shorter trips because his leg was sore.  He had full laryngitis by then, and the sore throat was still raging, so we treated it with Tylenol and honey tea, which helped a good bit.  And then we went home and packed him off to 4-H camp, something he’d been excited about for months.  And they, very graciously, changed two of his classes at the last minute so to meet the activity restrictions we’d only just learned about.