One Year Anniversary!

One Year Later, The Trans-Siberian Railway and Conquering Congenital Brain Defects is a Thing We Did.

Christopher had Chiari decompression surgery one year ago today.  So of course I'm making myself cry by compiling a video of how far he has come.  I may have mentioned that it was a much more difficult recovery than we anticipated based on how amazingly well he did with the surgery itself.  He was only in the hospital for 72 hours, after all!  The hard part didn't come until he started really walking more than a couple blocks six weeks later.  But we knew how important it was to get active again and regain a full range of motion as soon as possible.  So he trained all summer to run a kids triathlon with his brother and good friend.  He did a half-day tennis camp at the community center the first week of July.  He wasn't allowed to swim competitively, but he did a youth developmental class once a week so his neck muscles didn't go into shock the first time a HAC coach asked him to do the butterfly for swim team in September.  It was hard on him, it was hard on me, but.it.worked.  By mid-August, we knew he was doing so well that our medical goals for the year were full participation in swim team and school, no modifying our workload for cognitive fatigue.  We mostly succeeded, although it honestly took until 9 months after surgery for him to stop needing extra sleep at night.  Christopher still gets migraines on occasion.  But never a Chiari headache.  Loud, chaotic situations still exhaust him, and a cold still runs him down a bit more than I would like.  But I realized in the fall that he got a cold and it was just.a.cold.  The coughing and sneezing hadn't ratcheted up his headache to the point he was non-functional and I was debating taking him to the emergency department.  After a year and a half of that, it was amazing.  And his neurosurgeon, Dr. Groves, agreed.  No more regular follow-ups or MRIs for 3ish years.  If he develops symptoms that concern us, she still has a low threshold for ordering a scan at any time.  But neurology appointments twice a year for migraine monitoring are the only regular brain follow-up he needs for several years.

Christopher's external scars are minimal.  He usually has the strength and energy of any 11 year old athletic boy.  He has the academic fortitude to read, write, and 'rithmetic with the best of his peers.  He is more introverted than he started out two years ago.  He is still highly susceptible to motion sickness.  He sometimes gets nasty migraines.  But he is also still very, very young and I think his future is likely to be quite bright.

And we made our bucket list goal, made the summer of 2016 on one of his darkest days:  We got him healthy and strong enough to go to Playa del Carmen, Mexico, to swim with his favorite animal, the whale shark.

Vladivostok! Exploring the Scenery

Pretzell PT—when he started getting a sore back from being a couch potato, we started taking walks, but also fun activities like baking soft pretzels to create a bit of activity.

May 15, 2017

Christopher officially no longer has Chiari headaches.  We went to his post-op with Dr. Groves today, and confirmed that he is experiencing residual neck muscle pain from surgery, not actual Chiari headaches.  Not even when he plays hard outside.  She admitted today that she had her doubts going in, but he’s completely convinced her.  I don’t think anybody really understood how good he’d gotten in subduing his own pain until they saw him sail through PICU.

So, we are nearly six weeks out from surgery and scheduled to see neurosurgery, neurology, pediatrics, and pain clinic this week.  Whew.  But how did we get to here?

Overall, the surgery looks like it’s been life-changing for Christopher.  The constant pressure, which he says was like a banging, is gone.  

Three weeks ago, at his regular outpatient PT, he was discharged from all PT services.  After running him through his paces, Hannah says he didn’t regress at all compared to when she saw him two weeks prior to surgery.  There remains a slight deficit when he hops on the left leg, or does left leg standing balance with eyes closed.  All other functions are equal bilaterally.  The neurosurgery PA says they generally don’t say a deficit is permanent until we reach 12 months from the original AVM resection.  So he may regain some of that by August.  But they agree, it’s such a specific deficit, there are very few places it will ever show up (like a yoga tree pose, eyes closed).  Dr. Groves, while thoroughly pleased with his neck function right now, says he’s currently at 60-70% tensile strength in those muscles.  At 6 months, he’ll be at 80-90%, they’ll never fully regain their strength due to scar tissue formation.  So, in a few months, if neck stiffness is causing significant discomfort, we may need to revisit the idea of PT, but for Chiari, not the AVM.

About a week after the discharge, Christopher started getting piercing headaches behind his eyeball.  It’s usually only one, but not always the same eye, and is sometimes behind both sockets.  We ended up calling the on-call neurosurgeon one evening when he had a level 8 headache that wasn’t responding to pain, as per his discharge instructions.  After listening to his symptoms, we were told that while eye headaches after a Chiari decompression aren’t common, they aren’t unheard of, and he only needed to be treated right away if he needed better pain control.  He opted to try sleeping in his own bed, and with the help of relaxation essential oils diffusing in his room, he slept without trouble.  A week later, though, we were still having trouble.  Stephanie, our favorite PA, spent over 30 minutes on the phone with me asking detailed questions, but ultimately decided he was having daily migraines.  This happened after the AVM surgery, and the migraines mostly disappeared after six weeks, so Stephanie felt like his threshold for migraines was similarly lowered by this procedure.  She almost put him on an oral steroid, but because he was only 2 weeks’ post-op, opted to have us give Motrin every 4 hours around the clock instead to keep the inflammation in check, and hopefully the migraines.  That worked beautifully.  But as we’ve weaned off the strong meds (Dilaudid, Valium), and gotten him down to only 3 med doses a day, he’s back to daily eyeball headaches.  They do usually respond to Tylenol plus our Migraine Essential Oil Blend (roll-on vial filled with 3% Plant Therapy Tension Relief diluted in almond oil, plus 3 drops Valerian oil), but they hit about 4-6pm every day.

Also about a week after surgery, he started having nosebleeds.  We tried treating with saline nose spray, but they’ve been getting more voluminous and more frequent.  Today’s was bad enough, I suddenly wondered if his headaches are sinusitis or allergic rhinitis, not migraines.  So we had the pediatrician take a look, since neuro types don’t generally do that sort of thing.  His nose is completely inflamed, but no signs of infection or allergy.  The pedi thinks that while the Non-Steroidal Anti-Inflammatory effect of the Motrin was great for the migraines and soft tissue pain, it has thinned his blood enough to exacerbate his nosebleeds (he’s always gotten them in the winter).  For now, we’re treating with Aquaphor, but the pediatrician wants us to ask the neurologist about an oral steroid to calm both his nose and the eyeball headaches.  He just didn’t want to mask any symptoms before Dr. Langdon, our lovely neurologist, sees him tomorrow morning.  This is the hard part.  On the one hand, he's Chiari-free.  On the other, the very meds we were told to administer to help migraines are triggering nasty nosebleeds.  I'm sure we'll get it sorted, but it does muddy the picture.

Normally, Dr. Groves would wait 6 months to do a post-op MRI, but since Dr. Tamargo wants to see him in August, she’s scheduling an appointment for the MRI and visits with herself and Tamargo then.  I told her we could wait until September, but she laughed and said, “Dr. Tamargo is more important than I am, we’ll do it in August.”  She’ll then continue to follow him yearly for awhile before she discharges him to 3-5 year appointments to see if anything else crops up.  The odds of him ever developing scoliosis are now greatly diminished, but she’ll continue checking.  The pediatrician says they plan on checking him every six months.  Luckily, that’s an easy check, no equipment necessary.

Otherwise, Christopher is free to swim for fun, to walk, play on the playground, whatever he feels energetic enough to do.  We began schoolwork again after two weeks and are back at a full schedule.  He isn’t having any trouble completing his schoolwork most days.  In fact, he is finishing the last few exercises of sixth grade math in Khan Academy, having finished his textbook last week.  But he is likely to still have low energy and some neck pain for 2-5 months.  This may be an easy brain surgery, but it still takes a good six months to fully recover.

Vladivostok Station, Preparing to De-Board

April 12, 2016, 11:15am

Christopher's nest during recovery.

A year ago today, we woke up at 8am in Inova Children’s Hospital to receive an official diagnosis of Chiari I malformation.  That day, the idea Christopher might need brain surgery to relieve his headaches was scary and mind-boggling.  He was pretty emphatic it just wasn’t something he’d allow to happen.  Six weeks later, he’d decided it was worth trying, but by then, we’d discovered the AVM.  April 12 will probably always be a surreal anniversary today.

As expected, Christopher was discharged from Johns Hopkins Hospital first thing Sunday morning.  Actually, we were rather shocked at the efficiency—we were home by 9:30am.  Dr. Teresa, the resident shadowing Dr. Groves last week, came in just before 7:00 to do a final neuro check on Christopher and declare him fit to go home.  She’s a bit more conversational than Dr. Groves.  From her, we learned the nystagmus he developed Saturday is very common after Chiari decompressions.  It started as double-vision when he tilted his head a certain way.  Now, it mostly presents as blurred vision if he tries to read the small print in a book.  He can read clocks and televisions just fine.  It should dissipate on its own in a couple weeks.  But I did ask Groves prior to surgery if he might have any vision issues this time.  “Oh, no, this is a totally different procedure than last time and won’t go anywhere near actual brain tissue.”

I’m pretty well convinced that neurosurgeons compartmentalize the brain.  As in, “we aren’t touching the occipital lobes, where vision lives, so we won’t see vision changes.”  Last surgery, the swelling in the frontal lobe put pressure on the eyeballs, which created double vision and blurring.  As the fluid resorbed, his vision returned to normal.  His opthalmologist, at his post-op follow-up appointment, told us this is a very common side effect of brain surgery.  This time, they separated and retracted his neck muscles to gain access to the skull and cervical vertebrae (and dura).  He doesn’t like chewing because the neck muscles are so sore his jaw muscles working aggravates the neck spasm.  Somehow, his eye muscles have lost perfect synchronicity, which contributes to nystagmusus.  Very common after Chiari decompressions, but not something that triggered when we spoke with Groves.  This time, I was amused.  Neurosurgeons don’t always speak the same language we do, no matter how specific you think you’re being.

But Dr. Teresa also confirmed something we’ve suspected for a long time—even among Hopkins neurosurgeons, Groves is one of the best.  The resident told us Groves’ technique is absolutely impeccable, and she felt it was a major reason Christopher didn’t have any nausea following the procedure.  As infuriating as her conservative, step-wise approach was for all of us, I’m glad we stayed the course.  She was right, every step of the way.

Alfred has spent a lot of time cuddling with Christopher on the couch.  Pet therapy is real!

Once home, Christopher and I took a 4 hour nap on Sunday.  Monday, we tried to establish his post-op routine while I made his various post-op appointments.  Yesterday, his good friend and neighbor Will came over to play, while I ran to the doctor for routine bloodwork and a foot X-ray.  It turns out that sleep-deprivation and stairs don’t always mix.  But my great toe isn’t fractured, just swollen and bruised because the arthritis is back and didn’t appreciate being stubbed.  Stupid toe.  Christopher took a short walk with his daddy to walk the dog.  Today, we went to see the Power Rangers movie.  I think the walk is shorter than what he did yesterday, and he took his pillow, but he was too exhausted for lunch afterwards and just wanted to come home.

If fresh fruit were a Snickers bar, Christopher would weight 300 pounds.  A big shout out to the team at Accenture for sending an Edible Arrangement.  Perfecto!

Christopher doesn’t have a great appetite, but I’m not certain whether it’s the narcotics making him apathetic, or an actual side effect of the general anesthesia.  I suspect the former.  His taste buds are intact this time, and he’s pretty good about getting chocolate milk, fresh fruit, and a salad every day, so I’m not too concerned.  A week after surgery, he has bruised eyes from the exhaustion of recovery.  But his neck range of motion returned to nearly normal Monday, so he’s moving gingerly rather than robotically.  Dr. Groves told us to expect to use the heavy lifting pain meds for two weeks before we started weaning down to just Tylenol and Motrin.

Healing is hard and exhausting work, but Christopher's mood is generally pretty good this time around.

Tomorrow he checks in with the pediatrician, we see Groves and the other specialists again in a month.  We start math in a week, then add a subject per week until he’s back to his full schedule.  Christopher can swim for fun in 6 weeks and play actively with his friends once he feels up to it.  No regular classes or swim team practices for three months, though, Groves doesn’t want that much exertion while he heals.  It will also be that long before we know just how successful the procedure has been in alleviating headaches.

So this is our new normal for a few weeks.  

April 8, 2017, 10:05am

Nintendo Switch, Making Hospital Life More Fun.  Trust Pitsenbargers to Bring the Good Toys.

Christopher had a great night.  The bradycardia he had the first night is gone.  His neck got pretty stiff yesterday, so he kind of moves like a robot, turning his head and trunk together.  But PT came yesterday to do a full evaluation and they’re thrilled.  He isn’t just moving in the normal range for post-Chiari decompression, he’s at a superior level.  They also did a screening to see if OT should visit, and cleared him based on his successful completion of all fine motor and activities of daily living (ADL).  So no prescription for outpatient PT services, just gentle home stretching exercises every 1-2 hours until he next regularly scheduled PT monthly follow-up in two weeks.  Should something to change at that point, his regular PT, Hannah, can handle it.  Because they didn’t cut the neck muscles, just separate the fascia between them prior to retraction, there’s no risk of scar adhesions forming without PT.

Pain control is excellent.  He wanted a fruit cup, the mango that came with his lunch was truly nasty.  He wanted to walk with me to the cafeteria, which is a long trek from the pediatric tower (Bloomberg).  He quietly told me as we were nearly back that it was too much and he hurt a lot.  No anxiety or irritability or stress, just a quiet statement.  So of course, PT showed up shortly after we got him back into bed.  But he was happy to do everything they asked.  His only IV med is the steroid at this point, everything else is by mouth.  Switching the Dilaudid to a pill every 4 hours is giving him much better pain coverage, hopefully that continues.

Hopefully, because he’s still asleep.  I LOVE Hopkins Peds General Floor.  His night nurse shifted his med schedule last night to line up with his neuro exams so she only had to come in once every 4 hours, she kept all the lights off, so he just went right back to sleep.  This is their standard modus operandi, not just a great nurse.  If you have a choice of hospitals for a several day inpatient stay, I’d definitely ask about night care. The studies on maintaining normal night-time Circadians clearly demonstrate shortened recovery time and less dependence on pain meds.  There’s a whole host of reasons that isn’t very practical in the ICU, but anywhere else, I feel like it’s a crime to be barging in every 1.5 hours.

Dr. Groves came in to see him just before 7am.  She is still shocked at how “surprisingly well” he’s doing.  He’s already meeting most of their metrics for discharge, so she’s put in the orders for tomorrow so they can have the pharmacy start filling his prescriptions today.  She says that seems to be the rate-limiting step in leaving.  Should something develop today where he needs/wants to stay an extra day, she has no problem keeping him, but she says there’s really nothing more here than what we’d be doing at home.  So, woo! and hoo!  He’ll probably need to stay on the Dilaudid/Valium for 2 weeks, liberal use of Tylenol/Motrin for several weeks after.  We’ll follow-up with Groves at 4-6 weeks.  He can return to active play about that time, just no regular classes like ballet or swim team.  Too much exertion.  He can return to gradual school work in 2-4 weeks, letting his energy be our guide.

He woke up at 9am!  He pretty calm about it, but he is at a level 8.  Morning meds are on board except the Dilaudid, that requires breakfast.  But Heather and Bryson Pitsenbarger arrived with Star Wars drones, so he is now properly distracted (and the Valium is kicking in).  I’ve ordered breakfast, I should have just guessed what he wanted while he was asleep, but it should be here before 10:30am.

Even Box Drones Need Calibration!

The X-Wing is on the floor, the Death Star is hovering in the background.  

April 7, 2016, 11:15am

Nothing says "Happy" like getting out of PICU.

PICU overnight was not a total nightmare.  First off, Nurse Olivia was amazing.  When the cement in Christopher’s hair dried enough to start bothering him, she offered to syringe wash it.  She spent 45 minutes gently lavaging the hair and removing the ook with a comb while keeping the incision site clean.  It still definitely needs a shampoo, but he was comfortable enough to lay on it again.  And the incision site stopped oozing, we switched to a new pillowcase afterwards, which stayed clean all night.

But it’s PICU, so they’re doing checks every 2 hours and the alarms aren’t silenced.  We got to hear about it every time the computer didn’t like the respiratory rate or blood pressure readings it was getting from the arterial line.  Christopher didn’t believe us when we told him that using his Dilaudid pain pump wasn’t going to prevent him from going home on time, so he suffered more than he needed to.  He receives Valium and Tylenol every 4 hours without choice, so he wasn’t handling pain completely med free, but it could have been better.  At 6am, we gave up attempting to sleep and he watched the original Tron movie after hitting the Dilaudid button.  The muscle pain in his neck is settling in now that the anesthesia is fully out of his system, though, so he had a rough period around 8am.  He was in enough pain he couldn’t focus on listening to his audiobook the way we did last night.  His range of motion is definitely restricted today, but he’s getting in and out of bed to use the restroom fine.  He’s had no nausea of any kind.  From neurosurgery and pain management’s perspective, he’s fantastic, so they actually approved orders for him to transfer to the general floor even before morning rounds.

And that means—we’re on the general floor!  Before 9am!  I didn’t even think that was possible in a hospital.  Arterial lines aren’t allowed on the general floor, so that is out, much to his delight.  Pain management wants to try transitioning him to pills, which also pleases him.  IV Valium burns going in.  Last night, it didn’t bother him much, but the most agitated he’s been, was his last Valium dose.  As luck would have it, pain management was in the room to witness it, so they switched him to oral Valium before his next dose.  The only delay was making sure he tolerates solid food, as he wasn’t allowed anything except popsicles last night.  Supermom that I am, I bought him a chocolate croissant with my chai latte and scone this morning so that I’d have food ready the moment they allowed it.  I had a feeling it wouldn’t happen before breakfast time.  He was thrilled and ate the whole thing.  No nausea, no weird tastes, great appetite.  So apparently we can blame just about all of the digestive trouble last go-around on Keppra.  He is a mite more irritable than normal, but no massive roid rages like last time, despite being on IV Dekadron again.  

His lunch order arrived, a bacon cheeseburger and salad.  He’s eating it in between naps, I think his Circadians are backwards!  No worries, last night was rough and he needs the sleep.  He just got released from live monitoring.  They are trying to wean him off the PCA pump, which may give better pain coverage, as pills last 4 hours instead of quick IV burst.  He may also graduate to 4 hour neuro checks soon.  There will remain a concern for the development of meningitis and CSF leaks for a week, but his sodium checks have been clear so far, nor is there oozing in the incision site or bubbling under the wound.  We have the same room we had last time we were on the general floor, and since Hopkins separates the floor by age group, we don’t have any crying babies nearby, only school age kids.  This makes everything so.much.quieter.

Seriously, it’s hard to believe he just had brain surgery.  Everything is really quite relaxed.  I haven’t had much time to knit today, but I have nearly completed Section 1 of my blanket.  In 2.5 rows, I’ll begin the lace section.  Last surgery, I couldn’t bring myself to knit because that meant time to let my thoughts wander.  They raced, not wandered, to very dark places, so I didn’t place a single stitch after the AVM.  It didn’t help I was starting a new project that required learning new stuff, and I had no confidence I had the focus at that juncture for acquiring new skills.  This time around is a much different experience for us all.

The beginnings of a blanket, it's ~40" wide.

Vladivostok Station, The Chiari Decompression Surgery

April 6, 2017, 11:30am

A little light reading before brain surgery

We have learned so much about pre-op, people.

First off, nobody cares if you puke before surgery.  You can have all the gastroenteritis you like out of either end, just don’t develop any respiratory symptoms within 2 weeks of your surgery date.  I know this because, for 3 of Christopher’s 5 procedures, he’s had gastroenteritis the week of surgery.  The PACU nurse said hopefully today’s surgery will stop all that.  So maybe there’s a connection between Chiari and gastroenteritis.  Christopher is certainly the most sensitive member of the family in that area.

Second, screw bedtime.  Calories are way more important.  If your child can eat until midnight, make sure they eat as close to that as you can get.  Waiting in PACU before surgery sucks as an adult, nobody needs a hangry child.  To this end, we took Christopher to Cold Stone Creamery and had him pick out a sundae at 9:45pm.  He only ate half, but that’s okay, because then we started Pirates of the Caribbean and I handed him 3 buttered mantau (Chinese steamed buns) at 11:40pm.

Third, wake up early enough to get in 8 ounces of clear, sugary liquids at the last minute.  We were told he could have clear liquids up until 2 hours before we had to report to the hospital at 6:45am.  So at 4:15am, I woke that child up so he could come downstairs and drink 8oz gingerale before the cut-off.  See, Calories are important, see above.  Also, sore throats after intubation are Christopher’s primary complaint the first several hours he’s awake.  He cared far less about the sore legs from the angiogram catheter or the headache from the brain surgery than he did the wick sore throat.  Adding insult to injury, it was hours before they let him start sipping fluids or popsicles to ease that sore throat after the AVM surgery.  So.  Hydration.

Fourth, don’t let your older child fool you into thinking they don’t need stuffed animals for surgery.  Maybe they no longer take them on vacation with them, but if fluffies still live on their bed, a few should go to the hospital.  Heather Pitsenbarger and Summer Yen saved us last time by getting Christopher a giant orca (apparently the best body pillow ever) and a neuron (instant rapport with medical types).  He initially told me he didn’t need them this trip, but when I countered, he didn’t insist.  Both fluffies had prime positions in both his waiting room chair and on his PACU bed.  The neuron, by the way, excited his anesthesiologist so much that she asked to photograph it.  She’s also a brain researcher studying the effects of brain injury, so she’s now buying giant stuffed neurons for her researchh team this Christmas. (Side note:  Are all Johns Hopkins anesthesiologists brain researchers?  Or just the ones that work on neuro cases?)

Notice the critical placement of fluffies while he reads.

Following these rules, for the first time ever, Christopher didn’t complain once about being hungry.  They’re using a nasogastric tube instead of just an esophageal tube this time, so who knows what his throat will feel like.  We may be trading a sore throat for a swollen nostril and a nose bleed.  Just like last time, he’ll be under anesthesia long enough to wake up swollen and rashy, but it should dissipate within a few hours.  The NG intubation is due to the fact that he’ll spend this surgery prone (on his stomach) with his head/neck pinned in place.  The neck angle Dr. Groves wants isn’t conducive to a standard esophageal intubation.  Having read his reports from the last two procedures before they came to visit us, the anesthesiology team was already prepared for Christopher to have emergence delirium and will give him fentanyl as necessary to prevent it.  His bed in PICU has been confirmed and a very nice concierge spoke with us to see if we needed a tour.  It wasn’t so scary watching Christopher shake as the nitrous oxide (laughing gas) took effect.  For him, it’s a misnomer, it doesn’t make him slap-happy.  As an observer, it would be easier to watch if he went to sleep via IV push.  But he prefers they put the IV in while he’s asleep, and he’s a young enough patient, Johns Hopkins Hospital policy is to let him choose.  As a parent, I try to give him agency over his body wherever possible, and this helps him feel like he’s got some measure of control over the situation.

Unfortunately, Christopher wasn’t quite able to finish The Fellowship of the Ring before his brain went mushy this morning (yeah, yeah, 4 hours’ sleep, blah, blah, blah).  But he’s really close.  He did, however, really make the anesthesiologists laugh when we were talking about their dosimeters and Marie Curie characterizing radiation.  When noted that she got a unit of radiation named in her honor, he shot back with a crack about her dying of radiation cancer and a unit not doing her much good after she’s dead.  Touché.  

We are currently about an hour in to surgery.  Dr. Groves did warn us that we may see a recurrence of some of the issues that cropped up after the last surgery because those neurons are weaker and more susceptible to injury.  It would be a regression from his current baseline, not a complete re-injury, and since she made us wait so long between surgeries, it should be minor.  But we did make sure he understood he could wake up with some left side weakness.  The surgery won’t technically affect his vision or attention span, but until he clears the general anesthesia completely from his system, he might not be reading much.  So we’ll take things as they come.  He probably won’t be eating until tomorrow to prevent vomiting tonight as his body recovers from the initial shock of the decompression.  Now we wait.

April 6, 2016, 2:30pm

Christopher is out!  

Christopher is resting comfortably.  Last time, he thought he'd sleep through PICU and not remember the experience.  This time, when he had no expectation of it, he might well sleep through most of his time here!

Dr. Groves removed the bone from his posterior fossa and C1 as planned.  The dura was thickened and when she used the ultrasound probe after removing the bone, she found the CSF flow was still very restricted.  So she placed a bovine pericardium patch during the duraplasty, which restored pulsation nicely.  In the short term, this means Christopher will have more pain in recovery.  There is now a risk for a CSF leak and meningitis (either bacterial or chemical) that wouldn't have existed without the duraplasty.  The PICU attending is a seasoned physician who has treated lots of Chiari decompressions with duraplasty, however, so I feel confident we have an incredible team.  They aren't too concerned about his blood pressure this time around, but are keeping the arterial line in to monitor his sodium levels, which can be problematic following duraplasty.  The key to his recovery will be getting him up and walking tomorrow.  Dr. Titsworth, one of the other neurosurgeons on the team, has already been in monitoring him for neuro and pain responses; we remember each other from the AVM resection.  

This diagram shows the side view of where the posterior fossa was removed (craniectomy) and the spine of C1 (laminectomy).  Image courtesy of Mayo Clinic.

In this diagram, you can see where bovine pericardium (dura patch) has been stitched into the existing dura.  Dr. Groves says she prefers bovine tissue because, in the ten years they've been using it, they see patient cells migrating into the patch and becoming one with the piece.  It also, in her experience, produces the least amount of scarring.

So far, Christopher is resting very comfortably and is just barely waking up.  He reports a sore neck, but was able to prop himself up to swallow his acetaminophen dose at 2:00pm.  The incision at the base of his neck is very minimal, they did a great job minimizing the stitches.  The PICU attendings and resident spent a LOT of time asking "how we got to here", although it's obvious they read his whole file first.  I think we got the A team, even his nurse seems more experienced than last time.  He is on Dekadron (steroid) again for a few days to control inflammation, but no Keppra.  He was given a large fentanyl bolus as he was exiting surgery to begin pain control as he clears the anesthesia.  No emergence delirium today!  I'd like to think he's just become a pro at this.

I'll try to get a better picture when he's awake and can turn over, but the incision site is much smaller than we anticipated.

The pain management team has also been in to check on Christopher.  In addition to IV valium around the clock to help with the neck muscle pain, they've given him a dilaudid PCA pump so that he can administer his own pain control.  His respiratory rates are still pretty low (his computer isn't happy), but nobody seems too worried about it.  They have his monitors silenced, so we hear the noises of the patients and practitioners on the floor, but no awful beeping.

It will be 2-3 months before we know what his new baseline is for headaches, but I'm hopeful we're almost off the Transsiberian Railway for good.

Pulling in to Vladivostok

Headed to Johns Hopkins in the pre-dawn hours.  Orca and ganglion fluffies, Star Wars blankets mandatory.

April 6, 2016:

It’s been over a month since we saw Dr. Groves and she agreed to do a cervicomedullary decompression of Christopher’s Chiari I Malformation.  

I can spell it better than I can say it.

Christopher was released from weekly PT in early February, and still shows only minimal balance and precision issues on the left standing leg.  But the absolutely remarkable headache relief he got from acupuncture started eroding as he returned to his favorite activities.  Both behavioral psychology and his neurologist asked him, in September, to begin acupuncture to alleviate headaches.  He was extraordinarily resistant, as Dr. Groves had explicitly told him that nothing external relieves a Chiari headache.  But his headaches have three components:

1) Pain produced from the compression of the bony skull (posterior fossa) on his brain stem and cerebellum—the root cause of the Chiari headaches

2) Pain produced from the muscle tension of defensively hunching against the compression

3) Pain produced from a positive feedback loop in the brain as a by-product of chronic pain.  This pain, while purely biochemical in nature, feels absolutely real and is indistinguishable from pain with physical causes.

The goal of acupuncture is to reduce or eliminate Pain Types 2 and 3.  Studies show that acupuncture sites show both an increased blood flow and a release of the body’s natural opioids (painkillers).  There is also evidence that the relaxing touch of the acupuncturist brings relief, just like in massage.  Christopher, despite the urging of his medical team to try acupuncture, was certain it was voodoo and only agreed to do 6 sessions as part of his behavioral plan to discharge behavioral psych services.  After the first treatment, his daily baseline level 6 headache dropped several notches for 3 hours.  A week later, the second treatment dropped the headache for 3 days.  At this point, while he had decided acupuncture actually did work, he wasn’t sure it was worth the discomfortort of the needles to continue.  Week 3 acupuncture dropped his headaches for 6 days.  And Week 4 gave him a full 8 days of relief.  By the time we reached his mandated 6 sessions, Christopher was an acupuncture convert.  His baseline headaches had dropped to a level 1-2, and he was only have 1-2 days a week where the headache spiked to an 8 from barometric weather changes or activity, instead of nearly every evening.  So we continued weekly acupuncture treatments until January, when he started twice monthly sessions.

Just prior to that, in December, his rehab physician, Dr. Korth gave Christopher permission to return to all developmentally-appropriate activities except full contact sports.  Tag! Cops and robbers!  Monkey bars!  I even got grudging approval for skiing and ice-skating provided he wore a helmet and had careful supervision.  In January, Christopher received permission from his swim coach to return to full swim practices, so he began swimming 60 minutes, 3 times a week.  As we had an unseasonably warm winter, he began playing on the playground at co-op the Wednesdays he felt energetic enoughh, and started playing Nerf and other games outdoors.  We went skiing in January, once for a day trip, again for a 4-day family holiday.  Christopher did need a semi-private lesson his first ski day to relearn how to turn with the left leg, as he’d lost the muscle memory and ended up skiing backwards down a green after attempting a right turn.  Oops.  Clearly, I should have checked his slalom skills on the bunny slope first.  By the end of our ski week, Christopher had done another semi-private lesson to learn parallel turning and get cleared to ski blues.  He was skiing like a demon!  But he also developed a wicked headache after the third day, anxiety attacks, and extreme nausea driving down the mountain.  The exhaustion and headache spike lasted several days once we were back home.

So when we went to see Dr. Groves February 28, she felt comfortable attempting a Chiari decompression this spring.  Christopher is physically strong, has only minor deficits in the left standing leg, has only minor cognitive deficits, and exhibits strong exertional and barometric exacerbations of his symptoms—classic Chiari traits.  She reiterated that he may well always have a baseline headache, but that she hopes to eliminate the spikes.  She hopes to only remove the bony tissue of the skull base (cranioectomy) and C1 spine (laminectomy), but Hopkins is very aggressive in doing duraplasty if the ultrasound after bone removal doesn’t show the cerebrospinal fluid (CSF) flow rates they feel are minimal for significant symptom relief.  Failure to do a duraplasty in the first decompression attempt is the primary reason for second attempts.  While only a 10-15% risk, they’d prefer this be Christopher’s only Chiari decompression procedure, so the threshold for opening the dura and putting in a synthetic patch to create extra room (the tough membrane surrounding his brain won’t naturally enlarge after the bone is removed) for CSF flow is extremely low.

This surgery is extremely painful.  We were hopeful that since he’s already had a craniotomy and vascular brain surgery, Dr. Groves would tell us he already knows the worst.  But it turns out that retracting the neck muscles to gain access to the bone for 3-4 hours produces both muscle weakness and lots of pain.  And nausea.  They will give him both IV zofran and valium for the nausea and muscle pain in addition to fentanyl and acetomenophen.  Because he’s no longer a stroke risk and she’s not touching actual brain tissue, they can also use IV ibuprofen for pain relief.  But we’ve been warned their best efforts will be at the limit of blunting the symptoms.  On a more positive note, he should only be in PICU overnight this time before transferring to the general floor.  We do expect a total 3-5 day stay at Johns Hopkins, but both Dr. Kramer and Dr. Groves confirmed that inpatient rehab stays are not part of Chiari decompression surgeries.  The first two weeks are likely to be pretty bad, but by 6 weeks, we should know what his new baseline is.  Dr. Groves plans to return him to most activities by 3 months, and full contact sports by 6 months’ post-op.  She’ll let him get in the water to swim for fun at 6 weeks, but told him not to do swim team this summer.

Christopher feels like enduring this is better than the alternative of living with his current headache status.  We didn’t have the direct surgery-related anxiety attacks we had prior to the last surgery (no fear of the unknown), but he has been more anxious and more easily frustrated leading up to surgery.  Furthermore, after a final ski trip St. Patrick’s Day weekend, he went back to level 8 daily headaches for about two weeks.  He’d asked to stop acupuncture because he didn’t get the magical drop in headache level each time he went anymore and his baseline had creeped up to a 3-4 even before the last ski trip.  But when we scheduled two acupuncture visits in a week, we finally broke the level 8 cycle and returned to a 4 baseline.  It turns out that acupuncture, while still more effective than anything else we’ve tried, is only so good at mitigating the effects of a normal 10-year old boy’s daily activity and energy.

As a side note, we stopped noticing significant memory lapse events in January.  A neuropsych re-evaluation March 13 by Dr. Megan Kramer confirmed that his memory and attention skills are now commensurate with his age.  Interestingly, while his performance is somewhat variable, with complex tasks often earning above average scores, while tasks he perceives to be easy, often only scoring in the average range.  But the perseveration and impulsivety noted right after the AVM surgery in August are now gone.  Verbal and mathematical reasoning both score in the superior range (99th percentile).  Flexibility and efficiency in problem-solving and emotional regulation are still uncomfortably low.  Christopher will likely always need spell-check, but we’ve been encouraged to attempt a spelling curriculum that uses context to encode the rules next year before giving up on the subject.  Dr. Kramer has officially recommended that Christopher be given 150% time on all assignments, tests, and projects to accommodate his need for rest breaks to avoid physical and cognitive fatigue.  It is too soon, at this point, to know whether this is purely a Chiari effect, or residual healing from the AVM resection.  

As homeschoolers, we technically don’t need official 504 codification of these recommendations.  As a parent, it is affirming to know that letting him take each day as it comes, scheduling his energy as a balance between reading assignments on the couch and desk work, shortening assignments as necessary is exactly what both neuropsychology and neurology recommend based on his scans, symptoms, and cognitive performance.  In the long game, it’s important to document these accommodations now so that if he shows a significant drop in his annual ITBS scores over past years following standard administration this summer, we can codify them through the Seton Testing service we use so that it’s part of a long-standing record when it comes time to take the SAT, ACT, PSAT, and AP exams.

So, as we enter the city of Baltimore,

One last time

Relax, have a drink with me

One last time

Let's take a break tonight

Hamilton, One Last Time

Chugging toward Vladivostok

Traveling the Transsiberian Railway (A Movie of His Surgery and Rehabilitation).

Six and seven weeks’ Post-Op, October 10, 2016

Drs. Ahn and Tamargo couldn’t stop grinning, they were so pleased with his progress.  They were so interested to know when he first walked without a wheelchair, and other questions.  Dr. Tamargo did confirm all Christopher’s worst thoughts about neuropsychology by telling him their diagnoses are pure conjecture.  Dr. Ahn was more careful, saying the testing was done too soon to be certain, and would need to be re-tested.  They gave him full clearance to return to any and all of his previous activities, “as though he’d never had brain surgery”.   A standard neurological exam (think touching your finger to your nose, walking a straight line) showed only minimal impairment.

Additionally, they took Christopher off Keppra.  Hallelujah.  Most of his taste issues went away within days, but his pediatrician, Dr. Kapoor, had to put him on Zantac to alleviate the severe stomach and intestinal pain.  We nearly did testing for C. diff, but the Zantac kicked in so quickly it wasn’t necessary.  I’ve fought reflux for decades, but I didn’t connect his pain to that, so I’m very grateful the pedi did.  The anger and anxiety disappeared pretty quickly, too.

We saw Christopher's headache specialist October 10 and his physical medicine physician 1.5 weeks ago.  Langdon says, night and day, he's a different kid than she saw this summer.  Korth had only ever seen him the day after surgery when she assessed him and recommended he go to Kennedy Krieger for inpatient rehab.  It took her 20 minutes to connect Christopher with the kid who couldn't move in the bed (he had a hoodie on, so no visible craniotomy scar).  And then her jaw dropped.

While the neurosurgeons feel he can return to any activity he previously enjoyed as though he hadn’t ever had brain surgery, both Dr. Korth and Dr. Langdon have some concerns for getting him to the one-year surgery anniversary.  But, as bad as things were, everyone is completely amazed at his recovery thus far.  We are no longer walking through a living nightmare.  Christopher's headaches and mental state are better than they've been in over a year.  He still gets exhausted easily and we have to carefully plan his school day around outings and therapies.

There was some need for oculovestibular therapy to correct some residual vision issues from surgery.  Luckily, his outpatient PT caught it and prescribed exercises so that they corrected before we were able to find and schedule OVT.  Dr. Korth is also very concerned that his left ankle remains very weak, affecting his entire trunk balance and stability.  She doesn’t want him participating in recess games like tag or soccer for a year.  That did.not.go.well.  She did give Christopher permission to begin a ballet class, so I’ve spoken with Skye Ballet, the studio Alexander attends, and they’ve agreed to let Christopher join.

N.B.  His first class was a major success!  He felt so good to be jumping without a spotter and to just be part of a regular, active, group of boys again.  Furthermore, his behavioral psychologist is thrilled because of all the research showing major connections between ballet and memory.  Win-win.

Three Months’ Post-Op, November 22, 2016

We had the first follow-up MRI today, along with appointments with Dr. Ahn, the vascular neurosurgeon, and Dr. Groves, the spinal neurosurgeon.  When Christopher was shown the before and after images of his AVM site, he exclaimed, “Look!  Even my ganglia have an Olympic sized swimming pool!”  Such a swimmer.

More importantly, the MRI does.not.show any regrowth of the AVM.  They can begin to regrow in as few as 30 days, so this was welcome news.  The overall chance of regrowth is only 5-10%, so Dr. Ahn is optimistic it will never be an issue again.  Still, Christopher will need a repeat cerebral angiogram when he’s 15 to make sure.  That way, if it does return, there’s time to remove it and rehabilitate without affecting Christopher’s trajectory for college.  Again, Dr. Ahn smiled during the entire exam.  He’s a fairly quiet Asian man, so it’s amazing to watch how pleased he is with Christopher’s progress.  Honestly, we are, too.  The brain of a little kid is a truly amazing thing….

Dr. Groves’ appointment wasn’t until after lunch, after we’d done the first two appointments.  Christopher grew noticeably quieter and more agitated as it approached.  We were really hoping she’d be ready to schedule a Chiari decompression in January, Christopher has been working really hard in PT and in shortened swim team practices towards that goal.  Dr. Ahn had just told us he saw no reason to wait any longer, he’s fully healed from the AVM procedure.  But no.  Dr. Groves, we’ve learned is extremely methodical.  But we met a patient at Kennedy Krieger who, despite a devastating spinal cord injury from a motorcycle accident, will walk again because of her caution and skill.  So, while Christopher really hates her pace, he has the utmost trust in her abilities, thanks to a 21 year old’s recovery.  Hindsight being 20/20, we also feel that she was probably more honest with us regarding just how tough the AVM surgery was going to be than the vascular docs.

At this appointment, she warned us that she thinks she can make the headache spikes go away with surgery, but not necessarily move the baseline lower than what he achieves with acupuncture.  His rehab physician said the same thing in December.  Furthermore, because general anesthesia itself causes memory, attention,  and appetite changes, Dr. Groves wanted Christopher to wait 6 months from the AVM resection to be put under again.  There is evidence in children that too many anesthesia events, too close together, can make the changes permanent.  He'd just started doing regular swim practices again, but only at 30 minutes, so she also wanted him swimming full practices before another surgery as an indicator he was back to his pre-surgery strength and conditioning.  She doesn't anticipate he'll need PT this time, although she always requests a PT consult in hospital, but she does want him to be as strong as possible before he goes through another major surgery that will sideline him for 6-12 weeks.  She did discuss details of the surgery with us, so I don't think she doesn't want to do it at all, just that she is very conservative and doesn't want to create unreal expectations.

Six months' Post-op, February 3, 2017

Christopher's recovery remains pretty spectacular.  To look at him, only his PT knows his weaknesses, and maybe his swim/ballet teachers.  We even took him skiing in January!  His first ski day, at Bryce Resort, was a bit bumpy, because he lacked the muscle memory for turns using the left leg.  A semi-private lesson with his brother cured than in under 90 minutes and he was back to skiing greens like a pro.  Two weeks' later, we went to Snowshoe for the week, and when he was bored of greens, a semi-private lesson to clear him on blues was in order.  He had trouble in the lesson putting together the components of parallel turns, instead of the snowplow turns he's used up to this point.  But the next day, he nailed it and was flying down the mountain with great control--and parallel turns! He also went swimming, climbed the rock wall, spun in a Eurobungy that week.  We didn't plan the trip to coincide with the six month anniversary of the AVM surgery, but it was a great way to spend the week.  Rehab medicine and PT have, obviously, now cleared him for all but heavy contact sports.

Christopher's headaches are fairly stable.  His baseline Chiari pain has bumped up a bit to a 2/3 now that he's doing full swim team practices, skiing, etc.  He has a Chiari headache spike to a 7/8 on co-op days and whenever the barometer is very high or low (strong storm system days), so at least 2 days a week.  He isn't having full-blown migraines, but does get small break-through ones on bad barometer days.  The craniotomy site also often develops a headache on storm system days, but his neurologist feels his head is young and plastic enough, it will fade with time and not be a permanent weather vane.  He is still doing acupuncture every 2 weeks.  

Christopher has a full school schedule and is doing great in his school work.  Many days we have to play Tetris with the order of his work to compensate for his energy level.  Mondays always cause trouble because he swims for an hour, then goes straight to PT, and comes home physically exhausted.  So he does a lot of reading and some of the written work has shifted to other days.  When I told his neurologist my concerns that, at current stamina levels, he'll have trouble completing high school and college coursework, Dr. Langdon assured me it isn't problematic.  She has high hopes for Chiari surgery (more on that later), but says that she has lots of chronic headache patients without a surgical option, and she writes 504 accommodation plans for them even in college.  Mostly, she eliminates busy work and asks, for example, for 20-30 core quality math problems instead of 50 total.  This is pretty much what I've done with his schedule, so that's comforting.  English is a bare-bones reading and handwriting operation at this point, with his Latin studies doing all the heavy-lifting for grammar and vocabulary.  Comprehension and composition come primarily mostly through his history activities.

We discussed all this with Dr. Langdon this week.  She agrees with everything Groves told us, but doesn't think there's a reason for further delay or to avoid surgery altogether, now we're beyond the 6 months.  She thinks he will get significant relief from surgery, even of the baseline, but thinks he'll likely always be a headache patient.  Migraines, once triggered, often don't just disappear--even if you remove the proximate cause.  Langdon thinks revisiting Moores, the Inova neurosurgeon, could help us feel more comfortable with specifics, but would prefer Johns Hopkins do the procedure since that clinical team has all the notes and experience of his last surgery.  Since she has privileges with Children's National and Inova, not Hopkins, this advice is not earning her money.

The symptoms that earned Christopher an EEG are periods of 5-7 days of extreme clumsiness that happened in October and then in November.  I initially thought they were occurring because he was spending less time resting on the couch between school and PT activities, his brain was having to work at a new speed to control his body, and needed to learn to work faster.  This is the AVM recovery/multi-tasking explanation.  Growth spurts also cause clumsiness, and he's grown nearly 3 inches the last six months.  Langdon says they could have coincided with really bad headache weeks, as well.  Chiari malformations can put enough pressure on the brain stem to cause clumsiness, too.  But around Christmas, he started dropping things with just the right hand.  It went from every few days, to daily, to twice daily over three weeks before I called his pediatrician.  She was very concerned because it wasn't bilateral and asked me to call Langdon because she thought he needed new brain scans.  Langdon agreed and sent us to the ER for an MRI with contrast.  That was clear of any signs of swelling or bleeds.  The Chiari looks the same as last April.  In the absence of a structural cause for the clumsiness, Christopher is probably normal for him.  But since major brain surgery can cause seizures, and small seizures can present as clumsiness, Langdon felt an EEG was in order.  We'll get those results in 10 days.  When I called Johns Hopkins to schedule his next appointment with Groves after the MRI, I told the secretary briefly about it, she spoke with Stephanie, our favorite PA, and they asked he be seen this month instead of in the next three.  That is scheduled for Feb 28.

N.B.  Those EEG results were also normal.