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