LCH-Free At Last

We got great news this morning after Julia’s latest check-up MRI yesterday: there is no more evidence of active LCH anywhere in her body. The lesions in and around her skull have disappeared, and the ones in her spine are healed except for small compression fractures in four vertebrae. These may heal in time, but even if they don’t, they aren’t affecting her ability to walk. She has two more chemo treatments, with her last one being August 9th. After this, she’ll undergo progressively less frequent check-up MRIs to make sure it hasn’t come back.

Hearing the news was almost surreal for me. The pediatric oncology department hadn’t even looked over the results yet, since they received them the previous evening and I asked about it in the middle of the following morning. The doctor read a few excerpts from the report for the first time out loud as he answered my question. Talk about an endorphin rush! I couldn’t help smiling, and I almost couldn’t help crying. Maybe. A bit.

We are extremely grateful for this outcome and for all of the support we’ve received through this challenging process. Julia sure has a lot of friends!

18 Months: Toddling, Thinking, Talking, and Treatment

Today, Julia is 18 months old. Coincidentally, it is also Father’s Day—my second as a father, and a great time for me to appreciate that fact as Courtney and I enjoy our delightful and fascinating daughter.

Over the last five months, Julia has begun developing more confidence in movement, new cognitive abilities, and lots of new language skills. She’s got a bit more hair, although not quite enough for us to do typical girly things with yet. She engages in a variety of playtime activities, loves books and music, and has a memory that constantly amazes us. Her day-to-day changes have felt minor, but a side-by-side comparison is remarkable.

Toddling

She progressed from timid walking to the toddler’s clompy version of a run, and she wants to climb everything (which my parents frequently point out to be a genetic trait inherited from her dad). She climbs onto the couch, dining chairs, stools, the fence around the deck, the rocking chair in her room, her Bumbo, and her high chair. This can be a bit unnerving, but we try to let her do everything except what would be inherently unsafe no matter how old she is. I’d much rather let her learn while we’re watching and ready to catch.

She has begun carefully navigating low steps without help, which shows improved balance. Only a few days ago, she repeatedly sat down on a box and then stood again by shifting her center of gravity forward enough to stand—also forward enough to fall over without correctly standing, but she got it every time without help. It’s gratifying to watch her learn the little skills that adults take for granted.

Also, I’ve had the fatherly pleasure of getting to play a little rougher during the evenings, tossing her onto couch pillows or rolling around on the rug with less fear of breaking her tiny self. She giggles and laughs like crazy and says “Again!” until I eventually give up, so I know we’re in agreement about what constitutes “fun.” I will treasure that kind of play forever, and I hope she will, too.

Thinking

More than three months ago, I ran some errands that included getting a haircut and picking up a new pair of glasses. When I returned and walked into the kitchen, Julia popped her head up from where she sat on the couch 20 feet away and stared me quietly for five seconds or so. Then, she made her “surprised” face with a baby gasp, and said “Gwasses!” At 15 months old. (She didn’t say anything about the haircut, but I’m not complaining.)

It’s abundantly clear that Julia is making great progress in her brainy bits. She is intensely curious, studying every new thing or person that comes her way. She also remembers events and details to a surprising degree, and obviously recalls them regularly. She correctly identifies a few abstract thoughts and emotions like “sad,” “happy,” and “funny,” and uses those words (and ridiculously adorable facial expressions) to communicate those ideas to Courtney and me.

She remembers what we’ve told her not to do, and almost always refrains from doing it or stops with a quick word or motion from either Courtney or me. Or, she’ll start to do whatever we’ve told her not to (such as reach for an outlet or open a cabinet), then repeat our “No no!” command and pull back. It’s pretty entertaining.

I wonder sometimes whether she has started to dream coherently at night, too. When I wake her in the morning (or she wakes by herself), she usually starts jabbering about random things that she has no apparent reason to think of based on the objects visible from her crib. Who knows? It will probably be a while before she understands what a dream is well enough to tell us about hers.

Talking

Julia’s language skills have continued to grow. Her vocabulary is bigger, her pronunciation is great except for the “L” sound, and she will repeat almost any single word we ask. If she wants something, she can often tell us what it is, and she can always tell us what it isn’t. She’s good at saying “no” as the answer to a question, but she thankfully hasn’t got to the point of using it to defy a command (much, anyway).

One of the funniest aspects of her speech and developing personality is that almost everything she says comes out as an enthusiastic squeal, because she really is just that excited about telling people stuff. Also, she doesn’t only talk to people; she will literally tell anything to anything else if she’s excited about it. The examples below illustrate some of this. (You may notice that all of the words and phrases below end with exclamation points. This is entirely accurate.)

“Wift! Pet!” —Since I exposed her to my recently acquired Oculus Rift VR headset and the Ocean Rift experience, she frequently wants to get back into the environment and pet the manatees (which *cough* feel surprising like daddy’s arm and sometimes nibble her toes). Even though I have to hold the headset for her, she seems to love using it. Every time she enters that fascinating world, she lasts longer before pushing out from under it with a decisive “Ah done!”

“Biwd a wight!” —one of my proudest shared accomplishments so far: she likes to sit on my desk and help as only an 18-month-old can as we build a light. First, we put an RGB LED, resistor, and two jumper wires on a small solderless breadboard. Then, we connect two alligator clips (complete with “Om nom nom!” motions) to the wires and turn on the power supply. Ah, young engineers! We inevitably follow this process with making our hands glow different colors by cupping them around the lit LED.

“Daddy! Happened!” —the precursor to an explanation about something that happened, usually followed by “Bonk!” or “Pinched!” or “Tripped!” or “Ouchie! Head!” She loves to recall and relate things that happened to her.

“Bayah button!” —finding her belly button, or that of a stuffed animal or doll. For some reason, she’s fascinated with belly buttons.

“Daddy do it!” —asking me to do something either for her (helping) or instead of her (copying or demonstrating). Corollary: “By sewf!”

“Outside!” —her oft-repeated request to leave the house, whether to swing, get the mail, climb on the patio table, or mow the lawn. Her love for the outdoors continues to confound her mosquito-hating programmer of a father.

“Back seat!” or “Panjer seat!” —asking for us to let her climb into the back seat or the passenger seat. She’s also learning to include “Civic” or “Fusion” in her request.

“Moon! Airpwane! Cwouds!” —telling the moon about the airplane and the clouds, or telling the airplane about the moon and the clouds, depending on context. She really needs to decide on prefix, infix, or postfix notation when it comes to sentence structure.

“Rock! Airpwane, uhver one!” —telling the rock in her hand about the second (or third or fourth) airplane she’s seen flying overhead.

Courtney suspects that many babies have this type of excited interest in everything, but most just haven’t developed the means to relate it to anyone yet. That seems plausible to me, though I have no real idea. Regardless, her enthusiasm is entertaining and infectious.

Just today, on the way to a Father’s Day family gathering, I had a Tom Woods Show episode playing in the car, and the Constitution came up. Julia caught this and said “Constitution! Wead it!” Now, lest you think she’s been indoctrinated with Tea Party talking points: she had recently pulled the pocket Constitution off the shelf in our bedroom, after which Courtney told her what it was, and she asked to read it because that’s her default reaction to any book or book-like thing. (They got through the Preamble before she got distracted.) Her statement in the car was due to recalling that event with Courtney, not urging people to read the Constitution. Even so—wow! Moments later, we got her to say “nullification,” which is perhaps a more important political tool to become familiar with early in life…

Treatment

We’re nine months into Julia’s treatment for LCH, with a little less than three months to go if all goes well. Her last check-up MRI at the beginning of March brought very good news: all of the (few) lesions in her skull have continued to shrink, and the radiologists noted that many of the lesions in her spine, some of which looked like fractures, appear to be healed. By all accounts, she is responding very well. She has another check-up MRI scheduled for the 27th of June, a little over a week from now.

As Julia has grown, the doses of Vinblastine and Prednisone have increased slightly to match her age and size. During her more recent tri-weekly chemo appointments, we’ve noticed more pronounced side-effects from the drugs: less desire to eat normal food, more difficulty sleeping through the night, more attachment to mommy, and heightened sensitivity during the “on” weeks. On the other hand, some of these symptoms also reportedly occur in most babies around this time as a result of brain development and new teeth, among other things. Julia’s behavior is close enough to the norm that we can still gratefully call her reaction to the LCH treatments “minor.” She’s doing remarkably well.

At the same time, we have had some medical challenges. Due to the heightened consequences of an infection with an internal port, doctor’s orders are that she absolutely must have a blood test (counts and cultures) anytime she has a persistent fever over a certain threshold. With a teething baby, fevers are relatively common. Since January, we’ve had to go to her regular clinic once and the ER three times as a result, most recently this past Friday. Two of the ER visits happened to be during a brief trip to California in February for the Gallifrey One Doctor Who convention. Go figure. Fortunately, she passed every test with flying colors, never showing any signs of infection or other risky symptoms beyond being too hot for her own good.

For now, we are continuing to enjoy every moment with Julia, and hopeful about what the next few months of treatment will bring. We’ll keep looking for new ways to pique her curiosity, and maybe I’ll find some time to finally go through the last nine months’ worth of baby pictures and get them online.

Words in Winter

Over the last month or so, Julia has clearly entered the babbling stage. This, frankly, is awesome, because she’s the most adorable creature in the world even without an incomprehensible monologue of cute baby sounds. It also reminds me a little of this. Her language is a lot more fun to listen to though.

In addition to the babbling, she’s developing a real vocabulary of words that we can use to communicate. We’ve compiled a roughly chronological list (in terms of when she started using each word), which Courtney posted to Facebook a little while ago. I’ve copied this into a table here for convenience, and added a few new ones since her post.

Word Julia’s Version
baby (correct)
daddy (correct)
hi (correct)
bye (correct)
boot boo
balloon boon
moon (correct)
mama (correct)
nana (correct)
mail may-oh
bowl boh
dish diss
dish disses
no (correct)
walk (correct)
home hmm
Mia (correct)
buckle buh-kuh
bug (correct)
ball bah
PCB peh suh buh
Cheerio dzee-oh
water wawa
dark dock
duck (correct)
bottle bah-ohh, baba
movie moo-wa
remote wee-mo
eye eye
ear eww
necklace neh-nee
uh oh (correct)
boo (correct)
E-I-E-I-O yie-yie-yoh!
snow noh

She’s also developed her own substitutions for certain things, where she doesn’t say the correct word but she certainly has the right idea. These substitutions are usually based on either a sound the thing makes, or else the sound we or she makes while using the thing. This leads to some funny associations, as well as quite a bit of overlap.

Thing Julia’s Version
swing weee!
spinning chair
snow sled
Vitamix grrrr!
vacuum
pick-and-place machine
kitchen mixer
drill
lawnmower
weed-eater
mouse ee ee!
snake ssss!
sheep maaa!
owl hoo hoo!
monkey ooh ooh aah aah!
cow mooo!
lion RAAAR!
anything dirty eww!

Not bad for a 13-month-old.

Besides the language development, she’s making some interesting but logical associations. Since Courtney has her call up to the upstairs room (office) window after going for a walk in order to get my attention in the middle of a work day, she now associates “window” with “daddy.” Pictures of houses with upstairs windows in her board books now elicit cries of “Daddy!” while she points to them. This was confusing to us at first, but it makes perfect sense.

One of Julia’s most recent accomplishments—just this morning, in fact—is the ability to use a straw to drink out of a cup. We hadn’t really presented her with any real opportunities to learn this before (no particular reason), but she picked it up after only a few awkward seconds of chewing on the straw, then leaving it in her mouth and waiting for something to happen. She’s almost a pro now, though she begins each “suck” with a giant wide-open mouth before chomping down on the end. I guess she wants to make really sure that straw is really in there.

She is developing an appreciation of living vicariously, choosing to push her dolls in her own swing rather than sitting in it herself, and immensely enjoying watching our neighbors sled down the hill in our connected front yard. She still stares at the lingering patches of snow in that part of the yard and yells “weee!” like she did when we were watching the the first time.

We’re also noticing some habits of cleanliness appearing in her everyday activities (yay!). At the end of her bath times, she helps put her bath toys back into the toy bucket while singing along as only a pre-vocabulated one-year-old can to the abbreviated clean-up song. She stops to inspect every little black speck on the floor, often saying “eww!” repeatedly after apparently identifying dirt. (This can be a time-consuming activity when a large portion of the floor is Scotch pine knot wood paneling.)

She’s continuing to grow in all the expected ways, aside from being a relatively low height percentile for her age. She hasn’t managed real balanced walking yet, but she goes all over the place with the walker she got for Christmas, and she can take six or seven steps on her own when we coach her. She just gets too excited about the whole thing and usually tips over forward in squeals of glee. Sgweels, if you will. She’s nearly to the “toddler” stage, but not quite yet.

We have more adventures ahead, so stay tuned!

Julia’s First Year

Our baby girl is one year old today. It’s been an incredible 365 days, no doubt about it. We had a great party this afternoon which Julia thoroughly enjoyed, although she refused to participate in any sort of “smash cake” ritual. She barely even touched (literally) her cake, focusing instead on the mice made out of strawberries that Nana brought.

Anyway, to commemorate this wonderful occasion, I’ve put together a video of moments in her life between birth and now. It’s just under four minutes long. Compacting an entire year of a firstborn child’s life into four minutes is tremendously challenging, but I’m happy with the result. Julia grinned and laughed at it, which is all the affirmation I need.

Enjoy!

One Week Shy of a Year

Today marks the end of Julia’s 51st week with us. We’ve got Christmas and her first birthday party coming up soon and requiring some time and planning effort, so I figured I’d take a short break in between things and post a little update before all of the festivities.

Julia’s second check-up MRI results came back a couple of weeks ago and showed all good news: there are no new items of concern, and all of the LCH lesions on the previous MRI are either measurably smaller or show no growth. This progress means that she can switch to the maintenance phase of her treatment, which means a lot less chemo and a lot less Prednisone. She can finally settle back into a more normal sleep pattern (gradually, with interruptions every few weeks). Her next check-up MRI will be around March.

On a different but also happy note, she’s started to say “dad-dee!” in a manner which makes it obvious that she knows what she’s saying. Also, “bay-bee!” when she sees either herself or another baby. It’s awesome. However, she also says “dad-dee!” when we tell her to say “mommy,” which is a little less awesome. We’re still working on that, for Courtney’s sake. Julia: despite the downward trend in linguistic skills of the average American child, I don’t think you can get by in life with only two words.

Actually, make that three words. We’re teaching her to say “please” and use the circular-hand-on-chest ASL sign to match when she wants something. She can get “peez” out sometimes when she’s not in so much of a hurry that she’s squealing with delightful anticipation. I suppose, being totally honest, that “dad-dee,” “bay-bee,” and “peez” with a lot of pantomime might actually keep someone alive for a while in a crowd of observant people.

She’s also starting to eat more normal food and less formula and milk, and on a more regular schedule. We try to feed her basically what we eat when we eat it, focusing on the healthy stuff. We figure, if she doesn’t even know what ice cream or refined sugar is enough to ask for it or recognize it, why introduce stuff like that? She absolutely loves berries, kiwi, apples, and almost any fruit we put in front of her, along with many vegetables, salmon, and chicken. Her little chompy face is so adorable, it’s hard to make her stop—although, she has developed an “I’m finished” signal on her own, which consists of taking whatever food she still has piece-by-piece, leaning over the edge of her high chair, and testing whether gravity still works.

She’s still not quite walking, but really close. She crawls everywhere all the time, stands on whatever she can, cruises around with remarkable speed, and tries to climb the stairs whenever I accidentally leave the door open (or sometimes even bangs on the door until we open it so she can access them). She’s definitely all about motion.

It’s amazing that she’s almost a year old, but I wouldn’t say that it felt like it went by faster than it did. She is, as always, an absolute joy to be around.

First Thanksgiving

I have a lot to be thankful for, but right now, I’m focusing on a simple, special experience this morning. Courtney had a challenging night with Julia, who kept waking up at roughly 90-minute intervals because of Prednisone effects. Therefore, I took care of the sleep-averse child for the first couple of hours after I got up, to let Courtney sleep a bit longer.

Julia and I played with her toys for a while, climbed the stairs to my office a few times, discovered the “rattliness coefficient” of the new baby gate at the top of the stairs (calculated as [sound intensity per rattle / distance required to make one rattle], usually expressed for convenience in units of dB/mm), drove the “Julia boxcar” around upstairs at reckless speeds, investigated the contents of my trash can, performed some up-down exercises to maintain our respectively girlish and manly figures, and wrapped up with a hearty breakfast of Cheerios, raisins, and oatmeal, all of which she daintily scooped with her fingers into her waiting mouth. After all, what other way is a baby to eat? Certainly not with a spoon. Spoons are for sissies.

Normally, I don’t get to spend that much time in the morning with her because of work, but since today is Thanksgiving, that restriction doesn’t apply. Without a pressing list of tasks to get done, I was able to enjoy every minute. It’s an appropriate day for this. I am extremely thankful for Julia, for the bundle of happiness that she is, for the moments like this that I get to spend with her—moments that aren’t just experienced with distractions, but with focus and intentionality.

Julia, thank you for being my happiness spigot, and so much more. You’re amazing, and I’m beyond thankful to have you here.

Autumn Adventures

November is my favorite month. The weather here is incredible. I felt the same way when I lived in Orange County–I loved that time of year for weather-related reasons too, even though the weather there is pretty different from the weather here. The heat has died down, the humidity has receded, Daylight Savings ridiculousness is back to normal, breezes are plentiful, the bugs are gone, and the colors are unbelievable. It’s just pleasant.

Julia loves being outside, and she takes advantages of any opportunities we provide for her to escape the confines of our home. With the great weather, I’m actually happy to go out and walk with her more often than usual. It will get cold soon, and earlier darkness will preclude after-work strolls, but it’s great while it lasts. Then, we get to move on to nightly coziness inside with a fire–which is something else Julia has become very fond of.

It’s been a little over two months since the last post about Julia’s LCH. Since that time, many things have happened. To start with, we proceeded on the diagnosis and advice of the local doctors here in Roanoke concerning treatment (those at Johns Hopkins in Maryland didn’t have anything contradictory to say). That meant weekly chemotherapy. Her Broviac central line placement surgery went better than expected, in that the surgeon was able to find a port small enough for her and make it work. A port is preferable to a central line for multiple reasons, so we were thankful for that outcome at the start of her chemo journey.

The chemotherapy itself has been less detrimental than we feared. She doesn’t appear to have any negative reaction whatsoever to the weekly chemo applications, and the only visible side effect is from the Prednisone, which wreaks havoc on her body’s willingness to sleep for extended periods, and increases her appetite. This is tough, but it’s nowhere near as bad as it might have been.

The treatment plan was for six weeks of weekly chemo, then a status check MRI, then six more weeks of chemo depending on the outcome, then another MRI, and then 40 weeks of maintenance at a reduced frequency. The first six-week milestone has come and gone, and Julia made it through the MRI like the experienced trooper she is (outpatient procedure this time, so no hospital stay nightmares). The results were mixed: measurable reductions in all of the skull lesions (yay!), no significant change in the existing spinal lesions, but two new lesions in the spine and one in the sternum (boo!) which were not on the previous MRI. The doctor suspects that those appeared between the previous MRI and the beginning of the chemo, which was a period of about a month. We have only one data point right now, so until the next follow-up MRI a few weeks from now, we can’t know.

Julia, for her part, has done wonderfully. She’s continuing to grow in all of the expected ways. She finally figured out how to scoot on her tummy, then graduated to real crawling on her hands and knees a few weeks later. All of a sudden, she’s mobile! And good grief does she like to move. She’s contained in a play area much of the time, but she needs a close eye on her every other waking moment.

Equally exciting is her developing ability to pull up to a standing position on anything and everything. She can’t stand and balance alone, or walk, but she has become very adept at finding surfaces that give enough grip for her to lift her self with–including cabinet doors and the front of the refrigerator. Unless she’s fascinated with something on the floor, she’s probably either crawling somewhere or standing and looking around, or “cruising” all along her play area fence. It’s only a brief matter of time before she starts walking. Just today, in fact, she was using a dining room chair as a de-facto walker, pushing it around in front of her while she stepped behind it. (It was an accident at first, but then she got all excited and started doing it on purpose after a few seconds.

Her most recent accomplishment has been scaling the stairs up to my office all by herself (with supervision). That’s 15 whole steps! She can manage the whole thing without help most of the time. Only twice has she needed some help to maintain her balance. For being as small as she is, it’s pretty impressive to watch.

She’s also eating plenty of solid food now, especially Cheerios, raisins, peas, and apples. She’ll eat almost any salad ingredient we give her. She insists on feeding herself, but also insists on using her hands for everything, including mashed stuff. Spoons inevitably get pushed away. We’ll have to work on that. At least she’s eating well, though.

In mid-October, we got to take Julia to Colonial Williamsburg for a weekend along with Courtney’s parents. Julia had a bit of a rough time being in an unfamiliar place during the nights, but she thoroughly enjoyed being outside for most of the days, and she loved getting to see some of the animals found there (sheep, horses, and the many dogs that visitors bring). She also had great fun “conducting” the live musical performers at the taverns. She might have accidentally stolen the show on a couple of occasions.

Julia also participated in our recent Halloween festivities as a green-tinted version of Navi, the Kokiri fairy from the Zelda game Ocarina of Time. Courtney went as Link, as seen in the awesome photo at the top of this post. Julia made a great fairy in her adorable costume, though she hasn’t mastered the process of saying “Hey! Listen!” at the most inopportune times. She just…makes baby noises, which I guess is a reasonable substitute at her age.

For now, we’re enjoying her rapid development and working through the weekly unpleasantness of chemo treatments. The next medical milestone will be her second check-up MRI. The next developmental milestone will be either her 11th month, or walking. At this point, we’re not sure which will come first. Either way, life is still exciting for all of us. Only a few more short weeks until her first birthday!

The Bumpy Road to Langerhans Cell Histiocytosis

Julia has just experienced an unexpectedly rough seven weeks. She’s been diagnosed with single-system multi-focal Langerhans Cell Histiocytosis (also known as LCH), present in the spine and skull. Her particular type of LCH is, so far, one of the low-risk presentations which has a very high curability rate and excellent prognosis. LCH is not technically a cancer, depending on who you ask, but it is usually treated with some type of chemotherapy. Julia’s type requires a year-long staged regimen of Vinblastine and Prednisone, which–as chemotherapy goes–is fortunately mild.

The pun in the title of this post had more of a humorous component when I thought of it, before a few of the later events in this story unfolded. I’m keeping it anyway, since it still fits. Here’s the surprising tale of how we got here.

Day 1

On Thursday, July 2nd, we noticed a bump on the back of Julia’s head. It appeared suddenly, without any apparent cause; she hadn’t fallen over backwards or hit her head on anything that we knew of. It also didn’t have any discoloration or bruising, such as what you’d expect from external trauma. We decided to wait a few days to see what would happen to it, and then visit her pediatrician on Monday if it was still around by that time.

Day 5

After the weekend came and went and nothing changed (other than the bump getting ever-so-slightly larger), we called the doctor on Monday morning and took her in a couple of hours later. He and another pediatrician in the office looked at the strange bump and noted that it seemed both smooth and mobile–apparently good signs for a bump like that–but recommended that we get an ultrasound for further analysis. An MRI or CT scan would have provided more helpful images, but those tests would have been overkill based on the information available at the time.

Day 13

On July 14th, we took Julia to the hospital for her ultrasound. When we asked the technician after she acquired the images, she said it looked like it could have some cystic components. The official analysis the following day from the pediatrician was that it definitely looked like a cyst and should probably be removed, since it hadn’t gone away on its own. Given the position of the bump, he recommended consulting a neurosurgeon.

Day 26

On July 27th, we took Julia and the digital ultrasound images to see a neurosurgeon. She confirmed that the bump looked like a cyst that needed to be removed, but requested an MRI to determine the complexity of the surgery necessary to remove it. The occipital bone at the back of the skull is close enough to the brain and spine that any potential nerve or vein involvement could increase the technical difficulty of the removal procedure.

Day 33

Due to the inexplicable rules on medical machine time allotment when scheduling certain types of procedures, the neurosurgeon had to schedule the MRI as an inpatient procedure. Therefore, we headed to the hospital on the evening of August 3rd to settle in for the night, waiting for the procedure itself to be done in the morning. Julia had to be NPO starting at 4am since she had to be completely sedated for the MRI (good luck getting a 7-month-old to hold still for that otherwise), and general anesthetic requires no food or drink to minimize risk of suffocation should something go wrong. Not only could we not feed her, but for ridiculous administrative reasons, we had to stay at the hospital overnight at the same time.

Late the following morning, Julia received the planned sedation, then the MRI. She had no issues with the procedure or test, and recovered well after so many hours without nourishment. She downed about 6 ounces of Pedialyte and nursed for a while, and had some formula. And burped. A lot.

Break for Software Engineering Rant

The kind of workaround they made us do for MRI access is not acceptable. It is inefficiency of the highest form in one of the worst environments possible due to the insane costs of various types of healthcare–regardless of whether my insurance has to pay for it or I do. The neurosurgeon warned us that it might be necessary to do this in order to gain access to the MRI resources she needed in the time frame required, and apologized in advance. The scheduling assistant at the hospital apologized as well. I explicitly told her (as an observation, not that she could do anything about it) that it was truly insane to have to do it this way, and asked if there was any possible way to achieve the same MRI time without physically showing up at the hospital until we truly needed to be there. She said no.

I have built back-end software (from scratch) to manage scheduling of multiple resources among multiple people. I even came up with a computationally efficient way to find availability in a schedule by looking for what isn’t there. This is really not that hard of a problem to solve. Use a priority queue to manage urgent patients and delays. There is no reason why anyone should have to undergo an outpatient procedure as an inpatient because of some administrative rule that says you’re not allowed to access the MRI machine quickly as an outpatient, when they clearly have the resources available for it and for keeping you in the hospital at the same time. It’s mind-boggling.

Separately, there is a communication issue that the administration or doctors or possibly nurses need to fix (though the nurses we had were very good, so I don’t think this is on them). Regardless of how efficient or inefficient your testing and surgery processes are, whether it takes 30 minutes or two hours or two months to get an MRI done, it is paramount to correctly manage expectations concerning what will be happening. We were originally told that Julia’s MRI would be at 8am, and the NPO requirement was based on that. She didn’t have it until about 11am. It’s a big deal to keep a 7-month-old baby from eating or drinking for that many hours in a row, especially when it could have been much shorter if we knew what was actually going to happen.

I understand that urgent and unexpected things come up, especially in a hospital environment. Sometimes the people or machines you planned to use get booked for more time-sensitive cases, and there’s nothing you can do about it. In those instances, it is important to let affected patients know ASAP that their procedure(s) will be delayed due to resource availability issues stemming from unforeseeable circumstances. But that is not what happened to us. There was no advance notice until it was too late to affect Julia’s nourishment, and the notice didn’t come with any explanation for the delay. Why couldn’t they have just scheduled the procedure originally for later in the morning? Maybe there was some reason, but if there was, we never heard it. We sent some pretty specific information stating all of this through the feedback mechanism provided by the in-room TV system.

Carilion: please fix this. Get somebody else to fix it. Get me to fix it; I can write software. Please, just stop doing this to people.

End rant.

Day 36

After a rejuvenating Wednesday at home between hospital visits, we headed back to the hospital at 5am on August 6th for the cyst removal surgery. During the course of the initial discussion of what was to happen that day, we got our first bit of unexpected bad news when the anesthesiologist asked for confirmation of “which ones we were going to be removing during this surgery.”

Which ones? Plural?

The MRI results from two days earlier had come back and shown not just one but three or possibly four lesions in Julia’s skull. None anywhere near as big as the one on the back of her head, fortunately, but still present. Nobody had mentioned that to us before we showed up, so it was a bit of a shock. We only knew about the one we could see. The MRI revealed a small lesion on the upper left part of her mandible, and one in the middle of her sphenoid bone, and one more small anomaly that couldn’t be definitively identified. Due to the unexpected findings, the neurosurgeon also ordered a full skeletal survey (series of X-rays of her whole body) to check for anything wrong elsewhere.

With this news firmly planted in the most worry-prone areas of our minds, we left Julia in the hands of the anesthesiologist and surgeon to work their magic on the one big bump on the back of her head, and returned to the surgery waiting room until they moved us up to a dedicated room in the pediatric ward.

The excellent surgeon performed the cyst removal procedure with perfect results, and Julia handled everything as well as could be expected. She was back with us just a couple of hours later, sans a small bit of hair and with a new gauze patch on the back of her head–much less frightening than we were afraid it might be. The removed mass was sent off to pathology for full analysis, but the initial results suggested that it was indeed exactly what they thought: a benign aneurysmal bone cyst (which sounds awful, but actually isn’t).

Late in the evening, a team of two RNs came to get Julia for the skeletal survey. We were hopeful that we might actually get to go home that day since Julia was nearly back to normal behaviorally speaking, but no such luck. They decided to keep monitoring her, and (per the above rant) keep her as an inpatient to expedite anything more they might need to do based on additional discoveries. This became standard protocol over the next many days, much to our chagrin and consternation.

Day 37

The skeletal survey results that came in on Friday revealed nothing more of interest except for one small abnormality in the T-8 vertebra. The neurosurgeon said that if it weren’t for the unexpected additional lesions in the head, she would have ignored the T-8 anomaly, which was so subtle that it could have been a random shadow on the X-ray. However, she ordered a second MRI to focus on the thoracic vertebrae to get a clearer picture.

The sedation and thoracic spine MRI occurred later that day. That was the 3rd application of general anesthetic to date. (By this point, we’d learned most of the pediatric nurses’ names, and everyone on the floor recognized Julia. We’d also determined that the cafeteria food at Carilion is remarkably good.)

Day 38

On Saturday, August 8th, the results from the second MRI came back and showed some abnormalities that they didn’t see on the X-ray before, but which also did not look like the same bone cyst that they took out of her head. The T-8 vertebra in particular appeared to be somewhat collapsed. The “shadow” from the skeletal survey X-ray was not just a shadow; it had a definite cause.

After this, we spoke to a resident oncologist/hematologist who had been made aware of the findings so far, and she expressed her concern based on the symptoms that Julia might have Langerhans Cell Histiocytosis. She explained some different ways it might present in Julia’s body, and told us that it would probably require chemotherapy if she turned out to be correct. To continue investigating this, she and the neurosurgeon together ordered a bone scan, a full spine MRI, and a biopsy of the T-8 vertebra.

Day 40

After one full “downtime” day in the hospital waiting for the availability of a single anesthesiologist who could meet the planetary-alignment-like requirement of being available for three separate sedated procedures in a row, we got the remaining scheduled tests done on Monday, August 10th. As it happened, the first test (bone scan in Interventional Radiology) did not actually require Julia to be sedated, so they did that one first. The biopsy and MRI happened many hours later, and took about four hours start-to-finish.

The doctor who performed the biopsy elected not to use the T-8 vertebra due to the high-risk nature of the procedure and the fact that the T-12 vertebra provided a much larger and safer target. The T-12 appeared on the MRI with the same “lit-up” properties as T-8, so it would have the same fundamental LCH qualities (if any) as T-8.

We finally returned to our now-familiar room on the pediatric floor, reveling in the fact that Julia would not have to endure any more tests before we could go home–even if that turned out not to happen the same day, which of course it did not.

Day 41

On August 11th, the final results came back from the original pathology of the aneurysmal bone cyst, confirming that it was clean and nothing more than what it appeared to be. The neurosurgeon and oncologist also got the imaging tests done the previous day. The bone scan and MRI showed only a little more of the same things seen from the second MRI, namely that there were a few additional sections of the spine that lit up the same way as the previously identified vertebrae. With a summary discussion with the neurosurgeon concerning the results and what to expect in terms of later analysis and follow-up visits, Julia finally received official permission to go home.

Courtney and I unpacked, showered, and rested (along with Julia) as best as we could, with the help of both of our sets of parents. We also kicked into high-gear “research mode” for anything and everything we could learn about LCH in preparation for the scheduled meeting with the oncologist on Wednesday of the following week, to discuss the results of the T-12 biopsy which would be available by that time.

Day 47

On Monday morning, the oncologist called to let us know that the T-12 biopsy results had come back initially positive for LCH. She recommended additional “stains” to rule out and/or confirm various possibilities, but she wanted to let us know in advance of our appointment, and so that we would be aware of the results and of additional appointments she requested for us in advance. These included an ultrasound of the abdomen to rule out liver, kidney, and spleen involvement, a chest X-ray to rule out lung involvement, and a consultation with a pediatric surgeon who would be responsible for a Broviac line placement for chemotherapy delivery. (Julia’s body is too small for a typical port, and arm-vein-based delivery is not recommended for Vinblastine due to its harsh effect on skin and nearby tissue.)

Day 49

On Wednesday, August 19th–my mom’s birthday, no less–we had the official diagnosis and treatment discussion with Julia’s oncologist. By this time, we already knew the fundamentals of almost everything she told us. The new elements were the specific chemo regimen recommended for treatment, and the official classification as single-system multi-focal LCH (which, given the possibilities, relieved us somewhat because of how much worse it could have been).

Today, we’re pursuing a second opinion from Johns Hopkins up in Baltimore just to get further confirmation and expertise. We anticipate being told the same thing that the local doctor told us, but since JH actually has an LCH-focused group within their oncology department and they work with it more often, we want any additional insights they can provide before starting a chemotherapy regimen.

A Silver Lining

Julia’s LCH diagnosis ultimately came after investigating the bump on the back of her head, which had nothing to do with LCH. It would likely have been another 6-8 months before we would have noticed any symptoms from LCH, during or after she started walking. Despite the fact that she had to endure two ultrasounds, three MRIs, surgery on her head, a skeletal survey, a bone scan, spine biopsy, and endless pokes and prods for lab work and vital checks, I’m grateful that we caught it now rather than a lot later. On top of that, since Julia is still so young, she has no way of knowing yet what lies ahead for her, and so she has no fear or anticipation of any kind. It would be harder for all of us if she did.

Also, Julia has been incredibly resilient through all of this. She is consistently as happy as usual (which is a lot), eating normally, sleeping more or less normally for an 8-month-old, and just generally enjoying life. The surgery on her head is healing well. We are constantly amazed at how well she is doing despite what she has had to go through so far.

The next year will be difficult for all of us, especially Julia, but we have a great support network here with our family and friends. For those of you reading this who have already provided support, prayer, food, cards, and more: thank you. Although the circumstances aren’t what any of us would hope, it brings out amazing acts of kindness.

Father’s Night

It is 1:52am. I just spent the last 25 minutes being awoken by a crying baby, making a bottle for her, changing her diaper, feeding her, rocking her, cuddling her, and finally putting her back to sleep in her crib.

You know what? I couldn’t be happier this Father’s Day, not even at 1:52am, because I get to be among those celebrated today, because my beautiful daughter Julia is here.

I am a father. Interrupted sleep from time to time is nothing compared to that.

On Silly Baby Nicknames

Without any specific intent to do so, I’ve begun using a variety of nicknames for Julia. None of them are the kind of permanent nickname that sticks for years, but I use all of them often enough that they aren’t just one-off silliness. Maybe eight-off or nine-off silliness.

Different people have all different kinds of nicknames for their kids, formed in creative ways from funny circumstances, or sometimes with deep personal meaning. I, on the other hand, have become more of a nickname literalist. Now, these names are not without any sentiment or emotion, but they tend to be imminently practical and speak to something about the situation at hand. A few are timeless things that are true all the time, which I feel compelled to mention via nickname, but most are not.

All of these nicknames follow a basic pattern of adjective-noun, where the adjective describes something Julia is or exhibits, and the noun is invariably one of head, face, pants, or butt. For some reason, these four words just lend themselves to nickname suffixes. You can sometimes cheat on the true “adjective” requirement if you pick a phonetically similar word that gets the point across. It is just a nickname, after all. No need to be too strict.

All you have to do is pick whatever descriptive word applies at the moment, choose a suffix to go with it (though some work better than others depending on the descriptive work), and BAM! Instant nickname. Good candidates for babies include cutie, stinky, fussy, cranky, silly, barfy, fancy, poopy, chompy, smiley, happy, sleepy, giggly…the list goes on.

While some of these pairings are odd at best, some of them are downright hilarious. At least, they are to me, when saying them it a silly voice at Julia’s adorable face–even when the contextually appropriate nickname is cranky-pants. Heck, especially when it’s cranky-pants.

I’ve taken the liberty of putting all of the possible pairings in a table below, because that’s how I roll. Also, as an exercise, I’d like to see if there are any awesome ones I’m missing out on.

head face pants butt
barfy barfy-head barfy-face barfy-pants barfy-butt
chompy chompy-head chompy-face chompy-pants chompy-butt
cranky cranky-head cranky-face cranky-pants cranky-butt
cutie cutie-head cutie-face cutie-pants cutie-butt
fancy fancy-head fancy-face fancy-pants fancy-butt
fussy fussy-head fussy-face fussy-pants fussy-butt
giggly giggly-head giggly-face giggly-pants giggly-butt
happy happy-head happy-face happy-pants happy-butt
poopy poopy-head poopy-face poopy-pants poopy-butt
silly silly-head silly-face silly-pants silly-butt
sleepy sleepy-head sleepy-face sleepy-pants sleepy-butt
smiley smiley-head smiley-face smiley-pants smiley-butt
stinky stinky-head stinky-face stinky-pants stinky-butt

I’ve bolded the ones that I tend to use on any kind of a regular basis. I’m sure you’ll agree that some of the combinations above just don’t work for one reason or another. Statistically, it looks like I get the most mileage out of face and pants. This doesn’t surprise me. They’re hilarious.

I mean…barfy-pants? How can you not laugh?