Julia's journey up until now...
Updated: February 2020
October 2018 we noticed Julia (an otherwise completely normal, happy, healthy, one year old) had an increasingly stiff neck. We brought her to the ER the day before Halloween 2018. She hasn't left since. It's been 460+ days and counting. About a week later, the day after my dad died to cancer, Julia was diagnosed with cancer: clival chordoma, an extremely rare, aggressive, difficult-to-treat cancer, with a poor prognosis. They gave her 30% survival with treatment, 0% without.
The tumor was wrapped around her C1 vertebra, and because it was bleeding, immediate surgery was required. Removal surgery was performed through the back of her throat. It required two fat grafts to pack the tumor area (those are the scars on her belly and thigh). Because they removed so much bone, her spine/skull was destabilized, and she was kept sedated until a follow-up spinal fusion surgery could be performed. They fused her C4 up through her skull (that's the big scar on the back-middle of her neck/head), and this required a halo neck brace which she kept on for 7 weeks. The halo was bolted to her head with 8 titanium rods (those are the round scars: 2 above each eye, 2 behind each ear). They also installed her chemo port (which has since been removed) - that's the scar on the right side of her chest.
While performing surgery, they discovered the tumor had already punctured through into her spinal column, and it was near her brain stem. This area was inoperable. Chemo was the plan, but during recovery, she suffered several complications. Pneumonia, long-term sedation, lack of nutrition (lots of fluid-only days), and surgical recovery, had caused her to become so weak that she could not support herself off of the breathing tube. In addition, she was experiencing a fluid/mucus build-up in her lungs. Due to her extremely-limited movement (from the halo), and overall weakness, she was unable to cough. Due to the surgery location (at the back of the throat), they were unable to properly suction. All of this kept her in an unfortunate cycle: the longer she was sedated with the breathing tube, the weaker she got; the weaker she got, the longer chemo was delayed; the longer chemo was delayed, the more the remaining cancer grew. To break this cycle, a tracheotomy was performed (this is why she doesn't talk). At the time same, they also gave her the g-tube in her belly. After all this, we thought we were finally about to begin treatment... but then she started spiking fevers.
All labs continued to be negative for infection. Around Christmas 2018, her eyes stopped responding to neurological checks. Immediately, they determined she was experiencing increased pressure in her brain. Right at the bedside, they drilled through the top of her skull to relieve the pressure (this is the scar on the top-center of her head; recently re-opened). They put in a temporary external shunt, and tested the fluid to discover an infection had reached her brain, likely through the surgical site in the back of her throat. This increased pressure caused a stroke (this is why she has left-side weakness, most noticeably in her left arm and face). Due to the cancer's location, and the likelihood of blockage/future pressure issues, they installed a permanent shunt (this is the scar and bulge on the left-side of her head; recently re-opened).
NOW, in early January 2019, we thought we were finally about to begin treatment... but then her right eye started to close, and over a few days, she quickly lost ability on the right side of her body. This was concerning as the stroke only affected her left side. An MRI determined the cancer had "exploded" in growth, fully consuming the area inside of her spinal column, and compressing her brain stem to nearly nothing. We were told nothing more could be done. Any swelling, like that from surgery, radiation, or chemo - would kill her. We were told she had days to live, and she wouldn't survive the week. She was put on hospice and comfort was the only focus.
During this time, her vitals became real bad real fast, and she fell into coma. She could not move any part of her body; her eyes, down to her pupils, were both motionless. We planned her funeral. But since we couldn't "do nothing", we started treating her ourselves with alternative therapies. I had done the research on options just three months prior for my dad. To everyone's amazement... she didn't die. Her vitals stabilized. They didn't improve, but they stopped getting worse. Three weeks went by and we requested another MRI. This image showed that while the tumor continued to grow, the growth rate had slowed to a crawl. Given this seeming period of stability, we pushed for chemo. We were told that if "by some miracle" the cancer was reversed, which "doesn't happen" at this stage, Julia would be brain dead (they said it more nicely than that). They said the damage to her brain stem had been severe and "developmentally devastating", and that she would never recover her abilities and would have no quality of life. But Reed and I insisted, even though I was personally terrified that we would succeed "just enough" to keep Julia in this permanent state of coma forever.
Over the months of February and March, Julia responded amazingly well to this combination of chemo + alternatives. She fully came out of coma. By April, she was declared "neurologically perfect". She retained all her memories and mannerisms, and while physically limited, was clearly the same Julia as before. On MRI, the tumor continued to visibly shrink during this time, fully releasing the pressure on her brain stem. The tumor reached a point in April where it no longer shrank - it didn't move at all - the hope was that it was dead.
At this time, she was transferred out of the PICU (Pediatric Intensive Care Unit) and into the PCU (Progressive Care Unit). Over the spring and summer (2019), Julia continued to push through and work on her strength. She was physically recovering from a LOT! Spinal fusion, stroke, coma, brain stem cancer, (not to mention exhausting chemo), and over half a year of bed rest. She slowly started to regain muscle in her core and even in her stroke-damaged left side. She also starting making huge improvements with breathing. She was doing very well with the HME (being detached from the vent), and quickly worked up to 12+ hours a day "on her own". This was amazing progress toward one day removing the trach.
During this time, she smiled and laughed again, played with toys, loved on her baby brother, and became obsessed with Peppa Pig and her iPad. By fall 2019, she had regained enough strength to FINALLY sit up in bed on her own! Once she had the muscle and stamina to do that, we couldn't convince this girl to lay down. She had been laying bed for nearly a full year, and she was OVER it. She started going on stroller rides outside of her room (and felt fresh air for the first time in nearly a year). She was making progress in communicating using her picture board. They even did one session with a "stander" which is a first step in relearning to walk. She was doing such a great job!
Twelve rounds of intense chemo ended in September (due to a body's limitations they can't just go indefinitely). Her MRI's continued to show stability for 6+ months, so the hope was the cancer was gone, and it was just a dead mass left on the images. The focus shifted to proving Julia was a good candidate for acute rehab, to help regain some more lost abilities, and then finally bring her home. She made major improvements once chemo was out of her system... For a few weeks.
But then, near the end of October, several weeks after chemo ended, we started seeing concerning signs. Her right eyelid started to droop again. She sat up for shorter periods of time. She tolerated the HME (breathing without the vent) less. She didn't move her stroke-damaged arm as much. She slept more. An MRI was performed just before Halloween. At the very meeting we were supposed to discuss discharge plans, we were told the cancer "exploded" in growth. It was everywhere. All around her brain stem, down her spine, and in her membranes. We were told there are no more options. At best, we could do palliative chemo in hopes of slightly extending her life while keeping her comfortable. We chose that route, knowing of at least one case study where a child responded "remarkably well" to this combo. But we also did a crash course of additional alternative options, and have pushed for a complementary approach.
Since the cancer started growing again, Julia's condition has deteriorated quickly. Over November and December, she lost all of the ability she had recently regained. Her right pupil became out of sync with her left, and the eyelid closed for good. Two weeks later she starting acting really bizarre, and they determined she was experiencing an unrelated shunt malfunction. She was transferred back into the PICU. They fixed all that (that's why they re-opened both shunt scars on top of her head). After that, her increased weakness led them to discover the large pocket of fluid collecting near her right lung, which they installed a drain to help clear out. The fluid was found to be cancerous. She lost the ability to sit up on her own, and roll over, and she stopped using her left side completely.
Over the month of January 2020, she became completely paralyzed from her eyeball down. She didn't so much as twitch a finger or toe. She barely moved her mouth. She stopped fighting during care (which she hates), even her vitals stopped reacting to being touched. An MRI determined the palliative chemo had no effect, so it was stopped. We upped her comfort measures. She's on a ton of painkillers to handle any sort of discomfort she might be experiencing. We opted for a third chemo. It's very gentle, and while not expected to work, there is (again) a single case study where a child with Julia's cancer responded.
Status of February 2020. Her health continues to decline. It is a daily struggle to regulate her vitals and fluid status (she swells randomly, particularly in the abdomen). Her vessels are leaking. She is unable to receive feeds because they all come back up, so she's currently on IV nutrition. Due to a septic shock episode in January, where her kidneys failed and she was at immediate risk of death, she now has three drains managing her swelling (one in each pleural space around the lung, and one in her abdomen). We continue to focus on comfort. Our current goal is to keep her alive and stable long enough to give this last-chance chemo the opportunity to do something. The next MRI should be in March, and while we don't expect it, we pray to see improvement.
Julia has shown the world that she can fight back from coma and 'un-shirnkable' cancer once before... so we're hoping we can find the right combo to allow her to do it again.
You can follower her daily updates here on her website, or on my Facebook page:
Last update: February 2020