As some of you may have seen via Lacy's updates on Instagram, we brought Savannah into the hospital on Sunday, July 30th due to some ongoing concerns related to her trache site. We didn't have a day-time nurse on Sunday, so I was up early with her. In general, she requires a lot of attention due to her inability to swallow and risks of aspirating these excess secretions. This means a lot of suctioning of fluid out of her mouth and out of her trachea using an in-line suction catheter. On Sunday morning, she was having some signs of discomfort: high heart rate, facial twitching, and dropping oxygen saturation. These symptoms can happen for several reasons; sometimes it's because she's got a mouthful of saliva and needs suctioning, but other times it can mean she's experiencing some pain or discomfort and possibly having a neurological episode. For some additional context, she also received an upsized trache about 2 weeks ago due to her growth.

Sunday morning, she was having these symptoms of something being not quite right. Her heart rate was up, her oxygen sats were trailing, and she exhibited a rhythmic twitching in her jaw and corner of her mouth that has been associated with "epileptic discharge" in the past (essentially a sub-clinical seizure that doesn't quite meet the definition of a bona fide seizure.) I went to suction her in-line catheter and encountered some resistance that felt like an obstruction in her trachea. I was further alarmed when some blood came up the catheter.

I put her on oxygen that morning to help get her sats up and monitored through the event, which went on much longer than they usually do. Most of the time, these events can last approximately 15-20 minutes, but on Sunday she had two separate events that went on for 1.5-2hrs.  On top of these events, the inability to get the in-line suction catheter to the designated depth was alarming because it meant we couldn't get the catheter as deep as it needed to go to clear her trachea of mucus and ensure she kept a clear airway.  After the second event that lasted about 2 hours, and after discussing these concerns with her ENT, we decided to take her in to Children's Hospital in Minneapolis. The plan was to send a fiber optic camera down her trachea and take a look at where the trache tube is sitting, looking for signs of irritation, granulomas, or possible "shelving" (scar tissue that can develop on the inside of the trachea where the end of the trache tube rests against the trachea wall.)

After a frustrating and unproductive visit to the Children's ER, we decided to transfer Savvy to Gillette, where most of her regular doctors reside. They are more familiar with her case at Gillette and we were hoping we could get the scope of her trachea done quickly. Unfortunately, the ENTs did not see this as an emergent event since she was well-saturated at that point and wasn't exhibiting any of the symptoms she'd shown earlier in the day. They'd be able to get the scope completed first thing in the morning on Monday, and so Savvy had to stay the night at Gillette.  Additionally, due to the neuro activity we decided it would be best to get a new EEG (electroencephalogram, a monitoring test of her brain activity that can detect seizures). One of us needed to stay with her overnight, so I sent Lacy home to get some (hopefully) good sleep in our own bed while I took the convertible sofa in Savvy's room. We were both already exhausted from a 12-hour nursing shift.

They got her hooked up to the EEG at 3:30am Monday morning and monitored her until we went home Tuesday afternoon. The ENT team came in a little after 7am on Monday to perform the scope of her trachea. Everything looked fairly good. The walls of her trachea showed some irritation but nothing of serious concern. The main issue was that her suction depth appeared to be set too deep, and the obstruction we were hitting was likely her carina, the cartilaginous branch where her trachea splits into each bronchus of each lung.  We adjusted the suction depth and hopefully will not encounter any further issues on that front.

The EEG, on the other hand, showed some activity that was more concerning. She has quite an active EEG baseline, with a lot of background noise and occasional neurological electrical activity that is indicative of seizures. Savvy doesn't have any muscle tone to speak of so her seizures do not present in the way you would expect. She does not exhibit muscular rigidity or shaking or eyes rolling back into her head. She doesn't really exhibit any symptoms, as far as we can tell. Occasionally she gets the rhythmic jaw jerking motion, but it doesn't appear to be a one-to-one indicator of a seizure happening. There have been seizures identified on her EEG and when you look at Savannah there's no indication anything is wrong. Underneath the surface, though, it would appear her brain is severely malfunctioning. The way it looks on the EEG and the way the neurologist tried to describe it makes me think of a malfunctioning circuit board, with sparks, pops, fizzes, and smoke.

That likely means a couple things:

1) these seizures have probably been happening for quite some time. There is no way to know how long other than her last EEG, which was in 2020, did not show seizures; only epileptic discharge was identified on that scan.

2) she likely has a condition known as Lennox-Gastaut Syndrome, a kind of epileptic encephalopathy. You can Google it or click this link to learn more, but it's a severe form of epilepsy. There's an illustration you'll find when you Google it comparing a healthy EEG to a person with LGS and Savannah's looks very consistent with LGS. The disorder does not have a cure and can sometimes be unresponsive to treatment. There is currently no known disease-altering therapy that would modify the EEG results to be more nominal.

I asked the neurologist what the risks are of this disorder with a patient like Savannah who doesn't exhibit the clinical symptoms of seizures, and he replied that there's still risk of further brain injury occurring. it's in her best interest for us to attempt to get the seizure activity under control. To that end, we've tried a couple of things thus far that led to an increased duration of her hospital stay (we went to the hospital thinking we'd be back that night; we ended up staying for two nights instead.) She's been on an anti-seizure medication called Keppra for years, so we first tried increasing that to a larger dosage. When that didn't have any effect, we went to the maximum dosage. That still did not make a material impact on her EEG readings. The next step was to try an entirely different medication called Depakote. This is what required her staying a second night in the hospital.

Thankfully, they were able to change her to inpatient status, which meant neither of us were required to stay with her that second night. We were both still exhausted from an insufficient previous night of sleep, so this meant we could go home and sleep in our own bed and be with the older two girls for Monday evening.

We were back in the hospital with her Tuesday morning and spoke with neurology. The Depakote also did not have any effect on her EEG. For a neurotypical patient that isn't responding to medications, they would typically opt to put them in a medically-induced coma to calm down the brain until they can find an effective cocktail of medication, but it's doubtful we'd even go that route with Savannah. The plan is to try another medication called Clobazam, which we started a bit after noon on Tuesday. It takes weeks to get up to adequate levels in her bloodstream to have an effect, so we planned to discharge Tuesday and continue administering that at home, then follow up in a few weeks for another overnight EEG to see if the Clobazam has had any effect.

The EEG team came in to remove the electrodes from Savvy's scalp, which are held on with a rather foul-smelling-but-effective adhesive. After the electrodes were removed, Savvy was treated to a nice warm shower in Gillette's tub room. She got a good scrubbing and a hair cleaning and treatment before being wrapped in warmed towels. We were on our way home a half hour later.

As I may have mentioned, Savvy didn't exhibit any of the symptoms she had on Sunday while she was in the hospital. Once we got home, however, it felt like we'd been sucked right back into Sunday all over again. Savvy struggled to stay oxygenated and had a high heart rate. We had that familiar feeling that something wasn't right. I was able to get some junk out of her lungs with some deep suction, and then she more or less stabilized and was able to rest peacefully.

Our directive with Savannah has always been to give her the best life and care that we can for as long as we can, so long as she is still conscious and sentient, (i.e. "in there.") Lacy and I have no interest in just keeping an empty body alive, but neither of us have had any reason to believe that's where Savannah is at. She still gets mad when you turn off Bluey. She still hates when you suction her nose or wash her face.  She seems to react to her surroundings and gets worried if she thinks she's alone. We have every indication to believe that she's still in there, and so long as that remains the case, we will be there to facilitate her life in every way we can for as long as we're able to. She's already far surpassed doctors' expectations of her since her birth.

All of that being said, given the waveforms on her EEG, it's impossible to try to understand what her reality might look like from her perspective. Given all of the misfiring synapses, is seeing life through her eyes like watching a video with vastly insufficient network bandwidth, buffering every half second and resulting in a garbled and pixelated picture? Does life come to her only in bits and fragments? There is truly no way to know. It feels like all we have is speculation with Savannah unable to verbally tell us anything.

The risks of LGS are significant. It puts her at risk for SUDEP (sudden unexplained death in epilepsy, which is basically what it sounds like; an abrupt, unexplainable death of someone with epilepsy that doesn't seem related to a specific epileptic episode or accident. Just a sudden, unexplainable death with no warning. Think 'SIDS' but for epileptics.)

We're truly wandering around in the dark trying to navigate all of this. We weigh every option we have against our prime directive with her, and though we often question ourselves and our decisions in hindsight, we always remind ourselves that we made the best decision we could with the information we had at the moment. That's all we can do. Though we're, as I said, wandering in the dark, we generally know where this journey is going to end; what we don't know is whether the path ahead of us is shorter than the path behind. We've experienced 4 years of uncertainty, and as someone who needs to know things this has been a very trying and humbling experience.

So here, fumbling around in the dark, we stumbled across a small piece of the knowable. We got another couple acronyms and more drugs with odd names to add to the glossaries in our heads. But even having happened upon that piece of knowledge, the act of knowing it results in even more things we don't have answers for. Something that became apparent to me while studying for my degree in physics is that the more that we learn, the more we come to realize how little we truly know and understand. This has never struck as true for me as it does now.

Thank you for reaching out to ask if you can help us in any way, and to let us know we're in your thoughts!