Refusal to Panic

I always knew Cyrus was a high risk for aspiration. And it’s something that I stressed about constantly. Especially when we have given him oral medicine because to keep any of it from spilling we give it to him on his back. And by the end of administering the meds (a milestone that seems to take longer and longer to reach every day as Cyrus has learned to fight us tooth or nail), he’s almost always coughing with glassy eyes.

In the last six to eight months or so, he’s had more days where he just sounds “watery” where it’s like he’s got a ball of fluid in the back of his throat that just sits there. I call him Dragon Baby when he gets like this because he just sounds like he’s growling! And every so often, he’ll cough this ball of slime up. It’s rather gross. His nurse at school commented on it too – how he struggles sometimes to cough or clear something in his chest and when he is occasionally successful that he will cough up a ball of phlegm.

Anyway, his pediatrician has always been quick to reassure me that his lungs sound clear during practically every visit, and he’s never seemed to have a sudden fever or a bout of pneumonia. But she put in for a swallow study anyway this past February. (And apparently with aspiration, risk of infection is only part of the problem. The other half is that your lungs aren’t equipped to handle the acids and bases of your foods like your stomach is – and exposure to such chemicals can cause your lung tissue to erode). Only Stanford never ever calls us back when she orders stuff. Or they do, but it takes like six months!

Meanwhile, last May when Cyrus’s seizures were spiking once again, his neurologist recommended we look into getting a Vegas Nerve Stimulator (VNS) implant. This is a rather nifty device that can be used to interrupt a seizure in progress by swiping a magnet across the implant and using electromagnetic induction to induce a current into his Vegas Nerve. Apparently, this was an accidental discovery and no one really understands why it works in countering certain types of seizures. It also doesn’t always work well for all types of epilepsy, and we have no idea if it’ll work for Cyrus as we can’t find any record of an ADSL patient trying one out. But, if it does work, it could potentially take the place of one of his sedating medications (or prevent a new one from being added) without any of its own sedating side effects.

But that isn’t to say it doesn’t have any side effects at all – it is a machine that you’re putting inside your body. The two on the list of potential side effects that concerned me were “additional difficulty swallowing” and “sleep apnea”. Cyrus already has difficulty swallowing and we have suspected in the past that he has sleep apnea as well, so we didn’t really want to exacerbate either of these issues.

The neurosurgeon reassured us that the device is now a lot smaller than it used to be, and none of his patients has had issues with sleep apnea from the implant, but agreed that a swallow study should be done as soon as possible. And apparently when a neurosurgeon refers you for anything, Stanford will call you almost the very the next day!

So, we had our swallow study this past Thursday, and can I just geek out for a moment about how cool this imaging technique is?! Basically, they added apple flavored barium to his food – the barium is dense enough so that it can show up on an X-ray. Then while he was eating in a tumbleform chair, they took an X-ray video! And then they can watch where the food goes as it goes down. It was very cool watching Cyrus swallow his foods via X-ray! 

The trials were kept short so as not to expose him to any more x-ray radiation than necessary, but apparently not much video was needed because Cyrus failed this test spectacularly almost immediately! He is apparently a “profound silent aspirator”. This just means that when he swallows a portion of his food is going into his lungs and he’s not coughing or doing anything to clear his lungs (or let us know that there’s stuff in his lungs). In addition to aspiration, some of his food just sits in this throat (in the pharynx), and builds up there, and it takes him a long time to clear it, and the more build up there is here, the more he aspirates as he continues to eat.

Right after the trial concluded, and before any of this had been explained, Stanford’s OT, Cyrus’s regular OT, the technician, and the radiologist all disappeared into the hallway to confer with one another. Once I realized this had happened, I cautiously approached and asked if I was allowed to be a part of this conversation.

And this OT – she was the sweetest! Apparently, she was asking our regular OT for advice on how to deliver bad news. She really seemed to feel like she was handing down a death sentence. And it was hard to reassure her that, “No, it’s okay. We were expecting this to eventually be the case. Give it to us straight. We received an actual death sentence a few years back and have survived so far!”

She explained Cyrus should never get more than a tablespoon of anything by mouth. That he needed a g-tube (requires surgery), and should get an NG tube (a tube down the nose and into the stomach) as quickly as possible in the interim – she would have preferred yesterday. Even our own OT seemed just so very sad as we walked back to our car, offering an ear if we wanted to talk about it.

And I appreciate them both so very much for their compassion and their sensitivity.

But I can’t feel upset myself. If anything, I feel vindicated, because I’ve been pushing for this for months!

Our OT, literally called us that night to check in and give advice for how to feed Cyrus more carefully until we can get a tube placed. She was very concerned about him continuing to take food and meds orally – she suggested that we reach out to our neurologist and see if there was another method to deliver the meds until a tube could be placed. She called again Friday morning explaining that she’s trying to get a referral to a GI (Gastro Intestinal) Specialist as soon as possible, as well as a referral to Complex Care (a program where kids who are medically complicated with lots of issues and specialists have a doctor or a team that are responsible for coordinating all the different doctors’ treatment regimens). Then she contacted me a third time, when one of her contacts was out sick to tell me she was going to reach out to someone else instead. I just so appreciate that she is making all these calls! Goodness knows, I hate making them myself!

Our pediatrician is also trying to get us a referral to GI, and she suggested if she couldn’t get soon enough, we could just go to the ER to get an NG tube placed there.

And I am baffled by these reactions. Give his meds another method? Like what? IV? Injection? Rectally? And the ER?! Really?

I just don’t feel the urgency. I don’t understand the panic. In fact, I refuse to panic!

I know that aspiration is dangerous. I have read so much about it over the last few years, and have stressed about it constantly as mentioned previously. But here’s the thing. If Cyrus aspirated so profoundly during his first ever five-minute trial, he’s been aspirating profoundly for months and months! Probably longer!

And I have already learned that one missed dose of medication results in terrible status seizures. And he can’t not eat – that’s how he stays hydrated!

So yes, aspiration can slowly and surely kill him. (Why do you think I’ve been so worried about it?!) But seizures and dehydration can do it faster!

A week or two of eating and taking meds the way he has been, seems insignificant in the grand scheme that is caring for Cyrus. It’s not like we’re not going to get a tube in as soon as we can. And it’s not like we’re not going to do the surgery to install a g-tube! We have a long-term plan in place!

And if we can’t get a GI appointment in the next week or so, then sure, we can go to the ER as a last resort. This weekend we already have plans! We’re doing another medical test – a sleep study! We got this appointment by sheer luck off a waiting list with the hopes that it could happen before any surgeries! So, I wasn’t going to turn it down!

But these medical professionals and OTs are all so panicky! Do I have my priorities all mixed up here? Am I underestimating the danger?

And don’t worry. I haven’t been sitting on these questions. I actually asked our pediatrician straight out if this was the case on Friday. She never got back to me.

I figure that’s answer enough.

Sleep study, here we come!

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General Update:

Cyrus closed out his first mini school year on July 13^th after starting in April. I think he’s missed it! Although, it might also be that his mom keeps dragging him to doctor’s appointments and weird medical tests during his month off. And of course, PT and OT continue even during vacations! But he doesn’t see his teacher and nurse on a daily basis, anymore and I know he would greet them both with smiles (usually after hissing at me for putting him in his chair).

We try to do fun things too though – like trying out swimming and going on playdates with other small children.

He was fitted for a new wheelchair a few weeks back, and will be receiving a new Zippy Iris in the next 4-6 months! And we finally made a break through with our insurance company in terms of trying to get our wheelchair van, and have been promised that we will hear from them on or before August 2^nd with an absolute determination of whether they will fund a van modification or not. Either way, we win!

The seizures that peaked in April and May are back down to manageable levels with the addition of a fourth anti-seizure med. If our honeymoon with this med doesn’t turn into a long lasting healthy relationship we will probably move forward with the VNS. We might do that anyway if we can get surgeons and anesthesiologist schedules lined up enough to do both surgeries at once!

I don’t know if the sleep study went well. I have no idea how anyone can sleep well with all those wires and bumps, and things on your face! They also did not initially want me to give Cyrus his normal melatonin supplement “because it affects the way you fall asleep”. No duh! That’s kinda the point. And well, he just didn’t get tired – in fact was super active (and angry) and tearing out sensors… Eventually, the technician said I could give it to him if I wanted. And he fell asleep so fast! But then he was woken up at midnight and 4:30 to readjust more sensors, and then again at 6:15 AM because the study was over and they needed to take all the wires off. Though he fell asleep rather quickly in all instances (even during the removal of wires), I’m just not sure how representative the study was of his usual sleep behavior. Basically, he was so exhausted from the sleep study he came home, ate breakfast, and then took a five-hour nap. (The only time he normally takes naps that long is after some major status seizure).

The bath to get all the sticky stuff off his skin and conductive paste out of his hair was also quite the adventure! I don’t think Cyrus appreciated it very much. Such is the way of medical tests. Hopefully, though they got some good data if only in shorter bursts and it will give us good information in the future and will result in higher quality living on average days!

Mommy and Cyrus napping off the sleep study together!