Never Ending Bureaucratic Mazes

As you probably know if you follow this blog, we’ve been working on trying to get a minivan that can accommodate Cyrus's wheelchair for over a year. The difficulty here is that these vans are expensive. A new minivan is around 30-40k, and then the conversion is apparently an additional 24-32k on top of that. In my research, I also discovered that you could buy a used van for about 35k (so like the cost of a regular new vehicle). But then I learned from a special needs parent friend that the Regional Center will often help pay for these vans, but only if you get one that is practically brand new. They only want to invest this amount of money if it's going to last. So they pay for the conversion, and we pay for the van. This means for the same 35k we can get a new van instead of a used one. Of course, you have to be able to afford the new van, so this system is unlikely to help a special needs child that is in a family with less financial resources.... (but I will spare you my social justice rant for the time being).

But the Regional Center often refers to themselves as the payer of last resort. They will help you, but only if everyone else says no first. So we were assigned the task of obtaining rejections from our insurance company, three charities, and California Children's Services (CCS).

CCS was easy! They have a standard rejection letter on file that they will just hand to you upon verbal request!

The hardest part about getting rejections from charities was in finding organizations that might consider a case like ours. For the most part, this involved writing a grant proposal or filling out an application that explained what the funding would be used for and why that equipment or those services were needed. Once submitted it was simply a waiting game. I submitted three applications within the space of a month, and two of them responded within about three months. Both of them rejected us, likely on account of our income, which is apparently too high for such charitable causes.  But no matter, I only needed rejections anyway. (Maybe these charities will help cover the families that don't have the financial resources to use the Regional Center's program. At least I hope so!)

But the third charity never responded, after months and months. I sent in an inquiry about our status, and never got a response for that. I wasn't sure that the organization was even still active, and there was no phone number to call.

So, I found a fourth charity! One, that had updated information less than a month old! And spent a day writing another grant proposal. We never heard from them either. I wasn't too stressed about it though, because I was busy fighting with our insurance company!

I lost count long ago of how many times we tried to called them. I honestly have learned in dealing with insurance companies and specialty pharmacies that it sometimes helps to just call a second time so you are talking to a different person who will give you a different story. Over the first three calls, we made it deeper within their bureaucratic phone maze each time. But since we have learned how to navigate these departments, we’ve had the remarkably the same frustrating conversation over and over again. It goes something like this.

After asking to make a request to receive prior authorization in the funding of a modification to a van, we are transferred over to the Authorization Department. They ask for a prescription, which we give them. Then they ask for the ICD 10 code. This is a code that is supposed to identify the specific equipment that is being requested. When we don’t have one, they insist they are unable to process the request at all. My pediatrician didn’t know what it is and suggested we talk to the manufacturer. The sales people at Mobility Works insist that the code doesn’t actually exist, and that you just tell this to your insurance company, they will usually issue an automatic denial. Ours won’t do this.

We then requested aid from our social worker at the regional center in finding a code. She gave us a code, but it wasn’t an ICD 10 code. It was a diagnosis code that justified why he needed the van modification, but did not identify the equipment. The request could not be processed. But I was told directly, that if it doesn’t have a code, it’s definitely not covered under the plan. One phone rep suggested we talk to the Benefits Department and ask for a “Benefits Denial Letter”, which according to them was just a blanket – this is not covered under this plan – type of letter. And that it wouldn’t have to go through the formal authorization process.

Upon talking to the Benefits Department, they always tell a different story. Basically, that they can’t issue a Benefits Denial letter unless the request has actually been denied. When I told them the Authorization Department wouldn't even accept the request, they usually shrugged, apologized, and told me they can’t help me. But on one occasion, the phone rep suggested that I write an Appeal. They said, that if I submitted an Appeal the Insurance company would HAVE to rule one way or the other within 30 days of the request.

So, we did that. It was very similar to the grant proposals sent to the charities. And then received a letter back from the insurance company, that we can’t actually appeal a decision that has never been made. That they can only process an appeal on a claim that was previously denied. 

I turned to other Special Needs parent friends that have already been through this process, and learned that they had no such troubles getting their insurance company (Kaiser in both cases) to give them written denials.

After Cyrus had surgery this past July, and Anthem Blue Cross was courteous enough to assign us a Nurse Case Manager who would call us once a week to check on Cyrus through questions to see if she thought we should go to the doctor’s due to surgery complications. She also offered to answer any questions related to our insurance coverage. I asked her for help on this. She was horrified by my experience and promised to try to have a resolution within a week.

She and a social worker that also works for Anthem Blue Cross worked internally. They told me they tried to contact our social worker at the regional center (I think only to receive a code). They tried talking to the Benefits and Authorization departments both, to see if they could get them to write a letter, or if they knew some tricks for how I should be phrasing my requests to make better progress.

After three weeks, they both gave up and said they couldn’t help me. That my best bet was to call them myself and ask to speak to respective managers. My husband tried that, but they didn’t allow him to speak to the managers.

Since then, I have called again and had no success. I have also requested their policy of being unable to process a request without a code, in writing. They said they couldn’t do this because this policy was on internal documents that outlined job descriptions. I requested that they put it in writing that they were unable to process the request. They said they couldn’t do that either.

I was feeling rather frustrated for having poured months into this, to only have gotten absolutely no where. It is abundantly clear to me that my insurance company will not cover the van conversion. But they categorically refused under any and all conditions to put that in writing.

At this point, I was panicked. When we first started this process, getting a wheelchair van was in the "would be nice" category of priorities. But it has rapidly become a necessity as Cyrus has outgrown his adaptive stroller, and was fitted for a new one this past summer, and we're expecting his new Zippy Iris within few months. This new wheelchair will not fold or collapse the wave the adaptive stroller will. Once we have to use this (we're already cramming him into the adaptive stroller as it is), we will no longer be able to transport him anywhere. Not to doctor's appointments or anything. He will still be able to go to school because the school buses can handle his wheelchair, but we wouldn't be able to take him anywhere. He would be trapped at home. 

The panic was further compounded by the fact, that we're expecting another child. And once she is born, we won't be able to fit both children (the adaptive wheelchair and a stroller) in our current vehicles. So, we will have to buy a van anyway. And it seems absolutely ludicrous to buy a van without the conversion that can't accommodate Cyrus's wheelchair when we KNOW we will need that in the very near future. (Not to mention, that getting a van converted after the fact usually costs an additional $6000 for shipping costs to get the van to and from the factory). 

So, I reached out to our social worker at the regional center and explained all of this in minute and excruciating detail with all the documentation of what I had attempted so far. And like the nurse case manager, she was horrified by our experience. And she reached out to her supervisor to get special permission to skirt around the policy of needing a rejection from insurance. But she told us that we needed to get one more actual rejection from a Charity, and recommended Center for Independent Living. She said they would either have funding or not within their charity cycle and would tell me right away. She was correct! It only took a week! 

Our next step was to be assessed by an Occupational Therapist to establish that Cyrus does in fact need this. This was scheduled within two days of everything else being submitted. I was nervous that this one assessment would make all of this work pointless, but she wrote a beautifully detailed report that documented exactly why every other option was problematic or outright useless for Cyrus, and our application went through the Regional Center less than a week later. We were then told it takes about a month or three before the funding went through. 

But we went van shopping anyway. Picked out a slightly used Toyota Sienna with a power folding ramp, put a holding deposit on it, and hoped that we'd maybe have it before Christmas! Only to be contacted by the Dealership a week later, telling us he had received approval from the Regional Center for the conversion funding! He told us he had only ever seen one other application go through faster than this one once everything was submitted, and that we must have been put on the priority fast track list. Our request for our own financing for our portion has also already been submitted, and is expected to be completed in the next two days. We might have this van in time for our Thanksgiving trip (Like literally, we might have it the day before Thanksgiving), but we were guaranteed that everything would be completed by the end of the month.  

So apparently, the secret to success with these bureaucratic mazes is being neverendingly persistent. And if that doesn't work, lots of crying to social workers might open a few more doors. 

General Update: 
We made it back home from hotel living October 20th! And we are in the process of unpacking the second bedroom that was filled up with boxes of all our kitchen, dining room, and half of our living room stuff. With the eventual goal of setting up Cyrus's new big boy bed in his own room!

The seizure count might be slightly down. He was having up to five seizures a day for the last few weeks, but this week he seems to have stopped having them at school, but will still have them afternoon or evening. And the ones we are seeing are more severe than they've been with the tendency of wiping him out for an hour or so afterwards. This is probably due to the introduction a new anti-seizure med - Ficompa. This drug is apparently powerful, and only given once a day in the evening. But this might be why we still have some in the evening before his meds have been given, as the protection from this drug may be waning in these afternoon and early evening hours. Hopefully, as we move to the full dose over the next two weeks, these will drop down as well. 

Due to the chronic congestion, Cyrus's Pulmonologist referred him to an ENT (Ear Nose Throat Doctor). At this appointment we learned that Cyrus has freakishly large adenoids, and larger than average tonsils. This apparently makes it difficult for him to clear out any mucus in his sinuses once it is there. They told us we could surgically decrease the size of his adenoids and remove his tonsils, but they didn't push this option, and they said, he is likely to grow out of this "sinus crowding" within the next few years. So we didn't push for surgery. We don't want to do surgery again if we don't have to. In the meantime, we have added a bulky suction machine to our at home medical equipment collection. And after a little less than a week of using it a few times a day (Cyrus hates it!) his congestion has cleared up completely!

Our Complex Care Clinic recommended Cyrus for a diaper service that would pay for all his diapers, since he is likely to continue to need them for his whole life. The diaper service called and said they needed rejections from our insurance! (Nooooo!) I didn't want to touch that, so Kyle was nice enough to make the request yesterday. Anthem Blue Cross didn't even blink! They said, "Yeah, sure. Where do you want us to send that?!" They didn't even ask for a code! It took only one phone call! I'm going to be happy about that! And I'm not going to scream in irritation... 

Cyrus's granulation issues at his stoma site have finally seemed to settle. His skin looks a little red and irritated in the area, but the tissue has stopped trying to grow around his button, and leakage from the button has dropped down to almost nothing.

Cyrus's newest peptide formula did not go well. Once we moved over to the new formula exclusively, the vomiting did disappear, and there was only a little spitting up on occasion, but he had constant chronic diarrhea for over a week. His school sent him home for this too until we got a letter from the GI clinic, saying, "Hey, while we're messing with the diet, some diarrhea is expected and not a sign of infection or illness. He's allowed to be at school". 

But then, when the diarrhea lasted for a week on a formula that "almost no one has issue with", GI is all worried he might actually have an infection now. The pediatrician on the other hand, just thinks the pre-digested formula is running through him too fast. And she recommended switching formulas again - to something in-between high fiber and pre-digestion. She recommended Nestle's Compleat organic real food bend. So I ordered six days worth of that so we could try it out before I ask the GI nurse to change our formula prescription. I don't want another month's supply of something that isn't going to work. We're on day two of Compleat! And so far, we've already seen some significant improvement!! His stool is still a bit loose, but no longer pure liquid. But we're also going to collect some stool samples to insure no infection as well. 

Cyrus's school has been closed yesterday and today anyway due to air quality concerns. Apparently, here in California we have "smoke days" instead of "snow days". But it's hard to argue with staying home when the Air Quality Index is rated at 220 due to smoke from an awful fire that is 200 miles away! Cyrus's diapers have been rather smelly as of late, and we can't even air out the apartment. Time for a diaper pail and an air purifier or three methinks. 

Hope all of you are well! And that you have a roof over your head that is not threatened by fire, food to eat that doesn't give you diarrhea, and air to breathe that won't cause lung cancer or COPD.

Emotional Needs

They tell new parents to narrate what you're doing whenever interacting with your new baby. Even though initially, they don't understand your words, eventually they will. And this constant narration serves a couple of purposes. It helps develop vocabulary, it helps them learn what is going to happen, and this helps them learn to trust you.

Over time, as it became apparent that Cyrus is likely to remain non verbal, I kind've let this habit slide. It's just hard when you don't get any response or social engagement. And part of me thinks, I should still keep up the practice because he probably does understand more than he is able to communicate that he does, and those benefits are still the same even if they are slower to develop. But interestingly enough, whenever we had company or were just in front of others I found myself narrating constantly! Every diaper change, what we're eating, that it's time for medicine, the expressions I see on his face, etc.

But why?!

Is it just like I'm trying to look like a "good" mom in front of others? Do I feel some compulsion to impress? I mean... maybe??? I definitely wouldn't discount it completely, and yet that explanation doesn't completely resonate.

During a hospital visit, after we had decided a treatment plan, the pediatrician turned to Cyrus and explained what they were going to do to him.

I told the doctor that he didn't really need to do this. That Cyrus didn't understand.

He gave me a soft sincere smile. "That's okay. I'm going to explain it to him anyway."

I felt immensely pleased at this response because this doctor saw Cyrus, recognized him as a person with thoughts and feelings, who deserved his respect.

I realized at some later point that same week, that when I was narrating in front of others it was a way to include Cyrus in the social interaction. I was doing it because I wanted our guests to see him! It was like I wasn't doing it to explain to Cyrus what was happening, but rather a way to give him a voice - to share his experiences with others.

And it works! Others will turn to him, ask follow up questions - engage with him more than when I don't do this.

But then, why don't I do it the rest of the time? When it's just the two of us? I still want him to be seen and included.

It's almost like I'm so used to serving as Cyrus's voice, that I forget he has one of his own.

That just because he cannot articulate his thoughts and feelings, doesn't mean he doesn't have them.

I was reflecting on our bedtime routine at one point. We go through the process, I lay him down in the crib, tuck the blanket around him, and walk away. On one particular evening, he immediately objected as I started to leave with one of his five distinct sounds that I think I can interpret.

I came back, put my hand on his chest, and told him it was time to sleep and time to dream. I stayed for just a moment, and he instantly calmed.

Huh!

It's almost like all he wanted was for me to say goodnight.

It was a good reminder. Cyrus may not always be able to articulate his wants and needs. But he is still human. A human with social and emotional needs. I'm trying to get better at learning how to meet them.

What happens when you brush a curly haired child's hair.

General Update: 

The last three weeks or so have been rough for Cyrus as previously mentioned. He had a cold a while back, and since that time his sinuses have not really cleared up completely. He seems to be struggling with intermittent congestion and it sounds like so much of the nasal discharge just gets caught in his throat. Kyle and I have both been experiencing a little mucus as well, and I can't help but wonder if there's something here in this hotel that we are all reacting to.

He had three doctor's visits this week with various specialists. First, a pulmonologist who confirmed that his lungs are clear (yay!), just that his sinuses have remained clogged. He saw no sign of infection and recommended we get a suction machine to help him clear things out when he is not able to do it himself. He also concluded that Cyrus's sleep apnea has diminished so much according to his last sleep study, that he no longer needs oxygen at night!

Then we met with GI to discuss volume intolerance. They suggested that Cyrus needs more water more often, and that perhaps his current formula has too much fiber in it, which is slowing it down through his digestive track, and when we try to feed him more he gets backed up. The water should help, and we are also considering changing to a different formula. Of course, the sample the dietician sent us home with does not actually appear to be lactose free, so I've been trying to reach them about this, but haven't heard back yet. I'm happy to say though, that just with the additional water we have been able to titrate his daily food intake from 700 calories a day to about 825 without any single vomiting, or even spitting up, incidents over the last three days. Our goal is to get him in the 900-1000 calorie range. But we're going slowly (don't want to get kicked out of school again for vomiting).

And finally, we met with the surgery team to check out how well Cyrus is healing from his surgery this past July. We were also supposed to learn how to change his g-tube button, but since we accidentally ripped it out a week ago, we apparently are now considered experts that already know how to do this. They cauterized his granulated tissue for a fourth time, and with the help of some steroid cream we seem to be keeping the granulation mostly under control. They gave us some extra g-tube buttons and syringes since we don't have access to our other extra supplies that we left at home  thinking we'd only be in this hotel for a week or two. Ha! We're now starting out week six! Hopefully, we'll get to go home on October 20th.

Meanwhile, we going to get out of this space and spend the weekend with friends who like, live in houses. Maybe make some gluten free cookies that this pregnant lady has been craving for weeks!

We probably should find time for a hair cut at some point...

Up For Promotion

We are super excited to announce that Cyrus is up for a promotion this coming spring as he will transform from an only child to an older brother.

After learning we were expecting, we have had the opportunity to explore yet a new side of the medical field - this time genetic testing of an embryo! I participated in a CVS (chorionic villus sampling) procedure. This involves collecting a sample of the placenta after it is formed. The placenta shares the same DNA as the baby, so this sample can then be used to test for specific disorders or chromosomal abnormalities without as much risk to the embryo. And they use an Ultrasound to pilot their instruments, so you get to watch it all as it's being done! (I'm clearly a sucker for cool imaging techniques if my last two posts haven't already made that obvious enough!)

And a cool bonus of participating in genetic testing was we got to find out the gender a whole month earlier than most expecting mothers. Cyrus is apparently going to have a little sister! Her name will be Eliana Kylie. Like, Cyrus, Eliana refers to the sunlight and we hope she will be another source of light in our lives. She is expected to join us in March. And she apparently won the genetic game of roulette in that she is not even a carrier of his disorder even though both mom and dad are.

We are incredibly excited to be growing our family, but also a tad nervous. Cyrus has stretched us and challenged us in a multitude of ways - introducing us to a world that we didn't know existed before he came into our lives. A world that involves developing various nursing and physical therapist skills, learning lift and carry techniques of fire fighters, navigating bureaucratic mazes, and writing grant proposals every other month. He introduced us to a world of wheelchair accessibility and the amazingness of medical technology.

But he has also spoiled us in that, in between he's pretty happy and calm. He is incredibly chill, and he doesn't throw emotionally violent tantrums. Nor does he really get into anything unless we leave things on the floor where he can roll over to it and accidentally grab it or get tangled.

And now, we may end up with quite a willful and mobile child. We are very aware that for awhile we will essentially be caring for two cognitive babies at once, one of which will be way too heavy! We may need to actually learn how to child proof our apartment (if we ever get to move back into said apartment!) and learn to manage an infant or toddler's emotions! And that van, that I've been talking about for over a year that would make are lives so much easier, now feels much more like a necessity! (Not that this fact makes our insurance company any more reasonable...)

Oh, and if I want this girl to inherit Cyrus's crib, I have to figure out where he can safely sleep. There are these beautiful safe beds that are designed exactly for his needs. You can even supposedly get your medical insurance to pay for them... if you get a prescription, write a letter of medical necessity, and get recommendations from a half a dozen specialists to establish medical need of every feature of said bed... (please excuse me while I run screaming in the other direction!)

But our long term stay in this hotel has at least shown us that Cyrus can be moved into his own room and sleep okay with some precautions, though there may be a rocky transition involving sleep strikes. Perhaps we'll try to do this over a school holiday.

Cyrus's attitude towards other children has always seemed to make him more engaged and aware of the world around him. We've seen this with his cousins, play dates, and with his peers at school And I am so excited for this to be a more regular thing!

And if I'm totally honest, even though I know better than anyone that there are no guarantees, I'm also excited for the chance to raise a child that is neuro-typical and perhaps not medically complicated. I'm excited to have that totally different experience. I'm also thrilled at how I know Cyrus will touch her life, grant her the opportunity to learn a patience and compassion that I have witnessed in some of my students when they have grown up in similar environments and situations.

And of course, I don't want to put any burdens or pressures on her (especially before she is even born!), but I am also excited for there to be another person in this world who cares about him.

I'm just excited that our family is growing! And that Cyrus's medical complexities were not the death of my dream to have more children.

General Update:
To clarify our living situation, our apartment complex had to "temporarily evict" us from the premises due to an emergency plumbing project underneath the building. They are paying for us to stay in an extended living two bedroom suite. The room is nice, wheelchair accessible, and they allow for pets! We have a little kitchenette. Of course, we miss our own space, and every day we discover a thing or three that we should have brought with us.

The cats took only a few days to adjust. Honestly, Cyrus seemed to take longer. The first night he managed to fall out of the bed (we don't have a crib here) despite my pillow barricade. He didn't even cry! I was so upset. He was fine - no broken bones or obvious injuries. We pushed the bed to the corner of the room and upped our pillow barricades even further. Despite our efforts, he absolutely refused to sleep at all for our first three nights here. But since then, has finally seemed to settle and that's just his room now. We've had no incidents falling out of the bed since.

Medically for him, life continues to be an adventure. He's has now had to get his g-tube site cauterized on three separate occasions due to excessive build up of granulated tissue. (It looks gross. I will spare you).

He appeared to initially handle his bolus feeds very well, but has since developed some volume intolerance. This just means if we feed him too much, he will vomit. Vomiting is never any fun, but the other challenge is he's not allowed to head back to school for 48 hours if he has vomited! And well... he's not actually sick in this case, so this has been frustrating. I also stress constantly that he's no longer getting enough food. Any time we try to creep over 700 calories we have had issues. Whereas, for the first month after surgery he was having no problem with 1000 calories a day.

And in the last two weeks he's had a case of a ring worm rash, two colds that kept causing him to miss more school, and this past weekend he appeared to develop an eye infection requiring antibiotic drops. His delightfully happy mood that was mentioned in the last post, has unfortunately not made quite as strong an appearance in the last few weeks, but I'm excited to report we've started to see glimpses of the happy Cyrus again.

Seizures are on the rise still with 3-5 seizures per day at the moment. And we added so many new specialists to Cyrus's care team, that our doctors finally recommended us for Complex Care! This a management team, that basically communicates and coordinates between all our specialists, so that you - the lay person parent - don't have to be as good at that yourself. I think this will be a good thing!

We also have wonderful friends who have asked unexpectedly to throw us a baby shower (You know I haven't had any time to think about it at all!). I think they know how frantic the last few months have been and are eager to support us through the next few hectic ones in any small way. If you would like to support us welcoming Elli into our family this coming March, here's a link to our Baby Registry that I created for them. Amazon has added a cool feature since the last time I did this, where you can "contribute" to an item to make it much easier for many people to split the cost of a more expensive thing. Super cool!

He might be excited too... 

She's a squirmy little thing! Seventeen weeks (about 4 months).

Test of Endurance

Cyrus's face describes it all! 

Our lives have been crazy enough that I've barely had a moment to think, let alone string together any words for a coherent blog post or four, but there's definitely been a lot to write about. Consider this to be the first of three or four updates I hope to have out in the next two weeks or so. This post is for the month of August.

A whole two days after our sleep study and less than a week after failing his swallow study, Cyrus was admitted to the hospital again for an NG tube placement. I could tell the moment we were checked into our new room that he was not thrilled to be there - it's like he recognizes the place! And perhaps he did, as this was our third visit is less than a week. The worst part of it was, there were orders on his chart that said he wasn't allowed to eat anything by mouth! There those medical professionals go all panicking again...

That order wouldn't have been so bad except the order to place the actual NG tube hadn't been put in yet, so the nurses couldn't proceed even though everyone knew that was the whole purpose of our visit! He handled the fasting better than I would have - by basically rolling over to his stomach and giving up on life for awhile - acting as the personification of apathy and resigned acceptance.

Tell us how you really feel Cyrus.

The actual NG tube placement was quite the spectacle, that involved three experienced nurses, and a nursing student, and of course me - who was just holding him in place as best as I could. And he handled it like a champ! He tensed for just a second, and then was fine. The whole ordeal lasted less than thirty seconds. In fact, one of the nurses turned to the nursing student and told him, "Don't ever expect that procedure to go that smoothly. There's usually resistance and screaming involved. Even adults don't like it."

What IS this thing?!

He did spend the next hour or so dramatically waving his head back and fourth as if to say, "What is this?!" With the NG tube placed we still had to remain in the hospital for a few days to confirm that Cyrus could tolerate the feeds, and to receive training as his parents in checking the tube's placement, using the new feeding pump, and learning how to re-tape the tube in place so that he couldn't just pull it out. They also wanted to do another barium study - this one to map the shape of Cyrus's upper intestinal track to prepare for surgery for his permanent G-tube placement.

Imaging Study with cool x-ray machines!

This was a study that involved another really cool imaging technique that I got to geek out over. It was incredibly similar to the first swallow study, but I think I also just enjoy getting to see the inside of my son's body - especially live as it is working!

Mapping Cyrus's Intestinal Track.
This one reminds me of the monster in Stranger Things!

Afterwards we were discharged from the hospital, and were privileged enough to spend a whole day at home (two nights!) before heading back to the hospital for surgery. We were told to arrive by 10:30 AM, and that surgery would be at 11:50 AM, and that Cyrus needed to fast for the twelve hours prior to the procedure. We arrived, checked in, and waited.

And waited.

And waited some more! With Cyrus getting fussier by the minute! I checked in at the desk multiple times, trying to confirm that they at least knew we were here.

When we were just 15 minutes shy of our scheduled surgery, I went back to the desk, to ask what was going on, and why we had to arrive so early. She checked the schedule then, and informed us that Cyus's surgery had been rescheduled the night before for 1:50 pm, and that we have arrived hours too early.

"Did no one call you?"

I didn't scream.

A few minutes later, I received news about another totally unrelated family emergency, of which I could do nothing about. And Cyrus, the ever chill child, was now outright crying because he was hungry. I also hadn't eaten anything myself because I had planned to go grab food the second he was in surgery.

At this point a social worker came out to speak with us. She explained that not only was Cyrus's surgery rescheduled as the person at the front desk had already told us, but that the two surgeries before Cyrus in the operating room had both gone longer than anticipated, and they were running an hour behind.

I did cry.

The social worker gave me a snack pack and some meal vouchers, insisting that I take care of myself.  She also pulled us into a private waiting room, and requested Cyrus be given fluids to keep his blood sugar levels up. As a patient with a metabolic disorder, they apparently worry that the extended fasting can cause his blood sugar to crash unexpectedly. Unfortunately, having extra sugar in your blood does nothing to actually satiate you. And he continued to cry inconsolably for hours. Until around two in the afternoon when he cried himself to sleep.

Three hours after that (so now two hours later than running an hour behind, and more than five hours after the time we had originally been told), Cyrus was wheeled away for surgery. Kyle and I were able to have a real dinner before heading back to the hospital for our post-up surgery consultation, and to hang out with a very dopey three year old.

The surgery apparently went well, and Cyrus recovered quickly from the sedatives. He woke up  much calmer than before (sedatives were helping I'm sure) and we learned from an extremely friendly surgery recovery nurse how to give him his medicine through the brand new tube. And it was so easy! No thirty minute wrestling matches required!

He spent the next three days in the hospital to be sure he was continuing to heal well, to make sure he could tolerate this new method of feeding, and so that mom and dad could be trained on how to take out and replace his g-tube button. He hated being trapped in the crib during this time, so I tried to make a point of taking him out and sitting with him on occasion, even though this was difficult with all the monitoring cables attached to him still. This only placated him slightly, as what he really wanted was to roll around on the floor.

But once he got home, he still wasn't pleased since he wasn't allowed to be on his stomach. The surgeon said he wouldn't want to be - that it would hurt. Yeah, Cyrus didn't care. I'm not sure he understood the cause and effect that being on his stomach caused the pain to increase. If I turned away for a second he would roll onto his stomach, and then immediately start hiccuping. So he probably felt the pain for sure and was unhappy about it, but that was still the position he wanted to be in.

See! He's totally thrilled! And not at all too big for his chair...

This lead to three really long days! We tried to keep him in the chair for as long as he would tolerate (far longer and more often than normal). Then we'd move to the floor, but would have to hover and guard him from rolling over onto the surgery site. He was so angry with us like all of the time, and one of our attentions had to be on him at all times. Occasionally, we'd get a small breather if he would let us get away with putting him in the bean bag chair, but most of the time it didn't work. It was emotionally exhausting after two weeks of emotional exhaustion! But come that Friday, and we were both free, it was so much easier!

Except he was still constantly pulling on the button and the tube both. It was actually fascinating to watch him try to grab things so intentionally! It would take him three or so tries, but he would get the corner of his shirt and pull it up. Then immediately go for the button. And I just sighed - feeling he picked a bad time to develop fine motor skills. We learned to put socks on his hands. This backfired (or paid off, depending on how you look at it) a bit as within a day or so, he learned to grip things through the sock just by pushing his fingers into his palm.

Socks!

I'm delighted to say that once things settled down, things got immensely better for Cyrus. It has been so much easier to give him medicine - something that was becoming a 30 minute ordeal twice a day, now can be done in 5-10 minutes. Feeding him is so much easier, and he's clearly feeling better. Like I don't think I realized how much the constant aspiration was probably irritating him and making him feel off. The three weeks following the surgery, we were treated to Cyrus's happiest version of himself - we've seen this Cyrus on many occasion, but never which such frequency.

He put on three pounds in the space of a month, and has just had more energy in general. He's more active and less fussy. It's been really nice to enjoy. He is far more socially engaged. He just responds more when people talk to him or smile at him. He's laughing more. He is in love with life! I've certainly never been able to collect so many pictures of a smiling Cyrus before! And we find ourselves wondering why we didn't do this ages ago!

General Update: 

Our test of endurance seems to be continuing with a batch of regrowing and stubborn granulation scar tissue at the surgery site, common colds plaguing Cyrus and mommy alike, continued battles with our insurance company, volume intolerance (which involves vomiting), increased seizures while our neurologist is on leave, and extended hotel living (we're up to a month now)! Yay! But more on all that later! In the meantime, we're hanging in there taking one day at a time!

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!

...

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!