"48 Hours"

There is a certain kind of patience and determination you sometimes need to manifest when you have to call the specialty pharmacy.

The specialty pharmacy is where you get prescriptions that are not typically kept in stock by a regular pharmacy near you, either because it is a highly regulated substance, or because no one else in the country needs that drug! So instead of picking it up, it has to be delivered to you! 

Sounds convenient right?

Not even close!!

Cyrus has been getting one of his prescriptions (Sabril - also know as Vigabatrin) through the specialty pharmacy. And there's been more than a few headaches involved in that - basically once every 6 months when the refills run out and the prescription is renewed, they always have trouble getting that first batch to us for various incomprehensible reasons. I don't even know what they all are at this point. (Kyle's the one that's had to deal with most of it).

So when I told Kyle that the pharmacy had called and they didn't carry Cyrus's new prescription (tetrabenazine - it's supposed to help with his uncontrolled movements) and that they had referred us to the specialty pharmacy and they would call us within 48 hours and that we should have our medicine within a week, he groaned.

That was June 15th.

They were nice enough to call me the next week way more than 48 hours, but who's counting? This was not a big deal because this new medicine is not crucial even if feeding him is way more difficult than it used to be. Now that we've gotten him to go to bed consistently with the melatonin (the movements were making it difficult for him to go to sleep - he'd like hit himself in the face right as he was falling asleep - made everyone miserable) this prescription didn't feel nearly as urgent. I somehow missed the actual call, but she left a nice message saying that she needed to ask me some questions before she could proceed with filling the prescription.

So I called them back the same day and I had the privilege of speaking with a gentleman who essentially told me that he had no record of them calling me at all and that he had no idea what they needed as there were no notes in my account. But if he had to guess, it probably had something to do with getting prior authorization for the prescription. And that I should call my doctor to make sure that happened.

Really? You want me to call my doctor because you might need help getting the prescription approved by insurance. Was it rejected or not? Can't you run it now and see if it is approved or not? The regular pharmacy can! No?

Then he tells me that once they have prior authorization someone will call me within 48 hours to schedule delivery. And I'm still not worried about it. We're actually experimenting with a alternative remedy to control the movements - and it seems to help quite a bit.  When we were on vacation we ran out and the movements exploded in intensity. It's made out of wolf spiders - we call it the spider medicine (actually called Tarentula Hispana). So now this prescription feels even less urgent.

A week goes by! And around this time I realize that the Vigabatrin doesn't have any refills left (Yay!), but I still have a lot of it left so still I'm not too worried, but decided that we should take care of getting a renewed prescription because... well, this is important and... you know - specialty pharmacies.

I do call the doctor's office at this point and Perla (who handles all the prescription and pharmacy shenanigans) is so completely on top of it. She calls in both prescriptions to the insurance and gets them prior authorized all the way through the year 9999 (I'm not even kidding), then she calls the pharmacy to confirm this with them, and then she calls me and gives me the pharmacy number and the stupid menu #s I have to press to get to the right person without listening to the never-ending itemized list (they do it based on what medical condition you have as they have over nine different divisions that handle different types of drugs - I'm sure that has nothing to do with their inefficiency at all). Then she gave me her cell phone number and insisted I call her if I ran into any trouble at all!!

So anyway, next phone call. Pharmacy says they have the prescriptions and the prior authorization, but there's an unexpected delay. And they would call me within 48 hours once they have resolved their system error to schedule delivery.

Another week goes by. I thought that hours were a standardized unit of time, but clearly I am wrong.

They call me again on July 6th. And yeah, now I know the exact date as I started keeping a log! So she actually tries to schedule a delivery, but the copay for the new prescription was $150, and I don't exactly get paid during the summer and I thought I was getting a 90 day supply so that amount wouldn't be too bad, but she said she was only sending a 25 day supply. She was nice enough to offer to apply for copay assistance and promised to call back... wait for it... in 48 hours!!

So yeah, that didn't happen. I'm sure you're as shocked as I was.

I called them yesterday, July 11th, more than a little frustrated, and now I'm starting to get worried as I've been watching the Vigabatrin supply dwindle.  I asked for an update on the payment assistance and if we could schedule delivery. This particular lady told me that the pharmacy will not apply for copayment assistance - that I had to do that myself. And when I told her that the previous person had said they would apply for it on our behalf - she insisted that they never do that. Then she gave me a few numbers I could call to request that help.

I asked if I could pay for the first month now and apply for assistance for future refills. At least that way we'd finally have the prescription and would know how well it worked so we knew if it was worth it. She said sure!

And then she quickly changed her answer to no, explaining that there's a problem with the prescription.

What do I need to do to fix this problem?

I have to work on it. The prescription is switching systems. I have to work on it and then I'll call you back.

(I wouldn't bet on it... )

Okaaaaay. Can I schedule the other delivery for the vigabatrin?

No, the whole account is locked up. I have to work on it. I will call you back.

Is there a number I can call to check up on the progress?

Just the main number...

The second I get off the phone I go over to our little home pharmacy station and count out all the vigabatrin we have left. We have ten days worth. Then I start crying, wondering if the bureaucracy and inefficiency of the pharmacy is going to cause us to run out of medicine - wondering if the withdrawal from the seizure meds will break our seizure free streak. If this lady on the phone knows what it's like to watch you kid have five seizures or more every single day - to go months without him cracking so much as a smile.

Kyle was quick to reassure me that we could always call Sabril directly and ask for a sample (we had to do this once before when the pharmacy was being annoying). And I figured I would call Perla the next day to ask for support and advice and started outlining the sob story I was planning on telling to the next pharmacy phone worker I would talk to if things didn't get resolved.

I figured I would call them one more time before I stuck Perla on them. (She doesn't take prisoners). And this guy was awesome! I called and said I wanted an update on what was wrong with my account so that our prescriptions could be delivered.

He told me that there was nothing wrong with the account. That they had just sent the prescription to the wrong branch to be filled. I asked him what I needed to do to fix it. He said I didn't need to do anything - just had to stay on hold while he transferred me to the Sabril (Vigabatrin) branch and they could fix in in just a few minutes. He also told me not to get off the phone until everything was resolved and scheduled. And I didn't even have to tell him my sob story!

Then I had the absolute privilege of speaking with Gabriela in the "Sabril branch". She asked me to confirm a few things, asked how much we had left, then noted that we had received our last shipment in early June and were definitely due for a refill, and immediately scheduled it for this week!

Then I asked her about the tetrabenazine. She said that it was on hold because someone was still working on assigning a grant to it - you know, to pay for the copayment, but that if I wanted I could pay for it now myself and they would ship it too! So they do apply for assistance for you!

So yay! Only took 28 days!! Longest 48 hours of my life!

I figure my new strategy anytime they start telling me something that doesn't make sense or is not specific enough for me to understand the problem, to just hang up and call back until I get someone that is skilled in clear communication and reading notes in their system.

In other aspects of our lives:

We took Cyrus on a road trip to SoCal to visit his uncle and another close friend of ours. He handled the long hours of driving like a champ - only would start fussing after I had gotten away with three hours straight of driving. And when we took him out of the car, he rewarded us with beaming smiles that lasted at least twenty minutes! So I guess we have a new trick for putting Cyrus in a good mood! Just torture him with a long car ride first (we did get to hear some pretty mournful and resigned sighs along the way).

Then right when we got home, we subjected him to a visit from three of his cousins and he handled that like a champ as well. We did take him to the beach and I think he found it a little too bright and or too windy as he wouldn't keep his eyes open (he was clearly awake) - he was also super cold so we had to wrap him up.

He seemed super interested watching the girls play both with toys and their food, or on occasion him! And then we have spent the last few days at home settling back into our routine as I am back at work for two weeks and Kyle is taking Cyrus to all his appointments. I hear he is flying through therapy sessions and being just a good natured little guy as always!

Our New Normal

Cyrus has continued to do incredibly well, somehow remaining seizure free for three weeks now! And as a result we have been able to benefit from his personality coming to the surface. As you know the first few days were incredibly happy - I think that coming out of his seizure fog, he was just in awe of the whole world!

Since then, we've also gotten to see the other side of the emotional spectrum. He's more opinionated in general. He seems to hate his medicine again, which makes administering it three times a day super fun. And he will sometimes object the moment we stop playing with him or put him down, even though our arms are throbbing with overstretched muscles. He constantly wants to engage and he's so cute he's just impossible to resist.

Last week we took Cyrus to a sensory and social class. The first hour is spent in circle time, designed to promote sensory play. This involves playing with toys, singing songs, and socializing with other children in the class. And despite getting up at eight in the morning (which is four hours earlier than normal) he did well. The best moment was when he turned to a girl sitting next to him and they locked eyes, maintaining eye contact for a solid 15-20 seconds. I have never seen him do that before. The second hour is devoted to motor play. Here, the children get to play on swings, ball pits, just rolling around on mats. His favorite was definitely the trampoline, which was enough to send him into constant giggle fits.

The rest of the day however, was ruined after getting up so early and exhausting himself in the class itself. He became king cranky pants and would not stop crying. Even once I got him down for a nap, he woke up ninety minutes later almost as angry before he went to sleep.

And you know what?! It was an exhausting day, but even then I couldn't wish for anything different. Knowing what it's like when your child doesn't cry - I'm telling you this is better! So if he needs to be held and soothed and rocked, we will do that. I'm getting really strong! The constant squats don't even phase me now! And when he is calm again when being held (though I cannot put him down without his face crumpling into tears) we're simply going to cuddle up and settle in with Netflix!

This week has been better. After confiding our going to bed at a reasonable time troubles to our pediatrician, she gave up some melatonin. We give it to him 30 minutes before bedtime, and it's magic!! He falls asleep naturally despite the choreiform movements. Cyrus has gone to bed by eleven for a whole week in a row! (Though mommy has yet to adjust to not staying up all night...) With the better and more consistent rest, the chorea has lowered in intensity and his mood has been more consistently on the happier side and life for all of us feels more sustainable.

Grandma is visiting this week and was willing to babysit, allowing Kyle and I go on a date for like the second time in six months! Yay!

The Power of Recognition

As I've said before, one of the questions that they ask on the developmental surveys is, "Does your child recognize you?" When I first read that question, I wondered how one would be able to tell. I mean, he was four months old! An infant! He can't say, "Hey mom!" How do others know if their baby recognizes them? And while I was sitting there agonizing over how to answer this one question (out of like fifty many of which were equally baffling), I thought of a moment just a few weeks prior.

I had gone to his crib to wake him up only to discover he was already up! And he looked right up at me, we made eye contact, and his face lit up into a smile. It was so cool!

I described this to the developmental psychologist, and she patiently explained that was exactly right! And immediately checked something off on her chart.

The only thing was I had only ever seen him do that once. And that one time, was before he ever had a seizure. But I did take comfort in other small things - like the fact that I could sooth him when he was distraught or teething. Or being able to make him laugh!

But a lot of the time, we'd get nothing. We try to play with him, sing to him, dance with him, or read to him, and so often there would be no response. So I'd put him down and he'd wiggle and squirm on the floor. It was honestly hard to engage with him as much as a I felt a child should need, just because it was sometimes like interacting with a wall.

We are still seizure free. Today we're working on day ten! I have no idea what changed. And I have no idea if it will last, but it's been amazing to watch Cyrus come alive. On days five, six and seven the choreiform movements have lessened in intensity, if not in frequency. And he's become so responsive! He's looking at things and experimenting with all kinds of weird sounds that I don't know how to emulate. And if you talk to him, he talks back!

And on day eight and nine he has been so happy! He's been smiling and laughing socially, or giggling because we were pushing the wheelchair fast and then slow and then we did a wheelie! We took him to class on Tuesday - something we've been doing for over a year and never had a problem because he's so calm! But this time, he was so joyful! He was cooing and laughing and smiling. I actually couldn't hold him as this seemed to set him off further. When I put him on the floor, he was still happy, but more quietly so. But if I or dad picked him up he'd just turn into a little laughing motormouth!

And I realized then, he recognizes us. He knows who we are. And he probably has all along even if he is not always able to show it. I need to always remember this experience. And I need to never take it for granted.

We hope this trend continues and that we get to keep getting to know Cyrus better! It certainly makes it harder to get things done, as he is present and he wants to engage and play all afternoon and night! (He's seems to hate mornings). But I wouldn't have it any other way!

A few other notes: 
On Tuesday, we had an excellent appointment with the neurologist who assured us that if he suffered brain damage enough to get rid of the seizures that he would be near comatose right now. So this change may just be part of his progression. She also told us that since September Cyrus has continued to surprise her with how well he's been doing - with how well he's been growing and progressing. So even if it feels aggravatingly slow to us at times, we have to remember that he's already defying the odds.

This powerful piece is really what has got me thinking about recognition this last week. And while, it's not related to our experience other than that, it's a good read about a difficult moment. A beautiful piece that I recommend reading!

__________________________________________________

Support Cyrus

The Impossible Has Happened!!

We are currently four days seizure free!! I have been so scared to commit that little fact to paper - I don't want to jinx anything after all! It feels a little surreal and almost impossible as Cyrus has not had more than one day in a row seizure free since September! And then there's also always the possibility that he is still seizing - either after we have fallen asleep trying to stay up with him, or that his seizures are just so small that they are not visible, which would still be an immense improvement!

And of course because things can never be totally simple, this blessing has been accompanied by a few new concerns as well. Cyrus has always had these bouts of hyperactivity or strange purposeless movements, usually after he's had a dose of one of his meds (clobazam). His neurologists refer to this movement as chorea or chorieform movements, and is perhaps maybe a result of his damaged basal ganglia. And since Tuesday of this week, this behavior has stopped being episodic and has been totally constant. If the boy is awake he is moving - he is moving his arms, his legs, and his mouth. Here's a couple of videos. The first is from Tuesday and the second is from last night (Friday). Perhaps you can tell me if you think it's staying about the same or getting worse.

This has caused a few problems. First off, he can't stay on his back and keeps rubbing his face all over the floor to the point of giving himself rug burns. I almost feel like we should transition to spending more time in one of his chairs, however the we've managed to break our on-loan wheelchair (it was already damaged so when this happened it was not hugely surprising. The thing is ancient!) so his headrest is not quite in the right position and I don't want him kept in it for longer than he needs to be for physical therapy reasons, and he hates the tumbleform chair - sitting there all day would make him absolutely miserable not to mention angry.

Which brings me to my second point, physical therapy has become rather difficult. His little body is completely out of his control - constantly flailing and it's difficult for him to do any of the exercises from tummy time to rolling down wedges or sitting up in a supported sit. Very frustrating. 

And most importantly, his ability to eat has significantly diminished. He is not sucking on the pouches and even when we spoon feed him, it takes him awhile to find the coordination to swallow. It used to take 20-25 minutes for him to eat about 10 oz of food. Now, it's like 45! And he gives us no signals anymore, it's hard to tell if he's hungry or if he wants more. So we've just been guessing based on how much food he was eating last week and how often he wanted it. There was one point last night where I felt we might have waited slightly too long as he seemed almost frantic when he was offered food, but I'm not really sure, as he has become even more difficult to read. We've not been able to get much expressive emotions from him in general this week, though he is still babbling when we pick him or talk to him or just 'cause. So that's good! 

Anyway, we conveniently have a neurology appointment on Tuesday and hopefully she can assuage my fears of a completely destroyed basal ganglia or at least offer us some options or strategies moving forward! 

In other news, I have just completed the school year and am absolutely looking forward to getting to spend more time with Cyrus and just be a bigger part of his therapy team! And maybe give dad a night or three off. 

__________________________________________________

Support Cyrus 

Even Getting Sick is More Complicated

As much as I hate teething and nursing a child through a cold, I actually slightly covet these moments as being an experience that other parents can completely relate too. These are just universal struggles that are understood by any who's had a honor and privilege of caring for a toddler.

But this week's particular bout of illness turned south and became a lot more frightening than Kyle or I had really anticipated.

Cyrus for most of Wednesday was vomiting a lot, even when he didn't really have anything left in his stomach he'd still go through this convulsing heaving thing that just makes me want to cry. I hate it when he vomits. It's one of the few things that seems to really upset him - it always comes up his nose as well and then he can't breathe, which makes it hard for him to cry, which just seems to upset him that much more!

In the evening, he couldn't keep anything down at all. The moment of panic was when I realized that that included his anti-seizure meds. I had given him a dose. He threw up within three minutes. And I followed the protocols of giving him a second dose if he throws up within 30 minutes, and he was throwing up before the dose was even finished!

This was compounded by the fact that he had a 102 degree fever and that reduces the threshold for seizures. When his meds were an hour overdue and he started having seizures pretty regularly. We gave him his emergency medicine which, thank goodness, is a suppository and that seemed to calm them for a bit.

I was panicking that it was only a matter of time before a status event* would hit. Part of me felt like we should just take him to the emergency room in anticipation, but going there unnecessarily seemed like a terrible idea as well.

The pediatrician wasn't answering her on call pages. And in my panic it took me far too long to remember that there was an on call neurologist too!

Thank goodness for the internet (and FaceBook in particular), which connected me with a group of families that all have children with the same disorder. Lucky for me one of my new ADSL parent friends was online, and able to chat and give me some advice and calm me down having been in similar situations before. She recommended keeping the diastat and car keys on hand, and wait it out at home as long as possible where he would be more comfortable. In general, don't go to the ER if you don't have to.

The pediatrician did finally call me back. I learned from her that you can deliver liquid medicines rectally as well! That you just have to go slow, 1 mL at a time, and that it will be absorbed quite quickly.

The neurologist informed us that if we needed to rely a little more heavily on diastat than normal just to get through the night, that one double dose wouldn't hurt him. She advised to wait an hour without vomiting, then try to give him some pedialyte. Then wait thirty more minutes and if he kept that down to redose the seizure meds only a few mLs at a time every ten minutes or so. That again, even if he got one double dose (as we can never be sure how much of the original dose he absorbed before he threw it up) he would be fine, if a little more sleepy than normal.

Two hours, three seizures, and another emergency dose of diastat later we were able to administer the full dose of meds over the course of 90 minutes. His fever had dropped to 100.3 degrees, and we were all able to go to sleep.

The next morning he woke up without a fever. Took to some food, though not with as much gusto as usual and was not followed by vomiting. His fever came back during this second afternoon and evening, but it was never as high and he only refused half of his meals. By this morning (three days later), all remnants of the fever are gone, he's still been a little sleepier and calmer than normal, but his appetite seems to be back!

*Status event - also known as status epilepticus, which refers to a seizure that does not break or resolve on its own for longer than five minutes (they can go on far longer than that).

__________________________________________________

Support Cyrus

Laughter Storms and Giggle Fits

There have been some dark days in Cyrus's life. Usually I measure them by how long it's been since he last smiled.

Our longest stretch was this last winter. From November 5th to January 11th there was not a single smile. During that time, I began to doubt I would ever see a smile again! There was this one picture, taken in early November that I loved! But I also hated it as evidence of something that he had lost. And that smile in January had to keep us tied over until February 20th. 

But a new medicine (clobazam - also known as Onfi in the US) brought them back. And smiling and laughing are a much more regular occurrence now. But usually they involve wild acrobatics of being swung around, thrown in the air, or dropped onto the bouncy bed. 

Cyrus has had a string of some not so happy weeks. He's been a little hoarse, very sleepy, and yesterday he threw up and had a spike in seizures and then stayed up until three am grinding his teeth together. 

I can also tell you that until today, the last time he had smiled was May 7th. 

Today, we have completely broken that trend! He has been so happy today! His voice is back and he has been smiling and laughing all day. He has laughed socially! And laughed spontaneously on his own while in his own world rolling around on the floor! We converged upon him immediately to share and multiply his joy. It was a pretty cool moment. And an even better day. 

__________________________________________________

Support Cyrus

"First Words"

At some point in my life I asked my mom what my first word was. She told me that I was at a doctor's appointment. The doctor walked into the room and according to the story, I smiled at her brightly and said, "Hi!" clear as day. My mom was shocked!

Of course that might have been because I was a scant two months old, and had never made any kind of coherent sound ever! I probably also had absolutely no idea what I was saying. It was just a beautiful and happy accident! 

And she has no other stories. That was the most memorable moment for her. 

At three months old, Cyrus stumbled onto some accidental words. He liked hard g's and would fall into "gook" and "guru" in-between the "agoo"s and "aroo"s and we would joke that he was a racist baby (and we'd have to train him out of that) or a spiritual baby (and hopefully he could impart his wisdom to us!).

Since September, Cyrus seems to have lost his consonants and really only makes vowel sounds. But today, we've had some happy accidents. He apparently said "ow!" when Daddy was putting him in the chair, which is ironic because he hates the chair! Daddy was not a fan...

And while I held him he kept spouting off "Oh? Oh? Oh?" It sounded just so much like a question each time. And it was just the coolest moment because he was almost silent before I picked him up, but when I held him he looked at me and just kept talking! And when I had to put him back down he got pretty quiet again. But then ten minutes later, I picked him up again and he jumped straight to an "Ooh," followed by a sharp and happy, "Oh!" 

Seemed very deliberate and I've never really noticed him doing this before. It was also made all the more adorable as the little guy seems to have a sore throat and sounds a bit hoarse. I guess even non verbal children can lose their voice. But yay for linguistic and/or social development regardless! I'll take whatever I can get! 

Now he's trying to bury himself in the floor blanket. He's been doing this all week! I believe his master plan is to turn it into a swimming pool with all this excess drooling.