Sleep Experiments and Teething

So after three nights in a row of Cyrus staying up until somewhere between 2 and 4 am, we came to the conclusion that we needed to intervene. So we started limiting the naps. It did cost on a few extra seizures first thing in the morning (when the meds have worn off) and late in the evening (when he's super tired) that were a little more intense than normal and we got to see some super irritated pronounced eyebrow ridges after being awoken from a nap!  But after two days he stopped having these and seemed to have adjusted completely! With bedtimes around 11:30 pm! Woot!!

And that lasted about a week.

Then he started teething again.  And now he wants to go to bed at midnight (most of the time), but the poor guy just can't sleep. He's whimpering and crying or chewing on anything he can get his... well, teeth on. And whatever he wants to chew on we have to hold in his mouth for him. Even the teething pacifiers only stay in for like 20-30 seconds before he needs help getting it back. He's not really sucking on it. He's chewing on it!! Which doesn't help it stay in place. We've tried teething tablets and oral gel, and they work for a few minutes. And if he's super tired they even maybe work long enough for him to fall asleep. We tried a teething necklace too, though sadly I've not noticed any difference. It's cute on him though! It's got the amber and every third bead is amazonite, which is supposed to "calm the nervous system" (shown in next picture). Seemed symbolically appropriate.

But anyway, I find myself wondering why the pain seems so much worse in the evening! I mean, who thought that was a good system? He's fine during the day, but the sun goes down and suddenly he wants to chomp on everything and the crying is so pathetic sounding... It makes me sad.

To make matters more interesting, he's been having the more intense breakthrough seizures again. Though really I still think it's because of the disrupted sleep patterns caused by teething! But his third tooth just broke through today! So hopefully, we'll have a more peaceful day or two at least before the fourth tries to push its way out. And if they insist on only coming in one at a time, hopefully the rest will wait until summer, so we can be nocturnal together!!

In other news, Cyrus was assessed for his equipment needs today. And we have received a prescription for a squiggle chair with both a kimba stroller base and high and low seating base (His will be green!). Apparently the chair will transfer back and forth between the two bases. The stroller is way better for getting around and the high and low seating base allows us to bring him low to the ground so he can be social when we're on the floor, or raise him high so he can sit with us at the dinner table, and can be used as his high chair for eating. Super excited about these, but apparently it may take 2-3 months before the order is complete, so we have to keep making do with our infant stroller. They are also recommending a bath chair, but that is a lower priority at the moment.

After his assessment, we went to the Pulgas Water Temple and took lots of cute family pictures. While we were there it occurred to me that Cyrus has never had the chance to roll around in the grass, and seeing as it's been recommended to expose him to high sensory experiences and sensations we did just that! He was definitely squirming around on the grass - I'm not sure he knew what to make of it. I found his reaction amusing. Next goal is to take him to the beach!! So we can experience sand! And waves!

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Emergency Protocols

I remember my very first earthquake as a teacher. In fact, I was a student teacher in the middle of teaching one of my very first lessons, when the walls pulsed. Everyone froze. The students, myself, the cooperating supervising teacher. All of us. Despite the fact, that we have all gone through so many drills. Each one littered with so many eye-rolls that communicated clearly, "We already know this Ms. Payne! We've been doing it every year since kindergarten!" In that moment though, we froze.

The master teacher was the quickest to recover with a quick, "Well?! Duck and cover!"

I have never scrambled to get under a desk so fast! More panicked about the fact that I had frozen in an emergency situation where I was partially responsible for 30 pre-teens, than I was about the earthquake itself.

And over the years, I have learned that the more clearly defined the emergency protocols are, the easier they are to actually follow in the moment amidst all your own anxiety.

And then when it's your kid...

I woke up one morning last September and Cyrus was having a type of seizure I had never seen him experience before. I definitely spent at least five minutes just hoping, or praying or whatever you want to call it, that he would come out of it before I called the on-call pediatric neurologist at like five in the morning. I don't like that panicked moment of indecisiveness where you don't know what to do.

Then I spent the following week asking every neurologist (and there were a lot of them) that walked through the door for directions on what to do during a seizure, and I never felt satisfied that I knew what to do. Maybe there isn't really anything to do.

It wasn't until we reached our outpatient two week follow-up that we sat down and hashed it out.

1) If Cyrus starts having a tonic clonic seizure for longer than 5 minutes give him the emergency medicine.
2) If he has three seizures within one hour, give him the emergency medicine.
3) If he needs two doses within eight hours of each other, give the second dose but immediately call 911 to have breathing assistance on it's way.

I felt good about those directions. Everything was quantified and has clear follow up instructions.

But I quickly learned, that the emergency protocol never feels well-defined in the actual emergency!

What happens when the tiny child starts having partial seizures instead of generalized tonic clonic seizures? Are those as damaging? Do they need as much intervention?

And yeah, he just had three seizures, but each one lasted like three seconds. Whereas earlier today, there was only one seizure, but it lasted three minutes and the convulsions were far stronger. These three seizures can not really be worse than that, can they?

So really, the emergency protocols are actually:

1) Do you feel safe right now? Or are you freaking out?
2) Is Cyrus awake and aware? Or does he seem totally out of it?
3) And sure, if it lasts longer than five minutes... (which has never happened since the two status seizures in September).

So yeah, yay for "well defined" emergency protocols...

This last weekend, the Sun King has been on a hunger strike or something and been showing all kinds of signs of dehydration (extreme sleepiness, little to no urination, urination is darker in color, fever, fussiness, etc.)

Saturday Evening's emergency protocols:
1) If he doesn't pee in eight hours or his urine is significantly darker, call the pediatrician.

Check and check!

During that phone call, she said to give 5 mL of electrolyte infused water or coconut water every ten minutes. And if he still doesn't pee in two hours, take him to the ER.

I have never been so delighted to see a wet diaper in my life!!

He's actually been doing significantly better the last two days. Eating and peeing again. Still sleeping more than normal, but when he's awake he's actually awake! Went to physical and occupational therapy today like normal, and apparently passed his feeding assessment with flying colors!! And practiced standing up again! (The picture is below! You can tell he is thrilled!)

He seemed to have some stomach bug last Thursday that involved multiple vomiting incidents (though each was pretty mild) that probably (in conjunction with one of our medication experiments) caused all of this. Will definitely be relieved when his full appetite and energy come back!!

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Therapists are amazing!

Watching a doctor wave a flashy wand across your child's line of vision, only to watch your child not respond induces a special kind of panic.

And having to fill out the developmental questionnaire three times in one week for three different specialist assessments is more than depressing.

Does your child track movement?

No, apparently not. 

Does your child hold his head up?

No.

Does your child make eye contact?

Sometimes?  I think? 

Does your child smile?

Yes!

Does your child have a social smile?

No... not really anymore...

Does your child recognize you?

I have no idea. And if I can't tell I'm guessing no? 

And the questions go on and on. The whole thing is really good at identifying in excruciating detail every little thing Cyrus is not able to do.

And so that very first time when his first therapist (she's a visual specialist) hovered right over him and just waited until he focused on her and then moved ever so slowly to his right and he slowly but surely followed her with a delighted coo... Well, that's a powerful moment for a parent.

Shortly after that she had him on his tummy, and just by tucking his arm out of his own way and he rolled over within seconds! Totally and completely for the first time! Beyond amazing!

And that's the thing about therapists, they have a different job than doctors. The therapist figures out everything Cyrus can do. It's not a binary checklist at all - it's a developmental spectrum and they figure out where on that developmental scale he is and then work with him (and train us to work with him) wherever he is to make progress toward those developmental milestones that come naturally for most children.

Cyrus has been receiving services from a visual specialist since August and he just started physical and occupational therapy this last January. The physical therapist works with him on gross motor skills (things like head control and walking) and the occupational therapist works with him on fine motor skills (picking up objects with his hands or eating). Due to my work schedule I have never had the chance to attend one of Cyrus' PT/OT sessions. This week being my spring break, I finally got to change that!

And I found myself floored once again! I don't know how they do it, but therapists totally know how to communicate what they want a nonverbal child to do! Kyle refers to them as baby whisperers. They tuck a limb here or there, tickle his chin or pat the ground and ask in that high pitched baby voice. And Cyrus understands what they want him to do. Sometimes he's excited to comply. Other times he whines in complaint! And boy do they get him to work! They get him to play! Or discover his feet or stand up (with assistance)!

His head control has improved so much in the last 12 weeks - he's far less wobbly than he used to be. Does he still like to arch his head back? Yes! And now he seems to be more aware and purposeful about it, so he'll do it all the time! But from a prone position he can totally hold it up.

He can roll down a slightly inclined wedge almost completely unassisted on his right side. On his left he still needs a little help. And he seems to enjoy it - loves getting to the cushy mat at the end where he can just face plant!

He will bat at toys or his own feet. He looks incredibly uncoordinated when he does so, but there is little doubt that it is intentional. He even has a favorite iPad app! Some barnyard game or something... I don't know what it's called.

And yesterday evening I was making faces at him and he was imitating me consistently three or four times in a row.

His seizures are still better controlled than they were last January with a seizure free Saturday this week and only small ones since then. So overall, we've been seeing only improvement in these last few months! So grateful for the doctors, and especially the therapists that have helped us make this progress!

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Starting to Notice

Cyrus is getting to the age where it is becoming obvious that he is unusual.

Because he is so calm most of the time, we will take him everywhere. To friends' houses, out on hikes, to that weekend spiritual retreat, or out to dinner, etc. We'll just roll the stroller with the carseat right up to the side of the table or next to my seat. The hostess will often ask if I want a high chair. And the polite ones seem surprised when I say no, the less polite get irritated as the carseat stroller takes up some space!

During or after the event people will come up and comment.

"I think it is just so great how he just sat there so calm and let you have a dinner together."

"Is he always so calm? You're so lucky! They're not all like that! We couldn't take our son to dinner until he was seven!"

"Just you wait, he's going to running around terrorizing the house before you know it!"

"Is that baby real?"

And for the most part, these comments don't upset me. They do push me into that moment of awareness - awareness that we live in a different world. Because sometimes I forget.

And I want to tell these people:

"Do you realize how lucky you are that your child has the ability to misbehave? I bet you never thought of that as a privilege."

"It must be so reassuring that it's almost a given that your child will live to see their seventh birthday."

"Yes, he's real! You think I push around a doll in a stroller all the time?! What kind of comment is that?!" (Okay, that one might've irritated me a little...)

But I don't say these things. I'm not certain there would be a point. Instead, I gush about how I know that Cyrus is one in a million (literally). And how adorably cute and wonderful he is.

Then there's the comments from people who have some awareness of what's going on.

"He'll be fine. You just have to have a positive attitude."

"The doctor's could be wrong..."

And I can't respond to these. I have no words to say. Instead, I'm just hit with a wave of sadness. But I don't express that either. Instead, I simply smile and nod. Because I can appreciate that these people are trying to be kind, trying to be optimistic and reassuring. It's not their fault that what they say is not comforting in the slightest.

Cyrus is very close to outgrowing his infant carseat/stroller combo. And when it's time to upgrade we may be getting adaptive strollers and carseats because Cyrus can't hold his head up, he doesn't have the core coordination to sit up on his own. I don't completely know what these tools look like or how obviously different they will be - they're so different depending on what the child needs and we're just starting this process.

But I do know that at some point, probably in the near future, it will be obvious not only that Cyrus is unusual, but also that something is wrong. And I think people will naturally avert their gazes and will not comment.

And I think I will miss the random strangers telling me how adorable my son is, how lucky I am that he is so calm.

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A Touch of Masochism

The last two weeks have been fantastic! For the most part we have learned how to stagger his medicines to cover the whole day and he's been having 2-3 days a week totally seizure free! Though we haven't been able to get two of those in a row, but we'll keep trying!

He's been pretty active, improving in head control, passed his last swallow study with flying colors, and overall he's been very expressive if not always happy. He's got two teeth finally! Maybe that's why he hasn't always been in the best of moods.

The only annoying thing is he is on a weird schedule. He likes to stay up until like one or two in the morning and sleep in 'til noon. So really, it's just the normal Payne family schedule. But the problem is Kyle hates that schedule (he's not so good at the sleeping in part so staying up late all the time is hard), and I am on the teacher schedule, which (while contrary to my nature) is what I have to be on for the moment. (Can't wait 'til summer!) And we've learned that trying to mess with the schedule just seems to cause more seizures, which in turn throws Cyrus onto a new wacky schedule. We like this one in that, he's been seizure low, if not exactly seizure free.

Today though, has been a bit of an emotional day, but really I only have myself to blame.

Cyrus started it by having just an awful day yesterday. We had to wake him up super early for a therapy appointment and this seemed to throw him off completely (See! You can't mess with the schedule!). He had more seizures, and even though none of them seemed severe, he was just so completely out of it all day, which just hasn't happened in the past few weeks. I think the bad days just hit me that much harder when we haven't had one in awhile.

Then this morning he woke up early all by himself demanding food, which he promptly threw up. A huge amount of it too. These events always make me anxious as I know that they will eventually become far more frequent as Cyrus's disorder progresses. So whenever he throws up, I'm always nervous that it's starting to get worse. I especially hate it when it comes out his nose because it makes him totally congested and I have to spend time clearing it out, which he hates! Though I think he prefers being able to breathe.

But I also have to remind myself that kids just throw up sometimes. And Cyrus has always done so a little more often.

Then just when calming down, I made that anxiety far worse by writing a death scene of an epileptic child in one of my unpublished stories. Seriously, why would I do that?! Masochist anyone? But let me tell you it is an awesome scene. Almost beautiful in a morbid sense. Definitely very real and very human, which is my favorite kind of writing. And in general, if the writing is flowing you don't fight it! You let it flow!

After writing this scene, I found myself in need of some research to flesh out some of what came right before in the story, so I went looking for the ADSL personal accounts from parents and starting reading those. These always throws me into a weird mental and emotional funk, but at the same time I find them reassuring as their experience can answer questions about day to day living that doctors can't because this disorder is so rare that they've never had another case.

So rare that I could only find four blogs - three of which are no longer active, and another that doesn't really focus on the child as it was written by the grandparents.

So I decided that I wanted to contribute to this tiny community by sharing our experience. I already have written about it a lot through gofundme and Facebook and I have no reason to believe I will stop. It was just a matter of bringing all of those pieces into one place. And blogger even lets me backdate posts so I can add everything I've written since June. Plus it'll be nice to have a place where I can go back and look at everything as well.

Hence this blog! The Light of My Life title refers to both my husband and son. My wedding vow in 2009 was "You are the light of my life, and I must always look to that light." And when picking out names for our children all of them had a meaning that related to the Sun or to light. In general, pictures of the sun (both photographic and drawn) make me think of Cyrus when I'm not with him.

Hopefully, if you're reading this, it has something to offer you.

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Cyrus Lee Brown - A Light In My Life

Cyrus Lee Brown arrived on February 15th, 2015. I know that not all mothers experience that instant connection with their newborn child, but I did. The second Cyrus uttered his first cries, my heart just about melted. I have never been so excited or grateful to meet anyone in my life! 

We chose the name Cyrus because it means Sun as in Sunlight! We just knew Cyrus would be a source of light in our lives. 

When he was four months old, Cyrus began manifesting a rare type of seizure, that are referred to as Infantile Spasms. He was having dozens of these seizures a day. We were lucky enough to be living near Stanford, and Cyrus was taken to Lucille Packard's Children's Hospital. Because this is a gargantuan teaching hospital, they ran every test imaginable from genetic tests, lumbar punctures, blood tests, EEGs, and MRIs, and were able to diagnose him within a month! He has a freakishly rare disorder called Adenylosuccinate Lyase Deficiency (ADSL). By freakishly rare, I mean that there have been less than 100 cases worldwide since 1989 when the disorder was identified. We're like number 10 or something in the United States. Genetically, the disorder is believed to have originated from Belgium. 

ADSL is a metabolic disorder that is caused by having the incorrect genetic recipe for Adenylosuccinate Lyase, which is an enzyme that is used to make a purine nucleotide called Adenosine. Adenosine is used in DNA replication, transcription, and to make cellular energy in the form of Adenosine Triphosphate (ATP). Needless to say, it's important and it will affect him severely both intellectually and physically. This metabolic disorder that is so complicated and so rare, that it is not well understood even by neurologists or metabolic geneticists (honestly, his care team had never heard of it until Cyrus's genetic test came back positive). There are several theories as to what specific mechanisms in his metabolic pathways causes the developmental delays and epilepsy, but the research is far from well defined or certain. And though several experimental treatments have been tried, there's been little in the way of success in treating the underlying metabolic dysfunction. 

His prognosis is unclear as he is presenting on a different timeline than any other documented cases. He has presented with seizures later than those have have the severe version of the disorder, and unfortunately far earlier than those who have the moderate version. We have been warned that he is unlikely to learn to talk or to learn to walk, and that his life expectancy is significantly diminished. 

Initially, I felt incredibly guilty and responsible. And then angry and devastated. But at the end of the day, I really didn't want to spend our time grieving something that has yet to happen. Instead we want to treasure each moment and Cyrus has definitely taught me to slow down and be more present. To let the other stressors and daily irritations roll off my shoulders. And to be joyful and grateful in the the tiniest of moments. He has made me a far better person and is definitely living up to his name. 

Birthday Party!

Cyrus had his first birthday party yesterday (two weeks after his birthday, but it was the soonest mom and dad could pull it off!) in the park. He was even awake for most of it! He is quite the popular little guy with lots of little friends that chased bubbles in circles around him and ate lots of delicious cheesecake with him! He sure is lucky to have so many people that care about him in his life! 

And last weekend he took his first ever vacation to Monterey for a spiritualretreat with mom and dad and got to make lots of friends and listen to awesome music!

He's been doing fabulously well these last two weeks. He's on a new medicine that seems to have brought back his expressions and emotions (as well as reduced the seizures)! He has started smiling again this week, making more cute little noises, and glaring at us whenever we make him sit in the tumble form chair for too long! His physical, occupational, and visual therapists have all three been thrilled at what they've been able to get out of him this week. 

So good news all around! Thanks all for the love!

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