Autism, epilepsy and song

Your love is a symphony
All around me, running through me
Your love is a melody
Underneath me, running to me

~Switchfoot, Your Love Is a Song

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My friend JoyMama has a darling daughter Joy who is Rhema’s “sister across the way.” Both our girls are window-dancers and are usually up to the same antics. They are the same age, and both have autism and epilepsy issues. Months ago JoyMama sent me a piece about the comorbidity of autism and epilepsy. According to the article, roughly one third of individuals with autism also have seizures.

JoyMama pulled the following quote:

“During development, the brain is trying to set up all these connections,” notes Ann Berg, research professor of biology at Northern Illinois University. “You’ve got these little neurons trying to sing to each other and then this crashing cacophony comes down over them.”

Then JoyMama wrote something that made my eyes smart and gave me goosebumps:

“Blessings on our beautiful girls and their hard-won songs…”

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I’ve written about it many times here. Rhema. Her language, her life is a song. Hope calls it “talking with music.” We don’t know or understand the words, but her serenade is ever present. Like a long musical, she intones her heart-thoughts; she sings every emotion. It seems to be a melody for her, for us, for the morning and night, for toilet paper and rain and air and macaroni and blades of grass; she hums to the world.

And when I think about it, it kills me and inspires me that she keeps singing.

To date, Rhema’s epileptic condition has proven untreatable. She must endure several EEGs a year, and for a child with autism and the communication paralysis and anxiety and all the sensory sensitivities that tag along, the EEG hookup can be sheer agony. So it was yesterday, and I’m convinced she fought harder than she ever has.

Can I say? I wish we didn’t have to keep doing this.

For 24 hours a device in her backpack will record every spike and wave, every electrical blast in her brain. Just a few months ago the EEG revealed that the “crashing cacophany” occurs on average 58 times a minute for my girl.

She repeatedly takes my hand and puts it on her head. It’s her way of pleading with me to take those awful leads off her head.

“I’m so sorry, sweet girl. We have to keep it on.”


This is the face of autism and epilepsy.

And this is the face of a brave little soprano opening her mouth,

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and singing through the storm her hard-won song.

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EEG-ness

“Cause it’s been fear that ties me down to everything
But it’s been love, Your love, that cuts the strings…
You make me want to be brave.”
~ Brave, Nichole Nordeman

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In the past few months there’s been concern that Rhema might be having seizures at school.

She suddenly squeezes her eyes tightly shut. She covers her face with her arms as if she cannot tolerate the light. Sometimes she’ll drop and huddle on the floor, immovable. Sometimes, after she’s “snapped out of it”,  she becomes upset and overheated and tries to remove her clothes. At school, her teachers call these episodes “breaks.”

With Rhema, it’s just so hard to know sometimes. Brandon and I have always assumed that these episodes were behavioral – just the latest odd thing in a slew of odd things she does.  For months we’ve gone back and forth, talking with teachers and the school nurse, talking with the staff at Children’s Hospital, video-tapping the episodes. We’re still not sure.

So we scheduled another 24 hr. ambulatory EEG. Sadly, her last EEG was… messy; her brain spiking on average 40 times a minute.

Yesterday my father-in-law went to the hospital with us and helped me hold Rhema down while she got the leads on. Rhema has had more EEGs than I can count since she was two years old. By now she knows the drill, but it is absolutely terrifying , even painful, to her.  

As soon as we enter the room, she is humming nervously and covering her ears.

Asking her to lay still while a stranger messes with her hair, measures and writes all over her head is like asking a teapot of boiling water not to scream. And then we wrap her in sheets and seal her up in a papoose (aka straight jacket.) All she can do is jerk her head back and forth, tears pooling in her huge brown eyes. The cold glue that smells like paint thinner, the texture of the gauze, the loud sound of the suction tool near her ears – it’s a sensory onslaught.

But she handles it well. While her head is being wrapped tighly, she says “Uh oh, uh oh.” She babble-complains, and I kiss her red-hot cheeks.  She calms  herself with a song of words I’ve never heard.

This afternoon we’ll go back to the hospital. Rhema has been so good. She has not tried to rip the leads off her head even once. She kept the backback with a special recorder on all night long, and she slept peacefully!

I’m just proud of my brave girl.

Juncture

I’ve collected lots of photos over the years of her in the hospital for EEGs. I’ve kept time in my heart and mind on all the effort spent making sense of the seizure activity in her brain.

  

The pictures remind me of our history, the long trail of medications in treating Rhema’s Landau Kleffner syndrome. Depakote, Keppra (aka Kepp’rage’, nightmare drug), Lamictal, Diazepam and combinations thereof and countless EEGs.

 

We’ve recently had a long period of respite in terms of Rhema’s epilepsy. But it has not been without cost. Just before Brandon deployed we made the difficult decision to put her on a high dose diazepam/valium protocol – the only drug that seemed to calm the continuous spikes in her brain. The medication can be highly addictive with long-term use. In fact, she went on it a couple years ago in the hospital and even though she had a clean EEG for the first time ever, doctors weaned her from the drug immediately and put her on another (non-addicting) anti-seizure med. The seizures came back with a vengeance.

We decided to go back on the diazepam long term, not knowing what “long term” meant but knowing it probably could not mean forever. We just knew this was a chance for Rhema. With her brain quiet, she could really learn and even gain language.

So it’s been over a year and I have not given much thought at all to spikes and absence seizures and EEG’s. Such a relief. In fact, only when I come across an old picture every now and then do I remember that we are on “borrowed time”.  

Last month when I called for a new authorization on Rhema’s anti-seizure med, her neurologist got antsy.

Time’s up. Time to deal. Time to re-evaluate. There’s always something, isn’t there?

We are at the crossroads again… facing what seems to me in my freak-out moments like an almost impossible decision.

Is the med still as effective as it was a year ago? Is she good and addicted now after a year on the drug? Will she go through some frightening withdrawal if we take her off? Will the seizures come back again? Will she lose all that she’s gained? Do we dare take her off?

Zero hour is almost upon us.

24-hour ambulatory EEG on Monday.

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“O our God… we have no power to face this… We do not know what to do, but our eyes are upon you.” 2 Chron. 20:12 



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On Expectations

“Just wanted to let you know the EEG results are back. Dr. C will be calling to discuss them.”

I check my little equation: Nurse will call = good news, Doctor will call = not so good news.

I wait for the rest of Thursday.

And all of Friday…

And all of Saturday and Sunday…

“Dr. C never called,” I say on Monday morning.

“No? He’s been really busy. I’ll send him a message again to call you.”

I wait for the rest of Monday.

And now, Lord, what wait I for? My hope is in thee… Ps. 39:7

“Dr. C never called,” I say on Tuesday morning.

“No? I’ll have him paged.”

I wait some more.

“Dr. C never called,” I say at the end of Tuesday. “I’m thinking maybe the EEG is clean? Because he hasn’t been in a hurry to call me… I just wanted to know before Thanksgiving…”

“Hold on,” she says. A minute later, she’s back on the line: “Dr. C wants to meet with you tomorrow. Can you come in to Children’s tomorrow at 10?”

“Ummmm, yeah. Yes. Sure.” Thinking, this can’t be good.

 

By the time we’re ushered into his office the next day, I have already:
~held Rhema for dear life on the elevator in the parking garage (she likes to bolt when the elevator doors open at every floor
~saved her from crazy Boston drivers at a busy intersection
~extracted her from every wheelchair (some occupied, some not)
~lost my parking ticket
~watched her run in circles in the waiting room, sip someone’s soda, jump on chairs, baptize herself in the water fountain, and try to take a drink from the fish tank.

Dr. C (whom we are grateful for) turns to me and casually asks,

“So did anyone ever call you about those EEG results?”

Um….  No, dude.

For a moment, I imagine myself a spurned lover at his feet. I waited by the phone for 5 days and you never called. WHY DIDN’T YOU CALL ME?????

The image is interrupted by Rhema pushing him out of his rotating chair so that she can spin in it. As I pull her out of the chair, she taps on the keyboard on his desk.

I make a joke: “She’s pulling up her results. The suspense is killing us.”

He laughs… kind of.

Then he tells me her EEG showed no seizure activity during sleep. Good news. Wow! The med is working.

Then he goes on explain that the EEG did indeed show sub-clinical seizures during the day and during drowsiness. I knew this might be the case based on her behaviors lately, and her school nurse had called about absence seizures.

Still, as I skim the report, my hands betray me; they’re shaking.

On cue, Rhema flops to the floor and begins to melt down.

We leave with a plan to increase her current med again and a new prescription for another drug to try. Dr. C says he’s sending our “case” over to a new doctor who specializes in sleep disorders and seizures. “Maybe he’ll have some ideas.” This, he says, after working with us for three long years to treat Rhema’s epilepsy.

Why is it that I keep waiting on things and people, doctors and drugs – anyone besides the One who knit her together in my womb, colored her like a mocha frappuccino, spirited her with an iron will, named her and loved before I knew her?

Ultrasound: Rhema at 29 weeks

 

My hands steady and a peace washes over.

My soul, wait thou only upon God; for my expectation is from him. Ps. 62:5

Sleep Deprived EEG

Update: Rhema is sick with the flu. EEG cancelled!

Rhema will have a sleep deprived EEG Tuesday morning.

That means I have to keep her up all night tonight and then drive her into Boston for her EEG, during which time they expect her to sleep.

A couple years ago when Rhema did her first BEAM study at Children’s Hospital, a technician sat us down and told us everything that would happen. It was 12:30 in the afternoon when he explained that he expected Rhema to sleep during the 4-hour study. Brandon and I stared at him for a moment and then, as politely as we could, laughed in his face.

“Oh, she won’t sleep.”
“Oh, yes she will.”
“Oh, no she won’t.”

He explained that he had been doing this for a billion years and was a master at getting the kids to sleep during the test. He said that once the leads are on, he lays the child down with mom or dad, turns down the lights, turns on music or a video, and the kid always sleeps.

We bet him a pizza our kid would not.

Needless to say, I have a handful of reports from BEAMs and EEGs performed over the past couple years that say, “Unfortunately, we could not achieve sleep.”

In fairness to our BEAM friend, I must admit that one particular day, Rhema was off her game and did actually sleep for a few minutes. She was quite disgusted with herself when she woke up.

sleep_eeg

But back to this week’s sleep deprived EEG. I called the kind folks over at Children’s and explained that Rhema is quite used to being up all night and then going through a full day of school (including an hour long bus ride each way) and after-school therapy without so much as a yawn.

It would be better to do the standard 24- or 48- hour EEG in the hospital – this is what we usually do – because she will sleep at some point in that period of time.

But the epilepsy floor is currently filled and we’d have to wait several more weeks for a bed to free up.

“I understand. I’m just worried she won’t sleep for the EEG Tuesday morning.” I said.

“We’ll try our best,” the woman on the phone said.

So there it is. All-nighter tonight. EEG in the morn.

As my blogging buddy Tiredmama would say, To sleep or not to sleep, that is the question.

(We actually really need her to sleep during the EEG, so that her doctor can determine whether her anti-seizure med is still working. Ideally, those beautiful brown eyes will close once the 32nd electrode is glued to her scalp.)

Anyone wanna bet me a pizza?

Hospital Time

backpack

We’re off to the hospital
for a couple of days
For long-term testing
of Rhema’s brain waves

 

Confined to one room,
hooked up to machines
Keep Bouncy Girl still?
Ha! What a scene.

 

We’ve got movies and toys,
books, puzzles and games
All with high hopes
she’ll be entertained

 

Children’s Hospital Boston
is a sobering place
So many sick children
We pray for God’s grace

 

Good results for our girl
We also pray for
Still hope for a miracle
of seizures no more.

 

We’re so thankful to all
Who send well wishes our way
I carry each one of you
in my heart… always.

 

(So don’t make fun of my poem!)

Ambulatory EEG

Cute backpack, eh?

Cute backpack, eh?

Yesterday Rhema got her own princess cap.

We’re at home for the next 24 hours doing an ambulatory EEG. Initially, we were happy at the thought of not staying in the hospital, but we were concerned about keeping all the electrodes glued to her scalp and the monitoring equipment out of the toilet (literally). We’ve had to be pretty inventive in the last few hours, but so far, Rhema is doing great!

The diazepam protocol Rhema’s been on for the last six weeks has brought about some interesting results. There’s no doubt that it has helped to calm the electrical storm in her brain, and there have been several amazing moments for us:
1) In the hospital, a high dose of diazepam essentially cleaned up her EEG. Spike activity was knocked down to 2%!

2) Hi!

3) Soon after she began the protocol, I believe Rhema spoke her first sentence. She wanted a popsicle and dragged me over to the freezer. She said in a sing-songy voice, “Iwant… …. …. … pop.”

4) She began greeting me. If I’ve been away for a couple hours, she runs to me with a smile, claps my hands together as I stoop in front of her and says “Yaaayyyyy.” Oh. my. heart.

5) This past weekend some people stopped by our house (2 adults and a 6 year-old girl). Rhema walked right up to them, and when they said hello, she repeated “hello” right back to them. Then, with interest in her eyes, she walked over to the little girl and gently patted her on the chest. Brandon and I had to pick our jaws off the floor. We’re still trying to figure out what caused that little exchange. We have never seen Rhema do that before. Ever.

Most of these events have been “Elvis Sightings” moments; they came out of the blue, and then they disappeared just as quickly, not to be repeated. 

And besides them, Rhema has just been Rhema.

In fact, the diazepam has actually caused some not-so pleasant side effects. It usually makes her hyperactive and agitated. She’s been unable to focus at school and we’ve seen an increase in noncompliance and tantrumming. She’s had trouble sleeping at night (but Kathryn prayed Ps. 4:8 and it’s been getting better!). And she has limited her already limited diet to about 3 food items.

Sigh.

Honestly, a small part of me was hoping for some earth-shattering miracle. I could see us on the cover of Children’s Hospital magazine with the headline “Medical Breakthrough” and Dr. BEAM beaming at our side. It didn’t happen. But that’s o.k.

I am just thankful. So thankful that God is God of both big and small miracles.

Only peace here in this heart of mine… and that, my friends, is one of the big miracles!

Thou wilt keep him in perfect peace, whose mind is stayed on thee: because he trusteth in thee. Trust ye in the LORD for ever: for in the LORD is everlasting strength.” Isaiah 26:3-4

Home Sweet Home

What we thought was going to be a 24-hour stay in the hospital turned out to be three days! My aching bones will be so happy to rest in my own bed tonight.

I don’t wish to bore with Rhema’s medical details. Something rather exciting happened, however.

Dr. BEAM and his team were willing to think outside the box, and I love them for it. Mara Mara said seizure disorders have been described like a radio losing its signal or a TV screen flickering on and off. The first night Rhema’s spike index was around 65% — better than her last reading (which was 85% – 100%), but still not great.

Dr. BEAM decided to try a high dose diazepam (valium) protocol on Rhema the second night. This is commonly used to treat Landau-Kleffner syndrome, a disorder in which epileptic seizures during sleep affect a person’s ability to speak and understand language. Dr. BEAM was hopeful that the protocol would improve things somewhat, quiet the electrical storm in Rhema’s brain.

But none of us were quite prepared for the result –

After an initial violent reaction to the high dose, Rhema slept like a baby. The next morning Dr. Duffy’s team excitedly came to us with the news that Rhema’s sub-clinical seizure activity during the night measured at 2%!!!

One doctor said (and I am nearly in tears as I write this),

“Last night her brain rested for the first time in a long time… maybe ever.”

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Over the next month, we will be giving her a much smaller dose of the diazepam along with her seizure med (and more EEGs in October) in an attempt to continue reducing the spikes. The doctors are enthused; hopeful that we may see remarkable improvements in Rhema if we can keep the spike activity down. Perhaps in doing so, Rhema will be able to acquire some language.

That’s a big PERHAPS.

But one thing I became convinced of while in the hospital and that is,

God is for us.

It may not always seem like it.  Life may be horrible; circumstances may seem hopeless. But God is for us. Seriously, ask me again in a month. If my daughter makes no progress, I will still tell you, God is for us. Because He said He is. And because His goodness is not based on my circumstances. “When I cry unto Thee, then shall mine enemies turn back: this I know; for God is for me.” Psalm 56:9

I once heard a story in which a man lay on his death bed with his family gathered around him. He had once been healthy, but was now dying because of some medical mistake. As the plug was pulled, his wife said to those in the room, “This is by no means a lapse in God’s goodness.”

“It is impossible for any human speech to express the full meaning of this delightful phrase, “God is for me.” He was “for us” before the worlds were made; He was “for us,” or He would not have given His well-beloved son; He was “for us” when He smote the Only-begotten, and laid the full weight of His wrath upon Him— He is “for us,” with all the infinity of His being; with all the omnipotence of His love; with all the infallibility of His wisdom; arrayed in all His divine attributes, He is “for us,”–eternally and immutably “for us”…” – Charles Spurgeon

By the way, Rhema was a superstar, enduring the confinement, the pokes and prods with a smile most of the time.  She only tried to chew the power cables a few times, only bit off 12 marker heads, only tried to stuff foam puzzle pieces up her nose a dozen or so times, only had 5 meltdowns, only got trapped under the hospital bed – and it only took 3 people to get her out – one time. She was AWESOME. 8)

And I know every word to every Dora video now.

For more Beyond Ordinary Blog Carnival stories, go to 5 Minutes For Special Needs.

This Is An Abnormal Study

The time has come again for us to go to Children’s Hospital Boston for an overnight EEG for Rhema. This is a very familiar procedure to many of us in the autism community, but for those who are unfamiliar I wanted to briefly explain what is involved.

A technician measures and marks your head with a red pencil. Then 32 electrodes are glued to your scalp one at a time; gauze is placed over each electrode with some sort of liquid adhesive and then cold air is blown through a small tube over each electrode. Then the head is wrapped in gauze-like material and a meshy cap is placed over the head. The whole process takes about an hour.

It is not a painful procedure, although Rhema always leaves with small burns on her forehead and scalp from the electrodes and adhesive. And for a child with sensory issues, the smell of the glue, the noise of the air tube, the sensation of being held down and having your head and face handled can be frightening and overwhelming. However, Rhema has had so many EEGs and BEAMs (Brain Electrical Analysis Mapping) in the past 2 years that she is getting to be an old pro at handling this. At her last overnight EEG four months ago, she did not even make a peep while the electrodes were being applied.

For 24 hours, Rhema’s brain wave activity is recorded and she is confined to one room. Her room is hooked up with video so that technicians can record anything they see physically and correspond that with what Rhema’s brain is doing. The hardest part is keeping Rhema entertained and distracted from yanking the leads off her head. We try to keep her from jumping off the windowsills or doing back flips off the hospital bed or trying to climb up the metal arm that the TV is attached to. 8)

At Children’s Hospital in Boston, a study that is often performed on children with autism or a regression in language is the BEAM study. The BEAM is essentially an enhanced EEG that uses a lot more electrodes than the standard EEG and also has the ability to “measure” auditory processing responses. The man who developed the BEAM is a doctor at Children’s, and although we have never met him, he has written countless reports on Rhema. The reports are full of medical jargon that our neurologist tries to explain to us, but there is always a conclusion section written in “English” so that the average parent can understand. The first sentence in the conclusion section for Rhema’s BEAM studies have always said: “This is an abnormal study…”

After Rhema’s last BEAM, I was really hoping to get a good report because of medication changes, dietary changes and the introduction of supplements. Instead the conclusion read, “This is a very abnormal study.” After a year and a half on anti-seizure meds, the spiking brain wave activity has only increased. In fact, the report stated that Rhema’s brain was spiking so much that they could not get a normal reading for more than 2 seconds.

A friend explains spiking like this: “Say you’re sitting reading a book and you have a massive seizure that knocks you off your chair. Your whole brain is not functioning due to the seizure and you no longer can read. With “spiking” it’s different. Again say your sitting reading a book, spiking would be someone consistently poking you in the arm taking your focus off your reading because you’re being irritated by the poking.” In Rhema’s case, her BEAM study showed that the brain spiking is occurring 85 – 100% of the time, and it is occurring on both sides of her brain, most strongly in the speech centers of the brain. There has been some speculation that Rhema may have a variant of Landau-Kleffner syndrome as her spiking increases significantly when she is asleep.

Phew. If you’re still with me, thanks for reading. The doctor that created the BEAM study is supposed to be the attending physician while we’re in the hospital, so we’re really hoping for the chance to finally meet him and pick his brain (no pun intended)!

For those interested, here is an interesting article on how EEGs are increasingly being used as a diagnostic tool for autism.