The Return of Atonic Seizures

The last 5 seconds of the video capture the drop attack.

For the past couple of weeks I've been trying to fool myself into thinking that Bertrand was just having some really big myoclonus. Nope. It's clear the drop attacks (atonic seizures) are back.

The last time we saw Bertrand have a drop seizure was in May 2010.  ACTH treatment, while it almost killed him, bought us 2 years of drop freedom.  Now, we're going back in the ring to fight them.

ACTH Course*

June 18, 2010 - Bertrand is currently in the hospital indirectly due to ACTH. He picked up RSV because his immune system was compromised due to the ACTH. Then because of the swelling (also due to ACTH but also due to the decreased GI motility from the ketogenic diet) it turned into pneumonia. Unfortunately all the symptoms were masked because of ACTH -- he can't really run fevers or produce a lot of mucus on ACTH. He may be discharged today with oxygen.

Even through all that, I love ACTH. He can now track me, is back to smiling and is working on "hi" and "bye bye" and most importantly "mama". :) Maybe he could've done that all with SoluMedrol too. Who knows?

Everyone has stressed the importance of taking the full time with the ACTH (or any steroid) course and taper, so we stuck with it in spite of high blood pressure (150/80 for a 2 year-old) and severe mood issues (I just started an anti-depressant because of this). As a 2 year-old, 35 lb., 36 in., male, this was his course:

May 01 - 0.56mL ACTHar gel twice a day (45U total)
**Continue for approx. 3 weeks.**
May 17 - EEG
May 24 - 0.56mL ACTHar gel once a day (23U total)
**Continue for approx. 2 weeks.**
June 07 - EEG
June 07 - 0.27mL ACTHar gel once a day (12U total)
**Continue for approx. 1 week.**
June 14 - 0.27mL ACTHar gel every other day
**Continue for approx. 1 week.**
June 21 or 23 - ? mg Prednisolone twice a day
**Continue for approx. 3 weeks.**
July 12 - EEG

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*October 6, 2011 - I just found this incomplete, un-published post. I'm going to post it, without edits, for posterity's sake. Bertrand's July 12, 2010 EEG was normal. By January 2011, his EEG was severely abnormal again.

Focusing on the POSITIVE: Bertrand's EEG

September 2009

Keppra only.

April 2010

6 months Ketogenic diet (4:1) and keppra.

May 2010

2 weeks ACTH (and ketogenic diet).

June 2010

5 weeks ACTH (and ketogenic diet).

Note: The preceding EEG images were taken during drowsy periods. And, the ketogenic diet is nullified by ACTH since the resulting steroids increase glucose.

Hospitalization update

Bertrand got his morning off to a bang with a large bowel movement. Unfortunately, the circumference of his distended belly grew to 71cm. Since his other extremities are now equally swollen, our concern is that he has generalized edema.

I asked for a physician's opinion on the possibility of heart failure. Heart failure is one cause of generalized edema, and heart failure is a side effect of ACTH. I felt that with his elevated heart rate, this had to be ruled out. After an examination, the cardiologist here thought his swelling was related to his pulmonary infection rather than heart failure. A veteran medical tech here also concurred that his symptoms are more aligned with pneumonia than a heart condition.

We pulled about 650 mL of fluid and gas out of his nose through a gastric suction tube. The upside of this is that Bertrand regained enough motor control of his right hand to scratch at the tube. (He hasn't displayed that much motor control since 8 months old!)

As the day went on, Bertrand seemed to regain more strength, and he can now stay just inside the safe blood-oxygen range without external oxygen support! (Unfortunately, he's doing this by breathing and pumping blood twice as fast.) His respiration is still "chunky" but it's clearly less so than yesterday.

But, he won't be going home until we track down the source of the fluid retention. Even though the staff told me they'd seen children twice as swollen as Bertrand, I couldn't help but wince when I touched his taught skin. It just looks painful.

Ironically, if I could choose to have the Bertrand I have today or the Bertrand I had two weeks ago, I might still choose the one I have today. Surface maladies aside, Bertrand has been more emotionally and socially connected these past few days than ever before. When I smile and laugh, he smiles and laughs. When I rub his head for comfort, it soothes him. When I walk across the room, he tracks me.

We're hoping that once we get Bertrand all fixed, that we'll get to keep all of these sudden and unexpected developmental advances.

EEG Improvement on ACTH

Bertrand's neurologist was shocked by the improvement shown on Bertrand's EEG today. It took until midway through the EEG to see the first spike. He had no clinical seizures during the EEG.

This was all to my surprise because Bertrand has been so miserable the past month (and in particular the past two weeks), it has been hard for us to see any improvement at all. If anything, we thought he had gotten worse because he is no longer sitting unsupported or playing with toys. He rarely smiles and no longer laughs. He just sleeps or moans or does both simultaneously.

I walked into the appointment ready to give up on ACTH and steroids, but with the news about the EEG improvement I resolved to give them a bit longer: 3.5 months. Yes, Bertrand will be on ACTH for 2 more weeks and then on prednisilone (a steroid) for 3 months.

Here are some other highlights:

1. ACTH and steroid titer schedule set (ACTH 1/2 dose daily for 1 week, then ACTH 1/2 dose every other day for 1 week, then Prednisilone 1.0mL twice a day).
2. Zantac 1.5mL twice a day in addition to current Prevacid 15mg.
3. See pediatrician ASAP to check for gastric ulcer. (Appointment Wednesday.)
4. Expect call from dietitian about weaning off the ketogenic diet.
5. Bertrand must lose about 2kg.
6. Schedule EEG and neurology in August to discuss additional medication, etc.

State of the Pookie Report: May 2010

I've been feeling ground to a pulp (this may have to do with being completely black and blue from a few days ago when my knee gave out at the top of a flight of concrete steps causing me to tumble all the way down them--ouch), but I owe you all a long overdue State of the Pookie Report. Without further ado, here it is.

May 17th

Bertrand's EEG was "much improved". As you can see from the photo below, there were still plenty of (smaller) spikes but most of the underlying wave pattern disappeared. This is great news! However, most kids on ACTH have normalized EEGs after two weeks of treatment, so Bertrand's neurologist believes that a normal EEG may not be in Bertrand's future. We'll see.

May 24th

Bertrand's ACTH wean began. He went from two shots a day of 45 units (90 units total), to just one 45 unit shot of the ACTHar gel in the mornings. He literally got back in the saddle, as his 'roid rage was sufficiently lowered to attend hippotherapy for the first time since starting ACTH. It was his best session ever--with him complaining whenever the horse would stop. :)

May 25th

Bertrand's ophthalmology appointment was attended by his representative/therapist from Utah Schools for the Deaf and Blind. Bertrand has cortical visual impairment (CVI) and is legally blind. This was documented for the school district so it can be addressed at his IEP meeting on July 7th. Later that day, Bertrand picked up his totally rad AFOs, and he now needs larger shoes to fit over them. Time for new shoe shopping! Yay! (I LOVE shopping for baby shoes. They're so darn cute!)

A portrait of Bertrand taken by Jessie Lynne.

May 26th

Bertrand had a great occupational therapy session. He has become much more willing to bear weight on his arms and let things touch his hands. We're finally being able to fully enjoy many of his touch-and-feel books. This is a huge, new plus. Unfortunately, we're seeing myoclonus and tonic seizures in his sleep. Hopefully, these will go away with continued ACTH, as we have not seen a seizure during his waking hours since... MAY 5TH! In equally awesome news, Bertrand's 'roid rage is toning down and he is finally starting to eat less. Whew!

June 2nd

Even though his immune system will still be technically suppressed, Bertrand will be re-starting his preschool. He will be attending Mondays AND Wednesdays from 8-11 to make up for the month he missed in May. This will be a good test for his tolerance of increased school time and of the changes/progress he has made since ACTH treatment.

Another portrait of Bertrand taken by Jessie Lynne.

June 5th

Matthew will leave on a marathon of conferences and family activities through the end of June. In an effort to save my sanity, Bertrand's Abuelito (my dad) and his Auntie Aury (my sister) are coming to visit and arriving the day Matthew leaves. Abuelito will be staying for a week but Auntie A will be staying through July 5th! I am so excited! While she's here, Aury will be attending the University of Utah's School of Computing Entertainment Arts and Engineering Summer Program! I wish I could attend the program--it sounds awesome! :)

June 7th

Bertrand's follow-up EEG and neurology appointment should be interesting. At this point he'll be two weeks into the ACTH wean. Bertrand's Abuelito, who is a neurologist, will be attending the appointments with us. (Hey, Daddy? FYI, you're attending the EEG and neurology appointment with us. Love you! Thanks!) At the neurology follow-up we'll be discussing the ACTH wean, the prednisone/prednisilone titer and the possible addition of Zonegran.

"The Boy Who Discovered His Lungs"

There once was a boy named Bertrand
With no fine motor or tears from his tear gland
Then along came steroids
And peace was destroyed
Tears and yells came hand in hand

Yes people, I am finally losing my mind. I want to believe (and so I will) that there are finally some positive changes coming from Bertrand's ACTH treatment. We haven't seen a myoclonic, tonic, or atonic seizure in 4 days--this tops his 3 day "seizure free" record on the ketogenic diet. And as for the seizures we don't count, his eye myoclonus and absence seizures seem greatly reduced. That said, apparently Tarzan is Bertrand's real father. This is the only explanation I can come up with for the unnatural sounds and volume coming out of my once soft-spoken little boy. Oh, and a wolverine is apparently Bertrand's real mother, but more on that some other day. :)

The Blood Pressure Saga Continues...

Bertrand on a walk with his great-aunt Shirley today!

Bertrand's pediatrician appointment was interesting yesterday. His blood pressure kept reading as very high in the office. To give you an idea, Bertrand's blood pressure was 152/82 and the high range for his age, height and weight is 105/65-75. Yeeeaaah.

This blood pressure was taken while he was sleeping, but having a lot of jerky movements, which can distort the reading. So we were given two days to get sleeping blood pressures for Bertrand before putting him on blood pressure medication and/or reducing the ACTH.

Guess what we got last night?

113/87! We were pretty excited by how low this was! This was taken on his ankle, and any leg blood pressure measurement is about 10 points higher than that from the inner elbow. Correcting for this would put the values at 103/77. Not bad, huh? :)

All in all, Bertrand is still very irritable and hungry, but his great-aunt Shirley and aunt Jess helped to distract him by going on walks... in the snow. Yes, it was snowing in May! (But fortunately it didn't accumulate.)

Jess and Shirley are driving across the country so Jess can start her nursing program at Johns Hopkins! We are all so proud of her! She unpacked her stethoscope just to take Bertrand's blood pressure. :) And, Shirley gave Bertrand multiple walks and single handedly finished curtains for our bedroom while B was napping! The energy of these two ladies is amazing! They need to drive through Utah more often. :)

ACTH Day Four

This is Bertrand's weary Mama reporting from the front lines.

Bertrand is doing better tonight! He had his first NORMAL BLOOD PRESSURE and PULSE! YAY! We thought he was going to have to go on blood pressure medication starting tomorrow, but he may have beat that. We increased his water and decreased his salt. Also, while he was a little monster today, I would say he didn't seem possessed, which is progress, right? :)

What else? His ketones are down to moderate. We saw tears again today--I always consider tears a good sign. :) And he's had only one or two seizures today, which isn't good but isn't bad. He's still acting alternately fussy and exhausted, but hey, his body is working hard. We'll see what his pediatrician says about all these things tomorrow.

Overall, I am not as frantically worried today as I was the past few days. (Mind you, still very worried, just not as much.) I think we'll be able to stay the course for the next 2 to 3 weeks. Also, looking at kids on ACTH with non-infantile spasms, the treatment can take weeks to see results, so that I was happy to hear that too. ACTH still has time to work on Bertrand.

Look at what I found today! Bertrand's baby announcement!

Look at that adorable baby chub!

Update: Irritable

It's a daddy post tonight because Bertrand defeated Cristina today.

ACTH-powered Bertrand is not something we were really prepared for.

He's almost constantly wimpering or crying. Holding him no longer comforts him completely, but it clearly helps him. He breaks into what would be tears if you try to put him down. The usual remedies like his favorite books and toys have little effect. I could only get him to sleep tonight by leaving his iPanda playing low-volume music.

I feel like I'm constantly searching for something else to comfort him, knowing that it's probably not going to make him happy either. I think Cristina feels the same way. Parents like to be able to comfort their child, but we can't ever seem to provide him much relief. This ineffectualness gnaws away at us.

He's hungry and thirsty all the time, but he refuses to drink water. (He's never liked it; I think it's a texture issue.)

So, to keep him from ballooning up, we've started mixing his Ketovolve with "thickened" water (which looks and feels like snot) and we're feeding him as frequently as he wants. It's about the only thing that's bringing him any peace right now.

The injections themselves are tough on all parties.

The needle is really tiny, but I guess that's because if we used a big needle, he'd have holes all over his legs. The problem is that we're injecting a cold gel, and it takes what feels like an eternity to inject it all. Meanwhile, we have to hold down an extremely pissed off Bertrand and keep him still.

I've already hit bone, bent the needle and had it knocked out twice.

But, I do think I'm getting better at it.

Tomorrow is ACTH Day 4.

Bertrand's seizure count today was pretty low compared to yesterday. Apparently, half of all ACTH patients go seizure free by day 4.

Our fingers our crossed.

24 hours of ACTH and a bit discouraged

The happiest day in my life. I didn't know then that it would lead to some of the saddest days in my life.

At 3:30PM yesterday, Bertrand began a course of ACTH gel injections. He has received three injections so far. He has had lot of seizures today--more than all of last week's seizures combined. It is a bit discouraging. We know that because he is still producing large ketones and his glucose still reads as low (consistent with a 4:1 ketogenic diet), perhaps we are not seeing the full effect of the 90 units of daily ACTHar gel. I can only hope that over the next few weeks (preferably days) we'll see the improvements that ACTH is famous for and not so much of the side effects that it is infamous for. Fingers crossed.