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Once Upon a Dream

We’re currently driving to Disneyland. I can’t help but think about how much has changed since last time we drove to Disneyland.

We came down in early December, having been aware for several months that Reese had epilepsy. We thought it was a “normal” epilepsy and naively weren’t all that worried about it – we were hopeful that with a little tweaking of meds, she’d be fine. Her seizures were myoclonic – blink-and-you-miss-it – so somehow we didn’t consider that while they were very short, they were wreaking havoc on her developing brain.

About a week before we left for Disneyland, we did a routine EEG and a day before we left for Disneyland, we got a call that the neurologist wanted us to come in immediately. I knew that couldn’t be good. I asked if I should cancel our Disneyland trip, but they said coming the day after we returned home would be fine. As I’ve said before, that Disneyland trip had a cloud over it. I didn’t know what was coming, but I knew it wasn’t good. We were committed to enjoying ourselves anyway and Reese loved Disneyland! Or so we thought. In hindsight, her personality at Disneyland (and the months before) was sort of comatose. She would get very serious on the rides – we thought she was taking it all in. She did seem to get a kick out of it all, but it may have also been a bit of stimulation overload for her.

When we returned home, we got the news – Reese’s epilepsy was worse than we had all imagined. I just wanted to be back at Disneyland – my happy place. I’m able to live in the moment at Disneyland. But instead, the rest of our December consisted of weekly doctor visits, starting a high dose of prednisone, having all the hopes imaginable after having our first seizure free day, being crushed when the seizures came back, getting immediately admitted into the hospital for a 10 day stay, starting a much stronger steroid, switching neurologists, stopping all current meds, starting several new meds, and starting the ketogenic diet.

The last few months have been an emotional roller coaster, to say the least.

Since we got the awful news, I kept thinking and dreaming about Disneyland. No matter where Reese’s development was, I felt she’d enjoy the sights, sounds… and smellilizers! I quickly decided that once she was off the medicine that suppressed her immune system (we were effectively quarantined for 3 months), we’d come back to Disneyland. I wanted to take Reese back to my happy place and just live in the moment again. I started counting down the days.

Last time we were headed to Disneyland, I could not have imagined all that would lie ahead for us. Late December when we were in the hospital and things were looking their worst, I could not have imagined that 4 months later, we would have gained seizure freedom by way of the ketogenic diet.

Well… we’re on our way! Packing for Reese’s ketogenic and other medical needs has been a challenge, but I’ve always been up for a good challenge. Especially one where Disneyland was the goal!

At this point, I think it’s safe to say I’m more excited about this trip to Disneyland than the average 6 year old.

A Hard Couple Weeks

If I’m being honest, the last couple weeks have been hard.

Reese has been fighting taking her medicines so every morning and every night have been a struggle.  It’s draining.  I can’t say that I blame her – they have a strong, awful taste that I wouldn’t want to have to take.  We do the best we can, but between the medicines and the supplements, there are just a lot.

Also, since weaning from the ACTH, her appetite has become nearly non-existent.  At this point, I’d be really happy (and surprised) if she ate 2 meals a day.  She didn’t have much of an appetite before the ACTH, but I was hoping that being so hungry while on the steroid would have some lasting effects.

Reese did some neurological testing a few weeks ago.  We knew she has at least a moderate speech and language delay, but we wanted to see where she was in other areas.  In the questionnaire beforehand, it asked Reese’s strengths and one that I wrote was problem solving.  She tucks things under her chin if she wants to carry more when her hands are full and once at a play kitchen, she couldn’t reach the microwave so she popped the sink out, flipped it over on the floor, and used it as a step stool.  Family and friends seemed pretty impressed with her problem solving abilities, so I don’t think I’m the only one who felt it was a strength.

We got the results and Reese has a global developmental delay – she’s around the 10th percentile in every area (including problem solving) except for gross motor.  For gross motor, she’s age appropriate.

It was a punch to the gut.  Something I thought was a strength of hers was actually not a strength at all.

While the seizures were uncontrolled, we would read stories about other children and how it was like someone hit a pause button on development.  Reese was improving (albeit slowly) so we thought this didn’t apply to us.  We realize now that it’s exactly what happened.

Reese had another EEG on Monday and then saw our neurologist on Tuesday.  Thankfully, the EEG was (again!) considered normal.  So now we are going to wean off the Vigabatrin (which was already a low dose) over the next couple weeks and then possibly start weaning off the Onfi.  Lately Reese’s coordination seems a little “drunken sailor” and we suspect it’s the Onfi, but don’t want to wean 2 medicines at once, so we decided to just tolerate her clumsiness for a few more weeks.

I mentioned to our neurologist the results of Reese’s developmental testing.  He was very positive about how far we’ve come and felt that she has time to catch up.  I made some throw away comment like “maybe considering the Lennox Gastaut and where we were in December, we should consider ourselves lucky that we’re even on the curve.”  He quickly agreed, which helped to put things in perspective.

Since getting the results of the testing, I’ve enlisted my sister (who is a Montessori teacher) to help me find new ways to help Reese continue to improve.  What a wonderful resource to have in our family!  I’ve already noticed that Reese’s attention span has improved, so I suppose the positive part of the testing is that it served as a good wakeup call.

I do believe that Reese will catch up a bit (exactly how much, I don’t know).  It just bums me out that she’s having to work twice as hard just to be where all the other kids are.  It feels like we’re farther away from normal than I thought we were.  But that’s okay.  Maybe I should have written that Reese’s strength is her persistence, and that is really an excellent trait to have. 

Glad That’s Over!

Reese amazes me constantly.

Every morning for the past 3 months when it’s time for her injection, she sees the needle and knowing what’s coming, holds her leg out for me.  She never cries.  She doesn’t even flinch.  As soon as it’s over, she says and signs “all done” and goes right back to playing.  Her bravery through everything these past few months has been awe inspiring.

Today was her last dose of ACTH.  We’ll never know exactly how large a role it played in Reese becoming seizure free.  We do know it played a large role in her adorable chubby cheeks.

I’m told that each vial was just shy of $50,000.  Reese went through 11 vials.

We are thankful for so many things these days… high on our list is good medical insurance and great doctors!

The Need for Community

Having a child with epilepsy (or any life changing medical condition, I would imagine) can feel very isolating.  Although I know I’m not the only one living this way, sometimes it feels like it – living EEG to EEG, days on end in the hospital without any advance notice, at times having more doctors appointments planned than social outings, and turning into the helicopter parent I never wanted to be (so guilty!).

This weekend, our entire family participated in a local 5k to raise awareness (and money!) for epilepsy.  It was quite moving to be surrounded by other people who are affected by epilepsy.  Everyone was so positive despite this awful common thread we all share.  It is clear that epilepsy affects the whole family.

I have enjoyed meeting other people in the same boat.  People whose heart stop every time their child flinches.  People who now all too well what to do in the event of a seizure.  And even people who understand the thrill of measuring out exactly 20.00 grams of butter on the first try!

I have long considered starting a local support group, and this weekend has made it more apparent that it’s something I’d like to do.  It’s hard to stay positive 100% of the time.  Really hard.  Feeling a part of a community during a time like this is so, so important.

Stay tuned, I just might do it…

 

2 Months Seizure Free!

These past few months have been unreal.  Was December just a bad dream?  Even still, I hope I never forget it.  I hope I never stop fully appreciating the small moments.  They’re not guaranteed.

Reese has a tricycle and has enjoyed being pushed along on it.  Yesterday, she started pedaling.  I can’t help but think that before all of this, I would have assumed that this was a normal skill for a 2 year old and thought nothing of it.  And really, it is normal.  But when I see Reese happily pedaling along so proudly, it makes my heart happy.

On Monday, Reese had a routine 30 minute EEG and I was feeling pretty good about things.  And then Reese had a questionable twitch.  I had mixed feelings about it – I was glad that if it had to happen, it was caught on the EEG.  But I was very upset that it happened.

I kept reminding myself that regardless of what it was, being seizure free for nearly 2 months is nothing short of amazing.  Unfortunately, it seems my happiness is pretty well linked with Reese’s condition.  So I was pretty distracted for the couple days until our appointment with our neurologist.

Great news – it was just a twitch!  Reese’s EEG is still considered normal!!

While in the hospital in December, our neurologist described Reese’s EEG as “scary.”  Scary to a neurologist means VEERRRYYYY scary to the mother of that child.  Boy, oh, boy… we have come a LONG way in just a few short months!

Life Goes On

We’ve been doing this for a relatively short amount of time, but I’m feeling like we’re really hitting our stride lately.  One thing that has helped us stay focused is how well Reese has responded.  It’s also been helpful that she’s been relatively compliant.  Sometimes she’s not thrilled about eating her butter and that can be rough.  She also sometimes gets hungry and so therefore, cranky.

We were shopping at the mall yesterday and she wasn’t having it.  She didn’t have her cream from breakfast, so I went into Starbucks and ordered myself a drink and since there was no line, I explained our keto situation to the barista.  I was wondering if there was an easy way to order such a small amount (1oz) of heavy cream – if that was even a thing.  It’s not, but the barista was very helpful – she said that I could order my drink and ask for heavy cream on the side and that it would probably be about an ounce.  It was excellent!  And free!  I always have my gram scale, so I measured 30 grams and Reese was quickly back to being a happy camper! 

Since the beginning of this, I have been determined to not let this get in our way of enjoying life.  And today was another reminder that life does go on while keto!

Ending February on a Good Note

It’s been a busy week!  We started with an EEG, then finally had our second opinion with UCSF, and just had a follow up appointment with Dr C this morning.

The 30 minute outpatient EEGs aren’t my favorite.  I certainly like to know what’s going on with Reese’s brain, so they’re well worth it, but Reese is like me – she likes to get a healthy amount of sleep.  Keeping her up until 11pm and then waking her up at 4am is sort of rough on both of us.  Her EEG was fairly uneventful.  She got hooked up and immediately fell asleep (which is what they like).  She had a few slight hand or foot twitches that I wouldn’t think twice about if my son did it, but when Reese does, I always get a little nervous.  I was glad to have it on the EEG so we could see if they were anything noteworthy.

UCSF was another early morning.  During the drive there, Matt and I discussed how much different this doctors appointment would be than we anticipated when we asked for a second opinion all the way back in November.  At that time we were desperate for answers, hoping to find anything that would work, and possibly even interested in hearing about any relevant drug trials.  Now, I guess we just wanted to be assured that we were on the right track. 

At the appointment, we went over all of Reese’s history, the meds she was and is taking, and the improvement we’ve seen in the last month or so (both in terms of seizure control and her development).  The doctor was surprised that the ketogenic diet was working as fast as it did for Reese.  She was almost doubtful – she really hadn’t heard of it working that fast.  She said that usually they’re having to continually encourage parents to stick with the diet for at least 3 months to see possible results.  Our dietician has heard of it working that fast – she’s mentioned that she thought Reese is a super-responder.

The doctor at UCSF felt that if Reese hasn’t had developmental delays yet, she won’t start having them now while she’s seizure free, which was wonderful to hear.

We asked for a diagnosis since we still don’t really have one.  She felt it probably wasn’t infantile spasms, but was possibly Lennox Gastaut or Doose Syndrome, and if she had to guess, she was leaning towards Doose syndrome (mostly since Reese hasn’t had delays aside from her speech, even though Reese hasn’t had any atonic (drop) seizures which is a hallmark of Doose Syndrome).

The thing all of Reese’s neurologists can agree upon is that she’s a unique case and doesn’t fit nicely into any box.

She felt we could start tapering meds and by and large agreed with what Dr C was doing.  We have always had a lot of faith in Dr C, but this really just confirms that we’re in the right place and doing the right thing.  It was an encouraging appointment all around.

While in San Francisco, we went out for lunch on Pier 39 to celebrate and even went to play a couple of games at the arcade at the end of the pier.  It felt like our first normal day in a long, long time (although we were missing Nicholas!).

This morning, we had a follow up with Dr C.  He was very pleased to hear that Reese has gone over 1 month seizure free.  Reese has been doing regular bloodwork and last weeks came back with low potassium levels, which sometimes can happen on ACTH.  Also, her electrolyte levels were low.  Unfortunately, this didn’t surprise me at all – for the past week, she would wake up and be sort of shaky.  She’d drink a few small glasses of water and then be fine.  I found a carb free, sugar free, calorie free electrolyte drink and had just started giving it to her.  The day after the blood draw, the nurse called and requested we repeat the labs.  We did, and that night Dr C called (I maintain he doesn’t sleep!) and we added a potassium supplement since her levels were still low.  We went over that and discussed the continued taper of ACTH and then weaning from the Vigabatrin after that.

When we were in the hospital, Dr C was aggressive with meds and now that Reese is seizure free, he said he wanted to be conservative with tapering off meds.  I hate that Reese is on so many meds, but the thought of tapering off them makes me anxious.  I have gotten the taste of a seizure free life and I don’t want to lose it!  But I know that tapering off meds is in Reese’s best interest – we can’t leave her on 10 meds forever, even at the risk of having another seizure (if we learn that a medicine was more important than we thought, we could always add it back).  He felt that the meds helped, but that the ketogenic diet was doing the heavy lifting, and I agree.  I’m happy to go the conservative route on weaning from the meds.

We discussed how Reese’s speech was coming along and how her receptive language has improved as well.  During our visit, Reese high fived, waved at, and said thank you to Dr C.  He commented that she didn’t do that when he first saw her.  It’s true – she’s improved a lot in the past couple months since her brain has been given a chance to rest.  It’s so nice to have a doctor that sees Reese as a whole child and not only noticing her seizures.

But the absolute best part about the appointment: Dr C said that Reese’s EEG from 2 days ago had normalized.  Wait, what??  Like… normalized, normalized?  I still can’t believe it.

I am not much of a dancer, but I practically danced on out of the doctors office.

The Rest of Us Aren’t Keto

Does that seem harsh?  I’ve cut back on carbs, but I do still eat some in front of Reese.  I don’t draw her attention to it, but I have chosen not to hide it.

When we’d eat at Chipotle, I used to share my burrito with Reese.  She would nicely say “bite, please?” and I would oblige.  Then I’d have a bite, she’d wait her turn, and then she’d say “bite, please?” and I’d give her a bite and so on until she was full.

The first time we went to Chipotle after Reese started the diet, I thought it was going to be tough.  She knew the routine and angelically said, “bite, please?”  This time, I said, “No, this is mommy’s food.  You have your food.”  And that was that.  She didn’t complain.  (This is why I think that somehow she knows she’s feeling better and that the diet has something to do with it.)

My son loves applesauce and Reese used to enjoy it as well.  While Reese gets her food, Nicholas gets applesauce sometimes.

People ask us if we’re planning on going keto as well, and the answer is no.

My husband and I feel that this is just one more way we’re treating Reese as normally as possible.  Many people have different dietary restrictions – for example, Matt is allergic to nuts.  She’s going to be constantly surrounded by foods that she can’t eat and will need to know how to handle it.  We can’t keep her in a bubble, nor would we want to.  

Children adapt.  We probably don’t give them enough credit.

The Problem with Starting Keto on New Years Day

The problem with starting the ketogenic diet on New Years Day is that the ketogenic diet is a really trendy diet for weight loss right now.  It’s practically everyone’s New Years resolution!  And since it’s become so trendy, things are being marketed as “keto” or “keto friendly” everywhere you look.  Often, they’re actually not very keto at all.

Everybody thinks they know what the ketogenic diet is.  Truth be told, I would occasionally make a dinner recipe that had keto in the title and think I knew about the ketogenic diet also.  Often, those recipes are low carb but not actually high fat and fairly high protein.  Outside of websites specifically for epilepsy, I have yet to come across a keto recipe that lists the ratio alongside the nutritional facts.  I’ve since learned that keto for weight loss is vastly different than keto for epilepsy.

I consider myself a pretty live-and-let-live person, so whatever diet a person wants to follow is fine by me.  The trouble I’ve found is that people have heard about keto for weight loss and therefore think they know what Reese can eat.

But even if a recipe were to be a 4:1 ratio, I couldn’t just haul off and give it to Reese.  It would need to be planned and accounted for. 

I love that people want to get involved and offer to make keto recipes for Reese.  To be fair, if this were happening to someone I knew, I’d probably be doing the same exact thing.  But unfortunately, I can’t accept their offer and it bums me out.  I’m just not comfortable with any variation from what I’ve planned.  For me, the downside of straying from the diet is too great.

One night Matt and I decided to have dinner at a casual burger restaurant in our area.  As I was packing Reese’s meal to take with us, I considered getting Reese a burger patty at the restaurant as part of her meal.  Maybe it was a moment of weakness.  Or laziness.  Or perhaps it was wishing for more normalcy.  But was the ground beef 80/20? 85/15? 90/10? Was anything mixed into it (maybe a filler, or vegetables)?  What type of fat was it cooked in?  There were too many variables and I started to get anxious about it.  I have rarely had these moments, but fortunately, Matt was there when I needed him to keep me on track.  He reminded me if I don’t want to do it, just don’t do it.  Pack something from home so that we know exactly what’s in it.  So that’s what we did and the evening was wonderful.