Feeling Thankful

A few days ago, with Zane’s 2nd birthday nearing, I started feeling the need to print some of our pictures to put in Zane’s baby book.   Looking through all those memories triggered a letter to our neurologist, and I thought I would share it here as well.

Dr. Von Allmen,

Tomorrow, Zane turns two. I can’t even believe it. Two! Our daughter is ten, so I know how fast time flies. But, it’s different this time. As I try to put pictures in his baby book, I feel so much sadness. Not only has time flown by, but this time we were robbed of so many precious memories. We missed out on so many smiles, so many giggles, so many splashes in the tub. We missed out on spaghetti sauce faces and dropped toys just to see how gravity worked. Instead of cute videos of silly moments, we have videos of seizures for documentation. Instead of pictures of new milestones, we have pictures of EEG’s in the hospital. We missed out on so many silly little things that most parents take for granted.

When he should have been gaining new milestones each day, we watched the milestones he had already achieved fade until he had none. None. At eight months old, he was like a newborn. But, his body had grown, so taking him up and down the stairs or giving him a bath was like holding a twenty-pound limp noodle. Our sweet baby boy, once healthy and thriving, could not even hold up his own head. He was expressionless, and nearly motionless, except for spasm after spasm. He was gone. Our son was gone.

After having several experiences with different teams of neurologists (no less than 6), plus countless residents, at a well known hospital, you would think that someone would have gotten the diagnosis correct. Or, at the very least, that one of them would have scheduled a longer EEG. You would think that one of them would have listened to us about how the seizures were changing. You would think that one of them would have watched our short video clip. But up until then, we weren’t so lucky.

But, then we found you. You didn’t even know us yet, but you took a quick moment to watch a video clip that had been emailed to a nurse’s phone and handed to you by a resident. (Our first neurologist wouldn’t even watch the video during our scheduled appointment). You had us in within two days for Zane’s first 23-hour EEG. Sadly, it all confirmed my fear that it was, in fact, Infantile Spasms. But, you moved quickly to get us in, get it diagnosed and start an appropriate treatment. Zane’s path wasn’t an easy one, but thankfully, we found our miracle. (I never would have thought I could have been so happy to give my own infant shots in the leg each day for months, at thousands of dollars each. But, once it started to work, I was)!

Within a few weeks, we started to see his smile come back, then his giggle. We started to see milestones he had lost return. Then we saw new ones. Now we have have cute videos of laughter and pictures of that spaghetti stained face. With lots of therapy, we helped him learn to crawl, learn to eat, and learn to walk. Now he’s jumping, dancing climbing and nearly running. He is becoming quite the toddler, testing limits with a smirk on his face and a twinkle in his eye.

I know from online forums that many babies with I.S. don’t have the same outcome. So, I do know that some part of this may be luck. Yes, Zane is lucky. But, part of his luck was finding you to be his neurologist.

So, from the bottom of my heart, I thank you. Thank you for listening to us, thank you for being kind and most of all, thank you for helping our son. He would not be where he is today without you and your wonderful team.

With much appreciation,
Holly Guillaume

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Concerned… but…

If you remember, there were a few days that Z was previously inconsolable on ACTH.  But, then, it was as if the medicine miraculously did what it needed to do, and then he was fine again. He started smiling, interacting, gaining milestones.  It was amazing.

Then comes yesterday, the day I had been dreading: the wean.  Z only went down 10 units on his meds, so I was nervous but at the same time, not really expecting anything to happen at this point.  But last night he was up a lot, inconsolable at times.  I couldn’t help but worry that somehow whatever had been done at the high dose was coming undone. No one seems to really understand how or why the medicine works, so I don’t know if that could even happen like that.  But it’s where your mind goes when you’re deliriously tired and afraid.

But then we both finally got a little sleep and are both feeling better.  And then…
Z rolled over from back to front for the first time ever.  And then… he did it a second time.  Yay!!!!!!!!!! Go baby Z!  It seems like such a small thing to most.  But, to me, it’s huge.  HUGE.  It brings tears to my eyes.  (Well, to be honest, it brings tears streaming down my face.) I so totally needed to see that this morning! 😉

Baby Z is an AMAZING boy!  I love that little guy!

Making Progress

We had a visit with our neuro today, and then had another EEG.  Unfortunately they had to schedule the visit before the EEG, so I haven’t gotten confirmation that the EEG is still “normal”.  But, his progress leads me to believe it is.

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The APRN (advanced practice registered nurse) who is very knowledgeable with IS, saw Z and was telling the resident how great baby Z was progressing.  He said, “you should have seen him a month ago before starting the ACTH.  He was more like a 1 or 2 month old.”  Sad, but true.  But, now, look at him, holding up his head.  They mentioned he’s probably at a 4 month old’s level, which is a lot of progress for a little guy to make in 4 weeks.  I know he’s got a long way to go, but he’s just doing so well!  I know it’s strange to be celebrating an 8 month old holding up his head, or smiling, but that just shows you how bad it had gotten.  😦

The resident was from the adult neuro dept, but was on a pedi rotation, so wasn’t very familiar with babies and IS.  You could tell he didn’t quite know what questions to ask or how to interact with little ones.  But, in talking to him, he realized he knew more than he thought he knew about baby Z.  He is a friend of the resident who first saw the video I just happened on a whim to send to my friend who is the adult neuro nurse, and he saw the video when she had shown the attending (who is now our wonderful neuro).

 

We started the weaning process from ACTH this afternoon.  So, I just can’t help but do everything I can to help/hope that the meds did what they needed to do permanently.  I feel like I’m crossing my fingers and toes, knocking on wood, and of course, praying like mad.  He has hardly had any side effects, so I wish he could stay on it forever.  But, unfortunately that’s not how it works.

 

I asked some other moms what happened after they weaned their little ones off ACTH, and these are some of the responses I got:

“My sons returned 2 weeks after wean. Our neuro chose to try Vigabatrin next instead of repeating ACTH. We still don’t have control at max dose, just a reduction. Checking into candidacy for surgery and may add oral steroids to see if it will knock them out. Going for our weekly EEG in the morning to see what we’re going to do.”

“Atticus started ACTH June 15. By June 26 we could no longer visibly see any spasms. Sept 3 was his last injection. Sept 10 his EEG came back normal. I am happy to report that Atticus is still spasm FREE!!!!’ That’s over 4 months now. Don’t give up hope. When one door closes, another always opens. Praying ACTH worked for you xo”

“My son took 30 units twice a day for 2 weeks, then tapered to 20, 10, 10 once a day, 10 every other day. The whole thing took 4 weeks. He was seizure free after the first dose. He’s been off it for 1 month now and has a normal EEG. An EEG during the taper showed no hyps but did contain a slow wave- that’s now normalized. It seems like there’s many different dosing schedules that all are considered potentially helpful. How are your baby’s brain waves right now?”

“My daughter was on ACTH and was seizure free for a little while but they came back and we tried the diet and Meds was seizure free again. But they came back and the Nero noticed that they are just on one side so we did the surgery was seizure free for over a year but came back and did the diet again seizure free but they came back so now we r on 4 different Meds talking about the diet again”

“We did ACTH for one month and are now on prednisone for 3 months and weaning. EEG was normal after one month of ACTH and we go back for an 8 hour EEG nov 19th. If there are any spikes we are not trying ACTH again and moving to sabril.”

“My son, Tripp was diagnosed with IS at 4 months. We are in our fourth week with the ACTH injections and have seen no more signs of the IS. Unfortunately, he has started having other seizures, we have seen three different kinds. They told us Wednesday that is was Epileptic Encephalopathy and his prognosis was not good. He also has a diagnosis of Optic Nerve Hypoplasia, but they were unable to find a symptomatic cause for the IS. Has anyone else been diagnosed with other seizure disorders?”

“Update on Billy.. seizure free for 4 months, started up again suddenly 5 weeks ago, keppra been upped & upped & upped, hes on his maximum dose for his weight/age now and he still having clusters throughout the day. I dont know why ? :(”

“Have any of you had your child’s IS start really early? My son was a little shy of turning 3 months when diagnosed, so hasn’t really met any developmental milestones…not even holding his head up and he’s over 6 months now. He doesn’t interact with me, smile, or even move a whole lot. Still trying to get control of the spasms with Vigabatrin and finally got an admit date for the keto diet on Oct. 29th. I know he’ll be delayed and I can deal with that, its just that I feel like I’ve lost my baby completely. For him to not even look at me much, show recognition of me in his face, or smile at me tears my heart to pieces daily. Anyone else’s child been like this?”

Then, other moms have come back years later with posts/pictures like these:

“I so clearly remember diagnosis day and being told my baby girl would never walk……she was running, laughing and having so much fun looking for leaves. Never give up hope, our babies are so strong!”

“5 yrs old healthy as a horse my little miracle man:) keep the faith moms n dads-you will get through this! Love and prayers to all of you!”

“My little boy was diagnosed with IS in April this year and to date is doing well! He is 6 months seizure free after success with ACTH! I just wondered is anyone else’s little ones very restless sleepers with IS! We’re getting up to him a few times each night! He wakes himself up wriggling around the cot to the point his pj bottoms came off in his sleeping bag and then he squeals the house down!! Maybe he’s just a terrible sleeper but just curious!!”

“Hi, just a positive story to share, my son (currently 3years 3months old) was diagnosed with infantile spasms at 7months old, got treated with two courses of ACTH Injections, then onto Epilem, and then onto Lamotrigine, Joel has been SEIZURE FREE as two years now, and is currently going thro the ‘weaning’ process to bring him OFF the medication, there is always the risk they may come back, but we can only think positive, he has been left with some brain damage, which has led to learning difficulties and global development delay and current screening for the ASD spectrum, but he’s a happy little boy who is out mixing with ‘normal’ kids at nursery and hopefully he will continue to thrive and grow!! Never give up..there is ALWAYS a chance the child can ‘outgrow’ it!! The picture is Joel now with his big sister ♥ so proud of him!!”

“A year ago today my son was diagnosed with IS. After a long 4 1/2 months we got the seizures stopped and haven’t seen on since. 7 1/2 months later I’m thankful for doing the research I did to find the wonderful doctor in Detroit that got my sons IS to stop when two other doctors couldn’t. I also am thankful for God pointing me in that directions even if it was an 18 hour drive!”

“Me again sorry!! We got results from zacks MRI the other day and whilst he has been having these spasms/ seizures for over 9 months now, he has absolutely NO brain damage 🙂 just thought I’d give some hope to anyone in particular worrying about brain damage. Not to say it can’t happen, but I’m over the moon as I thought for sure with his seizures there would be damage.”

“Michael has been seizure free for over two months now and we are so thankful but also so nervous! Does anyone have any idea of milestones we could be looking forward to in terms of not relapsing. Like how long do they have to go before they are in the clear or where the statistic drops lower… Thank you all for the support!!”

“For those of you who haven’t experienced the “new baby” I just want to let everyone know that it happened over night for us. The last two days we have a “new baby” I’ve never seen him happier in my life!!!! Before this he showed ZERO happiness, no emotion, except for crying, and he’s doing so much more than ever and his progress is 100%. PLEASE STAY STRONG, GUYS! It’ll happen! Pray pray pray!!! I’m praying for all of us in this group.”

 

 

I feel like it’s all over the map.  Some babies relapse.  Some don’t.  Some go on to have other types of seizures.  Some don’t.  Some go on to lead completely normal lives.  Some don’t.  There’s really no telling.  So, I’m praying like mad because I don’t know what else to do.

 

Roller coaster continues

Unfortunately, we’re not out of the woods yet.  Our roller coaster continues.

Baby Z had some spasms around midnight last night.  Then more around 9 a.m. this morning.  I talked to the neurologist’s nurse, who talked to the neurologist, and they don’t seem overly concerned.  I’m just supposed to watch him and if he has any more over the coming days, we’ll up his dose.  So, we have a plan.  It’s just that it’s hard to see.  I want him to get his miracle!

The rest of the day was good though.  He got to eat real food (I think I forgot to mention they took him off the ketogenic diet for now).  He was a lot more playful, did better at tummy time, rolled over, and was just bright eyed.  So, it’s definitely doing something, because he hasn’t been this active and playful for a while now, and that was amazing to see!

1st dose of ACTH

Baby Z had his first dose today, after having spasms this morning.  This time, the nurse walked me through it, but she actually gave the shot.  Poor baby Z… he is so used to getting poked now that he hardly even cries.  Breaks my heart that he is used to it.  Breaks my heart when he looks into my eyes wanting me, his mom, to help him, but I’m the one who is always there when he gets poked.  I sure hope he understands we are desperately trying to help him.

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About 30-45 minutes after the first dose, he had another episode of about 10 spasms.  But, it was too early for the ACTH to have taken effect.  Still… hard to see when you are desperate for a miracle.  The fellow came in and was commenting that everyone was curious to watch and see if the EEG showed any changes.

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5th Hospital Admission in 3 months

Here we are, back in the hospital, for the 5th admission in 3 months.

After some pretty crappy experiences, I know neither of us was looking forward to it.  But, I was excited to final get baby Z on ACTH.  It was actually the treatment I wanted first, but after seeing the whole fiasco with getting the stuff, I don’t think the insurance company would have approved it back then, even if we had tried.  I’m not very excited about having to give him shots myself, but I am hopeful it will help him.

First off, baby Z had a repeat swallow test.  He is still aspirating regular liquid without coughing.  So, they want to continue to thicken his bottles to nectar consistency.  But, I did confirm with that doc that it is a swallow issue, not a reflux
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