Checkup… and weaning OFF another medication!

It has been a glorious 2 months since our last neuro appointment.  It’s the longest we’ve gone without seeing a doc or going to the hospital since this all began late last June, which is a very, very, very good thing!  We didn’t even have to do an EEG this time since we haven’t seen any seizure activity.

Z was highly entertaining in the doctor’s office.  I was going to say he was in rare form, but that’s not true these days.  He was up and down, wiggling and giggling, and had an absolute ball crinkling the paper on the bed.  After a long wait in the room, and discussions with the resident, he was even still awake and going strong when Dr. V. came in.  That was fun, because so often by the time she gets in the room, he has long since crashed and gone to sleep.  She talked to him, held him, and seemed excited about his progress, as are we!

She even decided to move forward weaning him off his last IS medication… the Sabril.  He is officially on only one anti-epileptic drug now, which is a huge feat considering at one point he was on about 7 different mediations and treatments at once for the IS and it’s related symptoms.  He did have some small focal seizures during his last EEG, so he will remain on topiramate indefinitely… well, until he is 2 years seizure free on the medicine.

He will go back to the hospital for a 23-hour EEG in a couple of months, just to check everything out.  Seems weird that it’s a 23-hour EEG not 24-hours, right?  That’s just a loophole with insurance because of how they charge for a full day (ie. 24  hours).

Anyway… things are going well for baby Z at this point.  He’s still behind, and even our extended family doesn’t seem to quite comprehend that, judging from some of the questions and comments I’ve seen in email or on fb, etc.  It’s hard, and getting harder as he grows, to explain to strangers in a relatively short manner why he won’t hold his own bottle or why he’s not crawling or walking, etc.  But, none of that even matters to us after all he’s been through.  We’re just so happy to be able to look in his eyes and see a smile on his face!

 

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♥ our doc!

I decided to email our neurologist with a couple smiley pics and the video of baby Z rolling over.  I didn’t want to bother her but I wanted to share some good news.

A few minutes later (on a Saturday morning), I got a short but sweet response that confirms we have the right doc!  I love that I can call or email her when we have questions or problems, but it means a lot that she responds to a quick video of my 8 month old rolling over for the first time, and in all caps with 3 exclamation points, and then thanks me for sharing.  🙂

YAY ZANE!!!

Thanks for sharing!

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.

 

On the phone all day…

I was on the phone for a big chunk of the day and I found out that there were a lot of “hiccups”, as one person said.

When we were going to do ACTH a few weeks ago, and then switched to the ketogenic diet, I was told that Z was already approved for ACTH  and that all the docs had to do was call and remove the hold.  Unfortunately, that wasn’t exactly the case. The insurance company had NOT approved the treatment.  Because of the extremely high cost of the treatment, it wasn’t something my case manager or even her boss could just approve.  It had to go to the board, and it had to be studied by their doc to see if there were any other treatments that could be done instead.  But, we’ve tried everything else, and this is the best drug out there for this condition.  So, there was no way for them to tell us to try anything else first.  Thankfully, they did approve it, and thankfully we have already met our out of pocket maximum, because it is crazy expensive.  Like, more than our first house kind of expensive.

They had also tried to get a hold of the neurologist or someone in the office.  But, the only number they had was for the nurse who happens to work part-time right now because she’s studying for her tests.  The insurance company didn’t have any other numbers, and for some reason, they wouldn’t call me.  (That irritates me, because I had other ways to get in contact with the doc.)  But, our case manager wasn’t working on Friday and it had gotten passed on to her boss.

Once the insurance talked to the neurologist, they approved it to go to the pharmacy.  But, then the pharmacy needed to contact the neurologist.  Basically, there was just a lot of paperwork that needed to get filled out and certain critical people were out of the office and it was just a series of “hiccups”.

But, it did get approved!

Then the pharmacy called to arrange a ship time, and they wanted it to arrive on Thursday.  But, our admission got moved to Wednesday.  That’s when I had to bring out momma bear.  I mean, really, were they trying to save the cost of overnight shipping on a medication that was that expensive?  The hospital can’t just necessarily get us in to start treatments like this whenever we want.  But, they had an opening on Wednesday, and I didn’t want to wait another day or two… or four or five till the next week, just to save the pharmacy from overnight shipping.  Ugh, NO.  So, I was getting pretty firm, but remembered the old adage about catching more bees with honey… and I stopped myself and explained how old baby Z is, his condition, and how every day he doesn’t have treatment is another day of regression.  Then the pharmacy guy quickly approved overnight shipping so we could have the meds on Tuesday, and still make our Wednesday admission.  Phew!

 

Update on Ketogenic Diet

Baby Z was still having some slight seizures on the 3:1 ratio, so we went up to the 3.5:1 ratio.  Unfortunately, over a day or two, he became lethargic and his ketones were too high (meaning his blood sugar was too low).  So, we had to give him a tiny bit of apple juice, and went back to the 3:1 ratio.
I do think the diet helped, but it didn’t resolve the spasms completely.  So, we decided to proceed to ACTH.  We can always go back to the diet, but we need to get rid of the spasms (and hypsarrhythmia on EEG) completely if possible, and the ACTH is our best chance.

3rd day of Keto Diet

Once again, we started what we thought was going to be an easy day on the diet.  Here’s my little guy this morning, just wanting to be held and cuddled.

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Then, his EEG probes were removed.

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Unfortunately, it wasn’t that easy.  Instead, it looked like one of the probes had a sore.  It looked like it has blistered and broke open. Poor little guy.  No wonder he was in a crabby mood yesterday.  His poor little head was probably hurting and he couldn’t tell us.  I feel terrible that he was hurting and we didn’t even know.  Ugh.

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That morning, he started breathing strangely again. He had actually started that way back when we started the Sabril, and I don’t know if it was related to  the new drug or not, but it was literally 12 hours after starting the new med.  Back then, I had talked to our neuro about it, who did not think it was related, so she recommended I take baby Z to a pediatrician in case he was getting a cold.  The pedi could hear it and was concerned, so she did a throat x-ray.  The radiologist said that the x-ray showed either an infection or a developmental issue.  The pedi used a tongue depressor and flashlight to look down his throat but could not see an infection, so she referred us out to an ENT.  I called several ENT’s on the list our pedi had given us, but the earliest of them had a 2-3 week wait.  Except for one who could get us in a couple days later.  So, even though he didn’t specialize in kids (he saw kids, but didn’t specialize in them), I took the appointment.  But, he really didn’t do much.  Of course, during that appointment, baby Z wasn’t having any issues.  So, he used a tongue depressor, but basically said it wasn’t anything and sent us on our way.  Since then, it would come and go, but we decided not to stress over it since he had been checked.  But, when it started happening while we were in the hospital, I thought we might as well have it checked again.  I talked about it to a resident, who listened to his chest and determined that he did not have a crackle (which would indicate pneumonia).  She said that it’s not uncommon for babies to have soft cartilage in his throat, which can make it collapse.  But, his pulsox was fine, so it wasn’t causing any problems.  That’s basically what the pedi and ENT had determined a month or so ago. So, I considered the issue was dropped.

The dietitian and the neurologist’s assistant came by to talk and answer some questions.  They were really great, and answered everything I asked.  They were really, really great.  But, in trying to give me the information I needed, they started to overwhelm me.  They started telling me about all the negative things that could {likely} happen in the future.  I have already read all that stuff, but it’s different reading it about someone else’s child, and hearing it about your own child.  They explained that babies with this condition usually go on to have other kinds of seizures.  A lot of times they develop psychological and behavioral problems, may have developmental delay, could be more prone to autism.  I guess I just keep trying to be positive and hopeful that we are helping him.  Right now it’s easy, because he’s a baby and so far isn’t that far behind.  I mean, babies lay around and because he looks healthy, there’s no real indication that there’s a problem.  But, as he grows and it’s more obvious that he should be sitting/crawing/standing/walking, it’s going to become more difficult to pretend everything is okay.  They also told me that infantile spasms will eventually go away on their own, with no medication or treatment.  But, it’s not good when that happens… as in REALLY not good, because those kids have a lot of really bad problems.  So, it’s a good thing that we are doing all we can so early on.  They did mention that they were impressed at the research I’ve done and how much I know and understand, and they said that will serve baby Z well.  They said that some parents just can’t make a decision, get overwhelmed and don’t end up doing anything.  But, what they don’t know is that’s what I feel like doing.  I feel like curling into a ball in bed and crying. But, I don’t have time for that because he’s my baby and I have to do what I can for him.  Anyway, it was a rough conversation.

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A little while after they left, the nurse came in and told me not to feed him any more and that he was going for a swallow study.  The nurse has a french accent and I had to clarify, but sure enough, I heard him right.  Basically, the nutritionist and neurologist decided to have the swallow study done to make sure baby Z wasn’t going to aspirate his new formula.  Because the new ketogenic formula is so high in fat, it can be more problematic if aspirated into the lungs.  So, we held off on the feedings, and 2 escorts came to help us get to the right place for the swallow study.  When we arrived, they had just taken another patient into the room, so we had to wait.  It wouldn’t have been a problem, except my little guy was getting really hungry.  So he waited a couple of minutes, but then let loose and started screaming for food.  I was trying to calm him, but nothing was helping.  When they finally got us into the room for the test, they were trying to explain the test to me over a screaming baby.  I tried to get the gist of it and they let me slip on a shield so I could stay and watch.  If it wasn’t *my* child, it would have been really interesting.  But, seeing your child’s insides on a computer screen is just weird and wrong.  They started feeding him the {glucose free} barium, and he was starving, so he gladly took it.  Clear as day you could see it on the monitor going down his throat.  Until it didn’t.  Yup.  He aspirated… and he *didn’t* cough.  That’s a problem.  Then they took that consistency of barium away, and gave him the next consistency, which they called “nectar”.  It was just a little thicker, and baby Z took that one just fine with no aspirating.  Phew.  But, they only gave him enough to make sure he wasn’t aspirating, and then the test was done.  I picked up baby Z and was walking out of the room and the doctor told me she would call and talk to our dietitian.  As soon as we walked out of the testing room, they closed the doors behind me, and the doctor and everyone somehow disappeared.  I was left with a hungry, crying baby, and had absolutely no idea where I was.  It was kinda like a horror movie, with these long, nondescript hospital hallways.  I started walking and tried my best to remember how I got there, but I wan’t doing very well.  Baby Z just kept getting louder and louder, and I kept getting more and more emotional.  I thought that we were coming just for a basic watch as he started a new formula.  Easy-peasy, right?  But, nothing about this has been easy.

Someone pointed me in the right direction and I eventually made it back.  But, by then Z was crying and I was sobbing and we were a freaking mess. When we got back to the room, I pulled the curtain, fed my baby, and just sobbed and sobbed.  Then I heard the camera turn so it could see us, and I soooo wanted to rip the camera off the wall.  It is so unnerving to be watched all the time, and especially when you need a moment to pull yourself together.  UUUGGGGHHHH.

Then, unknowingly, the neuro resident came in to talk to us and I was still sobbing, and a mess.  I had to ask her to give me a minute.  When she came back in, she told me baby Z was now going to have a chest x-ray to make sure he didn’t have anything in his lungs.  But, the x-ray came to our room, so at least we didn’t have to get lost in the hospital.  Then the dietitian came to our room and explained that the thickener they used to have is no longer available so she needed to find another option and would have to re-balance the formula to account for the new carbs in the thickener.  But, I told her she needed to bring us something for the night so baby Z could eat, wouldn’t aspirate, and then she could finalize the new balance tomorrow.  Thankfully she agreed and brought some thickener in right away.

Then the pediatric team came over to take a look at baby Z and check him out.  They reviewed the chest x-ray and decided to put him on a reflux medication.  That’s when I stopped them.  I explained that my daughter had reflux.  I know what reflux looks like.  She would spit up… A LOT!  We went through lots of formulas before finally finding one that would work for her.  She had to sleep with a pillow under her mattress and had to take medication.  Baby Z does not have any of that, and I don’t want him getting another medication unless it’s truly necessary.  But, then they explained.  Basically, when a thin liquid goes down the throat to the stomach, there’s a sphincter that closes off to keep it down.  But, all babies have some sort of reflux because the sphincter isn’t tight enough.  Some babies don’t have any outward symptoms of it (like the symptoms my daughter had shown).  When the liquid is thin, it easily moves back up past the not-fully-closed sphincter and can go down the windpipe.  But, when the liquid or food is thicker and heavier, gravity helps keep it down so it doesn’t come back up as easily. So, the thickener in the formula combined with the reflux meds should get him the help he needs.  The dietitian just has some work to re-balance the 3:1 ketogenic ratio, because both the thickener and the additional medicine add carbs (and remember, even a tiny little bit of carbs can throw off the whole balance of this really strict diet.)

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