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!

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.

Then, his EEG probes were removed.

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.

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.

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.)

2nd day of Keto Diet

It seems like it should have been an easy day, but I think in general when you’re in the hospital, no day is an easy day.  Last night, baby Z was poked every 2 hours for blood sugar.  They just prick the fingers (or in his case, the toes), like you see on the commercials for diabetes.  He was so exhausted from getting the IV and getting his blood drawn yesterday, that he actually slept through the pokes during the night.  But, he was up at 2 a.m. for a feeding (which may become our new normal because he’ll need to eat more often on this diet).

Today though, his IV came out, so they had to put another back in, and they needed another vial of blood too.  At least the nurses learned from yesterday that he really is a hard stick, so they called the transport team to come do it.  Even they had a hard time, but they finally got it in, and they actually listened when I told them NOT to pull pressure to draw the blood, so they let it drip into the vial and that worked much better.  

Baby Z took the whole thing well.  In fact, he took it too “well”.  He didn’t cry when they put the band around his arm before the poke, nor did he cry during the actual poke, and she had to push the needle in, pull it partially out, re-push it in, pull it out, over and over, and still didn’t get the IV in.  So then her partner found another spot and did the same thing, but they finally got it.  But, baby Z didn’t cry during any of it.  It was actually quite depressing, because it was like he lost the will to cry because he couldn’t control the situation, he couldn’t get away, and his own mother wouldn’t help him.  UGH.  My poor, sweet baby.  I try to tell him over and over how I am trying to help him, and that I’m so sorry when they poke him.  I wish he could understand just how much I love him and want to help him.  But, all he knows is that *I* am the one who is always here when bad stuff happens to him.  He won’t even look at me after.  😦  But, I just try to snuggle and hold him and talk to him about what is going on, and tell him over and over how much I love him.

Even though he didn’t cry during the blood work, he had a rough day.  He cried a lot.  A LOT.  He didn’t want his food, his paci, or anything.  But, he did want to be held a lot, so that’s what we did most of the day.

The neurologist told us that he is having less clinical seizures (the ones we can see) and is having more sub-clinical seizures (the ones that aren’t noticeable).  He is still showing hypsarrhythmia in the background of the EEG. But, the neuro said it is moving in the right direction.  So, I guess that’s something.  

As far as the diet, today he started having more of the ketogenic formula than the regular formula.  He’s still not making ketones, but maybe in a day or two.  His blood sugar checks and specific gravity have been fine.  Tomorrow afternoon he’ll transition to all ketogenic formula, and we’ll learn when and how to prepare real food at a 3:1 ratio.  We’ll also get a list of approved products to use for pain/fever medicine, teething medicine, lotion, shampoo, toothpaste, etc.  His real food will consist of 3 parts fat (butter, cream, etc) to 1 part protein+carbs, so it should make for some unique recipes.  It requires a very precise digital scale, not just to 1 gram, but to 0.1 grams.  I ordered it from the Charlie Foundation, per the recommendation of our dietitian.  If you need one, look for it half way down the page here:  http://www.charliefoundation.org/resources.html

Abby and Joel came by tonight, and it was good to see them both.  I wish we could see them more, but it’s just so hard to get Abby enough sleep coming down here each night, with Joel in traffic to get home, pick her up and then head all the way into the city, stay a bit, then head home.  She’s been through a lot, and I’m not sure if it’s the stress, the new daycare, having a really busy weekend, or just being in school (or all of the above), but I’m crossing fingers that she stays healthy, because tonight she was complaining about her ear hurting, and she sounds a little congested.  Joel really needs to be at his “new” job, but obviously I can’t take her to the Dr., so hopefully she stays healthy and kicks whatever she’s getting.  Needless to say, I didn’t let her hug and kiss on her brother, which is really hard for her, but at least she understands!

Here’s our less than homey digs. lol

A bad day and a tissy fit

It has been a l-o-n-g day, and not a lot has gone right.  Z and I were up and on the road by 6:30 a.m., and we spent nearly 2 hours in heavy morning traffic before arriving at the hospital.  Still, I was in a pretty good mood, because I was about to start a new treatment for Z, and was hopeful this would be ‘the one’.  But, when I checked in at admissions, they told me I was supposed to have checked in last night, on Sunday.  I explained that I was NEVER told Sunday.  My daughter’s 9th birthday was on Saturday, and her best friend of 8 1/2 years turned 9 on Sunday, and we also had an additional party for some other really good friends on Sunday.  So, I remember specifically asking for Monday or Tuesday, and the Dr.’s assistant said Monday would be perfect because the Dr. was getting ready to go to a conference, and Monday would be the last day that she could still be here for the entire treatment.  Regardless, it really wasn’t that big of a deal to me, because I figured it would work out.  That is, until someone called me at admissions and chewed me out for not getting there on Sunday.  I was instantly defensive, because I was NEVER told Sunday, and her tone really pissed me off.  Then she made it sound like they were going to turn us away because they no longer had a bed for Z.  I explained that if there was a bed for us on Sunday night, and treatment was supposed to be several days long, shouldn’t that bed still be reserved for us on Monday morning.  She started making up other B.S. and continued to berate me for not being there on Sunday.  I was then told to wait and she’d see what she could do because she had to get the *new* date (of 12 hours later) re-approved with insurance.  AAAARRRRGGGHHH.  Then she mentioned that she did have a message from the Dr’s assistant on Friday explaining but that she wasn’t there on Friday.  Uhm… so WHY are you berating me because YOU should have had someone covering for you on your day off.

So, we sat and waited.  Me, Z in his stroller, the big ol’ diaper bag, a bag I’d packed with items I’d need for 4-5 days, my laptop bag, and another bag with all the other bottles, formula, diapers, etc.  Baby Z was getting hungry, so I had to start prepping a bottle in the middle of the waiting area with all our stuff, when we should have been in our own room that was supposed to have been ready for us then, on MONDAY morning.  We waited about an hour, and finally got into admissions for the paperwork and we were then escorted to our room.

Then, the real ‘fun’ began.  I explained to the nurses that baby Z was a hard stick.  But they must hear that a lot, because they didn’t seem to believe me.  Even when I explained everything in detail, like the fact that when an IV was put in, you can’t draw blood from it or it would pull the whole thing out, or the fact that if they tried to stick him, they’d end up sticking all 4 extremities before getting the IV and blood.  Or, the fact that he had to have an ultrasound in order to get an IV in at Texas Children’s.  So, what happened?  Well, they stuck him and didn’t get anything, so then they stuck him again and at least they got the IV in.  At least they did listen about pulling blood from the IV, so they didn’t, but it did require another (3rd) stick to try to get blood.  When they did, it quit flowing and they didn’t get much, but they decided to stop and see if the lab could make due, otherwise it would have required a 4th stick.  UGH!  (Which it later did because they didn’t have enough).

Once all the blood work was done, then baby Z had to get hooked up to all the EEG probes.  He doesn’t particularly like having that done, but this tech must have had a lighter touch, because it wasn’t so bad.  Or else baby Z was already exhausted from all the screaming during all the blood work.  So, it took a while, but at least baby Z wasn’t screaming the whole time!

Once all that was done, we waited for the dietitian.  Then we waited some more.  Then we waited and waited and waited some more.  The neurologist stopped in to see us, and we chatted a little bit, but nothing was really happening so I didn’t really have any questions.  So, she left and we waited some more.  Finally the dietitian came in to see us.  Basically, she went over everything we had ALREADY discussed on Friday, and then she said she had to figure out the combination of regular formula to ketogenic formula.  Uhm… I thought that’s what she was supposed to do Friday.  But, okay.  I thought it was going to just be a few minutes.  But, we waited, and waited, and waited some more.  Finally, around 6:30, she came back and had worked out the details.  But, she said we were going to wait until the next day to start.  What?!?!  I questioned her philosophy, but she said it would be better to start in the morning.  I guess I see her point, that he’s going to be woke up every 2 hours for blood sugar checks.  BUT… that’s going to happen tomorrow night, so why not just start.  It just felt like a waste of a day, and I don’t really want to be here on Friday just because she wanted to wait.  So, I convinced her that we should get started, and baby Z got his first “dose” of his transitional recipe at 7:30 p.m., after the dietitian had left for the day.

The dietitian had given us an easy recipe of 4 ounces of regular formula to 2 ounces of ketogenic formula, for the first day, making a 6 ounce bottle.  But, she had specifically told me that he could eat whatever he wanted on the ketogenic diet, as long as he stayed at the right ratio.  I had explained that sometimes baby Z sleeps a lot one day and barely eats, then does the opposite the next day.  So, he doesn’t have a typical 24-hour type of schedule.  Anyway, she was gone, baby Z drank his entire 6 ounces really fast, and was still hungry.  I knew he didn’t need another 6 ounces, so I started mixing 2 ounces of formula in our room, and walked to the refrigerator across from the nurses station to get 1 ounce of the ketogenic formula.  But, then the nurses saw me and told me he couldn’t have any more formula (or anything) for 4 hours.  WHAT?!?!?  All the while, baby Z is in his room screaming, because he’s hungry and waiting on me to bring him more food.  I explained what the dietitian had told me and I explained that he just need a couple more ounces.  But, they wouldn’t let up.  So, I had four nurses very adamantly telling me that I could not feed my baby because it was a very strict diet.  I was getting angrier and angrier, because that is NOT how it was explained to me.  If it had been, I would have been sure that the dietitian had formulated it so he could have 8 ounces, or feedings more often, or SOMETHING.  But, they wouldn’t listen.  Finally they offered to call up the dietitian. At that point I was pissed.  (Remember, baby Z is down the hall screaming at this point).  I told them that they better do that, then I stormed down the hall and I SLAMMED the door, and pulled the curtain.  Then, instead of ripping the flipping video camera off the wall like I wanted to do, I took a deep breath, picked up my screaming baby, and tried to calm him.  A few minutes later, two of the nurses walked in and pulled the curtain back and nicely told me that they were wrong and that I *could* feed my baby.  AAARRRGGGHHH.  You’d think they’d have experience with the ketogenic diet and would know this stuff.  But, whatever.  Even though they were in the wrong, I am completely embarrassed at my reaction.  I guess it was the stress of the day and not being able to feed my hungry baby, when the whole point of being here was to feed him this stuff.  But, I acted like a toddler, and it’s embarrassing.  Luckily though, the nurses seemed to understand, and they commented that they understand and that they have babies too.  Now it’s become kind of a running joke, because each time I walk out of the room, they ask how I’m doing and smile, and we all kind of laugh.  That was not the ice-breaker I had in mind, but, hey, whatever works, right.   HA!

I talked to my hubby, and we feel that all the ducks were definitely not in a row today, and that the hospital really needs to work on that. (That’s not exactly how our conversation went, but I’ll save you from some of our expletives!  😉  I’m just not sure how to proceed, or who to turn to.  We definitely think they need some feedback so they can improve.  But, how do you go about that when you may have a long-standing relationship with these people and you don’t want to offend them?  How do you give diplomatic/creative criticism???  We need them on our side, to help baby Z.  But, we also need them to have their ducks in a row, so we can feel confident in their abilities, and more importantly our little guy (and other little ones) in the best way possible!

Tough Decision: ACTH or the Ketogenic Diet

We had our 2nd clinic appointment with our new neurologist today, Dr. V.

Since baby Z is not doing any better (and is actually doing worse the last few days), it is time to make a change.  Our next options were either ACTH or the Ketogenic Diet.

J & I  have discussed this at length, and we have changed our minds on multiple occasions, but today we chose to move forward with the ketogenic diet.  Baby Z seemed to make some progress when we removed all the cereal and fruit juice, and had him eat more protein.  Based on that fact, and the fact that the ACTH is a very aggressive drug that has a whole laundry list of really NASTY side effects, and also requires daily injections, we feel that this less invasive, less aggressive treatment is a better option.  But, when I say less aggressive, I do not mean that the Dr is taking a less aggressive approach to getting rid of these nasty little seizures, because the ketogenic diet has been shown to have good results, and she would not have offered it as an option if she didn’t believe it could help him.  It’s just not a nasty, aggressive drug with terrible side effects.  So, if we can get the results we need with this option, it’s all the better for baby Z.  We can always move to ACTH if the keto diet isn’t working, and that will be our next move, if the Dr. doesn’t see a remarkable improvement (meaning NO seizures) within the next 2-3 weeks.

If you want to know more about the ketogenic diet, there are some links in the resources tab.  Basically it’s a very strict “diet” that has a 3:1 or 4:1 fat to protein/carb ratio.  I had to explain to my nine year old that it’s not a diet like the ones you hear about to lose weight, just a strict guideline on certain foods.  For right now though, because he’s so little, it will be mostly just a high fat formula, and later we’ll add in some other foods.  But, we will have to weigh and measure everything, and apparently we will even be given a list of other products that are keto-friendly, such as toothpaste, shampoo, teething medicine, fever reducer, etc.  Even the slightest shift in the ratio can bring seizures back.  So, it’s going to be a bit of an undertaking.

Baby Z will have to be hospitalized for several days while he is put on this diet.  They have to get baseline blood work, and then will be checking his ketones and his blood sugar as they steadily get him on the right ratio.  It sounds like he will go in early on Monday morning, and should leave by Thursday or Friday.  It seems kind of strange to be hospitalized to be put on a “diet”, but it’s no regular diet.  Plus, since he’s so little, they just want to make sure to get it right.