Myoclonic seizures vs jerks

Some time before LJ’s surgery but after his birthday, we saw his neurologist.  It was just our annual appointment but between visits, we had raised LJ’s dose of Clonazepam because his jerks were becoming more frequent.  Plus, his therapists at the Rehabilitation Institute (RI) felt these jerks would start interfering with his ability to do things independently. 

At our last visit, LJ was diagnosed with myoclonic seizures.  Myoclonic (MY-o-KLON-ik) seizures are brief, shock-like jerks of a muscle or a group of muscles. 

I expressed the therapists concerns, along with my own.  They seemed more frequent when LJ’s Botox was wearing off, or when he was really tired and hadn’t had his medication in a while.  I’m not sure what I was hoping for, but then the neurologist started mentioning Keppra.  I had mixed feelings about this but he reassured us that we’d only start the Keppra after the EEG verified they were indeed seizures.

The following Monday (I can’t believe he got us in that fast!), Louie took LJ to get his EEG done.  The nurse asked him to stay afterwards because our neurologist wanted to talk to him (which just shows me how awesome our neurologist is… I mean, even the nurses were surprised he was coming down).  He told Louie that the neuro-something (I’m not sure what they’re called) had to review the results, but he did not think LJ was having seizures.

When falling asleep, does your body ever jerk, startling you awake?  That’s a myoclonic jerk.  Hiccups are also apparently a myoclonic jerk.  Imagine how annoying it would be to have these jerks… all.the.time. 

I suppose I should have taken this as good news but all I could think was… now what?  Before, when we believed they were seizures, there was an answer.  And as much as I didn’t like the idea of more medication, there was something

So I did what many other mothers do.  I googled it.

Turns out, we’re already doing the treatments that many sites suggested.  He’s on the exact medication, albeit the lowest dose, and he receives Botox.

Wait, I thought.  Botox?!

I guess it makes sense.  From what I gather, a jerk is a nerve reaction as opposed to a seizure which would be a reaction to electrical charges in the brain.  I get why it’s happening to LJ; he had significant brain damage.  In fact, everyone expected him to develop seizures.  Hell, I’m sure they still expect him to.

I need to do more research on them, although I have a feeling I won’t get too much more information then I already know.  The most frustrating thing when dealing with things like this, seizures, hell, even depression, anxiety… it’s just a PRACTICE.  The brain is still a very unknown territory, especially when it comes to things like damage.  And then there’s conflicting arguments, for example HBOT (hyperbaric oxygen therapy) where many many parents swear by it, but just as many, if not more doctors say it’s a load of crap and probably just a scam, with how much is charges.  How do they know?  How do they know how the brain is reacting to this treatment?  No, it’s not an exact science, but neither are the drugs you’re prescribing my child. 

Which also makes me wonder how they read LJ’s EEG?  He will always have abnormal brain activity due to the amount of brain damage.  The EEG will never be “normal”.  Do they compare his results to “normal” EEGs?  Is that really an accurate thing to do considering the circumstances?  This reminds me of how they keep putting LJ (and all the other kids with special needs) on the same growth chart as typical kids.  In case you didn’t realize this, MY KID IS NOT TYPICAL.  Why they do this when there is a CP growth chart out there, I have no idea. 

Sometimes I get overwhelmed, thinking about LJ and his needs.  But then he’ll smile at me and it all melts away.  I know this is just one of many things we’ll have to deal with on Our Yellow Brick Road, but we’ll deal with it together.  He’s worth it.

So much for taking some stress off

Well, we’ve already been through 1 nurse and 2 agencies.

From the start, I knew I’d want to meet the nurse before having them assigned to us.  I’m glad we did because the first nurse they sent us lived 80 mile away.  At the time, we were asking for 10 hours a day, 5 days a week.  It’s a lot but financially we’ve been hit pretty hard with all of LJ’s therapies, annual appointments, and his surgery.  Right now it’s a busy time at my work, so I was hoping to try to catch up on some hours.  When school started, we would reduce the hours. 

We decided to go with a different agency, since the first one didn’t seem to use common sense (hello, what would we do in the winter time when it snowed?). They said they’d send someone out Thursday but they weren’t sure what time, so they would call before hand.  No one showed up.  No one called.  It wasn’t until the next day that the agency called and explained that he didn’t have all his paperwork in, blah blah blah.  Ok…

So Monday rolls around (the day of LJ’s EEG, so we were sleep deprived) and I get a call early in the morning.  The lady asks, do you not need a nurse today?  I’m like, what is going on?  No one told me you were coming and I told them I had to meet the nurse before signing up with her.  She apologizes and I feel bad for her coming out for no reason.  But really, I was not with it that day.

We finally meet her the next day.  She’s perfect.  Plenty of experience in pediatrics, plenty of home nursing care experience, has kids, seems very sweet.  I tell her about the schedule change when school starts and she nods.  She didn’t start until Friday, which I find odd, since she was suppose to start Monday, she should have had her whole week free.

Everything seems to be going well.  She tends to do things her way, which is alright.  There are a few things that we’d like to do our way, for consistency and so LJ can anticipate what’s happening.  I start talking about school, routines, therapies I’d like her to start working on, after she’s had some demonstration with our therapists.

Last week, LJ had to have a follow-up GI appointment.  His button has been moving too much which has caused the skin to grow weird (I wasn’t there for the appointment so I’m really not too sure about what I’m talking about). They sprayed some stuff on it to get it to fall off and instructed us how to clean it and use tape to keep the tubing from moving the button. 

Louie’s pretty accurate when he relays information to me from doctors.  Sure, sometimes he forgets to tell me things, but what he does tell me, is usually correct. Even if I’m there, we tend to have different perceptions of what the doctor says, and he’s usually correct.  It doesn’t bother me like it once did; he’s just better at science then I am!

Anyway, the point of that was that he was showing the nurse how to do some of those things, and she would talk over him.  He’s get a little irritated but would get back to the point.  Then she said, well I don’t know how much longer I’m going to be here with the school schedule.  Ummmm…. okay.

Needless to say, that upset us a bit.  First of all, we mentioned this to her and she didn’t make it seem like a big deal.  Second of all, we wouldn’t be spending all this time training her if we knew we had to do it all over again.  The agencies had offered a temp nurse until we got a permanent one and I said no, because training in itself takes a lot of time.

She was off and on the phone that day with the agency, even after Louie told her it wasn’t her job to get us another nurse and that we’d take care of it.  Later, after she’s off, she calls me and tells me that she really is in love with LJ and our family and that the agency called her to tell her that we actually wanted more hours then she thought.  I told her I’d talk to Louie about it and I’d see her tomorrow.  Louie just said he would talk to her about the hours we needed.

This morning, I realize it’s pretty quiet and notice it’s 10 after 7 and our nurse isn’t here.  Ok… I give her some more time then I call her.  She tells me that she took herself off our case on Wed and finished out the week (she left early on Friday) and didn’t they send someone else?  I told her that I was upset because no one told me AGAIN what was going on.  She said do you need someone? And I start to talk, although I’m not sure what I was saying but she just starts talking over me again.  Finally I said, don’t you worry about it and hung up.

The thing is, I feel bad about this!  I mean, obviously she wasn’t a great fit with our family, but I liked her and I hate that our relationship ended like that.  At the same time, I’m furious that no one told me what the heck was going on!  Don’t they think it’s important to inform the families?  We were even going to use the same agency, but Louie was just like, forget it.  We’ll use one our therapist’s recommend.

We have 2 weeks left with our Early Intervention therapists and one week with our Rehab. Institute (R.I.) therapists.  Luckily, the therapists at the R.I. are putting together some stretching guidelines and therapies to work on.  I really wanted our nurse to have a good understanding of LJ and his disabilities so they could better understand him.  School starts in 3 weeks, which seems to be our deadline now.

Obviously, there was a huge communication issue, regardless of how many times I tried to clarify it with her.  Did I miss something?  What do you guys think?

G-tube and help

Ok, mamas, you were right.  After a week of being at home with the new g-tube, I admit one of my thoughts was, “I wish we’d gotten this sooner.”

On Thursday, we had a follow-up appointment with GI, just to make sure feedings were going well.  They are.  We aren’t even up to our target goal (8 ounces, 5 times day) and LJ had gained 2 pounds since Friday.  2 POUNDS!!! Granted, it’s the 2 pounds we lost this past month, but still.  2! POUNDS!  It’s a huge deal for us.

I can already see a difference.  His ribs aren’t protruding as much.  His delicious cheeks are even rounder, fatter.  He seems to have more energy.

The best part is, we don’t have to fight at meal time anymore.  I downplayed eating time with him a lot.  It was stressful trying to make sure he ate enough.  There were battles when he was too tired.  At one point, I was practically force feeding him.  Many times, we would all end up in tears at the end of the meal. 

But there were times he did really well, too.  Like the past few months.  And this made me really question our decision.  But after 5 illnesses this year, his eating and drinking habits suffering a little more after each one, and after the last illness where he lost 2 pounds, we knew what we had to do.

And it was the right decision for us.

We don’t have to worry about changing his schedule because of all the therapies we have, daily.  We just hook him up to his backpack and go.  We still feed him by mouth when we can… which brings me to an exciting part…

We’ve qualified for an in home nurse!

I’m nervous about bringing someone in my home, into my family (we have lots of hours) but I’m excited to have help.  I’m excited that someone will be able to do with LJ all the things I don’t have time for because of Noah or trying to work.  I’m excited he’ll have more individual play time.  I’m excited to have another person I can talk to about our life that will get it after a few days with us. 

We decided to meet the nurses before just signing up with one.  I’m glad we did because the first one was kind of bomb.  Then again, we’ve had such great therapists and have sought out great doctors, that our expectations may be kind of high.  I know that anyone will fall in love with LJ, but we want someone who’s passionate about him and their job.  Someone that will be a part of our family.  Someone that will relieve stress and not add to it.

G-tube

It happened a lot faster than I expected.  Last week, we went to our very first G.I, after a small battle.  See, our surgeon already gave us the approval for surgery, but he wanted us to be sure of our decision, so there’d be no regrets.  Well, the day before, the Nurse Practitioner calls Louie and tried to finagle out of the appointment (ok, so it wasn’t on purpose but it ticked me off).  Long story short, she cancelled our appointment, without telling us.  It was pure chance that I caught it the evening before.  Then they tried to move the appointment to a different time, but I wasn’t having any of it.  At the end of our conversation, she told me frankly, “You know you’re seeing NP, not a doctor.” Yes, I knew this.

It was obviously an important appointment, since the doctor did indeed end up in the appointment with us.  We discussed the PH Study (surprisingly, normal…), his feeding habits, his recent gagging issues, his other conditions that would affect decisions regarding surgery.

In the end, we all agreed to do the G-Tube (feeding tube through the tummy).  It was scheduled the following Wednesday.  As in 2 days ago.  It was quite a scramble to get everything together. 

The surgery went well.  The first day was the hardest.  Typically, surgery sends you home the next day, but G.I. was concerned with re-feeding syndrome, which basically would lead to heart problems.  Surgery wanted to send us home the next day, even before the surgery, regardless of the note.  I made sure that didn’t happen, which got us switched to the G.I. team the next day.  They’ve been a lot more cooperative. 

I was nervous, scared, and excited.  I doubted myself even though I knew it was what we needed.  To take the pressure off, to have enjoyable meals, not worrying about calories.  But surgery is scary.

While we waited for him, I couldn’t help but feel this was only the beginning.  We’ve already been told by orthopedics that we need to do some tendon surgery for his hips but we postponed, wanting to give Botox a chance.  But mostly, we felt he was awfully young for such a procedure and would mark the beginning of many surgeries to come.

It’s a little unnerving to think how accustomed to hospital stays I am. 

Tomorrow, LJ will probably be discharged.  Tomorrow is also the 3 year anniversary of the first time we got take LJ home from the hospital, 2 weeks after he was born.  From the same hospital.  Just thinking about it fills me with mixed emotions.

A prayer was whispered everytime the chopper took off for the child they went to transport

 

Dermatologist

We took LJ to his first dermatologist appointment, thinking it would be futile (ok, that was just me).  I was wrong.  In fact, it was a pretty good experience, despite being 30 minutes late (damn construction!)

When we stopped by the height/weight room, I groaned inside.  How many times do I have to explain to people that LJ cannot sit or stand on his own.  He’s in a fully supportive pediatric wheelchair that many professionals recognize, but that doesn’t stop them from their barrage of questions.  After explaining something 999,999,999 times, it gets old. dsalfjdsjf

This time, though, the nurse waited by the standing scale, where Louie picked up LJ and got weighed together.  Then Louie weighed himself.  She did the math and reported he was 23 pounds some odd ounce.  Wait.  Did she just say 23?  They reported that at the PH Study, but they didn’t sound sure, so I took it with a grain of salt.  I know one pound doesn’t sound like much, but to parents who are constantly adding calories anywhere they can to a boy who hasn’t budged in weight in what, 6 months?  A year?  It’s incredible.  Especially after the recent illness and lack of appetite that followed.

So we described the “rash” to the nurse (red, hot extremities, seemingly for no rhyme or reason, either both hands, one hand, both feet, one ear, etc) that typically last about 5 or so minutes.  After a short wait, a few people shuffled into the room, one I recognized as the doctor by her coat, despite her laid back appearance.

She listened to us and examined him, first addressing a lesion (and what I thought was a mole).  She immediately diagnosed him with mastocytoma and said to watch it.  She then gave us a list of drugs he should avoid, which honestly surprised me.  But really, I never paid attention in science class so what do I know?

Then she diagnosed his rash as Erythromelalgia.  Holy crap, that’s what my Google search came up with!  I thought.  As she explained what was happening, I tried not to let my eyes glaze over and actually try to understand what she was saying.  I tend to be a hands on kind of learner though, so when I got home I went back to Dr. Google.

In case you’re wondering: Erythromelalgia, also known as acromelalgia, Mitchell’s disease (after Silas Weir Mitchell), red neuralgia, or erythermalgia,[1]:816 is a rare neurovascular peripheral nerve disorder in which blood vessels, usually in the lower extremities(or hands), are episodically blocked, then become hyperemic and inflamed. There is severe burning pain (in the small fiber and sensory nerves) and skin redness associated with the return of blood flow following the transient vasospasm. The attacks are periodic and are commonly triggered by heat, pressure, mild activity, exertion, insomnia and stress. Erythromelalgia can occur either as a primary or secondary disorder (i.e. a disorder in and of itself or a symptom of another condition). Secondary erythromelalgia can result from small fiber peripheral neuropathy of any cause, essential thrombocytosis, hypercholesterolemia, mushroom or mercury poisoning, and some autoimmune disorders. Primary erythromelalgia is caused by mutation of the voltage-gated sodium channel α-subunit gene SCN9A.*

So from what I gathered, it could be an abnormal brain thing or not.  The triggers seem pretty consistent with LJ.  Insomnia, stress, exertion… even heat seem to trigger it for him.  He seems uncomfortable but probably not in too much pain.  We are to treat with cool packs, which since LJ doesn’t really like cold things, won’t be awesome.

Another mystery solved!  It sucks not knowing and understanding what’s going on with your child, especially when they can’t tell you something.  I’ll read all the science books in the world to better understand what goes on with LJ. 

Here are a couple of pictures that I forgot to bring to the appointment (but they let me email them!).  Poor baby.

*From Wikipedia.org

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