Archives for category: hydrocephalus

Today is Lizzie’s birthday and she is now eight-years-old.  I made a list of Lizzie’s progress.

Miss Lizzie – Age 8

In addition to everything on her “can” list last year,

Lizzie can:

Cruise her railing on her bed.

Turn her feet the right way while standing in her crib.

Open and empty a drawer (much to Mommy’s chagrin).

Operate a light switch when prompted.

Move her chair along if she has a wall or dresser to push/pull off of.

Stand and empty toys and books from bins.

Lizzie can not:

Walk independently

Talk (except for the occasional Mamama and All Done)

Use the bathroom

This year Lizzie started:

I’m walking here!

Walking with a gait trainer at home.

Riding a tricycle at home, that we push for her.

Sitting up better, for longer, and sometimes sits to play rather than laying all the time.

Getting better at handling foods and liquids by mouth (More swallowing, less coming back out, still not more than a couple of times before boring/tiring).

This year Lizzie had trouble with:

A repetitive bubble on her g-tube

This year Lizzie received:

NO new diagnoses and no seizures for 7 months today.  YAY!

1 new seizure medicine :  Topamax, which brought the total to four with Phenobarbital, Keppra and Trileptal.  Then we said goodbye to Trileptal (three) and now are weaning Phenobarbital.  Hopefully she’ll only be on two next year.

Check out my cool new ride!

A gait trainer and a tricycle.

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I hung up the phone yesterday and I woohoo-ed, surprising my son who was at the back of the van. Lizzie’s gait trainer has been approved, which means that in about a week and a half, she will be walking in our house for the first time. This is such exciting news. Of course, as I was woohoo-ing, I swear I heard my son mutter, “Godzilla,” under his breath. This is no doubt in reference to the destruction of Lego villages he sees in the near future as his sister becomes more mobile. I had to laugh. 🙂

I spoke to Lizzie’s neurosurgeon’s office today, and here is what I found out:

1. He has a lot of experience with VNS as he has put them in.  Which means if Lizzie gets one, most likely, he will do it.  I breathe a sigh of relief here.

2. He had to read up on the shunt/VNS paradox after I called.  Then he found the same study I did, and came up with what may be a solution.

3. There is a newer programmable shunt valve that is less sensitive to magnets, and would be a solution to the shunt reset issue.

Unfortunately, this would mean:

1. Lizzie would have to have shunt surgery – and her working shunt would be disturbed, and her working valve replaced.  We would then have to wait at least a month to make sure Lizzie’s new valve and shunt are working properly before we could even consider the VNS placement.

2. More than one surgery – so multiple chances for infection.  Also twice the trauma for surgery itself.  And more time away from school and therapy.

3. More than one new device – so multiple chances for equipment failure which means refer to #2 above.

4. If the valve fails, or just doesn’t work for Lizzie for some reason it would mean another surgery to correct the problem.  And this would be a new problem we created, because there is no problem with the equipment she has now other than it is incompatible with the new device.

5. If the VNS doesn’t work for Lizzie, we would have done all this for nothing.

If, however, it worked, it would mean:

1. Less severe or less frequent seizures (or in some cases, no seizures at all (dare I dream?)).

2. Possibly less medications, so less side effects (which in Lizzie’s case seems to mean more abilities).

3. Fewer worries about seizures (although the worries will always be there).

4. An easier way to intervene when seizures do hit (a magnet swipe across her chest vs. a rectal medication that wipes her out).

This is a really tough decision.  We have to do an EEG and meet back with our neurologist at the end of next month, then I’ll know more.

VNS or Vagus Nerve Stimulation is a treatment for epilepsy.  It was recently suggested to us by Lizzie’s neurosurgeon, and is something we are considering trying.  With this treatment, a small device, (like a pace maker) is implanted into the chest and has wires that wrap around the vagus nerve; a nerve in the neck that goes into the brain.  With stimulation from the device, seizures either stop, or can be slowed down.  Some people actually report no seizures or a significant reduction in the occurrence of seizures.  The device can work on its own, or it can be activated by a magnet swiped over the chest at the onset of the seizure.

If medications continue to not work for Lizzie this may be an option, but it does scare me.  This is a serious decision, as it is surgically inserted and gives shocks to the nerve to stop seizures.  I am nervous about the magnetic factor of the VNS, as Lizzie’s shunt is also controlled by magnets.  I read online that there was a study conducted in which 100 trials were performed and 78 times the VNS messed up a programmable shunt’s settings.  Highly discouraging.  This means that we could get the seizures under control and go into shunt failure.  I definitely need to call the neurosurgeon on Monday to ask about this.

I also read that the VNS is not recommended for those with heart issues.  Lizzie’s heart is fine, but her aortic valve is bicuspid (having only two parts) instead of tricuspid (having 3 parts, as it should), this means that her valve works harder than most people’s to get the same job done.  Bicuspid aortic valves, as it has been explained to me, can over time become weak or stiff from the overwork.  They can fail or leak and require surgery or replacement.  Or you can live with it forever with no issue.  We have Lizzie’s monitored once a year by her cardiologist, and so far it has been fine.  My question now is can the electrical impulse from a VNS cause issues with this specific heart issue?  I guess I will also be calling our cardiologist on Monday.

It’s a lot to think about.  I really want Lizzie’s seizures under control, but what risks are worth taking to achieve that goal.  I would also like to reduce the amount of medication she takes for her seizures, as this seems to cause delays in her skills and development.  But again, what is it worth?  I definitely have to do my research on this one.  I find that the older Lizzie gets, the harder some of these decisions are getting.  She is physically stronger, and these procedures and treatments are not life or death but rather quality of life, and that makes it much harder to decide.  The question of risk versus benefit becomes much harder when both sides are close to equal.  Those earlier surgeries were easy to decide because it was surgery or she could die.  Easy decision.  But this one, implant the device – risk the shunt or heart, or stay on meds that don’t work 100% and risk holding back her abilities and having seizures.  Although we could implant the device, and still have seizures, if it doesn’t work for Lizzie.  Tough call.

We will be taking our time with this one.

Today begins National Hydrocephalus Awareness month.  Our family has been aware of hydrocephalus for the last 7 1/2 years.

The funny thing is that hydrocephalus is honestly not the most immediate of Lizzie’s issues these last few years.  In the beginning of Lizzie’s life, hydro was everything.  We watched and we measured month by month to see how big Lizzie’s ventricles were, and how much more “water” was in her scans, and how much less brain matter.  Hydrocephalus was the big question, and the end all be all of her health issues.  When she had her shunt infection in October of 2004, it was the sickest I’d ever seen at that point in time.  I remember she was gray, limp, and non-responsive.  Our pediatrician took one look at her and said, “Go to the ER.”

But since we’ve had her second shunt put in, things with her hydro have been in control.  Don’t get me wrong, I still think of her hydrocephalus as a serious concern, but it is not something that is first on our minds.

Now-a-days, her epilepsy is far more prevalent in our daily lives.  Seizures are the biggest danger we face.  Just one of those can send us to the ER in a heartbeat, and can cause her to have to stay in the hospital.  Besides the epilepsy, there is the recurring infection in her g-tube, which is not just a nuisance (because it refuses to be resolved), but it is also a concern because obviously it is an infection and infections for special needs kids can be dangerous.  Then there is Lizzie’s cerebral palsy, which is the main cause of her mobility issues, and also the muscle weakness that causes her swallowing and speech delays.

So, hydrocephalus is always in the back of our minds, but not the first thing that comes to mind when I think of Lizzie’s health.  And yet, hydrocephalus is the mother of all of Lizzie’s health problems.  With the exception of her bicuspid aortic valve, the hydrocephalus caused all of Lizzie’s other problems.  Lizzie’s hydrocephalus damaged her brain from the pressure built-up before birth.  That damage led to her epilepsy, caused her cerebral palsy (which caused her scoliosis and hip displasia), her speech and feeding problems and her cortical vision impairment.

Even though the hydrocephalus is not our biggest direct cause of Lizzie’s issues it is the indirect cause of all of the conditions that are.  Hydrocephalus affects 1 in 500 births, and is not something that most people are familiar with.  It is something that is silent, and not well publicized, but can really effect the people who have it.  So, my Facebook profile pic will be wearing a blue ribbon for hydrocephalus this month, and I will be too, because hydrocephalus is a real condition with real effects on people’s lives, and people need to be aware of it.

Our journey started more than seven years ago, when I was still pregnant with Lizzie.  There is nothing like going for a “higher level” ultrasound to look for a possible heart problem in your unborn child, and being told by the technician that your daughter’s heart looks fine.  I was soaring.  I thought, “I have a daughter, and she’s going to be okay.”  Then minutes later, the doctor comes in with a very serious face and says, “We have a problem.”  He explains that the problem was not with her heart, but in her brain.  So much for soaring; I felt like I had hit a brick wall.  They didn’t tell me what was wrong yet, because I was alone.  I went into the building’s foyer, and waited for my husband to arrive.

That day they told us about hydrocephalus.  A condition that meant our daughter’s brain was full of too much cerebrospinal fluid, which was putting pressure on her brain.  They told us that we had to watch the progression, that it might resolve itself before she was born, and that there was no way to know what hydrocephalus would mean to our daughter or to us.  It was all a big wait and see.

The rest of my pregnancy, we watched the fluid build-up.  Every month we talked to a different neonatologist in the practice, and every month it looked worse to me.  Worse meaning: more black area of fluid and less whitish-gray brain area.  I mentioned this to one doctor who basically told me, “Well, I do know more than you, because I have a degree, but yes it’s getting worse.”  Okay, tell me I’m stupid; then tell me I’m right.  They never discussed ventricle sizes with us, and I didn’t know enough to ask.  No one would tell us what any of it meant.

When we got closer to the due date, they suggested we meet a neurosurgeon.  Our first meeting did not go well.  He spent the entire visit explaining the horrors of spina bifida to me.  After I sat there numbly taking it all in, I managed to mumble something like, “But they told me she only had hydrocephalus.”  The panicked surgeon consulted his nurse, and then apologetically explained he was having an emergency surgery scheduled and things were not going well, and I realized he was having a bad day.  He then explained hydrocephalus to me and what our options would be.  We were told that sometime shortly after birth a plastic shunt (VP) would be inserted into her brain that would carry the surplus fluid to her abdominal cavity where her body could reabsorb it, and that most likely this would be permanent.

Near the end of my pregnancy, a C-section was scheduled, because it was determined that her head would be too large to pass naturally.  However, even in the womb, my daughter had a mind of her own.  Four days before we were scheduled I found myself in the hospital, in pain, and waiting for someone to deliver my baby.  Emergency deliveries kept bumping us.  I thought, “What’s more emergent than a difficult delivery of a medically challenged baby who needs to deliver C-section but is trying to come naturally?”  Hours later, we finally delivered, and my daughter was whisked away to the NICU (neonatal intensive care unit) before I even got to see her.

When I met my daughter, six hours later, the NICU nurse informed me, “You have a very opinionated young lady on your hands.”  These words and the other we heard before she left the hospital twenty days later, “stubborn,” would shape our journey.