Luna’s Story: Update #2 – Never. Give. Up.

A 6-minute video.

How can a 6-minute video video catalyze so much activity?

1:30 into the video I see one process happening in Luna’s brain; the happiness in her eyes melts away while fear and terror are evident as her brain struggles to stop a raging electrical storm.

After a 75 sec complex partial seizure,  a separate process is evident: Infantile Spasms which clusters for several minutes.

It is a remarkable video, one that captured her seizure disorder at a very early stage, and before almost all mediation.  I am not sure I would have been so insistent Maria continue to seek second opinions after the Norwegian healthcare system more or less gave up on Luna.

If you are unfamiliar with Luna’s story, here is the first post from November 2014.  I want to share some of the highlights of her journey in this post.

Nearly 6 months after the onset of Luna’s epilepsy, she received her first 24 hour VEEG.  Prior to this, it was only short EEG’s without synchronous video.  By this time, courses of steroid and hormone therapy (synthetic ACTH) were tried with some effectiveness, but almost immediate relapse upon completion. Luna was on several conventional anticonvulsants and Sabril.

If they captured the overnight VEEG data before all this intervention, what might be different?  Difficult to say, but very interesting to the point of heartbreaking to consider.

MRI impressions were normal and did not correlate with the clinical presentation.

Finally, negative targeted genetic and metabolic testing rendered Luna’s case more or less closed in Norway: etiology unknown.

Take the pills, accept her as she is.  She will be disabled, was Maria’s translation of what she was told.

Luna 11-6-2014

I advised her: Do Not Give Up!  I always felt there was hope for Luna.

Why?

Impressions from early EEG tracings found epileptiform discharge activity in both hemispheres, but more in the left hemisphere, and very close to the midline of the brain and almost always with overweight and pre-dominance in the left hemisphere.  Often there was bilateral slowing.  The slowing was frequently found to a greater extent in the left hemisphere.

To me, the remarkable video was a sign that all the generalized activity could be irrelevant if there was a focus discovered.  This theory is difficult to prove given the focal events ceased after the steroid therapy.

An analogy:  Imagine if you were spraying water mist or hair spray on your child’s head.  And you were standing on their left side spraying towards the head around ear level.  As you spray, you move the spray nozzle around, and as you get near the top of their head some of the spray easily falls on the other side of their head.  At first, it would be clear that some of the spray from the left side fell on the right side.  But if you just never stopped spraying, after a while it is impossible to determine if you sprayed some from the right and some from the left independently, especially if you were brushing the hair during this time.

Maria began looking outside of Norway for help.

Helsinki, Finland has a well-known comprehensive epilepsy treatment facility.  Maria engaged the doctors there as did I. Uninterested, they referred her somewhere else in Sweden that was not a good fit for Luna.

2014 began with a trip to Bonn, Germany.  The financial cost was high.  But, the emotional toll was higher, as once again negative MRI findings ended the investigation despite the presence of a focus in the left hemisphere found in a long-term VEEG.

Dr. Sassen reviewed the early videos and agreed about the focal nature of the episode in the one very remarkable video.  Why then did he not recommend more diagnostics?

Luna was weaning steroid therapy during this time and was experiencing seizure control; so no seizures were captured during the VEEG.  This lack of clinical seizures was likely to key reason additional diagnostics were not performed.

I remember the defeat in Maria’s words in emails, the misery, and emotional turmoil in the family.  Chronic seizure disorders in young children are well-known for disintegrating the family unit.  During this period Maria realized she had a marriage built on sand not rock.

By the summer of 2014, Maria was in contact with Dr. Von Allmen and Children’s Memorial Hermann Hospital here in Houston.  In parallel, Dr. Simon Harvey from the Royal Children’s Hospital Melbourne, Australia, also reviewed her case.

Dr. Harvey insisted she get a PET scan.  In a matter of days, Dr Harvey in Melbourne Australia ordered a PET scan for a child from Norway to be performed at St. Thomas hospital in London, England.  You can’t make this up!

The results indicated she should be a good candidate for surgical intervention. Finally, I felt like she broke through an invisible barrier in getting help for Luna.

Where can Luna receive such surgical care?

It wasn’t long before the USA became the only real option and Children’s Memorial Hermann Hospital was the natural selection.

They quoted $125,000.00 for investigation and surgery, far more than anywhere else.  But, Luna could get quick access to care here and could not elsewhere.

Maria created a fundraising page through one of the internet-based fundraising sites.  The response to Maria’s call for help was dramatic and surprising.  God’s plan indeed.

Like every step along Luna’s path, accessing pledged monies wasn’t without significant challenge.  Global political tension between the US and Russia made transferring Russian monies difficult with credit cards and wire transfers – the kind of transactions needed in this situation to quickly fund the planned events.  Her friends came through with great success!

We don’t know all the donors, but whoever you are, you played a vital role in changed lives.  Thank you for your kindness and generosity.  Not only did you help change Luna’s life, but you illuminated a potential path for others like Luna.

A pause.

Maria didn’t have the necessary funds CMHH demanded.  And, yes, ‘demand’ is the correct word.  Global political tensions and resulting monetary policy restrictions delayed the transfer of some pledged funds.

What to do?  Make the trip, or wait until she secured the funds?

I remember telling her “Just get here.  And, we will figure out the rest.”  This was our moment we have been waiting so long to happen.

Despite many setbacks, roadblocks, and barriers, Maria and Luna made it to Houston.

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After evaluation and consultation with Dr. Tandon , the chosen path at that time was a TPO resection/disconnection.  The hope was that remaining cortex was not implicit in the epilepsy.

A hemispherectomy was discussed as the likely procedure to provide the most control, but also with the most consequence.

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The procedure had a remarkable positive effect on Luna, but unfortunately it was clear she needed more help as her epilepsy continued uncontrolled.

Devastated, Maria returned to Norway with Luna to collect her emotions.  Here was a post I made just before she departed.

Emotionally knocked down, she stood back up.

Maria reorganized her life and relocated to Houston seeking further care for Luna.  She secured a job such that the company paid for the transfer.  She leased a house, and a car.  And began engaging in all the things that go along with living in the US.

[This person, this Mom, Maria, has a wealth of courage and love in her heart.  She used to get tired and say “I have no forces left”.  I always chuckled at her word choices, but she did have forces left.  She is human yes, but has superhuman ‘forces’ in my book.  As I reflect on all she conquered to get real help for Luna, our journey with Savanna pales in comparison.]

Luna diagnostics second round CMHH 2015

American medical insurance in place, a new round of surgical evaluation was initiated.  The results were confounding.  Discharges still in the [connected] left and right hemispheres.  Dr. Von Allmen recommended a larger resection, likely including some motor cortex.

Dr. Tandon wasn’t confident that would help but was willing (as I understand it).  He recommended a palliative procedure with the intent being to slowing down the epilepsy progression and perhaps illuminating the focus more clearly, without serious consequences.

The discussion devolved to a point where Maria was left with less than ideal confidence in the plan of care.  Dr. Von Allmen, frustrated, referred Luna to another pediatric facility.

Luna’s case I guarantee while perhaps not one-of-a-kind, is extremely unusual in presentation and overall path of care.

Using the MEG study from Houston, the team in Austin (Dr. Clarke and Dr. Lee) went to work.  They performed another 24hr VEEG and installed several depth electrodes.

The Austin team ultimately followed a similar path suggested by the epileptologist in Houston, which was a larger resection.

Prior to the surgery, I heard discussion about the ‘incomplete’ or ‘not optimal’ nature of Luna’s first surgery.  Parts left connected that ‘were missed’ according to accounts of discussion between the Austin team and Maria.  I struggled with the motives of this discussion.

These statements sounded like conjecture, a moment to elevate one’s self without any responsibility. That would soon change once they too operated on Luna.

I think in general Maria felt discussion with the surgeon in Austin was what she needed most.  I was not present, but the account of the conversation sounded very positive and reassuring.  It sounded extremely specific in what was ‘done incompletely’ previously, his plan to ‘fix it’, and a near guarantee Luna would be ‘seizure free without motor skill loses’.  Who wouldn’t want that in this little world, right?

[Luna’s case unfolded such that it appears Dr. Tandon was likely correct in his assessment during the second surgical consultation here in Houston.]

On August 14, 2015, Dr. Lee per his language, ‘completed the TPO disconnection’ in Austin, Texas at Dell Children’s Hospital.  Luna is such a strong little girl!

Luna Second surgery

Unfortunately, Luna seizures started again very soon after this surgery.  And Luna experienced severe hemiparesis, even 4 months post-op.

A corpus callosotomy (the palliative procedure rejected in Houston) and a VNS was implanted for an extra measure of control during a third surgery shortly after the second one.

After the corpus callosotomy, the seizure presentation was remarkably focal in nature. Only right arm and leg involvement during the events.

This was a big and positive change!  And it indicated, the right arm and leg were still connected to the motor cortex to some degree.

[Why the palliative procedure?  After all the diagnostics in Norway, Houston and Austin, no one could say for sure that the discharge activity onset was only in the left hemisphere.  This procedure could positively determine this with minimal deficits.  This procedure can stop or slow the progression of the epilepsy by closing the pathway between the hemispheres.  This procedure won’t stop seizures, but it can stop focal seizures from generalizing.]

The fourth round of evaluation revealed what we all prayed for all this time:  All discharge activity was localized in the left hemisphere!

Amazing!

Now, it appears complete hemispherectomy is Luna’s best option.

January 29th, 2016 Luna underwent total left hemispherectomy, during her fourth surgery.  This radical surgery enrolls Luna into a very small sorority of patients worldwide.

Luna Feb 2016-8985

[Epilepsy surgery timing is a research field all its own.  The mantra is ‘the sooner the better’.  But a misstep can lead to unintended disastrous consequences.]

What does this mean for Luna – a hemispherectomy?  No one really knows.

When you study the situation, the outcome is a spectrum, with underlying etiology being a big factor.  Therapy methods and theories are evolving as well.

Medical science can’t tell us why Luna’s left hemisphere produced epilepsy.  In this light, Luna, and others, are ahead of science to some degree.

 

Luna stroller february 2016

For sure, it means Luna will function with half a brain, unlike you and me.

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It also means Luna now can achieve her best ultimate outcome with the greatest chance at living seizure free and possibly medication free.

After 13 months here in the US, Maria is starting a new life.  She remarried and is moving to Dubai, UAE with her new husband Roman and plans a return to Norway.

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Luna Feb 2016-9057

Luna Feb 2016-9038

Luna Feb 2016-8980

Romans 8:31  “What, then, shall we say in response to these things?  If God is for us, who can be against us?”

I cannot lie, I found this time of Maria and Luna’s departure quite emotional.  Watching that remarkable video brings me to moment of profound clarity.  Where would Luna be today had I or someone else not responded to Maria’s call for help in the summer of 2013?

Thank you Lord for leading me into this family’s life.  Thank You Lord for inspiring Maria to post the videos of Luna when she did.  Without Your guidance and leadership, all of this would not have happened.

All of this from,

…a 6-minute video.

-Luna’s friend

4 Months Seizure Free and Counting…

4 months since complete TPO resection.  No seizures that we know about.  IMG_7229

This is great news.  6 months is the industry bellwether.  Seizure freedom 6 months after a surgical treatment to remove a seizure focus, generally indicates an upcoming long Honeymoon period if not permanent cessation of the seizures.  Specifically, it means the dysplastic lesion was completely resected.

Savanna’s blog has a new page titled “Savanna’s Story“.  I have re-written her story from start to finish.  It is not a compilation of the posts.  Those of you who have met Savanna along her journey or have known her from the beginning, I invite you to read her entire story here.  “Like” it, if you choose.

Also, I have upgraded the “About Savanna” page to an abbreviated timeline of events, with a little  commentary at the end.

Thanks for all your prayers, support, and comments,

-ken

Our baby is home….

Savanna was discharged early Friday morning and we made it home by lunch time.  It was so nice to be home, feeling like we were back in the driver’s seat again with her care.  Savanna is doing better, but still has a long way to go in terms of recovery.  She is in a lot of pain, and the best we have to manage the situation is ibuprofen and Tylenol.

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The past few days have been rough trying to manage her pain.  It comes and goes.  We seem to get good moments all of a sudden, and then as quickly as it became good, it deteriorates into crying and fussiness.  She has absolutely no interest in taking a bottle, so we are back to bolus feeds only (through her feeding tube).  This is tricky when she is just agitated beyond consolation, and squirming like you would not believe.

I decided to make some adjustments to her medication to see if we could level out her mood without too much sedation.  I split her onfi back to TID (tri-daily) versus morning/bedtime only.  And I went ahead and started scheduling .5mg of ativan, TID.  This has helped tremendously.  She is much happier and playful, with a lot less ibuprofen.  Once we get completely off the dexamethasone (steroid for her stridor), that will help this situation too.

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She used to crawl by doing a bunny hop, versus alternating opposite leg/arm movements and now she is crawling like a normal baby.  Albeit not much, but when you see it, it takes your breath away at first.

She is eating, like a horse.  She is chewing well.  Against normal intuition as a parent, dicing food into incredibly small pieces enables babies to simply swallow food versus chewing.  One piece just the wrong way and she chokes or her gag reflex kicks in full force.   So, the O/T (occupational therapist) we have recommended giving her larger pieces, so she would not naturally want to swallow the food, but rather naturally realize she has to chew it first.   Even if she doesn’t swallow it at first, that’s okay, she is getting over the aversion to having food in her mouth.  This has been a long process with Savanna.  A process that honestly, we almost didn’t even notice with our other kids – that is how different they are from a developmental standpoint.  It is all good.

One step at a time.

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These guys are three pees in a pod.  They are the best of brothers, and worst of enemies when it is time for ‘warfare’ – aka ‘playing wrestle’.

Tristan, Brandon and Austin are such good helpers.  They love to do things for Savanna to help us.  Most of the time, it is a good help.  Sometimes, like when Brandon wants to spin Savanna on the sensory swing, things get a little out of control.  I picture in my mind puke spraying out from Savanna splattering the walls all around her as she spins wildly…. and then… well, I slow down the swing of course.  We have yet to see the puke, but I have to admit, she likes to spin.

They just crack me up with their antics and how Tristan (the oldest) can interact so well with Austin, (Savanna’s twin and the youngest).  He is so protective and makes sure Brandon does not hit him too hard, or ‘wrestle’ him too hard.  They drive me to my wits end, especially when Savanna is having a really difficult time.  Then they turn right around and do things that are so touching and so thoughtful.

Sometimes, I have caught myself making parenting mistakes with Tristan (oops! did I say that out loud?)  Yes.  When my patience is the thinnest, I act my least thoughtful.  I am so focused on something going on with Savanna, that I have said things in a voice that was not called for, but was heard.  These situations are difficult.

Like seeing a picture you shouldn’t see…you can’t ‘unsee’ it.  Once I hurt his feelings, it can take a long time for him to get over it no matter what I do or say.

In the beginning, he (Tristan) would ‘forgive’ (more like forget) pretty quickly, but not now.  He is older and smarter.  His feelings are genuinely hurt, and most times it goes into the next day.

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I am learning as a parent and person in this situation.  It is still a work in progress, but this ‘stay-at-home’ situation is far more difficult than I thought it would be when I signed up for it.  With that, of course, goes the reward far beyond what I could ever achieve in any company, at any level.

Seeing Savanna learn and progress, knowing that I am big part of that effort, just can’t be explained in words.  There are not words that I can put together to characterize the emotions involved, especially since this last surgery.

So, overall, Savanna is progressing and doing well.  We are really anxious for the next few months to see what happens.  We continue to learn as a family what it takes to care for someone like Savanna.  More challenges are forthcoming.  Just as I have said before, God is preparing us for the future with the situations of today.

A special thanks goes out to Grandpa Squiz, Grandma Lou, Mama Barbara, and McKenzie and Eric for being there when we really needed help.  They allowed us to devote 100% of our time to Savanna during the operation and recovery period in the hospital.  Their help was a blessing.

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Breathe Eat Poop

Savanna Hospital Second Resection-5Savanna is three days post-op  since her second resection for epilepsy.  It is too early to tell about her seizure activity, but we see activity that is very concerning from our perspective.  We have been told to give it another 3-4 weeks before sounding the alarm.

Savanna is moving around now, and her demeanor is dominated by discomfort and irritation versus acute pain and rage.  This is good.

Still in the hospital, here we sit.  Like waiting in the in the airport terminal and the boarding status just keeps getting delayed.  Then delayed again.  Then delayed again.  Ugh!  The reasons to continue to keep Savanna in the hospital seem convoluted and mired in bureaucracy.  We know our daughter.  We understand their genuine concern.  But life in the PICU for someone like Savanna (and her family) is corrosive.  She is ready to go play, roll around, and be a slightly-delayed toddler.  The PICU was supposed to be a pitstop for her for the first 12 hours and then she was to be moved on to a more outpatient facility for monitoring and released 12-24 hours later.  What a disaster ensued when we were admitted.

The unexpected respiratory situation confined Savanna to the PICU for 3 days, tipping the balance of the our emotions resulting in a more combative relationship with the staff than one that needed to be harmonious.  She was having problems I admit, but looking back, the initial 12 hours of care should have been in the PICU and then Savanna should have been moved to the general floor for care.  Maybe some of the nurses/doctors out there who follow this blog can elaborate on what I am saying.  Or, maybe I am off base too, and by all means let me have it.  Comments are not moderated.  But, the PICU staff is used to seeing a certain type of clientele.  Being the #1 trauma facility in the region, most patients here are completely different than Savanna (and so are the parents).  By day 2 in the PICU, we had scuffled with 3 doctors and 2 nurses.   Looking back, I am not sure why it took 2 whole days to get in that much trouble – it could have been accomplished in less time.

The doctor overall in charge (the neurosurgeon) had been called twice in the middle of the night during the first two days too.  These unfortunate actions create an environment of  ‘us against them’.  The heat from the friction generated at times is tremendous.  And the sad part is that it didn’t have to be that way.  The thought that any one person knows 100% of what a baby like Savanna needs is naive.  I say that without callus and arrogance,  but rather a humble realization that the above statement includes me (the parent) too.  Near the end our crime and punishment in the PICU, I asked the neurosurgeon, “What is required for Savanna to be discharged?”  He replied simply “Breathe, Eat, Poop.”  He elaborated, but I thought it was a great title for a blog post.

I was ready to take Savanna home now, but I also knew a brief stay in the EMU was more or less required.  I tried to wiggle out of it, but the sentence was handed down and time served was not enough punishment.  Savanna’s journey continued in the EMU.  Luckily, the EMU is like ‘Celebrity Hospital for the Rich and Famous’ when you are there for observation without EEG equipment installed.  It can be a desolate place too with little to no activity at night – very unlike the PICU.  What is odd though, is just when you think it is safe to pick you nose, you realize you are being watched through the video system.   You are reminded by the sound of the discretely placed camera panning and zooming under remote control of the EMU nurses.  Haha.

Breathe.

No it is not a call for us as parents to take a deep breath, as though the person holding us underwater decided to let go and allow us to surface.  Savanna has to maintain Oxygen saturation level of at least 90% on her own.   Savanna is still struggling somewhat with stridor, which is due to the swelling in her upper airway.  This is a potential result of being intubated for nearly 12 hours.  It is not permanent, and will take some time to dissipate.  It has to be remedied or markedly improved for discharge.  As of now, the ENT doctor and neurosurgeon are happy with how she is breathing and feel this is not holding her back from moving forward.  We checked this box.

Eat.

So, with her right vocal chord not closing all the way, there exists significant risk of aspiration if she were to spit up or vomit.  It presents a delicate situation with respect to feeding her given the nausea from the pain medications and anesthesia.  If she is enraged and hyperactive due to pain, she could aspirate while feeding.  If she is sedated too much, she could aspirate while feeding.  Dilemma.  The neurosurgeon does not want her sedated post-operative as it could mask presentation and symptoms of complications.  But, if she can’t be safe in a crib that looks more like a cage more than a place to sleep, what is the point.  His answer is just to try to console her and redirect her pain with magic.  We need her to be manageable and not in so much pain.  We have four children, I can’t just sit with her for hours at time and try to keep her from being in pain with a little tylenol.  There should be some common ground here, and I feel like I am being fought tooth and nail about keeping my daughter healthy while not in a lot of discomfort.  And, the weird thing as this was not the case at all after the first surgery.  Maybe I am missing something as ‘just the parent’.  I don’t feel like an irresponsible parent, though there is some innuendo in the conversations with the different doctors.  I have to say it is not appreciated after all we have been through with Savanna.

How does this rant apply to feeding?  Okay, so if she really is in pain, it is really difficult to feed her.  Bolus feeding is difficult to impossible as well.  She has a G-tube, why can’t we just use that you might ask?  Savanna does have a peg style g-tube, but not a fundo.  So she can still eat normally as the stomach doesn’t have a ‘reverse flow’ limiter – if you will.  So even bolus feeds are in question now, which I think is reaching too far in maintain Savanna’s admission.  Even during Savanna’s most difficult times, sedated far more than now,  she never had a problem with reflux with bolus feeds.  But my historical experience as a parent is being ignored for the most part.  I know why, but it is still frustrating, especially when I am right.

So, I still have a speech therapist consult scheduled tomorrow to prove I can feed my child safely.  Amazing.  An empty box that I have to get checked.

Poop.

I wore my lucky ‘poop’ shirt to the hospital and Savanna had an extra dose of miralax this morning.  This box is checked, a couple of times over I might add.

Breathe, eat, poop.  Breathe, eat, poop.  Kind of sounds like a meditation phrase.  Let’s go Savanna, find your happy place!

-dad

Recovery from a Second Resection for Focal Cortical Dysplasia: Day One

Having taken care of Savanna after her first epilepsy surgery, I thought I knew what to expect.  I was misled by my own experience about the potential difficulty immediately post-op.  My bewilderment is like that of parent who’s first baby sleeps through the night in 3 weeks while child number 2 doesn’t even come close to such behavior.

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Savanna was very healthy during the procedure, but it still took nearly 12 hours to complete.  The craniotomy (opening in her head) was basically the same as her previous except one small relief cut around the vertex of the incision at the top of her head to allow more access.  The neurosurgeon told us that Savanna had significant scaring in the dura underneath the cranial bone which resulted in a longer procedure time than planned.  After surgery Savanna was extubated and began to experience difficulty breathing known as stridor (a wheezy, whistle sound when breathing due to constriction).  This resulted from her larynx and retinoids below it being very inflamed, which constricted airflow.  Imagine trying to run or workout while breathing through a straw only.

 

Throughout the night she was really agitated as the anesthesia wore off and the stridor kept her from breathing well.  Finally, after many doses of sedative and painkilling medicines, she really went downhill – so I thought.  I worried about her safety that night one time in particular.  Rebecca was right there and even though an RT was working on Savanna, things spiraled out of control – or so we felt.  Rebecca ran down the hall to find the critical care Fellow that was managing her case, and forcibly brought them back to the room.  I think there were some expletives used, as she too was really scared.

Savanna was in tachnycardia.  This can be really scary, but also normal presentation with a very agitated child.  For sure, with the stridor, the crying hurt as her throat was sore, and this increased her agitation, which results in more crying.  Add the constriction to the windpipe and you have a recipe requiring action.  With respiration rates in teens, heart rates in the 220-230 range, and bp north of 130, she was really unhappy.  They would administer a mg of morphine and she would settle down for about 10 minutes and then awake as though someone was cutting her leg off with a butter knife.  Then they tried something else, same result.  Everyone involved was amazed after several doses of versed, morphine, norco, the addition of precedex drip, there was still only minimal control of the situation.  Rebecca and I could not have been more relieved that we had clear contact with the doctors overseeing her neurological care.  They called the appropriate people in the PICU and we felt (even if it wasn’t the case) this made a difference in her care that night.

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Perspective:  Most likely, the Attending Physician, Fellows, Residents, Interns, and Nurses had not seen more than a few cases like Savanna’s in their lifetime.  And guaranteed, the only thing similar would have been the type of seizure disorder.  The etiology and child would likely be very different.  Some statistics for those interested in my rant on ‘perspective’ – (based on aged data, but the best I could find)

  • 4,000,000 live births in US according to census bureau.
  • 1:4000-6000 live birth rate for babies diagnosed with Infantile Spasms.
  • Result: 1000-2000 total babies are born that will be diagnosed with Infantile Spasms, total in the US.
  • The gender ratio is 60:40, boy:girl  ==  There is only 400 to 800 female babies per year born in the US like Savanna.
  • There are ~ 22 babies within 1000 square miles of Houston that are born like Savanna each year:
  • Ratio of 10-county (1000’s sq miles) population versus national population
    • 6,100,000 : 350,000,000 = 1.7% x total births = ~68,000 people born in this area
    • Factor in 1:5000 average, ~ 13 girls born that will develop IS.
    • Factor in 70% symptomatic diagnosis, 9 girls born who will develop like Savanna in Houston area (1000 sq miles) each year.
    • There are 2 locations qualified to treat this condition in the Houston area.

Even if you add to the number of children with FCD (focal cortical dysplasia) that did not develop IS (infantile spasms), it is a small number in any given year.  So from my perspective, Savanna is very special, even if some of the doctors who care for her don’t see her situation worthy of study when dropped into their lap.  I am not suggesting they ‘waste’ a lot of time understanding the her history, but more than a look at her current chart might give some perspective to her situation and our potential knowledge as parents.  But nonetheless, I find myself ‘just the dad’, with a perceived  ignorant and irrelevant opinion not worthy of anything except keeping to myself.  A situation that would seem to warrant 60 seconds of briefing by looking through her most recent neurology clinic output note devolves into one that they are forced to go back and look or face a difficult road.  I know other parents out there have felt the same way at times.  I can’t understand why the situation has to be so difficult.

The following day, we were assigned a nurse who deemed herself all-knowing as to what Savanna wanted, although she stated the opposite.  Her hypocritical actions combined with her statements didn’t sit well with me and we decided not to stand for it.  Rebecca took on the confrontational role and initiated a nursing change.  This particular day was stressful enough already and we did not need the attitude from the nurses treating us like we have never taken care of kids before or that it was our fault the night shift crew did not do the job just as she would have done it.

So here I am, shortly after the first 24 hours in the PCIU, realizing that my baby is probably going back to the OR in a couple of days to deal with the airway restriction that is hampering her recovery while facing the fact that I am confined to this location against my will.   To top off our sour experience, Dr. V arrives around 5pm to check on Savanna.  We had never even seen the attending physician that day even though they, ’rounded’ on Savanna.  No doctor or nurse even told us what ‘the plan’ was despite several attempts to find out ourselves.  Dr V, says she is going to contact the ENT she wants involved, then leaves the room to make the call.  Magically, the attending physician comes in to speak with us, and said he had already set up the consult.  Ironic.  Not sure exactly happened outside the room during those few minutes, but it just seems odd that all that happened within about 10 minutes.

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But, here we are, Second Resection in Recovery – Day 1

-dad

Sometimes, Less is More: Less Brain Tissue with Focal Cortical Dysplasia Is More Freedom From Seizures.

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We have been blessed with witnessing a profound new beginning for Savanna after her first resection at 13 months of age. Today, God willing, we are expecting a new child to emerge from the operating room again as she undergoes … Continue reading

Every Single Aspect of Savanna’s Life Is Better….

December 22, 2012

All seemed well for a trip with Savanna, so we head off to celebrate Christmas with the majority of our family in Louisville, Kentucky.  The drive goes pretty well, and we even went straight through from Houston.  The first half of the mileage consumed 3/4 of the total time, as it seemed we stopped at least every hour, for at least fifteen minutes.  Someone had a dirty diaper, or a wet diaper, or had to use the restroom, or needed something more to drink – (thus all the bathroom breaks).   My regulation as Dad was overridden by Mommy.  It was all fun looking back.  We arrive at Grandpa and Grandma’s house at around 4 in the morning, and it was really nice to be done driving, after 22 long hours.  Everybody seemed healthy, and we made it without any incident with Savanna.  Our arsenal of controlled substances and medical gear remained packed!  Savanna had already endured a difficult December winning a battle with RSV and a UTI, so we were looking forward to good times.

The next morning we get ready for a short trip across town to visit more family.  We got everyone loaded in the 18 degree weather into our Honda Odyssey.   We back out of the drive, and put it in drive, and nothing happens.  It was similar to when you try to accelerate on ice, only the wheels are not actually spinning.  The van doesn’t move.  Wait it’s in neutral, uh…, no it’s drive?  (Son of a b^%$&! I thought.)  Oh wait, then it starts to go, but it is already crystal clear something is seriously wrong.  Being a mechanical engineer, all the thoughts of probable cause for our lack of ‘going’, run through my mind.  No the car didn’t need some Flomax® (ha ha).  With some feathering of the accelerator pedal, the transmission seemed to function a little and we limped along.  Not far into the little trip to Mama’s house I knew two things: 1) this is about to be a real pain in the butt given its Friday before Christmas, and 2) regardless of how it unfolds, it will be very, very expensive.  After about 5 days, I bathed in the glory of being right, (okay more like drowned in the truth).

We managed to borrow a car from my mother which enabled our journey, but certainly highlighted the value and efficiency of a mini-van such as the Odyssey.  When you have a little army of small children, it’s just so much easier to make any trip.  We go on about our way, cringing at the fact that we just sold a 2011 Odyssey that was on lease to reduce our monthly expenses, and now here we sat with major problems with a 2006 that we thought would carry us along for the next year or so without major investment.  Oh well about that plan, Merry Christmas (and bah humbug!)

December 24th, Christmas Eve

We make it through the really long day of Christmas Eve visiting everyone possible.  We made several visits to family, attended a Church service, the Tristan, Brandon, Austin, & Savanna partied like champs, Savanna was laughing and playing, and then at 11pm, Austin vomited every single thing he ate that day (which was a lot apparently), and then some.  Gastroenteritis (stomach flu) had struck our family.  The next day Austin was a handful, completely uncomfortable as the diarrhea started.  We went through about 15 diapers in 4 hours before he seemed to be ’empty’.  That night, he continued with the vomiting, and then Savanna started exhibiting the same symptoms.   Austin is in the 75-80 percentiles for growth, so we were never worried about him missing some meals.  Savanna on the other hand, while physically healthy, these situations just tend to progress abnormally in terms of the overall effect on her body.  Since the brain surgery though, she seems to respond much more normally than we are expecting.  Despite the unpleasantness of being sick, it has been reassuring to watch her contract, battle and overcome typical sickness with normal response.  Literally, I have seen her start to get sick and almost without thought, I am packing a suitcase, thinking about how much of what medicine to pack, etc.  This process seems to be a thing of the past, and it is truly a blessing in disguise.

We wondered where he got it, as nobody else was sick when we arrived.  Assuming it was Rotavirus, you can think back a day or so before symptoms arrive.  For us, it wasn’t difficult to determine a culprit.  Here is a great ‘visual’ story.  Anyone that has taken a little child into a public restroom (specifically a men’s room) – anywhere – , knows it is such an experience.  Our boys want to touch and pick up everything in sight.  A urinal is like a new toy, and what is the really cool bright colored thing down inside?  Treasure!  Must have!  Yes sir!  A truck stop along a major interstate is the perfect safe harbor for viruses and a great transmission avenue.  I am sure he contracted it in one of these places, despite our best efforts to use the facilities without touching much.  Incubation period fits perfectly, and the first person affected in our family is currently the worst offender in terms of touching everything he shouldn’t.

(Rebecca) Needless to say, during the third evening in a row that Austin was sick, and the 1st for Savanna, Ken and I were each holding one of them, nearly dressed back in our Christmas outfits because everything else by that point had been puked on and was in the wash, about 3 in the morning, all we could do was look at each other and bust out laughing.  It was a time to either laugh or cry, and at that moment, we chose the high road.  Now there was something special about this stomach bug.  Usually, stomach bugs last for 24-48 hours at the most, but on Christmas Eve, the headline article on the front page of the “Courier Journal”, Louisville’s local paper, was “ER’s flooded with rare strain of 7-10 day viral gastroenteritis!!!”  By this time, we realized that Austin’s stomach bug was in the 72 hour range…  We had 2 more kids + ourselves to go and a 16 hour (more like 23 hour) car ride home.   Grandma, Grandpa, and Rebecca had already been struck by the virus, and fortunately seemed to shake it off in <24 hours, but we feared that the 4 kids would not be so lucky.  We decided that the best thing to do was to plastic wrap the car seats, put garbage bags on the floor, pack towels, and try to push through to drive home praying that we’d arrive without a car full of vomit and 4 miserable kids.(end sidebar)

The most disappointing effect from the illnesses, was that we didn’t feel comfortable trying to schedule more time with more of our family, knowing how this virus is very contagious for some time after symptoms.  We spent several days more or less ‘holed-up’ in Grandma’s house.   While this Christmas was different, as we weren’t living in the hospital, I had earnest plans to spend time more time with our family this year.  Savanna’s situation has changed me personally and this is one area I wanted to work on in terms of personal development for our family.  Never again are we going to be so far away from family during times of celebration.  Life is just too precious and for many, it is taken for granted.   There will always be another Christmas, right?  Yeah, hopefully so…  But there will never be another for us where we are thousands of miles from our extended family, alone, unless medically necessary.

We decided to head home on the 28th, as Savanna is starting to get worse in terms of not eating, and still vomiting.  We are performing basic urinalysis with the chemical strips and are monitoring the specific gravity of urine and watching it rise – meaning she is becoming more dehydrated.  We start inject water into her using the G-tube, and she continues to just vomit it up.  We are genuinely concerned at this point, and feel the need to be closer to her doctors.   We packed the van and while I was really getting nervous about her health (and whether or not the van was going to make it home), we left somewhat nonchalantly.

December 30, 2012, Sunday

An uneventful trip, but a speeding ticket a few hours from home just put the icing on the cake for this trip.  Savanna is really in trouble at this point and we can’t manage to keep any of the Ketogenic formula down, as she vomits is up as soon as we bolus feed it.  (Yuck!)  We spoke with her doctor, and decided we would stop the diet, feed her fluid with electrolytes and glucose which would stop the Keto diet.  So we started the Pedialite® fluid, along with others.  She seemed a little better the next day, so a trip to the EC was avoided.  This was the official end of the Keto diet for Savanna and this was really a good thing.   After a couple of days slowly pushing electrolytic fluids, she regained strength and recovered.

January 4, 2013, Friday

At my direction, the medical supply company came to pick up the infusion pump we had been renting.  A non-moment in terms of task related activity, but a huge mental rainbow as it marked the end of a difficult period.  It meant she could eat on her own, (all P/O – post-oral- feeds) without concern of aspiration.   It also meant, it could be difficult to feed her if something goes sideways with her health.  Yes, we could get another pump quickly.  Yes, we could bolus feed her too but it was still a significant mental moment for Dad.  It was quite emotional and difficult to explain why.  We have learned to appreciate each small victory.

We decided to use some skymiles from our Amex account to fly in Tristan and Brandon’s previous daycare family Mom and daughter (Irma and Paige) from the 4th to the 7th.  Tristan became really close to Irma during our most difficult times in CA with Savanna.  Irma was a second mom to Tristan and Brandon, and in many ways, to Rebecca and me as well.  Tristan made the connection more than Brandon as he was older.  He can often be heard saying he wished he was at Irma’s when things aren’t the best at our house for some reason during the week.  He is convinced the money in his piggy bank is enough to buy him an airline ticket back to CA to visit Irma for his birthday.  Some of these lessons are difficult…  Anyway, it was a great visit and we thank her greatly for giving us some time.  She always said she built walls around her so she would not get too attached to the kids as they are with her until school starts, and then she typically doesn’t see them anymore.  For some reason, Tristan broke through that wall.  We call every now and then and Tristan still has a desire to see her.  Rebecca and I were actually able to go out that Saturday night while Irma watched the kids.  We danced at a studio operated by someone we knew from many moons ago back.  It was a great night.  Hopefully, we can figure out how to make it back again in the future.  We have mentioned “angels” who have helped us through this journey, and Irma, Paige, and Kevin Fisher have truly been that to us.  They haven’t just watched the kids, they have become real family to us too.

January 16, 2013, Saturday

Savanna had an excellent couple of weeks and we learned more about how to help her with her motor development.  She smiles regularly and is very engaged with her surroundings.  We have been working hard on the transition from laying to sitting up.  She sits up well now if you position her, and is getting stronger every day.  On January 16, she was admitted to the hospital for an outpatient 23 hour EEG/LTM.  This was a monitoring session that will help us to see how she has responded to the surgery.  The anticipation of the results was intense.  While we thought we would get a couple of moments with the doctor to just go over what they were seeing, it was an outpatient procedure, and the doctors don’t round on those patients.  It was very deflating for me, but if there was ever a time when the age old adage applies it is now: sometimes no news is good news.

The next two weeks were filled with joy in the house.  It was clear that every single aspect of Savanna’s life is much improved after the surgery.   On January 19, Rebecca was feeding Savanna, and managed to get her to eat one and a half containers of baby food.  Savanna did not fight it that much, and at times seemed to enjoy it.  This was a milestone for her, as it had never happened before, or it had been more than a year since such activity.   Later that weekend, Savanna was eating Cheetos and cookies with aggressiveness.  It was such a great sight to see.  Rebecca was outwardly excited and emotional, while my emotion was a little more inward and guarded though just as strong.  We were at our friend’s house, Eric & McKenzie Montague (more angels), and Rebecca jumped up and down more excited than a little kid at Christmas.

January 30, 2013

We were in the middle of our morning workout session on the 30th, and Savanna was really happy and engaged.  Then suddenly while I was working with her, it looked like she could sit up on her own.  I stood back, and like letting your 4 or 5 year old go on the bike with no training wheels, I let her go.  She went from her back to her belly, then to sitting up in many, jerky uncoordinated movements – but she made it!  She was so happy with herself, and so was I!  The vast majority of children blow through this milestone with little input from the parents or knowledge with respect to the mechanics involved in the movements.  It was a really rewarding experience and I felt like all the effort and sacrifice in terms of time not spent with the other kids was paying off.  I only wish Rebecca could have been there to see it happen.  She did it again later that day during an afternoon workout session.    This experience has shown me that most major milestones like this are going to take a lot longer to achieve for Savanna.

January 31, 2013, Thursday

Our Au Pair exchange student, Andrea Ramirez, that was helping with childcare had gone home to Columbia on the 23rd, so I was on my own during the week now.  What a change for me personally.  Anyway, I had some apprehension about taking all four kids to the doctor appointments, especially the epileptologist, as they tend to require several hours of waiting before you see the doctor.  I went for it, as I could not find help that day for the kiddos that I felt was affordable.  We arrived early for the epileptologist appointment to get the reading from the EEG.  We waited in the waiting room for about 45 minutes and then we are called back to the room.  I have a double stroller, plus the two older kids.  The exam room became really small, really quick.  The wait in the room was two and half hours before we saw the doctor.  This is typical in this field of practice.  Two hours in, I had run out of all of the food I brought.  There were remainders of cheerios, grapes, blueberries, cheetos, and raisins on the floor….multiple cups of water consumed by each kiddo, because ‘Daddy, I’m thirsty’ – and the resulting numerous trips to the bathroom… (public restroom from above again, yes!)

Then finally, the doctor appears.  The conversation was swift, as it was clear the majority of the family was completely done with being there in that small room.  Savanna had finally given up and fallen asleep in her car seat.  Then I got the official news…

Unfortunately, as we suspected, Savanna is still having seizures.

While not a complete shock, it was definitely disappointing and difficult to hear nonetheless.  Apparently, a single seizure was recorded during the 23 hour monitor, which was much like the previous ones but clinically so subtle I didn’t notice it as the caregiver in the room with her.  The good news is that the onset is right where they thought it might be given the abnormal tissue that was left intact after the first resection.

(Rebecca) While Ken was amazingly managing all 4 kids at the doctor’s office waiting for Savanna’s test results, I was driving home from a 4 day business trip to Louisiana.  We were connecting by phone, as I had hoped to hear the Dr’s comments directly.  The few actual minutes when a doctor walks in to speak with us can be a whirlwind.  So in this instance, I wasn’t able to listen in.  Ken and I only talked briefly after he got the news, as he had to let me go to manage the kids.  After hanging up, my heart just sank.  You try to be realistic despite the emotion involved.  When the doctors came in after Savanna’s surgery, and told us they hadn’t been able to remove all of the dysplastic brain area, I remember somehow knowing that this is the path we would eventually go down.  But, I had always hoped, like every parent of an epileptic child, never to hear about another seizure.  The guilt of being a working parent in this situation, and not being there with Savanna…with Ken…as he received this news is just impossible to describe.  (end sidebar)

So now what?

First, review of the expert side opinion.

We knew that abnormal tissue was still present when they closed the first surgery.  Savanna had blood-gas saturation levels in the 60%’s at the end of the surgery (not good), lost half of her blood supply, and received a couple of transfusions – so it was time to call that procedure done.  We were advised a second surgery is highly probable.  When?  Well, it is difficult to say.  Savanna will tell us when.  If her development is progressing well, and there is good seizure control with medication, the operation is on hold indefinitely.  This is just as simple as it gets.

Second a review of the non-expert opinion (the mom and dad theory)

The abnormal activity was greatly reduced after the first 2 resections (the first procedure) and we are now seeing a rapidly increasing pace of her development, globally.  We could not be happier with her progress and without a doubt every single aspect of her life is better, far better.  Could the second surgery be planned proactively?  Will it be as difficult leading up to the next surgery as it was with her first?  It was hard news to hear that seizures were still present, and there was a moment amidst the craziness that the world seemed silent around me.  I didn’t know what to think, how to act, or how not to act, what questions to ask.  Questions, we had many, answers we had few.  This sounds odd, as one would think I would be comfortable in this situation given the constant exposure.  I couldn’t remember what questions Rebecca and I had for the doctor and all I could do was listen and absorb.  It was a disappointing performance from a parental perspective as I re-live the visit.   Looking back, it was not a good decision to take all the kids with me to the clinic visit.  A ‘do-over’ would result in at least the older kiddos to be dropped off somewhere trusted, regardless of the cost.   At any rate, nothing changes with her care except the change of AED medicine.  (No big deal right?  Wait for the next post.)

The new plan…

Our doctor wanted Savanna to start on another AED, and titrate down the Sabril®.  We had already started decreasing the Sabril® on our own anyway since she was doing so well and initially on a really high dose.  Then it is really a wait, watch and see event as we watch Savanna’s developmental progression.  Like she did in the past, she will let us know when the situation needs to be addressed or when she needs help.  I only pray that process is not as difficult as the first time around.

I called Rebecca on the way home from there to give her the news and I could tell she was going to cry, and I learned later she did.  She had many more questions right away than I did, and I think Savanna would have been better served that day by having Rebecca in the office than me.  It was a sinking feeling not having the answers to Rebecca’s questions, but even more so, knowing that I simply didn’t ask them at the time.  This would be a good time to be able to use a mulligan.  It has resulted in my reflection on my ability as a father and caregiver. Am I really the right one for the job?  She is so strong, intelligent, and beautiful.

I meet Rebecca at home with my car load of craziness, and amidst the reunion of mommy and kids after Rebecca’s 4 day trip, Savanna sat up on her own again.  This time, Rebecca got to see it, but it was really a bittersweet moment to say the least.

Saturday, February 2, 2013

Savanna is really having a hard weekend.  We suspect a UTI, and a basic urinalysis indicates high levels of white and red blood cells in the urine.  Classic sign of a UTI (even without presence if high levels of Nitrites), especially since she has been screened for most all other types of abnormalities of the UT.  We start a coarse of antibiotics, as we keep those on hand now just for this occasion.  The rest of the weekend and majority of the next week is really difficult as the pain seems to be high with this type of infection.  Conventional pain medications don’t seem to help, but Uristat® seems to ease some of the pain.

Tuesday, February 5, 2012

Savanna is teething something fierce and really having a hard time.  Pain medication is barely affecting her, and then she starts having seizures.  My heart sank.  I was very concerned with what I was watching.  Could teething pain provoke such activity?  We already knew she was having seizures, but didn’t really know if pain would trigger such an episode.  Administration of Ativan® interrupted the seizure activity.  The situation wasn’t emergent, but why let her seize unnecessarily if the trigger was teething pain.  We had the drugs, experience with administration, knowledge of side-effects, and the inclination to use them more quickly than not.  Somehow, I think knowing the history and the fact that she is our child creates bias – (no way!)  Most doctors would probably not have given what I did, but I lost no sleep over my decisions and got reassurance in the decision from the epileptologist later.  Rather, I lost a lot of sleep wondering if this was the next step in her journey and it was now ‘go time’ for the next procedure.   Was this the beginning of the end of her second honeymoon period?  No, not yet…  By the weekend, the situation had calmed considerably and I got much needed support from Savanna’s epileptologist.  We felt much better about Savanna’s situation, and she returned to being very engaged, happy, and moving forward again with life, a scare, but not a setback.

Some additional commentary:  Just when you think it is really bad…

Savanna is under the care of a number of specialists, such as a Pulmonologist, pediatric GI doctor, etc.  They usually have clinic days that typically consist of a very short period of only a day or two per week.  Just when I think our situation is really difficult, I have one of these specialty clinic visits.  The wait times are long, regardless of the type of doctor you are seeing.  On the 28th of January, there we were, waiting around 12:30 for our 1:30 appointment (a little early).  Then the procession of other patients starts to arrive as they fill all the exam rooms at 1:30.  Most have unimaginable disorders and some have traveled hours to be there.  These kids have disorders that render most of them to highly specialized electric wheelchairs or special strollers.  Most of these kids have disorders that will end in death long before their parents.  (Savanna may still may be in that category.)  But, Savanna is so young, that in her car seat, it is difficult to discern the severe nature of her problems just yet.  This world, while great if you are healthy, is very cruel if you are not.  Sometimes I continue think about why God allows such terrible situations to exist and the answers remain mysterious.  The suffering these kids and families endure is amazing when you talk to them first hand and begin to picture what their life is like.  I have realized through these clinic visits, that our life and situation isn’t so bad, especially now.  Every time I think our situation is ‘so difficult’, I remember what I saw in the waiting room during our last ‘specialty clinic’ visit.  Few people I meet in the waiting room of the ‘specialty clinic’ have such an opportunity for relief as Savanna was afforded, and I can say after her first procedure, every single aspect of her life is better.

Happy Valentine’s day,    

-Ken and Rebecca

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