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

Posted on March 1, 2016 by kenlininger

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.

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.

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.

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.]

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!

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

[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.

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

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.

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

Three Years Later

Posted on December 10, 2014 by kenlininger

If I could have a conversation with Savanna, who has ASD (Autism), and she could magically understand things the way we understand them, just for this conversation, it might sound something like this:

Me: Savanna, you can play with all the toys in the bins, not just dump them out and throw them.

Savanna: What do you mean, the toys in the bins are not just for dumping on the floor?

Me: (Silently operating a 6-12 month random baby toy in front of her, for the millionth time…)

Savanna: Oh, I see! Can I try?

Me: Savanna, not every hole and fabric loop is for inserting your finger. But it is okay if you do.

Savanna: Daddy, I wish I could stop doing that, but I just can’t.

Me: Savanna, the words I say aren’t just weird noises, they actually have meaning. They can even be put together to form what we call sentences that convey thoughts, feelings, instructions, etc.

Savanna: Oh, I just thought you just like to hear yourself make these noises.

Me: Savanna, the food we serve you at the dinner table is actually for eating, not throwing.

Savanna: Oh. Why didn’t you tell me? Maybe I will try to eat some of these things. Daddy, how come the other kids throw the food too?

Me: Well, … its complicated….

Savanna: Daddy, you mean it is difficult to do your daily tasks when I insist that you hold me all the time? You say your back hurts, why didn’t you tell me?

Me: Savanna, I tell you this everyday.

Savanna: Oh? I never understood that before.

Me: I know.

Savanna: Daddy, why do you get so excited when I put a square shape into a square hole? It’s not that big of deal you know.

Me: Well, I think it is really great and I was actually trying to get you to do it again by showing positive praise.

Savanna: Oh? Sorry, I just didn’t want to do it again.

—————————————–

Savanna’s (special needs) Journey formally began December 19th, 2011 when she was diagnosed with a seizure disorder. Her disorder took away her normal life by arresting development in every way. A symptom of an abnormality in her brain, her disorder proved medically refractory. At 18 months old, she underwent a left TPO resection (removal of ~70% of the left hemisphere of her brain) to mechanically control the seizures, which it has done. It has given her a second chance at life; her best chance. Three years later, here we sit.

I find my situation surreal today. But it is very real. Three years after it all began, I administered the final dose of her last medication. What does the future hold for Savanna with regard to seizures? No one knows for certain. She is nearly 21 months seizure free and not on medication, indicative of a long period of control in her future.

Three years later, I find myself learning how to teach in a whole new way. I never thought I would know so much about physical therapy, occupational therapy, speech therapy, and ABA. She is 39 months old, functioning at about a 2, maybe 2.5 year old level, far less with regard to expressive speech. This sounds not so great, but my heart is filled with joy! She makes strides everyday.

The power of prayer and His word have led us to this point. We made faith-based, selfless decisions and accepted the risks and consequences in search of the best chance for Savanna to thrive. God was holding our hands and leading us even when we couldn’t understand the path or the advice clearly.

While she is now considered “normal” by many, Savanna’s brain development is different from a typical child. The way different parts of the brain work together is possibly different. These differences are difficult to measure, as is the effect of these differences on her ultimate outcome.

Yes she is “smart”! She is learning in her own way, on her own schedule.

She is learning to speak! It has taken nearly 2 years to get her to appropriately say simple words like Yes and No. She loves the power in the word no, and it has become her standard response to a question before reconsidering then following her “no”…”yes”. She has a mixed receptive/expressive speech disorder that when coupled with her cognitive delays makes communication very difficult at times.

I remember when Tristan, our oldest, was about 1 year old. We would drive around and he would say “bus” when he saw a bus of any kind. It was a very short time until he understood “yellow school bus”. Savanna just started to say “bus” in the car when she sees one. And often times, she only says “yewow (yellow)”. She knows the golden arches well, and says “pway pwace” every time (unless we pass it on her blind side). It is very reassuring feeling as her parent to hear this, as it means to me, she is learning.

I sat down to write this long-overdue post, and realized so much life had been lived since the last post. Maria and her daughter Luna visited from Norway for three months while they sought treatment for Luna’s epilepsy. While they were here, my mind just paused. Every thing just kind of slipped away and it has taken some time to remember all that transpired. A few highlights…

She and Austin celebrated turning 3 recently. On their birthday, Savanna decided to take us for a walk. She took each of our hands and proceeded to pull us along. Rebecca and I were beside ourselves, as she had not ever done anything like that. She said “Hi” to the camera when prompted. It was amazing.

She really enjoyed being part of making the cakes. She cracked her own eggs with very little help. It just warms my heart seeing her understand opening a wrapped gift, not a simple concept I have learned. Turning three meant she “graduated” ECI. And should we continue with publicly funded help, it transfers to the public school system. Her awareness of and participation in her environment is so dramatically different, it is difficult to compare to one year ago.

Celebrating Halloween this year was a little different for her. She participated, a little. But the experience was bit overwhelming, and she is always exhausted at the end of the week from the intense ABA programming.

We went to the Second Annual Pediatric Epilepsy patient/caregiver reunion. She was much more active this time. Her neurosurgeon helped her put her shoes back on after exiting the bounce house. How cool is that? I think he was a bit amazed how well she is doing, maybe not. Definitely, it reinforced the decisions made in her case. Some pictures and a link to a youtube video made by the hospital (I have short spot in it…)

Link to CMHH video of the 2nd annual pediatric epilepsy reunion.

We supported Maria through Luna’s surgery and visited Maria in the hospital a day when Savanna’s school was closed. Savanna walked all the way into the CMHH pediatric day surgery waiting area from garage 5. She has never done that; I had to take a picture.

We went to Galveston beach at the end of October, and she loved the water. It was still warm, and quite clear this day. She enjoyed the waves, noticed the kites flying, and was interested in “helping” build a sand castle. We love Houston!

She enjoyed Brandon’s birthday at Chuck-E-Cheese, especially the large indoor play structure. We almost couldn’t get her out of that thing!

These activities have all been more fun (for her and us) since starting ABA. Her expressive language development has exploded. She talks to us now. In general, she can express her feelings in words. I admit, we don’t always understand her approximations, but its a start right? But for sure, she wants to talk; she can talk!

She has many words now, when just a few months ago she had none, no fault of the speech therapy she received for 24 months. She wasn’t ready then, simply put. The therapists always said the information is going “in” and one day it will all come “out” in words. They were right.

Rebecca recounts reading the ABA plan with Savanna’s “6 month goals” before she started ABA. There were goals like “say 20 single syllable simple words” and “make 5 animal noises”. She was scared that might not be possible in just 6 months time. A miraculous 2 and 1/2 weeks later, Savanna was talking! She’s blown past her 6 month goals, and we are convinced that she will be able to do anything she decides she wants to be able to do. Amazing!

She puts 2-4 words together now appropriately without prompts. She is able to parrot many words we say, even if she doesn’t understand them. She struggles with certain phonetic sounds, such as the “k” sound, like in the word bike. Her approximation isn’t even close on this one. The speech therapist notices this and feels she will get it, but it is just not happening as quickly as the other sounds.

We believe the ABA has helped dissipate her OCD behaviors and it has certainly smoothed transitions between activities and people. We are more cautious now of situations that can derail a “good mood.” And during ABA (40 hours a week) she has 100% 1:1 supervision, which helps redirect her when she starts such behavior.

Sometimes we exploit the OCD behaviors at home, for example if she doesn’t want to get dressed. You can almost always find a top or dress with a bow on it, (with a fabric loop), show her the loop with your finger in it, and she will usually be okay with wearing that outfit. I am sure some out there would say this is a bad idea. At this point, I am picking my battles.

Sometimes, this happens…

Suddenly she was very upset! What happened? What was wrong! She demanded I hold her – or else meltdown. I went about my work (holding her) which is usually enough.

She refused to be happy.

She refused to use words.

She cried, then she screamed.

She was a snotty mess. I didn’t know what was wrong.

Finally, I figured out the pantry door was open and the light was on. This bothered her terribly, but she did not want to go take care of it herself for some reason. So, 15 minutes of complete breakdown, for what? I don’t know.

And, that’s the point, sometimes we just don’t know what is going on in her head.

She does eat without throwing (too much) these days, though her diet remains severely self-limited. If you serve her fruit (except watermelon), she will protest, period. If you are adamant she try it, you should be aware of imminent flying food. I guess this is somewhat normal?

Oh those shape sorters. She understands shape sorter toys these days. But, conceptually does not understand shapes via their names.

These days, we work more on color matching. We sort 2D and 3D objects, matching like items. We work on letter and number identification. All of these tasks start simple, with organized aligned rows of items. But the goal is for Savanna to sort/match items from a random unorganized pile, with only minimal prompts. She has done so well, so quickly, we now work on puzzles too – a much more complex challenge.

Her fine motor skills increase daily it seems. She can thread a very small string though really small beads, or holes in a board. She can cut paper with scissors with some help and prompting. (wow! right?)

She can randomly draw with crayons/markers for a couple of minutes. If you prompt enough, she can make horizontal, vertical lines, and circles.

If the other kids want to color, she too is interested. I set up a space for her on the table, but she usually loses interest quickly. This situation is disappointing and frustrating to watch.

A character trait you cannot teach: Desire to Independently Learn. Yep, she has that one!

It is glorious! It is almost to a fault though as she refuses hand-over-hand assistance.

She has the ability to sit and focus for a long time with a therapist or with us as a parent. But make no mistake, it is very hands on. You have to be right there to keep her on track. The minute you think you can step away, well….

ABA has indicated she is ready for potty training. They don’t want to wait since she has exhibited so much interest in using the bathroom. We have seen this at home too, and have agreed to work with the ABA center to develop a routing for Savanna. We are excited about this addition to her routine.

She can play with her brothers for longer periods of time (without me!!) on a somewhat regular basis. She will do this when the activity is something she prefers. She LOVES the trampoline and the swing set.

She loves her platform swing too, and this has become a staple in her sensory diet – every day. Not sure how our annual trip back to the VA and KY (where this won’t be available freely or otherwise) will unfold without this resource.

She can jump now! That’s right, leave the ground with both feet at the same time (and land appropriately)! I know it sounds trivial, but this is very difficult with children with proprioceptive deficits or dysfunction.

Austin and Savanna play the “rib-bit” frog jumping game sometimes. I think it is their own language, lol. And she jumps! The trampoline is very helpful for developing her leg and core muscles. We also use the Sure Step SMO braces to help correct the pronation in her feet. A recent gait analysis showed significant pronation, poor symmetry, and wide gait indicative of low balance and tone in her core.

The scientific evidence shows we need to work diligently at correcting her gait before the age of 7, after which correction is more difficult.

She loves to get in the middle of the action with the boys. When she is in a good mood, she is right there with them. She is often the first one hurt and crying. I generally push her back “into the octagon” and gently give the boys some additional operating parameters. A pillow fight for example, is a great way to experience a lot of heavy sensory input.

Here is a video clip of her swinging. A few months ago, she could barely push the swing around and still hang on. She is a bit tired here, but manages quite well. Important to note is that she understands to get near the center of the platform to reduce the centrifugal force from spinning. It is there she can comfortably sit and eat, or whatever (notice the chips sitting in the center). She loves her swing!

This next clip is hilarious. A good use of the physio ball (not found in the instructions!) It builds leg and foot strength, increases core strength, and increases balance and spatial awareness, all with a little fun. The amount of input from the adult greatly changes the amount of input from the child. You can plainly see the differences between the Austin and Savanna here. The motor planning is happening for both, but the results with regard to timing, strength, and overall execution are vastly different. Still, she has come so far! And I am proud!

Once again, they conspired, as they usually do on a rare quiet Sunday afternoon. We were busy cleaning around the house like a bumble bees and suddenly the eerie silence is softly broken by intense laughter from outside? Uh-oh. They snatched the physio ball from the official place of storage, and managed to get it on the trampoline. Yeah, that’s right, as if the trampoline itself wasn’t enough.

I would like to say “Thank you” to all those who have helped us with Savanna’s care. Without your input, your guidance, and your wisdom, Savanna would not be where she is today.

Dr. Gretchen Von Allmen, Dr. Mary Zupance, Dr. Nitin Tandon, Tammi Rainwater with MHMRA, George Michel with MHMRA, Nikki Dupont with Reach Healthcare TCG, Dana and Jennifer at The Speech Emporium in Cypress, Dr. Allison Arthur with TCPA, and all the therapists at Tangible Difference Learning Center in Katy. To our parents and family, Thank You for supporting us.

Thank you for your interest in helping Savanna; helping us as a family. We greatly appreciate it.

Have a Blessed Christmas,

-Liningers

Dare to Dream

Posted on August 7, 2014 by kenlininger

1 year, 4 months and 7 days since the completion of Savanna’s TPO resection for seizure control.

Savanna recently had an MRI to examine the surgical site in her brain and a 23 hour VEEG to observe and characterize events and behaviors for a 12-month post-surgical follow-up meeting with her neurologist.

Happy! Happy!! Happy!!!

The MRI impression is that the surgical site appears unchanged from the imaging results at 6 months post-op. No signs of problems related to the surgery or anything additional elsewhere in her brain. The EEG revealed that concerning behaviors were not related to epileptic activity.

Just playing around during my EEG.

Yeah, I have done this before…

MRI day. Not a happy camper.

This means she is what we call seizure free (SF in the internet world). In the medical community, Savanna’s outcome is still Class 1a on the Engel scale. This is as good as it gets in terms of seizure control. Awesome!

The Shape Sorter. Again. Again. And, again.

The Shape Sorter. A must for a toddler during a 23 hour EEG.

Well, what does this mean in her everyday life?

It means the power of prayer is real. I believe God works through us all. While He allowed Savanna’s suffering, He answered collective prayer through actions of her doctors inspired to empower their God-given intelligence and ability.

It means we are witnessing development which we might not have otherwise.

I moved the stool myself, and turned on the light!

It means we are witnessing His divine power as Savanna climbs out of the valley of global sensory reintegration created from forced normalization of her brain activity and subsequent reorganization of the neural network. Savanna thankfully exhibits a persistence and perseverance that you cannot teach. At times, she wants to learn; she wants to show us things; she wants to exist in our world. This is when the camera comes out, because we knew it was in there and we are so happy to see it come out. And then at other times, it is just difficult. She disconnects, and seems to wonder aimlessly through her own world on a path I just cannot see or understand.

For the first time…

For the first time, she is eating and drinking on her own in a manner that is adequate to allow her to thrive. Six weeks ago we removed the feeding tube. Savanna is now 100% orally fed. She is able to try food in larger quantities and react to the textures, smells, and tastes. For the first time, I have been able to really hone in on how her diet affects her mood and behavior.

For the first time, we are observing fine and gross motor skills that are close to the low-end of normal. If you were to see her on a playground, you would not immediately see a difference between her and other children her age. But, it is there, lurking just beneath the surface of awareness for not only the casual observer, but also for her. It is significant, and it is serious. Sometimes these complex deficits rear their ugly head with an unusually awkward fall, or sudden, invisible playtime-ending problem. In general, I am highly in-tune with Savanna and plan accordingly. But recently, for the first time, she is often perceived equal in ability to her twin brother.

For the first time, we are witnessing dramatic acceleration in her receptive communication. (This is where she understands simple instructions or auditory communication.) Her cognition is improving daily, which allows learning new sign language at a quicker pace. At this point, the communication is her biggest barrier to a happy life (for us too!).

For the first time, we are seeing times of appropriate social interaction with her siblings. She exhibits persistence worthy of saying she is ambitious in catching up developmentally. She has moments of appropriate empathetic reactions to others. At times she is even deliberately mischievous, just like a normal 2 year-old! I see her interacting with her twin brother like I have not in the past. They sneak out together after breakfast to get on the trampoline, or play crazy games of laughing out loud and running from wall to wall in the house. It is so awesome to see knowing what I know, and something I was beginning not to expect at all.

A short video clip of Austin and Savanna on the trampoline… Austin can open the door, so they conspired, snuck out unseen. Savanna’s compulsion with closing doors meant, I didn’t immediately notice they had left! Then, I saw them. I saw playing. I heard laughter. I felt the need to start the camera.

We recently went on our summer pilgrimage to the homeland (KY and VA). This has normally been quite a trip for us, slathered with worry (from me anyway) and hampered with impediments related to Savanna’s condition.

For the first time, I did not pack a suitcase full of DME (durable medical equipment), or supplies related to tube feeding. I did not pack a duffel bag full of medication, most for “What if?” scenarios.

Extra Goldfish, Pringles, and apple juice replaced cases of enteral formula (not exactly a nutritional even trade, but I will take it!) I did not pack back-up stuff for back-up stuff. I took 2 oral syringes, a bottle of prescribed medication, and some rescue medication for good measure. For the first time, I did not install the roof box to carry all the extra stuff. It actually seemed kind of easy and normal travelling on an insanely long 3400 mile road trip.

Savanna had many great periods and days on the above mentioned “vacation”. She did have a few bad days too. While most want to see her as ‘normal’, this is something I just would never mention about our other kids knowing what I know now. No, she didn’t seize, but once we lose the happy place, it sometimes is difficult to get it back in the same day. On those days, all the best laid plans start to boil down to “Who is going to hold Savanna?” I know it sounds simple, callus even to the reader/parent who might think it is not a big deal – holding a child. And to those readers, you are right – I am whining. But, I think the analysis and commentary are relative.

Rebecca and I are both still somehow in ok shape after this journey. We are experiencing the physical woes all too familiar to parents of special needs children. Holding Savanna is not difficult per se`. Holding Savanna for extended periods standing up, moving around, trying to complete the normal tasks of life, all while cantilevering away to balance her (as she naturally leans away instead of into us) is a different ball game altogether. Our backs are paying the price, and we are more conscious now, giving each other time to workout regularly. We find the workout routine is less about vanity or leisure, but more about necessity to build muscle to heal and mitigate current problems and perhaps delay further injury.

Here is a very short clip of her on the tube on the lake.

Do we dare let ourselves dream about what might be for Savanna? Before we drift off into never-never land, I would like to share some other “firsts” during this last few months.

For the first time, I can see the emotional derailment and predict the ensuing behavioral train wreck with good precision and fair accuracy. More clear now are the signs of disengagement from her environment. She loses all interest in things right in front of her. She shows no interest in many items that typically soothe her, except a very specific blanket that has a magical calming effect. She will not just want to be held, she will demand to be held. And if you can’t (or won’t), the path to the train wreck begins.

The path has stages and factors that affect how quickly you arrive at your destination, which is the behavioral train wreck or urban term “meltdown”. She will whine first, that kind of “I’m not happy whine…” The whine becomes a cry. This process can take while, but we are learning it can also happen quite quickly.

The train has derailed at this point. I have learned it possible to avoid the worst outcome if I intervene appropriately.

The cry leads to stumbling or stammering like a drunk all while dragging her blanket. She will either 1) run into to something like a cabinet corner, wall corner, or door jamb on her right side (where the dense hemianopsia affects her) or 2) fall down hard by tripping on the blanket or something in the sea of ‘things’ on our floor that seems omnipresent. This leads to the scream of “Hold me now!“ and that of “Damn that hurt!”

By this point, I have modified my plans for the day somewhat, and am holding her (sitting if possible).

It is difficult to recover from this series of events. I try to push through some days and it ends up generally alright. But, sometimes it doesn’t go so well. Sometimes this process takes 5 minutes. And, other times 5 hours. It really depends on things I am not knowledgeable about yet – or I would have addressed them intelligently.

At times I witness her eye deviate to the right (opposite what was observed before the resection) and this tempers my elation about her future. Diagnostics (mentioned in the beginning) have indicated nothing abnormal about these behaviors, but it is troubling as a parent given the history.

I joined the support group that our epilepsy program sponsors and attended meetings for the last few months. If you want a dose of reality as a parent in my shoes, this is the prescription. This group has been a great find though, as I learn more each time I attend.

For the first time, Savanna was evaluated independently (without me present) for ABA therapy, ST, and OT through an ABA provider locally here in Houston. ABA is Applied Behavior Analysis, and a method of intervention and therapy for those suffering the symptoms of ASD (Autism Spectrum Disorder). It sounds weird, but I liked it and I disliked it all at the same time. I knew she would struggle with new people and new activities. But, I also knew it would be a very good judge of where she is functioning with respect to interacting with the public.

The Preschool Language Scale (#5) and Functional Communication (Revised) Profile toolkits measured Savanna’s communication abilites, and resulted in a mixed scores (all pretty low) higher/lower for receptive/expressive communication respectively. This agreed with the evaluation results done by ECI at 32 months. The Verbal Behavior Milestones Assessment and Placement Program toolkit showed the way for an ABA therapy plan, recommending 35 hours per week of this type of therapy. OT skills measured using the The Peabody Developmental Motor Scales where she qualified for applying the Beery-Buktenica Visual Motor Integration subset revealed a score just a tick below normal in one category but, quite low in another. A calculated quotient score result was “poor”.

I received the reports in the mail and was not surprised at their conclusions, I was disappointed though, I have to admit. I interpret their conclusions with caution though, as none of the therapists were familiar with Savanna. But, this was a first: Savanna on her own for about 3 hours with people she had never met, doing things perhaps she may not have done in “just that way”, and no train wrecks. It was a good experience overall. (Thankfully, they were all familiar with dealing with kids with such challenges.)

Overall, this news about seizure freedom along with critical thought about our faith precipitates a giant sigh of relief. It allows moments where we take a deep breath and absorb the joys of life. Her experiences, our experiences, so early in Savanna’s life make witnessing her achievements that much sweeter.

Finally, we said goodbye to Ruth, our Au Pair from Australia. She was a big help with the kids. Maybe one day we will go down under for vacation and pay her a visit. May God Bless you, Ruth.

Ruth, our Au Pair from down under, has returned to the outback. She was so much fun, and we miss her a lot.

-dad

A Message from Mom: Dear Dr. Von Allmen…

Posted on January 15, 2014 by kenlininger

First a little context….

We set out on our trip to Kentucky and Virginia for Christmas early, so Rebecca could squeeze in a work trip to Kettering, OH – which meant we would be compensated for traveling. A day after we arrived in Louisville, she left on her business trip. Scheduled to return on the 18th, she called me to tell me she leaving at 12:30 pm.

I got a call around 5:30 pm from her saying she was now just leaving. I was frustrated. I was sick. Savanna was sick. Tristan was sick. It was becoming unpleasant as Savanna was a complete handful. We all lived through it-

A few weeks later back home in the routine, she sends me an email with a response to a letter she wrote to Savanna’s doctor. The timestamp was 3:28 pm on the 18th of December. But she told me at 12:30 she was on the road? Something must have happened in Kettering that kept her there but also allowed her the time to compose such a beautiful letter. She never mentioned to me that she sent a letter. I sent one too, but not nearly as articulate (sorry GVA!) Rebecca rarely has time to contribute to the blog directly and this wonderful piece deserves sharing.

It captures her thoughts and feelings at this time of the year. I completely agree with her expressed feelings about Savanna’s treatment, the regard for her doctor, and the wish to help others. I might have a wider field of view with regard to ‘how she is doing’ than Rebecca, but that is just me. More on this in a future post…

…………………….

Dear Dr. Von Allmen,

I want to thank you with all my heart for the amazing care you have given Savanna (as well as me & Ken) through our journey. When her spasms began, I was afraid to even dream that she might be one of the rare “lucky ones” who could be spared from a lifetime of seizures & the many associated challenges through a miracle surgery. I prayed like I’ve never prayed in my life for God to guide us and give us the strength and energy we needed to survive and care for her. I know in my soul that he led us to you. The confluence of factors that had to come together for us to move to Houston, for Savanna to get the right diagnosis and treatment as quickly and effectively as she did could only have come by His hand. Why she (we) were found worthy, I will never know.

I have so much admiration and respect for you, as a mother, as a woman, as a doctor, and as a bold entrepreneur in your field. You saved Savanna’s life, her quality of life, and in doing so, mine as well.

She’s doing amazing. We traveled to Louisville, KY for Christmas, and it’s pleasantly reassuring to see her go through the common childhood illnesses of Strep throat, ear infections, and the like.

She’s happy. She’s healthy. She teases us by walking and looking back over her shoulder to make sure we are watching / following her. She dances and shakes her booty on request. She waves and blows kisses. She plays cars with Austin, and wrestles with Brandon. She giggles when thrown in the air or on the couch. She runs and giggles from “I’m gonna get you” when I chase her and eventually give her zerberts on her belly. She’s putting things into containers & taking them out. She’s starting to show an interest in books with a few torn pages as casualties. She’s social and likes to explore and meet new people. She snuggles. She climbs up and down the stairs. She’s starting to make more sounds in addition to “mama & baba”. She’s starting to use more inflection, and saying pa pa, da da, … She’s starting to do puzzles. She’s graduated from AFOs to less intrusive orthotics. Ken feels good enough about her progress that he is talking about possibly going back to work next year, which will allow me to possibly cut back to 20 hours / week, down from way too many hours.

None of this would have happened without you, your dedication, your training, and your willingness to aggressively treat her.

Savanna was first diagnosed on 12/19/11. We spent that Christmas Eve & most of Christmas Day in the hospital before Ken broke her out. It was hard to hear “Merry Christmas” that year. My dad wished us a “Blessed Christmas”, and we found that to be more appropriate.

If you have anyone going through a similar situation that needs someone to speak with, especially at Christmas time, please provide them with my phone number.

Have a Wonderful, Merry, and Blessed Christmas with your precious family. If you ever, ever wonder between long hours or thankless tasks, if what you’re doing is worth it, know this, it absolutely is, and I’m so grateful to you, Dr. Tandon, and your entire team for what you’ve done for us.

Rebecca

6 months seizure free…

Posted on October 1, 2013 by kenlininger

Six months ago today, Savanna underwent a complete TPO resection led by her neurosurgeon and epileptologist, Dr. Tandon and Dr. Von Allmen respectively.

The process started very early in the morning. About 3 pm we spoke to the epileptologist. Tears were shed. Hugs all around. Then we did it again at around 5pm with the neurosurgeon.

Each day, I thank the Lord for giving us a fresh new day, and pray it will be filled with happiness. (I don’t think he hears me everyday?) Each day I also ask for forgiveness for my sins of yesterday and His help to be the best husband, father, and friend today.

What has changed since the last post at 4 months?

Savanna and Austin turned 2 years old on September 20th. The picture to the left is very different than a year ago! A couple of days prior, we participated in a Memorial Hermann Hospital marketing campaign highlighting the benefits of MEG technology. Our local ABC13 filmed the event and interviewed us and our doctor. The 90 second news clip is here. Rebecca’s full 12 minute interview resides on their website.

Foremost on my mind today, is that I don’t believe she is having seizures or even epileptiform activity, but I cannot say this for certain. She has reached 6 months without a clinical seizure that I have witnessed. Most doctors and surgeons would harbor a guarded optimism about her future, but at the same time would suggest this is a big milestone given the circumstances.

She has almost completed cutting her two-year molars. While difficult, it has been sweet to see her struggle through this period with relative normalcy. She simply could not experience this in the past.

She walks around the house at will. 2 months ago, she could only take a few steps, seen here.

She has learned to stand up on her own, without help from furniture.

She can safely navigate steps. We do not knowingly let her do this alone.

She can focus on an activity for more than 3 minutes regularly. This has been a huge step forward with regard to self-regulating her behavior.

She can point with her index fingers and can operate simple cause-effect toys.

She has started to deliberately put toys into a container, in a very controlled environment.

She can roll over somersault style, a bit sideways, but she can do it.

She understands when you are not looking at her. For example, when you holding her while talking on the phone, she will deliberately put her hands on your face and try to turn your head to look at her. Or, she will ‘look around the corner’, to see your face. When she does this to me, it feels great as Savanna is still decidedly a Mommy’s girl – if Mommy is in the house. This indicates increased awareness and it is exciting!

All of these milestones are wonderful to watch. This 6 month milestone is a breath of fresh air. It is reinforcement for me that the surgery, the hard work every day with her sensory diet, and the physical and developmental therapy, is working together to allow her to thrive. When I reflect on the what she has learned and how quickly, I believe the therapy has helped immensely.

It is not all cake and ice cream though.

While she is eating well, she is very finicky about what she eats. And, she still won’t reliably drink thin fluids. She will not suck at all. This is very frustrating. Yes, you can sit and spend 1/2 hour forcing her to drink 3-4 ounces of fluid if she is really thirsty. But she will aspirate – a lot. Right now she is very healthy and seems to clear her lungs well. I am not without pause here, as throwing caution to the wind is dangerous. All it would take is a moderate cold combined with aspiration of fluid filled with bacteria (such as milk or formula) and you could have a serious problem. I will be trying another feeding clinic in the coming months.

Now that she is walking, the visual field cut is becoming more clear. She has really hit things hard where it was obvious she was walking and just didn’t see the table or corner of the wall just off to her right side I know these incidents will dissipate as she learns to compensate. But, it hits really home realizing her visual field cut is large enough to most likely prevent her from obtaining an operators permit for a vehicle. I know some of you might think it is odd that I would think about that or mention it now. But she is doing so well now, that it doesn’t seem out of reach – much like it would seem for a normal 2-year-old.

I have noticed Savanna ‘pausing’ at times, and so have the therapists. I say ‘pausing’ not staring because while she is paused, she is not focusing on what she is staring at. You can put your hand right in front of her eyes with no response. These events don’t seem to affect her motor function and are short. She does always seem to respond to the sense of touch. Is it partial seizures I am witnessing? Probably not, but I am not sure. I am sure we will catch some of these events on an upcoming 23hr VEEG.

She has stopped saying the consonant sounds she was saying at 4 months. She was saying about 5 sounds with and without prompting. Now, she has other noises that include sounds like what you might hear ‘Curious George’ make, and a growling sound. Is it a regression? Is she intently focused on her new-found independence being able to walk, that this has been sidelined for now? Or is it…. something else?

She flaps her hand a lot when she is excited. It looks suspicious. Is she really saying ‘Hi?’ Or is it…. something else?

She has a fascination with putting her finger into holes. It dominates every therapy session and at times takes away from the session. Sometimes, without a fidget toy that has holes in it, I can’t even get through a therapy session. She finds dimples on plastic toys that looks like holes. She is fixated on finding assembly screw relief holes on toys, cloth loops, etc. She can be obsessed with inserting her fingers into such holes. I have to say the last week this activity has lessened. It was cute at first. It enabled her to regulate her behavior somewhat at first too, so I encouraged it. Is it just a developmental stage? Or is it… something else?

I recently took them to the pediatrician for the 2 year wellness checkup and flu shots. I completed the M-CHAT (Modified Checklist looking for Autism in Toddlers) and she scored really high for ASD. Because I have done my homework, I knew this was coming. Yes, she is delayed, but the loaded questions regarding ASD are not about development, they are about ASD. Her exome sequencing indicated a significant chance for ASD. And, ASD manifests in significant percentage of children diagnosed with Infantile Spasms. An evaluation is forthcoming in her near future.

If you were to see her today for just a short while after not seeing her for some time, these things I mention would not be part of the memory. I believe the questions about ASD are challenging to definitively diagnose or dismiss at this stage. That said, I cannot ignore what is happening or hope it just ‘goes away’ as that would be foolish on my part. I cannot ignore the science that I have subscribed to thus far. I believe early diagnosis and treatment is the best course of action.

She surprises me with her development at times.

She inspires me as a leader daily.

Her encouragement, although ambiguous at times, motivates me to lead her to success versus micromanage her challenges.

A short note about the rest of the kids.

Tristan started Kindergarten this year. He loves it! He has always loved to learn and thrives in the structured environment. He is playing soccer at the Y this fall and is doing quite well. He recently ran in a Boosterthon FunRun race and ran about 2 miles. Mommy and Brandon cheered him on.

Brandon is something else. On the cusp of turning 4 on October 7th, he continues to say really off the wall funny things. He has always done this, but it continues to get more entertaining. Right now, he is participating in PRE-K4 soccer at the Y – (a very questionable use of funds 🙂 ) His first game, he just walked right beside the coach and held his hand. His most recent game, he at least ran and was able to kick the ball. He is very shy as it turns out, and I guess in Tristan’s shadow I didn’t realize it. He is protective of Savanna while being playful in way that she just loves. Hearing that deep belly laugh from Savanna when Brandon is playing with her is wonderful.

Austin is doing great. He is so intent focused on keeping up with Brandon during the day, and the ‘big kids’ at other times. He has a lot of new words now and is growing up fast. It feels like his first year was just a blur. I am glad we have some video to reference!

Ruth, our Au Pair from Australia, is working out really good. It has enabled me address a hernia repair that was long overdue. Our daily routine is working out well enough for me to start working out again and it feels great. Rebecca and I have enjoyed a couple of date nights – we almost forgot what that was like. Ruth has really been good for our entire family.

Savanna's Journey

Infantile Spasms. Partial Epilepsy. Surgical Treatment. ASD.

Tag Archives: Dr. Von Allmen