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

Luna’s Story: An Update

Posted on November 23, 2014 by kenlininger

The meeting with the neurosurgeon prior to the surgery described the treatment scenarios on a scale of least invasive to most invasive, along with a likelihood of success in Luna’s specific case. The chosen path was not the most invasive, leaving a portion of her left hemisphere connected and functioning with the hopes it is not involved in seizure onset. This decision was based on experience and the best medical science available today. It was not a mathematical formula with a guaranteed outcome, but rather a first step. One that if successful, leaves her the most natural motor function possible. If you didn’t read her initial story, you can find it here.

On November 6, Luna underwent what is called a TPO resection/disconnection in her left hemisphere. A known consequence is significant visual field cut in both eyes on the right side, a dense right sided hemianopsia. The actual procedure went very well and Luna recovered quickly. Here is a picture of good news from the waiting that day (Savanna was not particularly photogenic this day!)

Luna like most in this immediate time following surgery, is quite unhappy often. She doesn’t understand what happened to her or why. Her head itches, and probably hurts, but she can’t tell us in a way we immediately understand. She cries, and we do what all parents do, we just try to make it better any way possible. Her behavior and demeanor reminds me so much of Savanna during these days. I wish the pertinent doctors could be more aware of what this is like. When you try to speak with them about this, they really just don’t seem to care or have anything to help in the way of guidance, wisdom, or advice. I have come to realize they just don’t really know how hard it can be; how exhausting; how stressful. It is not to a fault in any way, it is just reality. You just can’t know unless you live through it personally.

Maria is returning to Norway completely emotionally drained, mentally exhausted, and physically hurting from holding and carrying Luna far more than you would ever carry or hold a normal 19 month-old.

The thought of being on a couple of airplanes for nearly 18 hours is just daunting to me, but she is determined.

So how is Luna?

The incision site looks great. She healing physically very well. She is not experiencing any complications.

Luna is much more calm overall. She is more focused and engaged with her environment.

She is changing rapidly during this time of recovery.

Some noticeable suspect activity has been seen. Luna needs more time to heal before officially ruling on this suspect activity, and a chance still exists that this activity will dissipate on its own.

She has some sensory processing issues she needs to work through, a process very difficult with the constant disturbance in her brain. Now she has a real chance to overcome these challenges, and in the short two weeks post-op we are already seeing these changes.

Luna has to tell us what she needs, metaphorically speaking.

Prior to the surgery, I worked and worked with Luna to stand and take steps. The hypotonia in her feet and legs is significant. She stands but with locked legs, and muscle grading is poor – much like our Savanna experienced. After surgery, she is so much more focused and able to “think” about something such as walking. Here is a short video that is just amazing. I could not have done this with her a couple of weeks ago, without more or less carrying her through the process. She would have tried to turn left circles the entire time and cried while doing it, or as Maria calls it ‘protesting’. Now, she is very motivated.

What do you feel as a parent in this situation? What do you do next?

Well, first, you have to learn to wait. This is harder than it sounds, because the academic body of evidence is growing to act sooner rather than later with pediatric cases.

As a parent, your world hinges on the surgical success, something that can take some time to appropriately cast judgement.

You research others’ stories with an obsessive compulsion that can render you insane. With red eyes, you seek out that other child who looks just like yours; has seizures just like yours. What was their treatment path and outcome? How can I interpolate and extrapolate their path into mine? You fixate on their story, for better or for worse. But this is what we do as a parent of a child with intractable epilepsy in this ‘Google-centric’ world.

While not entirely healthy, you come up for air once in a while and find scholarly articles to educate yourself in an objective manner. Then you remember discussions with the pertinent doctors, the ones you put so much trust into already, and let their guidance help show the way. You go back and re-read diagnostic findings, again to help educate yourself.

You second guess every decision made in your child’s care. You realize that you cannot go back and relive anything, try anything differently, act differently or more quickly. This time has passed and all you have is the future, on a new path post-op.

To use a very American analogy… At this juncture, as a parent, you have left the college sports ranks and joined the professional athletes. When you run through the tunnel onto the pro playing field/court, the same game is very different. And as a pro, success means embracing this change. You have to expand your mind. You must adapt to a larger set of variables, more potential consequences, and then re-calibrate your mind with regard to what “success” really means. Going back to the college days, is no longer an option.

On top of all this brow-beating and compulsive ‘googling’, Maria has secured a work position here in Houston for the next couple of years and sold her beloved house in Norway. She returns to Norway today to finalize her move to the USA for employment and spend much needed time with her other daughter. We will get to see her again in the future and look forward to seeing Luna’s progress.

IS Awareness Week: Luna’s Story

Posted on November 7, 2014 by kenlininger

Here in Houston, an 18 month old girl from Norway named Luna awaits surgical intervention to control her epilepsy. Her mom knowing full well this is Luna’s only chance to get real help. She has experienced more than a year of uncontrolled seizures, with only a few brief periods of control on steroid therapy. She is suffering from Infantile Spasms, a very catastrophic form of childhood epilepsy.

About a year ago, I came across a post in a FB support group from a desperate mom looking for help. She posted videos, and asked for assistance and advice about interpreting the video and what she should do. Already at this early stage, she was questioning the Norwegian doctor and plan of care. I reviewed the spine-tingling video, and saw right away a likely cluster of IS, and a second process that seemed very asymmetric in clinical presentation. She was so similar to our Savanna, but yet so different.

She explained the treatment plans in place from her neurologist. She translated documents for review. I advised her how cases like hers are handled here in the USA at a facility familiar with IS, the kind of tests usually ordered and why, and why it is so important to move quickly and accurately with the workup.

As the workflow progressed in her treatment, at a small hospital in Oslo, I remember experiencing a sinking feeling after her clinic appointments with her neurologist. It just didn’t sound like their doctors saw the situation as an emergency, nor did they have the resources to appropriately evaluate Luna or treat her such that she would have the best chance at seizure control.

Two things I learned over the past few years dealing with IS/childhood epilepsy: 1) Time is Critical, and 2) Workflow (the process) is Important – especially when the cause is idiopathic or cryptogenic. This is not to say a symptomatic case should not be treated similarly. Etiology can play a significant role in therapy choices or schedules.

It became clear after about 6 months, Luna simply wasn’t going to get the best chance at seizure control in Norway. We talked frequently and the idea of traveling away from Norway for help was born.

I kept having to remind myself, that Luna is in a socialized healthcare system, and this can be the way it is in these extreme cases in such a government-controlled healthcare system. While Norway does have a more appropriate hospital for Luna, it was the access to the services that seemed draconian after seeing what is possible in other countries, especially the USA. For those that have lived through the nightmare of IS, can you imaging having to wait for nearly 6 months to get your first 24 hour EEG with video? Me either. Until that point Luna only underwent only 15 minute EEG diagnostics, without video.

High dose steroids were the only therapy that had a positive affect; producing brief seizure free periods. Several other drug trials failed to control Luna’s epilepsy and also resulted in marked negative side-effects.

Even a trip to Bonn, Germany, resulted in a “come back in six months” outcome, after diagnostics produced results insufficient to justify additional diagnostics. Here in the USA, those same results most certainly would have justified an additional scan or two. I remember being so let down by the results of that trip, beside myself at how different other countries view IS.

Finally, 10 months after her journey began, she was able to get an FDG-PET scan, (her first one!), only after a epileptologist in Australia called in the order to a facility in London. Another expensive “self-pay” excursion to another country, much like their visit to Bonn.

By that time she had also contacted our doctor here in Houston, and had arranged a consultation date.

Access to surgical services looked viable in EU and in AU. And the doctor in Melbourne seemed confident about what Luna needed. Suddenly, the outlook looked good. Timing became an issue in the EU as surgery was many months away, and access to socialized services in AU seemed out of reach after some effort.

Two months later, she arrives here in the USA, after having raised nearly all the necessary funds to cover the cost of a “self-pay” surgical workup and epilepsy surgery.

A streamlined plan consisted of a week of diagnostics followed by a week of information review, then surgery the following week. Celebration!

Then suddenly a question during the VEEG/LTM: Has she been tested for CDKL5 mutations? In that one second, the entire plan appeared jeopardized. A thorough review of records produced no test results. Calls to the Neurologist in Norway produced no immediate answers. Surgery now on hold, a comprehensive targeted genetic panel was initiated. It took three weeks to get results, during which time the Norwegian Neurologist finally confirmed she was tested for a host of genes in question, and was negative. Exhale!

So we have a new schedule for a surgical date. All is a go, again!

Then the unthinkable: she gets sick. A common cold, that of course produces the cruddy cough, and the sound of doom: congestion. Then it produced ear infections, which was icing on the cake.

How she made it nearly 5 weeks in our house without getting sick is beyond me. We are performing every possible prophylactic measure to get her healthy as the battle with Anesthia continues. Yes, battle is the correct terminology. The procedure Luna is going to undergo requires several players on the team. One is the Anesthesiologist. They really don’t care at all why she is there or the reason for the procedure. It is so frustrating when a doctor who’s only involvement in her care is that day’s events can derail concrete plans, for a clear runny nose.

There are two types of people in this world: 1) Those who say Yes, I can help! and 2) Those who just say No.

It takes work to say YES or be positive. It requires assuming more risk. It often requires making difficult decisions and sacrifices. It is a conscious choice to say yes and be positive.

It takes zero effort to say NO and be pessimistic. It requires assuming no risk. It requires no further decisions or sacrifices. It is easy to say No and be negative.

In this case….. It’s an over the phone diagnosis: your surgery is cancelled, and I really don’t care what you think. Call you neurosurgeon’s office and reschedule two weeks after her last sneeze. Just the Friday afternoon call a neurosurgeon enjoys about a Monday procedure.

This position minimizes the risk assumed by you and by the institution. Which is interesting because the other doctors and the institution have agreed to allow the neurosurgeon latitude to operate – which may not work – and yet still leave her with permanent deficits. But, wait! Patient Safety!

When in doubt, pull this one out: It’s All About Patient Safety! Sorry, but no surgery. This is like liberals using the race card in a debate when the know they have lost on the facts. It is infuriating and in this case quite insulting. Right, it is much safer to continue to suffer a catastrophic seizure disorder than the potential of a sore throat after surgery. Right. Any parent of a child like Luna arriving at this moment sees right through this argument. Enough said.

We have finally reached The Big Day. An emotional blender full of tears, anguish, and hope. All the sacrifice. All to arrive at this moment in time where you kiss you child goodbye, not 100% sure what is going to happen. May God keep our children safe and guide the hands helping Luna today.

Today is Luna’s last day to suffer from IS.

You can follow her journey on FB here.

Savanna's Journey

Infantile Spasms. Partial Epilepsy. Surgical Treatment. ASD.

Tag Archives: TPO resection

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

Luna’s Story: An Update

IS Awareness Week: Luna’s Story