His journey has been a maze of doctors, specialists, therapists, theories, blood tests, diet modifications, neuropsych testing, IEPs, weight worries, anesthesia, EEGs, medication experimentation, communication aides, AFOs, misdiagnosis and missed diagnosis…tears, pain…oh the pain…worries to a depth so deep they could only be known by other parents of children with significant special needs…the disappearance of finances, the slowest implementation of help possible….the feeling of desperation and isolations…who hears your cries for help?
Still through it all…his smile and laughter have remained infectious. The ability he has to touch another, to reach the unreachable while holding his own. His perseverance rivaled by none. His brain, rattled by that missed diagnosis. That missed diagnosis….
Landau-Kleffner Syndrome, as described by Friends of Landau-Kleffner Syndrome (FOLKS):
“Landau Kleffner Syndrome (LKS) is a rare form of epilepsy that only affects children, and causes them to lose their understanding of language. The main epileptic activity happens during sleep and is usually not obvious to others. It can be seen on brain wave recordings (EEG, electroencephalography). There may, however, also be visible seizures at night and/or during the day. LKS may also be referred to by a variety of related terms that describe its effects.
As the condition is not well known and has complex effects on language and often also on behavior, it can take some time before the whole picture is recognized both by parents and professionals and so it can take some time before LKS is diagnosed.”
Jackson’s first noticeable seizure was on December 1, 2006, 6 days before Olivia was born; he was almost 4. His second October 1, 2007…9 months to the day after the first. With medications started, epilepsy was looked at no further. Despite this medication, despite our pleas for professionals to investigate the reason for our son’s continued erratic if not non-existent sleep, despite his obvious regressions, our concerns were dismissed. Years later, in 2009 we’re finally heard, a 24 hour EEG ordered and the diagnosis of LKS (Landau-Kleffner Syndrome) was given. Likely, he’d had LKS for 4-5 years.
5 years of constant assaults to his brain. 5 years of irreversible damage. 5 years of his suffering.
Over time, Jackson’s brain activity of spikes and waves morphed, reformed, stretched, regrouped and ‘matured’. CSWS (Continuous Spike and Wave during slow Sleep), ESES (Electrical Status Epilepticus of Sleep)…these terms were used interchangeable within the same conversation with our epileptologist. It would seem Jackson’s form of epilepsy changes with each EEG. During this time, he’s been forced to consume numerous medications in various combinations and dosages, yet the EEGs would not improve.
Then, this past June, after his most recent EEG, it all got worse. It seems as Jackson’s “epileptic aphasia” (yet another term) was maturing, it was growing into a monster now described as intractable seizures. He hit his all time high of 95% of his nights AND days in seizure or pre-seizure activity. 95% of his life spent with his brain under constant assault… a constant attack from itself, damaging…damaging. 95% of his life spent in almost constant spike and wave state. His EEG showing activity nearly every minute. What would happen when this medication finally stopped being strong enough to hold back this monster?!? (We’ve found that answer as he’s starting having noticeable seizures again.)
His epileptologist was once hopeful that he would outgrow this, as happens with LKS around age 15 (though not before causing permanent damage). Now, she hesitantly admits… she can no longer hold this hope for him.
So, here we are. We know our boy, our first born having held all the hopes and dreams an infant unfairly carries…our first born is struggling to just be, seizure free, in every breath. Here we are, knowing our boy will not outgrow this. That his brain’s rogue activities are now classified as refractory, uncontrolled, unresponsive to numerous medications, intractable; intractable, as in there’s no medication to prevent it. Here we are with very few options.
- Ketogenic Diet (already refusing to expand his own diet coupled with weight concerns, all medical professionals agree this is not a plausible option for him)
- Vagus Nerve Stimulation (implanting a device to ‘zap’ the brain to stop the activity – used in conjunction with medications that have proven to not work for him)
- Brain Surgery (enough said)
- Experimental Trials (enough said)
Then there’s IVIG. Intravenous Immunoglobulin infusion. Not in the set “guidelines” insurance carriers reference, but recognized among epileptologists as one of the very few treatments for LKS, epileptic aphasia, intractable seizures. Obtained from thousands of plasma donors, this pooled source is processed by removing antibodies (immunoglobulin), chemically treating it, then filtered to remove viruses. This mixture, Gammagard liquid injection (a.k.a. IVIG) runs from a tube, through an IV and into Jackson’s arm, manned by a pump.
The process will take us from 8:00 am to 4:30 pm… 8 hours. For two days in a row. With positive results leading him to need this every 2-3 months. His first round is scheduled for September 20th and 21st at the Children’s Hospital. For a conclusive, unbiased decision of the effectiveness of the treatment, before moving towards more invasive procedures, a follow up neuropsych exam and an earlier 24 hour VEEG (Video ElectroEncephaloGram) on October 17th will be done.
The estimated cost of the IVIG therapy is $ 5,182 per day. For two days. $ 10,364. Insurance has denied this, for Jackson’s manifestation of epilepsy is rare. It is not found in the “guidelines” before them. We have appealed. His epileptologist has sent a letter and supporting documents. I have sent a letter. We are on hold for a funding source for this procedure…that we HAVE to do, and will STILL do…and that we don’t know how many more we’ll have to do.
This is an estimate, we don’t know what this total will turn out to be, nor do we know what additional expenses might come out of this, how many of these infusions he might need through his lifetime, nor whatever other medical expenses might not be covered in the future…he does, after all currently hold 4 diagnosis (Cerebral Palsy, Epilepsy, Autism, Sensory Processing Disorder) and who knows what else.
Throughout all the ordeals and constant state of crisis our family has been in, the support has come from very few places. Few walk our journey with us. Few understand, are compassionate, self-educating and involved with our family. Few accept our family. My online family, my “crew”, my blog friends have become very precious friends. Other parents of children with significant special needs, each spanning every diagnosis Jackson holds and more, some having typical kiddos, but openly receiving me, Special Happens, my family…they have awarded me with the highest honor with their selfless activism for Jackson’s cause.
For Jackson, with Jackson, they fight his epilepsy and all his medical conditions. These friends have waged a campaign of supporting Special Happens with their writings, temporarily relieving me of my blogging duties so I can focus on my son…but most importantly, they have spearheaded fundraising efforts to cover the costs of the IVIG treatment. Concerned with the possibilities Jackson’s diagnosis’ and prognosis’ hold, they have decided to not limit their efforts for a single treatment, wanting to cover any other therapies or medical expenses not covered by insurance or Medicaid now and in the future.
In the coming days, I’ll add more about IVIG, Jackson, insurance battles, Medicaid and our infusion experience. In between, I suspect, you’ll have the chance to read other posts from various other bloggers…some known, some not.
If you would like to contribute to Jackson’s Fight Against Epilepsy (and Autism, Sensory Processing Disorder and Cerebral Palsy), I would be forever grateful for your donation. Yet, times are hard, for this we are aware. A note to me, the hubs, the youngers (Olivia and Braddock) or most importantly to Jackson, would be just as priceless.
On the sidebar is the widget for donations on ChipIn if you’d like to donate. You can also grab the widget and place it on your site. Don’t worry if you don’t see your donation immediately in the total, it takes about 24 hours for the widget to update. All monies will be deposited to a Fund designated for Jackson’s Continuing medical expenses that might not be covered now and in the future.