In 2009, Jackson was diagnosed with Dravet Syndrome after having genetic testing done for the SCN1A mutation. Prior to the genetic testing, Jaxs had a diagnosis of medically refractory, idiopathic, cryptogenic epilepsy. This is a fancy way for the doctors to say that they “do not know what is causing his seizures, nor do they know how to treat his seizures because they are not responding to the medications or to other medical interventions”. Jackson has suffered from these seizures since October 21, 1999 (4.5 months old).
There are many signs and symptoms accompanying the diagnosis of Dravet Syndrome. The presentation of Dravet can be life altering and will effect many areas of functioning and abilities. The challenges can vary from one child to another.
Jackson suffers from the following:
• Neurological Dysfunction with frequent seizures
• Cognitive Delays
• Expressive Language Speech Delays
• Dysautonomia: difficulties regulating his body temperature & breathing irregularities while sleeping
• Autistic-like characteristics and behaviors
• ADD/ADHD Combined Form
• Sensory Integration Disorder
• Orthopedic issues with awkward gait and flat feet
• Possible Cardiac issues from the repeated seizures and work load on the heart
• Brain injury related to the ongoing seizures
• Inability to recognize danger or to care for himself
• Side effects of medications including lethargy, drowsiness, and aggressive behaviors.
Jackson is taking a large cocktail of medications and the modified atkins diet since December 2009. All of these medications and treatments have been ineffective in controlling his almost daily seizure activity. In November 2011, the insurance company, through the EPSDT/Medicaid approved a medication approved for use in Europe which has shown efficacy in the treatment of Dravet children: stiripentol. The approval was based on compassionate use basis since there is no effective pharmacological treatment available in the US FDA approved market. With the addition of the stiripentol, the treatment has proven to also be ineffective, along with the other FDA approved medications. Jaxs still suffers from status epilepticus and frequent seizures.
This is the list of medication Jackson is currently taking:
• CBD 20:1
• depakote (Valproic Acid)
• rufinamide (Banzel) added 9-2013 to aid with the benzo wean
For the past 14 years, Jackson has been on medication, failed neuro surgery, and many alternative treatments. To date, Jackson has failed 50+ mono and poly-pharmacological medication treatments. To date none of the medications have done much to control the seizures. Many times Jackson actually experiences more seizures with the recommended doses or higher doses of the medications currently on the US market. Sub-therapeutic doses of the medications seem to be more effective with seizure control. All changes (increases, but especially decreases) must be very slow to prevent reoccurring and ongoing seizures.
In January of 2005 he had the vagul nerve stimulator (VNS) implanted in hopes of conquering the seizures at UC Davis Medical Center in Sacramento, CA. For three months, he was seizure free and doing well. But, like the medications, the VNS stopped working to control the seizures. It is still implanted in his chest like a pace maker for the brain. The device was upgraded in 2012 with a new battery replacement indicator to avoid miscalculations of the battery strength and battery life.
In April 2008, in hopes of once again stopping the seizures, Jackson underwent major surgery at the Children’s Hospital of Philadelphia in Pennsylvania. After extensive testing, the decision was made to remove the section of the brain where the seizures appeared to be originating from. The team of surgeons removed the right frontal lobe just anterior to the sensory and motor strips and completed a 2/3 anterior corpus callosotomy. Within 18 days the seizures were back, but Jackson was strong and determined. He was discharged from the hospital and rehab just 6 weeks after the surgery was complete and was back in school to finish off the school year. Although the surgery did not stop the seizures, the length of the seizures have curtailed to less than 2 minutes on average. Prior to the major surgery, Jackson suffered from seizures which were over an hour on average.
In November 2012, Jaxs cocktail began to include a trial of CBD rich butter. His dosing has increased and he is beginning to show signs of cognitive improvements, lessened seizure intensity and quicker recovery from seizure events. His VEEG background is now showing normal without slowing or spike waves. He has completely weaned from phenobarbital (highly addictive barbiturate) Klonopin (highly addictive benzodiazapine) and Onfi (highly addictive benzodiazapine), Stiripentol, and a decrease in depakote by 50%. Jaxs is also no longer on the ketogenic diet with 5 additional nutritional supplements.
Despite all of the medications and other limitations, Jackson has the ability to wake up every morning with a smile and a warm greeting: “Good Morning Mom!”. I am blessed with this greeting even on the mornings when he has seizures before waking up; sometimes he has 10+ seizures between 2 am and 6 am and still wakes with a smile and willingness to tackle the day. He is the inspiration and sunshine which empowers me to continue each day to fight for his needs, inspires me to request access to appropriate medical care and hope for the future for him to live the happiest and best life possible.
Jackson needs safe access to the state approved medical marijuana with high CBD and low THC to control his seizures in both states in which he resides. However, the family has recently made the decision to relocate to Colorado from NJ because Governor Christie has no compassion and is destroying the New Jersey MMJ program.
Hope for the Future:
CBD is a molecular component of the cannabis plant that has many healing properties which can benefit those suffering from intractable epilepsy like Dravet Syndrome. Medicinal grade CBD should be used to treat Dravet Syndrome with the supervision of a qualified neurologist.
You can follow Jackson and learn more about his journey with Dravet Syndrome at http://www.jacksonstormes.com/
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