Jackson Stormes:A Difficult and Amazing Journey 

Living with Dravet Syndrome and all the complications

Meet Jackson Stormes     

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) and his second seizure was on November 3, 1999 when he was started on the first narcotic medication in an attempt to control his seizures.

Jackson is being followed locally by Dr. Eric Segal at the Northeast Regional Epilepsy Group in Hackensack, NJ

Since 2009, Jackson has meet with Dr. Charlotte Dravet from France and Dr. Ian Miller from Miami Children's Hospital and the Brain Institute in Miami, FL

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
  • Ataxia
  • 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.

Currently Jackson is taking only one pharmaceutical at a very low dose for his age and weight since beginning the CBD treatment.  In the past 15 years the pharmaceutical 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 seizures.  He has been benzo free since November 18, 2013 and barbituate free since Feb, 2013.

This is the list of medication Jackson is currently taking:

  •      - CBD 20:1
  •      - Diamox

Progress with CBD Treatment:

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.

 Medications and/or treatments tried before MMJ:  Phenobarbital, Klonopin, Tranxene, Onfi, Versed, Diastat, Valium, Lorazapam,  Tegretol, Depakote, L-Carnitine, Topomax, Keppra, Keto Diet with many nutritional  supplements, Zonegram, Zaratin, Felbatol, Ritilan, Lamictal, Diamox, Vitamin  B6, Prednisone, Clonodine, Gabapentin, Banzel, Stiripentol, Prozac


Surgical Intervenations: VNS, Right Frontal Lobectomy, 2/3 anterior corpus Callosotomy


Ongoing Needs at This Time:

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. 

At this time Jackson has been approved for nursing services with EPSDT/Medicaid to keep him safe while awake and asleep because of the risk of increased risk of injury resulting from seizures or SUDEP.  The same insurance company is currently paying for the stiripentol (FDA Orphan Drug) with a prior authorization through the EPSDT/Medicaid program for compassionate use. The approval of the stiripentol will decrease the cost of Jackson's care with less FDA approved medication which are ineffective in controlling the seizures, decrease in frequency of emergency room visits, less med-flights to level 4 epilepsy centers, and avoidance of lengthy hospitalizations from intractable seizures.  The nursing services decrease the cost of emergency rooms visits and hospital admissions. Both the nursing services and the medication improve Jackson's quality of life, allow his older sister to be a normal-typical teenager, and should provide the opportunity for mom to continue with her nursing education and ability to earn a living as a RN to support the family.

Currently Jackson receives nursing care including the administration of the Stiripentol and other FDA approved medications through Loving Care Agency.  Atlantic Private Care is unable to administer the medically-necessary, doctor ordered stiripentol without IRB interference and politics.  

Jackson's Medical and Treatment History:

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 MIND Institute 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.

Current Treatments and Options for Dravet Syndrome:

At this point in time, there is no cure or known effective treatment for Dravet Syndrome.  Some children with Dravet Syndrome have achieved seizure control with a cocktail of medications or treatments.  The same cocktail may not work for another child.  To date, there are three treatments options for some children diagnosed with Dravet Syndrome:

      1. Ketogenic diet with medications available in the United States;
      2. Stiripentol (FDA-Orphan Drug Status) used in conjunction with Depakote and Onfi (clobozam).
      3. CBD rich medication cultivated from the medicinal marijuana plants.

 

Research continues and Dravet non-profit organizations are taking great steps to organize parents and professionals to continue in the pursuit of answers for our children to improve their quality of life..

                                   

Resources:

Dravet Syndrome for Dummies Website with summary of Dravet facts and information.

Charlotte Dravet, MD summary of information and knowledge.

2010 Dravet Family Conference in Greenwich, CT with Charlotte Dravet, MD.  

 

Hope for the Future with Dravet Syndrome:

 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.

CBD can be used with or without THC.  

CBD (cannabidiol) and other cannabinoids work on the endocannabinoid system with the CB1 and CB2 receptors.  It is neuroprotectant and has other benefits as well.

 
 

 

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