Dravet's & Prevention.Jackson Stormes, known as “Jaxs” has Dravet syndrome

Jackson Stormes, known as “Jaxs” has Dravet syndrome

Jackson Stormes, known as “Jaxs” has Dravet syndrome, a complicated and devastating form of epilepsy caused by a genetic mutation, SCN1a. In the past 18 years, Jackson has failed over 70 pharmaceutical combinations, two brain surgeries, multiple trials on the ketogenic diet, and the vagul nerve stimulator. In 2012, Jackson was started on cannabis: high CBD and THC in approximately 20:1 ratio and he thrived and did so well. He was weaned from phenobarbital, Klonopin, on, stiripentol, Depakote, and the ketogenic diet. Although there was a medical program in New Jersey, it was not up and running, nor would it prove to be functional for Jackson’s medical needs. In 2014, Jackson and his family relocated to Colorado for better access to the life-changing cannabis medication.

Shortly after moving to Colorado, Jackson began to decline, and the local health professional did little to help because Jackson was on cannabis, especially the THC. Despite the situation, I continued with the cannabis and made changes to address the new diagnosis of Juvenile Parkinsonism, declining skills and abilities, and loss of progress gained with the cannabis treatment.

Jaxs went from a 20:1 CBD to THC to increase the THC to lower the ratio to 3:1 CBD to THC. He was getting cannabis for emergencies, seizure clusters, or times of illness to bridge the lowering seizure threshold. Cannabis was the safest option for Jackson, after all, he had failed 98% of the known pharmaceuticals used to treat seizures or Dravet and even tried some extended shot options without success.

Last summer, a few additional pharmaceutical options were attempted with one new drug and retrying another drug from the past he had failed, in addition to the cannabis treatment. The pharmaceuticals were not expected to help much with Dravet, but worth a try and come with many severe known side effects, but the cannabis was not working well to control the seizures. Jaxs was declining, and few noticed or cared. Jackson’s seizures were getting worse with longer seizures occurring more frequently. He could not walk without support, he lost almost all of his speech (had over 200 words counted in 2013), could no longer assist in any capacity with his activities of daily living, no longer able to feed himself, and he just stopped eating with these new drugs.

The fycompa was the first new medication with devastating effects. His body contorted more, and he lost more skills and abilities. He could not move or reposition himself, and he was locked in his rigid body. He still had the seizures with the Dravet and the Juvenile Parkinsonism, but now he had more seizures. He was utterly GT fed and was throwing up everything all day, every day. The formula was healthy, organic, non-GMO, vegetarian, and covered by Medicaid. He needed to stop the fycompa medication as it was not controlling the seizures, but making him worse overall.

Since the cannabis was not controlling the seizures and now the emesis was problematic, he was losing weight and now considered a failure to thrive barely holding his weight at 100 pounds. Jackson was retried on felbatol. All of the side effects continued with the emesis, failure to thrive, increased seizures, and worsening of the dystonia and Parkinsonism. Jackson ended up in the hospital where they stopped the felbatol with no plan. They had no plan because Jackson was on cannabis and had already failed all the other drugs, Some of the meds, like the felbatol, have been tried more than twice. The neurologist sent him home from Denver Children’s Hospital without a replacement pharmaceutical, refused to increase the very low dose of the other pharmaceuticals he was on, because of the cannabis medications. Jackson’s seizure got even worse.

We discussed stopping cannabis, but he was not on any significant dose of a seizure drug and stopping cannabis would be dangerous and life-threatening. I was told by the neurologist during an outpatient visit; the “I got him on cannabis, I could get him off of it.” Without prescriptive authority, I cannot adjust or change his pharmaceuticals, and without their help, I cannot change the cannabis. He continued with daily seizures, emesis, weight loss, and failure to thrive.

The acceptability of cannabis, especially THC for children, by some physicians is a game of smoke and mirrors. At the end we found a great adult Epileptologist to assume Jackson’s care, including the cannabis use as a seizure treatment. Jaxs was a hot mess with the seizures so out of control; he was close to suffering a deadly seizure. With the help of this Epileptologist, his pharmaceuticals were increased, and a new one was added: Briviact, a relative drug to Keppra, but without the side-effects of rage and behaviors. The seizure activity went from a subclinical seizure every minute with multiple 3-5 minute seizures daily to one per week with no subclinical seizures. He was stabilizing and getting better with this adjustment in the anti-seizure medications. Jackson was still completely tube fed, and the specialists wanted to give him a permanent j-tube to address the still constant emesis.

Finally, he had endured enough distress with the emesis, and all formula was stopped. A blend of real food prepared by mom consisting of spinach, beef, garlic, and almond milk. He stopped throwing up. A few weeks later, he began to want to eat by mouth. Without the help of doctors, other than adjusting the pharmaceu- ticals, we were figuring out what was going on and healing Jackson.

It was decided Jackson would enter the fenfluramine drug trial. For this medication, he would need to be cannabis-free. This new adult Epileptologist was willing to adust medications and to safely take him off of cannabis in a controlled environment. We were not afraid of the cannabis, but more afraid of the seizures. The cannabis was stopped for a few days, and there were no seizures. The seizure medications were increased to maintain seizure control, but not any benzodiazepines, narcotics, or other dangerous drugs he had been weaned from with cannabis in 2012.

With the cannabis break, the oversaturated cannabis receptors could rest and some of the skills which were lost over the past three years since moving to Colorado were coming back. Taking a break and carefully selecting an appropriate treatment plan to control the seizures has allowed for Jackson to begin eating better, gaining 20 pounds. He started walking everywhere, with transitions, transfers, and balance. Things are being placed in the microwave, sink, or other hiding places with glee. His smile is bright, and he pushes himself to re-learn what he lost.

Why is this Important?

Cannabis is a medication and was responsible for healing Jackson when he needed it, however as expected; his needs are constantly changing. While he is better off at this time without cannabis in his regimen, I will be keenly observing for any changes that clue me in that he needs a change in his treatment at which time I would again start the process of determining what ratios would benefit him. What we have learned and will discover with time, is that cannabis can be complicated and can be confusing, especially for complex children. The Parkinsonism is regressing.

That cannabinoids were messing with the dopamine levels and/or receptors causing dystonia, tremors, shuffling gait, and other neuromuscular concerns are given though cannabis, or CBD, is not toxic or deadly, at what dose does CBD stop helping and make symptoms worse or more difficulty In Jackson’s case, it appears that higher amount of CBD for prolonged periods of time made his symptoms worse. In patients who are non-verbal, dosing can be more difficult with finding the sweet spot, correct strain, or appropriate ratio.

Because research is limited to cannabis in America, it is still unknown why a cannabis break is important for chronic, debilitating, and devastating disorders. It is known that cannabis breaks do allow for the Endocannabinoid System to reset itself, but it is unclear why this break is so important for chronically ill persons. Although it is consistent with Dravet to be pharmaco-resistant, when medications stop working after a short period, cannabis was not as sensitive. It took four years before a break was needed and changes necessary. What is clear? We need to be able to do research and understand cannabis as a medication, applications, pharmaceutics, and more.