Lennox-Gastaut Syndrome

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Living with LGS

My husband, son and I had just finished eating lunch.  My husband asked me if I enjoyed my meal.  I replied, “yes”, but I told him that what was actually controlling my thoughts and dominating my mind throughout our lunch was, “what is the quickest way to leave this room?” My husband said he felt the same.

To back up a bit, our family was on vacation cruising through the bright blue waters of the Caribbean.  My husband, son and I were eating lunch in the fancy dining room of the cruise ship.  We were supposed to be at ease, relaxed, and enjoying our vacation.  Our 24 year old son is diagnosed with multiple disabilities, he uses a wheelchair, cannot talk and is diagnosed with Lennox-Gastaut Syndrome (LGS) which is a rare, hard to treat form of epilepsy.  It’s the LGS that has my husband and I on our toes.  It is quite normal for our son to have several seizures throughout his day.  Unfortunately, our son’s life, our lives and our other children’s lives are all affected by these seizures.  Everything we do as a family we plan for and take into account how that activity will affect him.

It’s this extra planning, unpredictability, and worry that has elevated our senses into overdrive after 24 years of living this life.  When we arrived at the dining room we asked to be seated in the far corner of the dining room, where it’s quiet and the eyes of strangers aren’t focused on our son, rather on their own families and fancy food.  We can “tuck” Andrew’s wheelchair into a less prominent place. We want to have this time with our son, enjoying our lunch and good food.  That is why we are here.

What we can’t predict though is when a seizure will come.  We can’t predict what type of seizure will come.  We can’t predict the strength of the seizure.  So, we are on edge.  We’re on edge even more because we’re surrounded by people.  We search for the exits.  We search for the easiest path for the wheelchair to take through the maze of dining room tables and chairs. What’s the quickest way out of this room so that our son can recover with dignity?  Because If the seizure does come, our son’s arms and legs jolt and shake violently.  Our son gasps for air, his eyes roll to the side.  Andrew’s seizures are powerful and loud.  When a seizure comes, for my husband, son and I the happiness and mood of the moment changes in an instant.  Is our son okay?  Do we need to intervene with emergency medication?  Will more seizures follow?  Our questioning and arguing of whether we should have our son out and about now dominate the main topic of conversation.

On this day we actually left the dining room without seizures. Thankfully.  Something so simple, a gift, and a great blessing in our days living with seizures.  A simple meal on a vacation with our family.  Lunch on this day is a success, and a relief for a mom and dad who live their lives knowing the next seizure is looming.   We wish for even more moments just like this.

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Online support when you're living with a rare disease

The year was 1990. My younger brother Michael, age three at the time, started having seizures out of nowhere. I was seven years old; I had never seen a seizure before and thought he was going to die. I had no frame of reference at that young of an age to understand what was happening to him; what is a seizure? What does it do to my little brother’s brain? And why (oh WHY?) did he continue to have dozens of seizures per day despite taking a few different medications?

These questions ruminated in my young mind, but even more so in my parents’. They asked questions during their routine visits to the neurologist, and unfortunately, their regular trips to the emergency room, but were left with little to no answers as to why Michael was becoming more and more severe by the day and why his seizures were still so uncontrolled. At the time, my mom worked at our local library, and turned to periodicals and encyclopedias to learn as much as she could with the limited information she had access to. As a reminder, this was 1990, before the internet’s golden age, and before you could google your questions or symptoms and get answers in nanoseconds.

It was a version of the Encyclopedia Britannica where she found a paragraph under the epilepsy section that described Michael perfectly: Lennox-Gastaut Syndrome. A rare and catastrophic epilepsy characterized by “mental retardation” (the term they used back then), multiple seizure types, and a distinct EEG pattern. My mom’s fears were confirmed when she followed up with Michael’s pediatric neurologist and received the formal diagnosis at one of his next visits. Not knowing anyone else who had a child with the syndrome, she felt alone and desperate for more information.

Fast forward 17 years. The year is 2007, and the internet is ubiquitous. Facebook was gaining enormous popularity and other social networking sites (SNS) (i.e. Yahoo! Groups and Myspace) were being used as a way to connect with others electronically who shared similar interests, or of course, a rare disease. Other SNS would soon emerge in the next few years (such as The Mighty!). And boy, this time was ripe and ready for an LGS Foundation, which provided an excellent opportunity (finally!) for families to connect with one another online, to feel less isolated, ask questions, and form friendships with others who “got it”.

2007 was the year that I founded the LGS Foundation, the first non-profit organization in the world dedicated exclusively to Lennox-Gastaut Syndrome. The impetus behind starting the LGS Foundation was my brother of course, but it was also compounded by nearly two decades of my family feeling isolated and scared. Michael was 20 years old at the time when the Foundation was launched, and we had never met, or spoken to, another family facing the same disorder. That all changed with the formation of the LGS Foundation and the ability to connect with families from all over the world through our website and social media channels/ social networking sites. The connections that were made through these platforms undeniably lead to the Foundation’s growth and what the organization has become today.

This leads me to the topic of social support. Social support is defined as the way in which a person feels supported, especially during times of hardship or crisis, and how connected a person feels as a part of a supportive social network. For those of us living with a rare disease or caring for someone with a rare disease, you may have experienced increased social support from using SNS without even realizing it. I think one of the greatest things about social support from SNS is that it can be instantaneous and can transcend distance, time, and other barriers. Snail mail? Who needs it when you have instant messenger!

Sometimes, I need to take a step back and think about what life was like before the internet. How did our family manage those feeling of isolation, the lack of instantaneous information, and the dearth of information available to us as patients and caregivers? I’m not sure, but we did. Thank goodness for the internet and what it is today, and thank goodness for other families, like you, who are willing to offer the support that we all need through social networking sites and websites like this one.

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CBD Crash Course: The Hype Lines

Navigating the Cannabidiol

(CBD) product landscape as a patient can be overwhelming.  Fancy websites with glittery products hint

that by adding CBD to your health care regimen that YOU TOO could live the life of your dreams.  All your pains will just go away and vitality

is yours for the taking.  CBD is being marketed in everything from

hamburgers to pillows, and active wear to coffee.  Everywhere, people are talking about the

wonders of CBD.  When we listen carefully,

we hear consumers talking about www.foxnews.com/health/just-how-safe-are-cbd-products-experts-weigh-in and products that www.kmov.com/news/cbd-consumers-concerned-about-products-wit....  Patients

are also talking about www.projectcbd.org/medicine/cbd-drug-interactions/p450 they didn’t get warnings for and being misled

about potential negative consequences, like being dismissed from pain

management over a failed drug test.  Patients

don’t have the luxury to think about supplements like CBD as nutrition.  We have underlying issues and conditions that

these supplements can legitimately affect, especially when it comes to immune

system function.  It is important that we

treat cannabidiol just like any other therapy and ask the same questions we would

for pharmaceuticals.

Cannabidiol

has been an amazing tool and according to a blog.arthritis.org/news/patients-tell-us-cbd-use
, many with arthritis and autoimmune arthritis diseases

are trying it out.  A statement by www.who.int/medicines/access/controlled-substances/Cannabidi...
found that CBD had no potential for abuse, a good

safety profile, and most adverse effects were from pharmaceutical interactions

and underlying conditions.

Unfortunately, those of us with Chronic illness and fighting terminal

illnesses fall into those last categories.

We are almost always on pharmaceuticals and have underlying conditions

or symptoms we need to accommodate.  It

is up to us, the patient and consumer, to question claims being made, ask for

science, and check for interactions.  Always

double check the information being given with a trusted member of your medical

and/or legal team.

CBD works

because it interacts with receptor systems in our body’s cells.  While this is important for a healthy person

to know, it becomes essential when someone is considering CBD to help with

chronic/terminal illnesses.  A lot of

these interactions are desired, but people with chronic illnesses need to know

what to expect upfront.  Knowing there is

a risk to mitigate, whether legal or medical, can make sure the patient has the

best chance for success.  Here are some

common misleading marketing buzzwords that contribute to hype and general

misunderstanding.  Marketing is designed

to make you feel safe about a product.

All products have hype, and the CBD industry is no different.  Their products have some serious hype, and it

is our job to dig through it.

Here are the top 6 patient

picks as the “most confusing hype-lines”.

1.

CBD,

even from hemp and isolates, are not “legal in all 50”.  Internationally, the same ambiguous situation

exists.  Always check your local,

state/province, and federal/country laws before choosing a product.  You

may find there are THC limits, local areas may have banned hemp CBD, or some

other complication exists.  Most

CBD sales sites claim hemp CBD products are www.buscherlaw.com/state-hemp-legality, and will cite either the 2014 Farm Bill or the

2018 Farm Bill.  The 2018 Farm Bill did

deschedule cannabinoids found in hemp, but it placed them under the FD&C

Act, giving the FDA full regulatory authority.

The FDA classifies all cannabinoids as “www.fda.gov/media/112426/download
”, which makes them not so legal when introduced into

commerce.  Just as before the Farm Bills,

hemp and hemp CBD is subject to state legality.

This has created a very ambiguous legal framework that no patient could

really understand to comply with.  Add

this governmental nonsense to the constant hums of CBD marketers trying to

practice law and it becomes next to impossible to follow along.  Websites and hemp traders will sing about the

federal deschedule but forget to mention that many areas still consider hemp

CBD and CBD isolate to be illegal.  For

example, check out this statement from atg.sd.gov/OurOffice/Media/pressreleasesdetail.aspx,

“Current South Dakota law makes industrial hemp illegal and all

forms of CBD oil illegal.  The only exception is the prescription drug

Epidiolex which was recognized by this year’s legislature as a controlled

substance under SB 22.  Governor Kristi Noem signed that bill into

law on February 19, 2019, with an emergency clause, therefore having the law go

into effect immediately.

This action leaves any other use or possession

of CBD oil as a violation of state law.”

Always check with the local

authorities to see if CBD is legal in your area.  Legalities are complex and “legal” doesn’t

always mean “legal”.

2.      You will often hear phrases like “FDA

Compliant” or “FDA Approved”.  The only

FDA approved CBD product is Epidiolex.  If the product is marketed www.fda.gov/consumers/consumer-updates/it-really-fda-approved, walk away.

Epidiolex is a prescription CBD tincture that is rarely prescribed for

Dravet Syndrome and Lennox-Gastaut Syndrome and is the only FDA Approved CBD

product.  Also know that hemp CBD

manufacturers are still www.fda.gov/news-events/public-health-focus/fda-regulation-c... to come out with their regulations, and as

of this publication, they have not yet done so. (March 2020) “FDA Compliant”

can mean many things.  Their farm may be

FDA compliant in some way, not necessarily relating to hemp.  The packaging could be FDA compliant.  This phrase does not imply the product is

approved by the FDA, or that the product is more trustworthy.  Right now, any implied FDA stamp of approval

should raise eyebrows.  Make sure the

company is clear about what they are being “compliant” about and whether it is

relevant to hemp.

3.      In the United States, CBD (even isolate

use) is illegal in minors unless you participate in the state medical cannabis

program.  Make sure you consult with a lawyer that specializes in

CPS cases before you make any decisions.

It seems so

contradictory that www.youtube.com/watch

use when you can purchase it in every corner store.  Unfortunately, every state has a loophole

that allows them to investigate, and only a few states have taken steps to

protect families from CPS interference.  If your state has a medical cannabis program, protect your

child and your family.  We observed that

investigations involving parents who are actively on the state registry tend to

get their cases dropped.  Unfortunately,

a lot of doctors and CBD companies are telling parents that they don’t need

medical cards to give their children hemp CBD or CBD isolate.  Those parents are finding out the hard way.  If a salesperson is forceful and insisting

that you don’t need a medical card, this is a red flag.  For more information and

sources, check out this article themighty.com/2019/10/cbd-children-legal

4.      Watch carefully if you start hearing

phrases like, “You have an endocannabinoid system, so you need CBD.”  Yes, you have an endocannabinoid system, and

yes, it could plausibly be deficient.

However, the www.projectcbd.org/science/cannabis-and-immune-system, and not something you just want to throw

stuff at.  Right now, everything is

theory and pre-clinical and only the most popular theory (endocannabinoid

deficiency) is getting press time.  There

could be other issues like over stimulation, receptor issues, and more at

play.  Not every issue is going to be an

endocannabinoid deficiency, or specifically related to what CBD has to

offer.  Research is finding new

endocannabinoids, new receptors, and new functions all the time.  Chronic illness is hard on our body, and we

don’t have the extra leeway if we make a mistake.  Be on the lookout for those implying that CBD

is something your body needs. This is a commonly used phrase with no real

meaning.  Your body also has an opiate

receptor system.  That doesn’t mean you need

opiates or insinuate opiates are “good for you”.

5.      Be wary when someone implies CBD is safe

because it is from a plant.  Always

check with a pharmacist to see if there are any important interactions you need

to be watching for. Make sure

you critically think about any blanket statements being made.  As children growing up in the hills, we were

always told not to eat random berries in the woods because they could kill people.  There were mushrooms we couldn’t eat, and

plants we had to take great care not to touch.

Trying to make someone believe an active chemical compound is safe

simply because it’s a plant is nothing more than a comfort zone marketing

ploy.  The truth lies more in the

middle.  Cannabis does have interactions

within the body.  If it didn’t interact

with the body, patients wouldn’t be fighting so hard for access.  Cannabis works on multiple receptor systems, www.projectcbd.org/how-to/cbd-drug-interactions, and can even cause some adverse

reactions.  That is okay.  Often these interactions in the body are how

we get relief, start healing, and can then talk to our doctors about lessening

the pharmaceuticals.  I’d rather someone

be honest than to find out when liver ALTS measure 588.   We watch for interactions and side effects

with every other active therapy, whether supplements or pharmaceutical.  CBD and cannabis are no different.

6.      Is CBD from hemp or CBD from “marijuana”

better?  Guess what marijuana with a THC content at 0.3% or lower is?  Hemp.  Cannabidiol

the molecule is the same whether it comes from something someone calls “hemp”

or something called “marijuana”.  The

only difference is going to be THC levels.

Most CBD extracts come from low-THC cannabis, or hemp.  Whether an extract is effective depends on

how your body reacts to it, and what other things are in the extract keepitlegalcolorado.org/targeting-therapy not whether it was sourced from hemp or

“marijuana”.  If your body responds

better to higher THC levels, then CBD from “marijuana” (or CBD with higher

levels of THC) would be better.  If you

respond to low-THC CBD extracts, then hemp could be the way to go.  The plants are all cannabis.  Patients really should know what cannabinoids

and terpenes are in their products anyway.