a little of everything

First and foremost: thank you! I was totally overwhelmed by the response to my last blog and I apologize for not having gotten back to everyone! I realized I should have had ALL my ducks in a row with what I needed before I put all that out there. I have been hard at work ever since trying to figure out what I need and how to best go about all of this.

On that note, along with other epilepsy parents, we’ve created a group called Hope for Children with Epilepsy in North Carolina.photo

You can follow what we are doing on Facebook at https://www.facebook.com/Hope4KidsNC

We are following the lead of a group in Utah who is pushing for the same thing, to legalize the import of high CBD/low THC Cannabis oil. One thing that you can do to help us accomplish our goal is to write to your legislators. Email is ok, but actual letters sent via snail mail are what they pay most attention to. Its especially wonderful if you actually sign it by hand and use your own words rather than any template I create for you.

To find your NC legislators, click here and enter your address. It would be great if you sent letters to both your NC Senator and Representative. Thanks so much in advance!

The points that would be good to include are:

1)  that you support us, either Maddie Gorman and her family, or Hope 4 Children with Epilepsy, in our mission to bring new treatment options to children in NC with severe epilepsy

2) that you support the use of High CBD/Low THC Cannabis oil for treating severe epilepsy that doesn’t respond to conventional treatment

3) you could state how our story, or the story of anyone with severe epilepsy, has impacted you

4) let them know that you want them to contact me or Hope 4 Children with Epilepsy to discuss ways to make access to this lifesaving medicine legal for suffering children in NC. They can contact us at hope4childrenwithepilepsync@gmail.com, https://www.facebook.com/Hope4KidsNC or you can share my blog address with them

5) let them know that you will follow up with an email or phone call to their office to discuss the subject

I’m still trying to figure out what all else we’ll need to really get the ball rolling here. If you happen to know any lobbyists or aspiring lobbyists in NC that want to work for free to try to accomplish something amazing, please let me know or pass them my info! Part of the reason that I published that last blog when I did (before I was 100% organized) was that I had filmed a TV interview that was supposed to have aired that night. I wanted to have something up on the blog which explained my position on the off chance that the news piece didn’t fully represent my views. The piece was delayed and is now supposed to be broadcast on November 26th during the 11pm newscast on the CBS station in Greensboro NC. I’ll update y’all if it changes again and I’ll post the link to it once its available online.

 

In Maddie news, there has been a lot going on here as well. I am pleased to say that while she is still having drop seizures, they haven’t increased dramatically. That was my initial fear…..that once they came back, that they’d quickly get back to previous levels. For now they seem to be holding steady. I am truly devastated that they are back. But having seen them so much worse before surgery, I am still grateful to the relative safety that surgery has provided her. And for all of the amazing things she has been doing since that scary day in July.

 

Aside from the re-emergence of her drop seizures, there is another issue that has created a lot of difficulty in our life right now. I mentioned before that she had lost weight and that feeding wasn’t going well. Two weeks ago, we finally got in to see the Feeding Team at UNC. They had lots of advice and tips on ways to get her to eat, but after evaluating her, they felt that for the time being, it was important that most of her meals come from pureed foods. They felt she wasn’t chewing enough and that she was pretty much swallowing food whole. The good news is that she is still swallowing well, but we don’t want her to think its ok just to swallow food without chewing. So we have been pureeing foods ever since. This is a LOT of work. And it makes each meal extremely slow, especially since she is fighting eating at times still. So it takes a lot of preparation for me to be ready with nutritious, but fattening pureed meals to feed her 4 times a day. Especially since she is still gluten and dairy free and we try to eat all organic. So I spend lots of time in the kitchen fixing really strange stuff.

 

The Feeding Team also suggested changing her reflux med. It seems unusual that she would have had changes to her reflux (yes, our precious kiddo has been on high doses of reflux meds since she was two due to all of her chemo and then seizure medications) due to brain surgery. But is IS possible that she is so much more aware since surgery that she notices the pain of the reflux more since surgery. And that the pain might be why she decided to stop wanting to eat. The stopping eating could have then led to weakness chewing which may be why she stopped wanting to do that also. She saw a dentist last week and she is also getting in her 6 year molars right now and that can be very painful, but they didn’t think that would be reason enough to stop chewing entirely. So we started on the new reflux med. Two weeks into it, I can safely say that her eating has been consistently getting worse. She is now waking up many times a night and having trouble going back to sleep. That is a sign of reflux in a child that can’t describe the pain. So just today we changed back to her old medicine to see if we can get back to where she was sleeping through the night. She is eating the purees very well, and is much happier not being asked to chew as much. But part of this process is to teach her to chew again, so that eventually we can get her back eating normal foods. Her weight is just holding steady, but its better than losing.

 
We also saw the neurosurgeon and plastic surgeon at Duke to follow up on the leakage of cerebral spinal fluid from about a month ago. The neurosurgeon seemed to think it was a fluke and that it is unlikely to happen again. She will have a repeat Brain CT scan on Dec 18th to verify that there are no active leaks or fluid build up. Her incision had closed back up by the time we followed up with them and they both felt it looked good and closed this time and that there were no signs of infection. So again, we were told to just watch it, so that is what we are doing!
As always, we are so thankful for all of you wonderful people who follow Maddie’s story. Please continue to keep her in your prayers that her seizures do not increase and that we have NO more surgical complications.
Wishing you all a wonderful Thanksgiving holiday, hopefully surrounded by family or friends!
Love to everyone,
Liz, Brandon, and Maddie

Epilepsy Awareness

We’ve got lots going on, but I’d like to take a break from my normal updates to address something bigger. November is Epilepsy awareness month. It is also Maddie’s birth month. It is usually a month that brings out lots of emotions and reflection in me because it’s hard to get excited about your child’s birthday each year when your child isn’t progressing typically. Each birthday often just highlights the massive gap between where my child is and where she “should” be. Oct 29th was the anniversary of her leukemia diagnosis and so it’s always a bit tough this time of year for that reason as well.

This year as my contribution to epilepsy awareness, I’d like to take you readers through a synopsis of what epilepsy has meant for Maddie and our family. When she was diagnosed officially in Feb of 2009, we thought her seizures were just a temporary side effect of her chemotherapy and that we’d be able to get them controlled with medication of eliminating chemo. How wrong we were.

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Playing just after she turned two, a few days before the seizures began

She had been on her first anti-epileptic drug (AED), Keppra, since we first started seeing seizure-like behaviors. It did nothing but disrupt her sleep and make her behavior terrible. The seizures only got worse. After the official Infantile Spasms diagnosis, her first treatment was high dose prednisone, a steroid. This helped to reduce the seizures but stopped her from sleeping entirely. It also made her scream or cry pretty much the entire time she was awake. I would put her in the car and drive her around the 485 beltway in Charlotte during the wee hours of the morning in an attempt to calm her. That was the only thing that kept her from screaming. Well, that and breastfeeding. I’d guess that she was feeding 8 hours per day at least. And in between would eat an entire bag of Five Guys French fries by herself. She gained almost three pounds in one month’s time. It reduced her seizures a little, but because they were not gone completely and the known side effect of psychosis was becoming problematic, we began a long slow wean, during which we started her next AED – Vigabatrin. We actually saw an exciting 72 hours without seizures right as we started this drug and that led us to raise her Prednisone again in hopes that the combo would stop the seizures for good.

Instead, Maddie ended up in the PICU with life threatening sepsis. One side effect of steroids is that they mask the signs of infection. So by the time she popped a fever, the infection was rampant in her blood. We certainly knew steroids were risky in a child being treated for leukemia because she was already immuno-compromised. But with the type of catastrophic epilepsy she has, they felt it was important to treat it aggressively as soon as she was diagnosed.

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In the PICU when we finally got her off the ventilator and breathing on her own

Maddie almost died that day. And it took her a long time to get back the personality and spunk she had before that PICU stay. When she was healed, we finished the wean of steroids. Vigabatrin alone did nothing for her seizures. She stopped sleeping well during steroids, and it only got worse with the Vigabatrin. About that time, we began to see the emergence of a new type of seizure in her. This meant that despite our efforts, her epilepsy was actually changing for the worse.

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Precious as always, but sporting a little steroid weight

The next two drugs we tried were Clonazepam and Topamax. Clonazepam slows everything down, most importantly the brain. It did reduce seizures, but only when we had it at such high levels that she basically slept all day. So we pulled back to a low dose on it and moved onto the next. Topamax made her lose her appetite completely. I guess we were lucky she had some weight to lose from the steroid trial. It did not improve seizure control.

After failing those drugs, it was decided that she should re-try high dose steroids, but this time we would try ACTH, an injected steroid. So for 7 weeks, I shoved a syringe into her little thigh muscles myself to deliver this powerful treatment for her epilepsy. It reduced seizures enough that we escalated to the VERY max dose, but even then, we never got anything really resembling seizure control. It caused her to gain five pounds, not ever sleep more than three hours at a time, lose the ability to walk because of muscle atrophy combined with the weight gain and have extreme behavioral and emotional issues.  

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This is what high dose steroids do to our girl

After failing this drug, we flew to Detroit for the first time to consult the Infantile Spasms guru, Dr Harry Chugani. Maddie was found not to be a candidate for any type of epilepsy surgery after two PET scans. So we returned home with plans to try one more drug and then a less conventional treatment.

The next drug she tried, Zonegran, actually helped reduce the amount of seizures in each cluster. Zonegran  is one of the drugs we look back on as having been good for her – but it affected her cognitively, she seemed to have trouble remembering and using words, she had night terrors and the appetite issues persisted. We hit her max dose on it and were nowhere near having good control of her seizures and so we moved onto her next treatment, the Ketogenic diet.

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On a quick trip to Myrtle Beach for the USMC Ball before Brandon left for Afghanistan, she is playful and fun despite seizures and side effects

I had really high hopes for this treatment. It is thought of as one of the more natural ways to treat epilepsy, and by the time we got to it, I was feeling extremely frustrated with the lack of results from AEDs and all of the side effects she had to endure during each med’s titration and wean. At this time, she was still on Keppra, Clonazepam and Zonegran. That’s the trouble with epilepsy and AEDs. You often think you are having some seizure control and are afraid to make changes. Every wean seems to make things worse than before you introduced the drug, so at a certain point, you just keep giving them to your child (following your Neurologist’s advice) because it seems more dangerous to remove them.

Upon starting the diet, we had a few days of increased clarity and energy. But that was the end of her good results. Despite tweaking it and following an extremely strict regimen for 6 months, we never again saw anything good from the diet. She actually lost many of her skills while on the diet. As it was happening, we were constantly reassured that she was just lacking energy and that when we tweaked things to get her energy levels back, she would be the Maddie we knew and loved. That was never the case. When we finally called it quits, she was functioning at below a 9 month level in most cognitive and motor skills. I could leave her on a bed and she did not have the skills or motivation to sit up or roll to get off. She was three.

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Her third birthday “Keto cake”

While she was on the diet, we weaned her Zonegran. It increases the risk for kidney stones while on the diet and there is some evidence that the diet works better without many AEDs. Unfortunately, that only led to more seizures. So when she came off the diet, she was still on Keppra and Clonazepam. The next drug we tried was Lamictal. It comes with a risk of Stevens Johnson syndrome, so we titrated up extremely slowly to be sure she did not get the rash that indicates this dangerous condition. When we got to the target dose of it, seizures had only worsened, so we immediately began weaning it. At some point around this time, we weaned off of Clonazepam and onto Clobazam, a newer benzodiapine which has less sedative effects and supposedly patients do not so quickly need dose increases because of their tendency to cause physical addiction.

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I can walk again!

If you’ve stuck with me this far, thank you! I frankly hate reliving this….but I am putting all of this out there for a reason….

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One of her many EEGs to monitor seizure activity. She is not a fan

The next drug we tried was Banzel. Maddie was extremely blessed to experience 2 months without seizures. It was an amazing time. I remember running my first half marathon during this time and praying and reflecting back on the two years of seizures and thanking God for finally giving my child a chance at a normal life again. That was really the only time during her battle with epilepsy where it really seemed like we might actually be able to beat it.

So while Banzel gave us an incredible two months, it was not without struggles. Every time I increased the dose, she would completely quit talking and walking for a few days. It was obviously very scary to watch, but with each increase, I saw fewer and fewer seizures. It felt like something good might be happening. So we stayed the course. And eventually the result was good.

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Fun exploring at the beach during a time where she wasn’t falling constantly!

But that is one of the hardest things about epilepsy drugs. We, as parents, come to accept that there WILL be some undesirable side effects of every drug we try. We just hope the results outweigh the negatives. And for Banzel, they did. She is actually still on Banzel to this day (three years later) because we are afraid things could be worse once we remove it. But the two seizure free months back in late 2010 were the only good times we got out of it.

When the seizures returned, they came back worse. We quickly escalated doses of Clobazam and Banzel in an attempt to reassert control. We spent close to a year tweaking things trying to get it back. But we never got there.

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This EEG was right after the seizures returned when she was only falling rarely

Her sleep difficulties had led us (during this time) to try prescription sleep meds too, Tizanidine and Chloral Hydrate. Her doctor felt that if we could get control of her sleep problems, maybe we could reduce the seizures, since she was prone to them when tired. Both of these drugs just came with side effects, and neither fixed the underlying sleep issues. She actually experienced severe withdrawal symptoms while weaning off chloral. Eventually, we weaned Keppra and after 2+ years of awful sleep abnormalities, and we finally got decent sleep for a little while. We never really suspected it as the cause, but clearly it had been contributing to her inability to maintain a normal sleep schedule. I also then put my foot down that she would never again be on more than three AEDS at a time. I realize that sounds silly. Now, I think I should have said no more than two…..but at the time we still felt she NEEDED Banzel and Clobazam and we were afraid to see what would happen if we took them away.

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Another trip to Myrtle Beach for the Marine Corps Ball, just before another deployment

The next med we tried was Vimpat. By this time I had grown weary of the constant titrations and weans and how it wreaked havoc on every aspect of her life. We went extremely slow. This drug did not improve her seizures at all, but during this wean, a new, much scarier seizure type emerged.

Her next treatment started once we had moved to Raleigh and when we were near Duke and UNC and could get to a good ER more quickly. Despite having sworn never to use them again, we decided to try high dose steroids once more. So this time, we agreed to have high dose “pulses” of steroids, for three days per month. We started the first course in the hospital so that she could be monitored closely. We didn’t see major improvements, but were encouraged to give it more than once to work. After the second round, we called it quits when there was no measurable improvement.

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EEG monitoring at Duke while starting high dose steroid pulse therapy

By this time, we have run out of “good” drugs to try. There are a few that work well for other epilepsies, but not hers, that we can still try. There are a few others we haven’t tried that are contraindicated in her because of a genetic mutation she has and another because of her history of leukemia. So we decided to re-try a drug that had worked reasonably well for her in the past, Zonegran. After her second dose, Maddie went into her first episode of Non-Convulsive Status Epilepticus, essentially a seizure that doesn’t end. Luckily, Diastat, a benzodiazapine that I have kept on hand for years to use as a seizure rescue drug, stopped the NCSE without us having to go to the ER. That was the end of our second trial of Zonegran.

That is the point when we really began seriously considering surgical options, either the Vagus Nerve Stimulator implant or the Corpus Callosotomy surgery. We flew to Detroit for a second round of testing to make sure she was still not a candidate for any of the better outcome resection surgeries. She was not. After being sedated for the PET scan there, Maddie had her second episode of NCSE. At that point, the thought of trying any new pharmaceutical treatment was extremely scary, given that anything new introduced into her system seems to start episodes of NCSE.

We made the extremely scary decision for Maddie to have the Corpus Callosotomy surgery. As most you know, she had this surgery on July 8th. Her results were outstanding at first and she made immediate progress in many skills for the first time since she was two. It was amazing. But seizures have been gradually creeping back in, and just last week she began having a mild version of her drop seizures again. She is falling from them now, but I can usually catch her, as they are a little slower than they were pre-surgery. She hasn’t really started losing skills yet, but she seems frustrated a lot (possibly because she knows a seizure is coming).  So it is time for us to make a decision about her next treatment.

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Asleep in the PICU at Duke post CC surgery

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Her incision runs almost ear to ear, this pic is taken before all of the complications started with the wound draining

It will be an extract from the cannabis plant. The reason for dragging y’all through that whole history of pharmaceuticals is so you can see that we have not arrived at this decision lightly. She has failed almost all of the conventional treatments available to us and so we have decided to try something else. As you may know, medical cannabis is not legal in the state of North Carolina. I plan to work as hard as possible to convince legislators here that children like Maddie should have access to what has been life-saving medicine for so many other children with intractable epilepsy. I desperately want to be able to stay in our home, with my husband (and Maddie’s dad), near my family, where we have a great routine and a great support system. But we are not willing to wait so long that we allow for her seizures to get back to where they were pre-surgery. It is just too dangerous. So if I am unsuccessful in getting the law changed to allow Charlotte’s Web extract to be imported, Maddie and I will leave Brandon here and move to Colorado where she will have access to this medicine, which has been so successful for many patients like her.

Intractable epilepsy has shattered us in many ways. At this point, with Maddie about to turn seven and functioning under a two year old level, it is virtually impossible that she will ever catch up. But we cannot continue to play the same game, trying drugs that only slow her down even more cognitively. One of the most exciting things being reported by parents of kids who are just starting on Charlotte’s Web is that many seem to be more awake, alert, and interactive no matter the effect it has on their seizures. I am not Pollyanna-ish, I don’t assume this is a miracle drug for every child with severe epilepsy. But the fact that so many children who have failed most of the conventional AEDs are having such success with it makes it something very different than our other options. There is NO single epilepsy treatment that has the success rate that CW is having for kids who have failed the number of meds our kids have on average.

I do understand completely that not everyone will understand this, without the benefit of perspective we have gained due to Maddie’s epilepsy. I hope that you will support us anyway and support my fight to get the import of this extract legalized here. I will not be fighting to legalize medical cannabis as a whole, as that is a much bigger issue, one which cannot really be addressed in NC for awhile because legislation was just rejected unfavorably in April. What I am seeking is for an extract of the plant, which has less THC than the legal level in hemp products (which are legal to import), to be legal for us to import from Colorado (where it is legal to grow and be used medically). Because of the extremely low level of THC in it, it does not produce the “high” that is associated with marijuana use. It is instead high in Cannabidiol, or CBD, which has been shown to have neuroprotectant and possibly even neuroregenerative properties. This high CBD extract has potential to improve quality of life in patients with neurological problems other than epilepsy as well. But it is hard for much research to get done because of all the legal issues surrounding its growth, testing, shipping, financing etc. There is finally a drug being tested in clinical trials that is plant derived and is high in CBD. Unfortunately, we live nowhere near the trial hospitals and Maddie doesn’t meet trial criteria, as I understand them. I am extremely pleased that there is a drug in the pipeline, but we don’t have the 3-5 years it will take to get it FDA approved to waste.

To read more about Charlotte’s Web, read this. If you have time, watch the CNN Special report done by Dr Sanjay Gupta, entitled “Weed”. It is available here. I could cite loads more articles and new research being reported. If you are interested in helping me make this a reality for kids like Maddie in NC, please post your email in comments (it won’t show up unless I approve it) and I’ll email you back to talk about how you can help. You can also email me that way if you just want more information. As an extremely over-protective mom, a Republican and a proud Marine Corps wife, this is not a battle I ever envisioned myself getting involved in. But I would do anything for my child.

At home, recovering from CC surgery

At home, recovering from CC surgery

Love to everyone,

Liz, Brandon, and Maddie

More news

I just wanted to quickly update about how Maddie is doing. Two weeks ago, we ended up in the ER unexpectedly due to swelling under her scalp near her incision from her Corpus Callosotomy surgery in July.   The neurosurgery resident didn’t seem very concerned, but when the ER attending felt it, he wanted her rushed into a CT scan. It was a little unnerving, especially since 3 doses of Versed given via IV weren’t putting her to sleep. To keep her still in the CT scanner, she needed to be asleep. After 3 hours of trying to get her to sleep, they eventually assembled a sedation team and we got the scan.

The results showed that it was leaking cerebral spinal fluid. The good news was that it appeared clean, not infected. And they felt that though it was unusual, it would resolve on its own. We were sent home with guidance to watch her carefully for fever or any other sign of infection and to follow up with Neurosurgery in 2 weeks. So we will see both the neurosurgeon and her plastic surgeon at Duke on Thursday.   She has been doing normally for Maddie since then and the swelling has gone down. Her incision reopened and leaked again just for two days after the swelling appeared. We kept it super clean and I’m hopeful this is the last of the post-surgical complications. I’ll try to report back soon after the doctor appointments.

In other news, her EEG from last month showed lots of improvement. Unfortunately, since then, some of her old drop type seizures have reappeared. She has not been hurt yet, but now we know we are going to have to pursue our next course of treatment sooner rather than later. We do NOT want them to get back to how they were pre-surgery.

While this is devastating, I am trying really hard to keep reminding myself of all the good things that have come out of this surgery. The biggest and best thing is that Maddie is now most of the way potty trained. She actually did it mainly by herself. She just decided she no longer liked going in her diaper in front of people. So we had to leave her alone a few times of day for her to go. And when it was clear how much she was controlling it, we got an adaptive potty (where she is strapped in) and she began using it immediately. She still doesn’t understand the need to stay seated on the potty, but with the seatbelt and padding, it is a safe place for her right now. I sincerely hope the drops don’t progress enough that it is no longer safe for her to continue with this great new big girl activity.

November is epilepsy awareness month. I’m working on a long post talking about Maddie’s battle with epilepsy and I hope (when I post it) that you’ll share it with others. I sometimes still can’t believe how much epilepsy has turned Maddie’s life upside down. Most people do not know how serious epilepsy can be.

Love to you all, Liz, Brandon, and Maddie