May | 2017 | ChezPerky

Archive for May, 2017

Whole Family Support

Posted in Uncategorized on May 23, 2017| 1 Comment »

We haven’t fully moved Abby’s epilepsy care to CHOP, but we are 90% of the way there, and the thing that has convinced us that we are doing the right thing is that they have an approach that treats the whole family, not just the symptoms, the diagnosis, or even just the patient in front of them. They recognize that epilepsy affects not just the child whose medical record number and insurance plan they can bill, but the whole family who walks through their doors. The realize that they can treat the symptoms – the staring, the shaking, the electrical interruptions in the brainwaves, but there will still be significant impacts on the quality of life of the child, the caregivers, the siblings, and the child’s teachers and social peers. This is no small bit of understanding. Not only do they understand these things, but they actually are working to do something about it. Their ketogenic diet program includes the parent and child as equal members of the treatment team (and I’ve seen evidence of this being true and not just talk) – I’ll talk more about this later, but today, I want to talk about a different treatment modality, and one which I think many parents never consider – psychotherapy.

CHOP has a dedicated neuropsychologist as part of their Pediatric Regional Epilepsy Program (PREP) who is dedicated to treating pediatric epilepsy patients. While knowledgeable about the ketogenic diet, her role is not limited to the keto team. We would have consulted with her whether or not we were considering the diet (in fact, we had the appointment with her scheduled before we had pulled the trigger on the diet admission). Kids and families coping with epilepsy are coping with a number of issues – the emotions surrounding the realities of any pediatric medical diagnosis are significant, and all the more so if that child has experienced a crisis of that diagnosis as we have or has an intractable diagnosis, as Abby does. But there are also not-insignificant impacts of medication on a child – medication side effects include mood changes, but even the physiological changes (such as hair loss or weight changes) can have an emotional or social impact for which a child may need help finding coping mechanisms. Additionally, there are other social impacts that no one ever speaks about – or at least no one ever spoke to us about until we came to CHOP – what do we do if Abby gets invited on a playdate or for a sleepover? Do we explain her diagnosis? HOW do we explain her diagnosis? Do we send her rescue medication (which is administered rectally) and risk scaring off the other parent or just tell them that if there was a true emergency, they should just call 911, which is what I did before I had the rescue med (and would still do in the event I had to administer the rescue med anyway)? How do we find and train babysitters who are competent enough to take care of four kids, one of whom has intractable epilepsy and another of whom has ADHD and is autistic and another of whom has asthma, but all of whom are, at their core, basically normal, healthy kids? How do we, as parents, find any time to ourselves to get away from it all? To relax? To take three minutes to NOT worry about how we are going to find a babysitter in an emergency? How do we find support to ensure we have a sufficiently supportive IEP? How do we know that the neuropsych testing done at the school was thorough enough?

All these questions and more are reasons that CHOP has a neuropsychologist dedicated to the epilepsy program. At first I thought, “How do they have enough work for her?” but now I realize that she is probably severely overworked! I am grateful that they have her. We had a 90 minute intake appointment with her and while it was sufficient, I feel that we could have spent twice as long in her office and still filled the time. She recommended a couple of books, suggested that we review Abby’s IEP together to see if we can strengthen the supports in it, suggested having CHOP do their own neuropsychological testing (six month waiting list for that, but that’s okay – if she’s got better seizure control by then, it may be good to have it redone at that point anyway), and recommended that she do weekly CBT with Abby to help her with some coping skills for Abby’s mood and anxiety issues, and also to help get her “buy-in” to the diet as we draw closer to the program admission.

The more involve we become with CHOP, the more proverbial Kool-Aid I drink, the more I start to feel hope again. I am not sure I realized how dark and twisty I had been feeling for the last six or seven months until this fog started lifting just a bit. The last week (has it only been a week) has been such a huge mix of emotions all at once relieved, terrified, hopeful, scared, excited, and anxious – I’m ready to move forward, though. Through all of it, I’m mostly hopeful. Mostly the anxiety I feel is a good anxiety – I’m anxious that there’s so much waiting time. I just want to be moving forward and not sitting around anymore. I want to be taking steps toward better health, toward answers, not sitting in this endless quagmire of questions. It’s time for the fog to lift.

I am so grateful to have found a team that values the WHOLE Family. I don’t doubt that our previous neurologist also valued us as a whole, but the institution simply had fewer supports in place to hold us all up. It’s time to give up our sentimentality for a single caregiver and move to an institution that can take on the burdens we have been juggling all by ourselves. (who am I kidding? It’s mostly been me, not “us” – that’s the nature of being a mom, and it’s not because my husband isn’t willing; it’s because we moms micromanage this stuff.)

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Ketoge-what?

Posted in The Keto Chronicles, Uncategorized, tagged health, ketogenic diet on May 19, 2017| 3 Comments »

When Abby first got her diagnosis, it wasn’t supposed to be a big deal. Her seizures would be simple to treat and she would outgrow them in a few years and voila! But something didn’t feel right, so I took her to another doctor who revised her diagnosis. Her seizures weren’t quite as straightforward as the first hospital had led us to believe and the EEG report was inconsistent with the EEG itself. Her revised diagnosis made it less likely that she would outgrow this in a few years, though the unpredictable nature of the brain makes that sort of prediction guesswork at best. The revision did explain why the first med wasn’t working. So we moved to another. And that had horrifying side effects and didn’t eliminate every seizure. So we switched to another which wasn’t perfect but had fewer side effects and stabilized her even though it didn’t eliminate all the seizures. But then it stopped being effective so the dose went up and up. And then she was in status epilepticus, possibly because of medication toxicity. So we reduced the dose of that drug and added another. And she still isn’t seizure free and the side effects are awful. We are now transferring her care up to CHOP, but I will admit that although I appreciate their wholistic team approach, nothing – not one thing that they’ve suggested is something that our previous team hadn’t already suggested, so it’s not like they have any answers either.

I’m not sure where along the way we started hearing about the ketogenic diet. Our previous epileptologist ran the keto program for that hospital, but it still wasn’t something we talked about much – until recently as our options started slipping away. I didn’t know a whole lot about it except that I had been told it was like Atkins on steroids and I knew I wanted nothing to do with it. As a matter of fact, the way that we even broached the topic of putting her in the program was with a conversation that started with me saying, “And I know you’re ‘the keto guy’ but no way, no how am I going down that road. NO.” He didn’t push but did take the opportunity to discuss how it might benefit her and how we might go about figuring out whether it was for us. And the more I learned, the less I liked it, though I was beginning more convinced that we didn’t have any other better options. She had failed four AEDs (antiepileptic drugs), had been in status, and has seizures that are severely affecting her quality of life. There aren’t a lot of options for intractable/treatment resistant epilepsy.

Still, I didn’t really know much about the diet and the more I learned about it, the more impossible it seemed. A fellow at the hospital where she had a recent inpatient video EEG suggested that even if the diet didn’t cure her, it might help us get her off of one of the medications that is causing such severe side effects. It was a compelling point, so when we went to meet with the team at CHOP about getting genetic testing and transferring her care up here, we talked about the diet. CHOP’s program is a truly team-oriented wholistic approach that treats kids with a variety of modalities, but also addresses the issues that the whole family unit is facing while coping with the impact of epilepsy. We all came to the conclusion that if I didn’t at least explore the option of shifting to the keto diet, then we would be doing a disservice to her.

And, so, I went to CHOP’s monthly Keto Class for parents and caregivers earlier this week I received so much information about the diet, how the team works, how to enroll in the program, what we could expect our lives to look like. I’m not a doctor, and I’m not a nutritionist. I’m just a mom. So this post will *not* be a how-to manual for the ketogenic diet. I’m just going to give the highest level overview I can based on my (limited) understanding. I’m not going to get into the physiology of why it works either because although I basically understand it, I’m certain I would muck up the science enough as to make it worthless. So…. here goes…

If you do a search for “ketogenic diet” on any given day, you will find that most sites refer to a weight-loss diet that involves cutting carbs significantly so that your body goes into ketosis (the breakdown of fats in the liver produces something called ketones, which is called a state of ketosis; presence of ketones means that your body is no longer burning glucose for energy and storing fat, but is instead burning fat). There is general guidance on these sites that suggest what your intake should be for optimal weightloss in terms of intake of fat, carbs, and proteins (macronutrieints). However, they don’t talk about the specific ratios and restrictions of a Classic Ketogenic Diet used for treating medical conditions – most commonly, epilepsy. We’ve known for thousands of years that fasting stops seizures. When your body is low on intake (food/energy), it has a natural defense mechanism which allows your body to convert your fat stores to energy. When this happens, because you’re burning fat, you will produce ketones. In either 1921 or 1924 (I’ve seen differing accounts), Dr. Wilder at the Mayo Clinic posed the question of whether one could trick their body into producing ketones all the time without prolonged starvation. Indeed, the ketogenic diet doesn’t starve the body of calories, it starves the body of carbohydrates, which decreases glucose and insulin levels and increases ketones as the body starts to burn fat instead. The Classic Ketogenic Diet used for epilepsy is a medically prescribed diet. It isn’t a weightloss plan (though calories can be restricted for optimal weightloss if the patients aren’t already at an ideal weight). It is an extremely precise diet calculated specifically for each patient’s individual needs. Not only is it low carb, but it is extremely high fat.

The weightloss versions of the diet that I’ve seen have recommended a 70% fat intake to 5% carbs and 25% protein (around a 2:1 ratio of fat to combined protein/carb). The medically prescribed diet for epilepsy generally strives for a 4:1 ratio, which is 90% fat. Because fats are so calorically dense, meals are quite small (but, I’m assured, quite filling) and it is a calorie restricted diet. This is not about guesswork, as a weightloss diet might be. The 4:1 ratio is precise (once stable, the ratio may be shifted to 3:1 and sometimes even 2:1). How do you achieve that 4:1 ratio so precisely? All food is weighed to the tenth of a gram on a very sensitive scale to ensure that the ratio is strictly adhered to. All meals should be fully consumed to ensure no deviations from the ratio occur. There are virtually no “free” foods, there is no “cheat day”. It has been described as “the most restrictive medically prescribed diet to treat any condition, ever,” by medical professionals.

Why does it work? This is where the medical/sciency stuff is not totally beyond my understanding, but is beyond my capacity to explain it to anyone else. There’s an increase in GABA and a decrease in glutamate in the body which affects how nerve cells send signals to other cells. There’s a bunch of magic and jargon that happens here involving synaptic functions, electrical responses, neuronal excitability and hocus pocus, bibbideebobbityboo, somehow burning fat instead of glucose equals a change in brain function. Voilà! The changes in the brain equal fewer seizures and more hocus pocus because sometimes it works better than others and poof, you hope she’s one of the lucky ones.

Yes, the science is all more specific than that, but I’m not a 9th grade chemistry teacher, I’m not a neurologist, and I’m not, sadly, a super genius. I’m sure I’ve already butchered enough of the science and I don’t need the entirety of the internet shouting “SHE’S WRONG! IT IS OUR SACRED DUTY TO CORRECT THIS AT ONCE!”

duty_calls

Suffice it to say, I know it’s more complicated than all that, but hocus pocus seems like an easier explanation.

I am still in horror at the sheer amount of fat my daughter will have to ingest. I have severe gastroparesis and fat is the enemy in a GP diet, so it’s turning my stomach just to think about it. These kids on the ketogenic diet eat a ton of oil and mayonnaise – often just on their own – which makes me want to yak just thinking about it. I know that my biggest challenge is to stop making “that face” every time I hear the word mayonnaise and to sell this as “look at all these delicious foods you get to eat!” but even the neurologist admitted that the diet is pretty unpalatable at first. (However, CHOP is the only program in the country (world?) that has a “Keto Kitchen”, though. And they brought in a bunch of culinary student interns to work on recipes, teach parents, etc. The keto program director then hired “the best of the group” first on a part time, but now full time, basis to act as their Medical Chef Educator (aka Keto Chef). They teach quarterly cooking classes, develop palatable recipes, and help parents adapt recipes for their kids…. overall I couldn’t ask for better support in the “palatable food” department). Still, it’s hard to imagine my daughter eating mayonnaise or drinking straight canola oil and straight heavy cream. But, apparently, that will likely be our reality at some point in the not-distant future.

It’s overwhelming and scary and leaves me wondering why on earth I would do this to our family. And to myself. My health is so tenuous some of the time that I know this will take a huge toll on me in ways that will likely interfere with my ability to focus as much as I should on the rest of the family (though sometimes that’s the nature of crisis – for years Sam got 50% or more of my attention and the three girls had to split the rest, but now Sam is pretty stable so he needs less individualized attention, while Abby is in more of a crisis state right now and takes up a great deal of my focus much of the time. Sometimes it is Ellie, sometimes it is Tobie. Right now I’m actually juggling several major issues at once for different kids, so it’s getting to be quite challenging).

So why would we do this to our family? Because the alternatives aren’t great for treatment resistant epilepsy. Let me back up and give you some statistics/information from a slide that the neurologist provided at the CHOP Keto class.

1. Only about 0.5-1% of the population has epilepsy. This amounts to about 50 million individuals world-wide, and over 3 million Americans. (epilepsy is defined as recurrent, non-provoked seizures)
2. Between 60-70% of patients will become seizure free with any AED (you could pick one out of a hat), on the first try. This means that about 30-40% will fail at least one medication. After failing one drug, patients have only a 10% chance of the next drug working.
3. After failing 3 AEDs, there is less than a 5% chance of becoming seizure free with another AED. Abby has failed four AEDs so far.
4. The risk of sudden unexplained death from epilepsy (SUDEP) increases with every year a child has epilepsy. The risk is further increased for children with treatment resistant epilepsy who have untreated seizures for 5 years or more (Abby has had some level of untreated seizures for nearly 4 years – we have never gotten her down to zero seizures).

These, among other things, were the statistics that convinced me that no matter how overwhelming, anxiety-producing, or scary this massive undertaking feels, we owe it to our daughter to move forward.

And so, I did. Yesterday I sent the email to the keto team officially opening her record to work toward an admission to start the diet. There is a lot of homework we have to do before the admission can occur, as well as supplies we have to acquire prior to scheduling her admission. I have already started work on some of the paperwork. Some of the work has to be delayed until our insurance change happens on June 1 (my husband just started a new job) so that we aren’t working through prior authorizations with one company and then having to start all over again with the new one when it changes over. But the trigger has been pulled and we are moving forward. More later on the task of completing her file for the admission process, among other things. But … time to get back to the task of mothering.

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Dusting off the Blog

Posted in Epilepsy, Ketoge-what?, ooblahdeeoohblahblahlifegoeson, Uncategorized, tagged anxiety, Epilepsy, ketogenic diet, Kids, motherhood on May 18, 2017| 7 Comments »

For a long time, I’ve thought that I’d like to get this blog going again, but something is always in the way. Still, I feel the void left by not having this outlet that I used to have during my darkest years. For all my years struggling through infertility, this was my place of solace, of solidarity with other (far more clever, witty, intelligent, and strong) women going through the same journey, albeit each with our own paths. Even after my children were born, I found joy in documenting all the little firsts, the struggles, the joys. But the realities of my life started to make me feel too heavy to make my fingers move across the keyboard with quite the same regularity. Until, one day, my cathartic blog posts became so infrequent as to stop all together. Life as a special needs mom, life as a chronically ill mom, life as a disabled ex-professional-turned-homemaker… I couldn’t bring myself to write much more than quick, smarmy FaceBook posts about it – brushing off the challenges as hilarious little vignettes in the screenplay of my life. But without the catharsis of this process of journaling, no matter how many people support me, I too often feel alone with the challenges we face, when I know from experience I most certainly am never alone.

So, this is me. Wife, mother, sick person. Unabashed advocate for the needs of my children – who are all special, though some are more needy than others. If you don’t remember me, well, that’s because I wasn’t that memorable, or well-known in the first place. Let me reintroduce you to the cast of characters in my adventure. Maybe next time I’ll throw in some photos, but mostly I don’t want to put that kind of pressure on myself just yet. I just want to write and see where this takes me. So … the cast of characters:

The J-Man: Not really a current cast member, but an alum who never leaves my thoughts very long, so I feel the need to mention him since he is bound to appear periodically. Our first son, the J-Man came into our lives when he was a year old. We were asked to foster him for a year, but we had him for nearly seven. At the time that his genetic donor (I can’t bring myself to call her his mother anymore) called me and said she wanted him back, we hadn’t heard a single word from her in over four years. It was a shock. We spent a year litigating to ensure a safe, secure, and emotionally sensitive transition from our household to hers. But I recently found out that he’s not even living with her anymore. As I understand it, she can’t stand the sight of him, so she dumped him on a relative. When I found that out, I stopped calling her his mother. *I* am his mother. I always was and always will be, even if I never see him again. We have no contact with him.

Sam-a-lama-ding-dong: (he hates when I call him that, so I try not to do it too often). My vroombunctious 9 1/2 year old boy. He is so many things that it feels inadequate to simply mention his diagnoses, but I would be remiss in failing to mention that he is autistic and has ADHD. He probably is also bipolar or at least has some sort of mood regulation deficit. But none of those things are who my son is. He’s sweet and kind, funny, though he doesn’t always mean to be. Rigid, but working on it. Smart. So smart. He’s just a kid in all the ways that matter.

Ellie-bean: My sweet Ellie is also 9 1/2. She’s smart and curious and chatty and considerate. She is filled with empathy, quick to laughter, extremely thoughtful and has 87 questions about everything. She and Sam have a particularly close bond, which is such a joy. Ellie has pretty severe asthma, but she never lets this get her down, so I don’t either.

Abba-Dabba-Doo-Bah: My other 9 1/2 year old (yes, they’re triplets. No, they aren’t identical. Yes, they are natural – they aren’t made out of a space aged polymer.) Abby is ALL of the feelings. She’s funny and sweet, but moody and sad a lot of the time. She is quick to laughter, impulsive and very in-the-moment. She doesn’t dwell on the past and isn’t bogged down by worry about the future. She is simply carried by her particular mood right here, right now. Sometimes that makes life particularly easy for her, but othertimes it means she’s stuck in a sea of confusing emotions. Complicating all of this is that in addition to having ADHD, she has what is called “Treatment Resistant Epilepsy” (or intractable epilepsy). She is the reason I’ve decided to restart this dusty old blog. Our path toward trying to treat her epilepsy has been a difficult one and we are about to embark on the next leg of our journey by enrolling her in the medically prescribed ketogenic diet program in hopes of finding relief for her and hopefully improved quality of life for all.

The Tobe-ster (Tobie): My 7 year old demon-child. She gives the best hugs and smooches and is still super snuggly. She’s the only one who still wants to be tucked in at night and only Mommy does it right. She’s self-assured and bossy. Quirky and fun. Happy as long as she’s getting exactly what she wants every minute of every day. She is… special. She’s 7 going on 17 going on 27. She doesn’t like to work too hard, but she does love to play hard. There’s nothing that delights her more than dirt and puddles. Except, perhaps, mud puddles.

Seth (no nickname): Husband extraordinaire. Pharmacist. Father of the Year. Seth just started a new job that takes him away all week so he’s only home on weekends, which makes some of this stuff even more daunting, but he is always there when I need him (just sometimes via phone instead!) and he is a very present father despite his current employment situation. I’m grateful to have such a wonderful partner in our parenting adventure.

The Plot: Over the last several years, there have been a number of times when things have been so funny or joyous or challenging that I’ve had invisible posts written in my head that never make it to the cyberspace version of paper. But now, we are about to take on a new challenge – one which will test us in new ways. We are about to enroll Abby into a medically prescribed ketogenic diet program. This will require a significant lifestyle change for the whole family, but most especially for Abby and for me. The impact will be felt by us all, though, and I feel that I would be doing our family a disservice if I didn’t document the journey and use this as a resource for working through ALL THE FEELINGS that come along with such a complex and overwhelming path forward. I doubt the blog will always be about the diet – I have three other complex kids and a difficult medical condition of my own, so I’m sure there will be much of those things as well.

I hope that ultimately I’ll find this space to be a positive one, but for the moment, bear with me as I work through all the feelings I’m experiencing right now. I admit, most of them aren’t positive: fear, worry, anxiety, etc.

But … for the first time in a long time, there’s hope as well.

More to come!

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