Turkeys Down

A rather southern friend of mine (shout out Susan Johnson) introduced me to a colorful turn of phrase I like to repeat to myself whenever external sources have me feeling blue, "Don't let the turkeys get you down!" (you have to say it slowly and wisely with a slight southern accent -- see if it doesn't work for you, too) has been a mantra that makes me smile and remember not to take myself, or any other turkeys, too seriously. Everything will be okay.

Trouble is, this phrase hasn't been working as well for me lately. The turkeys are pretty big and serious looking (not at all funny) and their combined weight has been getting me down. I don't know that everything will be okay and I am having trouble coping with that.

I'm also starting to miss Tigger. Very soon he will be returning to Colorado for 5-6 months of final specialty training. He will be taught to recognize and respond to the physical signs of Simon's clinical seizures (on a related note we figured out that the reason he has not yet been able to alert to a seizure before it has happened is probably because Simon is having seizure activity all the time. Tigger probably thinks it is normal... the kid must always smell like seizure!), he will be learn how to protect Simon and keep him safe, and he will be trained to assist Simon with balance and mobility.

All of these things are very important and the intensive training is a necessary step, I know, but I am still dreading the separation. Over the past 6 months Tigger has become an intrinsic part of our family -- his exuberant but gentle presence makes our home a happier place. Additionally, we've seen improvements in Simon's language and empathy when he is with Tigger, and he and Simon have steadily become closer.  

Simon's "too big doggie" gives the best hugs.

We need Tigger's help now more than ever, and I am as sad and worried now as I have ever been. I have some big turkeys pulling on me. Fortunately, today I didn't need to look any further than Simon's backpack for a lift. 

A friend's son, who has also battled epilepsy and who has been through the testing and surgery we may be facing, sent Simon a sweet stuffed puppy to brighten his day. Simon told me it was a "not too big doggie" and it does have a striking resemblance to our "too big doggie." N2BD will be good to cuddle with in the months ahead, when I am sure Simon's bed will be feeling conspicuously empty.

Earlier this week I got a lovely card from a family friend with a gift intended to send Brian and I out on a date, since she also knows from experience how stressful a situation like ours can be on a relationship. Over the weekend my sisters-in-laws have called to see how we are, my parents have been helping us cope with our over-burdened schedule, and my friends have checked in and sent love.

The truth is that hands are all around me trying to lift me up, and as I realize this I am reminded of something I said at the "TiggerFest" party we threw earlier this summer to recognize all the kindness we had been shown as a part of our journey to bring Simon and Tigger together: 

"This year, we have gone from feeling frightened for our son's well-being, to being embraced and uplifted by our family, friends, community, even people we don't know -- together, we have experienced a true miracle, and I can't thank you enough for being a part of it. I will carry this in my heart always and I know that years from now, when Simon is encountering more challenges as we all inevitably do, I will have the story of what happened here to remind him of how loved he is, how great people can be, and how miracles really can happen."

The challenges have come sooner than I'd hoped, but the rest of what I said rings true. All I have to do when I am feeling afraid and overwhelmed is to remember that sunny day when my heart was bursting with gratitude for how much love we had been shown. All I have to do is look around to see how loved we still are.  Knowing this gives me hope to believe that if one such miracle could happen, more are possible.  

A very special friend from another very special friend -- thank you for this "not too big doggie" Ian Moore!

What Is

We tried to take the weekend to rest, to regroup, to catch up. I'm not entirely sure we succeeded, but we tried. We spent time with friends, attended church and Olivia's field hockey game, caught up on work and homework, and we got as much sleep as we could manage.

Brian and I tried to talk about how we are coping with our new situation. We are worried about each other. We acknowledged the cosmic sense of unfairness we feel -- Simon does not deserve to be faced with so many challenges. He does not deserve to go through all that he already has, much less all he yet has to face. In his sweetness and innocence he has done nothing to warrant being subjected to so much frightening medical intervention, tests, drugs, and surgeries. He should not need to spend so much of his precious childhood in hospitals and doctors' offices. He does not deserve to have his hard-won abilities threatened by this monstrous disorder. He does not deserve to have his young life endangered.

We don't deserve to go through this. No one does. We are frightened, and angry, and overwhelmed. We tried to comfort each other, but in the end the best we could do was sit beside one another silently crying. Which, in its own way, was comfort enough for now.

But, being me, I can't sit still for long. Maybe one day I will fall back, but not yet. Now is a time for doing. So I am spending a lot of my time researching, and talking to multiple neurologists. We learned a lot in our meeting with Dr. Riviello and I need to figure out how to act upon it.

We learned that Simon's ESES is pretty severe and has most likely built gradually over time (how much time no one knows). I actually saw surprise register on the kindly doctor's face when he scrolled through the EEG and saw the unceasingly wild and wavering lines. He diagnosed Simon as having CSWS, a syndrome within the syndrome of ESES. Based on this, and on Simon's repeated status seizures (which the doctor was also very worried about), he feels we need to proceed aggressively with treatment.

This is what a normal EEG looks like during sleep

This is what Simon's EEG looks like during sleep.

The first thing is to try to control the epilepsy with more aggressive drugs and to frequently test their efficacy with overnight EEGs. If the more standard epilepsy drugs do not work, we move to high-dose steroids that are so heavy-duty they have to be given inpatient via IV for 3 days at a time. Ultimately, however, based on Simon's history of refractory (drug resistant) epilepsy and Dr. Riviello's experience with kids like Simon, the projection is that the effect of the drugs will not last indefinitely and we will most likely need to pursue a surgical solution.

Regarding surgery, we got more bad news when the doctor reviewed the MRI of Simon's brain. The best case senario would be to pinpoint exactly where the seizures are originating on the EEG, and then in reviewing the MRI find a corresponding area of damaged tissue which could be isolated and surgically removed without risking further harm. We do not have a best case senario. Although we know from the EEG that Simon's seizures are primarily coming from the right side of his brain, and though we can clearly see the damage done from the stroke, it is diffuse throughout the right side. Dead tissue is mingled with healthy tissue and we can't easily tell what Simon is using and what could be removed to help him without creating further injury.

So.... what do we do now? First, we start with drugs to try to control the syndrome as quickly as possible. We started this last week and are continuing to make adjustments and get bloodwork to test levels. Once Simon is at a therapeutic level we will get another EEG. If this works for now, great. If not, or if it stops working, we move to the steroids. I have already discussed setting this up locally with our neuro.

In the meantime, for the past 3 days I have been calling and emailing with multiple neurologists to try to find ANOTHER doctor to help us with the extensive and highly specialized testing that will need to occur (and which can take a very long time to do) to determine if Simon's epilepsy is operable and, if it is, to "map" that surgery for us and work with a neurosurgeon to implement it. Currently, I don't know where we will end up for that. We are discussing options in New York, Washington, Philadelphia, and Cleveland.

Right now, I don't have time to sit and cry silently. Though I think I have always been tough, and for a long time I have been trying to be a super mom (for both my kids) and an impassioned advocate for my special needs son; over the past two weeks I have felt myself evolving into something else entirely. Harder, more intense, more determined. A dragon mom? A warrior mom? I don't know yet. I don't quite recognize this more ferocious version of myself. Though I am reluctant to embrace her, I am also glad she is there. I think she will hold me together, and I hope she has enough strength to hold Simon, and Brian, and Olivia, too. I am frightened of what is to come, and how our world may change (has already changed), but I know I cannot stop it or change it or wish it away. "It is what it is" (a phrase I have a long history with) but also I know that I am who I am, and I am going to kick It's ass.

I hope.

Simon and Tigger take Manhattan

Today we went to meet the ESES specialist in New York. We decided to take the train to make things as quick and easy as possible, and because we thought Simon might like it (he did). Finally, after about 10 hours of traveling and 2 hours of meeting with Dr. Riviello at NYU, we are home. 

It was a very long day, but a successful one. Thank you to everyone that reached out to us today to tell us you were keeping us in your thoughts and prayers. Overall we learned enough to simultaneously give us hope and terrify us. I daresay that in the two hours we met with the good doctor we learned more than most doctors will ever know about ESES. Indeed, as Dr. Riviello pointed out -- this condition is so rare (it effects less than .2% of the patients presenting with childhood epilepsy) that it is often up to the parents to become the authority on their child's condition in order to care for them. Fortunately, that is a role I am already used to.

And it is a banner I will take up again in the morning. Right now, we are weary in every sense. Simon and Tigger have been tucked into bed and Brian and I are tucking into some sushi and a beer (or 2). We keep starting conversations about what we learned.. and then dropping them. We are still processing it all. We don't yet know what to think, much less what to say.

Rest assured, however, another post will follow soon. I will share what I know, what I have learned, and what our next steps will be. Thank you again for reading, and for opening your hearts to us. We feel the love and are using it to help us stay positive and focused on one step at a time. Our little man needs us all in top form. 

We took the train and got an early start this morning. Simon was super excited to be on a train -- he kept singing the "Chuggington" theme song and offering to take everyone's tickets. Tigger wasn't quite so keen on the experience. He settled down after a little while, though, and took a nap. Good thing since the walk to the office from Penn Station was just a tad over a mile.

Tigger and Simon made their typical grand entrance to an adoring crowd at the NYU Epilepsy Center. They are rockstars... Brian and I are just part of the entourage.

As usual, we bribed Simon with a sandwich to get him to cooperate through the 2-hour-long appointment. Tuna on rye from an authentic NYC deli... what could be better than that?

My boys.

Bye-bye city!

Naptime on the train for a very tired Tigger (Brian fell asleep too...). It was a long day after a long week. Now, it is definitely time to get some rest, take a little time to process all of the information (and emotion) we are faced with, and then--sharpen our swords and our determination. We will beat this. We have to.

Making changes

 

This is a photo of Simon's current night-time meds. It is practically enough to make a small meal of and it takes almost the same amount of time to prepare and feed it to him. The capsules need to be broken and combined with yogurt and fed to Simon. The liquids need to be taken slowly to prevent gagging, and the chewables come last... after dinner mints?

The "me" of two years ago would have been horrified to be pumping my son full of drugs. The me of today is just grateful that there is something to help Simon outside of the MAJOR brain surgery which is already being discussed. I'm hopeful that this cocktail is the most I will have to administer, but I know that more meds are on the horizon and will be our next steps in treatment.

Today we met with Simon's neurologist to review the actual EEG, get bloodwork, discuss new medicines (and their side effects) and schedule our next EEG for later this month. We pulled together paperwork and older test results for our meeting with Dr. Riviello on Thursday. We discussed how we could work all together as a team moving forward. Again, I realized that the me of two years ago would have been horrified to be surrounded by so much medical intervention, tests, and hospital time. The me of today is just grateful that we have the resources to get the help we need.

Sometimes I think that the me of two years ago would not recognize the me of today. I wonder if she would look on who I have become with a mixture of pity and/or misplaced judgements. Occasionally I'll see a reflection of my former self -- the confident and self-congratulatory mother of relatively healthy and well-behaved children --  in the eyes of another woman and I realize how far I have come and how far I yet may have to go. I see all in a moment how much I have grown, and I silently pray for that woman I don't know, that woman I was, that she may never have to walk down this path with me. I pray that she may gain the wisdom of a kind heart without ever having to go where others (including myself) fear to tread.

The EEG

It has been a while since my last post, and a lot has happened in the interim. Summer turned to Fall, schedules got crazier, and Simon has had a few more seizures. Not knowing what else to do medication-wise we decided to do an overnight EEG (electroencephalogram) with Simon to see if we could capture any information on his seizures that would help lead to more targeted treatment.

The EEG itself is non-invasive -- basically a whole bunch of sticky electrodes are placed all over the head and chest of the person being tested -- but it was still pretty traumatic for our little guy. I did my best to make the test as easy on him as I could, preparing him through viewing social stories and you tube videos of the process, and then trying to put a positive spin on it (I called it a "hospital sleepover with stickers!" I even brought popcorn and a new video), but he wasn't having any of it. As soon as he saw a hospital bed and a bunch of wires he imploded and we spent the first several hours of the test just trying to calm him down and hook him up. Eventually, we succeeded and he did fall asleep. The next morning the technician unhooked us and we were sent home after being told it would be 2 weeks before we could see any results.

I doubted we would find anything from the EEG. Our doctor wanted to keep Simon on his meds for it to get a "baseline" view and he had no seizures while we watched over him (all...night...long). I left thinking that the effort was futile and we would have to repeat the process another time without medication in order to learn anything.

I was half right.

We will be repeating the process, many more times, but not because we didn't catch anything. As it turns out, we caught a lot.

Monday I got a call from our Neurologist saying that the results were in, they were interpreting them, and she wanted to let me know that they had noticed some abnormalities. She asked me a lot of questions about Simon's development and progress. She specifically wanted to know when we started to notice any autism-like behaviors. She explained that there are epilepsy syndromes related to the abnormalities they found and she needed to do more research to be sure of what we were dealing with. 

I tried not to freak out (too much).

Over the course of the week there was a lot more discussion between myself and the KKI neurology team about Simon's EEG. Finally, he was diagnosed with ESES -- Electrical Status Epilepticus of Sleep. People who have this condition experience many, many sub clinical seizures (a sub clinical seizure is a seizure in which the brain activity is epileptic but there are no outward visible signs of seizure). Sometimes they have them while awake, but they mostly occur while sleeping. Over time, the constant seizure activity can erode a person's cognitive and physical abilities. It can cause language and communication delays (and therefore is sometimes mistaken for autism) and behavioral changes. It can even cause psychosis.

We learned that for over 85% of Simon's non-REM sleep he is having seizures, and that he also experiences them while awake. We also learned that this condition is very, very rare. So rare that there isn't a statistical prognosis or a set course of known effective action. So rare that no matter how many times I google it I only come up with the same handful of articles (which mostly state how rare the condition is). So rare that most doctors have never dealt with it and there is only one person with enough understanding and experience in it to be considered an expert. 

Fortunately, that expert is no further away than NYU, and doubly fortunate he seems to be a very nice and knowledgeable physician who doesn't mind answering a very worried mom's millions of questions for nearly an hour this morning. His name is Dr. James Riviello and we have an appointment with him on Thursday of this week. 

So, we are headed to NY in a couple of days and I am picking the blog back up. I know that somewhere out there there is another worried mother whose baby has just been diagnosed with a rare disease... maybe even ESES. For her, and for the many people that love us and hold us in the light, I'm writing again. Pray for miracles -- we need them.