Diagnostics

Have an appointment for Cole today. Something's up, I just don't know what.

Back to being a detective.

What do excessive thirst, interrupted bowel routine, lethargy, poor appetite, constipation, excessive urine output (that is colorless), decreased ability to handle overstimulation, increased myoclonic activity and fever spiking equal??

I don't know either.

Hypercalcemia? Skeqwampus depakote levels? Unbalanced nutrition?

Your guess is as good as mine :)

Messy

Sorry for the utter confusion I possibly caused by the last post.

Things around here have been messy. There have been tastes of bitter and sweet. Things have been interesting emotionally and physically. I think the dishes have been done twice all week. I have had to let go of one nurse, interviewed a replacement...and she was our first no-show for her shift. So I told them to not send her again. Interviewed another. Decided to give her a chance, she is working out okay...however, is afraid of dogs. This could totally be an issue in the future, but she says she'll "try it out". Hopefully she can get used to the idea by Nov, I just need a qualified person at this point! Our main nurse has decided she doesn't want to work on Sunday...who does? That is the only day that I absolutely need someone, because I am gone at church. Yep, on the first week free to go to church all three hours, what do they do? Make me the Gospel Doctrine teacher! If that doesn't make you laugh, I don't know what will. Following someone who used to literally write Music and The Spoken Word. No pressure! Back to this week...then the weekend nurse quit because she doesn't want to drive an hour each way for a 5 hour shift. Can't say that I blame her...but still, I actually liked her! She can't do the day shifts because she is pregnant and will be on maternity leave right when Cole starts school. Had someone call and cancel an evening respite shift, the morning before, that I have been planning since nursing even started...scrambled and replacement #2 did some pinch hitting. SO-I am hopefully moving forward with replacement #2 for Tues, Thurs. In the middle of striking a deal with the M, W, F nurse for every other Sun, she puts in her two week notice and says that she can no longer work with Cole. AHHH! We really liked her and I seriously thought we were going to have her for years. Cole wakes up every morning asking specifically for her. So, having new Tues, Thurs take the other Sun and maybe just the whole she-bang. She has said she is available M-F. So, now just need someone to do Fri night and Sat and the other Sunday, right? Doubt it will be that easy. THEN, the agency now says that they can't have the nurse take the dog to school. Say what? I told them in the very beginning that this is what was going to happen. It's three hours, with a highly trained, very obedient, very useful dog! I promise that it will make their job even easier, not harder. We'll see what happens. I am grateful that I have this problem. It has just all hit at once. It's making me wonder if it is worth it...someone remind me that it is!

On a high note, we had our first double date in a year and a half last weekend. It included lovely company, good food and some spontaneous bowling (in dresses)! Saw Julia Child's kitchen, what a special moment for me, and the original Star Spangled Banner. Went to Relief Society and got to eat free food, always a pleasure. Took Cole on an outside play date and he survived! Met another 4 Paws family that lives in our area and is also going to be in our class in October! I also got to turn in my 30th birthday present that Brian bought before we actually moved here and we realized that cost of living is not just double, but triple what we're used to. Que sera sera. We still went, saw Mr Groban and it was awesome. Also got to indulge in my new favorite right before, Chop't. Seriously, I have thought about it every day. It's probably a good thing that the closest one is 40 minutes away. I am already addicted. The Cobb and Steakhouse salads are practically life changing...and don't get me started on the local cream soda! I'm hungry...next stop, Cafe Rio! They have a brand-spanking new one within comfortable driving distance! It is a scientific wonder that I weigh what I do right now. I am prone to eating the most fat-filled delicious foods, just small portions before the nausea kicks in. Where is the girl that could polish off an entire burrito enchilada-style by herself? I cleansed last week, but still not up to full eating capacity. I don't care that I have not weighed this much since I was 18...I just want to eat and have my clothes fit! Neither are really happening on a regular basis right now. Thanks gallbladder...you are awesome, not really.

I'm seriously contemplating returning to work part time. This is a whole 'nother ball of wax that I just can't ramble about yet, because it would likely be incoherent. Just take my word for it when I say it has been a struggle. A lot of back and forth with the Public Health nurse and the doctor trying to prepare for the Medical Review Board. Knock on wood, cross your fingers and every thing else that it turns out okay.

Same stuff just different packaging. Complicated and messy as ever. We are never vanilla. More like those crazy ice creams that have 8 different flavors in one. Rhubarb and Lime Cardamom frozen yogurt sounds insane, right? Sometimes though, it is the unique combination of flavors that makes something surprisingly sweet. Sticky, messy...but sweet. Just like our life and our little boy.

Sole

It's no secret that I love shoes. Funny, since I hate wearing them unless I am going somewhere important. You will always find me barefoot in the house. What does that say about me? I'm not sure I want to know! :)

These are my favorite. Black Reef flip flops, or slippers as we call them in Hawaii. Classic, simple, no-frills. No bows, buckles, bold colors, just two thin pieces of fabric in between my toes. Comfortable. Completely molded to my feet. Can go with anything from jeans to a skirt. I'm not even sure how old this particular pair is. I just continue to replace them when they wear out. Tried and true. The go-to shoe. I could pick one pair of the many beautiful shoes I have, most of which my husband has gifted me with, but you will find these on my feet the majority of the time. I remember once when I was home from college for Christmas break, I was wearing these in the snow. My father slipped me a $20 bill with the admonition, "Go buy yourself some shoes". I had other pairs, I just wanted to wear these. They were familiar.

I wish that everything was so simple. I know exactly which shoes I am going to put on if I need something comfortable. If I am going to get the mail. If I am going to the pool. You can't really ruin them. If they get dirty, just run them under some water and they are good to go again. Sometimes in life I wish that you could just wash away all of the dirt and reveal a nice sturdy foundation. Something that feels good and doesn't take a lot of work. I know them and they know me. We are sole mates (pun totally intended). It is nice to not add another thing to the list when it seems "The List" just never gets shorter...only things added.

Why can't decisions be as easy as going for your favorite pair of shoes?

You Can't Please Everyone

The Parable of the Old Man, The Boy, and The Donkey

There was an old man, a boy and a donkey. They were going to town and the boy was riding the donkey, with the old man walking alongside.

As they rambled along, they passed some old women sitting in the shade. One of the women called out, ''Shame on you, a great lump of a boy, riding while your old father is walking."

The man and boy decided that maybe the critics were right so they changed positions.

Later they ambled by a group of mothers watching their young children play by the river. One cried out in protest, "How could you make your little boy walk in the hot sun while you ride!"

The two travellers decided that maybe they both should walk.

Next they met some young men out for a stroll.

"How stupid you are to walk when you have a perfectly good donkey to ride!" one yelled derisively. So both father and son clambered onto the donkey, deciding they both should ride.

They were soon settled and underway again. They next encountered some children who were on their way home from school.

One girl shouted, "How mean to put such a load on a poor little animal."

The old man and the boy saw no alternative. Maybe the critics were right. So they decided to carry the donkey.

As they crossed a bridge, they lost their grip on the confused animal and he fell to his death in the river.

And the moral, of course, is that if you try to please everyone you will never know what to do, it will be hard to get anywhere, you will please no one, not even yourself, and you will probably lose your ass (donkey).
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This is one of my favorite parables. I used to try and be such a people pleaser. I followed all of the rules, tried to make others like me, do what I thought that they would want. In the end, I have realized that no matter what I do, how I do it or when I do it...someone, somewhere is going to have a different opinion.

and that's okay, because I probably have an opinion about what they are doing too; I just keep it to myself :)

Who'd Of Thunk It?

This time last year....
We had just barely gotten rid of the drop seizures. Were cautiously going without the helmet. Full fledged on keto. Finally had an oxygen concentrator in the house. Were seeing an increase in overstimulation issues. Only using Versed, Diastat wasn't working. Were still on house arrest. Lived in the west. Still didn't have official Dravet diagnosis.

Now...
Have a g tube. Not on keto. Have not only oxygen, but two pulse oximeters. Have 5 rescue meds we are rotating. Dravet. Have nurses. Free from prison. East coast dwellers.

Yesterday Cole swam in a chlorine pool, outside. Went to a restaurant. Then met up with some Dravet friends at IKEA...all in the same day, and without a nap. Normally, just one of those things would have been considered super risky and would have most likely caused a tonic-clonic seizure and a day of recovery. Today? He's fine and dandy. Slept for 13 hours because he had too much fun, but up and bright as a light bulb!

Mind you, he wasn't totally cooperative during his swimming lesson. Climbing in and out of the pool. He was also terrorizing the restaurant. Trying to use the carpet sweepers to "clean", running to the waitress station and trying to push the keypad. Wanting a credit card so he could slide it through the designated slot. Laying on the filthy floor instead of eating. A nice person who was staring at us the entire time, stopped by and told me that she thought he was adorable and that her son used to act just like him. She told me how that son is now in a Level 4 Gifted and Talented Program, so it meant good things that Cole was so "busy". Hmmm....didn't want to hurt her feelings, but we know better. He ran around IKEA like he was being chased, knocked over a few displays and got into a little trouble with a suitcase. Still, he did it. We did it.

The perfect place for an impromptu family reunion! Some were visiting from the southern part of the state and we couldn't miss the opportunity to see them.Who would have ever thought it was possible? I sure didn't this time last year.

Dravet.org

The IDEA-League has changed their name to dravet.org!

Still a wonderful community that we are grateful to be a part of, still up-to-date incredible information...just a different name.

One of these days I am going to get Cole's picture taken so that it can be on the website. They keep asking, and I keep getting busier! I need to update his story and update this site too. Falling on the priority list as other things come up, but hopefully eventually others who don't read Epilepsy Warriors can get hope and feel validation from reading about our little warrior.

Check out their new site. It is very user friendly and has great information about Dravet.

Review

Last week was stressful. Trying to orient nurses to our routines, Cole's seizure types and house rules is more difficult than you would think. It takes a trained eye and lots of practice to decipher what is a seizure, what needs intervention, what doesn't, etc. Then you have to add stuff like where the sippy cups are, which book Cole has to read before a nap, and how to use the 4 remotes for the television, DVR, DVD and playroom ancient TV. I know, we need a universal remote for everything in the house..would you like to buy us one? :) Seriously though, these things take time. On top of it my gallbladder has been reminding me that it really needs to be removed. Haven't cleansed for a while...need to add that to the list. Who likes to schedule time for throwing up though? Even if it does make you feel better in the end! I don't have time for surgery, so it has to be sometime soon. Really people, TMI..I know.

I have also been working on the Medical Review Board for school and had to create or help draft a lot of explanatory documents. One of them is from the doctor, and it was based on this list.
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I need you to express in this letter that Cole is a complicated patient. He has been diagnosed with an SCN1A mutation, and has symptoms consistent with Dravet Syndrome, so that is his main diagnosis. He has other needs that are taken care of by specialists, but we consider his neurological symptoms and all things directly related to his genetic mutation his primary disability. It takes a lot of time to get to know him and all of his various triggers. He has 7 identified types of seizures. Tonic, absence, tonic-clonic, myolconic, atypical absence, atonic, complex partial. To understand what type of seizure Cole is having and if it needs intervention, takes a trained eye and detailed observation over a long period of time.
-It is not certain just how much Cole understands or loses pre, during, and post seizure. He has had to re-learn skills in the past and others with Dravet have been known to lose skills completely. He will require a lot of repetition and patience and we are not sure exactly how much he captures in between his brain "turning on and off".
-He needs constant, 1:1 vigilant supervision to ensure his safety. He is at risk for falls and needs to be constantly assessed and monitored for seizure activity.
-He has no sense of danger and will put himself into situations frequently where he could be hurt. He will run into the street, jump into a body of water, can unlock and open doors, will immediately accept strangers, etc. He will need someone to intervene when he has compromised his safety and remove him from the situation.
-His oxygen has been known to drop very quickly and he needs to be assessed continuously with a pulse oximeter during major seizures and needs O2 support to avoid hypoxia. He must have an oxygen tank available at all times.
-Attending to him during seizures is a skilled need. His rescue medications have lost efficacy previously, so we have to rotate them on a periodic basis. What works for one seizure, will not always work for the next. It takes someone with medical training to be able to assess when to use them, how much to use and which drug to use. Someone who works with Cole on a daily basis would have this knowledge. He receives rescue medication through 4 different routes (rectal suppository, oral, intranasal, via g tube) to understand the procedures attached to which route is appropriate at what time is also a skilled need.
-He has a g-tube which he uses daily. He also can receive some rescue medications through this, so it requires monitoring and assessment. He also needs to have supplies available such as syringes, extensions, pill crusher, medicine cups and water for his g tube.
-Cole has sensory issues and will get overstimulated easily. Excitement, noise, patterns, textures, light, temperature and other things are triggers for his seizures.
-His overstimulation issues need to be considered during transportation. He does not do well when traveling, so the least amount of time on the bus would be preferred. Passing cars, light coming through the window, shadows from trees, all of these things can be triggers for seizures. We have tinted windows and he wears his FL 41 lenses when we are in the car. He also needs adequate heating and air conditioning as he cannot regulate his body temperature appropriately.
-He has special dietary needs.
-Cole has many features of dysautonomia including photosensitvity, temperature sensitivity, elevated heart rate and occasionally excessive thirst. All of these need to be constantly monitored and need intervention with special equipment such as Fl-41 lenses, a cooling vest, thermometer and a heart rate monitor located on his pulse oximeter.
-Cole will most likely need nursing for the rest of his life. Dravet is never going to go away and he will continue to need to be assessed and monitored for seizures, even if he looks like he is doing well on the outside.
-Dravet by nature is always changing and things rarely remain stable for Cole. There is constant adjusting of medications. New seizure types, new triggers, etc. Even though Cole presents as a happy, talkative child he still requires a lot of care and things can change in an instant. He could have a major seizure at anytime, so he is always at risk for losing skills, brain damage, or even death.
-Cole is often immunocompromised and will become ill when others do not. He will miss a lot of school due to illness, so it is important that he has as much opportunity as possible to attend.
-Cole struggles with new people and new places. It overstimulates his brain and it is a trigger for seizures, so to have the chance to have people that he is familiar with in an unfamiliar environment will be helpful for his overstimulation issues and can bring him comfort. Someone who is familiar with him will also be able to know when to remove him from a situation of overstimulation, to allow his nervous system to recover and prevent seizures. This continuum of care will provide Cole and his parents comfort and perpetual feedback on his condition.
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Heavy to see it all written out like that...and that's just the stuff that needs consideration for school.

However, some really great things happened in the midst of multiple reality checks and bouts of nausea.
*Brian and I went to dinner. By OURSELVES, without having to arrange for someone, teach them every thing and then worry about it all night. It was lovely, except for the whole I-felt-like-I-was-going-to-puke-the-entire-time thing, really...it was lovely besides that. We were going to try for a movie also, but I wasn't up for it. We calculated that it has been a year and a half since we have done dinner & a movie. Before that time, it was a year. Yikes! Having the weekends free if we want them has been kind of overwhelming. Date? What's a date? I guess we're going to find out ;)
*Took Cole swimming 3 times. He did awesome.
*Still no major tonic-clonics! Huh? Grateful...but huh?
*I went to a fabulous girl's only pajama party. It was spectacular, including the atmosphere and company.

So, in the midst of all of the crazy new there has also been good new...and some of the same old, same old.

We are a circus. Never boring.