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.