Again it has been a while since I sat down and typed out any sort of update on how things are going. When it is quiet on the pages of this blog it is anything but quiet in our house. Well except when Lucy is sleeping, quiet is a must if we want the world’s lightest sleeper to get any rest.
Speaking of sleep Lucy’s body has decided that it would like to have some, the words and I quote, “Mama…I tired” have been muttered from the sweet lips of our little girl. Lucy has been plagued with manic episodes for months. She will go days with little to no sleep, sleep for a few hours usually during the daytime, and then go days again with little to no sleep. Trust me when I say we have tried everything to get her to sleep during these cycles, the reality is that Lucy’s brain chemistry is different, changing. In addition to being awake comes changes in her behavior that are both concerning and heartbreaking. Her little mind is working overtime and as a result she is more sensitive to her environment and the people around her. She has a tendency to fixate on one thing, and until her mind is satisfied we are all going to hear about it over and over and over…again. I write about this with a bit of humor to my words, but in reality it has been some of the most difficult times in our house. Lucy has been extremely volatile, you never know when or what is going to change her mood and because of this we are all extra cautious around her. I have had to be the “mama bear” in certain situations and cancel appointments or limit her exposure to certain things or people in an effort to try and keep her clam and comfortable.
Two weeks ago we had follow up appointments with neurology and Dr. R, these appointments will go down in history as two of the most challenging appointments of all time for everyone involved, especially Lucy. Lucy demonstrated to both doctors her new and concerning behaviors, screaming inconsolably for hours all the while trying to do a neuro exam. What I need to express to you is that her heightened behaviors are not something you can try and discuss with Lucy, there is no negotiating or discipline that is going to make them stop. Our difficult reality is that the disease is starting to take her mind, the increase in seizures, the manic behaviors, dramatic sensory issues, irritability and agitation, and her worsening autonomic dysfunction are all as a result of disease progression. Dr. R sat with Drew and I during our appointment and admitted to us that this is perhaps the most difficult thing to have to deal with. We can treat a line infection, but this, this is not going to be easy to treat. It was an emotional appointment for all of us including our Dr. R, our options for Lucy are all things that are going to change who Lucy is, but in reality Lucy is changing and she deserves comfort. Admitting Lucy is always an option during these times to try and sedate her and keep her calm, although not my first option Dr. R wants us to know that it is always an option. We have made quite a few changes to some of the meds in Lucy’s med schedule in hopes of improving some of her issues. We switched her Risperdal dosing to an injection form vs. an oral medication because she is no longer absorbing meds through her intestines. As difficult as it is to have to give her a shot once every two weeks, Risperdal has been a life changing med for Lucy. We rearranged her anti-seizure med dosing in an effort to keep her blood levels of these meds more constant. We increased her Keppra dosing hoping to control the break through seizures that Lucy is experiencing, and added 100mgs of pyridoxine (IV B6) to help ward off the “Keppra-tuide”. We also increased her clonidine patch to two full patches vs. the one and a half she has been on for the past year in hopes of gaining more autonomic control(this is a crazy high amount of clonidine for a little one). Lastly we added in a narcotic for chronic pain control.
All of these med changes combined, a calm environment, a strict and organized med schedule, and routine seem to be helping Lucy. Some moments are better than others, and we are always thankful for a new day.