Neurology

When Jonathan was doagnosed with a heart Defect in 1990 I thought that was a fairly vital bit of him gone wrong...lets face it if the heart stops thats it!
Jonathan's heart Defect was major and failry complex, BUT the cardiologist and Cardiac Surgeon explained it to me in detail, drew me diagrams and helped me understand what the problem was and how different drugs would help to regulate things and what surgery would achieve. I was able to watch a scan and see the faults in his heart and afteerwards see the improvement. It all made sense and was fairly easy to follow.

Fast forward a number of years and I have an MRI picture of a brain, I dont understand what bits are what, the varying shades and little specks of light and dark dont mean anything, I dont understand what bits do what and why the different specks of light and dark make a difference.

What is perhaps even harder to grasp is that the professional, the consultant, the Doctor who we rate highly for his knowledge, his compassion, his desire to help, He doesnt really understand or know why nor can he tell us what drugs will deffinitely help because of any specific action they have. He has more understanding than I do but he admits it is mostly triall and error, guess work and wait and see.
There is no magic fix operation that will turn a damaged faulty brain into a properly functioning one. The brain will not heal it wil continue to deteriorate. The symptoms we are seeing can equally be caused by faulty electrical signals in the forward part of the brain or by faulty control of the brain stem.
Eve has been presenting with some worrying symptoms for a while, most have been put down to Autonomic Dysfunction. This means that her brain stem is not controling the functions that are automatic, the ones we dont think about, Heart Rate, Breathing, Sweating, Temperature control etc.
However we have in the last few weeks had some very concerning issues that may or may not be Brain Stem and equally may or may not be Epileptic in nature.
I was able to give a very detailed description of what happened on two occasions. The second being more involved than the first and both being very frightening.

Eve stopped breathing, and didnt start following the normal tricks to start her. On the first instance I had called an ambulance, and was talking to Ambulance control before she made any effort, Then she was slow to recover but once she did recover although she was weary and wanting to sleep she was failry much back to her normal.

The Second time was preceeded by a period of Tachycardia, her heart rate was racing over 180 beats a minute and she was not looking well. She then stopped breathing and was unresponsive and took a while to begin breathing again. Once she did she began to twitch, her right arm and leg rhythmically jerking for between 10 and 15 minutes before she melted into my arms and slept.

Both of these episodes could have been triggered by a lack of control from the Brain Stem or from an epileptic event from the more forward part of her brain. There is no way of knowing for sure without catching one on an EEG. The treatment for the Brain Stem movement disorder is the Drug she currently takes which is causing her problems with it's side effects, increasing this drug further is not an option. Indeed the decision was made today to actually reduce the night time dose and increase the morning dose by the same amount to see if we could balance the effects good and bad.
The Drug that would give more control with less of the troublesome side effect would not necesarily control the Myoclonus that the first drug has controlled so well. However if these other events are truly Siezure then it would give more control of those, if not then it wont really help at all. Adding in a second drug has it's own draw backs, and the fact that Eve has Leighs Disease means she is at high risk of developing siezures if these arent already.

All of these drugs have a sedating side effect to a leser or greater degree, and as far as I can make out the degree of sedation has as much to do with the child as it does the drug. Eve sleeps most of the day already, even when not asleep she rarely has the energy to keep her eyes open when not needed. So adding in something that may cause further sedation isnt something we want to do lightly. On the other hand uncontrolled Seizures are going to add to the degeneration and so not controlling them isnt really an option either!

The outcome of this long and much more in depth consultation than I can possibly relate acurately here, is that we are going to continue with the original drug in an equally divided dose, however if she has a further episode of prolonged apnoea with altered behaviout afterwards then we are to contact them and start a change to something else.

Phew....are you following?

Rosie....well we have agreed to keep things as they are for now!
Thank goodness for that!