MRI Results & Decisions

We met with our neurologist yesterday to review Macayla’s MRI from the 8th. There is a significant amount of atrophy in the brain compared to the last MRI in June. The atrophy is throughout the brain in all areas with the exception of the temporal lobes. These have some atrophy, but not nearly the amount as other parts of the brain. This is the area associated with memory and what a blessing that this area has held up so well. Her memory is still good and she still recognizes everyone and can even anticipate the next scene in a movie because she has memorized it. Hopefully, this will last much longer so that she will be more comfortable as her sight is lost. It will be more comforting for her if she can remember our voices and where she is and even be able to envision the scene in a movie from memory as she hears it even if she can’t see it. Her vision has been more of a concern lately and the MRI definitely shows thinning in the occipital lobe (back of the head) where the visual cortex is located. This may be why she is having some vision loss and inconsistency in sight. This is the stage that seems to be moving so quickly. Our doctor stated that this will seem to move quickly because we can outwardly measure her deterioration by the skills and abilities that disappear. Later, she will reach a vegetative state and things will seem to slow down. The brain stem will most likely deteriorate slower because it is more resilient than the rest of the brain and this will help maintain some of the more basic functions.
All of this past week, we have been challenged to think more and more about end-of-life decisions. Battens disease destroys the brain and as that happens there will come a point when the brain will no longer be able to keep a major system of the body going. At some point the brain may stop telling the lungs to empty fluid or the kidneys to function or the liver to do its job. The loss of a major system would be fatal. At present time, medical technology can augment some of these systems. If the lungs can’t function, then a person can be intubated and the lungs can be sustained by machine. However, with Battens the question becomes, “How many bodily systems do we sustain before we accept that the disease has killed her?” Macayla’s disease is fatal and it will shut down a major system in her body at some point. We are exploring the legal, ethical and medical aspects of Macayla’s future and we encourage anyone who is responsible for or caring for someone with a potentially deadly condition to consider the same. It is better to try to settle that up front as much as possible. Each situation will have to be considered as it comes, but we hope to have a general direction and decision made that will guide us through those moments. It is hard to always know what you will do until you get into a situation, but I hope by thinking about it now, we will be better prepared. Our honest prayer is that Macayla will either be completely healed in this life, or that she will be able to peacefully pass into the next life without discomfort. Either way, Jesus has her in His hands.

Snow D...uh...Slush Day

Jacob was so excited about looking out the window yesterday morning and seeing a world blanketed in snow. The sun wasn’t up yet, but snow reflects so much light that we could see very well. It gave us an unexpected day off from school and we ate a big breakfast and went out to play in the snow that was quickly converting to slush as rain came down. It didn’t stop Jacob from throwing snow balls at mom, dad, and Macayla. He soaked every layer of clothing and was freezing, but he didn’t want to stop. He actually threw a snowball at me in anger when I told him he had to go inside because he was too wet! We bundled Macayla up and put a poncho over her and her wheelchair and let her see it. Jacob liked being able to throw snowballs at her poncho. It was fun.

EEG, MRI, Tube and Speech

Macayla has been turning to the right in a rigid fashion lately where her head is turned as far as it can go and one of her legs will extend and become rigid. Sometimes it would last as long as a minute and we have been concerned that it was a seizure of some sort. We did an hour long EEG in the office and she didn’t perform. Macayla has done this before. We see a strange activity and go in for an EEG to see if it is seizure related, and Macayla will stop doing the very actions that got us there. And then it seems the EEG cures her for a few days after and the frequency of the strange episodes decreases. But now, she’s back at it and we still don’t know what it is. It could just be what her brain is going through at this moment. We will have a MRI in a few days and that may show us where atrophy may have advanced in her brain. We also just had her valve replaced on her feeding tube. It was a very simple procedure. I get the “willies” with the feeding tube thing for some reason. Cleaning around it (especially when a hair gets wrapped around it) just is not my cup of tea. But changing out the valve wasn’t that bad. We also started therapy in Anderson and met a new speech therapist who has opened up a whole new world for us. She has a vast amount of information about augmentative communication devices and ideas. These are devices and techniques that utilize the skills Macayla does have to try and communicate and interact with her environment. We wished we had learned this a year ago. There are devices that can allow Macayla to turn the TV on and off with a button she can manipulate. We are beginning to learn about ways to utilize the motions that Macayla does on her own now to make her world become more interactive. For example, she likes to dangle her feet when she sits in her wheelchair. We can put a switch near her feet that will turn something on (TV, toy, light, fan, etc.) when she dangles her feet. The hope is that she will begin to associate the movement with the result and make the movement more intentional when she wants to communicate. This is important for kids like Macayla who cannot learn new motions as well as before. It’s an amazing area of the speech therapy field to me.