Meagan did really well with the rest of her ICP monitoring considering the discomfort of the bolt. Friday morning the PA came by and helped Dr. R take the bolt out. They had only seen two spikes in ICP during the night when Meagan was sleeping which was odd, but not enough evidence for them to conclude surgery was needed. I still feel in my "mommy gut" this is a neurosurgical problem with structure or something that is not obvious, but I can't sit there and change Dr. R's mind. I will admit that I did get emotional when he said to my face was "nothing neurosurgical he could do" for Megs to try and help with her newfound weakness. I cried. Plain and simple. All the frustration of the last month and a half, plus not having a clear cut answer, plus having the answer I feel deep down is the culprit be pushed to the side all came out. Very unusual for me, but sometimes things come to a point where the emotion takes over. It appears now that we will have to somehow "prove" he needs to do some sort of surgical intervention, which is very frustrating. To be fair, Dr. R has always been good to Megs and had her best interest at heart - but I really have a gut feeling he is giving up in some ways on trying to help alleviate her current problems. Not sure if it's fatigue, being too attached, or just that he needs her to be in dire straights before he tries anything invasive, but in any case, as a mother who knows her child, it is extremely frustrating. In my heart I do believe that neurosurgery of some sort is necessary to help correct or at least attempt to correct her latest setbacks (and my hunch is on that shunt that while "working" may not be working ideally for her at this point in her development).... but, because of the reality, I had to accept Dr. R's conclusions (for the time being) and move forward with the next step to figure out her muscle weakness.
Following his explanation of everything to us, he said he would be passing us along to Neurology. Of course. When you have a complex kid with a new problem - what's the easiest thing to do? Pass her on to another specialist. We rolled with the punches, though, and agreed to meet with the hospital Neurologists. Dr. R suggested running a panel of blood tests checking for several different genetic markers, muscular abnormalities, neuro muscular markers, etc. etc... We were fine with exploring this because though I have my hunches, I do not know for certain - so ruling out anything myopathy or syndrome related is a good step. We did the in hospital consultation with the neurologists there. It was a lot of me head nodding and saying "oh ok" while they were talking, while at the same time realizing it was very clear we needed to forget them and go through Meagan's own neurologist for the testing. I was not impressed at ALL with these hospital neurologists and decided we would do the blood testing through Dr. F who has been her neurologist since almost birth. We got the list of tests from the hospital neurologists of what they wanted tested/ruled out. They ordered TSH plus free T4, Amino Acid profile, Carnitine Total and Free, Creatine Kinese Total and Serum, Organic Acid Analysis - Urine, and Acylcarnitine Profile, Plasma. A lot of complicated words having to do with anything from neuro muscular disorders, to metabolic disorders, to simple imbalances in her body's chemical make up. She went Thursday to have blood drawn and urine taken to get these tests going. So now we wait for those results which should be back to us early next week.
Being that we are essentially in a waiting game, Meagan's PT went ahead and asked to have her arms braced. Week by week she is losing strength in her arms and elbows, and her ligaments are being stretched beyond any healthy level. We were able to get in yesterday to get Meagan fitted for arm braces. Due to her current motor issues, and also her tiny size, she ended up having to be casted so the orthotics specialist can custom make her arm braces to both offer her ligament salvaging stability but also facilitate movement so she can continue to try and explore motor development. Meagan of course did not like being casted, but we were lucky enough to work with the same orthotics specialist we had for her AFO's so she did ok. The specialist was excellent with Meagan at distracting her, singing to her, and doing almost anything else he could to make her feel comfortable and relaxed and we were so thankful he was there. We should hear back this week if her orthotics have been accepted by insurance, and then the timeline of when they should be completed for her. She really won't be happy about these braces - but - whatever we can do to protect those ligaments is worth it. The last thing we need is long term, long lasting damage to very essential arm movements and connections she will need in the future for her everyday tasks.
Once Friday came and went, I really was comfortable or at least getting comfortable with Dr. R's assumption that this must be the cause of some myopathy or neurological breakdown. We had started a ton of reading, research, and acceptance on our part that Meagan has some other stuff going on. The older girls had a dance competition - Meagan was ok throughout the day - a bit fussy and sleepy, but who can blame her with the music going all day..etc. That night, Meagan was her normal happy self playing with my dad - singing, being silly and having a great time. Suddenly I hear my name being called - I came into the living room and Meagan was fairly lethargic but also fussy. She looked awful and I noticed the large soft spot on her head was extremely swollen and hard to the touch. I put her upright to try and help with gravity but her spot didn't relax and she became increasingly sleepy. At that point I decided to call the on call neurosurgeon. He advised us to watch her at home and if she changed behavior any more to come in to the ER. We watched Meagan for a while longer with her propped up in our bed and she remained lethargic and would not open her eyes. We decided it was time to head to Children's.
Luckily my parents were visiting so we could get in the car immediately. We started to drive and Brian sat in the back to keep an eye on Meagan. Her soft spot was still swollen and hard and she refused to open her eyes or rouse easily. Once we were about 20 minutes into the drive she started to stir a little. We pulled over to a gas station on the side of the road and parked so we could both evaluate her. Her soft spot was still tense, but not swollen and rock hard like before - and her awareness was improved. She was able to be woken up a little bit even if only for a second at a time so we talked about whether to continue to the hospital. If we got to Children's and she wasn't in the worst state she would have been, all it would mean was us telling them what happened, and possibly some unnecessary testing and sticks, so we decided since the situation seemed to be diffusing we would take her back home and watch her through the night. I am pretty sure I didn't sleep at all that night. As the night went on her soft spot continued to relax and she slept. Around early morning, I noticed her soft spot was back to it's baseline - very squishy and relaxed.
Fast forward to a few days later when I was driving the girls to dance class. Reilly suddenly yelled at me from the back seat that Meagan didn't look very good. I glanced in my mirror and noticed she looked "off".... the girls tried to wake her up but she wouldn't budge. I then saw that her soft spot looked large and hard. I pulled over at the next possible place and got out of the car to check on her. I took her carseat straps off to help wake her up - but she didn't move. She looked completely lethargic and out of it. I sat her up even more than she was and her soft spot did not relax. I was about to call Brian to see if we needed to rush her in but noticed her spot then started to pulsate a bit - it was tense but a lot less hard. Over the next several minutes, her soft spot relaxed and she slowly came around. Once I was sure she was ok, I strapped her back in and continued on to the girls' class, keeping a very close eye on Meagan.
I'm not really sure what happened during these two incidents - they did not look like a seizure to me and I have no other explanation to what happened. All I know are the facts - and the fact that both times her soft spot swelled up and got rock hard indicates structural/pressure issues not neurological issues. It almost made me frustrated at being thrown back to Neurology but what is one to do. I am thankful each time resolved on its own - but at this point when you have a kid as tricky as Megs, you almost want it to stay just long enough for the doctors to be able to see what you are talking about so they can have an actual picture in their mind of what we are trying to figure out. I did take pictures and videos so we have "evidence" in case it is needed but more than anything I hope these incidents to not happen again. They were quite scary with her so unresponsive.
That's really all as of now that we know. It is extremely frustrating to not know what is going on with her because it blocks us from having a treatment plan in place to conquer these new difficulties. To look at her, generally, you would never know - she is still happy, silly, and such a sweetie. But to see her losing skills and have trouble with her strength/fatigue level is very frustrating to me. She is so determined but her body is starting to not cooperate. Admittedly,this new issue is also worrisome to me. Why would she suddenly be losing muscle strength now at almost 3 years old? Why are just her arms showing weakness? Why is she looking smaller but not losing actual pounds? Is this weakness progressive? Is it short lived? Is this connected to her decrease in swallowing? Is it permanent but stopping at her arms? There are just so many questions and no answers as of yet. I am impatiently awaiting the results of her blood work this week. If there is an answer there, at least it's an answer and then we can decide what we are going to do to work through it. If there isn't, it will be back to neurosurgery with the normal results saying "Please help."
It's amazing to me to see Meagan's spirit in spite of some of her motor setbacks as of late. She still scooches around the room, ...and when she falls over just says "hi mommy" and has the biggest smile and will continue to play while laying down. She still does try to go over on to all fours to attempt crawling again, but when she does, she ends up accomplishing a big face plant right into the floor. Matter of fact the other day when this happened, I said "Oh Meagan, are you ok?".... she looked at me with the biggest smile and say "Sorry mommy!" Break my heart!
Meagan's smile and spirit are absolutely telling of her inner soul - no matter what happens along the way, she is ready to push through and figure out how we can work through it. Luckily we have a great PT helping us through this and trying to help Meagan figure out how to work through her weakness while at the same time being mindful of saving her ligaments from damage. Quite a frustrating few weeks as we go back and forth between specialists trying to figure out the root of Meagan's recent motor issues. However, I'm thankful that I'm the one along the way helping to figure this out. Because the fact that I have to seek the answers for her means she's mine. And I really wouldn't have it any other way.