Tuesday, April 7, 2020

No alarms, no appointments, and apparently no answers...

Braeden finished his 6 weeks of radiation this past Thursday.


It has been nice not having to go to Duke everyday, and it's been even nicer not to have to set those early morning alarms.

He is having a pretty quiet week, and has been working on school work and trying to get caught up on all of his missed assignments.

His last Avistan infusion is next week. 

He has a new cold spot on his right ankle, that can't be explained. This means that now all 4 of his extremities are showing some sort of unusual symptom.

His steroid "hunger rages" have definitely calmed down. This makes the short order cooks,who live with him, jobs easier.

Dr. Smith, the Neurologist called us on Saturday. The call was long, and we still have no real answers. Plans were made on Saturday for Dr Smith to get in touch with Dr Hussain, and schedule a test called SSEP (Somatosensory evoked potential).This test will measure his electric signals of sensation between his brain and spine. They will be looking at 3 different waveforms to try to pinpoint where the new issues are originating.

1st waveform will measure the brain's response to the the electric pulses.
2nd waveform will measure the spine's response to the electric pulses.
If the 3rd waveform ends up being slow or abnormal, it is likely the issue lies within his spine.
If both the 1st and 2nd waveform are abnormal, this will mean that the testing is unable localize the issue. And we are back at square # who knows by this point.


I think that we all are frustrated with the lack of answers. As his Mama, I want this crap to be done. I want Braeden to get back to his normal routine. I just want him to be a kid without the weight of this unknown demon to bear. I know his medical team is working hard to try to get the answers to his case. I told Braeden, he didn't have to be SO unique. Like really kid, quit stumping all the brilliant doctors at Duke and across the world.

Blogging allows me to have a visual timeline and record of what is happening with Braeden.

Blogging allows me to vent my thoughts and feelings.

Blogging allows me to tell you all, that I love you and I appreciate your support and prayers.


Below is a small part of Dr. Smith's visit notes, after seeing Braeden. I know it's a confusing read. I included part of his notes for my records. Feel free to skip to the end of this post for a funny Braeden-ism.

"This is a perplexing, and very unfortunate, case. I reached out to a neuromusuclar colleague here at Duke to discuss this case further, and I still feel that central (cord) pathology could explain the symptoms. This is largely due to the intact ulnar and median SNAPs, normal median CMAPs and relatively preserved ulnar CMAP amplitudes (right) - all in very weak muscles. Furthermore, there are other weak muscles that demonstrate normal findings on EMG (ie, RIGHT EDC). The low-amplitude LEFT ulnar CMAPs could also be explained by the underlying severe muscle atrophy as opposed to true denervation. To some extent, there may be a combination of both peripheral and central etiologies.

I do not have an answer at this time to explain Braeden's clinical condition. This is terribly unfortunate given everything this young man has had to endure. Regarding further testing to possibly help with localization, media and tibial SSEPs may be helpful, especially given the low concern for a significant peripheral neuropathy. Lower extremity nerve conduction studies could be performed prior to SSEPs to rule out a tibial neuropathy (which would confound tibial SSEP interpretation), but given the lack of any concern for such on exam, I do not feel that this is necessary.
Finally - I suppose it is possible that a "Hirayama-disease-like" picture could be contributing as well. Given his known C6 tumor, he does not meet the definition of Hirayama disease. But, a dynamic (flex/ext) C-spine MRI might be informative if it demonstrated significant anterior displacement of the cervical cord upon neck flexion. This is a long shot, but it is the only other consideration that comes to mind. There is no clear cord atrophy in the C6-T1 region in my opinion.
I am sending several screening labs for "idiopathic" peripheral neuropathy, but Braeden's clinical picture really is not consistent with that diagnosis.
I will discuss my assessment with Dr. Landi, and if he agrees, then we will proceed with SSEPs and consider flex/ext C-spine MRI. "




I will leave you with a "Braeden-ism"

In the car, on the way to Duke the other day. I told him how brilliant his answer was to the question I had asked him. He replied, "Mom, it must be all of that brain enhancement I had when I was a baby. You know? Those 3 little brain surgeries."

This child has my whole heart.

#BraedenBrave
#MrUnlucky

4 comments:

  1. He has the best heart and the best attitude. He is a true gem.....he shines brighter than anyone. Love y'all so much.

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  2. He has the best attitude. It's got to be difficult for him to keep it together all the time. Braeden is so wise. I learn something everytime you post. Thank you again for keeping your far away family & friends in the loop. I will continue to keep y'all in my prayers!

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