Ava came through her MRI beautifully. She managed to undergo sedation without needing a breathing tube, and that made her recovery much, much faster. Isn't she the sweetest little Valentine?
Immediately after her procedure, we met with our Neurosurgeon and Neurologist to go over the results. This was a difficult meeting because the results are heartbreaking. No one really understands what is happening in Ava's brain, or what it means. They don't understand why she's declining or know if it will continue. We do know what we told Ruby and Elizabeth this evening: that Ava's brain is very sick, and getting sicker, and we need God to do a miracle. In the mean time, we are trusting God for wisdom about how to best care for our sweet Ava.
Because the results were so confounding to the team, until now, we haven't laid out all the details. But the time has come to give you more details, because things are getting more complicated. To put today's results in context, Matt's going to take over and try to give you a clearer picture of the results of Ava's previous MRI and the subsequent brain biopsy results. So put on your thinking caps because this is going to get very technical! But if you can hang in there, know that you are blessing us by getting the scoop so we don't have to rehash all the details in person over and over again.
Based on the previous MRI and biopsy, we know there are four primary problems in Ava's brain. Unfortunately, most of these problems have progressed based on today's MRI. Going forward, keep in mind that on an MRI scan, abnormalities can be revealed either by the color ("signal") seen or the by the shapes/structure seen.
1. Ava's deep brain structures and the areas surrounding her ventricles show "enhancement" when a contrast material is given by IV. "Enhancement" occurs anytime blood vessels are leaky and the contrast material leaks out into the brain. "Enhancement" is typically a sign of infection, inflammation or tumor. Because she's not showing signs of infection and her biopsy didn't show clear tumor cells, inflammation is the most likely culprit. However, none of the doctors or radiologists here have ever seen this pattern, so none of them can even venture a guess as to what might be inciting the inflammation.
Today's MRI showed that every area of abnormal bright signal is significantly brighter, and that new areas are now involved. An especially problematic question remains: if this is inflammation, is it the primary cause of her problems and decline, or a helpful part of the body's response to whatever it is that's going on?
2. On her prior scans, the areas surrounding the ventricles have been too bright even when contrast material has not been given. Often, when this is seen in a kid Ava's age, it is due to a lack of oxygen at some point, usually from birth trauma/asphyxia. When that's the cause, the involved brain tissue dies and is reabsorbed over several weeks, leaving empty space where it once was. Although the area around Ava's ventricles have been too bright in a similar pattern, the brain tissue had been hanging in there and NOT disappearing. This certainly means that the areas weren't injured by lack of oxygen, but what was injuring them was unclear.
Today's MRI suggested that the disappearing has begun. It seemed to indicate the the damaging force has been present strongly enough and for long enough that these central regions of the brain have begun dying off and shrinking.
3. In addition to all the color/signal abnormalities, Ava's brain also shows structural/shape problems. First, the same areas that have the "enhancement" appear too "full." Not quite like a tumor or a mass, but bigger than they should be for her age. This could be equivalent to swelling from the inflammation, or it could be because of tumor-like cells infiltrating between the normal cells. The biopsy was supposed to help figure that out. It showed abnormal "astrocytes," but the pathologists could not agree if they looked tumorous or not (the vote for tumorous was 3 of 5). Astrocytes are the nurse cells of the brain and they are supposed to live in and around the neurons to help keep them well-fed and happy. On Ava's biopsy, they were too numerous and appeared filled with abnormal clumps of protein.
On today's MRI, the enlarged areas did not appear to have enlarged very much. Everyone is taking this as a sign that the abnormal astrocytes/nurse cells many not be as tumorous as some thought.
4. Also related to brain shape/structure is the fact that part of Ava's cortex is not folded/pleated the way it is supposed to be. These malformations of the cortex are the probable cause of Ava's seizures.
These abnormally folded areas have not changed much. In addition, Ava's 24hr EEG last week showed quieting of many of the areas where the seizures appeared to come from. That's a bit of good news, and for it we're thankful.
Thanks for your prayers today. We are still absorbing the news and have a lot to process in the days ahead. It blesses us that you're with us on this journey. We are being sustained by your prayers and support.
The LORD is the One who goes ahead of you; He will be with you.
He will not fail you or forsake you.
Do not fear or be dismayed.