I have been really glad I started this blog. It has been such a great way to deliver news to people and also to feel supported by so many people. There are still posts, though, that I don't like to write. It's definitely easier to share hard information in one place than go through it with everyone individually so I'm glad I have a blog for news both good and bad. But I still find it hard to actually sit down and type out bad news. So forgive me for my delay in telling you about Max's MRI results. We got the results back from Max's MRI on Thursday and his brain is 'worse' than it was 6 weeks ago. It seems as though his brain is 'shrinking' or that the cells in his brain are progressively dying off. They expect this will continue to happen at least for a while still. At some point (maybe 2 years old?) it may/should stop. And what damage it does to Max between now and then we just don't know.
Why did this happen? Well we don't know for sure but I can tell you one of neurologists opinions:
When Max was 3 days old he had a pulmonary hemmorage or lung bleed. It was on Christmas Day. It was the same day Addison started breathing completely on her own without any oxygen or support. It was the first day I ever held either of my babies. It was the day I was discharged from the hospital. We went to my parents' house and opened presents and ate dinner that evening. Then we headed into the hospital to see the itty bitty not even 4lb babies. When we got there and went back, Max was in distress. He wasn't breathing correctly and had a horrible painful look on his face and blood was coming up his breathing tube. It was, up until that point, the most terrifying thing I had ever seen. I remember feeling that day that I understood the intensity of the love of motherhood in the joy I felt holding Addie in my arms and the terror I felt watching Max struggle.
The nurse practioner talked to us and I cried a lot and they got Max stable seemingly pretty quickly. No one explained things in the level of detail then and there that they would later to us so I don't know all the details of what ventilator settings changed etc. I just know he stabilized and I felt better. I also know they did a head ultrasound and didn't see any bleeds the next day and that was good news. And that they told us Max was in 'time out' for the next couple of days requiring a lot of oxygen and sedation and not being able to handle any holding or handling. Then Max got better and we got to hold him on New Year's Eve and then began all the mystery of Max like his low breathing rate and tight muscle tone and then that led to all the testing which accidently uncovered their kidney disorder and I suppose the rest is history...
And so I moved on from that Christmas Day experience. So many seemingly bigger or more chronic things came up and it stopped being the most traumatic thing I'd seen after all the times I've witnessed my babies stop breathing or end up on ventilators now. In fact if someone were to ask me about their medical history, I wouldn't even mention the lung bleed because I didn't think it was that significant.
Turns out it might have been very significant because that lung bleed might have been the moment that Max's brain started shrinking.
We don't know that for sure and most likely we will never know for sure. One neurologist seemed reasonably confident that oxygen deprivation during that lung bleed when he was so premature and fragile was what caused the start of progressive brain atrophy or cell death. Any time the brain is deprived of oxygen for any significant amount of time, damage is down. depending on how long the brain is deprived and the age of the person and the reason for the damage, the deprivation can cause a small and localised or specific area of damage to the brain. Other times it can cause a 'global' damage meaning the whole brain is impacted. This is what we've seen in Max and is obviously a harder situation to predict the true effect of. Sometimes the damage will happen briskly and all at once and you can immediately see what has happened. Other times a few cells die at the time but it sets off a domino effect whereby more and more cells will die from that point onwards. How fast they die and for how long they continue dying, well it depends on the person so it's impossible to know. It seems that Max's brain experienced global and gradual, progressive cell death.
However, our other main neurologist seems less accepting of this view that the shrinking is caused by the oxygen deprivation from the lung bleed and is still looking for a more genetic explanation. And so we've been running a ridiculous number of tests on Max. In one day, he had a skeletal or bone survey, an opthamological workup, blood cultures and the start of his 23 hour EEG, all in between Hemo of course! So far we haven't found anything leading us towards a genetic or alternative diagnosis. So unless we can find an alternative diagnosis, I think we will end up with cerebral atrophy due to neonatal anoxic brain injury (or brain cell death following a period of no oxygen to the brain as a little baby).
What all the doctors do agree on though is that every one of his MRIs shows a smaller brain volume than the MRI before. And of all the body parts you could have shrink, the brain might be the worst one. Having said that, the brain is a very complicated organ and it also is a heavily localized organ whereby certain parts of the brain have been allocated for certain functions. And interestingly almost all of your 'survival' functions have been stored in an area called the brainstem at the bottom of the brain. On top of the brainstem is the cerebrum which is responsible for actually Living now that your brainstem kept you Alive. And within the cerebrum there are four lobes which each have their own roles. This is a very simplified explanation BUT at the back of the brain you have the occipital lobe for vision. On the sides you have the Parietal Lobe for sensation and the Temporal Lobe for memory and emotion and at the front of the brain where your forehead is you have your Frontal Lobe for movement and problem solving. At the moment we see no indication that Max's brainstem is affected. I say that with no real confidence because the neurologist did ponder aloud with us whether it looked a bit thin but I don't think we think his brainstem is shrinking. Which for Max's survival is really very important. The atrophy seems to be in his cerebrum and most pronounced in his frontal lobe. Which would definitely explain his inability to produce much voluntary movement.
So in terms of what the future holds for Max, it is hard to know because we don't know how much his brain will suffer in the next year or so (there is research to suggest progressive atrophy will slow down or stop after approximately two years after the injury). We don't have any reason to think it is an immediately life threatening situation. There are certain skills however that were he to lose, it could lead to a worse prognosis, such as losing his ability to swallow secretions. We expect Max to have lingering serious disabilities but again, we don't know at this point what that will look like. We know that although his MRI was worse within 6 weeks (which is in itself a bad sign to see visible damage in a relatively short amount of time), BUT we did not see any obvious differences in his behavior in the past 6 weeks. So the correlation between what is going on in his brain and how it will affect Max's outward behavior is far from clear.
Needless to say, we are greatly saddened by this news. We were very much hoping to hear that Max's brain was stable between the two MRIs and we feel very troubled by the idea of his brain shrinking instead of growing. I don't think there are any words that could properly express how we feel. I don't really think it has truly 'sunk in' and while there are moments where it feels real and acutely painful, no doubt the denial and uncertainty are protecting us to some extent.
In terms of what we actually DO now, well, not terribly much in that there is no real treatment for this. Which is hard for us. I feel like surely there should be some brain dialysis we could be getting or a brain transplant we could be working towards. We are so used to dealing with their kidney issues that involve so many treatments that the idea of there not being treatment with this is difficult. After these rounds of tests are done, life will just go back to normal and neurology probably won't see Max very often. (Max will be in the hospital for a couple more weeks but that is for inpatient Hemo treatments and not because of this neurological diagnosis.) But, we keep getting him therapy and just keep trying to teach his muscles how to move. Max is social and interacts with us. He tracks things visually. He, for the main part, is very medically stable despite complicated kidney issues. He is incredibly sweet, loves to be held and smiles so big his whole mouth opens. He is adorable and loved and there are many wonderful strengths of Max's we will be building up and working with. Although he is not currently able to produce a lot of movement, the body can be trained and we'll keep working on head control and reaching. We are also ordering a 'Kid Cart' for him on Monday which is like a 'medical stroller' or really, a baby wheelchair. I remember not too long ago when we started questioning what was going on with Max feeling incredibly daunted by the idea of wheelchairs and all those things. But it'll be really good for Max and we feel happier than we thought we would with this step for him.
The most important thing to pray for Max is that God will protect his brain and keep his hand over the parts that are most important for Max. We are doing this EEG brainwave study to test for seizures. We have not seen any indication of them but absence seizures can actually go undetected. We don't expect they'll find any now but we would ask you to pray every day that God will protect Max's brain from developing seizures. Another concern is with his swallowing. Max has never had a strong swallow and when he bottle fed we had to thicken his feed. He now has tube feeds and his new procedure to eliminate vomiting. His swallow may not be strong but it is definitely still working now. We are praying that God protects his swallow so that he doesn't end up in any trouble with choking on secretions etc. We are praying for protection over his brainstem so that basic body functions are not compromised. Furthermore, we are praying that God would put his hand over Max's sweet smile and his ability to recognize us and interact with us. I won't say that it doesn't matter whether Max can walk or talk because it does, of course. But the most important thing for Al and I is that he is happy.
Of course those are the specifics but we will also continue to pray for medically unexplained healing. We are praying that if Max's brain does continue to shrink that God will protect what is most important to Max. But we are also praying above and beyond what the doctors have predicted. We are praying that God will stop the brain cells from dying now and that Max's brain will start to regenerate and heal. We are not sure how God will work in Max's brain but we know He CAN heal Max in big or small ways so we'll just keep praying for both.
Please pray for us as we process this and deal with it practically but more so emotionally. If you're reading this blog you already know we have had a difficult year and we have a lot on our hands and it is difficult to fathom dealing with a whole new set of issues. But we know God is faithful and we know we can get through things we never would have thought possible. Al's mom is coming out tonight and we are excited to have her here. We are grateful for friends and family and support. We are very grateful for love poured out on us during this very difficult time and even more for love poured out on Max, and Addison of course. We understand people often do not know what to say but we do love to hear from people and see people. Feeling lonely certainly does not help so keep texting us, emailing us and talking to us and loving us. It makes times like this manageable when you have people walking alongside of you.
Thanks so much for all the prayers and please keep praying for us all. And for those of you who see us regularly and know Max, please keep believing in Max, giving him cuddles and soaking up his adorableness. Al and I are actively choosing not to let his MRIs define who he is to us. Max is still the most adorable boy in the world who lights up our lives. Nothing has changed that and nothing ever will.