September 9, 2014

Shrunken Brain, Mouth Surgery, and Finally

Whoops!  Sorry, Miss Peggy.  And anyone else who has been patiently waiting for an update.  Things happen so fast around here sometimes I am not even sure they really happened, never mind getting around to posting updates.
MRI... I was so confident the MRI was going to show absolutely nothing.  Then in the middle of the night before we were to go to CNMC I woke up with a crushing feeling in my chest.  I was absolutely certain the scan would show something horrible.  In fact, Addison's MRI showed no signs of stroke, lesions, tumors or brain damage which could have been the cause of his Infantile Spasms.

This leaves the question as to the cause of the high voltage waves on the right side of his brain.  There is nothing we can do at this time to know the cause or the prognosis of those brain waves so there is no real point in considering it.  We just have to wait and see.  In the meantime,  I am just going to assume it was a fluke related to weaning off the prednisone and trust it won't develop into another seizure disorder or a return of the Hypsarrhythmia.   

The MRI did show his brain has shrunken.   I am not certain of all the implications of this yet so to be honest, I don't think about it too much.  It can be caused by extended high doses of steroids.  His neurologist will be monitoring it with a repeat MRI in one year to see if it has returned to normal.   I spent some time considering if we would have made this choice had we known about the brain shrinking possibility.  And the answer is unequivocal, "Yes."  There really was no other choice.  The seizures continuing was not an option.

You have to keep a sense of humor at these times.  And when I think of shrunken brains all I can think of is Bloody Mary from South Pacific and those shrunken heads she sold.  Yes, I may be losing my mind.  But it keeps me from crying.

Our family mantra is, "The brain grows with use."  We intend to use his brain so much it will not matter how much it has shrunk. 

Meanwhile... We will continue to monitor all Addison's function and development from eating and sleeping to moving and learning in order to determine whether his cortisol levels are returning to normal after the 8 weeks of treatment.

And Speaking of... medication September 6th was Addison's last day on prednisone.  Only by standing in our house when we gave him the last syringe could you begin to envision how much excitement this created.  We look forward to seeing what Addison will do now that he is out of the fog of the hysparrhythmia and the oppression of the steroids. 

And speaking of what he can do... By Sunday Addison was reaching up in his bed to pull down on his toy elephant.  And on Monday he rolled from his tummy to his back.  Today he rolled from his back to his front, reached for a toy and brought his hands together to clap.  But the best thing of all was this.

It has been way too long since we saw that smile.  Addison's one neurologist said to me about a month ago, "I promise you will see him smile again."  When he gave us that big all ear to ear grin her words came back to me immediately. I was so excited I sent the video to her never thinking she might be concerned.  She emailed me back and said, "I thought you were sending a video of spasms.  Thank God you showed me the loveliest of smiles."  Thank God, indeed!

And other things to thank the Lord for... Addison has at long last, after 16 months of searching and praying and beating my head against the wall, had his mouth surgery.  Whew!  I mean what a huge sigh of relief. 

And God had to show us right up to the OR that this was His will and it was going to happen His way and we were going to have to trust Him to work out the details.  I am not exactly sure why the Lord worked it out just as He did but I have no doubt He was the driving force. 
We had already gotten verbal clearance from Addison's neurologist for him to have surgery even though he still had a few days left on the prednisone.  The anesthesiologist, neurologist and surgeon had spoken by phone and he gave them his verbal clearance.  But somewhere between there and the actual written clearance there was some kind of break down.  I am still uncertain as to why it was such an issue.  It took three of us (myself, the surgical center and the surgeon's pre-op secretary)  2 days on the phone with CNMC to get the correct paper work from the neurologist.  The time came for us to go to Connecticut and we still didn't have the written clearance.  It made me think of the Israelites crossing the Jordan in Joshua 3.  We had to simply go and trust the Lord to work out the details.  And He did. 

Right down to the woman we met in the waiting room when Dr. K came out to answer our questions prior to surgery.  I have done a great deal of reading on this type of tongue tie.  There are two parties of thought when it comes to follow up care.  One group says don't do anything.  The other group insists the mouth must be moved and exercised or the tongue will heal and the procedure will need to be repeated.  I seriously kept meaning to contact Addison's speech pathologist in CT to ask her opinion.  She worked with the surgeon develop this procedure for kids with Addison's situation.  That left me to think, if anyone knew what to do she would.  Except, we were side tracked by the seizures.  And we didn't think he was having surgery until right before hand.  Which was a holiday weekend.  And then we were so busy getting the clearances worked, making arrangements for our kids appointments while we would be away and packing, I completely forgot about the tongue moving business. 

So, when the surgeon came into the waiting room to talk with us I asked him.  And he said, "No, you don't really need to do that."  As soon as he left, a mom waiting with her children came over from the registration desk.  She said she had heard my question.  As it turns out she is a speech pathologist.  And she knows Addison's SP.  And she took the time to tell us she sees a lot of cases like Addison's which need to be repeated because they heal over.  And then she showed us just how to exercise his mouth.  Talk about timing a little bit.  She wasn't even supposed to be there that day.  Her kids had been scheduled for surgery a week or two before and someone had gotten sick or something and they had been rescheduled to the same morning as Addison.

Surgery went well for Addison.  We had been warned ahead of time he may need a stress dose of steroids.  His heart rate did shoot up and the anesthesiologist was a little concerned but he did not require the IV dose and about an hour post op the anesthesiologist declared him good to go. 
The other issue was his breathing.  Every time Addison has had anesthesia there has been the issue of him skipping breaths.  It could be caused by the prednisone, his small air ways or the anesthesia.  I believe this may be an ongoing problem and not related to, but rather complicated by, the anesthesia.  I think it may be something that happens in his sleep but is only noticed under anesthesia because we don't stand over him watching him sleep.  He has had episodes of cyanosis since he was born that is not related to his ASD.  From last fall, he went several months without any episodes that we know of. Then in April he had two pretty severe ones again.  His geneticist was concerned this it is related to a pulmonary issue related to Down Syndrome.  Back in May she had referred us to yet another specialist.  That whole issue was pushed to the side to deal with the IS.  We will see him in October and maybe shed some light on the situation.   Or maybe not.  I have concluded Addison's life is all about unanswered questions. 

Dr. K said he cut a lot more of the muscle than he expected.  He also said he couldn't cut as much as he really needed to.  Addison ended up needing 4 stitches which is pretty unusual.  So perhaps that gives you some idea how much tissue was interfering with the function of his poor little mouth.   Over the next couple of days we had lots of giggles watching him play with his new tongue.  It is really adorable.  And I can't wait until he starts puckering up those lips now that the tie has been released. 

Right out of surgery, Addison nursed well.  It was amazing for him to be able to eat like our other babies and finally get the sustenance his body needs.  It was such an emotional moment.   

After his initial feeding in the post op, nursing was quite an ordeal for him in the days following surgery.  I am told this is not unusual at all.  The changes in the mouth can make nursing a challenge when getting used to the changes in the mouth.  Because Dr. K had to cut so much, Addison had a lot more discomfort than is typical for this procedure.  For someone as tiny as Addison, we were quite concerned when he had gone nearly a whole day without taking anything at all.  We were traveling back to Maryland and had pulled over in a shopping center so I could feed him.  I had just spent an hour trying to get him to nurse and he wouldn't even latch on.  The whole time I was praying and praying.  Finally, We ran into the store to get some formula to get something else in him.  Although, we weren't really sure how we would get him to drink from a bottle or how he was going to swallow it considering he was still struggling to get medicine down.  The girls text right about then and I told them to pray for Addison to eat.  Right then I think I had given up faith.  One more time before returning him to the car seat I decided to try to get him to nurse.  But I didn't really expect he would.  The kid latched on as if it was never a problem and nursed for a full hour.  Later in the evening he did the very same thing again. 
When we entered into the idea of surgery back in June, we hoped it would not only aid Addison in speech but also help him with swallowing.  Dysphagia has been a problem for him since birth.  Trying to get medication in him for the last eight weeks drove home the seriousness of his disorder.  Speech became secondary to our desire for him to have the simple ability to swallow.  It has been tortuous to watch him gag and choke as we tried to get just 3 ML into him.  Sometimes it took up to an hour.  (Although, we did eventually develop some tricks that made it easier and quicker sometimes.)  I don't think even the doctors realized how very bad his swallowing issue was.  After surgery, the ENT nurse was trying to give Addison some Advil by mouth.  It took her 30 minutes to get it in.  Sigh. 

So we pretty much immediately found the surgery has had no impact on his ability to swallow.  I don't think it is lack of desire.  On Sunday I rubbed the tiniest amount of hummus on his tongue and he seemed to really enjoy it and even look for more.  Is it related to another swallowing issue in the throat?  Or perhaps he simply needs training to learn how to get food to the throat now that his tongue can move?  Just more questions.  We've already had two ENTs tell us they can do nothing more for his swallowing.  So this is a discouraging place to be.   

In steps God, again... So, the plan with our SP was another trip to Connecticut when Addison's mouth was finished healing.  That would be a time we could put together another program dealing with speech and feeding.  Initially, we figured we would get clearance for travel following his next EEG and hightail it out of here on vacation.  It didn't seem to make a difference if we delayed his follow up with the SP for a few weeks.  But now with the ongoing swallowing issues we weren't sure we should put it off another 6 or 8 weeks. 

It just so happens yesterday Addison's physical therapist mentioned she would be in Delaware on Friday to attend a conference to hear Addison's SP speak.  I shot a text over to Lori and asked if we could meet her in Delaware.  We spoke by phone last night so she could get up to speed on what's been happening with him.  She only has a very small window open when she will be in DE but she told us to bring him and we would meet after the conference.  And as it turns out, Trudy is already planning on being at the conference so she will be able to sit in on the meeting.  The idea of having one less trip to CT makes me happy! 

And speaking of happy.  It feels like Christmas.  After so long in the waiting place we are finally moving forward.  It may be fast or it may be slow and I really have no idea where it will take us.  We may end up stalled for a long time.  And I am sure we will face much more frustration and trials.  But, at least for now, it will be moving... somewhere. 


  1. So glad to hear some good news. We'll keep praying!

  2. So glad things are looking up! All three of my babies have had their tongue tie released ( but with laser at the dentist.) They didn't want to nurse much afterwards either. And we did do stretches and exercises too. Maybe the swallowing will improve as he gets used to his new mouth function? I know it took Arabella a while, and she never really learned to nurse properly as she was 14 months at the time of the revision.