Fight Or Flight

After a long day spent in Pensacola for Sammy's appointments, trying to figure out the origin of the sudden onset of the episodes he had over the past week, in hopes of creating a plan to avoid further life threatening concerns, it appears the specialists still find that our issues rest with his inability to effectively manage in "Fight or Flight" mode. His autonomic nervous system runs amok..yet again.

It seems our neurologist and cardiologist still think the episodes originate from a form of dysautonomia. This does not mean our pediatrician missed the mark by stating the event he witnessed might have started as a breath holding episode. He may have part of the trigger figured out but noone seems 100% certain on the entire chain of events. They think there is a misfiring in his brain that sets off a storm leading to bradycardia (very slow to no heart rate),a rapid drop in blood pressure, hypoxia and seizure. The trigger of this misfire is some mixture of external or internal stress, improper impulse control, involuntary breath holding, and hyper vaso/vagal tone. The behavior that seems like a seizure where his body is incredibly rigid and there is an obvious facial grimace and lack of consciousness, is likely a reflex anoxic seizure brought on by the lack of oxygen reaching the brain..not necessarily from not breathing but from a switch in how gas is exchanged and processed in his cells and is further inhibited from the heart not pumping it up to the brain at a fast enough rate. At least this is my understanding. The cardiologist said laying him down or him passing out and falling down should improve the ability to get blood into the brain faster as it stops fighting gravity. Stimulation with sternal rubs is key and getting to oxygen too.. but although hypoxia is a very important concern for avoiding brain damage, his concern is that the heart could stop beating and not resume. Sam has had concerning pauses in the past and we are now going to be watching to see if this remains a valid concern.

Our neurologist is ordering a continuous EEG to be done in their lab within the next week or so in order to see if we are able to capture an event. They don't think these are initiated by seizures but capturing one on EEG would bring confirmation. The Cardiologist has doubled his Glycopyrrolate (robinol) medication which has an anticholinergic effect by blocking the neurotransmitters which slow his heart rate. (Forgive me if my layman's understanding of these things is simplistic). But basically this medication lowers the vagal tone making it harder for messages to go out that slow the heart rate at dangerously low levels (a condition called Bradycardia). I like his cardiologist because he takes the time to explain things in a way that I can more easily grasp.

Until Sam's arrival I never even understood the nature of our heart rate and the delicate balance of our internal and biological rhythems. Everything has to be in sync or a traffic jam can occur. I liked how he described the heart as having one main job to do.. just to keep pumping and how the brain and nervous system are really the parts who act as the back-up pacemaker to ensure that the muscle pumps at a rate within the upper and lower limits. SO if the heart gets to pumping too fast or slow and that internal "fight or flight" is kicked into gear, the pacemaker or autonomic system needs to put things within the safe range. Sammy's does not always do this effectively and so if he decides to get mad and not take that next breath or take too many breaths, his internal autonomic switch needs to lay out the safe "fight or flight" limits but it seems to fail him and his heart rate plummets further dropping his oxygen saturation levels and bringing on a host of scary symptoms.

If on the new levels of medication, Sam has another episode than we will begin a 30 day holter monitering where an EKG is recorded during his events and based on those results, we might have to reconsider the pacemaker. This has been helpful to his Dr in the past. The pacemaker was advised a year ago for Samuel when he was having so many of these events but we saw the Robinol working and we opted to avoid the risks of major open heart surgery as it is with the placement of the pacemaker in an infant. The vessels of an infant are far too small for catheterization that is used on adults. This means the pacer has to be placed directly against the heart and will scar to the heart over time. The procedure is risky and I can't even imagine how we would feel doing this after seeing him nearly die following lesser procedures. This might also mean he would need more surgery later to change out batteries etc.. But that is getting too far ahead to consider right now.. for now, I will try to focus on the medicine changes and pray for no bad side effects. Those in of themselves are enough worry for this week. I wish I could handle things with more calm and grace, but honestly I don't think I could be anymore nervous after the stress of last week. I just don't want to see him having episodes so often as he did a year ago. It was a crushing time for me. On top of this, we are having normal family stresses of a new baby and such and I just have to keep remembering what is most important.

That said, I think my own "fight or flight" abilities are incredibly weakened lately. It would be so nice to say I have found the profound peace, the smooth ride (if you will), that I have so often sought with my planning and daily prayer... but I am just not there yet. When road blocks appear in my path or unsafe driving conditions loom ahead, I either take off in the opposite direction or worse I see red and can't even read the road signs pointing out the best route. I speed when I should slow down for dangerous curves and I find myself staring in my rear view mirror at things behind me that can't be changed. I lack the skills to survive when stranded on the side of the road and I can't seem to "fix" a single engine problem, let alone, change the tire when it blows. Basically, I lack the road savy skills needed to make this cross country journey alone.. so I find myself taking a lesson from Sam.. I will have to let God take the wheel on this one.

Searching for answers again..

SO it seems we are heading back down this road again... more endless questioning and confusion on Sammy.. I know God has taught us much in the school of life but I thought we had gone on summer break.. I guess school is back in session.

Here is what happened.. the quickest version I can type since I need some sleep and Max is needing to eat every hour on the hour.

We noticed this week that Sammy was a bit "off" but I could not put my finger on how or what was brewing. He was more fussy than usual. His coloring has been blotchy and he seems to be sleeping in odd patterns. He had some junky sounding periods after sleeping which would resolve after being upright. He has an open ulcerated area at the stoma of his G tube which is hurting him and causing him to yank at the tube.. so I thought maybe this was further pushing his mood a bit. Anyway, last weekend there was a moniter alarm for a brady that was asymptomatic. Then on Wednesday morning I was on one side of the house getting the girls ready for school and the nurse was on the other side changing a diaper when Sam apparently had a major hypoxic episode.. brady, seizure.. whatever you want to call it.. I am so confused at this point.. I just don't know. She described him exactly as I have seen in the past.. rigid, locked up and contorted. His skin purple and blue... not breathing and then going completely limp and then greying out and unconcious... the spelling on that looks wrong but I am exhausted and it is midnight.. so forget looking it up. I wish this thing had spell check sometimes. Anyways... he was out for maybe under 2 minutes and she put him on oxygen and then he started breathing again.

I guess she screamed for me but I could not hear her. This whole thing coupled with his recent funny behavior prompted us to call his Dr. but apparently he was out for the day and noone called us back. Then he fell asleep and we hoped he would get some calming rest. It was a few hours later when he awoke that we again noticed the junky breathing and arching pain. He did not want to be held and was irritated by everything done for him. We vented his tube and there were little black things in the fluid which seemed likely to be mini pieces of blood. Within the hour he seemed better and went out to play with his sister in the family room. I was in the kitchen when the nurse decided to puch in his meds while he was trotting around beneath my feet. I offered to hold him as he has been yanking at his G tube chords whenever they could be accessed. As I reached down to hold his hands, he instantly locked into the contorted rigid posture and stopped breathing and again became hypoxic. The nurse grabbed him up like one big solid log and ran him to the oxygen and again he went limp and grey in her arms. This one lasted about a minute and a half or so. WHY WHY WHY.. 2 events in one day after such a long time without incident?

Yes, we used to experience this constantly when he was a newborn home from months of investigation in the hospital.. and no they never fully understood these episodes.. but they seemed to respond to the medicines he was put on for seizures and for brady/ autonomic vaso vago syncope events. These have always been distinct from the bradys where he just drops his heart rate all of a sudden and turns grey..but I guess we came to think this was the same except that the heart rate maybe stayed down low enough to cause a hypoxic type of seizure. Yes, they have captured on on EEG during a code blue in the hospital and it did not show as a typical seizure but I am just perplexed because the one thing that did seem to be ruled out before has now yet again surfaced as a question.. breath holding or apnea.

Let me explain why this old theory is being resurrected. SO.. after having 2 of these events, we called the Dr yet again to see if we should head over there and they said probably not since our Dr. was out and the Dr covering was so unfamiliar with Sam. So.. I called the cardiologist and neurologist and left messages. The cardio nurse returned my call with a voice mail saying check in with pulmonology?? and they neurologist did not call right back. SO I made an appt with our pediatrician for the next morning hoping it was just some ear infection or something bringing on these symptoms.

During the exam, low and behold, Sam decided to perform for the Dr and sho him a mini version of this type of episode. I was actually somewhat happy to have him witness it. Again he looked incredibly scary and rigid and seizure like and hypoxic and then limp and then unconcious. This time, however, we did not have our oxygen and it was scary seeing him lay there so blue with no resources to help him. The Dr seemed stunned and did not even really seem ready to jump in to bring him back up himself. The nurse worked to stimulate him and within a minute he was back up and looking at us again. The Dr. threw up his arms and said it was beyond his realm of expertise and that we needed to see neurology and get on an anti convulsant right away. But then I explained that we are already on one (somewhat suprised he did not remember that since I am farely certain he has few kiddos as complex as Sammy).. but he said then that we needed another one and that he needed to not be provoked or made mad etc.. and that this could be very concerning behavior for a child with his underlying condition. He then explained that he thinks this was a breath holding event that led to hypoxia. BUT THIS SEEMED SOOO ODD TO US SINCE HE NEVER SEEMED TO HOLD HIS BREATH.. He went into the seizure looking behavior too instantly and it seemed completely out of his own control. He was not "there" if you know what I mean.. so chicken or the egg is again my issue.. seizure leading to hypoxia or hypoxia leading to seizure.. what happened?

I will not go much deeper into what our nurse had to say about it but let's just say that we are left very skeptical about this Drs opinion. This may be partly because he then seemed annoyed when the neurology office asked for a seizure medication blood check to see if he was at therapeutic levels. WHY would he question this request if he just got done telling me we needed another anti convulsant? Also.. further.. with his mito condition clearly being one where the crash of symptoms is worse with illness or underlying stress, why did he not order a CBC or dive a little deeper into the question of why this kid who has been mainly episode free for months and months all of a sudden starts having a string of episodes in one 24 hr period? Seems odd but I am not the Dr.. so I will have to ask these of the next Dr of which the buck has been passed to.. (sorry if I sound sarcastic or tempermental.. I am just so so so tired of this ping pong game of which new Dr will take a hit at the ball, with everyone hoping it falls under the next specialty brought into it).

SO.. then in final summation of where we are today. Luckily.. no episodes today and happy in the morning but very moody this afternoon.. We got a call afterhours from the nurse reporting that his seizure med levels had dipped below therapeutic levels and that the report would be sent to the neurologist. Then the nurse became concerned that it should not wait to be addressed till monday's appt so she called the afterhours neuro team and they (hearing the event info) decided to up his dose. Of course they and we have noo idea if our Dr is right and this is some kind of self inflicted life threatening breath holding event or if it is some reflex issue relating to his autonomic nervous system, a lowering of Heart rate/ brady or if perhaps it is a seizure. I just don't think anyone is entirely certain..but one thing is for sure.. they all feel these are very dangerous and he needs a management plan or to figure it all out quick. Until then, his Dr instructed that we allow no stress on him.. no pushing his buttons basically. He told Sam that for now he needs to be basically spoiled rotten.
Not so easy to do with 3 other kids including a newborn to consider..so.. praying for answers, peace and less frustration. Praying that the med increase stops the episodes and that he stays pink all weekend. :)

Maddox Alexander

After waiting so many months in great anticipation of meeting the little guy who had long been kicking me and keeping me up all night with his hiccups.. I am pleased to finally introduce our newest little man, our "Mad Max"..

Maddox Alexander Negron arrived January 24th, 2012, weighing 8 lbs 10 oz, and 20 inches long. He was a planned C section for 3 days before his due date. This was necessary as our local hospital does not have the resources to ensure a safe VBAC. Everything with the surgery went well and he was crowing loudly from the moment of his birth. He seemed to be crying, "Look Mom!.. I am here and doing great!" There is no sweeter sound!!

I know this is a graphic picture but I felt it really showed how caring the hands were that brought our dear youngest boy into the world.. it almost looks like she is praying as she holds his head in her hands before delivering the rest of him.

We were so thankful for the efforts made by our OBGYNs to ensure a smooth and safe delivery. They were so thoughtful in considering the bad experience we had during Sam's delivery and thereafter and keeping that in mind throughout my pregnancy and delivery. I felt far more secure in their care.

After delivery, Maddox was taken away to be weighed while they completed the tubal ligation portion of my surgery. Maddox will be our last child, officially completing our family. I was soon allowed to nurse Maddox in the recovery room and he took right to it. This was very encouraging to me. Things were going so well that it was a bit of a shock minutes later to be told that Maddox had been admitted to the NICU for monitering. This was devastating news for us as the memories from Sam's delivery came flooding back.

It seems that when they took Max to the nursery for his first bath, he had a dusky episode and a high rate of respirations. They wanted respirations between 40 and 60 and his were more like 100- 130. This is called Tachypnea and we later learned that it is not unusual for babies delivered by
C section. By not passing through the birth canal, they can often retain fluid in the lungs that is not squeezed out.

Looking at Maddox, he seemed great in comparison with what we experienced at Samuel's birth so this made it harder to have him in the NICU and away from us. I was very emotional about not being able to nurse him over the next day or so and the pain meds only made things even more confusing and emotional for me. There really is no way to express what it means to be seperated in this way from your baby after you carried them in your body for 9 months, all along anticipating the bonding you will experience in those first hours.

Then to make us even more concerned.. I watched as they measured a large port wine stain flat birthmark that Max had across his lower back and I asked the nurses about it.

They said he was scheduled for a spinal ultrasound in the morning and I immediately became concerned as I knew from my past experience at the March of Dimes that they were doing a check for spina bifida. This is because birthmarks and dimples can sometimes be a risk factor for an opening in the spinal vertebra underneath the skin. I knew they were just being overly cautious and that we would have likely known of any such risk as I had constant ultrasounds throughout the pregnancy and had such normal alpha fetal protein test results as well as taking my prenatals etc.. but after all we have experienced with Sam.. I guess I just could not handle the threat of anything like this. Needless to say.. it was hard night of worry and prayer.

Thankfully, the spinal ultrasound came back completely normal and furthermore his breathing began to slow to a safer rate. Labs that had shown some iffy immature white blood counts apparently looked far better and I was able to return to nursing Max. He was later discharged from the NICU in time to go home with me. So we counted this NICU stay just a bleep on the radar and were quite thankful to be blessed by no further concerns. At that time, however, I had no idea what was waiting for us at home..a house of illness!

Earlier in the week, Isabella left school with a bad cough, remaining pretty ill all week with cold symptoms. This was our first brush with illness in our home this season...which is really quite a miracle!

Maddox and I had arrived home on Friday to the house of gloom and doom..the dreaded RSV certainly had landed and Sammy was having back and forth issues as well. We were blessed to have my mom caring for Isabella and working diligently to keep the illness away from Sam. But even with his nurses keeping him apart off in his own room, he soon showed respiratory symptoms by Thursday of that week. The Dr saw them both but could not see the writing on the wall for Sammy until it was too late. They were on z pacs and Bella was doing breathing treatments but Sammy took a major downturn. By Friday night he was in full respiratory distress and could not keep his O2 saturation levels up even while on oxygen. The nurses were very concerned. We were up all night helping them and trying to decide what to do. He was miserable and obviously fading fast.

By the following morning, Sam had seen the pediatrician and Jason was rushing him over for an admission to Sacred Heart Children's hospital. I am thankful that one of nurses rushed over to help me pack his medications, protocol letters and equipment. It was not how I envisioned recovering from surgery or celebrating Max home, but I knew God would get us through it. We had the prayers and support of our church and community and for that there will never be enough ways to show our thanks. I felt like there had to be a break on the stress somewhere. Relief arrived when Tuesday Sammy showed signs of recovery and began breathing easy enough for his discharge home. This was a real saving grace as Jason had already missed too many days of work.. there is no vacation for the self-employed! I am not sure what I would have done during this time without the care of my mom, who rushed in to provide support with the girls.

With Grandpa Papi in the hospital..

Yay! Daddy and Sammy are finally home..

Once home, Sammy tried to take steps to walk over to me and promptly hit the floor flat on his face. He then did it once more hours later cutting up his lip which was followed by a low heart rate/ Brady episode. As in the past, we could see some regression from the illness and that we needed to help him ease back into doing regular activities. It took nearly two weeks before he was fully trucking around the house again and eating with a stronger swallow. He has slept most hours of each day and has required oxygen blow-by near his face to counteract saturation problems during sleep. Overall, however, Sammy is doing great and we are thankful that the illness did not take a greater toll on his overall health.

Sam getting back to walking in therapy..

After all this craziness..it was inevitable that Maddox would contract the same cold illness so I was not shocked when he began getting congested during week 2 of life at home. The Dr informed us the virus was all over town and unavoidable even with washing hands and wearing masks. Max proved to have a very robust cough that has been rather effective in overtaking the bronciolitus that the pediatrician had confirmed at our first followup appt. He had a day or two of bad junkiness in his chest and slower feedings, but still managed to make it back to his original birth weight by the 2 week followup appt. He does continue to have these fast breathing patterns which we first saw in the NICU but I think he is doing well overcoming the RSV, which apparently can be quite dangerous for all infants.

As we head into Max's 4th week at home, I feel so much more at peace. We are getting back on track and I look at my 4 sweet kiddos and feel utterly and overwhelmingly blessed. We have several friends who have also delivered recently and we are so happy for their families. Babies are such gifts from God!

We are getting into a routine with Max's feeding schedule and his little personality is starting to become more apparent. The kids have enjoyed getting to know their newest little brother and Sammy gently pats his head at each meeting and says, "Baby!." Max just seems content to watch all the activity constantly circulating around him. We are praying for the niece of our Pastor who was full term, now 3 days old, and has had some episodes of apnea. I pray for their family and that answers come swiftly. No parent is ever prepared for unexpected health concerns at birth and in my opinion it is the worst kind of pain to not know how to help your child. God's blessings on this baby and all new babies.