Neurology & Endocrine Follow Ups

We had been holding off on an update to get results from a few things, but it appears they are taking longer than expected to complete. So we will update on what we have from the follow-up visits last week. We conveyed our observations and concerns over the possible side-effects Elias has been exhibiting from the Keppra, his anti-seizure medication. Neurology agreed that these were likely linked and have added vitamin B-6 twice a day to his medication regiment. There has been some success with counter-balancing the Keppra with this vitamin. It is something we have to crush to be able to give via G-tube, so there is a small process to prepare it. So far it hasn't been too bad and our biggest worry was clogging, but it seems we are crushing it fine enough. We also changed the emergency medication and protocol for if he actually has a seizure again. We will be giving Midazolam. After we draw it up from the vial we will remove the needle and actually push the medicine into the buckle of his mouth - pocket between the gum and cheek. This will allow for quicker absorption into the blood stream. We are also going to give this the next time Elias spontaneously stops breathing to test if the clusters continue or we isolate the behavior. This will help to figure out is that issue is seizure related as pulmonary suspects. The biggest surprise from the appointment was to hear that his neurologist still feels strongly that Elias had meningitis despite the very well reasoned logic from Infectious Disease (ID). To recap, ID felt that Elias recovery time alone was too brief, there were no signs or symptoms prior to the seizure, and a seizure is not the initial presentation of meningitis. Furthermore looking more in depth at his blood work, there were not any of the other tells you typically see. Impressively his Neurologist brought up the point that Elias is far from normal when it comes to lab work, symptoms, and recovery. This makes for a very strong argument against ID's thoughts. Flashback to July 2008 when the tracheostomy and G-tube was placed. Elias was just a bit off, and it was a Mother's intuition nothing physical that made Katharine press the doctors to run tests the night before the surgery. His blood work showed no signals and his physical exams were fine as well. We found out that Elias had full blown pneumonia that went undetected because there were not any "typical symptoms." Elias had to battle for his life after crashing on the operating table. So what does all that mean....well medically, nothing. It means we don't truly have an answer and must proceed as seizures are a new problem that will strike randomly. It also reminds us and deepens the vigilance we use becoming concerned at the smallest of changes. In you or I it would present much stronger, in Elias it may appear business as usual. All-in-All we will simply keep doing what we have been doing, successfully. Before this we had kept Elias out of the hospital for an illness since April 2010, when he had a MRSA blood infection. That includes 2 winter seasons unhospitalized (ie; cold/flu/rsv hell) for a trached child that is amazing, as the doctors reminded us.
We are still awaiting the endocrinology results in reference to the possible adrenal insufficiency or dissolved regulatory response. This may mean another medication daily, a steroid instead of the stress dosing we are set-up with now. We are also going to be checking his blood sugars a few times a week for 4-6 weeks just to make sure things are working as they should. They do not feel this is an issue, but this will make them more comfortable in evaluating the big picture from an endocrine stand point.

We are beginning to see Elias level out a bit and regain some of his energy that has been difficult to watch. He has had a good week overall with the amount of time he will play and how he has felt. Part of it is adjusting and let's face it Elias was very sick so recovery will take time. The doctors did a wonderful job disguising the seriousness of things while he was admitted - giving it due attention mind you, but keeping our stress down and concern level low. However, now that he has improved they have not been shy telling us how very concerned they were and sick he was. Dr. Mehta, his bone marrow/FA specialist commented to us in clinic she has no idea how we handle it, but she is glad that we manage somehow for Elias sake. That is just it, it is for Elias' sake. She also reinforced how challenging and unique he is medically on all fronts. We appreciated the compliment and vote of confidence, which several of his doctors have made over the years. We told her that it helps to have great doctors caring for him and talented enough to handle his challenges and be pragmatic with his plans. They have provided us with the tools and we have embraced them. Without them, along with supportive family and friends we would not be half as strong as we are. It serves as yet another reassurance that we made the best decision coming here for his care and reminds us that he has some of the top doctors in the world - for their fields - working with him. We couldn't ask for more. Thank you for the continued prayers, support, and uplifting motivation. As I posted on Facebook this morning, "Tomorrow will be a better day because we are privileged to be granted the opportunity."

Day 5 - Closer to Discharge

Elias continues to improve each day. He is behaving like himself and reacting appropriately to actions which is a reassurance that any brain damage is nonexistent or very limited. We have been very happy to see his rapid progress and the doctors have been surprised at the rate he has recovered. While the spinal fluid is still being tested preliminary results suggest that it is a viral meningitis that caused the seizure. They still have him on a broad spectrum of strong antibiotics to ensure they are covering every possible cause. It is likely we will never know the type and source of this. Elias has a MRI scheduled in the morning. We do not expect there to be anything significant to report and we are hoping with Elias improvement they will let us bring him home after the MRI clears him.

Showing us he is better!

One of the other interests of note brought about by this is a possible adrenal insufficiency. This is when the body does not produce adequate amounts of steroid hormones (chemicals produced by the body that regulate organ function), primarily cortisol. It has us thinking that possibly this could be contributing to Elias' breathing episodes as well. We are going to discuss this with his Pulmonologist. It is a long shot, but hey it is a lead right? Back to the insufficiency....They are doing a test in the morning that should clarify if this is indeed an issue. Elias' thyroid numbers have been creating some concern the past month or so and we had planned another check this week before all this occurred. It is possible they were pointing to this. Not that this was the cause, but for those who know Elias and followed us you know that he has a way of showing the doctors answers to questions they didn't even know they were looking for. It has been this way all his life. He is just so nonclinical in so many ways that events like these usually uncover underlying issues. Due to this Elias will likely be taking medication to help manage this. We are meeting with someone from endocrinology in the next day or so for training on stress dosing (recognizing the need for and evaluating). We are building quite the resume for training medical experiences. Elias will also be adding anti-seizure medication as well to his regiment even though they feel the seizure was caused by the meningitis and is not a source of a new issue. We will also be given some training on effectively managing him, but as they pointed out, we did a wonderful job of doing what we did despite not really knowing what to do in this case. It saved his life and maintained his quality of life.

Elias has been his usual come back kid self. We are so grateful for his will to fight and survive. This has been an experience that will impact us forever - in a positive way. Hopefully, it will clear up some other outstanding issues and we can move forward. We are diligently working towards identifying, resolving and stabilizing all of Elias medical complexities and challenges not only to improve these, but give him the best chance to survive his bone marrow transplant when that day finally arrives. His will and fight is already evident, but will his body be up to the test? We believe we are taking a huge step to help that and will continue to do so. Elias has some incredible doctors and they are going to provide him the best chances. They are looking out for him and that gives us confidence. Our faith has been tested time and time again, but we hold strong to it and look towards it to provide us with the solid foundation we need to be the best parents we can for this amazing little boy. Your support, thoughts and prayers have been nothing short of incredible these past few days especially. But we recognize and appreciate everyone of you everyday. It is not always easy, but somehow we always manage to find a way. You provide us the courage and confidence we need to face each day and tackle each new challenge for his sake. As they say..."it takes a village..." Thank you for being one of those villagers.

A New Problem or One Solved?

We are trained to handle many emergency scenarios with Elias. We have had to save his life repeatedly over the past 2 years as he stopped breathing, but nothing had prepared us for today. We experienced a fear and sense of helplessness that words just can not adequately express. This was the scene outside our door at 1:18pm yesterday afternoon. One we never thought we would need and hope to never again. Elias had to be rushed to the hospital via ambulance.
As we updated earlier this week he has been having another round of breathing issues. Overnight we had to put him on oxygen and the vent was breathing for him. Through the morning he had periodic spells and we dealt with them as we always do. Shortly before 1pm he stopped breathing again. Katharine was bagging him and we had oxygen on. After nearly 2 min he still wasn't breathing on his own. We decided to move him back upstairs to place him back on the ventilator. We placed him in the bed and had the oxygen and ventilator in place when we noticed that Elias was looking off with his eyes to the right. He was unresponsive to touch or hands in front of eyes. Then he began to shake and shiver all over his body, eyes still not moving. The shivering became worse and we knew what was happen, even though it had never happened before. Elias was have a full blown seizure. We called 911 and requested a squad. Elias worsened as we waited for the ambulance to arrive. Our concern grew into a bit of panic. We were watching him slip away. We were frightened beyond belief. Once Elias was moved downstairs and loaded on the ambulance they raced him to Children's Hospital. As the driver told me, don't worry we will get him there loud and fast. Katharine rode with them while I finished gathering his equipment.

Once in the ED he was taken to the trauma bay. He was continuing to seize and had not stopped. It is estimated that the seizure lasted roughly 45 minutes. While the doctors can not be certain about possible brain damage, the trach and our ability to keep him oxygenated may have saved him, not only his life, but the brain damage or at least minimize it. Sadly it is too early to tell for certain. Only time will tell us what if any is there. One positive is his reactions when they touch and pull on key points of his body. Within 50 minutes he was having a brain CT and we were rolling into the Intensive Care Unit. Initially his glucose levels were 300+ in the ambulance and had risen to over 500 in the ED. The glucose in the PICU was trending down to about 300. Seizures will cause glucose to rise, but not like that. So endocrinology moved up the ranks of priority. It was so nice to see Dr Rose walk in who is one of the most active researchers and knowledgeable endocrinologist when it comes to Fanconi Anemia. She assured us that given known FA patients glucose and insulin regulatory issues it was indeed possible that the 500 reading was all caused by the seizure. They would follow up on this, but in a day or two when he was less medicated and more stable. It is another example of the unique and pragmatic tendencies little known and understood issues within FA create problems for medical professionals who do not know about this rare disease. At least for now we can focus and worry less about that problem. The PICU team has been great and immediately sprang into action with contacting and consulting many of Elias primary specialist. Several of which personally came into evaluate him. Often times the politics and egos of the hospital interfere with that, but given the circumstances and the mystery it has been extremely reassuring thus far that all the folks that know Elias the best are on this. It makes us feel so much more confident about his chances. Right now Elias has every culture and blood test known to medicine that could remotely apply being done, including cultures. A lumbar puncture was done to test for meningitis which he is already receiving proactive antibiotics to treat should that come up positive. He is also receiving a wide spectrum of antibiotics to treat any potential infection that may have gone undetected and cause this issue. They also have an EEG hooked up that will run for sometimes. He is of course on the ventilator and it has been back and forth on the vent breathing totally for him. His settings are higher than normal and he is struggling a bit with CO2 issues. For the lumbar puncture they had to sedate him so as of now at least Elias is out. There still is a lot of uncertainty mainly is this a new problem and concern to add to the list or is this the result of the breathing issues that have evaded us? Too early to tell and Elias certainly has an uphill battle the next 24 hours and not out of the woods yet.

We are exhausted and emotionally drained. Thank you to everyone on facebook for your absolutely amazing outpouring of support and prayers. It is so amazing how many peoples lives Elias has touched. We are humbled and grateful to have such amazing family and friends. You all are the greatest and you all have helped us stay strong through this. We may put up a good front, but believe us the tears of fear have been flowing today more than ever. We will keep you updated as we can. We have no idea what is going to happen tomorrow, but we will continue to pray that Elias will pull through yet another tough battle.

Hello?

This week has been horrible. It began on Sunday with the sudden passing of an FA patient from complications of the 2 bone marrow transplants he had a few years ago.  Every time another FA patient earns their wings are just somber reminders of how devastating and deadly this disease is. Our prayers are with the Barbier Family as they lay Justin to rest this week. Justin's mom has been a huge support to us and through Elias' journey. Our hearts are very heavy for her loss.

On to a very serious issue and potentially disastrous problem. We received a letter in the mail yesterday that was to notify us that Elias medicaid was being discontinued due to insufficient information provided during annual re-certification. We are not even sure what was supposedly omitted? We have left two voice mails requesting a hearing on this matter, but have yet to receive a response. Tomorrow is the deadline to request a hearing with continued benefits until a final decision is reached. The letter was dated July 11, 2011 yet we received it on July 18, 2011. It should not have taken 7 days for us to get this first off, but the hearing deadline is spelled out in that letter as 10 days from the date of the letter. I will call again in the morning hoping to get a call back or better yet an actual person. If they do not call back tomorrow I am not sure we would be able to get the hearing. This is absolutely critical that Elias maintain his medicaid. Elias has capped out long ago of our primary insurance provider. Without it we are finished and Elias needed treatments are in jeopardy, not that we are not already in financial turmoil from all variables and medical expenses anyway over the past 3 years. A lapse in coverage even briefly could create issues for supplies, equipment and services. Very stressful and worrisome this is, until we get it resolved.

I can not remember if we had mentioned this in a previous update so forgive the repeat if so. It appears the school system has misplaced or temporarily not filed Elias paperwork for enrollment. Without it he can not begin services. This was turned into the guidance counselor at the school prior to the IEP meeting which was April 28th. While it is irritating that the school system office does not have this, our primary concern is the fact that turned in included copies of Social Security Card and Birth Certificate required to register. After a week to allow them time to investigate and locate the file, this week they are dodging my calls and not returning voice mails.

Which leads me to another point. We contacted GI 2 weeks ago about concerns we had. It took them a week to return our call and then we were told his GI doctor was on vacation. If they had been more timely in returning the call that wouldn't have been an issue. So medication was ordered for a week to get by until the doc returned. We were told they would call yesterday to check in and get a report to pass on when the doctor returned today. You guessed it no call. I even asked them if I needed to call in when they originally set the expected phone call up. They said oh no we are making note of it now that won't be necessary. Apparently it was. Today has come and gone without a call as well. So tomorrow first thing I get to call and find out why and what the plan to treat Elias is. His speech therapist was also a no call no show today and while I have left a message with her, again still no call back. Bottom line is that it is becoming increasingly more common for his providers to just not call. Why is that? Are we that busy and if so why would you set an expectation of calling someone and just ignoring it. That is just plain rude and unprofessional, especially when it has been more than one day overdue. I know this is a big rant but quite frankly I am disgusted by the manner in which people provide service and hap-hazardously return phone calls. Sorry for the rant I will end it there.

If all that wasn't insane and hair pulling enough Elias felt like we needed a little more stress. He has begun yet another cluster of breathing episodes. While the number of times he has stopped breathing has been lower than normal (thank God) with everything else, particularly the medicaid problem, everything is a bit more charged. Poor Elias. We hate seeing him like this. It just sucks the life out of him. Hopefully the week will improve, but for now we just watch over Elias and try to go on with business as usual.

It is becoming more and more challenging to keep this blog updated. It seems as of late the schedule has been so hectic along with "stuff" (like Elias not breathing for example) just hitting us with the old 1-2! The fall isn't shaping up to be any easier, in fact it will be more nuts with the addition a preschool services here at home. We have been exploring other possibilities that might be easier, for instant - audio updates. If anyone has any suggestions we are open.

Since the last update Elias has had several more clusters of breathing episodes. Usually on the weekends it seems. It is as if he knows! Last weekend they began Friday at 4:30 and continued through Sunday Morning. So he has now had periods where he stops breathing 4 out of the past 6 weeks. This is the most frequent we have seen them in quite a while. All we can do is manage and try to keep the stress to a minimum during those times.

Elias has had a few sessions with his new speech therapist. It is a growing process and will take some time for them to learn to work with each other, but there has been some positive things already. We are working to expand his vocabulary with the picture exchange. At times the little stinker will turn it into a game of keep away as he smirks at you. He is a joker so watch out. In his listening therapy yesterday we witnessed something very interesting. She had a plane and a train. She showed and played with each individually with him. With airplane she would make an 'ah' sound before showing the plane. Then in the same manner did a Choo Choo sound with the train. She then presented both but rather than ask which one he wanted she made the ah sound, in hopes he would choose the airplane, and he did. We were not convinced fully that this was not merely a coincidence, so she did it again. Same result. So to try and substantiate what we saw she presented the two again, but this time made the Choo Choo sound. Elias just looked at her and did not do anything. She made the sounds again. After a few times he finally reached begrudgingly and touched the train, as if to say okay okay there is the train can we move on. So we are going to try that experiment over the next few weeks to prove it was not an accident. That he is beginning, ever so slowly to recognize even more sounds and correlated them to the appropriate item. If he continues to prove this theory correct it would be a HUGE step. So here is to praying it was real and effective.

Another issue keeping us from the blog updates is constantly having to chase down and follow-up on things that we are told have been done or should be done by various providers. One example, we are having issues once again with Elias' prevacid an the insurance needing prior authorization. The problem hasn't been so much getting the authorization in as it has been getting to the right person. This is due to the incompetence of the pharmacy providing us with the wrong information on what was needed, not once, not twice, but three times in the past 10 days. On top of that they have contradicted themselves in the information proving that at times they were just saying whatever to placate us and move us on for the moment. Ultimately this has cost them our business and frustrated the doctors office handling the PA, as they caused them 3 times as much work, most of which was not necessary. The part that truly angered us was not only the deception, but the lack of concern or willingness to ensure the information was communicated in a timely manner, as this medicine for Elias is potentially life or death. Why? One of the theories behind the breathing episodes is that reflux temporarily paralyzes the vocal chords, which creates a reaction causing him to stop breathing. He did have a cluster of episodes in the 10 days he has been without. Hard to say for sure, but if that is a physicians theory on the table for the unexplained along with very few others, you tend to take them serious, despite what you believe. Fortunately this morning we have filled the Prevacid and he will be receiving this once again.

This past weekend Katharine's mom and brother came to town for a visit. They will be with us for a week. Katharine's Aunt, Grandmother, and cousin flew up on Friday to surprise everyone. They were here until Sunday. It was a fun time surrounded by family. Elias was having such a blast. He barely stopped the whole weekend. He did have a blip on Saturday night, and was not doing so well, but bounced back by morning. Just reminding us that he is still medically complex is all. He showed off and demonstrated all the wonderful skills he is learning. The bubbles with the picture exchange for communication was by far the crowd favorite. There are some photos that we will work on putting up soon. We will update when we can and who knows maybe we will figure out an easier way. As we said we are open to suggestions if anyone has any.

other various sundries

Once again Elias’ blog has been unintentionally neglected. Tons of stuff going on with him right now, both medically and developmentally. We did get our IEP written for Elias to begin pre-school at home this fall. The doctors have said that it will be several years before they will likely seriously consider allowing him in a classroom, but there is the reality it may never happen at all. At home he will have physical, occupational, and speech therapies a few times a month through the schools. He will have an assistive technology person, who coincidently also happens to be a hearing impaired teacher and a pre-school teacher that will come in once a week. Between all those appointments, plus his other PT, OT, & SLP in addition to the therapies at Children’s that alone is going to keep us booked. Throw in the medical and we are in for a challenge. This IEP was rather straight forward and simple to put together. We are removing the cognitive and social elements from his education plan at this time as it is not appropriate for him at the moment. While we were initially surprised with how much service we were able to get, we have been warned to be cautiously optimistic that will remain that way. It has been strongly recommended that in the coming years we get a lawyer that specializes in this area to help with the process. There are advocates out there for parents, but a few trusted sources have said with Elias’ needs we need that extra notch to help oversight and push for his educational needs to be met. As with everything, despite some of the horrible tales, we are taking in everything with an open mind and filtering the lessons learned by us and others. Overall though it appears we are in a good school system for him and we feel positive about beginning this fall.

Medically, Elias is Elias. the good come with the bad and that is all you can hope for. He has had several episodes over the past 3 weeks where he has stopped breathing. It has been since March since we experienced this. He has been keeping us on our toes that is for sure. We are scrambling and cramming in appointments as Elias is having a list of surgeries next week. Why are we scrambling? because GI wanted to (back in March) move his already scheduled surgery up (originally set for June) sooner because of some issues they want to explore inside. It seems as though someone dropped the ball and if it had not been for us following up constantly, we’d still be waiting for a date. So now we are having the surgery about the same times as we would have had we let the airway team dictate. It has been a cluster and frustrating the lack of a plan GI has had with everything. But that is what we are use to and have learned that we much constantly follow up with almost all of his providers for things. It is amazing how much time that takes, which has been a major contributor to the blog being neglected. This round of surgeries has the typical airway bronchoscopies to assess his airway, an GI scope to help with some issues we are having in that area. He will have a cystogram and urethral dilation by urology. They will also be assessing his hypospadias and genitalia region for some potential corrective surgery plans. Finally there will be his annual bone marrow biopsy which checks for early signs of cancer and failure of the marrow among other useful info. There will also be a skin biopsy for a FA research project in New York. It will take a few weeks to hear back on the Bone Marrow results. We do not expect any surprises, but you can never be too cautious with FA. In the near future Elias will also have another MRI as well. We believe that about covers the surface of what is going on the past several unreported weeks. We will certainly try to do better.

Played Nice

Elias kept things even keel today. There were no episodes, so this round appears to be behind us. We will say that compared to what we are use to seeing this round had some pretty frightening moments. One of the episodes had his oxygen sats dropping to 10%!! Yikes!!! One of our friends commented on the verbage "used to" who is used to their child not breathing? Our response...Good Point!

Deep Breaths

Elias has improved over the course of this evening. We were able to take him off the ventilator and oxygen, but had to shortly after put him back. Then he improved again and has stayed fairly happy and "typical" if that even exists in his world. He even was able to skype with grandma for a few minutes. While we hope the end has arrived for this round, as we learned earlier he will turn on you in a flash. So here is to a night full of boring breaths and a weekend of nothing!

We will keep you posted of any changes. Thanks to all for the thoughts and prayers for our little man. We leave you with a mental image...Elias Tap Dancing??

Breathing or the lack there of

Just a quick update. Today has not been a good day. It is a harsh reminder how things can be great one moment and the next moment everything is crazy. Elias began having his episodes again where he just stops breathing. The first one this morning was the worst we have ever seen in terms of how blue he was. We hoped it would be one of those day where it happens once or twice and we move on. Elias has other plans! He has already had several episodes to this point today and we had to make the decision to put him on the ventilator this morning (typically only uses it at night). We decided with his Pulmonary doctor after the last round that this was a good management option. Unfortunately, even with an increase in the breaths per minute Elias is having difficulty maintaining his oxygen saturation levels. We have had to place him on oxygen in addition to the ventilator. We have tried to wean through the afternoon, but have been unsuccessful. His little body just isn’t handling things without it right now. He has been letting the vent do most of the breathing for him. He may just be so zapped of energy that he is allowing the machine to do all the work. We will keep you posted as things change. Please keep Elias in your prayers that this passes quickly.

Clinic Today

We just returned from Elias Pulmonary add-on visit. Dr Wood was pleased with what he saw today and said that he felt better having laid eyes on him. He really just wanted to be certain that Elias had not also contracted on of these nasty respiratory infections going around. That is not the case, thankfully. When Dr Woods nurse walked in Elias was walking around the room. She was so happy to see that. I think Elias gave us all a much bigger scare this time around. As always it seems he has bounced back as quick as it came on leaving no evidence. Regardless, we will take it. We discussed what we did here at home and how we should proceed in the future. No real changes despite everyone’s desire to figure this out. He did give us very high praise for making the decision Sunday to increase his ventilator support. That is what we will continue to do going forward. As he said, “Elias drives the bus, we are only passengers. We go where he goes.” We are not really doing much to chase down a reason – it is unfortunately a fact of life. You could probably draw a similarity for this as sugar lows are a part of diabetes. Despite your efforts to control, at times the body takes over and dictates. I suppose the intense difference in intervention makes that a stretch, but you get the idea. Thank you all for the support the past few days and prayers. These are always appreciated. We are pretty exhausted, so we are grateful this round is behind us. Until the next time we can all breathe a little easier – Pun Intended!

Wednesday’s MRI Scan Results

While we had planned on an update today anyway, it was suppose to be all positive updates. Apparently Elias found out, must have overheard while wearing his BAHA (kidding of course). Last night shortly before midnight Elias decided to stop breathing again. He has been due for a series of episodes as we have been on a pattern of roughly every 4-6 weeks for quite awhile. He made it through the night with his oxygen saturations staying at safe levels, but lower than normal –even for these episodes but he let the ventilator do 98% of his breathing for him. No oxygen had to be used so that is another positive. We have already contacted pulmonary this morning to see if they want to do anything. Waiting on a return phone call. The stress of it, while high & intense is manageable –from experience maybe, but the frustration level increases every time. We just wish we could figure this out so we can prevent them.

Elias’ New Years Resolution – To Breathe

Elias stopped breathing again today – 3 times and has already  demonstrated he plans to carry on with these antics this morning. We have already had to bag him back once. Overnight we increased the rate on his vent to 18bpm because he was unable to hold his O2 Sats above 90. Thankfully, no oxygen was needed and he slept through the night without an incident. He did however, let the ventilator do most of the breathing for him.

We have noticed a new behavior with these episodes so far – he is sticking his hand in his mouth like he is trying to gag himself. Makes us feel more and more like these obstructive in nature and not central as everyone is theorizing. We are going to see if he continues this with future episodes – albeit nice if he never had another, we are realistic enough not to allow that naive optimism get the better of us. Oddly enough, the hand gagging is a behavior we have seen in conjunction with his mysterious feeding issue. Another of Elias’ unresolved & unexplainable phenomenon. This opens a Pandora’s box to the possibility of considering a connection between the two in some fashion. Our inclination to consider this derives from a common theory doctors have offered in regards to each of these events as separate issues – Reflux!

Getting Back On Track

Things here have been busy. There have been a lot of follow-up and make up appointments to cram in before Christmas. The platelet antibody test came back positive, which is bad. It does provide an explanation for the dramatic drop. The good news is the counts are trending up despite the antibodies and IVIG is the first treatment, which Elias is already getting. The FA team is just going to continue to monitor his counts and increase his IVIG if needed.

Elias has improved a great deal, mostly in the last few days. He is beginning to get his energy back and becoming more active. The smiles, fishy kisses, and raspberries are back stronger than ever. He is also beginning to get back to walking. Wednesday night he was so determined and it was like watching him take his first steps all over again. He still is not walking as easily as he was before surgery, but her is able to walk across the room and spin around as well. When he stops it is more from fatigue than not being able to keep his balance and footing. That frustrates him a little, but he rests and then pulls himself up on the nearest stable object and goes again. We are glad we have not had to prompt him to do this and he is working this out on his own and at his pace. It shows his determination and will to conquer.

We have another busy week ahead with several appointments in the mix. It has been exhausting to be quite honest. We are still trying to recover just from being in the hospital so long and now this crazy intense schedule we have going on. Last week Elias also tested positive yet again for his chronic C-diff. Back on the vancomycin he goes. They are going to try a taper off it this time to see if that helps keep it at bay longer. We also started Diflucan to see if we can control and eliminate the feeding issues we have been battling where he sweats and retches with occasional vomiting. His GI doctor has a theory that with all the varied antibiotics Elias has had to be on almost constantly along with the colonization of different things in his body, that might be the cause. The Diflucan will treat certain types of those most commonly known to create issues like this. If after some time we are not seeing improvement they will likely scope Elias’ esophagus and stomach. Let us hope the Diflucan is the answer. We also had a day with about five apnea spells. Fortunately, they were relatively easy to manage and bag him back without having to go on oxygen. We have learned to just accept these as a stress in our life that isn’t going away anytime soon. All in all we are just staying busy, trying to get rest when we can, and generally trying to keep our head above water. We hope everyone’s holiday season is going well.

Apnea, MRI Results, & A New Concern

It has been a long rough day. Elias has stopped breathing 11 times in the past 26 hours. 10 of those have been since 2:30 this afternoon. He has needed to have supplemental oxygen to recover each time. Ironically, the neurologist called this afternoon to discuss the results of the MRI last week. There is no evidence of seizure activity or issues anatomically that would suggest a Central Apnea. That does not mean this still is not the case though. Of course, a cut and dry answer for Elias!? Never!!!!!!! The neurologist did ask a series of question about today's episodes to get some other information. She is pretty confident that this is not seizure related based on his interaction response during each episode. We also ironically just saw Pulmonary on Tuesday which I will update on that visit this weekend. Everyone agrees that this is not going to be an easy mystery and that handling this the way we have is still the best. It is just stressful, worrisome, and exhausting.
The only other finding to the MRI is a mass in the white matter of the left frontal lobe. While this type of thing could show up for a number of reasons, most of which are inconsequential, there is a need to treat it with respect. There is a small chance it could be a tumor of some sort. We will repeat the MRI in about 4 months. Even if this shows up again that doesn't always mean anything, but might prompt further testing. Even though she was downplaying this finding, it is a little unsettling. Just one more thing to be concerned about. It just never ends. We will keep everyone posted on this, but it will be a few months before we know anything more. Tonight we have other, more pressing concerns with Elias breathing.
At the time of this posting Elias is sleeping and on the ventilator. It is doing all the work though. We think he is so exhausted his body is allowing the vent to breathe for him. We had to raise the rate to 15 bpm to keep his Sats up but hopefully he will relax, breathe on his own when he can and be better tomorrow. Thank you all for you concerns, thoughts & prayers, especially for the newest issue.
By the way if you haven't seen it already, check out the Videos in the side bar to the right. There is a short new video of Elias walking and kinda babbling! It's adorable!
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Related articles

• MRI Scan (mycarouselofprogress.blogspot.com)
• Another Round of Apnea Episodes (mycarouselofprogress.blogspot.com)

MRI Scan

Elias had an MRI of the brain and cervical spine yesterday. Neurology ordered the scan to repeat a seizure protocol and rule out Chiari malformation. This was another attempt to discover the cause of Elias apnea episodes. The doctors want to compare it to the last MRI which was done in September of 2009 two days after the first appearance of Elias episodes. Elias did well through the scan and recovered quickly from the anesthesia. There was, as always, concerns that Elias would have to be admitted due to the risk associated with his sedation. His risk is much greater than most because of his airway issues. We have not received a report on the reading of this MRI yet and not certain when we will. If we have not heard anything by Monday we will call. No news is probably good news in this case though.

We had hoped to get the Functional-MRI as well yesterday, but it was not in the orders. We are not sure if it was because of scheduling or the neurologist that the red light was put on for this. The FMRI is the research scan we mentioned in Elias’ last ENT update. It was disappointing, but as we said before it was all for curiosity and not diagnostic. Another opportunity may present itself in the future.

Another Round of Apnea Episodes

After nearly two months without occurring Elias began another series of Apnea episodes. Thankfully as the posting of this update we seem to have reached the end of this round. He had 10 - 12 episodes this time around with nine of the ten from 6am – 6pm Friday. It was, as they typically are, very stressful with some of the episodes lasting several minutes before we could get Elias breathing on his own again. Half the time he would recover after a few minutes and start playing as if nothing happened. Other times he would just fall asleep because it had wiped him out. After each time we would just hover over him and anticipate the next episode to happen and we usually did not have to wait long. The first half of the day there was only one or two time Elias would need oxygen. Even then we had him weaned off within 10-15 minutes. However, towards the end of the day he was needing oxygen longer. He was actually on 1/4 to 1/8th of a liter from about 530pm until roughly 930pm just to give him some extra help and allow his body to not have to work so hard.