The Day After

Elias had another trip to the OR yesterday. This was all the standard follow-up procedures he has from time to time. He did very well during the 6 procedures that lasted around 2 1/2 hours. He woke up from anesthesia in typical fashion with a baseline of lower than normal O2 sats, but he quickly pulled them up by the end of the night. All the doctors were please with what they saw, at least those that could report.

Pulmonary & ENT are happy with Elias airway, that is in Elias standards. There is evidence of slight improvement in the compression, but nothing to get overly excited about. However, with Elias and his airway the smallest of victories is welcomed. Growth still is going to be his best ally towards achieving much improvement. That is something he has struggled with so that will not likely be helping anytime soon. Overall they said given Elias history they would accept this as the most positive overall bronchoscopy & ML&B they have performed. GI scoped him all the way to the small intestines and found no evidence of any fungal infections (which was a concern with all the antibiotics he has been on) or any thing out of the ordinary - again for Elias standards. The TEF repair site continues to look great and the stricture was not in need of dilation. His Nissen looks good as does his G-tube placement. The small intestines were a little tight, but that was expected with the failed colostomy take down and herniation of his bowel. We did not get to speak with Urology so we have nothing to report as of yet and it will be a week or two before we get results from his bone marrow biopsy & aspirate. He has been doing well today, a little tired from lack of nutrition yesterday, but overall his typical playful and happy self. We will update soon when we begin to have results come in from the testing.

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.

Thanksgiving Blessings

Last night we were able to bring Elias home from the hospital. We were quite shocked to say the least, but were not going to argue either. We believe they realized there wasn’t much else they were going to be able to do there that couldn’t be done by us at home. At this point Elias just needs time to recover from the sheer amount of stress these two surgeries have inflicted. He is still in quite a bit of pain and this in tandem with his platelet and hemoglobin losses really has him very tired and weak. The photos are from last night after we got him settled here at home. He looks weak and you can see the weight he has lost over the past three weeks. Weight he could not afford to lose. He lost nearly a pound and a half! He has shown a few signs of life and improvement. He certainly recognizes he is at home and we hope that translates into quicker recovery. We did get positive news that his platelets had rebounded some, up to 102 from 88! That is a relief to be heading in the right direction after such concern being expressed by his bone marrow doctors. He is still on pain medicine and we need to work on getting him back to his typical feeding schedule. Right now we are doing slow continuous feeds around the clock for a few days before we attempt to modify that. All in all we are just happy to be under one roof together. It didn’t really matter where, but it is much sweeter being here at home. On this Thanksgiving Day we are thankful for many blessings. We hope that you and yours are able to appreciate your blessings. We ask that you remember other members of our FA Family. There are many who are in the hospital this day for Transplant, cancer treatment, as well as, struggles with issues related to Fanconi Anemia. Especially keep the Lytles in your prayers as their little boy Joshua is fighting for his life today. The doctors are struggling to even keep him comfortable. Let there be a miracle, please! There are also a few families that have recently loss their children to this terrible disease. We are thinking of those families as well. Thank you for all of the continued thoughts and prayers. We hope that each of you have a wonderful Thanksgiving.

Over the Weekend

We know everyone has been anxious to know how Elias has been doing. He is improving, but very slowly. He was moved out of ICU yesterday, so that is a good sign. He is still in a great deal of pain and he has needed pain meds and valium every 2 hours. He will not cuddle with his teddy or blankets and does not want to be touched in any fashion, even by us. He pushes us away, literally. It is very sad to see. Thankfully the meds are keeping him asleep most of the time so that he can rest somewhat comfortably and heal. Elias hemoglobin has also been dropping since his emergency surgery on Friday night. It was 6.9 this morning, so a blood transfusion was ordered. The cultures from Friday all came back negative, which is positive. There really isn’t an explanation for the spiking and falling fevers. The surgeons say that nothing was infected inside so those types of fevers are not something they would expect. He has not spiked since yesterday morning when he hit 102 and has gone down slowly since. We have also needed to keep him on oxygen since the surgery, not too much thankfully, only 28% to help his tired healing body. He also has taken a huge hit to his platelet count. Before surgery it was 200, today it is 90. Elias FA doctor came in to see him and she is concerned by that drop in conjunction with his hemoglobin drop. It is something she is going to be watching very closely. They do not appear to want to take action unless he continues to drop down to 50. We imagine a platelet transfusion might be in order at that point.

As for when the heck we might be able to get him out of here, we have not been able to get anyone to even discuss the next step, which after stabilizing should be feeding. No idea on when that will start or how long it will take once we begin. This has certainly been a much rougher road than we could have ever expected. We are holding up as well as can be expected. There is obviously some disappointment, but as we said before the important thing is that Elias is safe. We appreciate everyone’s thoughts, prayers and support.

Emergency Surgery

Elias is now in ICU recovering from his emergency surgery. To follow up on the previous update, he was taken for the CT scan at 9 pm. Once he was done he was rushed into the operating room for emergency surgery on his bowels. The CT scan confirmed the mass that has been around the incision and stomach was indeed a hernia of the bowels. At some point they slipped up in between the muscle and was protruding and pushing up on his skin. The surgeons came and spoke with us while they prepped the operating room. They had no idea what they were going to find until they cut him open. They told us they had no idea how long it would take and what action they ultimately decide on again until the saw for themselves the inside. The surgery took several hours and it was almost 1 am before they came out to tell us they were completed. Sadly the colostomy had to be put back in place. The anastomosis, which is the area where the two portions of colon were reconnected from the colostomy is simply too narrow to allow anything, including gas to flow normally. The safest option was to put the colostomy back. The bowel had indeed receded and pushed its way up and fortunately it was caught before any perforation or damage occurred. The positive side to this is the surgeon noticed a section further down the bowel that is wider that he can in the future try again to reverse the colostomy. This can be evaluated again as an option after 3-6 months of healing. Honestly, after all that has happened the past 2 weeks now is not even the time to think about that potential, though it was nice to hear the positive feedback.

It has been a very long, emotionally draining and frightening day. This was suppose to be something to improve his quality of life and we suppose it still can be, but for him to have gone through all this to ultimately walk back out with a colostomy in place covers a wide spectrum of feelings and concerns. We are still waiting for the cultures as well, but Elias is on a much broader set of antibiotics until the source of issue is narrowed. It is possible that all this bowel issue caused the high fevers. That would be great if it was. It is still unclear how long Elias will remain in the PICU. We will have a better idea in the morning. It is also unclear how much longer we will be in the hospital in general. At least 2-3 days for post-op antibiotics and the pending culture results. Despite all the frightening moments and serious concern coupled by the fact we went backwards by redoing the colostomy, the bottom line is Elias is safer and on a path to comfort. That is the most important piece that we carry away from this. It was more the way he has suffered the past two weeks that gives us what we know is unfounded guilt, yet we have it anyway. Thank you all so very much for your thoughts and prayers today. It has provided us with much needed strength and focus.

Morale Buster

Well the train derailed today. Dr Levitt was pretty hopeful the pedialyte could start yesterday. Typically an X-ray is done before the decision is made but he was confident that wasn't necessary. Believing that was our first mistake. At 12:30 yesterday afternoon the nurse paged the doctor to find out when we would begin the pedialyte. She was told they wouldn't be, but no one could give us a clear reason why. We began to wonder if there was another communication flow issue because Dr Levitt apparently had no knowledge of stool being passed on Friday. Oops! Then Elias began to run a fever and have high blood pressures. This along with his sleeping so much was concerning. The surgical resident, which is separate from Colorectal, but included as part of the communication hierarchy (got it?) was paged at around 430p. As our facebook friends know they never came...still. The Colorectal fellow (a different one today) came in and he has helped get everyone back in the same page of the same book. He has been very helpful and given fantastic explanations for almost everything. Despite the confident no x-ray we were told on Fri the decision to do one today made sense, I just wanted them to realize they are mixing signals. Unfortunately, it showed that there is not gas in the lower colon yet, which is odd to them even because he is passing stool and yes audibly gas! This tells them that the reconnection area is still very tight and being small not a good idea to start anything in his stomach just yet. From here we are day to day with x-ray to determine if improvement is made. If tomorrow they can not begin clear fluid then TPN & lipids will begin according to them today. So as of now this has become another open ended hospital stay.

On the positive side from Elias, he has successfully sat up again a few times today and put his arms up for us to stand him up. He can do this for just a few minutes, but we are happy his spunk to do has returned. Even if that is all he does today it is so much better than what he has been doing. This did us both A LOT of good to see! That kid is something else, let me tell you. Thanks for the continued prayers and please remember our other FAmilies in your prayers. There are some going through some very difficult challenges and health issues as well! They are all fighters like our Elias! As one parent in our FAmily suggested last week FA stands for FIGHT ALWAYS!
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And So Goes the Weekend

We had foolishly become optimistic that we would make progress this weekend. We were hoping for clear fluids (ie pedialyte) to begin today per the attending Doctor, but somewhere along the line the decision was made not to do so and we are not quite sure why. The resident told the nurse that based on the fellow's assessment they will re-evaluate day to day. For some reason they do not round like other services, at least not with us. Maybe because we have to be on another unit because of Elias ventilator dependency? We saw the attending surgeon, Dr Levitt on Tuesday afternoon, but not again until Friday afternoon. The fellow has been coming in at 6am so there is not much discussion. We do not see anyone else unless we specifically demand it, which is absurd. Especially since the hospital prides itself on Family Centered Care.

This is day 7 of the admission and it has been 147 hours ( as of the time of this post) since Elias has had anything to eat. Our best guess on the reason to withhold the liquids still is there remains some distension in his stomach, but that is a guess. I won't tell you my other considered reason. If you want to know feel free to email me and I'll be happy to share. What little calories Elias is getting through the IV fluid is going towards the healing process. It is insane how much the child has laid still and slept, even if fitfully. He has zero energy.

Elias did show more life for a little bit this morning but it was sadly short lived. There was a tiny smile and a few fishy kisses. He also tried to sit up a few times, succeeding once and then turning pale white and literally crashing. Poor little guy. So we will just sit here again today and maybe tomorrow. If they are unable to progress on Monday then TPN and Lipids will be considered. We are hoping it doesn't come to that. While it provides needed nutrition when there is no other way it is very hard on the body. Thank you as always for your continued prayers and support. We will update as things change.
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It Works!

Elias had his first poopy diaper yesterday just before noon, which is a good indication that everything is working. That is not to say that he is not still in quite a bit of discomfort. His stomach is visibly distended and hard as a rock from all the gas and green bile is flowing from his stomach into a vented trap. We were able to stop giving him Valium last evening and over night. So that is a positive.

A brief way of describing what is going on is to remember that this is the first time his colon is working "as it should." There is a bit of a shock to the system and it needs to relearn its role in terms of functionality. In the meantime the bowel is rejection the natural fluids our body creates in addition to the fluid that Elias is getting via IV. The body is forced to defend itself against the rejection of the bowels until it becomes accepting. This comes by way of retching and at times vomiting in order to evacuate what is in the stomach. The reason his bile is a dark green is simply from the liver enzymes and fluid that deposit in the stomach which a normal working bowel would re absorb and push out. So while it is a hardship for Elias at the moment every aspect of what he is experiencing is validated by this adjustment. They still are not in a hurry to begin testing his stomach and bowels yet. We fear they may wait until Monday for this, but hope not. We did remind them the length of time he has been without nutrition. The nutritionist for the vent unit came in to visit Elias and noticed the low concentration he was on and wondered why. Apparently, being off the Colorectal Surgical Unit also means being neglected for little details like having the dietician (an automatic for every patient as they are part of the unit team) follow us to ensure his needs are met. Hopefully this will be addressed and adjusted.

So more sitting and waiting and wondering what the plan is. Hopefully, Elias will feel better, be more engaging and make progress today. If there are any developments we will update you then. Thanks for all the prayers and support.
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Not Much Improvement

There unfortunately is not much improvement to report. The plus side is all of Elias discomfort and issues are explainable and to some degree validated. It is just very difficult to watch him suffering like he is. Particularly, when we are so accustomed to seeing him bounce back so quickly.

The easiest way to describe the issues is that Elias has a lot of retained gas at the moment. It is filling up the empty space as shown on his x-ray, but is beginning to also put compressed pressure on his organs. This is causing additional discomfort. The incision and surgical site is not seeming to bother him. We are continuing the every 4 hour valium around the clock to keep him as relaxed as possible.

This morning has been particularly rough for Elias. He has vomited several time already. Considering he has not eaten since Sunday morning one could imagine how particularly painful that has been. He has also been crying out more than normal, needing more frequent suction, and unaccepting of any physical contact. This includes loving contact. Can't say I blame him though.

The goals for today are to keep Elias as comfortable as we can and to hope he finally passes stool and some of that gas. Thank you all for your continued thoughts and prayers. We greatly appreciate it.


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Day 2 post surgery AM update

This morning Elias was still uncomfortable and in some obvious pain or discomfort still. This is the longest we have seen him take to bounce back from a surgery, but the adjustment in his body this time around is quite monumental. We have been giving him Valium every 4 hours and the occasional morphine dose. This is the only way we can get him to relax. Poor little man!

This morning we traveled to radiology for a series of x-rays. They typically wait until stool has passed, but that usually happens by now. So they just wanted to make sure everything was fine in the bowels. Elias has not had any food or nutrition now for 72 hours. We continue to wait for stool before the slow process of working him into his feeds begin.

We will keep everyone updated as things come up. We appreciate all the continued support and prayers.
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Recovery Process

Elias is still uncomfortable and in a touch of pain from the surgery. We have not given any morphine since this morning. Valium has been the life saver today. He has napped and rested well and often through the day. The urinary catheter was removed late morning and that has helped tremendously to decrease some of his discomfort.

The surgeon came in early this afternoon to check on him. He was pleased with how his stomach looked (no swelling) and with Elias overall appearance. At this point we are just waiting for gas, stool, and/or both to pass. At that time they will evaluate when to begin his feeding. He commented again on the small and tight connection from the colostomy closure and his concern over proceeding too fast and causing damage.

Elias has given some smiles but generally just wants to be left alone right now. We can't blame him. He is on 3 different antibiotics too on top of not having eaten anything since 1030am Sunday morning. All in all though recovery is progressing nicely albeit slow going. Thank you all for your continued thoughts and prayers of support.
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No More Colostomy

Surgical update #2. Elias is in some pain and discomfort. He is getting morphine every 2 hours. The colostomy reversal went well, said the surgeon, but his bowel is very tiny. What does that mean? It means they are going to be more conservative with how quickly they resume feeds after he begins passing gas and stool through the rectum. The concern is that the point where the colostomy was attached to the rest of the bowel could open or not heal correctly if they try to press getting feeds flowing. This will keep us inpatient about another week possibly more. We are hoping that Elias will do as he always does and recovers quickly. Tonight there is also a urinary catheter in place which is causing him just as much discomfort. He has been asleep most of the evening only waking long enough to grimace in pain, cry a little and the morphine knocks him back out. Rest well and long little man you deserve it. The hourly bowel cleansings pre-op last night ended up literally going until 5 am, do 10 consecutive hours, so Elias got very little sleep last night. So he has even more reason to be exhausted. We are not positive but it seems like the catheter will come out tomorrow, that is our hope too. For now it is just sit wait for gas and stool and heal. That is the plan and being stuck in that crib is going to drive Elias NUTS! We will update tomorrow on the developments and improvements. Thank you all again for your continued thoughts and prayers. Everyone’s support is greatly appreciated. 

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Surgery update #1

Elias has been in surgery since about 1130am. The colostomy reversal portion. Which is the longest segment is last and being done now. Pulmonary and ENT did bronchoscopies and urology did a cystostogram with urethreal dilation.

Those three are complete now. The airway team said that not much has changed and they had hoped to see some improvement, but haven't. Elias airway is still very flat, small, tenuous, and at risk. They are still scratching their heads trying to figure out how this child is breathing as well as he is. We will accept the gift that he is giving and continue with caution as we have been. So that is positive despite no physical change. What we really need, and the emphasized this again today is for Elias to grow! Damn FA and its growth issues! It will make our GI appointment next week interesting.

We will update again when Elias is out of surgery and we know more about his colostomy reversal.
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The Term “Planned” Admission Used Loosely

Well this day pretty much went as expected, a crazy chaotic mess. We were told to admit Elias this morning at 10 am for pre-surgical prep. It took until six this evening for any orders to be put in the system for the nurses to do anything, so we sat there and no one could do anything or tell us anything. Why were we there so early if they were not going to start anything or even put orders in? The sad part is we had spoken with the colorectal nurse coordinator just a few weeks ago and rehashed the insanity and unprofessionalism that surrounded our surgery by the same team in June. She assured us that would not happen this time. Well she was wrong. Sadly the events in June were so bad and unorganized that the patient advocates office became involved. They sent us a letter outlining their review of the situation and that they would take action to improve this area. They too were wrong. Anyway, enough about that and we hope it truly ends with just today being a cluster…..

Elias is in good spirits tonight despite going through hourly bowel cleansings since seven this evening. This consists of placing a Foley catheter in the colostomy stoma and flushing saline in and drawing it out once an hour until the return is clear. Hopefully it will only take 3 or 4 times. He also has not had any food since 10:30 this morning and will likely not be fed again for another 24 hours or so. Still he is being an amazing little man, laughing and smiling.

An update on the Central line issue we informed everyone about last night….The CVC team examined the line and determined there was a weak area that needed to be repaired. This was a relatively simple procedure and done at the bedside. They cut the line beyond the weak point and fed another wire through and glued it together. That was a sigh of relief for them to be able to fix it. Though it was simple it is not without risk and much be watched closely to ensure nothing made its way into the line to contaminate it during the repair. We did have some issues and it took 3 repair kits with us literally covering his open line with a sterile gauze pad for close to 10 minutes while we waited for another kit to arrive. He appears fine and it has been used for labs and seems to be functioning well. We are very relieved that stress is removed.

We will post updates through the day tomorrow to update on Elias progress and recovery with his surgery. We are not certain as of now exactly what time he will be going in the Operating Room. Please keep him in your prayers the next few days, we greatly appreciate it.

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• Elias' Central Line

Disappointing Findings

Elias had another Bronchoscopy today and long story short the airway is not improved and still considered unstable and in Dr. Woods words, “down right scary.” They really can not figure out how Elias is thriving like he is. Basically, the vascular ring repair did not do even a fraction of what they had hoped for. We knew that was possible, but were hoping for the best. The trachea is still compressed significantly and of course there is the floppy tracheomalacia. Using the parenthesis symbols this is what a normal air way should be like if left is top and right is bottom: “()”  air flows through the middle. Elias’ airway looks like this “)(" air can not flow or is constricted. Add the Bronchomalacia a little lower towards the lung and things get ugly and out of control and takes very little to get Elias in trouble. In the case of yesterday’s episodes it was caused by an upper respiratory infection or virus of some kind. The cultures will continue to grow for 36 more hours, but the initial 24 hours does show white blood cells and negative gram rods. This more than likely is the early sign for an infection. As for the other times prior, well compression, floppiness, and unpredictable factors are likely the cause. That’s a fancy way of saying we do not know for sure and there is nothing we can do to prevent them from occurring. While much of what we just covered is almost review,  they were factors we already knew of, but we had hoped for a better outcome with the vascular ring release. The other disappointing factor is that there is really not much else we can do, but watch, wait and hope the compression and malacic areas mature and get stronger on their own. That is the really frustrating part to all this. The inability to explain or even prevent this from continuing to happen. We will always need to be at the ready 24/7 should Elias stop breathing again and that is the best that we can do.

If there is a bright side through all this, and we are really trying to find one, it is that the overall kind of sort of plan we were discussing Monday and it really will not change much. As we said they do not know how or why Elias is thriving like he is but we have to run with it and be cautious and vigilant to keep a watchful eye. We are going to continue the windows off the Si Pap. Tomorrow they hope to combine the two windows to create a daytime scenario without intervention and a night time usage of the Si Pap. He will also be getting pressure support on top of that, which is more like Bi Pap. The other component we are going to attempt is something Dr. Wood has had some success with in other extremely complex airways he has worked with. That is the use of a one-way valve or the passy-muir valve. We had tried this in the past but he is hoping the conditions inside have changed enough to have more of an impact. This will create a natural pressure to assist holding the airway open when Elias is off the Ventilator. It will hopefully provide enough edge to keep him off the ventilator for longer stretches during the day. In terms of when all this will happen to a point where Elias can return home is still very much in the air. It will be at least a few weeks. That too was a morale buster. I suppose much of this frustration stems from the feeling of going backwards almost to the point of guilt. We came into this without the need for any mechanical intervention and now we are being absorbed by the concept. What did we do? I think the resonating theme of all this is that Elias is going to have the tracheostomy much longer than we had originally thought. That too is difficult to come to terms with. The decisions we made did not involve all this. While we know it is not the end of the world it certainly changes the dynamics of things overall and the impact it has on Elias.

Despite the grim and disappointing news Elias did very well through the procedure. They also changed the trach tube back to a cuffless trach, Thank God! He recovered well and quickly and was butt-bouncing on his crib and moving all over within hours. That child amazes us to the very core. He amazes the doctors and of course family and dear friends. We have said it before, but today was one of those day that his terrific spirit helps us overcome the news and pushes away the negativity enough not to allow it to overcome us. I thank God everyday for allowing Elias the ability to show us his spirit while raising ours, especially when they do not feel like doing so. Mommy & Daddy Love you Elias and we are so sorry that you have to suffer and endure all of this.