Wednesday, December 19, 2012

What to make of this news..

Some very interesting news to share...not sure what to make of it or how it could even be accurate but.. Samuel had his exit Battelle assessment from the birth to three intervention program (his entrance assessments at 2 months putting him into pretty negative territory) as he is now 33 months and approaching the time when they transition a child into an IEP with the school system if they will need further prep to get them ready for Kindergarten due to their disabilities etc.. well I have to brag on my Samuel..our baby boy who had brain scans from birth showing encephelopathy and has had to fight through 17 hospitalizations, surgeries and more life threatening events than anyone I have ever met.. has just scored waaaaay higher than expected on his developmental testing and is coming out at an adjusted developmental age of a 48-57 month old on many parts. This is hard to believe for us since there are soo many things he struggles with physically, verbally and with feeding.. but apparently he is ahead cognitively, socially and receptively at this point. None of this should suprise me because he has just amazed us in the changes we have witnessed over the last year and rather in the last 3 months. Although I expect he will continue to require more therapy and as much effort as we can make in working past his challenges and although I know this score may actually make it harder to ensure he gets the help he needs, I am overjoyed that this assessment indicates he is doing so well. He only walked a year ago and now he is starting to run. Run Samuel Run!

Saturday, December 1, 2012

Tick Tock..

One of my favorite children's stories growing up was that of Peter Pan. Beyond the story's most notable characters of Peter Pan, Tinkerbell and Captain Hook, there is one who heightens the calamity and brings on giggles of anticipation from my kids every time he makes an appearance. His name is Tick Tock the Croc and this character lives for one motive.. to get one more chance to eat Captain Hook after getting his first taste when he bit off Hook's hand in an earlier altercation. He has an insatiable appetite, even having swallowed an alarm clock in his quest to be satisfied. With this clock somehow permanently part of his gastric system, Tick Tock can be heard ticking from a distance as he inches closer and closer to the prize he hunts: Captain Hook. Well, I share all this with you because our sweet boy has adopted his own Tick Tock in the form of his recent implantation of the permanent gastric stimulator. We hope it will be as determined as the Peter Pan character at meeting it's objective and taking out the villains in Sammy's daily life caused by his gastric paresis and dysmotility.

Sofia was actually the one to give his medtronic device a name.. similar to a pacer, there is a clock of sorts working to give off calculated electrical discharges at timed 3 second intervals into the outer lining of his stomach muscle. The makers of this technology can not fully explain why it works and scientific analysis is still in its beginnings. These devices have long been prescribed and allowed as a form of enterra therapy under a humanitarian use waiver by the FDA. You can imagine we were skeptical when first offered this as a possible solution to his seemingly constant and increasingly worsening problems associated with his slow gastric emptying and motility. Because Samuel had a nissen fundoplication done at 2 months of age to prevent any type of reflux or aspiration of stomach fluids, he has never vomited or refluxed in the typical fashion. He instead would writhe with feedings and when his g tube was vented, large amounts of stomach contents and sometimes intestinal contents and bile would come out into the open syringe. At first these episodes were few and far between but over time they were becoming an exhausting and debilitating part of his condition and pointed towards signs of disease progression that might mean more interventions in the future. Interventions such as permanent central line feedings which had only been needed for short periods in his past. I have blogged in detail however at the remarkable positive changes we experienced with the trials of the stimulator over the last four months.

So to bring you up to speed on the events of the last month.. After returning home with his last temporary stimulator, things went well for about three weeks and then we needed to head back in mid November for a check up with his surgeon. Four days before I started noticing some changes in Sam for the worse and on the drive down he became unable to tolerate the usual feeding amounts. The Dr found that the leads were faltering and removed the stimulator in his office. He then wanted one more trial at home for one week with no stimulator in place at all. Now he always reminds us that most patients continue to feel positive impacts for days or evens weeks following its removal. We think it started to cause him significant trouble within three days. By the Monday following our appointment Sam was running fever, lethargic, acting "off" and then dropping oxygen saturations even while on oxygen and while not dealing with any respiratory distress. Sam is not used to higher fevers as he is usually in pretty bad shape with low grade fever and we have to act aggressively to get ahead of what is plaguing him. We figured it was either A. a bacterial infection like ears which we could not find, B. a virus we picked up somewhere. or C. his bodies way of alerting to us that the body was not meeting the bodies metabolic needs due to the stress of the losing the stimulator benefits. We just could not know. Now I would suggest we should have done blood work or cultures that could have shed more light but I think our Dr from a practical position could not see how it would change what we were doing to support him, which was 10 day antibiotics (cefdiner) and lots of fluid and rest. The surgeon might have disagreed.. as he did not want to go to surgery not knowing the prior status pre antibiotics.. and I could see his point. Anyway, we ended up pulled off the antibiotics on Day four when he broke out in a rash. Now then there was the question of whether rash confirmed that it was a viral process going on or a reaction to the antibiotics. As the weekend came round and we were still scheduled for surgery on Tuesday, I worried we would be postponing. Sam ended up making a turn and with no fever by Friday evening, we were on for admission monday, surgery tuesday. The girls were out for Thanksgiving break and we made the plan to all go down together and support Sam through what they said would require three hours in the operating room and up to a week in the hospital.

My In- Laws lended us their spacious conversion van and we got Sam admitted on Monday. I left Jason there to get him settled in while the girls, Max and I found a hotel since there was no space at the Ronald McD house. It is no easy feat at night to unload a van and check in to a hotel with two bickering girls tired from a long day in the car and one hungry baby in tow! God gives us strength in the most curious of situations. I have to say that my girls learned real quick what I needed in way of their help. ;) I was so pleased though with the place we stayed cause they had a nice breakfast in the lobby that was healthy and the girls relaxed by a roaring fire while I took in all the details about Sam down in surgery that next morning. I was a little tenser because usually I am the one there to send him off but at the same time, I was glad Jason could be more involved in the caregiver role within this capacity because then he better understands what it is like for me when he calls from home and I am inpatient with Sam. Until you are in each other's shoes, this can be a delicate area of your coping as a couple. Luckily, we have always respected each other during these difficult periods and it has made all the difference. I have met so many families that fall apart when under the duress of having a medically complex child or stressful situation.

Then just to make things that much easier, in came our dear friend Amber and her family with rescue offers of taking on my kids while we were staying that day close to Sam in recovery. They even took Max which took a lot of convincing because I could only imagine how she and her husband would juggle their own three with my extra three. I mean, they have a complicated little guy of their own and enough equipment and oxygen and meds etc to run circles round what most kind neighbors would have in offering to keep a few extra kiddos.. but God must have been laying on them because she would not take no for an answer and soon all the kids were in their backyard and watching movies, having a grand old time. They even kept Sofie and Max through the night while I took Bella to her sleep study which was being done to check out some respiratory and daytime focus concerns.

Sam was in the OR longer than expected but they were having to work with a small abdominal space and they had told me ahead that this might be the case as he was the smallest they had worked with. His buddy Nate was not much bigger and about a year older and they ran into the same issue with him. So he was in for almost five hours. His recovery started out with good vitals, a lot of pain controlled by meds and blood suctioned by NG tube. His incision looked good and vitals were good in that first day. Second day was much rougher and although I expected that, I don't think we expected as much in the way of airway swelling concerns as he experienced. He was in distress and pulmonology was quickly brought on board but he responded well to the epinephrin and prednisone steroids they jumped up to get ahead of these issues. Later that day he actually had a pretty significant Brady event that had Jason and the nurse responding aggressively when he turned blue and stopped breathing at one point. This was something I actually was prepared for as these autonomic events seems to become more frequent when he is taxed and exhausted..metabolic crises of sorts.

By day three, things were looking up and we shared a Thanksgiving Dinner together around his hospital bed.. styrofoam meal and all. It was very graciously brought to all the families in the children's ward by Parkside Baptist of Gainesville.. what a kind ministry to share a meal with families by bedsides of loved ones when nothing in the hospital was open besides a vending machine. It may have been the most special Thanksgiving ever for this family.. just being together and supporting our little trooper. At that point Sam began asking for food and was eating jello and shaved ice in tiny amounts. He started back slow formula feeds and myself and the kids stayed in the ronald mcd house awaiting word on when he might get to go home with short visits to swap out sitting by Sam. It was on the weekend that we noticed he needed less morphine and then down to none. At the same time, however we watched him experiencing low heart rates, prolonged all the time and not just as a brief Brady episode. He usually has a heart rate around 100 to 140 when awake and it was trending in the 50s and 60s. No origin was attributed to this change but by Sunday afternoon when he had his first BM and was cleared to go home, he started having higher heart rates. We felt better making the trip.

At home Sam is doing well.. far better than we ever expected. He had a few Bradys on his first few nights home but respiratory has been great. His incision which was fully open to the abdomen is healing well and the box which was pocketed under his skin is not bothering him either. He has a few times tried to pull at the skin glue and we are trying to train him on safe ways to move and do things with the new device. He started back to therapy and they are going to incorporate how to best work around it as well. One thing I have to learn is all the things he should avoid contact with like cell phones and radios, microwaves and security devices. These can not only interrupt with the integrity of the device but some can cause physical harm. tHe device feels weird when you touch it from the outside but he doesn't seem to even notice it. His stomach is very distended and we re trying to keep him from jack knifing or twisting too much. He talks about his Tick Tock and the girls tease him that they can here him ticking down the hall.. of course it is like a pacemaker and does not make a peep.

On the agenda moving forward will be follow ups with most of his specialists coming do.. plans to maybe see my mom's family before Christmas and maybe setting up to meet with the Houston Drs at the mito clinic. I had put this off cause I just could not get all his records organized and sent over till now but things should get easier in days ahead. Sam is just showing us such positive improvements with both this stimulator and the supplemented oxygen he is now wearing through the night and intermittently through the day. I did get a copy of his sleep study results on this trip and he experienced 31 apneas, only 5 of which were obstructive. He had 81 oxygen desaturations and recovered well with oxygen placement. I couldn't believe how concerning this was as that was as that night he was doing great and feeling wonderful.. what must it be when he is sick? yikes.

So the last news I wanted to share is difficult. I am praying for a family we have followed for some time whose child had mito, complex 1 and was two days shy of his 6th birthday when he passed away suddenly. In many ways, I thought this child less complicated and was shocked when I found out he passed away this week. This is such sad news for his loving family and friends. Our friend Amber knew them personally and from reading their blog I felt an instant kinship to this sweet mom. I pray peace for them and God cover them with love and support. I cant even imagine this loss. I know I cant live in fear but it is terrifying to understand the tragedy this disease can purvey on little children.

Time to get moving as I am way behind on everything. Will be a busy weekend..Tick.. Tock..!

Friday, November 2, 2012

Birds of a Feather Spook Together..

Halloween was so much fun this year. I guess I was feeling a little crafty after our kids painted some pumpkins. My creative juices kicked in and I decided to make Samuel a costume. Not just any costume... A Dream-Come-True Costume! I have made a costume for each of my kids at one time or another and most of them required little skill but the fact that I made them myself heightened the anticipation of seeing them in their outfit. This was Bella in her ladybug at 7 months old.

So, I set out to make Sam a "Blu" costume. Blu is the Blue Macaw character in the movie Rio, which is a movie about exotic birds on an adventure in Rio, Brazil. The main character is a bird who happens to not be able to fly and within the great adventures of the larger plot, he acquires the inner strength and confidence to spread his wings and fly. The symbolism was not lost on me when I watched him falling in love with this movie. He asks to hear the soudtrack over and over and over... must be the latin in his blood! He loves to dance to it and it is like he becomes Blu everytime it comes on the screen. SO.. long story short I had a mission and little time to make it happen. I found a blog where another mom made the same costume and tried to figure out how I could do it with materials I had on hand. I worked on a cool head piece on Saturday night. I never knew paper mache could be so easy! It was made over the form of a plastic fireman's hat and some paper plates and foil. That needed to dry over night and I figured I would do the wings the next day after church. Well.. the hat had not dried and all of a sudden all my children were convinced that they should be other bird characters from the film as well. I was blowdrying the head and painting it, cutting a million little felt feathers and trying to please the diva in a 5 year old who wanted her Jewel character's beauty to shine through without covering her whole head.. ahhh!

A plan took shape.. there were even sketches by Bella! 4 hours and a ton of felt later, with burned-to-the-crisp finger tips from the hot glue gun along with lots of healthy laughter with my girls..we managed to turn out a flock of exotic birds in time to attend the Fall Fest at our church. I did cheat and borrow a parrot costume for Max from a friend. (Thank you, Tiffany!)

Bella won a cake on the cake walk and as an added suprise it turned out to be one made by our neighbor. Sam saw his sister play the donut game and had to try it himself. He actually got some nibbles off without hands! We then visited with cousins and checked out their cute costumes.

Later on the actual Halloween Night, we hit the road with neighbors and came home with loads of loot and some very tired little birds.

To hear the music Sammy loves from his favorite movie...
Click Here

Abundant October

What better time to experience change and appreciate abundance than in the month of October? The autumn season with the cooler weather, rich landscape hues, falling leaves and crisp breezes - all signs of harvest. Every year I look forward to the prospect of pulling on my favorite pair of boots paired with my coziest scarf, filling my mug with a hot pumpkin latte, and listening to giggles from little girls in my back seat bound for the local pumpkin patch. These cozy images are made even better by knowing we are on our way out of hurricane season and will experience the holidays just around the corner.

In looking back over the last month, we managed to get through a series of events that could have been impossibly difficult - some of them anticipated and others of the more unexpected variety. Our schedule just seemed incredibly packed this month but overall, I am overwelmingly thankful that everything is going well.

Here were some highlights of October for us..

The month began with a return to Gainesville for an appointment with Samuel's surgeon and a sleep study for the pulmonologist. While on the trek south, I enjoyed the quiet of my own thoughts, considering the prospect of signing on to do the final and permanent placement of Samuel's gastric stimulator during this trip. The process has already been a leap of faith thus far as each time we headed to the OR we were trying something so new to us and with little clear evidence or guarantee that it would solve his gastric issues. But we stayed the course through mishaps and illness, soon to find him vitally changed and doing so much better overall. He is now managing bolus feedings of 150 mls through his G port at one time, which had not been tolerated since he was 8 months old. He is digesting by stomach and not just directly into his intestines now as his gastric emptying is much improved with the pacer. We also see improvements in areas not related to gastric functioning. He began talking more, starting to run, singing and trying to count. His eating by mouth is still slow but I guess even that area is more than before. We saw him resting better and brightening up at the prospect of participating in family activities, which is huge for his siblings and his own quality of life.

Overall.. the temporary pacer on his GI has been going very well. He has rarely been in pain which is by far the best side effect. My take on this whole stimulator success story is that we are seeing success frankly because he is functioning better at baseline with less energy outlay. I think because he is not wasting energy on pain and a dysfunctional stomach, he is better able to take strides in other areas. He is trying to eat more by mouth now but this area is still a challenge and improvements have been slow. My greatest praise is to see him off the feeding pump for most of his daylight hours. He gets boluses during the day and then goes on his pump at night.

The drive down went ok but then he was tanked out and was asleep before his Dr. entered the room. At the appointment, they found his stimulator to be still working, which was suprising as they expected it to have dislodged by this point. We had noticed in the few days before the appt, he was having gut slowing days and questionable periods reminiscent of past issues, so his surgeon adjusted the settings up and asked us to go home and try that for a week and then come back for new probes (which meant yet again another endoscopy, this would be #4, to place new probes for temporary placement). He explained why we are still trialing it and should wait on doing the permanent placement. He said insurance is another hoop they have to jump through now and by fully testing the worthiness of this measure, we should have no issue there.

We followed this appt with dinner that night at our friend Nate's home with his sweet family. I notice alot lately that Sam is quite overwhelmed to be around lots of new kids and I think he probably needs more exposure to this for social development. I often forget how much time he spends in our home alone with his family and nurses. Meeting this family really was God working in our lives. It really helps to have such kind and supportive friends who share and understand this journey personally.

It was hard leaving their house and heading over to the sleep study since I knew Sam would hate that so much. They put a million electrodes all over his head and body with every other mask and sensor possible. I worried he would never fall asleep for how badly he was hating having all the wires and probes all over his body. But he did and then after an ABG (arterial blood gas) in the morning, we were off and on our way back home.

At home, we got back into the swing of things and Sam seemed better at the new settings. He attended some family activities and had more energy than usual. We have kept up his weekly therapy but made some recent changes to get OT back into our home environment. This helps him by allowing less illness exposure and a less taxing schedule. We had also noticed his 1 hour sessions out of the home were sliding down to half hour sessions, so this new schedule should help him get the most from his therapy. He still goes out of the home to see his PT, who has seen him since he was 3 months old. She is working with him on learning to jump and balance and lift his legs when he walks along with so many other new goals. Sam is particularly loving her little trampoline. I am also working on filling out some developmental charts to figure out where we stand in our progress over the last 2 and a half years.

It is quite amazing to see how far we have come! Sam has fought for every step along the way. Now watching Max leap through each of these milestones, I can appreciate how important they all are. It is hard sometimes to see it happen so easily for Max after watching Sam works so hard at it. I am expecting Max to walk on his own within the month and he is busy crawling and cruising all over the house.

One new change this month was that we had our transition IEP meeting to begin the process of transitioning Sam into the public school system. His Early Steps Program will end. It will mean many new formal evaluations in the months ahead and I am hoping his best interests are always at the heart of these discussions. He will likely attend a local school for therapy and receive hospital/homebound education until he is a little older and stronger. When they talk about what would be offered for him in the PreK D classroom setting, all I can think of is illness, setbacks and exhaustion. It is a 5 hour day and our boy is rarely ever awake half that time in a block during the day. Cognitively, Samuel is doing well. He understands things very appropriately and his communication is following with great strides. It is probably the most obvious delay. His gross motor and fine motor are coming along as well and the gap is far less noticable to me. There are things his typical peers are doing like riding tricycles and running without falling, but we will get there eventually. Recently Samuel was fitted for a medical stroller this month as well which will be outfitted to hold his equipment. I wish we could use a normal stroller forever, but as he grows, it won't be as adaptable and hold equipment effectively. This will allow him better options for when he just can't stay up on his feet.

Soccer practice and gymnastics have kept Sofia buzzing around. She has made goals at most of her games and really seems to be into it this year. Bella has been taking some time off sports as she struggles with some excercise-induced respiratory issues, most especially during allergy season. We are looking forward to a church cheer program that will start soon. She is into reading and singing in the choir. She also loves going to bible study. I have been attending choir practice again while she is in bible study. Jason even took over with the whole gang so I could attend a girls poker night. Book club is next week and I hoping to make that one as well, but I know I am really blessed to have such a supportive husband and sometimes it is just too much to ask of him after his hectic schedule. Our busy schedules are somehow holding together. We do the dance of Jason spotting me with the boys while I take the girls to their functions, but it would just be so much easier if Sam could handle keeping up with their pace and the exposures that come in those environments.

I have recently began venturing into potty training for Samuel. This is always a fun time for mothering! I think this may take awhile. He understands when he has to go but has a bit harder time getting onto an actual potty and does not always communicate himself clearly to ask to go, so this may prove difficult for him. It is also an opportunity to work on yet another new skill, that of helping him dress and remove his own clothing. Potty training boys is clearly unchartered territory for me and I have not mastered how to best explain the concept of aiming at the cheerios yet. :) I guess I will be seeking more advice from my friends with sons!

About a week after we came home Sam and I drove back down for the probe replacement. It was on a Sunday and I was thinking we would be back home late Monday or early Tuesday. I always hate leaving when the girls have so much going on but luckily our family pulls together to make it less of a disruption to their daily routine.

That first night in the hospital, the nurses got an IV placed suprisingly quickly for how difficult this always is with him. They had placed him in this plastic bean bag of sorts that molds around the child and then when a switch is flipped it is sucked dry of all its' air and hardens so he can't move a muscle. This is all done over in the "situation" room. Moms of complex kiddos know this room well and when they send you there you can figure the nurses are not feeling so lucky that day! Anyways, this resulted in Samuel sweating buckets of fluid and acting like we sucked the very life out of him, so we may opt out of the bean bag next time! :) I have to always remember that heat intolerance is very dramatic for him, so the plastic sealed around his body may not have been our best choice.

The position of his IV was not good that night as it was in the side of his foot and was kinked in such a way that all night the occlusion alarms kept beeping off at every move of his ankle. He somehow still slept hard for most of the night but then woke up mad and yanked his g-tube out. I had drifted into sleep in my chair and woke to a ton of people huddled all around the crib trying to place it back in. This is no biggie with a simple G tube, but with stimulator wires, it can be more of an issue. I figure this is when the probes were probably dislodged, because the next morning when they took him into the OR, they found that they were no longer in proper placement. Here is a pic of how Sam deals with pre-op nerves.. haahaa! Nothing like a little angry birds game time on mom's I-pad! Meanwhile there were adult men on the other side of the curtain crying over getting an IV for their procedures not realizing my 2 year old sitting there has had more procedures, surgeries and IVs than he could imagine. I am proud of my brave boy.

Leading up to the procedure, I met with the anesthesiologist as usual to do the paperwork and discuss the plan. The IV general anesthesia and intubation are really my concerns on each and every procedure even when it is even a simple, non invasive one. He has had many many anesthesia procedures and it never gets easier to let them roll him off to the OR. In this case, they were doing yet another endoscopy to thread in these little probe wires through his stoma and screw them into the stomach lining. Should be no biggie as we have done this like 3 times by now.. right? Never speak too soon. Well, I had explained about how Sam should not receive any gas (as I always do) since he has had adverse reactions in his history. This has so far been well-respected.. until there was little choice on this occasion. I guess when they got in the OR, the IV from the night before was still not working great and in order to find better access, they had to give him a little gas. They still were unable to secure a better IV before he was out of the OR and in recovery. It took longer than usual which had me a nervous wreck sitting in my hard little plastic chair in his room.

When he first came out, he seemed to be doing okay. We saw his surgeon right away and then again a few hours later in his room right before we started his meds and feeds. He was doing fine so he said to plan on heading home the next morning. It was about this time that the bottom seemed to fall out for Sammy. He started working harder to breathe, dropping sats pretty significantly. He was not tolerating even the slowest amounts of meds or food. He was acting delirious and in an angry haze, he was even trying to hurt himself. His IV had finally gone completely bad and we were dealing with nurses shift change and a new roommate admission. I was watching him decompensate and was shocked when the peds surg resident was less than aggressive in finding any solutions. We were trying blow-by of oxygen near his face but he was yanking his head in other directions. He was sounding junkier and junkier and retracting and nasal flaring.. obvious signs of distress for him. But again noone was doing anything. Then he lost his not-so-great IV for good. This can be life-threatening when you have no other way to keep him hydrated.

I asked the resident what the plan was and could not believe he actually said it was up to me whether they called a picc team in to try for another IV because he was so difficult for access. I said that I felt we had little choice since nothing was being tolerated in his tummy and he was already crashing. He acted like all balls were in my court and seemed hesitant to make any affirmative decisions. I guess he hated the prospect of a central line after such a simple procedure and of course that was not our hope either.. but we needed access so someone at least needed to try again for the IV. It was so weird. The nurses thought he was acting bizarre. Well then of course the stat team could not get one and then the PICC team struggled too. They finally became so concerned by how bad his sats and breathing were going that they challenged the resident to please notify our surgeon on how things had changed for the worse and to get on the ball for getting labs and respiratory to order oxygen by cannulla. He was at this point having tachy periods and was dropping sats into the 60s and 70s, usually coming back up but consistently not getting up to the 90s. It was interesting to hear the nurse telling the resident, "You handle this or I will" because I had so much more trust at this point in this charge nurse than I did in this young resident. He responded to questions on why he was not concerned by saying,"he does this at home." I was very quick to jump in at that point and tell them that "no, he doesn't just hang out in the 70s on sats at home!" I guess in a learning hospital, these situations are learning opportunities.

I was glad when our surgeon swooped in and started getting things under better control. Within minutes Sam was transferred to a higher unit with better monitering. He was put on higher flow of 2 liters of O2 and was finally responding. He had a chest x ray which showed some infiltrate from the gas but we knew that most of the junky breath sounds were from him not fully inflating the lower bronchi. He gets areas of atelectasis during metabolic and physiological stress. He began percussion therapy or what we call the "banging therapy" since they come round and just bang on him for awhile. Sam usually hates it at first but soon realizes it helps him. He then gets to keep the handmade banging tool himself. We knew he was doing better when he started getting up and banging all over his crib with this toy. He can be such a wild man!

Labs were pulled in this process and they consulted the pulmonologist. Two came by with differing views. Our pulmonologist said the sleep study results were pretty bad and that she thinks he chronicly traps CO2 and that we need to infuse him with some additional oxygen support at home and see how he does. She thinks this is all related to his aerobic vs anerobic respiration and how it is basically a cycle where as his cells don't process energy well, he then has weak bulbur and respiratory effort, he doesn't fully open the lower bronchi and then traps CO2. Sometimes we see low low sats but sometimes sats look okay but he still looks completely off in this weird way. It makes it hard to know when he needs help. The idea is to infuse him with the all-precious ingredient - oxygen, so that he has more energy to be more effective with his respiratory system and other systems overall. Of course our fear is that he would lose his personal drive to breathe without the mechanical support. And that is what we still don't know. For now we are using oxygen intermittantly as needed.

They tried to get an ABG (arterial blood gas) on four occasions but this is harder as it is deep in the artery. Each stat and flight team would fail and another would come in to give it a go until we finally all agreed that he had miserable circulatory system and we should just let it go since we might never secure the test. It would have been helpful info but not at the detriment to his need for rest. I think later that night we did finally get a decent VBG which looked pretty good but I did not agree with the nurse that a VBG was basically as helpful as an ABG for checking his blood gas. Anyway, he was already looking better to me and I felt things were on the mend. After discussion the situation with his surgeon, we all agreed this was really likely prompted by his reaction to the anesthesia. There is a medical journal study on how sevo flourane gas is an inhibitor of complex 1, which is one part of Samuel's respiratory chain that is already deficient in function.

In the days that followed we slowely saw improvement in the IMC and he was weaned down to about half a liter of oxygen, which is how we took him home. Before going home, he had a day where he wanted to get out of his crib a bit. So we walked down to the playroom to see the acquarium and play for a few minutes. It was only 5 rooms down the hall but I could see he was still struggling and tired. He did okay and even tried to paint a pumpkin with the child-life volunteers who brought breast cancer awareness pink pumpkins to the floor to be painted. Samuel went to lift the brush against gravity, but clearly just felt too weak. Moments later, he fell out of his chair flat on his face. It is hard to watch him feeling this weak. This is typical for him though following a rough patch, so I just knew we needed to take it slower.

We were blessed by so many friends praying for him that week and definitely felt loved and lifted, of which we can never fully express our thanks. Our stay ended with a happy visit from Sam's buddy Nate and his mom Amber right before our discharge. We all went out to lunch at Olive Garden on the way to the interstate and she was able to help me process some of the new changes with trying to utilize the oxygen more full time at home. It helps to talk with someone who has been there and knows the frustrations but yet is also seeking answers and solutions.

Since getting back home, it has been an adjustment having the long tubes all over the house.. but it is worth it when I see him having longer "up" periods overall. Some of our nurses have been more supportive of this change and others would rather not hassle with the fuss of getting the cannulla placed in his nose. It is still something we have to pressure him to do initially but bribes of lollipops for wearing his "noses" help and once it is on, he actually seems relieved and happier. This all has made me moody at times.. not at them, because I know we all hate to make Sam do things he doesn't love, but because I need to process this and still be sure to work at getting him better respiratory support.. even if it is not convenient and seems like a step backwards.

The day after we returned home though, I had a personal wakeup call that I needed to watch my own limits. Sam, Max and the nurse had joined me out to run to get his meds and of course all the no-sleeping and stress caught up with me. We were heading into Panera for some lunch and I started having a crazy pain in my diaphragm. Still not sure if it was low blood sugar or just not enough rest or what, but I had intense pain across my diaphragm, had a had time breathing and felt weak and tingly all over. I nearly fell over my own feet. I wasn't nautious but I felt like I was falling apart. Strangers were approaching me to offer help but I couldn't figure out what was hitting me so hard. I was starke white. I ended up back at my father in law's house on the couch letting him check my vitals because I honestly feared I was having some kind of heart attack... maybe I was just plumb worn out. SO strange. After a few days of weakness, I was back up and moving and about that time Jason felt like he was run over by a truck, so maybe it was a virus or bug of some kind. I am just glad that is behind us and we are all doing fine. It is in moments like those, that I feel the weight of being healthy and alive for the kids.

That first weekend back home, we had Maddox Alexander baptized in the contemporary service at our church. He was lifted up and surrounded by our loving church family. I snapped a few cute pictures of my little guy in what Sofie called his "girly dress". He really is a beautiful child of God and a blessing to our family. My heart overflows just watching him laugh. He is growing soo fast and is filled with such joy. He is 9 months and already all over the place. I love to hear him squeal and play with his siblings. He will be a great companion for Samuel. They are only 5 lbs apart as he now is nearly 23 lbs of chunky sweetness. It feels like twins to hold them but the weight is distributed differently and Sam is no longer babyish in facial appearance.

As for the rest of October, it has been a mix of running kids all over town to various birthdays and school functions. There was a Fifties Day at the school, of which my kids were dressed in outfits my mom lovingly made for them. Thank you, Nana! I loved attending Sofia's class activities and watching her with all her buddys.

I also had the privledge of attending Sofia's pumpkin patch field trip and one for Bella to the local orthodontist. Poor Bella says it is just not fair that Sofie has already had two long field trips while her grade did an hour at the dentist!

I will have to follow this post with some pictures from the fun we had this year making our own costumes for Halloween. Sam and I have another trip coming soon to Gainesville and hopefully we will know then when he will be getting the stimulator permanently placed. I just don't know what they will do until that can happen. It may mean returning to the GJ tube while we wait. I just keep praying we are making the right decisions.

This week, I am lifting prayers for the victims of Hurricane Sandy and all those working to help them. I am praying for a friend having surgery tomorrow and another recovering from one. I am praising several new babys and pregnancies and homecomings of loved ones and friends.

Ecclesiastes 3:1 (KJV)
To every thing there is a season, and a time to every purpose under the heaven.

Thursday, September 27, 2012

All The Right Reasons..

Here are the lyrics to a favorite song.. and I am listening to it and trying to clear my head of all the stress.. all the unclear things that lie ahead. It was a fav of ours before kids and somehow it still fits.

All the Right Reasons... by The Jayhawks

As I lay upon my bed, I begin dreaming
Of how it's gonna be the day that I am free
Once I settle like the dust upon the table
But then you came along
You helped me write this song

I don't know what day it is,
I can't recall the seasons
And I don't remember how we got this far
All I know is I'm loving you for all the right reasons
In my sky you'll always be my morning star

Like a tired bird flying high across the ocean
I was outside looking in
You made me live again
From the mountains to the prairies little babies
Figures fill their heads
Visions bathed in red

I don't know what day it is,
I can't recall the seasons
And I don't remember how we got this far
All I know is I'm loving you for all the right reasons
In my sky you'll always be my morning star

From the train in Manchester, England
Lightning fills the sky
As I watched you wave goodbye
From the mountains to the prairie little babies
Figures fill their heads
Visions bathed in red

I don't know what day it is,
I can't recall the seasons
And I don't remember how we got this far
All I know is I'm loving you for all the right reasons
In my sky you'll always be my morning star

We made a family trip a few weeks back down to Orlando and to give Samuel his first Disney experience before our season tickets expired and before he had to go in for a replacement of the external stimulator probes in the OR at Shands. This was our 3rd surgery and 2nd go at the stimulator to trial how it might improve his gut function and slow or stop the progression that could quickly lead to interventions we hoped to avoid down the line.

The week was incredibly special because Sam who so often has to stay at home and away from potential stressors like illness etc.. was getting to experience normal toddler activities and the joy and amazement that only Mickey seems to inspire at this age. :)

He was sooo happy, but more importantly his sisters were able to share this time with him and that was very special for me. Max, although only 7 months, also enjoyed the experience and was an incredibly happy little guy the entire trip. Our trip was made easier by the kind accomidations made by Disney to allow our stroller into closer access areas. We did not have to wait in long, hot lines and we had a few special encounters that would have been impossible with his health issues. We had to take each day in small spurts with long napping in the condo and short visits in the park, but it was overall, a fun adventure that I am most thankful we were able to give him. We were especially thankful to our in-laws for the use of the timeshare week at the condo as they have done for us in the past. It is nice to be in a place with a kitchen, that feels more like home when you are staying that long.

Our timing of the trip came in the second week of school, which was not ideal but as they have had to watch their brother whisked away so many times during emergancies and for surgeries they don't understand, I felt this was a worthy decision. We also might have had to go anyways since a hurricane was barreling our way and everyone here was boarding up their homes. It actually downgraded and barely brushed through so it was also a blessing to not return home to serious damage and needed repairs or devestation.

It was hard to transition from the parks to more Dr. appts and then admission to Shands for surgery but we packed up our crew and drove the two hours back to his children's hospital. He was again a brave little man for the ardous process of his IVs and getting through the no food preop requirements. It helped to have some of our past nurses that are so nurturing and understanding of little kids who experience this amount of poking and prodding. I was amazed that throughout the proceeding week he only experienced a few "downturn" periods as I call them and most were in the night with not tolerating feeds. We were in good shape for the procedure and luckily we had no negative outcomes. He rested alot afterwards and we prepared to head home within 24 hours of surgery. He was even tolerating a slow rate of formula on the pump with a plan to work up to trying boluses.

We did have one less exciting part to this trip. The pulmonologist came by and watched him as he slept. She immediately showed concern that we needed to be more aggressive with his respiratory needs. She identified signs of chronic CO2 with clubbing of fingernails and toes. His noisy breathing, which I thought was far far improved from what he did as a baby, was described to be PEEP breathing and indicated pressure problems with his gas exchange and difficulty opening the lower bronchi. We knew he had chronic atelectisis and with some tracheomalacia issues, I guess he is still having to work very hard to keep open his little airways. He sounds great during the day though unless he is sick and we have come to only need oxygen during and after illness and for rescue from his bradycardia events. Looks like she is questioning that now...

So we need yet another sleep study, this time more concentrated on breathing than on seizure concerns. He may need cpap at night and/ or full time oxygen by cannula during the day. Even knowing this might mean significant aid to his development and well being, I am concerned that he won't tolerate the mask on his face when he is well. He gets incredibly resistant and angry at anything near the face except during those periods where he is too weak to fight. I just don't know what it will mean for management in his home and around town. It is hard lugging so much medical equipment as it is... let alone those cannisters. I am always happy when I see his pulsox getting sats back up in mid 90s so I can disconnect it and give him normalcy after an illness.

We have been home now a few weeks with this external stimulator and it has been very interesting. I am thrilled to say he has allowed for bolus feedings several times a day again! YEAH! But I guess I had hoped he would be eating more by mouth. I know, one day at a time. It takes time. I am pleased though for the hours off the pump that this gastric stimulator has bought us. There have been times when it seemed to be difficult to tolerate the volume by bolus but overall we got up to 100 mls or 3 oz every three hours. Before he was doing 55 mls an hour by pump which meant all that time we had to lug it around with him. Now he uses the pump at night and we try bolusing in the day. If he starts getting gaggy or in pain or saying it hurts, we just back off and try later. Here are pics from a day Sam and his siblings enjoyed some time out on the sunny soccer field with friends.

I think one of the most significant benefits has actually have come in developmental strides. He is lifting feet off ground as he jogs on treadmill at PT and off the floor. He is talking more, repeating more words although the beginning of the word somewhat unclear. He is gaining weight better than ever and is 28lbs. He is now 30th % for weight. He is still not close to being on the growth curve for height but he is moving on his own curve. He is not jumping yet but is trying with exuberant effort. He understands counting to 3 and seems to understand sorting colors and toys. He loves to dance to music and is incredibly social. We have been trying to widen his horizons safely with small outings to the neighbors or on walks. We took him to a church picnic in a open outdoor setting and he loved kicking the soccer ball with his dad. Overall.. I am pleased. I am praying about this trip tomorrow.

We will be off to see his surgeon in the afternoon and then have a sleep study that night and then possibly his surgery with placement of the stimulator in his abdomen. I am feeling sick about it all but I know we want better quality of life for him, less pain and more motility. Those are all the best reasons. God be with us on this one.