Monday, February 10, 2014

Deep Breath

Sam has been trucking right along lately and just came through another battle with a stomach bug. It was pretty miserable around here for all of us but Sam actually made it through without too much trouble this time... thanks to lots of pedialyte and round the clock naps. Watching Samuel sleep is always difficult, with or without illness, and recently more discussion has surrounded the sleep breathing issues. What begins as a gentle tugging can at times escalate to a full out battle to breathe. I recall those first days of life when we peered over that bassinet in the Neo-natal Intensive Care Unit, meeting our 8 lb beautiful baby boy for the first time, listening to people defining obscure terms like "retracting" and "respiratory distress". It was all foreign to us then. We watched Sam struggle for every breath and noone could explain the reason. We still wish we could fully understand his breathing issues, but particularly those connected with sleep.

It is funny how we take breathing for granted.. it is supposed to be automatic but when you consider that the first thing we have to try to do during stressful situations is to take a moment to breathe in and out.. slow and evenly. To take one breath at a time. There is some feeling of control gained through measured breathing. The rise and fall of our chests seems rhythmic most of the time but once it becomes erratic it can rule us entirely. We can't do too many other things if we are having to focus on our breathing. Sam's breathing has a rhythm all its own and I guess he is used to it but it certainly takes its toll on us.

For me, breathing is at the heart of stress control. These days, I slowly breathe in the unexpected. The joys, the laughter and the saving graces. The concerns and disappointments. I feel cleansed by inhaling good news, even when I know there may be bad to follow. I try to avoid breathing in the smoke of negative thinking and denial. I exhale the worries, the things I can't control.. material and financial barriers. In my opinion, respiration really rules the kingdom and it seems our boy has an enemy at his gates.

Samuel's pulmonologist often explains that he exhibits clear signs of an abnormal, pathological imbalance of aerobic (with oxygen) and anaerobic (without oxygen) respiration. I personally had only heard the term anaerobic in association with those tiny organisms living at the depths of the ocean floor but it seems we all utilize both forms to some degree. Really these fancy words were meant to explain that Sam's cells rely too heavily on creating energy that does not mainly originate from oxygen molecules. His mitochondrial specialists think they have pinpointed defects in the oxphos respiratory chain which show that he does not convert oxygen and food properly into ATP.. which (put too simply) is how we make the fuel or energy our organs need to survive.

Sam depends too heavily on anaerobic metabolism and the negative of this alternative path is the toxic waste it creates and the effect that has on his body. We all use both forms of respiration but an example of relying too heavily on anaerobic would be to see the metabolic threshold breached by extreme athletes as they overload their aerobic ability to fuel muscles from mainly oxygen intake. Imagine that in the last leg of the race where the Olympic track runner has pushed forth to cross the finish line with almost impossible strength but then collapses after that final leg. They likely shifted beyond a normal amount of aerobic and anerobic respiration to depend too heavily on the anaerobic ability to meet the demand. The incredible fatigue they experience after this expenditure is natural and they will recover but they could not have existed long term on that method alone due to the damage it would place on their organs. It seems the cellular biproducts of imbalanced respiration sets off a chain reaction that ends in cell injury and cell death for kids with Mito. What we all think is so simple, the so-called building blocks of life, providing the cells with the nutrients while breathing in and breathing out... well, it is really so much more complex. I can study this stuff all day long, but it is in those late hours of the evening when I watch my sweet boy dreaming that I know how very complicated this mechanism is for him.

Sam is due another sleep study in the coming months. When Samuel breathes there is a unique "peeping" sound that is heard upon expiration (when he breathes out the carbon dioxide). They tell me this is due to his problems with proper gas exchange and specifically a long held suspician that his symptoms indicate Central Hypoventilation. Central Hypoventilation is a problem originating from the autonomic nervous center of the brain effecting his respiratory function which is where many of his other issues also originate. They call the collection of those issues Dysautonomia. Central Hypoventilation is usually caused by a Phox2B genetic defect (which Samuel does not have) or by damage to the brain stem or some other unknown source and we don't know it's relationship to mitochondrial dysfunction. We do fear the other related conditions but can't take in far reaching concerns as we still don't have the basics fully worked out. We know that one day when do understand how all the pieces fit together, the picture may not be one we want to see. Not knowing all the answers makes treatment more difficult. In the last 6 months we have had him on an additional med, Topomax, that is sometimes used for seizures but is the newest thing they are trying for apnea control. I don't think it has helped and would rather drop this one off our list but we await the nexr study for clues.

We continue the exhausting needle-in-the-haystack search for answers and going to Houston has seemed to breathe new life into this search. The team there wants us to undergo a new break-through set of testing called whole exome sequencing which might hold all the answers. We soon found this out of reach as Samuel's insurance swiftly denied the opportunity after we had provided Baylor with all of our samples for testing. It is a sad thing when the answers to what is plagueing your child and how to help him comes down to the cost of $15,000 testing that some analyst over at the insurance company has decided is not approved.. ironic since just a few days of his many trips to the ICU have cost far more than this test. Maybe we would gain answers that would help us limit such hospitalizations in the future or maybe answers that would help other children one day. So we wait and hope that maybe as this testing becomes commonplace as is expected, it will become feasible to attain for him.

For now, when he sleeps, over about 13-15 hours a day, we moniter his breathing and oxygen levels closely, raising his oxygen liter levels from 1 to 2 with signs of peeping or distress. We usually see improvements with the supplemental oxygen but it is hard to know if we are fully covering his needs. He expires his breaths with a tension that seems so unnatural, like each is held back by an invisible net. We have discussed BiPap with the pulmonologist and yesterday his peditrician described that he had seen kids years ago with similar conditions find relief in an iron lung. I hope further mechanical ventilation is not necessary and certainly hoping more invasive action does not become necessary in his future. I will never forget his surgeon telling me when he was 2 months of age that they were not certain they would be able to extubate him safely and were considering sending him home with a trache and mechanical ventilation.

Those were particularly hard days that I never want to revisit. This picture above is a difficult reminder of that very dark day when Sam was coded multiple times and we really had no idea if he would ever breathe on his own again. I thank God every day for blessings of overcoming dark days. I am thankful that God seems to keep breathing new stamina and hope into us each day. For some reason I think we have never needed it more than we do right now, which is ironic since Sam is more stable right now.

I pray for fresh breath of optimism and hope for all the people I know who need it and for strength for those experiencing very difficult trials. I keep thinking of a family I know who have had several losses since Christmas. My good friend's mother is bravely fighting cancer and yesterday was her birthday. I hope this next year is far better than the last few months she has endured. I am thankful for the blessing that we all are well now and hopeful that this season of illness passes as we know so many fighting respiratory and flu bugs. I am also praying my thanks for the gifts God has blessed me with and tonight that came in the form of a sweet boy laying on my chest, long eye lashes against his soft cheeks.. breathing in and out. Peace on Earth.. even if his breathing may not appear so peaceful.

Tuesday, January 28, 2014

Forces of Nature

Tonight our area braced in anticipation of a night forcasted for snow.. or at least some "freezing rain". We are such Floridians here... huddled under our blankets and wondering at how people up north ever survive the winter. Though not snow or sleet, we brave other elements.

Winter weather always brings me down. It seems like the cold and damp can permeate even the deepest corners of the mind. I am so thankful that a good cuddle on the coach with my baby boys, a good book and some hot chocolate can do wonders for a case of the winter doldrums.

We can hardly control the forces of nature. There are days when it feels like the forecast will shut us down, close our doors and snuff out the flame that was keeping us warm. I had one of those days today but as Sofie and Bella often are heard singing in our playroom from their favorite movie, Annie.. The sun will come out tomorrow.

Sunday, January 26, 2014

Game of Life

Yesterday the girls had an invitation to attend a friend's birthday party. The morning was a flurry of activity in our home. Max, who just turned 2 on Friday, was busy showing me just what a full-fledged 2 year old he could be. My nerves were wearing thin from not enough sleep on top of a head cold. The house was a wreck from a few too many days of falling behind with household flow since returning to work full time. It seems that both parents working coupled with 4 kids can mean certain things fall between the cracks, the floorboards and under a pile of dishes. Additionally our fancy 3 year old dryer has broken down for yet the second time and even sparked a fire. After paying almost $300 for the replaced heating element just last year, we have been trying to get a hold of the appliance repair guy to come back out since it is supposed to be under warranty but have had no such luck and we found out we are not the first ones to have this issue. (I sure wish I had seen the BBB ranking of F on the Honest Appliance Repair company before using him) Anyhow.. I have fallen way behind on laundry and although I am going to the laundromat constantly with heaps of wet clothes, it has hardly been an efficient exercise.

To top the pile that day, Sam was having tummy trouble since waking and I wrestled to get his medications pushed, his pump loaded and his oxygen on before he reached the break down phase of an overdue nap. His naps are like 5 hours long and he really doesn't do well overall if they are shortened. Sam had managed to transition to a twin bed from the crib this week without a single hitch and so for that, I am quite thankful.

Meanwhile, Jason has been spending the last few weekends helping his parents close out his grandfather's home as they transitioned him to a nursing home. This has meant weekends are spread a bit thin around here. With pretty significant car issues meaning we are making due with one car that itself is not in good shape at all with a wedged-on door and a singing belt, I was trying to decide how to share the car with Jason and still get the girls to their function which I knew they really wanted to attend.

We have been a bit like hermit crabs lately in trying to help Sam heal and recover from the surgeries, while avoiding more illness but unsuccessfully meeting that goal as each week some kind of bug hits each of us anyway. While focusing on the boys, I lost track of the time needed to mobilize the girls for their party and when I finally emerged, hoping to find them dressed and ready for the party, instead I found two little girls still in their PJs playing at 'The Game of Life' in our playroom.

Oh, how I felt like I had just played and lost at that same game! All I could do at that point was be OK with a little party tardiness. It seemed so ironic. Jobs, marriages, health, income, businesses, real estate, babies, insurance, crisis, foreclosure and the lottery of it all. Somehow the little pink and blue pegs that fit in that little plastic car don't do justice to what comprises Real Life. Funny, that I actually remember as a kid wondering what would happen to the player who had two many baby pegs to fit in their car...

Where are the backpacks, strollers, oxygen tanks, diaper bags, briefcases and the piles and piles of laundry? Where are the hospitals and therapists and countless appointments, homebound teachers, medical supplies and tubing coming out my ears, and all those binders of nursing notes? Do the parent pegs stay up wondering if the baby pegs will all keep breathing while they sleep? Where are the homemade lunches, signed school planners, reading packets and spelling lists? Where are the grocery store temper tantrums and where is the door on the side of the car that fell off or the burglar who came in to steal your iPad but really stole your sense of personal security?

Dare I ask... but where is Jesus on that board? Maybe there should be a chance card in the pack that reads, 'Congratulations! You were saved by Jesus, despite the chaos of this life!'

So.. after a long day, I am going to focus on how to stay the course. I want to purge the excess, streamline our routine and pray for best solutions to difficult challenges. I am praying for those I know who are also experiencing difficult times.. For a choir friend who has been in a lot of pain; our Sr. Pastor and his wife, who each lost a parent this month; for my friend's mom bravely fighting breast cancer; for another friend about to transition her kids into a daycare and for a fellow Mito family preparing to say goodbye to their little girl.

Saturday, January 11, 2014

Fighting a Bug

Sam has been fighting a bad stomach bug this week. Thankfully he has teetered through it without a total crash and I expect in a few days he will be back to his usual self. My biggest concern was over the fact that he just had the cardiac procedures and implanted devices which I know can put him at some additional risk of endocarditus and other concerns. As for these illnesses, we have really learned so much about managing his fluids and keeping on top of his oxygen needs. We have worked to keep him on his pedialyte even when he could not tolerate his formula. As we approached the end of the week, his sister Sofie got wind of the bug and then passed it on to Max as well. So, that has meant lots of fun around here for us all.

This week we also welcomed a few new nurses, so those adjustments while dealing with the bug have been a little tricky, but they seem to be adjusting well. We said good bye to a nurse we have had for over 2 years as she found a job in a clinical setting. She has watched Sam grow from infant to boy and has been a part of all the work he has done overcoming difficult challenges. She has actively helped rescue him from crisis and been there for him emotionally during many painful days. Sammy will miss his Miss 'tina as he calls Nurse Cristina.

School is back in session now and I am busy working full time at the house. I have really enjoyed diving into this new chapter with a new company and I wish this was not a temporary post. I have found I almost never leave the house though. So, I will have to find reasons to break away and get out a bit. There is just always plenty of work to keep me tied to the desk or else caring for the kids. From the messages and emails, I think people are beginning to wonder about whether I fell down a dark well! I really do need to make an effort to get out more for fresh air and some down time with friends. For now, I am just thankful for the work and in light of this recent bug, I am thankful Sam has avoided another hospitalization.

Tuesday, December 31, 2013

Finding Pace and Peace in the New Year

The countdown to 2014 is already in progress and I feel some excitement about celebrating tonight with my sweet husband and kiddos. It will likely be just pizza and monopoly for us.. long gone are our days of champagne and a dance floor, but all the same..there is much ahead and much behind to celebrate.

I have failed to blog in the last two months. We spent Thanksgiving in Houston as Samuel faced his pacemaker placement, bubble study and the possible ASD closure. I was glad that Jason, the girls and Max were all able to be with us for this trip. We all had a day together in Houston after Sam's preop appts and I felt this overwhelming joy.. when I watch them playing together, I can't help but know they may not always have this. They may not always have each other. It is an impossibly hard part of our reality but somehow when they are all together and bonding, I forget that ugly thought. We went to the natural science museum before we settled in at the RMH to help Sam rest up for the big day ahead. Sam had a rough night (as always on trips) but what was the hardest part was how cold the weather was that week. Getting to the hospital at the crack of dawn the next morning is no easy feat with babies, equipment in frozen, rainy weather. This Florida girl was shocked by the 20 degree front that came through Texas. I know, my snowbird relatives are laughing at me.. but we really don't even know how to dress for that kind of cold down here!

So Sammy was supposed to go in as outpatient but we pretty much knew that he would become inpatient just based on how he likes to handle stress and anesthesia every time. Luckily, the team at this hospital really knows how to handle Mito kids and their anesthesia protocols are fine-tuned with good management. Sam was so precious that morning as I sent him off to the Cath Lab. He kissed me hard and asked me, "Mommy, can you go in there with me?" and all I could say to him was that God would be in there with him. This just never gets any easier.

So Jason took the kids home as they were unruly and all over the waiting room. I sat there waiting for word and was so thankful when one by one each of the physicians and nurses involved called or stepped out with status. An hour or two into the procedures we found out news I was not expecting. We were having a bubble study done since they wanted to make sure the small hole (PFO/ ASD) we knew he had between two chambers of his heart was not going to be a problem for passing clots etc. So they ran bubbles up through one femoral line while a intra-cardiac echo that was in through the other femoral line could monitor the physiology and function of his heart. Then a cerebral Doppler was placed on his head that monitored the circulatory flow through to his brain. The bubbles that they inserted went across the ASD and apparently it indicated a severe shunt in the right to left direction which is in reverse of the usual flow. This meant, that the closure was indeed indicated. The Doppler on his brain actually also showed the bubbles went directly there as well which for me raised all kinds of questions and concerns. We are very lucky this little man has never stroked out. I sure hope the times when one side of his face was doing seizure-like chewing motions in the past.. hope that what we were seeing was not part of this issue. I am just not sure and don't really have the medical knowledge to connect all the dots that emotionally you worry about when you get this kind of news.

At least we knew the ASD closure was necessary, as we hated to have one more thing implanted in his little body. They put in a Helix Device to close the hole which is like opening a small umbrella on each side of the hole. Then they proceeded to go in through his shoulder to place the pacemaker lead to the right ventricle and the generator under the muscle. The cardiologist was wonderful as well as the anesthesiologist. They came out and said he was having a bit more trouble being extubated right-away as his airway is incredibly scarred and narrow. He had a seizure during the 6 hours as well. When he came out, the biggest issue was keeping him still as he was in a lot of pain and wanted to move his legs which needed to remain still for the next 6-8 hours. This was incredibly difficult to manage as he is only 3, after all and wants to move when he wants to move. Usually after his surgeries, he is far worse with his breathing and I was very happy with anesthesia's choices this time.. far less respiratory decline. I felt he was better overall, but the nurses in the IMC were more concerned.

He spent the next few days in Intermediate Care Unit working back up to his feedings. His follow up echos and EKGs looked good and we were able to go home the weekend following Thanksgiving. We had a semi-Thanksgiving dinner as a family in the RMH and met some really amazing families. It is incredible experience to be giving thanks over a donated meal, prepared by such loving hands in that setting where you know that every family is more thankful for each day with their child than most people will ever appreciate. One of the families we met while there in October who had a child awaiting a lung transplant was sitting a table away with their daughter in their lap. 18 months old and she was on ECMO life support when we last saw them. Today, she is just an 18 month old toddler fussing over what she wants to eat off her plate. She got her new lungs 6 weeks before and has a brighter future ahead. Sam played in the playroom with an older child who had also just had a lung transplant. She approached him because she told him she knew what it was like to need oxygen like he wears.. but how happy she was to not need it anymore. What a blessing.. purely thankful for the ability to breathe without help.

Just to keep our life adventurous, on the way out of Houston, the door to our van fell off the car... like the straw that broke the camels back. After so much stress that week, I felt it was almost just too much and I was going to fall apart. But I know it is better to laugh than lose it in those horrid moments and I pray things will one day be easier. Things have been pretty tough for us financially due to all Sam's needs and the demands of the trips, stress etc.. and then when normal stress comes along like our dryer that doesn't work or grandparents who are moving into nursing homes for dementia, you sometimes find that there is just already a pile to throw it on and it feels like too big a load.

Since his surgery, we returned home for a month of recovery and tried desperately to keep Sam from using his left arm and shoulder. This was nearly impossible and he experienced alot of pain each time he would forget to reserve his movements. His incision has healed well and I am especially thankful for this as we had been home a few days and turned to find he had ripped off his own steri strip dressing which was slightly angry and bleeding. His cath incision points are still very visible but we had no issues there either. He had a strange cough when we first got home but it finally disappeared. The biggest issue was his palor. He has had striking pale periods since the surgery, at times just generally so and others more marked and scary looking. His Dr. ran a CBC which showed somewhat low hematocrit, hemoglobin, red blood cell counts and MPV counts with high leukocyte counts but I guess for him and having just had the surgery, there was no concern for now. I think maybe his body could even just be adjusting to the circulation changes of closing the shunt. I can't imagine it was good that for all that time he has been mixing oxygenated and unoxygenated blood and shunting it the reverse direction... so I am glad the ASD is closed.

The last few months have been so much to process emotionally... We have long needed additional income to help with all the medical costs and I decided to return to working again full time. Probably one of the biggest hits we took when Sam arrived was my inability to be in an office doing an 8-6 job.. pretty difficult when you are always in the ICU and managing not only our kids but all of Sam's medical stuff, so I began doing communications work for our law office, which was helpful but I really needed a position with traditional benefits and pay. So.. I just made a shift this month and am working for a great organization from the house. Although this post is a temporary one, I hope to find a permanent one in the near future. It just has to be flexible enough that I am not fired when Sam lands in the hospital. Haha.. I am praying a lot about this change... more than anything praying it adds wider horizons to my outlook on life that admittedly has felt some big dips.. I really try so hard to have optimism for our future and Sam's.. it helps to find a place to pour out more of myself. I have to be honest in 2014 about tough realities that are hitting us pretty hard. I am thankful that we have a business we fight for and that we have income, but I am praying for our costs to become within reach of that, for more work, for more clients, for a fighting chance to save our son and give him what he needs. I am asking for the right answers to finding a manageable pace in this difficult life... to being able to be a balanced person that refuses to let the emotions or stress of life take over.

2014 is just a few hours from now and I think my resolution will be to find the best pace for peace in my daily life. If those little pacer generators that sit in my son's body are helping him to find a fine, safe balance, a rhythm for living a better and longer life, well maybe I can emulate this technology in my own life with some positive change.
Happy 2014 to all of you and yours! May you find peace and joy in the year ahead.

Tuesday, November 12, 2013


A Spy Kid, a Sofia-the-First Princess, a Super Grover and an Elmo all spilled out of our home this year on Halloween night. We visited with family and then set out to trick and treat with some friends. I was thankful there weren't many scary characters trolling the streets this year.

I am glad that my kids really don't associate Halloween with ghosts and goblins. They just know it as a perfect chance to wear a mask and be someone else for fun.. with no dark hauntings.

Since that night, I had a painful awakening that perhaps I am the one who is haunted. I had an unexpected encounter that forced me to realize that maybe some ghosts I thought were buried are still able to climb out of their graves.

I was walking along at this local function, watching my older children as they enjoyed the fall festivities when I realized the Obgyn who delivered Samuel was standing just 4 feet away from me. She had moved away quite some time ago and I guess she has returned. It is odd how quickly I could tell it was her since it has been almost 4 years and although she is a 43 year old woman, she was wearing platform shoes and had her hair in pig tail braids... but I knew it was her. All of a sudden I was transported back to a horrificly painful time in my life and it all came rushing over me. I felt physically ill.

Quite some time ago and after many many months of inner anguish, I thought I had laid to rest my anger for this person... her offenses, her lies and the insensitive way she treated me. I laid it all to rest because I realized it was all eating me alive and causing more pain than we were already facing. I forgave her because I knew God asked that of me. I just wish He would show me how to live with her in my vicinity now. I am not prepared for this hurdle.

Over those first couple years when we were in such horrible pain over the outlook they gave us for Sam's life, I worked hard to avoid thoughts of her and all the moments and decisions before his birth. It was harder to tuck away all the questions we were left to ask. I am not sure if those will ever be answered. I just know now we have chosen to live in the moment and not let the dark stuff pull us down. It doesn't help him or our family. I know that if I let it, it would tear apart my life with all that anquish. I have to let it go.. and I thought I had but maybe I am actually still haunted by it when presented in close proximity.

I couldn't make eye contact that day but if I could have approached her.. what would I say? I guess I need to keep working on locking this ghost in her closet.. but one thing is for sure. It will be awfully more difficult now. I am praying for strength against my fears of seeing this person regularly (and apparently it will be frequent since her kids go to the same school). I am asking God to help me again to not let my memories bring resentment back. She is just a person who crossed my life path.

Jason and I were sick this weekend and so I am hoping for more wellness for the week ahead. My sister's daughter celebrated a birthday and I lift her up for a most special year of growth ahead. Sending up prayers about Jason's grandfather who is in late dementia stages and has been in the hospital and now transitioned to a nursing home. I have a friend's son getting through cancer treatments and someone very special to me who just found out they have a fight with it ahead. We are preparing mentally for Sam's surgery coming up in a few weeks. I am praying for his good health in weeks ahead and that all planning logistics go smoothly.

We found out insurance won't pay for a $15,000 Whole Exom Sequencing testing that is at the forefront of available diagnostics for kids like Sam and I will post about that another day.

Wednesday, November 6, 2013

In a split second

I wrote the post below a few weeks ago but delayed in hitting publish due to the vulnerable nature of what happened to our little guy at home. It is a really long, emotional and rambling post.

October 3, 2013

Seconds seem like such inconsequential increments... until they feel like they are all you have.

Sitting in Sam's Pediatric ICU room I can hear the second hand ticking on the wall in an entirely too quiet ward that I know is inhabited by too many other parents passing the time just as I am. It is this all too familiar frozen, nervous state of worry.. the all-consuming fear for your child's life as you cling to the faith you have that God is in control no matter what happens.. a feeling I hope most parents never have to experience.

I know there are parents here desperately agonizing over mere seconds...
the seconds before that last smile..
before that last kiss..
before that wave of goodbye at the back door..
before the last lullaby.. or maybe even
before that last angry word.... all before life seemed to hang in the balance.

They are milling over their own responce time in seconds to a horrific set of events or maybe thanking God for every second they have left by someone's side during a long battle with illness. I know the worry of discerning what the meaning was of those few second pauses the Dr. took before giving a diagnosis or new treatment plan. There are people at the end of this hallway that are counting seconds till a surgeon comes out to share a good report. Sitting across from those people rejoicing in that news is maybe someone's husband or wife keenly aware that far too many seconds have past the projected time alloted for a simple procedure..and now they are worrying something has gone terribly wrong. All seems to hang in the balance over just a matter of seconds.

Our lives pass by, measured in seconds that add up to minutes and hours, days and months, ultimately the years that culminate in a lifetime... but it all starts and ends in mere seconds.

In sharing the events of the other night, the most difficult part is to recall how deeply we felt the seconds that mattered were escaping us. After facing quite honestly many, many life threatening codes and encounters for Sammy over the last 3 years, I have never in our home felt such assurance in a split second that he was gone forever. And by God's grace.. after over 300 seconds.. he was back to us. This happened twice in one night and yet God blessed us with his revival. I will try to share what happened two nights ago in our home that landed us here.. not only here in the hospital but here in this vulnerable state of concern and now waiting for his surgery.

That night..

I guess that night we were just winding down from a typical busy day and had sent the girls to bed, but they kept peeking back out for water or to pick a new book. Samuel seemed unscathed from a day of therapy, homebound school and a neurology follow-up an hour away. I fed the family dinner and we sent the girls and Max off to bed. It was a short while later that we gave Sam one of his many medications which always makes him extremely drowsy. He wandered around half awake as is usual for him. He was not on his oxygen but we give him breaks during the day and he is constantly on while sleeping. He always complains of his tummy before falling asleep and that night was no different. Jason held him and then he climbed into his little wooden antique desk chair. It is not uncommon at night for him to fall asleep on the living room floor or in his little feeding chair as his meds hit him fast and he often becomes a little irrational about being touched or even looked at. He likes to drift off on his own without being bothered.

We then usually move him back to his room to hook him up to his feeding pump, monitors, oxygen etc.. if he is not already on them. While talking to Jason about the day, I looked over in Sam's direction and noticed his head resting on the surface of his desk. Most mothers will tell you there is a recognizable feeling that comes over a mom when something is not right with their child. Like a 6th sense, I was suddenly struck with that unsettling feeling. I just don't know how long he was in this position before I realized that he seemed entirely too still. It makes me sick thinking about the time that passed.

Jason was closer to him and I heard myself ask, "Is he ok? Is he ok?" and then began to feel more and more anxious after he shrugged back and replied, "Sure, he's just sleeping." "Are you sure?" I asked. I don't know why I somehow remained frozen in time and did not move in those initial moments, those precious seconds and minutes. Jason realized my concern and reached over to touch him. He fell over like a bag of rice and it became clear that he was unconscious and not breathing. His color was horrible, greyed out and then blue in some areas. He was incredibly cold to the touch and his body had absolutely no tone. I could not find a pulse. We quickly began doing all that we know to do to save him and using all that we have for aid in our home.. which is more than most but of course nothing goes as smoothly as you hope. Nothing is right where you expect to find it and the situation becomes chaotic and highly stressful. At first we felt our efforts were futile... I watched my husband in horrible anguish that this could not be the last time we saw our son alive. After an incredibly long period, Sam miraculously regained oxygenation while bagging him with the ambu and we began to see some color returning and safer vital signs.

This was the longest period I have ever witnessed our son unresponsive and without signs of life while in our home. This statement sounds strange as I type the words because unfortunately we have shaved losing him too many times before...brushed up on what it looks like to lose a child and been blessed by God's grace. I wish I could erase all the ugly things we have seen in the last 3 years. This is a lonely and conflicting part of our life. I hate to admit it but there are moments I wish other people would have even the slightest idea.. because they would never ever take a breath from their child for granted again. That sounds horrible because most of the time, I feel this immense need to do the opposite.. to shield people from knowing too much. I often pray a prayer of thanks to God that Jason has been spared witnessing some of the more traumatic events just by the pure logistics of being at his office or home with the kids when we are away in the hospital. Here I am right now though feeling so thankful that we were together during this experience because I am not sure I would have made it through without losing it entirely and now it is something we can help eachother process. I am so blessed that God chose Jason to walk with me in this life. He is always so calm and collected, measured and wise. We somehow have managed through circumstances that would break most couples.

So... amidst all the chaos of that first event, we managed to reach 911 and stay on the line with dispatch until the first responders arrived. Sam had begun to breathe and regain color after about 5 minutes, but he appeared to be experiencing a seizure after this period. His eyes would not connect and his hand tremored with a twitch of his eyes. When I went to the door to let the responders in, I saw my daughters were curled up under the dining room table looking really scared. That is a haunting image for any mother. I took them aside knowing Jason was by Sam's side with EMS and I tried to get them back in bed and explain that Sammy was okay and that we just needed to cover him with prayer. They laid down to sleep not knowing we would be gone when they woke up.

Once he recovered from that first arrest, he was in deep sleep which seemed a postictal nature of possible seizures which I suspected where hypoxic in nature. We made calls to our neurologist and made the decision with EMS to hold off on an ambulance transfer. He was breathing and had regained a stable heart rate. We could have gone I guess but with his history, we hoped it was an isolated event and that the Dr. would take over about our next move. The local ER where the ambulance would take us does not instill much confidence in complex pediatric care management.. and the EMS team agreed. The neurologist nurse practitioner advised we go to the children's hospital that is 1 hour away if we had any other concerning changes in the night.

Soon after the first event and once EMS left, Sam's breathing became labored and noisier than usual. Jason was sitting with him and we awaited the arrival of the night nurse, all the while debating if he was stable enough to travel by car to the hospital. The night nurse arrived and just as we gave her a report of the situation, Samuel began to lock into a tonic convulsion and turned entirely blue. To us this event seemed more like it originated as a seizure. He was not breathing and again things were really bad. The nurse responded and was working to get his airway open with the ambu bag, while trying to get him to the floor so his head would be down for oxygen to the brain. He was unresponsive for several minutes and when his body did relax, he went grey and limp, he was not connecting or breathing on his own. The nurse's responce was far more aggressive on his small frame than I have ever seen but I was glad she was quick to take action. There is no way to tell another parent what it is to see other people trying to save your child. There is no way to describe seeing no life in responce to those measures.. and there are no words for the trembling, grateful feeling of seeing color fill their cheeks, eyes open at some point and looking back at you.

Sam was there in the floor when the same EMS team returned. After regaining consciousness, he was prepped for a transfer on the stretcher. I will forever be thankful for this one Paramedic who helped secure a ride directly over to the children's hospital, over an hour away without a stop at the closer ER, which is far less prepared for Sam. After all the stress, it was a stable trip with no other events.

Once there I braced as I realized they were looking to see if he showed signs of brain damage. Knowing these events might be just the beginning of more events or a symptom of some other illness, I expected a more proactive approach from the ER team. But.. as seems to be the case here locally, you find yourself telling them what you know might be associated with this situation from the past and what concerns you have for what might be yet to come as they blankly seem to wonder what your child's condition really is and whether they can google it. When Sam has had Bradycardic events in the past, they have typically proceeded illness or some stress factor so that is our focus after big events..stopping the worsening impact of the stress factor.

In the ER we delt with a long admission process, a few very kind residents, one less than understanding physician and one or two very caring nurses. We were left for long stretches alone in a dead silent ER ward. Sam was exhausted but breathing was far improved. I hoped that his detached mental mode was from the energy and stress of the events. Several teams attempted and could never get an IV placed and finally they decided to just get fluids in by way of his feeding pump because he was getting dehydrated at this point. The neurologist came by and ordered a continuous EEG with telemetry the next morning. Sam's mental state had been overall withdrawn but responsive. He was just very tired and we were hoping for no further events or signs of trouble. His breathing improved but his GI is still slow and much of his feed is leaking from the stoma.

One of the residents assigned to Sam was very attentive and kind. Going through all Sam's history, reliving it in a sense, can be very difficult with every new Dr assigned to him when these situations arise. I am thankful for all the caring hands in this hospital, but these days I can't help but feel they are often going through the motions. Maybe they have already decided his prognosis or maybe they are just not that familiar with the delicate balance that controls his health and how to best help him. I hate having to explain his history over and over and I grow tired of explaining that this stuff doesn't just happen out of the blue with him.. there is almost always a taxing stimuli. I live to control that part.. but why does it sometimes feel like they expect me to find the answers myself or just follow whatever course naturally occurs. Standing in the ER with our critical care RN night nurse explaining the events of the night to one of the physicians we encountered, I was left so upset when his first responce was to ask, "What course of treatment are you expecting us to follow at this point tonight?" I was left flabbergasted and deflated. I was glad for our RN who immediately looked at him and back to me and sent the clear message that we needed to know he was stable and safe... by GOLLY.. he had two extremely involved life threatening episodes that night! I only rant here because this is like turning the knife in an already extremely painful situation. Clearly we don't expect them to cure him.. is that what his Dr. was thinking we expected? After 20 some odd hospitalizations.. believe me.. even without a perfect understanding of his diagnosis, we know that management of his condition is the best we can expect for now.

Labs came back with elevated CPK, white blood cells and wonky neutraphils. I think all can be attributed to the stress of the events themselves but he has run some fevers as well while here.. so there could be an underlying infection somewhere I guess. I feel so alone in this place. I was so glad to see Jason able to break away and drive over today. He carries the weight of the world on those big shoulders. He is an incredible father who just wants to make it all okay for Sam, to fix this for his baby boy and remove the worry from his wife and children.

We know we will leave here without all the answers because.. as usual.. there are few answers to be had and we will just hope Sam's upcoming pacemaker placement makes the difference. I really should recap the last few months when we are back home.. since we have made some big changes in the direction of Sam's care.

Oct. 28, 2013
To recap since coming home and some big changes in last month or two..

Sam has excellent physicians over the last 3 years but unfortunately no real cohesive team approach. We struggle with having communication among the different specialties and so we approached a Mitochondrial Center through the University of Texas that provides a clinical multi-disciplinary approach. We went to Houston to meet with Dr. Koenig, of whom we have heard glowing reports from other patients. It is not that we are closing the door on the Atlanta Mitochondrial specialist who diagnosed Sam, but we are trying to see whether there are better ways to handle communication from a team approach. Already we have had more feedback from the new team and further communications to pass on to our pediatrician. I guess I will always need the wonderful folks at our tertiary care hospital 5 hours away and then maybe only a few times a year travel to the bigger centers for opinions of the mito-oriented team. Already we saw benefits when they checked in on us as this latest situation arose and are helping us as we prepare for the upcoming surgery.

As an aside, I realize we would be better off living in one of these big cities or places with close children's hospitals, but we would lose our family, friends, church, community, not to mention little details like jobs etc..

Overall, we have had a very busy few weeks across every part of our lives since Sam came home. By day 2 in the hospital Sam was doing better connecting and talking with me. He was not feeling well from a GI stand point and had some fever but we finished the EEG and were discharged home with the plan to get the pacemaker. The neurologist is pretty convinced again that these originate as bradycardia. He did not see much of the spike wave discharges on the EEG that he expected to see.. just a short period at the conclusion of the study. There was some slowing in portions of the brain seen in the past but nothing significant. He had no further cardiac issues while there and so we are left to wonder what the insult was that night that threw him over the edge. We have tried to keep things calm around here since then.. as calm as life can be for a family of 6.

Soon after the events, we made a trip back to Gainesville and stayed with our friends while Sam had his follow-up appts with ENT, Pulmonology, Immmunology and Dental. The first night was a little rough for Sam after the long drive. A slow GI after a long drive makes for discomfort and crying in the night for Sammy. I have a hard time knowing how to handle his pain during circumstances where we are away, in a hotel or someone's home. I know my tension over his crying in another person's home probably don't help and obviously some of the problems are likely linked to being out of his environment. This happens every time and the odd part is that it is often not during the drive lately but actually hours later in the evening.

One difficult part of this trip was meeting with the immunologist for the first time. It was good to have someone assessing his immuno status and considering how safe it will be someday for him to start getting into more open environments, like a classroom. It is difficult to realize what interventions may be necessary to make that a viable option for him. His little buddy Nate is getting what they call Sub Q infusions of donor immunoglobulins weekly. Some kids get similar support through IVIG in the hospital once a month. Even though I know it has helped Nate so much, I fear needing to take additional measures, but I know we will get all the facts and make decisions based on the opinions of those who know what is best. The immunologist pulled like 12 viles of blood on Sam for testing and then revaccinated him for HIB, Pneumocacol and Flu. The pulmonologist did mention that his liver was a little enlarged (something that has come up on and off before) and I am hoping that is just because of the stress of those recent events and not something we should be concerned about.

We drove home and Sam began having some low grade fevers, congestion and general malaise. He stayed this way for over a week. The pediatrician could find no real culprit and I honestly think the vaccines just threw him off his A game.. or maybe his body is still healing from the trauma of the events a few weeks prior. We are just letting him coast for now. He had to meet up with his surgeon a week later in Tallahassee and he was still not feeling very well. His surgeon increased his stimulator settings and we made it home in time for dinner.

During the course of these few weeks, the Houston electrophysiologist thought we might need to consider having the pacemaker surgery moved up but in the end, we have kept our original dates and I feel good about this, despite my nerves of some crazy event happening in these last couple weeks. I need him to be well rested and for our family to be ready to support him through this hurdle.

Amidst all the stress of the last month or two I was really blessed to have a few days with my highschool girlfriends at a beach condo reunion right here in the area. I can't even put into words what this meant for me. It really helped get my mind off life's difficult stuff and I laughed harder than I have in years. I am blessed by some very loving friends and I am so glad we have stayed in touch.

Sam will have a pacemaker implanted in a few weeks in Houston. At the same time, the electrophysiologist has advised that he do a bubble study to assess the nature of a small PFO or ASD (small hole between two chambers of the heart) that has a history of shunting blood, basically mixing oxygenated and unoxygenated blood. If it is still doing this shunting, he will close it surgically. This small hole is not uncommon in even healthy babies but could be contributing to fatigue and desaturation problems. When you have cardio pulmonary issues like Sam's, a small thing can be more complex, I guess. The pacemaker surgery was scheduled a few weeks before these latest life threatening events but now I could not be more convinced of its necessity. I hate the idea of another implanted intervention device but anything that keeps our little man growing and enjoying life is important.

I know the support measures we have had to take have contributed so greatly to his well being but I will never be comfortable with the idea that we can't just wipe away his history, ship away the equipment, formulas, meds and tubes..that we can't just remove the interventions and that he might just be.. normal. I know to appreciate that he is special and be thankful for the life-altering gifts that come with his journey.. but some days are so hard and I feel the sadness of the situation pulling me down. I have never been a "depressed" sort of person. I just think you can only hold in so much before the cracks start showing. I am slowly becoming more okay with being honest when someone asks how things are going.. smiling and just saying it is all great is not always very honest. Something has to give lately on alot of levels that I keep silent about.. and I don't have all the right answers except to keep moving forward. Pray for us that we don't let the financial nightmares of this life sway our faith in knowing that we will be okay and that God is with us.

Although I am leaving so much out of the last few months, our life is moving at warp speed around here and passing us by so fast and I guess I just take to heart that life can really change in a split second, which is why I hope you all treasure each one of those precious seconds with the ones you love.