Saturday, August 13, 2011


After returning home from Atlanta, I began to prepare for the upcoming first prenatal apppintment with our new Obgyn. This pregnancy has made me face many not so easily buried and painful memories of Sam's complicated pregnancy and delivery and I am having to let go of those fears and realize that this is all in God's hands. All I can do is what I have always done.. give this unborn baby the best environment and prayer and love that I have. I must also rest my trust with medical professionals, which is a tough subject since I struggle with how things were handled the last time.

I had a 1:30 appt and after a long, nervous wait, I went into the ultrasound room at 3:15. Jason surprised me by showing up, but had to leave a few minutes prior to the ultrasound as it was just too long a wait. I went in expecting to see a little 11 week old fetus with arms and legs.. a tiny little thing..the usual early ultrasound picture. Instead..

..this fully formed, larger than 11 weeks baby appeared and soon after the tech explained that I was most certainly closer to 16 weeks by her estimation. It was moments after this that I realized the sex of our baby.. there was little doubt and the tech confirmed..we are having a baby boy! This shot is from below the bottom and makes the sex pretty clear..

SO.. our family will be a better balance with 3 girls and 3 boys total (Jason and I included). Our family will be complete and balanced. The baby is looking good and we now understand why I was showing so much.. so early! The due date is January 27th and I will most likely have to have him by scheduled C section, as that will be the safe way to go following a recent C section. They won't perform VBACs here even if we wanted to risk it.

The appt went well, but was long as of course our Dr. had to get filled in on alot of Sammy's history and I had many questions myself. I feel really good about this doctor and I will not hesitate to see the high risk if anything seems concerning. For now, I am just praying for a healthy, uneventful pregnancy.

Sam is doing well this week, although doing alot of sleeping. He met his new physical therapist and will begin those sessions soon. Although I was sad to lose our longtime PT Miss P, I think Miss Q will be just fine. She was quite surprised by all he is doing now and is seemingly in my corner on the issue of whether we need a stander piece of equipment to get him vertical.. not so necessary now as he is showing off such wonderful new abilities! She did say we may need the aid of a reverse walker or similar equipment to make that next step towards walking. I am just so overwhelmed with joy at his new crawling, sitting and pulling to a stand skills! She said his transitions are phenomenal and although there are general weakness issues, we can build forward from here.

The only other new curious symptom is an eyelid swelling issue. Sam's eyes have always been a sign of something ominous! He gets pink eye with every ear infection and his eyes can be very telling about other issues, glassy and rimmed red when he is reaching his limit of physical activity etc. I can tell with one glance if he is reaching an exhaustion crash. The new symptom appeared Wednesday morning when his right eyelid plumped up big and swollen. It seemed like it was full of fluid and it was warm to the touch. I did not jump to take him in as the white area in the eye looked ok and I hated to head back when we were just there on tuesday. He did not want us to touch it and was quite defensive. We watched it all day and it seemed a little better by nightfall but had a slight worsening the next morning. That night it was almost entirely gone and so I figured we were done with the issue. The night nurse probably thought I was loony telling him to watch for it, since it was no longer there. However, the next morning it appeared in the left eye and this time his eye was almost swollen entirely shut. I was now more concerned again. But in Sam's usual odd fashion, the eye seems much improved tonight... so we will keep an "eye" on it for another day or so. Hopefully it is just an allergy and not some weird virus or cellulitus. Fun stuff. For now, I need a little shut eye myself..

Saturday, August 6, 2011

Tough Days

I have just returned home with Sammy from an emergency trip to Atlanta and I am still struggling with how to write about the emotions of the last few days. I guess it all comes down to fear and feeling helpless. I need to remember this scripture which I have heard and read many times before..

Do not fear, for I am with you; Do not be dismayed, for I am your God.
I will strengthen you and help you; I will uphold you with my righteous right hand.

Isaiah 41:10

First I should say I am always fearing any type of low heart rate/ bradycardia episode, but there has always been quite a spectrum of severity and duration of his symptoms during an event. There were pretty scary bad ones in the hospital following surgeries or during illness etc when whole teams have had to intervene and then quite a few in our home where Sam required stimulation and/or oxygen to recover. Others were completely self correcting and just the knowledge that his heart rate took such a plummet was scary. Color changes and behavioral symptoms are easy to spot when he is having a bigger one. All of them are scary for me but up until Wednesday, there had only been one or two other episodes where he actually passed out completely and only one was followed by no breathing for an unacceptably long duration in our home. That was back at the age of 2 months when I gave 2 rescue breaths myself and was on the verge of compressions when he roused.

I was not feeling well Wednesday morning, so my mom was over helping with the girls while I tried to ease into doing bills, paperwork sorting etc. I needed to head over to the pharmacy and the bank, so I popped my head in on the nurse to let her know where I was going. This nurse was a fill-in for our normal MWF nurse and it was only her 2nd day. I gather this made the turn of events even more difficult. As she turned to place Sam in his crib, I watched as his tubes were left to dangle between her body and the cribside. Then as she lowered his body, the GJ tube which leads down into the intestines, became caught and pulled out. The balloon which holds the GJ in the stomach had popped through. This has happened before and although frustrating, it is not the end of the world. The most frustrating aspect is that a GJ can only be replaced in interventional radiology under flouroscopy. For us, this can mean a trip back to Atlanta when it has entirely come out since our local hospitals do not have the tools for a mickey GJ placement. When only partially pulled, we can probably just head to a local ER to check proper placement. In this case, only the balloon and beginning of the GJ line had popped through.. at least initially.

I asked the nurse to please hold the tube against the stoma to prevent it from coming out any further. She was kinda new to this and floundering a bit so I was explaining what would need to happen next. She was holding it to him, when I turned back to the crib and found Sam's color was draining out and he was nonresponsive. He was turning grey, his eyes were glassy and his mouth, although open, was not moving air. In moments, his eyes rolled back and he fell unconscious. He was not breathing and his color was now completely grey everywhere. Words can not describe what he looked like at that moment. I thought he was gone forever. I went into pure "Go" mode and the nurse and I began scrambling for oxygen. She was quite upset to discover there was no ambu bag near the oxygen tank or the compressor. I began to panic too but as she was already doing enough of that for both of us, I moved my focus to him in the crib and tried to stimulate or bring him up somehow while she searched. He was a limp noodle..lifeless. I don't know how long this whole situation lasted but I imagine it was only a couple minutes before I realized I had seen the ambu bag below the crib the day before when our diaper supply was low. That is usually the only thing stored under there and so I remember thinking.."hmm why is that under here?" but yet for some stupid reason, I did not pull it out. Since then, the other nurses have told me that our night nurse had "cleaned" it to that storage spot. When I realized where it was I went to get the drawer open, quickly realizing the side rail had to be up to get it out... it is amazing what bumbling idiots you become in these moments. I remember myself shouting and fussing with this before I finally got to it and we started some blow by while rubbing his chest. At this point he started reviving and color came back. By the time we got a pulsox on him, he was doing much better. It was all such a nightmare.

During the course of events I had shouted "CPR.. Maybe CPR" but could not form thought about whether that should be our next move. The nurse thought she had felt a light pulse but honestly we did not know what it was or how slow it needed to be to warrant compressions. I have since learned that even if his heart rate was below 60 and he was this symptomatic, compressions might be needed to help bring up his heart rate enough to circulate the blood properly. I think I was first and foremost just thinking about oxygen.. he was just so grey.

After all this happened, it became evident that the entire tube had migrated out of his body and so I inserted an extra G tube I keep on hand so that his stoma would not close up. These can be inserted rather easily with the use of a kit and some saline in a syringe. After all the excitement, Sam seemed stunned but happy to be in my arms. I asked the nurse to hold him and read to him so I could call the Drs. Of course it was lunch hour and I had some trouble reaching people. His GI Dr. wanted us to board an angel flight and come to Atlanta's scottish rite for replacement and monitering. He did not want fluids going in through the stomach for fear that his episode was evidence of some kind of internal issue caused when the tube was pulled out. This made things more complicated since Sam can not go without proper fluids for any stretch of time. He wanted us to have an IV placed for the trip but of course we could not get that arranged quickly enough by our pediatrician locally.

So we made flight arrangements out of Destin through the amazing people at Angel Flight in Atlanta. They put it together in an hour and a half. They are true Heroes and I will forever be in their debt for the missions that have made it possible for Samuel to reach the best medical care available. I am quite humbled by the kindness of strangers. They really are angels sent down to touch the lives of innocent children. Our pilot said this was his first chance to fly someone since joining the organization over a year ago. He was so happy to be able to help. I could tell Sam was working in his life by giving him the opportunity to make a difference. God works in such mysterious ways. Even horrible circumstances have a purpose it seems. I do believe God would not let our son experience this pain without also allowing it to effect others in a positive way and also to help us not lose hope about the humanity of the world we live in. Sam makes me want to be a better person every single day. I am so thankful that others feel this power when they reach out to kids like him too.

We had some further trouble on the flight at higher elevations, both on the way and then again on the way back. It seems elevation poses yet another difficulty for Samuel. His sats dropped into the 60s and 70s and he turned a bit dusky but once our elevation was adjusted down, he quickly improved into the 90s again. I will have to keep this in mind for any future flights.

Our pilot personally drove us to Scottish Rite and we entered through the ER, where they were expecting us. Quickly, we realized Sammy was declining without much fluid intake. By hour 4, he was running fever, higher heart rates and respirations. He was hypertensive as well. With much effort, they finally got blood for labs and 4 sticks later, an IV in place. He had that in for about an hour before the whole arm puffed out like the michelin baby and he writhed in pain. This meant more and more sticks and two more bad IVs. We were about to be admitted to a room around midnight when the Dr came in and said an ambulance was ordered to come and move Sam over to the sister children's hospital at CHOA Egleston. We have stayed there before when Sam was once evaluated for the pacemaker. They have a wonderful heart center and are better prepared for cardiac issues. I was confused about the transfer but I guess they wanted to watch him for any other similar episodes. The ambulance ride was nothing short of frightening since a storm had popped up and I was in the front with the driver and Sam was in the back with the EMTs. The ambulance was flying down the interstate and I was feeling the effects of nothing in my prego tummy since lunch.

At Egleston, Sam arrived in a bad state of dehydration. They could not get any IVs in place and he was on the brink of a total crash. Finally they started a slow run of fluids through the G tube and he started perking up slowly. It was a slow day on Thursday and they held off on doing the GJ placement so he could be monitered by telemetry. He had an EKG and he bounced back and forth between the cardio service and general peds.

Those couple days back in the hospital were a reminder of why I HATE staying in the hospital for any length of time. The hard sleeper couch and constant people in and out through the night, coupled with mixing hospital food with morning sickness.. one change of clothes and no soft jams are irritating but really I just wanted to see Sammy perk back up and feel better so he could get back home to his sisters. Since getting home, he has slept alot and I have caught up on some rest as well although I can't shake the images of what took place the other day. I have an Obgyn appt on Monday and alot on my mind in that arena.

I am praying for a few others in the hospital right now. I pray for strength mainly, for them and their families. I know what it is like to feel powerless and experience a kind of helpless fear that only comes when it really is all in God's hands. I know I should not fear and God asks us to calmly hand our worries over to him.. but this apparently is something I struggle with, as it seems others do as well. I am thanking God for the help Sam had from so many good Drs and nurturing nurses this week. I seek guidance in my prayers about whether we need to go back to considering the pacemaker even with the risks of open heart surgery. I think if this level of event happens again at home, we will be forced to reconsider. For now, I am just praying his little autonomic nervous system will stay stable, that his health be good and that he will grow stronger with each new day.

Tuesday, August 2, 2011


We have some news. It seems I am "with child.".. #4. Although, honestly, I can hardly recall a time I have been "without child" in the last 6 years! After the shock and disbelief of this news, I have found myself repeating.. that, although not something we planned, it is a blessing. Every little life is a blessing.

We never intended to grow our family beyond the size of five. 'Family of Five' had such a nice ring to it. You can actually all fit in one sedan, right? It is sooo much easier to request a table in a restaurant..and you can even make a booth work with a chair on the end. As Jason and I were each raised between two siblings, three felt somehow the right fit for us. Then to experience the journey of this last year and a half.. well, let's just say I was feeling the limits of my mothering abilities and was further convinced of the beauty in the number 3. I would watch my sister rangle her 4 kids (all under the age of 7) and think to myself.."How is she not losing her mind?" Now here I am with #4 due before Bella even makes it to age 7. Yikes!

But as God has often taken the wheel during the peaks and valleys of my life, I know he has always brought me through and in the end I have always grown to fill the role he intended, even if it sometimes included a little kicking and screaming along the way! So.. when I started experiencing an unusual level of exhaustion, I went through a period of panic weighing how we could possibly be expecting.. so unexpectantly. At first, I did not want to believe the results on that pee stick, yet it was hard to deny the dark plus signs appearing on multiple sticks. It seems I am about 10-11 weeks along and as I am somehow showing in an absurdly obvious fashion, I feel I should start sharing the news.

I won't actually be seeing the Dr for another week as it has taken me some time to decide about who to use and how to best handle the prenatal care of this pregnancy. After an incredibly complicated end to the last pregnancy, we want to be sure that this child's care and my own are in the best hands and with a plan that will ensure the most healthy outcomes. For now, I am trying to get past what has been a pretty rough 1st trimester with all the exhaustion and morning sickness or all-day sickness, that is. I am sure some people think I dropped off the face of the earth.. since there have been days I could hardly leave the house.

The last couple weeks have included some special days too. Friends of ours were in town for a few days while staying with our other good friends. Our families had some fun together swimming and catching up. BBQs and golf were big hits as well as an interesting trip down to the beach on a most soggy, rainy day. I have tons of pics to download but here a few highlights..

After that busy week, we made a trip to Atlanta as a family for Samuel's follow up with various specialists. While I took him to the appointments, Jason took the girls over to the Atlanta Zoo. It poured that day and the girls were soaked through and through. They hardly noticed as they were just tickled to visit all the animals. It would not have been a trip through Atlanta without a stop at the Varsity..memory lane at my dad's favorite place to eat chili dogs before a ballgame. We were also very excited to see my friend Jenni, who not only found time to visit us at the Ronald McD House but also joined us on our trip over to the Fernbank Natural Science Museum. I may have to post more pics of this experience later but needless to say.. Bella only had to enter the Dinosaur exhibit and she was in pure heaven.

As for the medical outcomes of the trip, I felt overall that we made few changes to Sam's medications, routine or therapy, but that we were able to update these Drs on what our child looks like on the better end of his health spectrum. He has such extreme transitionary periods of wellbeing, making it hard for Drs to diagnose, understand or treat him. When he looks good, he looks sooo good. During these periods, people would have a hard time seeing all that lies below the surface. When he is not doing well, it is the most horrible thing to witness. As much of his life is summarized in a phone book of medical records from one crash illness to another, and as most Drs look at records first, it can be easy to surmise that his prognosis may not be so good. I often wonder what the physicians imagine before they meet him when records are sent over first. Then they all seem to show such suprise upon meeting the bright and beautiful, engaging little man that arrives in place of that patient they read about with so many issues. You see him on a good day or in this case, you meet him after the longest stretch of decent health ever.. and you are astounded and greatly encouraged by the difference.

Since he has done so well in recent months and is making so many new strides with development and movement, I had many questions about whether his condition might not be progressive, degenerative. The answers I got from his Mito specialist were mixed. Since they really have yet to fully identify a source or exact gene to hang a "primary" Mitochondrial Disease diagnosis, we are left to let Sam show us what he is going to do. They did request additional blood samples from Jason and I as they found some curious abnormality that is being investigated further. This worried me even more about the baby I am expecting. Apparently their work at studying his samples is constantly ongoing. We talked about how within the diagnosis of Mitochondrial Disease, there is quite a range of prognoses and clinical findings. There is a 20% figure attached to those cases where things are going to progress in quite a degenerative pattern with some waxing and wanning of symptoms along the way and these cases carry the highest risk of fatal outcomes. Presenting earlier in life does not help the situation. Then, however, there is another 80% of cases where the patient does have periods of regression and "crash" episodes but if they overcome the bad periods they continue along on their upward course albeit at a different pace. For these cases, they may have periods where they can't walk or are hospitalized for one thing or another, but then also have periods where they are seemingly normal and doing fine. I don't know where we will fall and frankly, I am still perplexed about what they actually do know difinitively. I do feel having a negative outlook will not help. We have had a few Drs that took one look at the suspected diagnosis and gave us pure gloom and doom feedback, others were not convinced of the diagnosis, and then others think we will teeter along with dips but hopefully manage his symptoms. Our mito specialist says this is all relatively unchartered territory and research is ongoing. Our local Drs just don't see this enough to say one way or the other.

SO.. on to a new and exciting topic.. Sam is now super mobile! He is crawling! First he got to a sit, then he lifted up on all 4s, then he army crawled/ scooted on tummy, now he is actually crawling up on all 4s. It does take him quite awhile to get across my living room but, by golly, he has figured it out! I felt like Professor Henry Higgens in "My Fair Lady" when Eliza figures out how to properly pronouce, "the rain in spain stays mainly in the plain".. quite a moment around here! He has even done a full pullup of his whole body weight at the coffee table from sitting postion with his feet off the ground. He is amazing... and then he is tanked and sleeps all day long. One day,I hope he will walk.

On a really sad note, we just found out due to strange insurance contract issues that our physical and occupational therapists have with BCBS, we will be losing them for Sam's therapy. We will be getting a new physical therapist, but ours was wonderful and I am concerned about this shift effecting his progress. It seems we are not getting a new OT and on this I am quite concerned. It seems we are in a disadvantage because we have an insurance policy for Sam. The hardest part is our PT has been with us since the beginning and now just as Sam is making such progress.. to lose her is very hard. Everyone agrees that Sam can not be exposed to the environment of their busy clinic, so we are between a rock and a hard place. BCBS, our insurance provider says she can not provide us services in the home even if they are not being billed. It is crazy but somehow, a therapist with no BCBS affiliation can come in..and I can't risk illness for Sammy in a clinic 5 days a week.

Speaking of PT, the advice obtained from the orthopaedic appointment in Atlanta supports that Sam could certainly be placed in a stander or make use of assistive devices as he could see no reason that his bones or joints were unable to support him. The Dr. did say Mito kiddos would be at great risk of severe scoliosis if they were unable to keep their bodies erect due to their general muscle weakness or power supply issues.. but that would be further down the road.

As for the GI follow-up, I am further convinced that his Dr is a perfect part of our team. He seems to even want to lead it. He has a great bedside manner and is a plan maker. He draws me little pictures to explain his ideas and he is optimistic about good outcomes. The new plan was to totally clear out his system with a major dose of miralax over two days at 4 full caps a day and then stick to a daily routine of miralax and colace unlike our current practice of laying off when he has a liquid dumping day. He wanted us to also do a pediatric enema but apparently our tiny town has no such modern marvel... so we just used a liquid suppository. You can imagine what fun those next couple days were for the nurses and I! The goal is to get 1-2 soft bms each day, which in turn should speed up the stomach's efforts, which should allow us to try bolus gastric feeds again one day. Then maybe later eat primarily by mouth in the usual fashion. I hope this plan works.

Now of course we could not go to Atlanta without returning with a souvenir... for Sam this was a bout of pink eye that went from one eye to the other and then included a stuffy nose that seemed to be draining his energy. He had a brady or two and then by day 5 he was miserable. The pink eye antibiotic drops were obviously not enough. HIs gut was involved and the respiration issues. We went into his Dr. and found out that once again Sammy has a double ear infection... more antibiotics. Luckily after a couple rougher nights he has transitioned to sleeping all the time and no major declining issues. I hope this means that for once the antibiotics are working.

The last bit of excitement to share was what a fright we had last week when Bella and Sofie somehow butted heads while playing and Sofie's nose and mouth erupted with blood and yes, even more fun, she began to vomit. To say it was alot of blood is an understatement. All is fine now and she must have just hit it in such a way as to disrupt an artery near the nose bridge. Such a different feeling to deal with an emergent situation for the girls. I sure am out of practice on that.

I am praying right now for a few families from my church and community. Our neighbor passed away this last week after a battle with bone cancer. I pray for strength for her husband, sons and grandchildren. I also just received news of the loss of a member of my church choir. I know both of these families need to be lifted up. Our lives here on Earth are unfortunately always too short but some people, like these women, have walked a path in life that was meaningful and inspiring to those around them. They will live on in the hearts of all who knew and loved them.