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.

Shiney Apples

This year marked the first year our girls would begin attending the same school together; Isabella in 2nd grade and Sofia in Kindergarten.

Sofia was initially quite nervous and unsure of what this experience would mean for her. I could see the questions forming.. Would she fit in? Would she make new friends? What about her old friends? How would she find her class?.. The questions were endless. I was proud to hear Bella begin reassuring her of all the wonderful new things Kindergarten had to offer. This little spark began to build in her eye and I knew she was going to LOVE Kindergarten. As she grew more and more excited, she flooded our breakfast room with a barrage of questions about field trips and lunch lines, P.E., not to mention all the rules and uniform guidelines.

One by one Bella flexed her big sister wings answering every concern. Frankly I never realized Bella had paid so close attention to whether striped socks were allowed or how the school was o.k. with no belts in the Kindergarten year. By this point, Sofia was so excited, she could barely contain herself. We ran down to the grocery store to buy a red apple to give the teachers and Sofie must have spent at least 20 minutes choosing the shiniest apple available. I hope that excitement never dies and that the shine for learning never leaves the apple of her eye.

As sisters go, these two have certainly been known to bicker like cats and dogs, but they have also shared far more together than with anyone else. They have learned so much from one another over the years and have grown to be each other's closest friend. When things are intense with their brother's needs, they seem to do well at talking about it together. They figure people outside the family might not understand. And then there are those times when for whatever reason one gets to go somewhere like a grandparent's house or a birthday party without the other and there is nothing more reassuring than to see them miss each other or run to the other explaining how it wasn't as much fun without having them there. Your heart nearly beats out of your chest.

So on that first morning back, I listened to this type of conversation all the while expecting to lose my emotions when they jumped out of my van. Suprisingly, I didn't even cry.

I watched them run off to the bus in the pouring rain and I felt this overwhelming relief pour over me.. and No, not relief that I would be getting a break from long summer days of 4 kiddos in the house! I was actually just releaved that at least if I could not be close to them myself during the day ahead, they would be close to each other. And close they are.. only a few doors between them. This made the transition all the easier for Sofia. I only followed them to school that first day and each morning thereafter, Bella walked Sofia to her class. As I watched Sofie race to catch up with her big sis and climb those tall bus steps, I realized it won't be that awfully long before they follow each other to college or lead each other down the aisle as Maid of Honor at their weddings. I hope they stay close in the years ahead. It is such a blessing to have a sibling to share life experiences.

It has also been nice having their cousin Keenan at the same school. They typically don't see him enough between everyone's busy schedules and as we now will be helping to care for him some of the afternoons, we look forward to some fun bonding ahead. I can see that this year will bring them closer.

Picking them up at the bus stop that afternoon, I thanked God for the gift of children with easy smiles on their first day, and that I have the privledge of hearing them gush over every detail of their day. This year looks like it will certainly be a good one!

The end of Summer is upon us...

I feel both excitement and sadness at these last days of summer..

We have made so many good memories. At the beginning of the summer we created a list of all that the girls hoped to do over their summer and although we could not cram every last one of those activities into these few short months, we made a pretty good stab at it.

They..

...went on nature walks.

...sunbathed and swam in the pool.

...curled up with good books together.

...had fun game and movie nights.

...played in Yamma and Papi's yard.

...had our first sleepovers both here and away.

...went boating.. many times.

...went to a few movies and even ate dinner in a theater.

...watched fireworks.

...had BBQs with family.

...went to the beach.. and then realized we took so much of the beach home with us each time that we might as well stay home and swim in our pool (without all the sand)!

...gardened with Nana.

...enjoyed visiting family.

...made special art projects.

...went to the playground for a picnic.

...went to the water park with cousins.

...tried paddleboarding.

...attended two different vacation bible schools. Both wonderful experiences.

...went to our favorite library.

...enjoyed a girlie spa day.. at home.

...went to Orlando as a family. Twice.

...played in the rain.

...made dinner for daddy and sometimes just ordered chinese :).

...attended birthdays and tea parties.

Overall, it was a very special summer. I am not really ready for the girls to leave on that school bus, but at least this year, they will go to the same school together. It will be different with just the boys here. And so we begin a new chapter..

New Frontiers Part 3

After leaving Shands with our new temporary gastric stimulator, Sam had some trouble in the car with gag like/ sneezy moments and then rocky breathing and mottled coloring. I had to pull over and set up blow-by and eventually his sats went up and he seemed far better. We got home and he was energetic and happy to be with his siblings. He did have some discomfort while sleeping and a few desats so we used blowby near him in the crib that night, but overall he was normal on Monday and even did quite a bit of eating by mouth. I figured he needed a few days to get over all that he had gone through with the procedure. I was really encouraged that the stimulator sis seem to be working. He was soo hungry and eating more than ever. We saw his pediatrician in follow up that afternoon and even his Dr. was excited to see Sam in such a great state and calling out to "Eat, Eat!." He agreed that maybe this stimulator would be a great aid to Sam.

That night, however, around 3 am in the morning Sam began having trouble. The nurse was using oxygen to help him, but he was not doing well. He was weak, moaning and dropping sats a bit, running fever of 100.6 or so (which is actually high for him as he runs sub). We had diarrhea that day and some residual on venting but it always flowed back in ok, so the stimulator seemed to still be working. He was clearly very lethargic and losing ground. He had one diarrhea early that afternoon. We then went to the Dr and he ran a CBC which was all clear and his chest x ray looked fine. He was at 102.5 fever by then. He was a rag doll, not focusing on us at all. They gave him a Rocephin shot in the Drs office and we went home. He did not get out of bed again all night or the next day, hardly moving a muscle.. very weak and lethargic. He looked incredibly pale and had more diarrhea. He was so pale, almost ghost like. He looked like he was getting thinner in the face too.. but his belly was quite round and distended. We were running his feeds at the usual rates and he seemed to still tolerate them..unusual for him during illness.

I took a rather rank and liquidy stoole sample (not my favorite mommy job) to the lab and awaited those results, in hopes there were no signs of c diff. All the while, he was tolerating the feedings at the same rate we used to put in his J port, but now in the G (there was no J at this point). The rate was incredibly slow for a G feed but we were still building him up to taking a normal bolus feeding and he was getting his normal amount. A bolus would be essentially the same as giving him a normal sized meal portion all at once several times a day, whereas for a year and a half, Samuel has been fed at 55 ml per hour of his formula over 16 hours of each day.

At this point we began asking ourselves, could the stimulator be stealing his energy? Could he be having some kind of non absorbancy or dumping phenomenon with the simulator? But that would hardly explain the couple good days and then the fever and diarrea. I even considered the hand as a source of issue, but it looked normal. Everything pointed to an infection but the CBC had looked good and we had already tried the old stand by broad base anitibiotic. Well, by Wednesday night, we could not hold off on figuring this out any longer. He was fading out on us. I think our night nurse thought he might die on her shift and was probably worried about when I would make the judgment about going to the ER. My problem is that our ER is just not well equipped for him. We went to the Dr. on Thursday and he had a comprehensive metabolic panal pulled and another CBC. It showed really bad dehydration and electrolyte imbalance... likely from the diarrhea but extremely dangerous with his mitochondrial disease. He had lost over 2 lbs in 2 days. The puzzeling part was his CBC still looked ok.

We were then sent over to the ER for an IV bolus of fluids and to take a life flight to Shands Childrens, about 5 hours away. It turned out that the life flight can't make it that distance so it had to be by ambulance. How would we pay for this later?.. who knows, but I knew I needed to just focus on getting him help and figuring out what had happened so suddenly. I really feared that it might be an adverse effect of the stimulator.

They had an impossible time placing his IV. I am convinced that most techs jump in thinking they can get it done on Sam in no time and act too hastily and blow every possibly good vein right from the getgo.. then the nicu nurses or stat teams have to deal with whatever is left.. every time. He just has no good veins left at all and being dehydrated made this problem even worse. Needless to say, the tech blew several veins and when she finally congratulated herself on her hard work at getting one (honestly, I think Sam did more of the work), our own nurse pointed out that it seemed to be infilltrating. The tech disagreed and said that she had just flushed it and it went in fine. Then our nurse asked her to turn off the pump and check again.. and guess what? Big suprise..there was a lump where it was infiltrating into his arm. We would have gone the whole way to Gainesville on this bad IV so I am thankful it was caught early. I try not to get agitated with people who are helping my child, because honestly I am so thankful there are people help. period. But the hard part is the spectrum of work ethic and professionalism and good attitude brought by the various people involved. Certainly we have learned that some are more careful, conscientious, understanding and open to feedback than others who seem to be just picking up a paycheck. That is hard to overlook when your child is being poked and prodded with needles and fighting for life. In the end, the paramedic from the ambulance company got our IV started.. so I dont think the ER should be getting any of the green$$$ on this one.. but they will.

The drive down went as smoothly as it could in that he had no bradys or desats, a common theme in this particular illness (unlike in his history). The paramedic was very nice and attentive. She said Sam's T waves on the EKG were really elevated, but somehow I think his has always looked like that. His EKG has always looked different than the typical, but I dont fully understand these patterns. She said it might be the electrolytes, which made sense. It seems Sam was hypokalemic with very low potassium at this point. His sodium was low, potassium low, co2 low, chloride low, Protein low, albumin low, basically it was all off but the potassium seemed to remain our issue throughout the next few days. It was 2.2 in the Shands ER but by Friday, after a day of IV fluids D10 and potassium boluses, he went to 2.6 and finally got up to 4.1. What is strange is that he went NPO and all diarrhea stopped but Saturday he drifted down to 3.0 again getting full fluids..and sodium went down too.. curious for sure. One I had never noticed before was his CRP level. It was elevated at a 52.8.. and I guess this is a marker for inflammation or infection in the muscles, but can be an important marker for cardiac concerns apparently but Sam seemed fine in that area. I think it was a sign he was inflammed in the gut and fighting gastroenteritus. At least that is what the Shands team decided. They think it was Rotovirus.. but I just read there is a Shigella gastroenteritus going around our county right now.. so who knows? They are convinced there was no connection to the stimmulator other than that he probably caught it while here for the surgery. They actually think the stimulator may have helped him not crash worse since we were at least able to keep feeds running on those few days at home. Within a day or so of being admitted to the 45 unit, Sam began doing much better, going from limp noodle to making eye contact and being more "with it". I am relieved we got help when we did.

The next day I decided to get something to eat since I am nursing/ pumping for Max and it had been almost 24 hours since I had eaten anything. The nurse was fine with me running downstairs to get something, but when I returned I found out Sam had pulled out his gastric stimulator... ugh. I don't think she realized I was thinking she would sit with him. I should have been more clear. They called his surgeon who came up and said he might still be able to fit him into the OR to replace it. What suprised me was him personally walking Sammy's crib down to the OR to get things expedited before the end of the day. They then tried to get it replaced and found his stomach was far too distended to screw the probes into the stomach lining properly. His surgeon is particularly good at explaining what is happening and why he does what he does. He said that when it wasn't working this time and taking too long, he decided to put back in a GJ and get out as the air they were putting in as part of the procedure was further distending him to the max. He said the probes kept going down to the pylorus and it would be best to only set these when it could be done right.

Watching Diego cartoons in recovery was about the only thing that helped soothe him.

Sam came out of the OR with a basketball for a belly but he actually was in less discomfort than last week's procedure. He calmed with time and finally got some rest last night. It is just exhausting trying to keep him from yanking on things or slamming his body into the crib from pain. We lost his IV that night and had to start pedialyte feeds through his J because no other access could be regained. This worries me as I am not sure what we will do about his horrible veins for the future. I know we should consider a balloon medicine port for emergency access and fluids. I hate to add another site available for infection, even if it is under the skin. This may be a decision better made now though than having to get (OI) in-bone line access during emergency.

As far as the stimulator, we plan to get it replaced when the tummy is not so badly distended. I did find out some interesting facts on this trip about our surgeon and the stimulator. First of all, our Dr. has put in 50 of these stimulators, and we may be the youngest, as most have been teenagers. There have been a total of three kids with mitochondrial disease, Sam and Nate being two of these. I also just read an article that he placed one in the diaphragm of a 3 year old girl with Pompe Disease as well.

She was the first child with Pompe Disease in the world to receive diaphragm pacing. I learned about this other metabolic disease, Pompe Disease, when researching stuff for Sammy two years ago. I saw a great movie with Brendan Frasor and Harrison Ford called Extraordinary Measures about a family with this disease. There were many parallels between this neuromuscular disease and Mito, especially how devastating they can be systematically. Anyways, this stimulator for her is being used to keep her off a ventilator for a large portion of her day. Amazing to have a surgeon breaking new ground and willing to explore new frontiers in medicine that could drastically impact quality of life for children! So thankful we have him working to help our boy!

We knew Sammy was improving when he began making eye contact, sitting up and playing with his cars. We watched RIO about 50 times over the weekend and then he wanted to get down to dance and promptly fell over.. this meant an extra bracelet to annoy him on his leg..it read: Fall Risk. Eventually he tried walking and soon was getting his sea legs back in order. Our friend Amber came by and walked the unit with him while I got some dinner. She has really been such a kind friend to us and we are so glad to know her and their sweet family. Trucking around the unit, Sam made several new friends and got alot of attention off his "go to town" hat. Of couse that sweet smile never hurts in winning over new friends.

We will have to work on toys like these in PT as Sam was frustrated that he could not move his feet on the ground to make the car go.. as he put it "GO.. CAR.. VOOM!!"

I am praying today for Sam to grow stronger each day. Praying about the financial stress we face with a medically complex kiddo. I am praying for all the families of the children on the 45 unit floor and hoping they get home soon to their families. I wish other moms and dads would tour hospitals like these and meet the amazing kids fighting harder battles than most adults have ever experienced. I think people take their lives for granted. They don't fathom how precious it is to be healthy. I pray for safe travels for many friends who are on family vacations. I am thankful for all the kind hands that have helped Sam this week and for the help of my mom and my in-laws who supported Jason with the kids at home. I don't know what we would do without them! I want to post a link to a radiothon fundraiser our friends are doing for Shands through the Children's Miracle Network. Nate is an ambassador and they could use any and all support. To help click on this link.

On a final note.. Sam is doing good at home today, but of course we got home and the girls are now running low grade temps with tummy trouble, so we apparently left a little good bye gift behind for them with love from Sammy. They are staying with Nana and soaking up her nursing care in hopes we avoid Sam having a reexposure to this bad bug. Decisions ahead include: when to go back for replacement of stimulator, when to reschedule his appointments that are all messed up by this unexpected hospitalization, and whether to go to Houston for full mito workup by their organized clinical team..

I will have to do a blog post on the girls and all the summer activities, more on Max, who is growing like a weed and is soo big that it feels like my 6 month old and my 2 and a half year old are twins. They are only about 5 lbs apart and wear the same size diaper now. I will update as well on what is new in Mito news.