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.