New Frontiers Part 2

On Friday, July 27th, with hopes of recapturing precious gastric emptying, comfort and motility, we had a temporary gastric stimulator placed by endoscopy at Shands in Gainesville, FL. I believe he is now the youngest child to ever have one placed, although it now seems we may be doing this a few times before we get the permanent one. Sam actually did well through the procedure, only mild desats into the 80s in recovery and over the next 2 days.. not too bad for him. This type of medtronic stimulator is meant to help with gastric dysmotility or gastric pareisis symptoms, usually those of adults with diabetes. It is not made to be used on kids, but we had heard about it from our friend, Nathan, who had one experimentally placed last year and has experienced remarkable success. It was a long, arduous process to decide if it was working in the beginning for Nate, as all mito kids take steps back with illness and taxing procedures. But once they had a stretch of no illness and could see him at home after recovering from OR procedures, they made the choice to proceed with a permanent placement. Nate still has slowing during illness periods but he eats quite a bit by mouth and only needs g pump feeds at night or to add nutritional support since malabsorbtion is an issue for kids like Nate and Sam.

Unlike Nate, Sam had already been forced to go to a J tube (tube into the intestines directly) over a year and a half ago when he just could not handle bolus feeds (large typical meal sizes) of formula in his tummy. Since then, we have experienced increasing issues with Sam's GJ feeds during illness but also when he was well. One of our biggest issues has been a result of success on the oral feeding therapy front. He has been working up to eating more orally from a dysphagia/ airway standpoint and is now frustrated by how little volume he can tolerate in his stomach. It is strange to shift from having a child who can not eat even a drop of water without turning blue as a newborn to finally being able to not only eat but drink from a cup. Yet it makes you upset to watch him grow so tired or in such pain from the act of eating, that he be unable to progress to eating real meals by mouth. All of his feeds are given by J (into the intestines directly) since he failed his gastric emptying testing but we do still put pedialyte and meds in his G (stomach port) and he trys small foods and small drinks by mouth now. So in hearing about this opportunity, we felt like taking a leap of faith and attempting something that could improve quality of life for Samuel. I think one of the main other reasons for us is our fear that if his gut continues on this course with no improvement, we could be looking at his requiring a central line or broviac port directly to the heart for TPN feeds which is incredibly caustic on the liver and body, as well as a huge risk for infection. Our surgeon is the one who placed Sam's original G tube and did his nissan fundoplication (stomach wrapped around the esophagus) at 2 months of age. Here is Sam's new stimulator as it was placed temporarily until we can decide to place it permanently entirely inside the abdomen.

The procedure for the temporary stimulator required intubation with endoscopy and mild intervention in relation to the permanent placement. With the temporary, the box is left on the outside and the leads of the probe are threaded through the stoma and screwed into the stomach lining.

That first night was pretty miserable for Sam with retracting, dropping sats and generally not tolerating feeds. We tried to make him comfortable on Tylenol but by morning we were having to give him some morphine to control how badly he was throwing himself about in pain. We figured this may have been the belly pain and cramping associated with the surgery or the raw throat from intubation, but it was a rather small procedure to be making him that upset.. and we soon realized the next morning what was really contributing to his extreme pain.

At shift change, Samuel was found to have a badly infiltrated IV. They were running potassium directly into his tissues..which is very painful. His hand and all the way up to the elbow were incredibly swollen with fluid. His hand was entirely white with darkish fingernail tips, cut off from the arm's blood supply by swelling and medical tape at the wrist. The photos don't give it justice and most were taken after we began treatment measures. We have had infiltration before but nothing like this. They called in a ton of people stat to evaluate it and slowly we got some capillary refill to his hand. The palm remained white though and they ordered 12 injections of Hydase into the hand. Hydase is made from sheep's testacles, apparently, and it spreads and thins out the infiltrate in the tissues. Amazing how many weird anitidotes we have for every possible pitfall in medicine! Of course in his many many hospitalizations, we have had many blown and infiltrated IVs, but nothing like this. It is hard to tell in these pictures the degree of acuteness, but I was concerned we could actually lose his hand.

After all this, he began to improve and the swelling went down but he was not moving it. They told me they could not be certain he would not have nerve damage or infection. A week later, it looks great, works fine and I think overall, it was a near-miss! Thank goodness for that Hydase and the shift changing nurse who caught it. I don't think the nurse responsible intentionally missed this, but I was less than happy with how she came back in later trying to make excuses... better to just apologize. We realize there are risks to every intervention and hopefully, our nurses always take these IVs seriously.

Sammy had some fun with the nurses blowing bubbles and we were blessed with a visit from Nate's mom, Amber.

By the next day Sam was tolerating more feedings and by that evening was trying food by mouth. He continued to have some junky breathing and retracting but he became more like his old self. He was asking to eat and seemed to have a real appetite. He also had more BMs than usual. I felt the stimulator must be working. Before we left he went from managing pedialyte, to normal pump feeds, crackers, mashed potatoes, oatmeal, eggs, water and milk.

Sam had many caring nurses over his stay in the 44 unit. It had been quite some time since we were here last and I am glad to have this hospital as an option for more involved support than we currently have in our area. I hate the distance we have to go for specialized medical care. Which is another topic that I discussed with Nate's mom: the decision about going out to Houston for more intensive, organized mitochondrial disease clinical care. They have a clinic there that meets several times a month for Mito kiddos where you organize all your appts for one week every 3 to 6 months and they call the shots on how your local pcp manages your individual needs. Sam could see specialties we dont have here such as immunology, endocrinology, pulmonology etc.. I realize I am really just frustrated by our lack of a cohesive team. Sam said "Bye Bye!" to his newfound girlfriend nurses and we drove home that Sunday with a plan to return after a month's trial on the stimulator... or so we thought.