Mitochondrial Disease Awareness Week

Welcome to yet another annual Mitochondrial Awareness Week! I say that with a tad bit of sarcasm because in our household, every day is awareness day. I know, very cliche, but it's so true. Every single day, at some point in dealing with my son who has Mito or following other families and praying for them during their children's struggles, I want to just go door to door or in public, I have this desire to start giving a Mito awareness presentation in hopes the world will gather, learn, and give. GIVE. That is probably the number one-A thing we need for Mito to become erradicated. Money fuels research. Money fuels awareness, but the awareness piece has to be in place to help bring in enough in research funding to make more dents in Mito.

How does Mito affect our household?
Not sure where to begin, so I will say that most of you are aware that Mito causes Garrett's severe constipation, delayed gastric emptying, sensorineural hearing loss, mental retardation, OCD, ADHD, Autism, anxiety, low muscle tone, developmental delays and failure to thrive.  Each day revolves around Garrett's care of his appendicostomy very first thing in the morning. It is his appendix brought out through his belly button with a tube in it for now. Later it will just be a hole or "stoma" where we feed a tube into him 6 inches or so and gravity feed 1000 mL of saline in hopes it manually flushes his colon. For now it causes him to poop, but not in the hopeful hour after the flush. Holding my breath this may possible work in the future. Next, it's get the kiddo to eat SOMETHING so he can take his meds. Not always the least bit easy when it's early. Next, it is morning meds: 3 pills to be crushed and mixed with one of the four liquid meds/supplements that make up the mito cocktail, along with a couple of gummy vitamin b3 pieces. Then pack his backpack to be sure he has extra undies, wipes, diapers, and pants. Keep in mind he is second grade age. Next, get him on the bus, which THANK GOD, he loves!!! Then it's getting his meds ready for the lunchtime dosing. On Mondays I can take his meds to school for the week. Each bottle (3 pills and two liquids at lunch) has to be in it's own prescription bottle and then directions alongside. And don't forget the 10 mL syringes for oral dosing!!! Fridays I pick up leftovers of the liquids and bring home to restock. He gets off the bus at 3-is and then it is two laxative tablets crushed into juice. Four p.m. is evening meds: three pills and four liquid meds, then one last med at bedtime. And sometime in there we are trying to get him to eat and drink..... Some days are better than others. I wake and change his youth diaper at least once nightly so he doesn't flood the bed. He has urine control in the daytime if his flush isn't all absorbed and peed out. At night he loses the rest of the flush liquid and usually soaks the bed. We have gotten good at layering chuck pads, fitted sheets and draw sheets. He has a developmental therapist/personal care assistant who can spend up to 40 hours per week with him, but that's pushing it for her. Garrett usually wakes between 4 and 6 the happiest person ever, but we try to get him to go back to sleep so we can all get some rest before the sun is up!!! Then it's flush, medicate, feed, and repeat. Every week, it seems we have an appointment for medical issues or maintenance. Then there's the everyday mental mess that is my son. He is loud, overwhelming to all of my senses, but incredibly cure and lovable!!!

While to those of you with normal kiddos may think the above description of our time with Garrett may sound overwhelming, I want you to know that our son has a more mild effect of Mito compared to so many other children and adults. Many families live in the hospital with their Mito kids, celebrating birthdays, Christmas and every occasion inpatient. Worse yet, many more families celebrate occasions at home but with the gaping hole of the lost child who has preceded their parents to Heaven because Mito stole them.

Tonight it's these families who have empty chairs at the table, empty stockings over the chimney and empty car seats in the minivan that I want to stick with you. Learn, love and GIVE. Look for ways to give at www.umdf.org

Blessings,
Clara-Leigh

Appendicostomy All Done!

I have done most updating on Facebook due to simplicity, but I do want to follow-up to share that Garrett had an appendicostomy on September 23. Surgery was longer than I thought it would be, but he also had his teeth cleaned, sealed and x-rays done. Teeth were in super shape! Great news!!

As for the appendicostomy, he was admitted to the children's hospital floor of St. Luke's the night before surgery around 2:00. After a few hours of questions, exams, more inquiries about his Mito protocol and docs between Boise and Seattle talking, an I.V.  and and nasogastric tube were placed so he could get hydrated well and have the NG tube to use to get 1.5 liters of Golytely down his tummy to get him all cleaned out. Friday morning he went to the O.R, after tons more precautionary calls and protocol measures were taken. He cannot have Tylenol, lacerated ringers IV fluid or a seizure med, Depakote. Apparently, he woke happily just after surgery but took a nap before we got to see him. This was Garrett's longest time under anesthesia, but he handled it well! He was fairly miserable the rest of the day with breakthrough pain, or maybe more sensory issues, but we kept ahead of his pain med dosing just in case. He has to get up and walk right times the very next days, and that was very tough!!! Then Sunday, his grandparents were ALL in town!!!' My folks live in our guest house all summer, but Grouchy's folks had been in Montana visiting friends, so hopped on a quick flight to visit. Just when it seemed a PICC line for TPN was going to be done because Garrett wasn't hungry or thirsty, he asked for food!!!! We came home Tuesday the 27th thanks to a hospital coordinator getting the home care supplies, pump, etc, delivered to our home!!! There have been and continue to be some bumps in the road, but we are holding our breath to see if this will work!!

Day of surgery, you can see the little tube coming from his belly button. In a month, this tube will be removed, leaving just the stoma and we will thread a tube daily. 

This was a week and a half post-op with some Versed on board so the tube that was acting clogged could be changed. His Tubie Friend, whose name and gender change almost weekly, is lying on top of Garrett. He had turned her mask into a bra before we finally got to see the surgeon!! Never a straight face for long around here!!

About a week after surgery, Shelby came by to bring cookies, and you can see Garrett is enjoying swing dancing and back to himself. He was actually back to himself about 4 days post-op!!! Amazing how fast these kids CAN recover, though not all Mito kiddos do. I should count this blessing twice daily!!

So each night, we use a Kangaroo enteral feeding bag and gravity flow 850 mL into his tube, which is through his belly button, using the appendix as a path to the cecum. In theory, this will eventually flush his colon. Not working so well yet, but time will tell. Surgeon has had to do scans and x-rays since surgery because Garrett was peering a lot but not having liquid through the rectum. Last night, before the flush done entering his body, he started having liquid stool, but nothing tonight. Scans with contrast showed that his colon is large and pretty useless. The saline was just going in and pooling in the cecum and then not moving through the colon. So he is absorbing it and just urinating it out. But tonight nothing, so I will sleep with one ear and one nostril open. Betting he urinates enough to float his mattress!!!! I will get up a few times to change him tonight in hopes he doesn't wake soaked because he gets so disappointed.

Should this not help him, the other option is to remove the colon and pull the small bowel through to the rectum and resection it. There would be two surgeries in that process. One would remove the colon and put a colostomy. So his stool would drain into a bag at a stoma site on his tummy. Second surgery a month or so later would close the stoma and pull the small intestine, that had been the site of the stoma, down to the rectum, create a j-pouch and then attach that to the rectum. So besides scars on his belly from the multiple laproscopy incisions, there would be no external sign that the plumbing isn't all normal. Some have success with this, but I would need lots more research and data and chatting with other parents who have been through this to make a decision of this magnitude. It's a much longer surgery than Garrett has ever faced. It is two surgeries. It is just a bigger deal than the appendiscostomy, obviously.

What else could be done? Well, I even talk about options because while the surgeon says to wait and see if this current deal works, I am not feeling hopeful in my  gut. Looking at his scans and test results with mom eyes, it just doesn't look promising. But who am I to say? Just going on gut feeling and the past couple weeks' experience. The other option is the undo the appendicostomy if it doesn't work and go back to how things were before. That's not cool either.


Garrett is back at school and acting himself!!! The largest blessing in this is that besides sensory issues and some pretty intense pain and frustration in the hospital for about 36 hours, Garrett has felt amazing!! Luckily, in a weird way of having to say it, he has no sensation in his colon, so even when backed up or if the laxatives cause any response that would cause most of us serious cramping, he doesn't feel anything!!! So he is a happy little guy, always melting our hearts and making me want this tummy mess to go away so he isn't bothered by it!!!

Godspeed,
CL