Marathon, that's what I feel the last 2.5 months have been. A non-stop race to accomplish everything with the only mode of survival being to breath slow and deep and just keep going. I left off the last post with me in a hammock, that was the last time I can recall "relaxing". The next day Boo was pushing 110% of everything we put into him through he J out the G and his urine output had dropped to 120mls for 24 hrs. We went in. Found out his potassium level was low enough (2.8) for the ER doc to thank me for not waiting till the morning and to comment that he was surprised Boo still looked as good as he did. His labs also suggested dehydration even though he had just received 1000 mls of fluid over the last 8 hrs. He was dumping ketones, protein and his blood sugars were dropping even on D5. They put him on D10 and infused potassium. Long story short (and trust me that hospital stay was quite the trip!) we went from hearing words like "intestinal failure", "Not sure what we can do" "might transfer to Cleveland" to "Oh what idiot missed the fact that on his first two x-rays his tube is coiled in his stomach?!?" OK so that wasn't the only problem he was having and didn't explain all of his symptoms but it was definitely a main contributing factor. It took them 7 days to figure this out! All the while Bennet got weaker and lost weight because all he was getting was D10. He was in a good mood and happy the whole time praise God but it wasn't pretty and I was a mess. It took an additional 24 hrs to get his feeds up enough to go home (with the additional help of Erythromycin). It was another full week before he was truly back up to his old self. Altogether, it was 3 weeks of summer spent with him down and out. Summer is normally our "good" time a time when we all but forget he has Mito and we relax, do things as family like go to the beach, have people over and take long walks downtown. Having him compromised at a "healthy" time hit us hard because it was a brutal reminder that now, even in summer, we have to have our guard up. This was the 5th time in 7 months that his gut shut down and he had to be hospitalized for it.
We addressed this with GI & Mito on our last trip to Cleveland earlier this month. GI was not happy about the fact that Boo goes so long in the hospital on only IV fluids. He had written a letter earlier in the year calling for TPN if Boo goes longer than 3 days "shut down" but the team here didn't want to follow it because it said "may be started" no "Should be started" and it also said "til J feeds can be resumed" well they consider 5mLs of pedialyte J feeds so there the problem was solved. I've never liked the idea of TPN so I wasn't about to fight about this. Well, GI made it very clear to us WHY I need to fight FOR it not against it. Got the message thank you and it does make sense. The reasoning is that if we can keep Boo out of any type of nutritionally compromised state it will result in less stress on his body and the less stress his body is under the easier it will be for him to fight infections and the less damage it will causes the mitochondria in the long run. He wants Boo on TPN if he is below 75% of goal feeds for longer than 72 hrs. He is hoping it will also result in shorter hospital stays and consequently, less risk of picking up hospital born germs. His Mito specialist was more than on board with this approach and would actually prefer he receive TPN after 48 hrs of reduced feeding tolerance. So depending on the situation and how Boo is doing we can decide which timeline to go with. If he is really sick and lethargic, 48 hrs if he is playful and happy 72. While I am happy that they have taken a "Lets fight this thing off at the pass" approach I am also very concerned and worried about the risks of TPN it's self. On the flip side we go into every appointment with much prayer to the Lord asking Him to guide the doctor's decisions and speak to us concerning Boo's care through them. So we rest in the faith that He has done so. There is a peace in this.
One more thing before I sign off :) We had the first Mitochondrial Disease Awareness Walk & Fundraiser in our area on Sept. 18th and it was a success! 5 Mito families got together to walk along with 220 family and friends! All together about $18,000.00 was raised for Mito. Most of it went to the Lucile Packard Children's Hospital to support the new Mitochondrial Disease Program that is getting off the ground under the leadership of Dr. Greg Enns. He is the principle investigator in the EPI-743 clinical trial and is also working on other Mito treatments in various stages of development. We've been in email contact the last few months and I have been so encouraged by his outlook and goals. If you are having a fundraiser and want the funds to directly impact treatment development I encourage you to look into this program. Their fundraising team made things so easy for us and they have assured me everything will go toward furthering the treatment development and not get tied up in the "Foundation's administrative costs".
The walk took a lot of work from me and was on the heels of Boo's hospitalization and only 2 weeks before our trip to Cleveland. All part of our marathon summer! I finally feel like things are winding down, we really have a great schedule for homeschooling, therapies, housework, work and family activities. Of course just because the marathon is over doesn't mean we don't have short sprints here in there but I can actually sit down longer than 5 minutes without 20 things clamouring for my attention! Time to go bake some bread :)