I don't know if any of you have seen the cartoon "The Emperor's New Groove" but if you have you will get this next bit. If you haven't you should see it, I laughed the whole way through and watched it again.
There is the opening scene where the emperor is describing his perfect life and how one person came along and wrecked it. While I'm making the same rant today. 2.5 years ago I had the perfect husband, two perfect daughters, a perfectly beautiful home in the country, a perfect business and was expecting the perfect son after a perfect pregnancy. Then Mito came along and ruined it all!! I did still have that perfect son but not the perfect pregnancy and my perfect son has less than perfect medical issues. No our perfect little life is upside down and feels especially so after the past three weeks! Let me recap.
Week One: Went to Cleveland and after 7 hrs of driving we where 1/2 an hour from our destination and the alternator blows and the battery dies and our car ends up dead on the side of the turnpike. A cop car, tow, truck, failed attempt at a rental car (who knew the Cleveland Airport would be booked solid on a Tuesday night?!) a private driver and three hours later we made it to the expensive Guesthouse for six hours of sleep (not RMH because of all times they would be full too!). There is a lot more to that story full of super frustration and kind people that really should be it's own blog post but for the sake of time we'll move on. Brochoscopy in the morning that ended with total gut shut down and an overnight admission. Once discharged then it was the GI appointment where we were told to just keep doing what we can to feed into the tummy but no pressure or schedule, this was a good thing under the current circumstances. Then the meeting with Dr. P where we found out that while the muscle biopsy was inconclusive he would treat Boo like he had Mito because nothing else fit and there were enough markers to suggest that he could very likely have Mito anyway. One of our specialist's back home familiar with mito coined the term "Uncatergorized Mitochondrial Disorder" and we've adopted it. Boo does act like a mito kid and responds to mito protocols when ill. We've been assuming he has Mito for months now so while it was difficult to hear that "this was it till more advanced testing becomes available" it was not the major blow of conclusively. That night the sleep study which, because of a forgotten piece of the order, was done with .5lpm O2 on the whole night. All the same it still showed work of breathing, desats and obstructive sleep apnea so we know he needs it. On the way back home Boo started showing signs that he was getting sick, he stopped tolerating feeds and started breathing faster and harder.
Week two: Since we where too far to turn back we continued on to pick up the girls from my parents and then hightailed it for home. Bennet did worse the closer we got. We stopped at the house just long enough to grab a few things and rearranged our packed car to have access to what we would need in the ER. We didn't even get the kids out of the car and headed off again for the ER. When I pulled Boo out of the car he was slightly warm. By the time we were checked in at the desk he was burning and I could feel him get hotter and sicker by the minute. They triaged him 5 minutes after we sat down and he was 103.5 bad news. I started urging them to give him Motrin...like last week people! They took him back to a room and Motrin was delivered. I'm sure if they would have rechecked the temp just prior to giving it he would have broke the thermometer. His O2 sats were low so they turned him up to 2, his heart rate was 180s and breathing so terribly labored :( Still, it took forever for the doc to come in, they sent in a student first. Thankfully we had our newly printed Mito protocol letter in hand just for these occasions. We are losing vein access in many places with Boo and he is usually such a hard stick that it has been taking up to 6-8 tries to place an IV each time. I'd finally figured out after the biopsy to start by telling them to not use his hands, they are shot, use the feet with caution and inner elbow is their best bet. Of course they still look at the hands until I tell them NO, you WILL NOT place an IV or try there period. Then I have to tell them that I need the nurse with the most experience, no training, no offence but he really does need this. Oh and yes we know what a port is but sorry not there yet. So we get the nurse with "nicu experience". The guy listened carefully to me as I explained what did and didn't work, took a couple looks around and went for the elbow, the guy got it the first stick! I could have kissed him. I then had to tell them that if they didn't hook up IV fluids and keep it running they would lose it within 30minutes. Well yes, yes I have done this a few times thanks for noticing! Once the doc came in he took a look and told us he'd been admitted. Then we waited 5 hours to get up to a room. Full hospital. The hospital is brand new, less than two months old so of course the room was amazing! I would have been able to appreciate it more though if my baby wasn't so terribly sick. The Motrin allowed him to sleep but his gut was completely unusable, he still had a very high heart & resp rate and the blood pressure was creeping up. By the next day he was on round the clock Tylenol and Motrin and it was still pushing through. We had to resort to using his J port for meds because they were just sitting in his stomach as was all of his secretions. (Boo can normally digest at least those) He was dumping ketones and his PH was a 6.0 and they couldn't find the source of the infection because his lungs where clear and all the swabs, blood and urine came back infection free. We didn't know what we were fighting...story of my life. This went on for 4 days with me butting heads with a worthless resident and the one laid back attending the hospital had who wasn't following the higher fluid protocols while at the same time claiming the problems we were still seeing could be a result of dehydration! Looking back I should have demanded talking to the attending familiar with Boo and making a bigger issue of it but I was so frazzled by how down right irritable Boo had become that I hardly had a fight left in me. Finally I did call one of our most trusted specialists, Dr.D, who came to the hospital. She immediately recognized the pain Boo was having every time he had to pee (for the past 4 days!) as high calcium and ordered nephrology to come down. Sure enough it was quite high. By this point Boo was starting to turn a corner and was tolerating pedialyte slowly through the J so they decided to hold off on full blown kidney work up and redo the urine in the morning as well as repeat blood draw. The kidney doc, Dr.S, has other Mito patients and this made me love her instantly. She felt the kidney problems were a result of the infection just as his gut was not working right neither were the kidneys and she hoped that as he improved so would they. She was right, once he started getting a little formula mixed in the levels started evening out but his PH was still just a 6.5 and it took Dr. D to save us again and finally up the fluids before it got up to his normal 8. By day 6 Boo had made almost a complete turn around which is nothing short of a miracle!! They sent us home on 24hr feeds rather than waiting for him to return to 18hr feeds and we were comfortable with this. We also monitored his urine at home with strips. He had lost 2 full pounds in 6 days.
Week three: We've slowly upped his feeds back to normal and just plan stayed home to help him fully recover. Nearly every time he's been that sick in the past we've lost a function or seen a medical regression. So far the only change is that he is back on a full liter of O2 continuously vs the .5 we'd weaned him to just prior to the bronch. We are praising God for this in a big way!! At one point in the hospital one of the doctors told us that the longest he felt comfortable with Boo on just D5 was 5 days. After that we'd have to look at TPN. I did NOT want to go there. Boo started getting 1/4 strength formula on day 5, tolerated it and avoided TPN. For how bad his gut had been the day before to suddenly tolerating really was a miracle. So now we are back to a semi-normal routine again and I even took him to feeding therapy today where he ate a new record of 5 fish crackers. I have my always happy baby back :)
So that was the last 3 weeks with loads left out but you get the idea!