Marathon

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 :)

When the Puke Hit's the Fan

There are a lot of posts that I have swirling around my head right now. (We got the stroller!! But that is another post) for today I have to write a quick update about an event in my day. First a little background. Boo has been sick since last Thursday but thanks to the port we've had infusion services come out for fluids and with a half functioning gut we've managed to stay home. Turns out he caught the flu or at least that's what I'm assuming because after spending a night with him retching every hour, not tolerating feeds and needing a continuous venting I spent the following night puking my own guts out while Hubs layed next to me moaning and groaning like he was going to die. (The man claims to have a high pain tolerance but I beg to differ at least when it comes to GI stuff). My mom, God Bless her! came up Saturday to give us some help in the way of taking the girls away for the whole afternoon. Pudge came home and spent the night puking and asking for water which she would promptly puke up again. It's been a week of weighing diapers, measuring fluids, taking temps, talking to doctors, coordinating services and paying attention to the healthy child who still wants to be loved and carried  for and suddenly has no playmates.

So fast forward to today. Boo's tolerance is dwindling, he's pushing nearly everything I put into the J out through the G and he's all but stopped peeing and he has high amounts of protien in the urine. We called three of his doctors, two agreed on the plan we followed: a last ditch effort of addtional fluids, gut rest overnight and maxing out the baclofen.The other uber-specialist's resident calls rather panicked and wants him admitted asap. But hadn't actually talked to the attending. So we went with the fluid plan because sorry Chica but I don't take orders from flustered, panicky residents who come up with their own plans. However, if this doesn't work everyone is in agreement we go inpatient tomorrow. Fingers crossed as even with nothing going into his gut he is still draining bile out the gport. So the nurse comes and stocks us up on a million supplies and trains me how to hook up his line.(Apparently after two weekend runs they are ready to have our house more prepared) Finally, after 4 months I feel like I have a handle on supplies, who to call, what to do etc. We got ZERO training at or after the placement hospital stay, not a shred of paper nada so I've been feeling really lost. As you can imagine my day was just about stressed to the max.

Pudge had been feeling great all day and just as I was about to send Hubs off for some much needed Chinese comfort food she looks at me with that tell tale face and says she doesn't feel good. I quickly pick her up to wisk her to the bathroom but get no further than the kitchen before Mt. Saint Pudge erupts. I nearly banged us into the walls as I went slipping and sliding through the fall out all the way to the bathroom (thankfully being a tiny house that wasn't far but it felt like it) she continued to erupt into the toilet. I yelled for Hubs who stuck his head out and said "On a phone call about to make a sale do you really need me?" Ug, no I do like to eat, go back to work....another eruption. At that moment I was so thankful for uncarpeted floors.  I stripped her down and put her in the bathtub. More to keep her from tracking her um...lunch...through the rest of the house than to actually make her feel better. I felt better after I finished cleaning the toilet, floors, walls, doors, side of the tub and the clean basket of laundry that she managed to hit. (who knew someone so small could produce so much?!)

After the world was again sane, Boo hooked up and peacefully watching Cars, Pudge clean and happy I went to get the Chinese Wonderfood and swung through Walgreens for toilet paper where God had a special treat just for me. The Ben & Jerry's was on sale 2 for $6.00 and to make it better there was a coupon good for $.55 off each. I bought two. Once I got home we had dinner and I let Hubs get the kids ready for bed while Ben, Jerry, Jodi Picoult and I spent some quality time in the hammock. Hubs knew better than to argue, he'd been gone all day and because he is really that sweet of a guy. It had been one of those days. Not unlike any other day in this household but one where the reality of our crazy life was getting to me.

Stroller Update

So back in May I posted about talking to our insurance about purchasing a retail stroller rather than a medical one. Let me tell you it has been a long road. Actually, the looonnng wait has not been due to the medical supply company or the insurance company but rather the retail stroller company. We found a children's store that could order one in and they were willing to order in a "floor model" for us so we could try before we buy. However, they tried to order it and the manufacturer kept stalling and finally after nearly two months told the children's store they wouldn't send one out till the fall. Drat... We could order one and have it in two weeks but the refund policy is store credit and if we bought it, it didn't get approved and we wanted to return it, we would have to find $1200.00 worth of baby items to replace it with. We had been waiting to submit it for approval to our insurance company until we had seen it but finally decided to just submit it and called our casemanager to discuss the options. If we get it approved and it doesn't work we can still submit for the medical version but we'd still be stuck with a shopping spree (not that I would mind that but Hubs might ;D) We decided to risk it, we are waiting for approval, then going to order it and if it doesn't work return it for carseats, a life time suppy of diapers and a new wardrobe.

Of course I'm kicking myself for not being able to have all this figured out in the first place because now we won't have anything until the end of July at best, perfect stroller weather will be half gone. Every time I leave the house and deal with the now very worn out, overloaded and hard to push current version I dream of the Mylo and it's one hand push. As we clatter loudly down the cobblestone streets (no really we turn heads with the racket made from the tiny wheel holding the O2 buddy board) I imagine the noiseless suspension of the Mylo carriage where the O2 machine will be whisked along by an undersling and not a board at all. Every time I push Boo next to a table I picture him having a seat AT the table instead of under it pointing to all the gum. It's like looking in the window of a fabulous italian restaurant, seeing the tantilizing pasta, smelling the wonderful sauce and then going home to mac & cheese.

While in Chicago for the UMDF Symposium we called a shop to find out they carried the Mylo and we could have easily gone for a test drive but they were out and had just sold the floor model the day before...REALLY?!? Speaking of the symposium it was great! It really should have it's own blog post but since this one has already become a book I'll save it. But for now lets just say that I had a great weekend with great roomies, learned lots of things that are going to be helpful, laughed a bunch, met so many wonderful people and enjoyed a great adventure getting home.

Well, time to think up something for dinner...I made a big breakfast so I am just so not motivated for dinner and will admit that there are SOME (very few but some) meals where having everyone tube fed would have it's advantages ;) 

Please pray for Eithene, Breylon and all the Mito warriors out there fighting tonight.

Cars, Surgery, Strollers

I swear I just wrote a post last week but apparently it's closer to last month..wow where does the time go? Since my last post Boo has had surgery to place a port a cath in his chest. This was one of the hardest things to do. We spoke with his hospital team, his regular ER nurses, his PCP and nearly all of his specialists who unanimously agreed it was time. He has virtually no vein access left and has frequent blood draws, iv fluids and they now want to give him TPN if his gut shuts off for more than three days...aka..everytime he gets sick. We decided to place it while he was "healthy" vs waiting for the next crisis to hit. The surgery went well, he was impatient overnight because of a sluggish (but not haulted) gut, we watched Cars 12 times in a row, then 5 min before heading home his port adapter came unscrewed so we got to watch Cars 12 more times while running IV antibiotics and running 24hr cultures which thankfully were negative! Because of that I spent Mother's Day in the hospital but it wasn't all bad. I had my coffee, we went out to the garden and Hubs brought Chinese :)

We also bought a new car...no I still haven't caved into a minivan. We have a lovely gray Chevy HHR which gives us just the extra space we needed while still giving us 24-32mpg which at $4.35 a gallon is already paying for it's self! At 188,000 miles the Passat was all worn out and ready for a new owner.

Now to find a new fullsize stroller to fit in the trunk. I found a stroller that does everything we need for Boo. Huge canopy, adjustable foot rest, full recline, headrest, reversible, table height seat, large enough undercarriage to accommodate his O2 and pump (with the addition of my own custom sewn sling) and less than 30lbs. It's called the "Mylo" but it's $1000.00 and the only store that carries it is 2.5 hrs away. So I got an idea. Called up his devel. Ped and asked if she thought Boo would qualify for an adaptive stroller. Yep she's thought he's needed more than the glorified umbrella Jeep Cherokee Sport stroller for a couple months now (why didn't she say so sooner?) Wrote the script and sent it to our DME. Then I called our private Ins. Case Manager. Our conversation went something like this:
"Hi Jan, how would your company like to save $2000.00? Boo's Doc just wrote a script for a medical stroller and for everything it needs to include it's probably going to cost at least $3000.00 however, I found a retail one that does all the same stuff for $1000.00 how would you like to buy that one instead?" Jan: "That sounds like a great idea thank you for thinking outside the box and trying to keep our costs down. You'll just need to send me the script, PT filled out form listing all the needs and a print out of the retail stroller specs and price compared to the medical one, I think it's a no brainer!"

The best part is she is going to work at getting it approved under the reimbursible medical expense allowance vs the DME allowance so as not to jeapordize our ability to get a fullout wheelchair later! Now to wait for the PT eval next week and send over the papers!

That's all for now :)

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Hospital stay #12 pic

One very sick little boy right before we layered the icepacks

Finally feeling well enough to play

Feeling a little better I moved him to my pull out bed to look out the windows

Boo wouldn't look at the camera

Can't seem to rotate this one of the girls zoned out to movie.

Just a few pics of Boo's recent stay.

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Viral's and Feeding Intolerance

Well we just finished an eight day stint at DeVos Children's again. Boo caught another viral infection. It started with a low grade fever that we controled at home with motrin and pedialyte. We hoped that by switching to just pedialyte through the gj-tube before the gut shut down would avoid it all together. Well that lasted all of 2 days. Mito kids are so crazy because one minute he was getting better and playing and the next he was burning up and coughing with dark green bile pooring out of the g port. The ER is always traumatic, there is the waiting room with a million germs coughing, running and crying all over the place trying to infect the host next to thhem. Then there is triage where I know they dont clean much if at all between patients.

There is only one advantage to having 17 ER visits in 28 months, the reception nurses know your kid by name and they don't make you wait in the waiting room or bother triaging you. Heck they practically call up to the floor and reserve a room the moment you walk in. Next they call around to page one of only two people allowed to IV Boo. He has only a couple IV spots left so no one messes around with them. Even with them it's getting harder and harder to get one that will stay good and they clot off within 20 minutes if fluids are not started within that time frame. Jon (our best poker) told us again that his veins are so bad we should consider a port. Ugg. He finally got labeled "viral infection with gut shut down and admitted." Because the hospital was full of cold and flu season patients the only open room they could find was in the PICU. Great, try and explain to visiting grandparents that while yes it says "Pediatric Intensive Care Unit" on the floor entrance your child is not actually a PICU patient. Although, Boo started acting picu when his fevers busted through the motrin and tylenol with ice packs being the only way to keep him at 101. He slept all night and was awake for a bit then slep for the next 16hrs. Freeked me out. Thankfully, the next day he was better (didn't need the ice packs, stayed awake a bit more and they moved us to our usual floor. The next several days were a rollercoaster of 2 steps forward 1 step back. He faught off the viral infection but it took another 3 days and the addition of a motility med to get us home. My thumbs are tired... I'll post about the great port debate later.

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You Threw Off My Groove!

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!