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!

Result wait

So we have officially gotten the results back from Cleveland and they are "inconclusive". There are some markers for mito or possibly another genetic or metabolic disease/disorder but they do not know which one of course. We have no idea what happens next until we talk to dr. P on the 17th of this month. "undiagnosed land" is so confusing, tiring and right down frustrating lately.

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Awaiting Results

So it has been nearly 8 weeks since Boo's muscle biopsy and we've only gotten some of the results back. On the way back home from AR we swung through Ann Arbor for an appointment there with Boo's Neuro. He'd gotten the incomplete biopsy results but didn't know what to make of them as nothing was clearly something. Small elevations here minor deficiences there. Aclycarnitines were probably the most interesting in that his Calculated Aclycarnitines were 44.8 instead of between 300.85 - 1078.55 but again, nothing diagnostic. So we wait for the rest of the results in hope that they will shed some light on the rest of these and give us some answers. All the while we were gone I didn't give the results a second thought. I knew I probably wouldn't have answers until the middle of February anyway. However, having some of them back and revealing nothing I'll fully admit to being discouraged. What if we put Boo through all this for nothing? What happens next? Do we keep searching? aka poking and prodding, xraying and the list goes on? How much do you "hurt" your child in order to help them? Hubs keeps telling me not to worry about it until we see Dr. P and HE tells us what the results do or do not say since the doctors that have weighed in so far don't know mito as well as he does. So we wait again. We have to wait 16 more days until we return to Cleveland for a bronchoscopy, GI follow up, sleep study and of course see Dr. P. The one thing Neuro in Ann Arbor did find out is that it IS possible that Boo has an Oxygen Affinity Disorder given his "without cause" O2 needs. He consulted with a hematologist there and recommended we have some tests done buy Boo's Hemo here to test for it. He thinks it would be secondary to whatever is primary or possibly in conjuction with his Elliptocytosis. Great, one more tree to bark up!

On a positive note today is my 28th Birthday and while I feel more like 48 and just plain hate being this close to 30 I did have a good day. My hubby called my Dad to get the recipe for my favorite childhood "panacakes" (Dutch pancake) and made me breakfast in bed complete with coffee, my dear daughters each made me a homemade card that I love, Boo gave me smiles when he squealed with delight at being allowed outside in the snow for a few minutes and God made me just sick enough with cold and flu symtoms that I had to lay around in bed and drink tea all day but just well enough that I wasn't completely miserable and could read, sleep and type at will. Of course the flip side was that the kids were barely let near me, Hubby took a forced day off work to play with the kids and I sat here praying that Boo doesn't catch what I have because if he did it would mean a lovely birthday overnight getaway to the ER. Speaking of a getaway, provided we have clear sailing, a friend of mine and I are going to go on an overnight next week to a beautiful house owned my some people from our church. It will just be one night but there will be no pumps, beeps, meds, laundry, dishes, alarms or any kids for either of us and we're both looking forward to it. As SN Mom's we need to feel OK doing stuff like this on occasion! For tonight though I'm going to snuggle up from a distance with Hubs and watch a movie.

Blog Failure

I haven't died. Although, I admit it probably seems like I did since it's been forever since I last posted. In my defence I've been busy! I could probably filling a blogging book with all that has been going on lately. In December Boo went to Cleveland Clinic to have a fresh muscle biopsy done. It was originally scheduled for Dec. 16th (They gave us 2.5 weeks notice) but then on Dec. 6th they called us to let us know there was a mistake, a double book and could we please be there on the 8th? Sure no problem we don't have a life anyway right? Away we went. A simple enough procedure but Boo had to be inpatient from the early morning of the surgery to the next evening due to O2 and feeding intolerance issues. It's had it's own lasting effects too as we still have not been able to take him of the O2 as much as we were able to prior to the surgery. Other than that he is back to his old self. In fact, he's doing great! He hasn't had so much as a running nose since his cold in early November.

After the rollercoaster the Cleveland run was we came home to a Christmas season in full swing. Celebrating Jesus's coming to earth is a big deal in our family and we had several parties and get togethers, multiple baking sessions and dozens of cups of hot cocoa. The kids loved it all. We topped it off with a trip to Arkansas where several of my relatives live including my Grandpa whom I love dearly and who's name I bestowed on Boo as a middle name. We've been here for nearly 3 weeks and leave tomorrow (Love having a DH who can work from anywhere!). It has been the best break ever. No doctors appointments or therapy sessions! I'm realizing just how much time they take up now that I have lived without them for awhile. It has also been amazing watching my 82 year old Grandpa play the same games with my kids as he played with my cousins and I growing up. You have to admire a man that will play hide-n-go-seek at that age! Boo is thoroughly attached and I think they are both going to miss each other when we leave. I'm going to miss the coffee and long games of scrabble. I will also miss all the space and sunshine!

One of the little things that made this trip easier was our medical supply company loaned us an Eclipse air concentrator. Let me tell ya folks, I don't want to give it back!! It's SOOOOOO much lighter, quieter and nicer looking than the behemoth that is at home and I took it everywhere we went. Plus, it fits in the car in front of his car seat so has virtually eliminated our need for tanks. We brought 4 small tanks with us as back up but have only used 1 in three weeks as I only used it to carry him into a store and lunch a couple times. The behemoth takes up a good 1/3 of the trunk and in our Passat that is valuable space so having it free made all the difference! I've also had more cruise bookings this week than last fall combined and it's hard to focus on work when I'm visiting with family I rarely see.

While it has been fun being away I am also feeling a pressure to be home. Two of our friends are in the hospital with their kids and neither one is getting great news. I'm wishing I was there to bring a meal or coffee or something. The burden on my heart for them is so great! Sometimes, I wonder how much I can handle when it comes to "bareing one another's burdens" as God tells us too. I pray often and find that doing something to help is what gives me the strength to keep going. I have decided though that I can not handle my four sis-in-law's petty drama! The boyfriend runaround, the "parents don't get it" shpeels, the bad choices AAAAAHHHH!!!!! I want to just slap them up side the head and say "Girls, really?! There is more to life then this!!" I'm cutting it out and even asked my hubs not to pass on all the "news". It has been a great decision.

So there you have it, a very small nut shell of what has kept me away from here. I have a lot more on my mind and heart but sleep calleth my name....

When your friends struggle

Today I read an update of a friend's son who also has mito and is going through a rough time. My heart breaks for them because I know the pain of regression, the uncertainty and raw fear of the unknown. It also breaks because I know that the next update could be our own. Mito changes things in moments and at anytime it could be Boo. Right now he is doing well. We've avoided the hospital twice now and I consider that a big deal! However I know it's not an "if" we ever go back to the hospital but more of a "when". It's just the nature of this disease. Right now it's our friends and we stand by them because we've been there too. Not always with the exact symptoms but with the same emotions and thoughts. Part of me can feel guilty when a friends child is doing worse than Boo. After all, he is doing amazingly well overall right now and to see another child with the same disease go through a rough patch while you sail by can be...a complicated emotion! You are happy your own child is doing so much better than the other and at the same time want that child to improve to the same place. Mito seems to be the instigator of complicated emotions on many levels though! Tonight I will be praying for the improvement of a special little buddy of Boo's and in the same breath praying that Boo at least stays where he is at. Because, I know it could be him next time.

Twice in a week!

So check it out people I'm posting twice in one week! I know earth shattering right? OK maybe not but it's a first for me here. I tend to keep very busy and I think it's my subconcious way of NOT obsessing about Boo. Because I'll admit when I start something I become totally obsessed with it until it's finished/solved. Well Boo's medical condition isn't going to get "finished or solved" anytime soon so I've dropped out of Google Medical University (and I was sooo close to my MD!) and enrolled in "Domestic Diva Academy" instead. Baking, cooking, sewing, knitting you name it I have it going on! As I mentioned in a previous post I made a dress for a date night. Well a friend of mine has a start up photography studio and invited me over to model my new dress. The only problem was that her studio is in her basement and is designed for kids which meant I couldn't stand up for pictures in my 4 inch heels! Here was probably one of the better ones (that's she's emailed me anyway) although the skirt actually came to about 6 inches above the knees when standing vs the super mini it looks like here!

So that date was FABULOUS!! Hubby looked really hot in his suit our friends were, as usual, a blast to be with and it felt wonderful to be a polished adult for an evening! I owe that date to a terrific sis-in-law who has tirelessly volunteered to be trained to take care of Boo for short periods and offers her services at least twice a month (plus she cleans and does laundry!). Without her we'd be starved for alone time.

Right now I'm back to my life in jeans, chunky sweaters, ponytails and fuzzy socks as well as beeps, buzzing and other alarms that come with Mito life. Boo has been more tired than usual the last few days and has required almost continuous O2 and I'm trying to decide if it's teething (three coming in!) the G-tube feeding trials (doing good at continous for a couple hours a day!) or if something else is up. So hard to tell! Well that's about all I had for today :)

Worry

I shouldn't worry about anything ever. No really!! I have a great family, wonderful husband and a God that has proven over and over and over again that He is taking care of me one step at a time. I can pile all my burdens on Him and He will give me the strength and support to walk through this life. So why do I find myself doing it?!? I'm not sure I can answer that other than to say I'm not perfect. I wonder about Boo's future and what it really holds. Sometimes I wish I knew the future so I could prepare better and other times I'm glad I don't. If you told me last year what my life was going to be like this year I probably wouldn't believe it. It's probably why God doesn't tell us our life story ahead of time but rather puts things in place to prepare us for it. My life has been...interesting. On the one hand it has always been perfect, happy, loving and filled with many things that most people never get to experience (like milking cows or walking the bridge of a luxury liner) on the other it had a lot of drama, hardship more drama and sheer stress from a young age. All of it has prepared me for my life now. The great parts taught me to be thankful, loving and loyal the rough patches to be resourceful, compassionate and strong in my faith because through all the rough spots God was there even when I didn't see it that way at the time. Now, I can look back and see His hand in it all and how it prepared me for today. I don't know what Boo's future or any of my children's future holds but I know that God is preparing me and preparing them for it through all of this. He has proved Himself faithful in so many ways, here are a few:

1. A great pediatrician, we hired him for our girls 1 year before Boo was born and never realized just how great a doctor he is until Boo arrived.
2. We got health insurance for the first time in our lives just 6 months and 3 days before Boo was born just making him eligible for coverage which is now passing the 1/2 million mark.
3. Our little house which my Mom and found, bought and remodeled only months before we sold our house and needed a place to live.
4. Sometimes out of the blue and from unexpected places God has paid our expenses whether from a "mystery" giver, a side job, a commission check I'd booked a year in advance or someone from church just wanting to help because they understood the burdens.
5. New friends, I've met some amazing people with even more amazing kids and have been able to minister and be ministered too through our friendships. Proving that good can come from the situation we are in through blessing others.

I do try not to worry about things getting worse. I do think there is a fine line between justified concern and care and worry which I'm sure I cross often but I hope that I can improve that, lay it at my Savior's feet and get some sleep knowing He holds my children in His hands.

Swimming and Sewing

Just when you start to think that things are on the upswing, that life is leveling out, reality hits. Yesterday we were invited to go with friends to a local community pool. Normally we shun public places from Oct-Apr but I thought that with all the bleach and chlorine a pool might not be so bad. Afterall, swimming is an encouraged exercise for kids with low tone like Boo. So off we went. All three kids had a blast in both the kiddy splash pool and the larger family one and it was probably the best family time we've had in awhile. Boo was particularly happy and splashed, kicked and giggled for an hour and a half straight in the 85 degree water. Our friends also have a little boy just two months younger than Boo and this is where the reality part comes to hit home. "O" as we'll call him was off playing in 12inches of water, walking around, running through the fountain, climbing up the sides of the pool and back in again while his mother sat on the sidelines watching him carefully. Boo could hardly walk though the water without loosing his balance and as hard as he tried he could not climb up the sides of the pool, let alone lower himself back in the water. So I gave him my hand and tada! A new confidence was born! As long as he had that bit of balance support he did really well (although he still needed lots of help climbing in and out) He could see what his friend was doing and wanted to try himself.

My heart broke as I watched this unfold around me and realize that while he's made really amazing strides with the O2 he is still not like other's his age.....yet. There is always hope! "O" did not have blotchy red and purple skin from the effects of the body going nutzo over the temp changes water to air, he didn't have everyone staring at his tegaderm taped belly, he wasn't wigging out the highschool age lifeguard who kept looking at us trying to figure out if he was really OK to be swimming, he could climb, walk and navigate the slippery floors without assistance. I can all but guarantee that an hour after getting out of the pool and being warmed up his core body temperature was higher than 96.1 and he probably wasn't having low O2 levels and a heart rate 10bpm lower than his normal low throughout the night and I imagine that if you fed him it would probably digest just fine instead of backing up and sitting there for 2 hours like Boo did. All this to say we have realized that if we are going to go to the pool again we are going to have to change some things. We'll have to take Boo out and warm him up more often, check his vitals and also not stay as long to begin with as it completely wears him out. This is hard because he LOVES it and did not want to sit still for even 2 minutes when I had to retape his tube guard. (we cover his tube site with tegaderm when swimming)  We'll see how it all goes next week and if we can find a happy medium between his health and his happiness. The girls also wouldn't like cutting our time short because of Boo.

On the Mommy front I have been pacing my wardrobe for a week trying to decide what to wear to the hottest date I've had planned in a very long time. Hubs is taking me to the nicest restaurant in town on Saturday night at 8:30pm along with another couple we are good friends with. The restaurant is having a special and our dinner is going to be 1/4th the price of normal so while we'll have to skip our next two date nights, it's within the range of being able to afford it by doing so. This has led me to agonize over what dress to go in. Don't get me wrong, I have a few cocktail dresses that would be perfectly suitable for the occasion that have been sitting in my closet all lonely since before Boo was born. So the problem isn't that I have nothing to wear, it's that I have nothing I WANT to wear. I've always bought clothes that are very classic in style so that I can wear them forever. That is where my problem is. I'd been wearing these dresses over and over and while to the rest of the world they still look really nice and totally Jackie O, to me they look....boring. It's my first big date in forever why wear the same ol, same ol? So I went shopping and bought three dresses in the latest, hippest and most trendy styles and brought them home to try on,  maybe this would give me the inspiration that I needed to figure out what to wear. I kept the bag and the receipt and tried them on. The first one....well lets just say it looked bigger on the hanger, either that or I am no longer as skinny as I once was. The second one was a nice little black dress but perhaps a bit 1996. The third one was the ringer, a dark purple, one shouldered, draping creation that hugged all the right places and none of the wrong ones and was about 6inches shorter that anything I've put on in the last 4 years or so. According to Hubs though it was ringing a few too many of his bells and he thought he might hurt someone if he caught them looking at me in it. (I have to admit, it was cut perfectly to hide what I wanted hidden and not hide the parts of me that still showed well.) Ahhhh so what to do? (I can't actually afford the dress anyway!) If you are me you sit in front of the computer with a piece of fabric from your stash wrapped around you and you sift through the latest Emmy Awards What's Hot list until the light bulb turns on and you head to the sewing machine. 2 hours and 20 minutes later you will emerge with a dress that is trendy, a bit shorter than you expected but doesn't cause the Hubs to want to lock you away from sight. My dress still needs some embelishments added but the structure is there so I'll finish it tomorrow and hopefully get some pictures up after our date. (And after I figure out how to do that on here) Time to sleep busy day ahead!

O2 our friend

So I'll admit, I'm not a great blogger! Seems just when things get stable and I think I can do something in a consistant fashion it gets crazy again. On October 19 we went to see the pediatrician (and to make a long blog shorter) he decided to start Boo on O2. That was the begining of the end for the day because just as we left the office Cleveland Clinic called wanting to know if we could be there in 1 week for his muscle biopsy. Uhhhh sure we can do that. So we started getting things in order, had the ped write up his report and fax it over and did the same at the ENT appoinment the same day. The ENT thought Boo needed a sleep study because he didn't think he was suffering from obstructive sleep apnea due to a throat issue, which was one of the things we were considering as a reason for the O2 need. As we left the office Cleveland called again about the O2 order. They wouldn't sedate him without knowing why he needed it to start with. So they scheduled him to see a pulmo and cardio in Cleveland because the ones here are nearly impossible to get into on short notice. So they canceled the biopsy and scheduled the new appointments for Nov. 1st

This was emotional for me because we had been waiting for the biopsy to be scheduled for such a long time and to have it and then not have it and to be bounced around so much in one day was draining. Because God is always in the details I wondered what we needed to find out before the biopsy.

That was a Tuesday and by the time the medical supply company got it together and delivered the O2, it was Friday evening. They took a monitor download the same day and it showed 33 events over 3 nights. This is not a terrible report, many children will have 33 or more episodes per night but for us it showed that there WAS something going on. After the O2 was delivered we packed up and went to visit my parents 1.5 hrs away. That night we started him on 1 Lpm and the next morning we woke up to a completely different child. The boy we always knew was inside came out. For the first time in his life Boo played without fatigue for FIVE HOURS straight. Normally he wouldn't be able to play for more than 30-45 minutes at a time without a break or a rest and this is in our tiny house where you practically have to crawl over the dining room to get to from the kitchen to the living room. (We placed the O2 concentrator by the fridge and the 25ft of tubing reaches every corner of the house!) My parents home has a flight of stairs and he must have climbed them at least 3 times and ran through the offices, guest space and of course tore up my parent's livingroom. (My parent's home is built over the offices and warehouse of the family business so this is a pretty large running space!) I couldn't believe the change. Day after day it was the same. We kept him on the O2 only at night, naptime and for long car rides and he was doing great! After spending a little over a week with my parents we all left for Cleveland for his appointments.

Since this "update" is turning into an "upbook" I'll shorten it to say that we took the girls with us this time because we were able to confirm a room at the wonderful Ronald McDonald House there the day before, arrived and had a great evening as a family. During the night Boo started struggling with congestion and we had to double his O2 usage. The next morning it was obvious that he was not in the best shape. For that matter neither was I. I didn't think I had much choice other than to press on with the plan though. Hubs was to stay with the girls at the RMH and try and have a fun day together between work phone calls while I shuttled Boo around. Our first appointment was with Cardio at 9:30am for an EKG and Echo. Both of which turned out fine and we were told to come back every year for a check up and not worry about his autonomic heart rate changes unless they stayed high or low "for a long time". Then off to pulmo after a 5 minute coffee break. I have to say, any peds doctor that sports a pink shirt and bright blue polka dot bow tie to his appointments to impress the kids  is a winner in my book. He was really interested in the whole picture and trying to figure out what part he was going to play in it. He also took time to make sure Boo was comfortable during the exam part. He wants a sleep study, a broncoscopy and a chest x-ray when we come back for the biopsy and was all for keeping him on the O2 if it was helping so much. Yay!! Another doctor who trusts parents! During the appointment Boo started screaming in pain and it took me awhile to figure out that he was bloating. I vented his g-port and dark green bile came flowing out. Great, just great, his motility had shut down. I stopped his feeds and dumped the bile and he was much more comfortable in about 10 minutes. The resident had been in the room during this time so got to see first hand what happens when Boo has a cold. I was secretly praying that we wouldn't end up inpatient. We got back to the house and I started him on Pedialyte and he seemed to perk up after a bit. Enough to play in the room while we packed up to leave. I felt SOOOOO guilty having a sick child in the room so we kept him sequestered and after taking him from the room I cleaned, then disinfected every inch and then cleaned again praying the whole time we'd leave no germs.

We went back to my parents as Hubs had more work to do there and ever since it's been like a children's hospital around here. We had to nearly triple Boo's O2 one night and during the times he managed to get his cannula dislodged he'd drop to the 80's. Pudge has also gotten very sick running high fevers and being very lethargic so at one point Hubs considered trying to figure out a splitter for the O2 because Pudge's sats where hoovering in the low 90's but then the motrin kicked in and they went back to normal. So now we know that Motrin has to be kept running for her because if it wears off she goes from sick to very sick fast. Princess has managed to stay fairly healthy and has only developed a dry cough so far.

So that has been the last fortnight here in the Mito Lane. A never ending series of adventures which has required an enormous supply of coffee, chocolate and of course much, much prayer!

Rainy days

I am a sun lover. The hot rays streaming to earth and warming my face while a balmy wind blows is my idea of heaven. When the sun is out I'm the energizer bunny. I bake, sew, play with my kids, catch up on all my house work, make plans for the week and just in general feel great.

Then....it....gets.....cloudy. Or....bracing myself....rains. For me, it's like the batteries get removed. I just want to sleep all day. I've started learning how to channel this. My Grandpa always said that a rainy day is too nice to be wasted on anything but drinking coffee and reading next to a hot wood stove. So I start the coffee pot right away on days like this and then put the kids in the bath tub. I catch up on some reading even if it's just a magazine. This usually wakes me up enough to be somewhat productive for the rest of the day and at least feed the children. Because we live in a state that is chronically cloudy in the winter and will often spend entire weeks without letting even a shimmer of sunlight through the gray skies I have to reinvent myself every fall. This fall is going to be no different because life goes on despite dark dreary weather especially with a Mito child. Boo still needs the same amount of care on a cloudy day as a sunny one. The positives are the cooler weather which he seems to tolerate better. Of course cold weather means sick season around here too which is reason #635 why I do like winter (cold being #1 and cloudy being #2). In fact in my opinion there are only 2 positives about winter...the snow is beautiful and I LOVE the Holidays! We spend from Thanksgiving to Christmas baking, sewing, partying, singing and just in general loving everyone. Although, the past two years the party attending has been preceeded with "is everyone there going to be healthy?" and Boo and I have inevitably had to be left out of a few because there tends to be someone every now in then that insists on coming even though they are sick. I'm not resentful though, I was probably guilty of the same at one point or another not realizing the impact it might have on someone else. It's all part and particle to having a Mito child. If I could put Boo in a bubble from October 1st to April 15th and have him enjoy it I probably would.

For today though, I'm going to rally and do some sewing while the kids take their naps. Either that or bake some more fresh bread. With a recipe this easy why not!?!

Rainy Day Bread
Mix together in bowl:
6 1/2 cups flour
1 1/2 tablespoons salt
1 1/2 tablespoons yeast
Add 3 cups of water and stir until mixed. It will be very sticky. Set in bowl for two hours or overnight.

Grease hands and shape into 2 long loaves on a cookie sheet covered in parchment paper and bake at 450 degrees for 30 minutes. Add a pan of water to the oven to give a great crust. Pull out of oven and let cool near completely before slicing to allow crust to turn soft and chewy. Enjoy!

My Gray Hairs

Actually they are white...about a dozen lovely white hairs sprouted in my bangs about 1 month after Boo was born and haven't gone away. I call it a stress mark. I'm sure after the last couple weeks I'm going to sprout some more although this time I've been chowing down B-complex like they were jelly beans. See this is what happens to you when you have a medically complex child that can't seem to make up his mind about whether or not he is going to be healthy or a bit unstable on any given day. Last week we were inpatient for 37.5 hours because Boo decided to sleep with his oxygen saturation levels (O2) between 90-95% for several hours. This was too low so I was advised to take him in. This was of course at 3 o'clock in the morning, hubby was out of state for business and my two daughters were sound asleep. I called my sister who woke her husband who would meet me in the ER parking lot. I packed a small bag for Boo and I and  woke the girls who handled the situation like champs. (As far as they know doesn't everyone get woken up in the middle of the night because their brother has to go to the hospital?) Of course I carried their half awake little bodies into the car and didn't think about things like shoes or extra clothes for them! Once at the hospital and done with the kid exchange we checked in and were taken back to a room within a couple minutes. Boo was fine once awake of course and the resulting chest X-ray and IV placement was enough to get him screaming. Once he fell back asleep they started to watch him while waiting for the labs. He did fine! After a few short dips to 94% he got up to 97 and stayed there. They thought that perhaps some mucus from the small lung infection they found may have been blocking his airway just enough to cause problems but then got cried out. All the same they admitted him for observation because he has a bad habit of being discharged and then coming back 2 days later to be admitted for a week or two. He was extra sleepy for the next day but otherwise did fine and they sent us home.

At home he continued to do well until a couple days later when he developed a minor cold. Again his O2 was tanking at night but this time we just kept waking him up a little or rubbing his chest. It's kept us out of the hospital. They downloaded the home monitor only to find out NO EVENTS!! I couldn't believe this so after much discussion found out from the nurse that the perimeters for recording O2 events was set WAY too low. The new machine we have is set higher but not as high as I'd like to see. According to an ICU doc he should not have O2 levels less than 94% for more than 3 hours so it would make more sense to me to set it to alarm at 93% than at 90% so we can tell if he is sitting low.

Anyway, this post is getting long so I will summarize it to say that Boo had 2 more terrible nights with frequent drops into the mid to high 80's% which resulted in my husband and I talking about the possible need for night time O2 supplementation and then comes along last night where he stayed above 97 the WHOLE NIGHT! I don't know if it's just  he's getting over  the cold now or if something else set him "off" for a while but now it's gone or what it could be. He's also started to eat cheerios again by the medicine cup full vs 3-4 at a time so something is getting better. We will of course have to continue monitoring him to see if he has more good nights in a row or goes back to what he was doing before which has been an up again/low again cycle for at least a month now.

In other news hubby and Pudge have come down with colds now so we'll see how healthy we can keep Boo since the house is so small "quarantine" is not an option like it was when we had two floors. The good news is that because everyone else needed to stay home just Princess and I went to church this morning...well kinda. She went to Sunday school, I ran the coffee window and we left after she was done. We stopped at our favorite coffee shop on the way home and split a muffin and drink. It was great to spend some one on one time with her again and it gave her a chance to ask me all the questions about Mito that she's been accumulating since we talked last week about a dear new angel named Samuel who had the disease. She's so smart and doesn't even know it! (I mean really, how many six year olds do you know that learn about sub cellular biology?) Well, I suppose this is plenty for now. I'll try to be more consistent with this new blogging thing so my posts don't read like a book all the time! (what can I say...I'm wordy!)

My week

So it's been a week since I've posted, not that I feel all that bad about it because I post when I can or when I feel like it whatever comes first. My week was so fazzled I'm surprised to be fuctioning at all at this point so here goes...

Today was a cold, rainy fall day and since we had gone apple picking yesterday I had promised the girls that we would make an apple pie. What I had not calculated into that promise was the fact that I had 4 loads of laundry to do, 2 sinks full of dishes calling my name, a floor that was missing the broom in a big way, all of the kids clothes to sort, organize and rearrange for the fall while calculating what I still needed to buy at our upcoming Mom2Mom sale on Saturday and oh yes, pack to go camping for the weekend and all before I was supposed to leave to run a couple errands and then meet up with friends for a long awaited "mom's night out". I had also forgotten that my beloved apple pealer, corer, slicer extrodinaire was packed away in our storage trailer 20 minutes away. Pealing, coring and slicing 12 apples by hand can't be that bad right? Wrong, I could have done the job on a whole bushel of apples with the machine for what it took me by hand for 12! But by golly this apple pie was going to be made! Afterall, I had told the girls that it was going to be our school for the day. Princess was to help read the recipe, count out the items, learn about fractions with measuring and organize ingredient categories. Pudge was to learn about different smells and textures, the colors of the different apples and how to properly lick sugared syrup off a spoon. Other then getting himself covered in flour Boo wasn't as interested in the process as much as he was in trying to sneak outside into the rain whenever I had to go out to the garage for something. (Remember, we have no basement so our garage is my storage, extended pantry and cold pack room.) What is it with boys and puddles anyway?!?

Speaking of Boo he had his EMG done on Monday which was....frustrating... It started fine in the prep room and they got the IV in after only the second try so wheeled us into the proceedure room where his IV blew just as they were trying to get the sedation in. He got just enough to get irritated not enough to knock him out. It took FIVE more tries to get it in again. 4 nurses and the sedation doc from PICU were working at it. They had to start O2 because his sats were dropping and his HR was in the 180's. Boo's screaming his head off and I'm ready to cancel the whole thing right before they got it in and the WHOLE TIME this is happening the Neurologist is in the corner of the room talking to his assistant about going to ArtPrize when and where do go for dinner and plans for the weekend blah blah blah. I understand there was nothing he could do to help but gosh either shut up or step out!

So then the test starts, he tests one arm and one leg for the non needle nerve part and three pokes on one leg for the muscle part. He couln't figure out where certain wires connected on the machine and often hovered over buttons and then push a different one and even admitted "We don't use this machine very often so sorry if we seem incompetent".....Say WHAT?!? You are telling me this at the end of a test that my son was just put under nearly an hour for after 7 attempts at an IV?!? This was an important test of Boo's nerve and muscle function that was quite invasive and now I'm not even confident that it will even be accurate! I'm still working on the "your fired!" letter but it's going to be sent! It took Boo twice as long as expected to recover his walking abilities and his O2 levels stayed low normal for 3 days straight. Then I get a call from one of his other specialists saying they want to schedule a repeat MRI and because of the Mito think it should be done under general sedation. Great! But this time let's admit him because I can't handle the stress of the aftermath!

Oh yes, we also heard from the Dr. at NYU who was being consulted about all this possibly NOT being Mito but primary Dysautonomia instead. Her note was pretty short and to summarize it even further she also believes that Boo has Mito and needs the muscle biopsy at Cleveland Clinic. It was the nail in the coffin for Hubby and in a way a confirming blow for me too. This lady knows her stuff, THE best in her field. While we've suspected it for a long time now and he has been treated medically as having Mito, since it's not confirmed we hold on to a lot of hope that it's not but rather something totally curable. As more and more top professionals agree with each other that hope slips away. So I put the call in to get the biopsy scheduled. Then I finished up the pies, sorted the clothes, folded the laundry did the dish, ran my errands, drank my coffee and cried in my car all the way to the restaurant to meet up with my friends. I fixed up my makeup and was fine the rest of the evening. They never even knew, which is what I wanted for tonight. To pretend things were normal. To pretend that I wasn't scared that things might get worse, that Boo's life might be shortened. God is so gracious though and reminded right as I was walking in that He will take care of Boo and me like He always has as long as I trust Him one day at a time.
This was a marathon blog I admit but it's what's going on here and here is busy!

What a day!

The problem with having a hectic life is that sometimes things get forgotten and today I forgot about a playdate that had been planned for nearly a week and I even talked about with my friend at least twice. Good thing it wasn't at my house! Lately it seem that if I don't have a phone reminder set I don't remember anything so I rely on it. Problem with that is you have to remember to plug in the phone to the charger every once in awhile (which I did) AND remember to plug the charger into the wall (which I didn't) this resulted in a dead phone this morning which I didn't realize until much...much...later.

The other thing that threw me off today was a last minute idea to get our car fixed while I was at a doctor's appointment with Boo. This took some logistics, drop the girls off at their Oma's first, get dropped off by hubby at the Docs next, walk 1 mile after the appointment to meet up with hubby so we could walk another mile to our favorite area coffeeshop to wait out the fix. While it was a beautiful sunny day and Boo was loving the scenery I was wishing there were more sidewalks! I ordered my favorite drink known as a Java Slinger and had them add an extra shot of espresso this brought the espresso shot count to five in my 16oz cup. Yes, ladies and gentleman I consider it the best drink known to man because I think the only way you could get more caffeine and sugar simultaneously into your system in under 16oz is by IV.

The day was not without some other upsets, the car cost $200 more to fix then they thought, Boo's feeding pump just kept alarming....and alarming...and alarming. Which got very....very....very....annoying! It was also annoying to have Boo grab the warm milk I ordered in addition to my coffee and spill most of it over himself. He didn't seem to even care.

His appointment had gone well. He's been having some issues with his breathing which we will continue to monitor for now seeing that he has some sort of viral infection going on in his throat although thankfully it is very minor because he isn't running a fever. I'm praying it continues to be minor! Otherwise, if the viral goes away and he is still having some difficulty then it might be part of his autonomic dysregulation. You just never know with Boo! Right now he is sleeping and breathing like he just ran a marathon. Ahhh...sleep...something I should be doing!

Maximizing Space

One of the great things about having a handy hubby is that when I get an idea he can put it into action. Being that our house only has 2 small bedrooms the girls have been sleeping on glorified camping cots and Boo has been sleeping on his mattress in the closet opening turned crib. There is no way twin size bunk beds would fit in their micro sized room and leave any play space worth standing in. Now, there is a corner of their room that had been turned into a closet space with the addition of rods and curtains which is where Boo has been sleeping. I'd gotten this idea in my head before but had waited for a good time to put it into action and as it turned out it was the perfect labor day project. We took the rods and curtains out of the space, spent $53.48 on wood, cut up an old foam mattress to make two smaller ones and installed a triple height custom bunk bed in the 4.9 ft x 2.5ft space. Voila! in 6 hours we had army bunks with a fun twist in that we also put up a cutout of a tree so now they all three sleep very comfortably and compactly in their "treehouse". This has left the entire rest of the room to be used for play space! We aren't quite done with it yet but I am excited to get it just how I want it because it's going to be fabulous! I'll put up pictures if I ever actually get around to painting it!