Updates W/ Pictures

What a week. We went to Wichita for thanksgiving and had a nice time. We spent time with family, did some basic christmas shopping, family photos along with Eli's 2 year pictures, eli's 2nd birthday party, and Bob and I even snuck in a date night.
Here are a few pictures from our weekend.

Then we got back to KC and Eli decided to remind us in a BIG way that we were back to the real world.  Sunday evening his blood sugar dropped and he was super fussy.  He was being put to bed and his feeding tube got ripped out.  I was able to get a G tube in and off to the hospital Eli and I went.  They immediately got an IV going with fluids and sugar.  The ER doc begged IR to come in so that Eli wouldn't have to be admitted during winter sick season.  He came in and got the tube replaced.  He ran the contrast dye in to check placement and accidentally ran it into the G tube side ( Eli's stomach.)  Eli choked and gagged.  The next morning Eli woke up with a fever, high heartrate, super fast respirations and a yucky sound in his lungs.  We had an appt with GI so he ordered chest xrays.  He also had us meet with the nutritionist as Eli is again losing weight.  Right now they are more worried about his fluid needs than his calories.  He's already getting twice the protein needs and 30% more calories.  He's getting a ton of fluid but still showing "dry" on all of his labs, even though he is constantly swollen and puffy.  The GI is also concerned about Eli's fundo and is ordering an EGD to be done in january.  Then we went for chest xrays.  They showed ok, although his pulmnologist says it sometimes takes time for aspiration to show up on film.  By the afternoon when his pulm called Eli's heartrate was 170 plus, and his respirations were between 45 and 60.  Doc said to put him on O2 and attempt to ride it out at home.  Eli couldn't handle a lower level O2 so is on double his norm.  Everytime we've tried to wean  him off his HR and resps shoot back up.  He's super tired and has been resting alot. 
We are holding well at home currently but concerned about the fact that we can't seem to get back off the O2. 
Eli also met with the dermatologist over a rash on his back and neck and a different type of rash on his head and ear.  The doc talked about what they were and what to do about them.  So now Eli has 2 new meds to help with all of that.
Here are a few pics of Eli these last few days.

The rest of us are ok.  Exhausted.  The kids are back into the full swing of school and life.  Bob is keeping up well with them and the house.  I'm staying busy keeping up with Eli and all of these things going on.

We are still awaiting Eli's care conference.  They've decided to invite a larger group of his docs and this takes a longer period of time.  We get more nervous by the day, especially watching Eli struggle.

Tomorrow is a HUGE day for our family.  Eli will be 2.  We are so blessed to be at this point and we cherish each and every day. 
Here is a pic of my little man taken this weekend by an amazing photographer!

I promise to get more updates up soon.  The kids have all just walked in the door from school and I need to get some more done around here.

Words

Eli's rough stretch is still going strong. He had a rough night last night and beeped alot. This morning after being off his feeds for 1 1/2 hrs his blood sugar was 171. Which for Eli is ridiculously high. Endocrine called. We chatted. A one time sugar like that isn't to concerning but if it continues they want to begin further testing and his HGH (human growth hormone) will need to be re-evaluated.

His new bed also came today. It got here early so he was able to take his 1st morning nap on it. He did great! Stayed perfectly in the right position and slept well with only ONE O2 drop!!! We are looking forward to seeing how he does tonight.

His primary medically complex doc called me to chat. Never a good sign. He was concerned about the fact that he got a refill request for the robinul. He thought it had been stopped. I was told it wasn't going to stop until we had the care conference and had a clear plan in place. He said that they were working to get the care conference set up and that there was more to the trach than because of Eli's need for the robinul to be stopped. They want it for secretions, for his central mixed sleep apnea, and apparently because they are feeling the need to get a clear cut long term care plan in place for Eli as they still strongly believe that the underlying issue here is progression. From the sounds of his doctor they have all already made their choices and are just hoping to get Bob and I on board.

This worries me. It worries me that they are planning for and thinking ahead. I know that "ahead" is there but I try not to think about it, I sure don't plan for it....Okay that is maybe a lie...I sure don't plan for it out loud. Please don't crucify me for saying that, but it's true. As a mother of a kiddo like Eli I know what the future can hold, what it is predicted (guessed) to hold, and what the possibilities are. There aren't scenarios rushing thru my mind of Eli walking across the stage with his diploma in hand, walking down the aisle with his bride on his arm, or being present at my funeral.

In the top of my closet there is a box, the weight of it is immense. It is full of books, books given to us by the hospital, by social worker and doctors and nurses. Bob and I know they are there. They are for that moment, that time where life is uncertain, where there is no turning back, no fixing it. They are written for children, they are to help understand life and death and all that is involved. The thought of them terrifies me on a daily basis. As they and EVERYTHING else are always in the back of my mind.

What terrifies me even more is that the doctors are so willing to talk about and plan for the future. They say these things outloud. They say them to me and Bob, they write them on Eli's charts, they discuss them together with all of his care team. They try to keep us grounded, keep us knowing, understanding. They try to push us to think like them, to talk like them.

I can't do that. Each conversation takes another piece of me, it takes another piece of my little man. Words spoken can never be taken back. I don't want these talks, I don't want to be grounded. I want to wrap my arms around my little man and my family and float off into the clouds, flying freely all around, immune to "care plan meetings".

This is what I want. This is not what I will get. I will prepare myself. I will prepare the only way I know how. I will go to the meeting and listen, I will talk, I will argue and push, I will negotiate and try to compromise, I will beg and plead, I will present evidence and emotion, tears will fall freely...Ultimately I will not win, my little man will not win, in all honesty the doctors will not win either, Mito will win...After the meeting I will return home and do the only thing I know to do, crumple into my husbands arms, cuddling my little man until his head is soaked in my tears....I will talk to those families closest to us, those who understand what it's like to never speak these horrible thoughts outloud....

....and finally then I will make formula, pull meds, clean syringes, run spo2 monitors, apnea monitors, bipaps, O2, walkers, wheelchairs, OT, ST, PT, sign language, AFO's, SMO's, glasses, hearing aids, and books....Eli's favorite....books. I will read them, touching the pages, signing the pictures he sees, laughing with him, hiding behind them, and reading them over and over until they are so worn out that we have to re-buy them. After so many books the words from the meeting begin to fade and the lines begin to fade on if they were actually spoken or if they solely existed inside of my head....This will begin to help the pain again fade back to manageable...until that next dreadful meeting.

The cycle is torturous....for bob and i, for our older 4 children, for our families and friends, for Eli's doctors and nurses and therapists....But we push forward, just as Eli does in his day to day life. Eli is truly my hero. He is the strongest person I have ever encountered. My children are strong and amazing and supportive and so madly in love with their little brother. Our community is amazing and without them we wouldn't be able to make it thru each day. I hope one day I can be as strong for all of you out there as you all are for me, for my family, for my little man. Thank you.

Individual updates

I apologize for how far behind I am. So much has happened and it has been hard for me to actually sit down and put it all together.
Things won't really be in chronological order as it's to much to keep track of that way.

Eli ~ We have met with the pulmonologist, cardiologist, and the ENT. They are all agreeing that the trach is the way to go. The ENT also is considering tubes in his ears. His left ear drum isn't moving and it is a different color and the doc believes it has fluid trapped behind it. He will most likely do the tubes during Eli's surgery in January. He will also either do a full scope or the trach, depending on the final decision. We are still awaiting the care conference. Eli also had a rough week last week. He stopped peeing and pooping. He also had a seizure. The first day he lost 17 oz, then gained 20, then lost 18, then gained and lost some more. He ended up in the ER on friday after no pee and the seizure. Lots of labs and IV fluids. He finally started peeing but it still isn't back to normal. He also finally had a movement after 4 1/2 days but again still not back to normal. He's been incredibly tired and has had alot of temperature maintenance issues. We aren't sure what is going on with him and we are hoping that it regulates itself quickly, because the doctors also have no idea what is going on.
In other big Eli news he is getting his Comfy Lift Bed!!! Some amazing Mito moms got together and held a fundraiser for my little man. They had the entire bed, plus shipping, plus a spare cover paid for in right over 24 hrs!! We are so blessed and so damn lucky to have such an amazing support system in our lives! I can't imagine this journey without them.

Emily ~ Life is fun with a preteen. I don't know how many eye rolls, hair flips, sighs, and her ignoring us that I can deal with. She can be the sweetest most helpful amazing girl and the next minute she can be a holy terror. It's fun times all around. She's trying to stretch her independence and we allow her as much space as possible. She now has her ears double pierced, wears strange feathers in her hair, wears sleeveless gloves, is addicted to black and neon green, and listens to music I have never even heard off! It's interesting to say the least. We give her this freedom as long as she keeps up with her responsibilities. She's still maintaining all A's and is keeping up her extras. She is in all the advanced programs, as well as now helping out every Friday in a kindergarten classroom with some special needs kiddos. She's very active in student counsel still and enjoying that. She's loving choir, and has even been selected for a solo in the Christmas concert. She's loving violin, and is preparing for her first strings concert as well. In other words she stays super busy with school, activities, and driving her parents insane!

Jace ~ He's doing decent. His meds seem to go in waves on if they are working well or not. We aren't sure why that is. We will be meeting with both of his primary doctors next week to discuss how the meds are going. His new IEP is in place and we are hoping to start seeing some changes. He's struggling a bit with Speech as he gets older. His stubbornness is starting to shine thru and his ST has had some issues getting him to participate and work hard. He will start his state testing in December and with the new plans in place we are hoping to see some improvement. He's still enjoying school and we are so thankful for that. He is finding some new activities to enjoy around the house as well, one of those being some craft projects and music! He also loves his playtime with Eli. They play brotherly rough and tough, which normally involves flying thru the house on Eli's car (with his helmet on of course). Eli loves this time and so does Jace!

Benjamin ~ He's doing decent. This last week he had a weight loss BUT all in all he has still gained 4 1/2 lbs. Which for Ben is HUGE. He's still horribly underweight but a gain is a gain! We are still trying to find ways to get more water into him, that has definitely been a slower process. He's doing ok in school, but still struggling to stay focused sometimes. He's doing decent with his spelling words, but not so great getting his homework done. He has made a friend at school it seems and has come home a few times very excited about playing with him. He's spending alot of time playing the Wii still and is loving it! He has also figured out some of the games on our cell phones and enjoys that as well. He's been working on some basic self help goals, mainly showering and getting dressed in the morning. He's not there yet but doing well learning!

Ariana ~ She's absolutely loving school! She has made many friends and enjoys the activities and the interactions. She's learning some new things and is very happy with it all! When she's home she enjoys playing with her dolls and has been enjoying some of the Wii games. Her favorite game is JumpStart and she's learning so much with it. She loves her mornings with the big kids gone so she can rule the big TV and do her things. She's still girly as ever and that's always interesting for the rest of us. Her hair is now halfway down her back and she loves it! Always fun for the rest of us to get to maintain it though!

Bob and I ~ It's always so hard to say how we are. Bob has had some major GI flair ups and has even ended up in the ER. Changed around some of his meds and it's doing much better. I've had multiple migraines lately. We've been busy with the kids and the house. I've been working my way thru 5 quilts, so far I have 3 1/2 done. I'm also working on a crochetted blanket for Eli. I even read a book this month!

This weekend we are going to go to Wichita for thanksgiving! It will be nice to get away for a few days and visit with family and friends. We will also celebrate Eli's birthday as he will be 2 on December 1st. My how time flies! More updates this weekend!

Cardiologist

Last night was rough. Eli's apnea monitor alarmed 28 times for high heart rate. Today he had his appt with the cardiologist. They did his ECHO and then we met with the cardiologist. The ECHO looked good. The clot is definitely gone and the calcified leason is mostly gone as well. Now it's simply scar tissue and a thickening of the heart wall there. The heartrate was again high.
The cardiologist agress that the robanol is causing heart issues. He wants him off of it. He also believes that Eli's trouble with secretions is causing a small amount of respiratory distress and causing an increase in heart rate as well. He says meds to lower the heart rate are going to have to many side effects for a kiddo like Eli. He's also very concerned about Eli's risk of developing cardiac myopathy. He says that basically each heart is born with so many beats. Eli is plowing thru his heartbeats at a ridiculously fast rate. Long term this is obviously a major issue and by long term he means we will begin seeing effects from it fairly soon. He agrees that a trach is the best option. He will obviously continue to follow Eli during the whole next stage of the process and continue to closely monitor his heart thru all of these changes.
So it seems as though we are basically out of choices. It's rough.
We are working thru all of our thoughts and feeling and fears.
Tonight is Emily's choir conference so we are going to try to go and simply enjoy that and try not to think about it all.
More updates tomorrow.

Ortho and quick updates

We had a very quiet weekend. Saturday evening Bob and I went out for dinner, shopping and a movie. It was sooo nice to get 6 hrs out alone. Very needed after these last few weeks.
Sunday we all laid incredibly low and didn't do a darned thing.
Today we have a new nurse shadowing. This morning was slow letting her get used to Eli. Eli had therapy with his speech path plus his sign language teacher. His IPAD was approved!!! Plus all of the extras that were requested. We are super excited!

Then this afternoon he had his appt with his new orthopedic surgeon. She says that when his muscles were growing in utero and as a baby they weren't able to grow properly which made his bones distorted. She says that ultimately he needs surgery. What she will do is cut both femurs in half and then rotate them to the proper place. Then they will be anchored down with metal plates and rods. Then he will have leg casts while they heal. She said that right now she wants to wait until he's a bit stronger. She's concerned that doing it now will cause way to far of a setback muscle wise. We are waiting to hear back on his care meeting for the trach. hoping to hear something tomorrow.

This evening Ari fell and got hurt. She was going over the baby gate and slipped and fell backwards hitting the back of her head on the tile floor. She has a massive knot and cried for a good 45 minutes. Alot of ice. No signs of concussion or anything else. We will wake her multiple times during the night, but I have no doubt that she will still have quite the headache come morning.

Tomorrow we meet with Eli's cardiologist. I'm anxiously awaiting this appt. This is the first of many times the trach will be seriously discussed, and his answers tomorrow will almost make our decision for us. I'm so damn nervous.
I will update tomorrow as soon as I know more!

Phone call with pulmonology

Just when I think I'm as emotionally drained as is imaginably possible I'm hit again.

We have been working closely with Eli's docs to figure out the secretion issues and the medication issues. The robanol is definitely helping with secretions but it's also causing issues with Eli's heart. Ultimately Eli needs to be taken off the robanol. Eli's pulmonologist called me today and chatted with me for almost 20 minutes. He believes that with Eli's diagnosis of Mito(and the progression involved), plus his esophageal motility, plus the hypotonia and the respiratory issues that we are looking at the need for a long term permanent fix. He told me he feels a very strong connection to Eli and our family and that he really wanted to talk to me in person but he believes we are hitting the point where we need to have a care team meeting and discuss a trach. He believes that this is going to be a more permanent solution for Eli that helps protect his heart. I began crying immediately and the compassion in his voice was overwhelming. He really really cares about what is best for Eli and for our family. He is calling together eli's primary docs as well as his geneticist and cardiologist and wants to sit down with us and go over what's going to be best.
I'm terrified. This is something we have been warned could be discussed some day but we didn't expect it this soon. We are going to obviously be questioning everything and push on what other less invasive options are available. In the mean time his pulmonologist is calling all of the docs and is going to check in with me on monday to get this all set up.
I will update more as we know more.

Burden of Life

Wow. I don't even know where to start. It's been a rough week for me.
The weight of responsibility of raising a kiddo like eli has hit me hard this week. I'm exhausted. Every moment of every day I have to be ready to give every drop of his medical history in order with ALL of the correct info, all of his surgeries and procedures, every name and phone number of every doctor(35)/nurse(2 in home and over 40 in office)and/therapist(8), every medication with concentration, dosage and time, and every single drop of info about blood sugar, swelling, seizures, sleep, vital and EVERYTHING else. If I miss ONE single fact it can be my childs life. To some this may sound like overkill but it's not. This is life with a complex kiddo like Eli. I have to maintain PERFECTION. So far I've not missed a single detail. I've always remembered every single thing at the exact moment that I needed it. I've always been able to have it all right there.
That being said though I'm TIRED. Beyond tired. Exhausted. My brain and body are nearing the end of what they can handle. I have maintained perfection for 23 months and I'm soooo scared I can't continue to do it much longer.
I have lost every drop of me and I have become an extension of Eli. I am Eli. Every doctors appt, every therapy appt, every progress or setback...the doctors and nurses praise me for how good I'm doing, they remind me that I'm the reason that Eli is still alive. I wholeheartedly believe that BUT it's such a huge burden to carry. It's lonely and exhausting and painful. oh so fucking painful.
For some of you I know this post won't make much sense, for some of you I'm sure you'll be nodding your head with tears in your eyes. I totally get that. I would have never understood until I'm living this life.
I sit here and look at Eli and tears automatically come to my eyes, my breath catches, my heart skips a beat. This happens virtually every time I look at him. The bond I have with him is amazing and I'm sooo very thankful for that. The problem is at the same time I almost find it hard to breath because the weight of it all comes crashing down upon me in waves. Sometimes my heart hurts so bad I can hardly stand to look at him and I briefly have to look away.

No family should have to live like this. It's not just rough on me. It's hard on Bob and the oldest 4 as well, just in a different way. Eli is my primary responsibility, leaving Bob primary caregiver for the oldest 4. That's not to say that I'm not involved in their care as well, because I am. But primary doesn't fall on me. It can't.

The top of my blog says "We've got what it takes, but it will take everything we've got." This is so true. I know deep down in my heart that we will make it thru this, but I'm so scared to know what will be left of me. I don't know who I am anymore outside of Eli. I don't know what to do with real "non-medical" life anymore. Life IS medical, beeps, medications, abbreviations, diagnosis, schedules, vitals, and dme's.

Life is keeping my baby alive.