Surgery here we come

I say surgery here we come with feelings of anticipation for a better future and feelings of fright for the worst case scenario.  The trach fistula has been colonized with MRSA and Staph which has been wreaking havoc on Meg for months.  After we had to cancel a long awaited trip to Minnesota's Mayo Clinic due to her waking up with every color coming out of her trach it was the last bout that her ENT could stand either.  The decision was made that if the infection came back after the last treatement that ended in early December the stoma would have to be closed. We have been running Tobi Nebs for over a month and had to start the bactrim again 2 weeks ago and it is  not doing much.  Surgery is set for the second Wednesday in January to sew her up.  The procedure requires intubation which is what makes me worry - we dont want to end up back where we came from and dont want to face "big" decisions.  She could never go through a trach again - we would not allow it for her.    Christmas came and went fast - it was very wierd to have a nurse in the house in the morning in the middle of our Christmas.  The boys were wigged out.  Better that I got to sleep though instead of being like a zombie in front of the Christmas tree.  I had to explain that to them so they could see that it was a necessary component of life for us to function and it is not what we "love" either.   We have a hearing coming up for nursing hours soon - had to appeal a riduculous decision that came down right before Christmas.  It is always something! Praying for a successful surgery and a great 2011 for Megan.

Dream Calendar

Megan's Dream Calendar Photo.  Well - I did not know it when we shot the session but we were just hours away to being told she was very sick with MRSA.  No wonder we could not get a smile and she was a bit wilted.  If it had been a week later it would have been great.  Oh well - I still love it!

Minnesota Bound

So the last month has been nuts.  We have determined that Megans trach site and right ear have been infected with MRSA and some other strange bacteria...she has been battling the dog ear smell as I call it since the end of July.  She has been treated with a/biotics but it kept returning.  Culture finally found it.  Infections Disease was lame about culturing - they said that of course it would grow bacterias because there would be skin flora in it too...ENT did it and figured it out.    We spent two days in the PICU for a new EEG with an epileptologist.  Seizures are off the charts again and have seen some infantile spasms.  No wonder with a raging infection...that is what happens.  Anything that stresses the body lowers the seizure threshhold and it creates a bunch of problems.  She finished her antibiotic this morning and I have seen some smiles in the last three days which means she is feeling better.  We reculture Tuesday.

Also have had new hip x rays and both hips are popping in and out.....we now will have to see the orthopedist for follow up and whatever else..I know it hurts.  She has been letting out some blood curdling screams.  But we will be going ahead with some botox in the legs to help relieve some of her muscle spasms that make the hip thing worse.  Cant do it until January 14th though - that is the next opening.  I guess it will go fast with the holidays coming and all.

This Saturday the 13th we are heading to Mayo Clinic for them to have a look and see at Meg and maybe get some new ideas - help??? I dont know - cant hurt to try and I have been having the feeling to do this for the last 2 years.  So I took the inititiative and faxed her information to them and they called me back and said - yup - we would like to consult with you on her.  It all fit in perfectly and it was so easy it must be meant to be.  I am always hopeful.  Like I said - nothing to lose and certainly she needs medical expertise far greater than I can get here.  They dont understand her at all.  What gets me is that because she is so pretty I get the stupid comments like "at least sheis pretty"  or they think she is not sick because she is not deformed or characteristic looking of a syndrome.....ignorance I guess!

One more reason to make this trip happen is Ryans prayer for Megan. 
Dear God, I hope that there is a cure for Megans mitochondrial disease in 2011 or 2012, if she is still alive to get the cure, and I hope I win my soccer game next Saturday.  Amen.

Talk about making you want to cry - what a deep thought process going on there. 

Ryan is finally wanting to participate in things outside of our house.  He is playing soccer and loving it.  Im so glad he does not feel like he has to be here all the time in case something happens.  James is loving high school.  He is experiencing a little freedom that is new.  I remember feeling too big for my britches and having to be reigned in a bit and now I am doing it to him.  But I must say he is much more understanding of it than I was.  I think girls must be worse.  His back brace should be able to come off around Thanksgiving.  He goes to the ortho surgeon a couple of days before and I hope his fracture has healed.  Then well start PT for his core muscles since he has not used them in several months.  Another set of appointments!Uhhh!

Well that is about all I can think of.  Anyway have to go start meds for Meg and vest treatments, etc.  She is awake and waiting!

Can't believe it is already October in a few days.  Where does the time go?  This past month has come and gone so fast my head is spinning.  I guess Megan being sick all month on and off , mostly on - has made it a bit more blurry than usual.  Hoping we are on the mend now finally.  She went through Rocephin shots (3), 10 days of Levaquin and has about 4 more days of Tobi Nebs left.  If this doesn't do the trick I guess we will talk to infectious disease about an admit for IV a/biotics.  Yuck - makes me sick to even think about a hospital stay!!  Ryan and James are taking turns with their stuff - who is sick, who hates school - who lost a binder with all of their work.  My patience for this stuff is very short these days.  Some of the stuff that is coming home from school is just absolutely riduculous. 

Still feeling kind of out of sorts with everything.  I am in a spot I don't really recongnize.  Not miserable - not happy - not anything.  Maybe just here....

I know what my responsibilities are - who and what I am responsible for and I am getting the job done.  I wish I could say I was getting it done with more UMPH though instead of just getting it done.. My get up and go got up and went???  That is what it feels like.  I don't like it so I have to fix this.  I want to feel connected to everything instead of just existing FOR everything. 

The next couple of weeks are more follow up visits, blah, blah, blah.

Ryan starts soccer this coming Monday - that is exciting - it will be his first team and I can't wait to see him play.  James is in a Boston Brace - dont know if I said that before.  He fractured his spine between L4 and L5 - probably in football but who really knows.  He hates it.  It looks like a medieval torture vest!!

That's all I've got!!!

Tragic Widnes tot Leoni's short life may lead to cure of rare condition - Runcorn and Widnes Weekly News

Tragic Widnes tot Leoni's short life may lead to cure of rare condition - Runcorn and Widnes Weekly News

Another one gone from a mitochondrial disease - two days shy of her fourth birthday. Crying for another lost little girl .

????

I dont even know what to write anymore. I dont even know what to say when people say - I hear Megan is doing great. It is one of the hardest things to answer without sounding like I am wallowing in her disease or a totally negative unhappy, unappreciative person. You would think this should be one of the easiest things to respond to....Great is not a word I would use to describe how Megan is doing...and I feel like an ass for even saying that. While I KNOW that she has made huge progress from last year with coming off the vent and having her trach removed, there is so much still going on every day. Seizures just plain suck. I am not even counting them through the day anymore. It is too sad because I know they are peeling away at her and we have tried everything to stop them. And we have the muscle spasms where she is stiff as a board and crooked all at the same time. It is getting very difficult to hold her and comfort her through these because of her size and her strength. She has a very strong right fist..I have great bruises to prove it. The crying fits from pain are also very painful, for all of us. I am so angry that nobody can stop her pain. It is so unfair to watch this. Yes - I am asking myself quite often why, why, why? And the disgusting stoma that wont close is driving her crazy - and me - it is an oozing snot hole that gets clogged up - so gross. I think she feels like her air is stifled at times because of it and it stresses her out, which then leads to the muscle spasm, which then leads to a seizure, and crying, and on and on - every day - all day - throughout the day. God help her if she does not go to the bathroom in the morning - we just add the stomach discomfort in for some extra fun. I probably sound like an insane person but it is how I feel. It's one of those days that I have had it with all of this and dont know what to do anymore which then makes me feel guilty because in other aspects of my life I know I have so much. Guess I might kind of be feeling like I'll be punished someway for feeling so pissed off. I really feel like punching my fist through a wall and screaming until I have no voice left. Ryan was trying to read Ripleys Believe It or Not to me tonight but couldn't because of Megan's screaming - what else is new. He takes the backseat again.....which I'm noticing is starting to wear on him. He feels unimportant. Sandwiched between James who is the oldest, in high school - funny, and between Megan who is so pretty and so sick and needs so much. I guess I would feel that way too - middle child and 4 years of this.

We do have some nursing and I get to sleep at night now. Huge bonus. It is helping me to feel more human. Day nursing is not so great I find. They have NOOOO clue what to do with Megan. The nurses that are here for a few hours a day seem to think if we just keep pumping drugs into her and request more and more, stronger and stronger it will help her. So what - drug her up like a patient in a mental hospital???? Would they drug their kid up that way? I think not. She is cognitive of her surroundings and loves, loves, loves me. So lets take that away from her too....they are callous and ignorant mostly. But I am in a catch 22 - I can not do this without people that can work a gtube, feeding pump, nebs, meds....a second person to help carry all of her crap to ALLof her 10,000 appts a week...it is exhausting. And it is stressful to have to manage all of their personalities - they are all women so lets face it - it gets interesting. Of course there is always the sunny disposition I am always supposed to have with them or else I am a bitch. It is hard to always have the sunny face for everyone around me - my kids, my husband, my mom and mother in law, nurses, doctors, therapists...........AAAAAAAHHHHHHHH! I just want to be left alone.

Ill figure it out - probably by tomorrow morning, and hopefully I'll deal well for another while...

I absolutely love Pat and all of my children and the rest of my family and would die for any of them- it all just is a bit much sometimes, thats all.

Is summer over yet?

 

Okay – I know it is August in Florida but I could not resist this winter hat.  Megan may get to wear it a couple of times this coming winter….  The good news about this hat is it is actually a size 3 which is about the right size for her age. We have always had to buy hats in the 6-12 month or 12-18 month size until recently! I think that is a good sign as far as head growth since I have been told it is too small for the rest of her!

James started high school last week and Ryan starts 3rd grade next week.  Looking forward to some quieter days without any boys fighting!

We just got back from the trip to Atlanta with the geneticist that specializes in mitochondrial, metabolic and inborn errors of metabolism. She gave us the swab kit that we will do early next week and send off to Philadelphia for enzyme studies. So far all of the enzyme studies done have matched the muscle biopsy results on other patients with the exception of two - which were both coincidentally processed at the same lab. Sounds like a lab problem on those two. I guess I will be looking for the enzyme studies to show the mito complex IV deficiency again. Hopefully it wont be adding any other complexes to our diagnosis. I will have to schedule Megan for an updated EEG, MRI and MRS. Like what we did in NY 2 years ago. It is important for diagnostic reasons. Right now having this blanket mito label is like being told you have cancer but that is it - that is all you get - no type....

The Dr. does think that she fits the Leighs Disease which is what I have always thought. It is not what I wanted to hear though. The prognosis is very poor. The majority of the children die within one year of onset of symptoms. Others live a few more....7 would be considered old for this disease although there are a couple I have heard of at 9 and 11. Needless to say I am very sad inside and I hurt so much inside.

I swear Megan gripped me tonight tighter than I have ever felt and I dont think it was involuntary muscle contractions. She had her little grin on. I hugged her so tight and kissed her little cheek and told her how much I love her and how special she is. She looked so content and like she understood somehow what I was saying or understood my emotions. I pray that she did and that she knows how much I love her. To look at her lying still sleeping you would never guess anything is broken. She is so perfect and pretty. And soon to be four. My mind started to wander today looking at the boys. I was thinking what age will they be when they lose their sister - how will they deal with it and that it is not a question of if they will lose her, but when will they lose her, and her Daddy - WHEN WILL I LOSE HER??????? I feel bad to even think it - let alone put it in writing. It feels like I am deserting her but I am not. I never will. I need to find my faith and trust that God will do what is best for her and for us and love her as much as I can for as long as possible.

Even though I was sad at the end of the stay in Atlanta, it was good to see Gwen, Mike and Erin. It was wierd with Amber not living there anymore but that is what happens - they grow up and move out. The boys had a ball with Aunt Gwen. While Pat and I took Megan to the Dr. they went to Stone Mountain with Gwen. At the top of the mountain carved in was GARRETT M. Ryan saw it and Gwen said he told her that it was for Megan Garrett. I thought that was really cool that of all things that was at the top, and that they saw it and got a picture of it! I guess she was kind of there with them in a way too.

Summer Already

Cant believe school got out for the boys last Thursday and summer has begun. The month of May was really busy with end of the year awards and ceremonies so I have not had much time to do much of anything.

Megan is doing okay so far with the trach gone. We have had some trials already that although were scary proved once again she is tough. A week after she was decannulated she came down with bacterial bronchitis - compliments of Daddy and James. James was down with it bad so I was really afraid of how it would affect Meg. She did need three abiotics but the fact that she did not need oxygen or breathing support via equipment OR a hospital is what counts. I spent an entire week doing nebs and vest therapy. I dont mean periodically either. Every two hours I did nebs and then vest that takes 30 minutes. So it was an hour of treatments every other hour. I think I almost went crazy at one point but reminded myself that it was okay since we were not in the hospital and at least at home doing it. NOOOOOOOOOOO sleep that entire week did not do much to help ease the craze.

Her stoma is almost closed. It is a little pinhole now and hopefully it will close over the next few months. Dr. does not want to stitch yet since it is invasive and requires a two day stay and cutting the skin inside the throat behind the stoma. I agree that she does not need that and we dont need to borrow trouble. She is getting so much stronger in the neck and trunk since the trach is gone. Sits up nice and straight and is activating her own head movement. It is subtle movements up and to the sides but from nothing that is huge. Keto diet is coming along. She is on ketocal now which is milk based and digesting it and growing!!! She is getting nice and full looking. Heavy feeling too.

Make a Wish was here last night to meet with us and we decided on a room redo. New wood floors instead of carpet that is gross from formula and meds. A beautiful outdoor fairy wall mural - some built in shelving to the cieling around the window. A beautiful little girls palace with much more space and hidden medical stuff. I get to relive my dream room with Megan. I am excited.

Traveling to Atlanta the week of the 28th to see the geneticist. Brand new buccal swab DNA test that screens for about 600 different metabolic mutations. Her mito geneticist is certain that a metabolic condition triggered the mitochondrial damage. Although the mito damage is the secondary disease it is the bigger problem not due to the nature of it. If we can find the metabolic primary portion - who knows. Maybe stem cells?? I have hers and Ryan's cord blood stored. Another big deal.

All in all it is moving along. Of course out daily challenges are still large they are much more manageable. Especially since we can move much more freely without equipment. Only a feeding pump and diaper bag. Woohoo!!!!

I know this is a run on but it has been soooooooooooo long.

Bye Bye Trach

Decannulation done.  Trach out as of 4pm on April 26th.  Hello Kitty is protecting the stoma which will take about 3-5 months to close, unless ENT stitches it shut first which is what I am hoping for.  We see him tomorrow morning.  Feel like a ton has been lifted off of us – and Megan looks so much better.  Swallowing everything is a bit of a challenge and so is clearing the stuff in her chest, but I think she is doing okay .  Really nice to hold her without something getting in the way.  Tomorrow is Pat and my anniversary and i cant think of a better present.