I often struggle with the title. But if I had to describe this week, the first word that comes to mind is "Overwhelmed". Of course Sunday's are always crazy around here especially when I don't have a nurse. Isaiah is either octopus (grabby boy of all tubes, meaning Avery's or retch boy).. This Sunday was a combo. And Avery was his usual "hot mess". I have to say that I truly miss my night. Getting the boys ready 7 days a week myself is a lot of work. All I have to say is thank god I have short hair and only take about 15 mins to get ready for work. Because I don't have time to do much more than that.

Work has been crazy for me. I have been seeing on average 17 patients plus doing some things on the side. Basically I could work 7 days a wk. with the workload I have right now. Every time I think I am ahead with work I get another few requests to be seen which means I have to drive at least an hr. to see one pt. Not to mention with all the snow days I've had to cancel more. Which means I have to work on the weekend to make up.

Meanwhile, Mr. Avery decides to act up during a pedi appt. So we are supposed to be going inpatient for 24-48 hrs for an EEG. The hospital is so short on beds and frankly I would not mind if they bumped us for kids who really need the beds. I feel that if Avery is having seizures, he has probably having them for sometime. They don't seem to be bothering him either. Although I do have my doubts????? A couple of my nurses and my mother in law feel that they have seen him having absent type seizures but I guess I have not noticed them. Almost wish they could put him back on the klonopin and see if things get better. I mean he has already been on it so it can't hurt right?? I just hate the thought of him inpatient when he is doing so well only to put him at risk of catching something else. Ok so maybe I am just being stubborn b/c we have been on such a great hospital free stretch and it is hard to go back even for a day or two.


So if we end up going in on Thursday we will hopefully be out by sat. On Sunday I am taking both boys to Rick's basketball game. It will be their first game ever. Hoping they are both feeling good and happy to see their dad doing his thing :)'

So much for "Simple Pediatrician Well Visits"

Avery was scheduled for his yearly visit with his pedi. yesterday.  Honestly, I was thinking I should cancel b/c of all the snow and my work schedule is just so crazy.  Plus, just as Isaiah was getting off to school the elevator lift was frozen.  So I thought there was no way I could go.  We ended up getting it working and off to school he went and off to the pedi. for us.  After being stuck in about 20 traffic jams we made it there just on time.  So I guess it was meant to be!!!!!!

Right away his pedi. noticed how great Avery looked.  He really doesn't see Avery much b/c if he is sick, he usually ends up inpatient.  We only make it in once in awhile.  Luckily it was booked for an hr. b/c we used that and more.  I really like his dr. b/c he is very thorough.  He literally starts from head to toe with every body system and adds in schooling and palliative care stuff.  So you can imagine the visits take awhile. 

I will start with the good stuff.  Again he thought Avery looked and sounded really great.  Best he ever heard his lungs.  YAY!! He ordered a slew of labs b/c Avey has not had them done since Thanksgiving. Got all the results today and everything looks incredible. Wooohooo.  His hemoglobin is better than mine and all his electrolytes and liver look perfect.  I was most impressed with his IGg level (checks his immune system).  It appears the weekly sub q IVIG he is getting is working wonders.  His levels were over 850 which is something we have not seen in a long time.  This is why he has had such a long hiatus from the hospital b.c his immune system is doing to well.  And it looks like his TPN is doing nothing but good for him which is great after being on it full time for over 2 yrs.  Another Woooohooo!!!!!!!

Onto the not so good stuff....One noticeable thing is Avery's back, poor kid almost looks like the Hunchback of Norte Dame :)  He is rotated, has severe scoliosis and kyphosis.  Basically his back and spine are a hot mess.  So sitting for the household is imperative.  He currently sits in a carseat which totally sucks.  He has a wheelchair but it is just not comfortable for him.  So our first priority is to get a comfortable seating system approved throug his insurance.  May be difficult but his dr. is all for it. 

I was concerned about Avery's hips b/c his right leg just doesn't look right to me.  He is rotated and doesn't like it when you move him too much.  A hip xray showed hip dysplagia on the right side.  Sucky, Sucky.  So we have to go back to see ortho. and the CP clinic.  Also on the xray, there appears to be some litle square thing somewhere on his spine.  I thought it might be the tooth that we never found, lol.  but it could be a big calcium deposit. 

Now onto the big one......During the appt. his dr. noticed what looked like multiple seizures.  They were tonic movements with him spacing out for about 20secs.   Honestly, the kid does this so often I just chalked it up to his weird dystonic movements.  I am not surprised he is having seizures b/c with Leigh's disease (progression of the mito into the brain) so many things are affected.  Well b/c of what he was seeing, it was time for a 24-48hs. EEG.  Yay, :(    The pedi. called our neuro/mito dr. and she asked if he needed to go to the ER and thankfully he said no.  He feels that they are not bugging him a great deal but it still needs to be investigated.  So we are likely going inpatient next week for the EEG. 

Off course we ended the visit with talking about advance directives.  I assured him that we have made all our decision over the Fall concerning Avery.  I like that he checks in with us about it.  It's also great that we are on the same page in terms of Avery's care and needs.  He is also in agreement that Avery continue to be home schooled as it is working out so well for him and his immune system. 

I know its mean but I equate Avery to a used car.  You can take it into the shop and you will find a million things wrong with it but you cannot afford to fix it all.  So you prioritize and fix the most important things that will keep your car running.  All in all it was a good appt. and we are trying to all do what is best for him.  It also shows what incredible care he gets a home too.

So this pretty much describes how Avery has been feeling lately. LOL

This is one of my favorite faces that he makes when he is not sure what you are going to him.  The funny thing about it now, is that since he is missing his two bottom teeth his little tongue sticks out in between them. 

He is kinda all over the map.  One day we think his tummy is hurting him so we have started gas drops (although not sure it makes much of a difference).  Another day he is very junky, requiring lots of suctioning and O2. Then other days you just don't have a clue what is going on with him.  He has been running low grade temps (99.5-100.6), nothing concerning but we keep waiting for the big temp to appear.  This is usually his MO before the **** hits the fan.   Although I have been saying for a couple of wks now that he is not right but he seems to recover on his own.  I'm actually pretty impressed with how long we have been home for (8wks).  I am totally whispering that though :)  We have at least made some strides in the constipation area which makes a big difference.  I started adding a heaping dose of miralax to the 80ccs of formula he gets a day.  Typically you need a lot of fluids with it and that was always the problem.  Somehow it is working for him so I am grateful for that.

The Nault's are moving forward with the Disney trip.  YAY!!!!!The volunteer has submitted the paperwork and we are hoping to get the nursing.  Isaiah is still an issue on the airplane but we will somehow get that figured out.  We are planning to go the end of Oct. or early Nov.  They ask you to pick 3 weeks and they will let us know which one was booked.  It is for 6 nights and 7 days.  Yowza, it's gonna be long but I hear that we will love every minute of it.  Although in our case, it will be filled with lots of drama and stories, lol.  It is going to be good for everybody, especially Janessa.

I have started a new little project/job!!! I know, like how do I even have time, right??? Actually it is something that I think I am going to be really good at.  It involves talking to parents of chronically ill kids and coping skills.  Right up my alley, huh!!!.  And I am a natural chatta box.  Ha ha... Honestly, I have been blessed with so much in my life despite the cards we have been dealt.  I have learned to become such a great advocate for my kids in a respectful manner and I want to help other families that don't have the resources to do that.  I'm pretty excited about it :)

As I had mentioned in the last two posts, Avery had been struggling with lots of vomiting and meds. coming out of his draining bag. On Monday he had his j tube changed ( a routine visit). I certainly feel like mommy of the year, not... Turns out his j tube which should be deeper in his intestines, was actually sitting into his stomach. Somehow his tube migrated from his intestines up into his stomach. I was surprised bc this has never happened in the 4 yrs. had his tube. At the same time I felt sooo bad. I was convinced that his tube was not the issue. Avery's motility is so poor that anything in his stomach will just come back out. He cannot tolerate anything into his stomach. He was also miserable bc he was not absorbing any of his pain meds. :(. Poor kid. At least I know it's the first thing I should check when he starts these vomiting spells. Turns out he was backed up AGAIN with poop and gas. So the pressure kicked the tube into his stomach. This poop thing or lack there of is just very frustrating. It is such a new problem for him and yet there are very few options other than surgerical. We all know how I feel about that. We are trying one more thing and it is working " for now". We may have one more trick up our sleeve but if these all fail then there may not be another choice. As a result of all his vomiting, he has been showing increased respiratory problems. He has been requiring oxygen with his bipap due to more desats. We will have to see where he goes with this.

Also, lil man lost another tooth. He looks so cute. But it is concerning bc he is young and there are no permanent teeth in sight. He also has a few loose at the top. He is going to look so funny with all his teeth missing for awhile. I have to get him into the dentist to see if his adult teeth are coming in soon.

In the meantime, we are waiting to hear from Make A wish. A lot of things have to fall into place for us to go to Disney. My biggest concern is the plane ride for the boys ( mainly Isaiah). Since he cannot go in his wheelchair and his carseat is not allowed, we will have to see what is offered. Janessa already said she is "not sitting anywhere near him" bc she knows he will be a complete wild man. Looks like I'm gonna be needing lots of wine for the flight, lol. I will keep you posted as to what is decided.

Toy drives, Make a Wish and decisions!!!

On Friday I was able to head into Children's with the Manager of Starbucks to deliver 3 large boxes of toys.  It just felt really really good to be able to give back to a unit that has been our second family for the past 4 years.  People donated such useful toys that will really help the kids pass the time while they are there.  I was also happy to not be inpatient with Avery, always a plus.  It's nice to be on the outside just visiting for once  Although I could not help but feel a little weird that is this what it would be like when Avery is not here :(   That feeling did not last too long b/c I got to see my favorite nurses and got a really nice compliment from one of the Attending Drs.  He said that he had been uttering my name alot last week as an example.  He said that I was the exception of the parents that know their child so well.  Basically, when I say that Avery has this or that going on it is usually true.  It was just really nice to get some positive feedback.  I just hope one day I can help many of these families to communicate better with drs. and nurses. 

Today, the Make a Wish volunteers came to start the wish process.  I was pretty much set on doing a computer for Avery with maybe a special seat.  Of course, Janessa let it be known that we have plenty of computers around here and that going on a trip would be nice.  My first reaction is to say "are you crazy girl" .  How could we even fathom handling two very involved boys in wheelchairs, on an airplane with retching cycles, TPN, respiratory problems (just to name a few).  Rick was right there with me.  Then the volunteers had shared some nearly impossible Disney wishes (granted they didn't have two handicapped kids) but they made the impossible happen.  Janessa immediately perked up as Rick and I are looking like "for real????"  Are we crazy for even entertaining this idea.  So then we started toying with a Disney cruise (another???/).  It came down to trying the cruise first, then Give Kids The World and a computer last.  I am told by many families that GKTW is amazing and so accomodating in every way.  They would even provide a hospital bed for the boys, bath seats, O2 and so many other things.  Our only major concern is that nursing has to be covered.  It would not be any fun AT all if we cannot take our nurses.  We actually asked for two nurses but I doubted that will go through.  I have to go through and make a list of everything that the boys would need and see if it can be done.  Once we submit it then we will see what MAW decides and I guess we will go from there.

This is exciting to think the Nault's (the whole family) could go on a vacation.  We once went to Disney when Janessa was 3 and Isaiah was 18months.  He has just gotten his wheelchair which got us ALOT of perks.  While Isaiah had an great time, poor Janessa got so so sick.  She vomitted the last 3 days and ended up in the hospital straight from the airport for 3 days due to severe dehydration.  She doesn't remember a thing.  So you can see my hesitation and fears.  But you never know, maybe the stars, moon and sun will line up and we can make this happen without any major casualities :) 

Here are a few pictures of the MAW volunteers and the gifts they gave the kids.  Unfortunately, Isaiah was out of the count b/c he is retching today :(  Its Sunday afterall and no nurse.  Just like clockwork.  We will have to be sure to not go on our trip on a Sunday. lol

On the medical side, Avery is hanging tight.  He has his times that he doesn't seem to be absorbing his meds and is a hot mess and then times he looks great.  No fevers or respiratory problems but still vomitting.  He is getting his j tube changed tomorrow so I will be interested in seeing the placement and his poop situation.

Too much celebrating!!!

As you all know, I am a pretty positive and upbeat person. So when things are going well around here, I have the tendency to celebrate. I mean how can I not, it's Avery first great Christmas and New Years at home. In fact, he has been doing fabulous respiratory wise. This is surely something to celebrate especially during cold/ flu season. But you would think with Avery's past history, I would have learned my lesson. Or at least celebrate quietly in my head, lol.

With all that being said, something is definitely going on with Avery. He vomited at least three time this afternoon odd b/c he usually only does it after meds. So we gave him Iv zofran to settle his stomach. He was happy but just off. Then tonight he vomited tons again and off course got it all over his central line dressing. So here we go with the respiratory issues. I'd be surprised if he didn't develop and aspiration from it.

I don't know why I get so upset when he ends up in the hospital. I should know that it's part of Avery's life. But you do get spoiled being home for a stretch so it makes it harder to go in. Guess when he is doing so well at home, I kid myself into believing he is getting better with time. Silly me thinking he can beat this nasty disease.

Im hoping with all the meds and good nursing he has, we can ride it out at home. Although one of my night nurses who covers 4 shifts a week is going home to Africa for 5 wks :(. I am really really gonna miss her. She is very good and does a lot around here and let's me sleep too. So I am going to be one busy and tired momma over the next 5 wks.

One huge silver lining is that me and the manager of Starbucks are delivering 3 boxes of toys to Children's 11 south unit. Thanks to everyone that contributed to the toy drive. I'm so excited!!!!!!hopefully Avery let's me deliver them by staying home this wk.

Happy New Years

It has started off with a first around here.  I can officially say that Avery has made his first New Years at home.  This is something to certainly celebrate.  When I look back at the last year, it has been filled with lots of ups and some tough downs. 

We did ring in 2010 with a quick hospitalization but then Avery gave us the longest hiatus ever, three long glorious months at home.  This was long enough to enjoy my husband's second State Basketball Championship.  I do have to say that I am so very proud of him.  He is truely living his dream both at his day job and his night job.  Rick has been one of the only coaches around this area to have won two State titles in Four yrs.  It gives me such pleasure to see him in his element and watch Janessa route on her dad and his players and of course be right in the middle of everything.  It is also a welcome break from all the medical stuff and caios in our daily lives. 

Just right after the celebration from Rick's big win, Avery decided it was time to have the spot light back.  This was certainly the down that I don't want to relive again.  He spent 6 long weeks inpatient and we thought we would lose him.  Here is a picture that I have never shared of him just after he was intubated.  I hated to see my little boy this way. No parent should have to see and experience what we did this summer.

This is just soon after he was intubated and was bleeding out.  I am just so grateful that God gave us another chance with our beautiful boy.  He was graced with the fight and spirit of both his parents, thankfully.  I am sure you all know that we are both bulldogs :)  The ICP/ICU staff at Children's Hospital Boston was tremdenous in bring him back to us. It taught Rick and I to take a stance in what we want for him and what we knew his potential to be.  I am proud of the parents we are to our kids and in the tough decisions we have to make.  These are never easy decisions but I feel we put Avery first and it shows.  As you can see he made a full recovery :)  here he is just days before his release with his favorite nurse Sarah who worked with him on both the ICU and ICP.

Look at that!!!!A true blessing, as if nothing happened. 

Obviously, this hospital stay will be with me forever and it made me really see that his time is limited.  We take everyday with him as a blessing and that is for sure.  Our little Hurricane Avery :) 

Isaiah, over the last year has become our "complicated kid".  My heart aches for him.  I mean, he is the happiest, craziest, lilttle stinker who suffers so much and we just cannot get it figured out.  While he enjoys so many good days and this dog has become his little best friend, his bad days are baaad.  We have tried everything over the past 5 yrs and it's just not working.  It is by far the most frustrating thing with this disease.  Could you imagine having a migrane for 12 days a month that cause your blood sugars to skyrocket and affects you whole system so much so that you cannot function.  I cannot imagine for one minute.  Yet this is what my handsome boy suffers from :(   I can honestly say the while Avery is so severely sick, Isaiah is getting slowly progressively worse.  It just scares me.  I just pray this year he finds some relief with it.

Janessa, my girl who seems to have grown into a woman this year.  She sure got us with this dog though!!!!!She is one smart cookie.  I know I always say it but I really do mean it, we were soo blessed to have had her first. Although she can drive me crazy at times with typical kid stuff, I am glad to be able to experience it with her.  I am just sad she is growing up so fast and I can surely see it this yr.  :)

If I can sum it up, 2010 has been a yr of many hard decisions but certainly more blessings. and many firsts (1st healthy Christmas)