This video was made by a Mom whose child passed of this awesome disease.  There are so many children and adults affected with this illness.  Some you would never even suspect.  Please watch this and pass it on to promote awareness of this disease my boys and so many others suffer from.  Thank you.  Hope you have some tissues handy though.
http://www.facebook.com/l.php?u=http%3A%2F%2Fwww.onetruemedia.com%2Fshared_facebook%2Fa8fc3b9e09e7792db8c92c%2F0&h=7155bcefef1cdc425c6a4280638d0e67

Sorry for the lack of updates

It was a busy end of the week.  Isaiah was only in the hospital for 24 hrs and I have to say it was the most productive short stay I have ever had with my kids.  Everyone was impressed that it had been 3 yrs. since Isaiah was inpatient.  They are all so used to seeing Avery that everyone seems to forget my other guy at home.  Well it was time to focus on Isaiah's needs and have drs. take his cycles seriously.  That was the purpose of this admission and it worked.  He was nearing the end of the cyle so he did not even look that bad but they thought he did (good job Zay). Although they did think he was handsome :)

By the morning we had seen Neurology, Metabolism, GI, Nutrition, Pallative Care, and Complex Care team.  They all came together to decide the best meds for preventive (daily med.) and abortive meds (at the start of a cycle).  All were in agreement that the frequency of his cycles were just not good.  Isaiah has tried just about every med. over the past four yrs. and nothing has really touched him.  With him having mito. it also restricts some of the meds. he can try.  Depakote being one of them.  The jury was out on this one b/c it could potentially damage the liver pretty quickly sometimes reversible and sometimes not.  So we all agreed that it was a total last resort.  In the meantime we are trying propanodal (beta blocker) as preventive and Imitrex as abortive but it can only be given once a week.  He will also remain on compozine and diastat  as abortive.  Today was our first trial of the Imitrex and I am not impressed but we will see. 

Next is his diet (on a postive note he gained 4 pounds he is finally in the 40s).  They will reduce the fats that are in his formula.  With the boys new diagnosis it is now thought that they need much less fats then they are getting.  We have a ton of appts. coming up to keep on track with what is going on.  All good things.  I am just glad is was only a 24 hr. visit b/c Isaiah is just plain wild.  I swear he might have even eaten his hospital bracelet, LOL.  He kept getting his skinny legs stuck through the slats of the crib.  I was worried he would break them.  It is very stressful being inpatient with him considering he is just about my size. Plus he was getting depressed b/c he cannot roll around :(  Avery is easier b/c he doesn't move (definetely has its advantages).

Meanwhile, I also had the drs. talking to me about Avery.  Last week he had a blood clot in his central line.  This is very concerning b/c he has quite the history of blood clots which have limited his line access to only the right side.  If this side ever got clotted we would be totally screwed.  The team had not heard of blood being clotted at the end of his line b/c he gets heprin regularly and his TPN is constantly running through it.  This weekend a nurse is coming to put TPA (extra strong heprin) in his line to make sure he has not clots.  It is just nerve wrecking b/c he is doing so well and something like this could really set him back.  I am just glad we are being proactive about it.   He has been junky more than usual and requiring bipap at least 3 nights a week.  He also has been having alot of very fussy days.  Things change from day to day with this guy but I am just grateful he is home. 

This update turned out to be much longer than I thought.  So thank you if you have made it this far.  Sometimes a couple of days in this house turns out to be novel of events. 

Just a quick update as I don't have my laptop. But I am actually inpt. with Isaiaih. I am at my wits end with his cycles. He retched all weekend and had 2 great days before starting back up again. It is really affecting his quality of life and it's time something is done about it. It should be crazy as we all know what a wild man he is. So diff. than Avery stays. I will keeped you all posted.

Counting our Blessings

We have so much to be thankful for as a family these days.  After all of the earlier swine flu scares this fall and my fears that Avery would get it, I am so happy to say this has been his best winter YET!!!!!!  Since Oct., he has only spent about 15 days inpatient.  That is certainly a record breaker around here.  I think it is between getting his IVIG treatments and him getting older and all the great care he gets at home and wehn he is inpatient.  Whatever it is, it's worth acknowledging and being grateful. 

Although Isaiah has had a really rough patch this winter with his retching, we have made alot of strides with his school.  Everyone is working together finally to get him communicating.  In the end that is all we want for him. 

We are so lucky to have such a great kid in Janessa.  She is smart, funny and a good sister even if she gets into my house paints.

Rick and I are so blessed to have such great jobs and supportive people in our lives.  It will be our 12 yr. Anniversary next month but we have been together for 19 yrs. (Amazing).  We have literally grown up together and have faced soooo many challenges along the way but we always get through them ... :)

Below is a clear example of why Isaiah can never be attached to anything, EVER.  I had him on a pulse ox last night b/c he was retching so bad. The other is Avery's haircut by mom and his skinny daddy.

Our Weekend.....

Has been filled with so much excitement from Winning the State Championship, Storms, Dogs, Sometimes happy boys and sometimes not.

Congratulations to the Central Catholic Boys Basketball Team on your Second State Championship in 3 yrs.

It sure was an exciting weekend to say the least.  Isaiah did his usual Frid. retching and Avery on game night was on 4 liters of O2 and satting @ 89-90%.  Thank god for bipap b/c he really needed it and it made all the difference for him.  I was just so nervous b/c he was doing so poorly during Rick's game and I was an hr. away in a bad storm.  Luckily my nurse was able to stablize him so I could enjoy the game.  Now the game was CRAZY, a nail biter until the very end.  We battled it out with St. Johns (a school we beat for the championship in 2008)  they certainly came to play.  We won by ONE point.  Talk about insane.  I am soooo happy it ended in a win though.  Janessa was right on the floor and has had alot of press too.  Homegirl has been on the news and multiple newspapers.  WTG Ness!!!!!!  She was also happy b/c she found the kind of dog we are going to be getting soon (cockapoo) we had daddy believing we were taking him home though.  Hehe....

After an exhausting and exciting night, I woke up to my nurse telling me that there is a tree on my van.  WHAT??????  Sure enough the biggest pine tree is laying on the hood of my car.  This is from the storm we are STILL having.  Luckily my van has no damage but we still have this darn tree blocking my whole driveway.  LMAO... Hopefully we will get the tree out of here by the end of the week. 

Crazy kids!!!!

Isaiah is back to baseline for now as if nothing ever happened.  Silly boy....

Avery was sooo happy to be out in the nice weather today even if it was just for a drs. appt.  He had a TPN checkup appt.  So Mr. Avery has been 30 lbs since the summer (me, I am fine with that b/c I have to carry him around) the drs, not so much...... I had to present my argument for not increasing his TPN time.  I really think the more time and calories he is on the more issues he has with it (ie. blood transfusions, gall stones).  Luckily he had his "high water" pants on today (he grew in inches) that was my winning argument :)  They did remove the stiches from his broviac as they were causing alot of irritation and possibly a line infection.  He was NOT happy about this b/c I am sure it did not feel to good but was certainly necessary.  Of course they strongly encouraged me to restart feeds so I am willing but not optimistic. 

Janessa, Janessa.......She is so excited about her dad being in the state finals that she decided her and a friend would make a "table for him"..... Of course mommy was otherwise occupied with the boys and gave the go ahead (even though they asked for a flat head screw driver????)  Needless to say, the girls used my house paints (navy blue, light blue,purple and tan) which were ALL over my white siding, walls in my downstairs, and I now have blue grass.  So let's just say Miss Girly girl is in BIG trouble.  She has been scrubbing the siding and even suggested to PAINT the siding white (no thanks).  She is such a great kid and we sometimes forget she is still a kid.  But I still have a blue and purple house now!!!!!!!

WOOOOHOOOOO!!!!!!!

CONGRATULATIONS TO RICK AND HIS BOYS ON THE BIG WIN TONIGHT AT THE GARDEN.  HEADING TO THE STATE FINALS ONCE AGAIN (2nd time in his 4 yrs of coaching, UNBELIEVABLE).  Janessa gets to be with her daddy every step of the way!!!!!!!!

SOOOO PROUD OF YOU RICK. 

-----------------------------------------------------------------------------------------

Onto the medical news:  Avery is doing great while poor Isaiah started a really BAD cycle today.  Not even the new med. they gave did anything for him.  His sugars were quite high and he is just struggling.  We actually have him on the O2 monitor which is highly unsual for him.  He is just getting worse and worse with these cycle ( they are now coming on about every 4-5 days).  This = very limited quality of life for him.  It just SUCKs.  I really really need a Dr. House for him.  Just not fair to such a normally happy boy.

Congrats to Rick and the Central Catholic Boys

It was a great night at the Boston Garden last night with the big win.  So now the boys head back to the Garden on Monday evening and he they win that game then its off to the state finals on Sat.  YAY!!!!!!!

Janessa is such a lucky girl being able to hang on the Celtics floor with her daddy as the water girl while mom had not so great seats in the stands.  She was even trying to get an interview on Fox News, lol.

Isaiah is back to baseline (the new med. did the trick)  While Avery has been super fussy the past few days.  No ideas why, just assuming it is his belly.

Tomorrow night is the big game night at the Fleet Center.  I am very nervous!!!!!! But it is also very exciting.  I am so proud of my husband, he has done such a great job all around and it is so fun to watch him and his kids in action.  Not to mention I have a new very skinny hubby on my hands.  He looks GREAT!!!!!

The boys are not so good.  Isaiah has some type of virus :(   He has been having consistent high fevers for the past couple of days.  Which in turn has started yet another retching cycle. UGH, UGH, UGH....  I finally had enough and called his neuro. dr. this am and asked for something stronger than diastat.  Now for those of you that don't know what that is it's a stronger rectal version of valium.  It's usually used to stop seizure but we have been using is unconventionally for the past few yrs.  Problem it that he has been retching so much lately that it doesn't even touch it any more.  So we are running out of abortive meds which is really bad.  So we are trying rectal compozine which is a very old med.  Gotta be honest, I am a bit nervous to try it with the listed side affects.  It just sucks all around.  But Isaiah is certainly a trooper.  He is the happiest sick kid I know :)

Avery could go either way. He is struggling with junkiness but manageable.  With Isaiah sneezing and coughing and green boogers, it's not looking so good for Avery.  We know he catches everything there is to catch so it would be a small miracle if he did not catch what Isaiah has.  Hopefully he can hold out long enough for the playoff game.  Who knows, he could opt out of this one :)  would be nice.

Looking at the bigger picture, Avery has done very well this winter in comparison.  Poor Isaiah has struggled tremendously and I so wish we could fix these darn retching cycle b/c I feel like he suffers more and it just breaks my heart.  But what do ya expect with two mito kids.  Fun, Fun, Fun......

CONGRATS TO THE CENTRAL CATHOLIC BOYS AND GIRLS BASKETBALL WINS TONIGHT.  GOOD LUCK AT THE GARDEN TO BOTH TEAMS!!!!!!!!!!!!!!!!!!!!!!

On the home front the boys are doing well.  Isaiah is retching little bits but he is pretty happy.  Avery has been more junky then usual but nothing is really showing itself.  I keep waiting for the other shoe to drop because he is usual inpatient and pretty sick during the playoffs.  I know I should not be even saying it b/c I would be jinxing myself but he seems to be handling cold/flu season much better this yr.  Just hope it continues so I can enjoy playoffs.

I will keep you all posted.....