All is going well at home. Besides me being freaked about the pig landing here (swine) LOL. We still have not gotten word about the kids pedi. office having the
H1N1 vaccine. Although Obama's girls are all set. Hmmm must be nice to be all set huh. Ha ha...

I am especially concerned for Avery his IVIG levels were low only a couple of weeks into the last infusion. Basically means his immune system is functioning at below average despite just having an infusion. I just booked another one for him for 2 wks and hopefully with the increased dose it will be enough for him to be able to fight off even basic bugs. Plus he is done with his toby nebs which is a medicated nebulizer (breathing treatment) to help prevent respiratory bugs. He is on it every other month and Nov. is his month off. So he will be running low on the defense side along with us not having the vax yet.

I just feel like the pig is looming over us. And I'm scared.......

I have to get some pictures of Avery in his big boy wheelchair. Will hopefully do that soon.

Hope everyone stays healthy.

Back Together

As you can see everyone is happy to be back home together. I swear Avery knew he was going home yesterday b/c he was up at 4:30 happy watching tv and continued to be the happiest kid especially once I put on his jacket and he hit the elevators of the hospital. He sure knows that means he is going home. That boy just laughed hysterically all the way down to the bottom floor (so much so that everyone in the elevator was laughing b/c he was). Sooo cute. That is what it is all about with our family. Being home as a family and not living in the hospital and making Avery and Isaiah's life the least medical life we can give them.

On that note, some food for thought: While inpatient I ran into Avery's GI dr. and I told him the circumstances of why we where there. He felt we really need to get Avery off TPN which would be wonderful but.... He "thinks" we should do an ileostomy to do that. Okay you are asking what the heck is that. I don't know much about it but I do know it requires cutting a whole in my babies small intestines and sticking a bag on his belly for drainage. NO THANK YOU. Rick and I are not into aggressive treatments such as this especially is noone can guarantee that it will get him off TPN. There is actually a good chance we would do that and he will still need some TPN. Not worth it in my book. I am sure many other families would disagree with me but this is "our stance" and the decision we make for our kids. It's not as if Avery doesn't already have enough going on and plus where the hell would I stick it, kid doesn't have much room left on his belly. As you can see.
Rick and I honestly don't think that it is something we will do for Avery. It is what it is. So for now we keep him as healthy as possible which I am a bit nervous about. Apparently, Isaiah's school distributed the H1N1 nasal mist which I was not aware of at all. Mind you in the open lobby for kids whom the parents had consented. Well the nasal spray IS a live virus and not good for kids with compromised immune system (being Isaiah and Avery). Luckily his nurse got him and her out of the area that they were doing it but the live virus apparently can live for 7 days. Of course I am freaking b/c we JUST brought Avery home who is not vaccinated for it. I am not sure I will send Isaiah back to school this week until I get all the details. This is the last thing we need before we have gotten the shot for the kids. I am sure I am being paranoid but this could be detrimental to Avery and perhaps even Isaiah. Hopefully I am just being a paranoid freak but you better be sure Janessa will not be getting this or going to school when they are doing this. I am just in shock that we got no warning of this. I just dont want to spend another day in the hospital for some time to come. KWIM?

It took a total of about 3 hrs. which is what we kinda figured. Normally a port placement could be up to an hr. The surgeon did have trouble placing the line but the concern was more for future line placement or lack of. He said this is definetly a problem down the road and if Avery were to need another line then he would have to go to the cardiac cath lab to have his veins dilated. Its a pretty big deal. So how we prevent this, is by being SUPER diligent about his broviac line now and pray he doesnt get another line infection especially not a yeast. If he gets a gram negative or positive line infection, it is easier to treat through the line without removing it with a broviac but much more difficult to do with a port (which is what he had before.

So very nerve wrecking but I am glad it is done so we can go home tomorrow and hopefully stay home longer than a month this time.

And he if FINALLY off

Avery had a VERY rough start to his day. He woke up screaming his head off looking like he was going to bite his tongue b/c he pain was so bad. His heartrate was all over the place. He settled within an hr. but I have never seen him that bad for no apparent reason. Of course the residents chalked it up to gas "as I rolled my eyes" .

We waited in preop for over 2 hrs. as the surgeon was running behind. But he is finally in the OR now. So please pray that they are able to easily insert a new line.

I will keep you posted.

More Waiting

Apparently there was no a surgeon available until Thurs. UGH!!!! It just baffles me that such a big hospital can be so overbooked but then again people do fly from all over the world to come here. I'm sure there are kids that need surgery who are much sicker than Avery right now, it is just a pain to have to hang there all week for a line placement.

Mr. Avery is surely being a little stinker. He was up ALL night whining and when he favorite nurse would come in the room he would flash her a big smile. So needless to say, I got very little sleep. Since I thought he would be getting his line on Wed. (I was scheduled to work), crazy momma combined 2 workdays into one. Yup, I MUST have gone mad. I saw 13 pt. at one place and went onto to see another 14 pts. at another place. THANK GOD for Starbucks. I came home tonight and so is Rick b/c it is not fun to get no sleep there, plus Avery has his fav. nurse he can harrass tonight :)

Hopefully a smooth line placement on Thurs. morning and out of that place Frid. I am just trying to run out of there b/c I am paranoid about the flu going around and I surely dont want little man to get it.

Look at Isaiah's school picture :) What a handsome boy he is if I don't say so myself.

And we Wait.........

Unfortunately Avery is not getting a new line whatever that maybe (whether it is a port or a brovaic) until Weds. UGH!!!!

It gets hard not to get antsy and go out of your mind when your kid feels perfectly fine and you are just waiting to get on a schedule. Avery is just mad that he is b/c he feels good. But he did get his new big boy wheelchair today. So at least he was able to get out of the bed and go for a walk which was really nice for both of us.

Also I learned that final diagnosis on the boys. It amazes me on how there are soo many people on this earth and yet Rick and I get together and makes babies with a disease that has only been found in 10, yes 10 people in the WORLD. Turns out Rick and I are both carriers who gave 2 copies to Avery, 1 to Isaiah and Janessa is a carrier. Now are you ready for the name of it..................................

Succinly-CoA Ligase deficiency. It is a Mitochondrial depletion syndrome with Methamolonic Acidemia.

You get all that..... I still have no clue what it means but it is an answer and something that will be useful for Janessa when she gets older.


So this is the phase in the game where we are ALL going crazy being in the hospital. I honestly don't know how other families do it who live here for months on end. My family goes stir crazy with anything beyond a wk. But I am certainly happy that he is not here longer b/c he is sick.

A week In the Life of an Avery Hospitalization

For some reason the boy loves to go in on Tues night so I will start there:

Tues: regular work day for Mommy and daddy. Getting ready to have that glass of wine at 7p then Avery decides to get real sick. Off by ambulance to local ER then transferred to Children's ER at 11p. Not in a bed until 3am. Avery is so sick he cannot sleep which means I get about 2 hrs. of sleep.

Wed.: Sick to my stomach b/c I am so sleep deprived. Dr. rounds fresh at 8am for which you find out why your child is sick. Mayham the first full day and a REALLY sick kid and you are really worried trying not to worry Rick.

Thurs.: Getting used to hospital routine and a child that is feeling slightly better. Work is starting to email asking when are you going to see some patients. Daughter asking when is momma coming home. Hubby missing you and you missing the family and your own bed and some sleep. Start to take melatonin by this point because you NEED sleep. Bored out of your mind and going stir crazy already.

Frid.: Going out of your mind for sure b/c you cannot take the food anymore. Tired of sleeping on a windowsill and waking up to your child being suctioned. Avery is feeling good and so ready to go home...but he can't b/c you are either waiting on blood cultures, test results or home care supply delivery and off course NOTHING happens on Frid. afternoon until Monday am. So you waiting anxiously until Rick can relieve you.

Sat-Sun: Daddy is on, Avery is feeling good and they enjoy football or whatever sport is on all weekend.

Monday:I seriously have to make up work but may have to relieve Rick b/c Avery generally does not go home unitl Wed. or thurs. So you get anxious about missing work with a child that is relatively better but your on hospital time now so you wait until you get the go home pass.

Tues-Thurs: hopefully home by then. Nurses want and need to work, You have a TON of work to do and so does hubby. Avery is laughing his butt off the minute you hit the elevators of the hospital. Janessa has had enough of you being away. Isaiah, well he is just glad that he did not have to share the spotlight with Avery for awhile.

BUT......

Without all the madness which is pretty much our norm is to be without Avery. This I simply cannot phathom. So for us we live the caios and look forward to the little mans smiling with his pearly whites at the end of the day. Mr. Avery has brought shear craziness to our lives but in such a good way. I just hope I don't go mad during it all. LOL.

I just wanted to give you all a glimpse of what a week in the hospital is with our family. We work it out somehow even during basketball season by the Grace of God. It is all worth it.
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Looking Good

Well not exactly that good, but pretty darn close......

Avery is feeling really good and so it appears that his cultures may be negative after his port removal. There is no growth to date which usually means negative. No fevers or anything so we are able to keep the temporary line until he gets his permanent one hopefully by Monday. He had the CT scan yesterday which was kinda comical. He needed to be sedated and there was not an anesthesiologist available so the attending did it. Funny thing is Mr. Avery was not that interested in being put to sleep so it took a few doses to keep him still but he never actually slept. Luckily it is a very short test and they got what they need. He is such a stinker.

The results were good and bad. The good is he has some good open veins on his right side that should be no problem to insert a new central line. YAY!!!!! The bad is the left side is totally clotted over and not usable to any line. UGH!!!! In terms of long term access we only have the right side and hopefully it is enough to last for quite some time.

The bug that Avery had is Serratia and it is debatable of where it came from. It generally lives in the environment and like water or moisture. This really bugs me b/c it could mean that the technique to access him was not sterile. He has a nurse come in weekly to change his needle and they are all very good and there is no water around when they are accessing him so it is just crazy to me. I have also heard it can be in his gut too. I guess it really doesn't matter but it makes me nervous b/c I need to prevent this from happening again as our access is limited and just no fair for Avery to be so sick. I think we may switch over to a broviac with is a central line that does not need to be accessed with a needle weekly. I just have mixed feelings about it b/c he could never go in the water if he wanted to. But if that is what is going to keep him access safe then it is what has to be done.

I will be going home for the weekend for a much needed break and to see the family and catch up on work. Avery I am sure will behave as he always does while I am away. He should be getting his IVIG infusion this weekend b/c his levels were low and could cause him to get another illness. They are increasing his dose and changing his infusions from every 4 wks to every 3 wks. Fun!!!!

Thank you all for your good thoughts and prayers.

Where to Start???

So yesterday started off as a bang and it pretty much continues with unexpected news. Bare with me as I know I speak medical as it is my second language and pretty much assume that everyone else does so I will try to simplify things.

As I said yesterday we learned Avery had a positive blood culture (gram negative rods) Basically its an infection in his blood that entered through his port (central line) Bacteria loves foreign objects for which the port is and quickly attaches itself to it. Any bacteria in your blood can make you very sick and even septic. This is what happened to Avery yesterday. There are three types of bugs (gram positive, gram negative and yeast) If I had to pick I would take the first one as you can try to treat through them sometimes but where the source of infection came from his initial infection around the skin, it made it really difficult to keep the port. But I obviously can't pick and Avery got gram negative rods instead.

He went into the OR about 3pm to have his port removed. I was very nervous b/c I know how difficult he is to get another one in. The other major problem is once they remove his port he still has to have access and his veins suck (you will see just how much). He needs to be able to get fluids, TPN, possiblly blood, IV antibiotics and IVIG infusions. It is not safe to put a new central line in until you have at least 48 hrs. of negative blood cultures. BIG PROBLEM.....

They have no difficulty in the OR removing his port but they still needed to get a couple of good IVs in him. Here the problem lies. After an hour and half the anesthesiologist used him as a pin cushion and got ABSOLUTELY NOTHING :( The kid looks like he has been hit with a nail gun. No kidding. She was quite upset b/c she had to stick him so many times and came out with nothing. She said all of his veins are shot. So we still needed access right, The surgeon had to put in a temporary central line back in the same area as his infection. NOT GOOD. Because there is a very high risk of it getting infected. SO this can only stay in for 5 days max. Another problem, he had a fever this morning so he still seems to be postive for infection. We also learned he was positive not only in the port but also in his bloodstream.

Another issue is the not only is it difficult to get IVs on him but we are extremely limited on central access (kid has only had 3 lines). The surgeon wants a head to toe CT so they can see what is what. This makes me very nervous b/c he NEEDS access. There are some other procedures to get it if you have to but they are very invasive. Problem for Avery is his history of major blood clots, drs. are very cautious about it.

Wheeewwww. You get that all..... The crazy thing is that little man actually looks pretty good today with some smiles even. He is just telling me that things will be fine momma. So we will see what the plan is for today whether we have to remove this line or not. Prayers for negative blood cultures and we can keep this line until he has to get a more permanent one.

It's amazing how Quickly things change

I really have to learn to stop gloating about how great Avery is doing. It gets me everytime.......

Yesterday he had a great day and he went to bed at around 5p and within an hr. of starting his TPN (IV food) be started to spike a temp. while sleeping. Within in a half hr. his temp went from 101 to 103 and his heartrate was over 200. So needless to say he called 911 b/c he just looked horrible and worriesome. I do have to say that the local hospital has really done a great job with him. He ran all the blood cultures and covered him (to the max) with IV antibiotics.

We got to Childrens at 11p and unfortunately had to go through the ER where we basically sat doing nothing until 3am. He is on his usual spot ICP and has continued to be totally miserable and some blood pressure issues. He is showing signs of being septic. By 8am the local hospital called saying he is postive for a line infection (gram negative rods) not a good one but a better than yeast. But because he is so sick they are pulling out his central line today. I am a bit shocked by this as I thought they could treat through it but the drs. as well as Infectious Disease are all in a agreement to pull it today.

THis is going to be a long week of no line, constantly losing IV and he is already getting a blood transfusion b/c he h/h has dropped so dramatically. I suspected he will be here for at least 1.5 wk

I really hate this whole line infection thing. It takes such a toll on his body and tough to see him have to get stuck so many times. Of course it is a double edge sword b/c he needs the central line for his survival and yet the risk of these infections can be very tramatic.

I will keep you all posted but I expect we will be going to the OR later on tonight. Thanks for reading.

Today I just feel really thankful :)

Mr. Avery has really come a long way in a yrs. time. It has to do with a number of things, the great care he gets, the TPN and the IVIG infusions. Frankly, I dont care what it is but that it IS. He truely has come into his own I tell ya. He is really trying to talk these days it is really something beautiful to see. This time last yr. I remember thinking he would not make it through the winter with all his respiratory issues. Now here he is 9 pounds heavier (almost caught up to his brother) and only a week hospital stay since late June. That is certainly progress for ya...... I am just soooo grateful to see him as a happy thriving boy. I am sure we will have more trips to the hospital but I have really come to appreciate the more we are able to stay home. It makes me wonder if he is getting better as he gets older. The same happened with Isaiah and it would be a gift if it was Avery's fate as well.

But for now I continue to enjoy my beautiful family and be grateful for everything we have because all in all LIFE IS GOOD........

We had a great time last night at the Celtics and what awesome seats. Thank you again Maureen. I hope you can see big boy this wk.

Avery's central line site continues to need monitoring. It has been oozing some yellow substance but we have been applying topical antibiotics daily and is seems to be healing. He is still asymptomatic of a line infection, Whewwww so that is great. Hopefully he is nice and healed by Monday when he needs to be reaccessed. It would be nice to make a yr. anniversary with his central line/TPN with only one infection that would be an accomplishment. Kinda sad, huh. But it goes to show you how we all are doing a great job with his line and I do think that is something to feel good about. I of course always fear that he will get another big line infection so we are trying our best to avoid them, although sometimes no matter how clean and diligent you are with the line there is still nothing you can do to prevent them.

Isaiah saw the pedi. yesterday and both him and Ness got their flu shots. The pedi. thought we should go back to the basics with Isaiah. Meaning we start getting rid of meds. that are not working and start from scratch. I do think this is a great idea. Sometimes once you start a med. it is really hard to get drs. to get rid of them. We obviously have not received any benefits from them so why have them on board. No easy answer but we have to do what we can to improve his quality of life.

Today was my first day off and I of course spent it at the drs. office with Mr. Avery. His site where the central line is was progressively looking icky. It was quite red with some drainage (not a good thing with a central line). He has not being having any fevers or anything so I am hoping it is just superficial. For now we changed his dressing and are treating it with topical antibiotics. The dr. also took blood cultures as well as site cultures. I really don't expect anything to come of it, although I better not act too confident. Hopefully, nothing comes of it and it will heal before we have to reaccess him on Monday.

Isaiah is back to not feeling great today. He was all set to go to school today and was even in his chair with his jacket on.....When he started retching again. UGHHH @%$%^ We are heading to the peds. tomorrow for him and Janessa so we will see if I get anywhere (highly doubt it).

We were lucky enough to get preseason Celtics tickets with a pass to go an hr. early for tomorrow nights game. Thank you Pedi. Pall. team :) So we will be taking Ness and a friend. It should be fun. So long as the boys behave themselves.

Hopefully I will reap the benefits for 2 days off next wk. Guess we will see.......

Happy 9th Birthday Janessa

Where does the time go??? I feel like it was just recently that baby Nessa was crying b/c she did not want to take the picture on the horse and now look at her practically grown with her friends :(

She certainly had a great long birthday weekend with lots of friends and dinners, and lots of presents. She sure is a lucky girl. We are of course guilty of spoiling her b/c it is easy to b/c we can only spoil the boys with our love and attention. Sometimes we have to be careful with spoiling Ness b/c it can surely backfire on us. But she is a great kid.

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On the medical front of things:

My poor Zay Zay has been suffering more and more from these horrible migranes. He spent his entire weekend in misery. His cycles are now happening at least once a week and I am beyond frustrated for him b/c they have gotten so much more intense. I am at my wits end b/c it is just not fair. Totally sucks to have to see him suffer like this. It has gotten to the point to the meds we use to abort (stop) the retching aren't even working so he continues to retch for hr. on end. He has a well visit with his pedi. on Friday and I really want to get on him about what we can do to help Isaiah with this.

Avery continues to do well these days. Last night he actually slept through the night for what seems to be the first time in yrs. Naw probably months but it is so good to wake and in the am and realize that you did not automatically jump out of bed 50x all the while hubby is snorning away :) He sort of had an off day today and he is coming up on a month from our last admission so I will not be celebrating b/c we know where that landed me the last time. LOL

Also some more interesting news on the whole genetics thing with the boys mutation. My genetics dr. called me this sat. sounding pretty excited that they seem to be narrowing down more specifically the type of mutation the boys have. So far they have not found it in Janessa or myself and Rick had not had a chance to get tested but the lab in Houston TX is very anxious to get Rick's tests done. I guess they have NEVER seen the type of mutation in males ever and it is a big deal. It is quite complicated and I don't even begin to understand it at all but my hope it that it not only helps my family but others to be identified.

Well that is it for now. I am excited to only be working 3 days this wk. WOOOOHOOO

Just a few pict. from my vaca. The first two are of a place I went shopping. So nice to shop outside in warm weather. The one with me, Michelle and Michael (right at the shore it where we saw a shark, CRAZY)


So in a few short days my baby girl is going to be 9...... Where the heck does the time go. Sometimes I swear she is going to be 12 b/c she is so mature for her age and she is already wearing my clothes. I am really enjoying this age b/c we get to go out shopping and being the girls. Before long she will not want to be with me so I better enjoy it while it lasts.

Avery is doing really well these days. He is so engaging in school and even made a picture finger painting. He is vocalizing alot and his head control has gotten alot better. I will tell you he just loves to be held by his momma. I spent the day home with the boys b/c I have no nurse and he would whine everytime I walked away until I picked him up. Now I was all for that when he was smaller but now homeboy is 32lbs of dead weight and full of tubes, LOL... He is so cute though.

Isaiah has had some real pooping issues these days and we are really trying to get a handle on it. I suggested to the dr. that it could be bacterial overgrowth in his gut from being on reflux meds for so long. The dr. agreed and started him on flagyl. I am hoping it makes a big difference. I just enjoy being home with the boys, it is sooo easy to make Isaiah laugh (he is so funny). He kills me how he steam rolls all over poor Avery who could care less. As you can see


I have to say I cannot really complain these days as things seem to be "normal" for us. I am excited to be down to 3 workdays next wk too. Say a little prayer that I survive another birthday party, Ha ha.....