Early Christmas Gift

Avery is going to stay home with his family which is exactly where he belongs :)   His lab work came back on thurs as slightly better but the ultrasound was unchanged.  It's suspected that his bloodwork may have been worse originally b/c he has an infection in the bile duct leading from his gallbladder.  Since he was started on the IV antibiotics on Tues, it seems to be making things a bit better.  He is still acting perfectly happy of course.  Such a trooper and a blessing :)  So for now,  no surgery and we will just repeat bloodwork and the ultrasound and hope for the best.  I am just so happy to not be spending our Christmas at the Children's Hospital resort.  Life is Good!!!!

Merry Christmas and Happy New Year's to all from the Nault's......

It's that time again

We have just hit the 4 week mark since Avery's last admission and here we are upon the next one :(.  If you remember from the last post I was taking Avery in for a liver ultrasound and some labs.  Well, the results were even worse than I expected as were his labs.  Since coming off the medication(actigall) in Sept. which is supposed to stop his gallbladder from slugging, he has developed a complete obstruction in his gallbladder.  Basically, there is no place for the bile to go from his stomach so it backs up into his liver.  It's creating very high levels of bilirubin (jaundice) in his liver.  Right now his liver is pretty angry b/c his gallbladder is not working b/c it's so obstructed with sludge.  He is actually starting to turn a bit yellow especially his eyes.  By looking at him you would not know that he has all this going on and I am sure if you or I had this issue we would be pretty miserable.  Not him so far, he is still pretty happy.  But something still needs to be done rather soon.  Of course soon brings us right up to Christmas.  UGH!!!!

I am so grateful to have my pediatrician. He has really grabbed the bull by the horns when it comes to making quality of life decisions with Avery's care.   I texted him last night the results of the ultrasound and by the morning he was on the phone with Children's making up a plan for us.  Mainly trying to keep him home for Christmas.  The dr. that called me last night to tell me his labs were quiet bad compared to last week wanted to admit him right away.  Having been a veteran of the Children's Hospital system, I know what happens towards a Holiday weekend.....Absolutely nothing!!!! There was no way I would let him be stuck in a hospital for Christmas and he would likely not get anything more than he can get at home.  My pedi. made that very clear to the dr. that called last night (before even talking to me). That's why he is so good for us b/c we think alike.  They agreed to let Avery start IV antibiotics at home and back on the medication he was previously on.  He has to have another ultrasound and more bloodwork on Thurs.  If his numbers look a lot worse then we may end up inpatient anyway but I am hoping that we can get him through the weekend and be admitted on Monday.  Once he is admitted they may do further studies and will definitely either take out his gallbladder or hopefully just put a stent in it (which would be less invasive).  Again it all depends on how bad it is when then get a better view and if he gets sick before than.   One of the biggest symptoms is extreme itchiness.  I really really hope he does not develop that :(  My poor lil man just cannot catch a break.  It's always something.  We could use all the positive "Stay at home for Christmas" thoughts we can get.

On a happy note :)  Master Avery received an early surprise from Santa.  Only we have no idea who it is but we were so happy and appreciative to receive such an awesome gift... He got 5 tickets to see Sesame Street live in the front row no less!!!!!!! Whoever is Santa, they sure know my lil man b/c he LOVES Elmo.  I cannot wait to go.  Both boys are going to love it.  Thank you from the bottom of our hearts it put such a positive spin on such a stressful day.

My posts are few and far between these days. I think because Avery has been in the hospital so frequently, when he comes home I just want to get back to our regular routine. I feel like I live, breathe, and eat the medical life and it's just too much. In fact since his last hospitalization, I have made it clear that we are simplifying Avery's care. If specialists want to see him then they can do it while he is inpatient. I am really trying to limit the amount of trips we take into Boston. I'm lucky to have a pediatrician that is will to take over most of Avery's medical decisions b/c his plan of care is spot on with mine. We want him to spend as much time doing the things that he likes, ie school. Avery just really enjoys being there and the kids love him too. He has been doing great since he has been home but his labs aren't looking so hot. His liver numbers are still high and we think he could be having gallstones. A couple of months ago he was taken off a medication which would prevent this. Because he get lipids (fats) in his TPN daily, it can really do a number on your liver and gallbladder. So he is getting an ultrasound on Monday to see how & what his liver is doing. Hopefully it's as simple as restarting the medication. He doesn't seem to be in pain which I would expect if he had gallstones. I'm hoping it's just some sludging of his gallbladder which is a simple solution. Otherwise Avery is happy and doing well.

Isaiah has FINALLY returned to his baseline. Poor kid was sick with a cough for a cowpoke of weeks and then had a period of daily retching. I just hate to see him whiny and miserable bc he's usually such a happy kid. Janessa has been busy as ever between girl scouts, drama, and student counsel meetings. She recently had a teacher conference and I'm so pround of the girl she has grown to be. She made the high honors list and even got a card sent by her teacher praising her for being such a great kid and an asset to his classroom. Just makes my heart smile.

I started counseling a few weeks ago and it's just what I needed. I look forward to going. It really helps me get through my week bc as you can see there is never a dull moment in my life. Superwoman I am NOT, I'm just a mom, wife, and a woman trying to get through the day of this difficult life.

I know I have been horrible about blogging.  Sorry to leave you all hanging but I just hit a wall this week.  This crazy life finally caught up to me this week.  I honestly don't know how I have done it this long without any help.  Things finally came to a head earlier this week and I decided to start seeing a counselor. Man,  it was the best decision I made for myself in a long time.  This guy is perfect for me, he has such a calming effect and that is just what I need right now.  I'm going to start seeing him weekly, it will just be my time every week to vent.  This life is not going to get any easier so I need a way to continue to deal with it and try to keep my sanity at the time.  Cuz is momma is happy so is the family.

Onto Avery, he recovered pretty quickly from RSV.  I honestly thought it would be a death sentence for him b/c it is such a tough respiratory illness to recover from.  Well not this little dude!!!! He was able to avoid intubation despite all the extra fluids they gave him in the ER (which he is still working off) and was able to wean off bipap by the end of the week.  He is still having some issues with his liver enzymes being elevated as well as his lungs being wet from the excess fluids.  However, he was able to come home yesterday.  My nurse has certainly earned her money today b/c he is very respiratory all day.  I'm hoping he  does not stay on this path b/c this house really needs a break from sickness.  Speaking of which, Isaiah still has a lingering cough and just not feeling great.  I'm wondering if he had RSV too?  Poor kid also spent the whole weekend retching.  Also I have been sick myself :( I kinda expected it b/c I felt so rundown last week.

There truly is never a dull moment around here.  I wish our life was boring for at least a week but it has continued to be nonstop.  I am just glad I now have an outlet b/c I'm gonna need it.

No rest for the weary

Since I posted last weekend things have yet to settle down around here.  Isaiah continued to spike fevers and his cough became very congested.  This is all very unusual for him so I was concerned by day 6 of the fevers and not to mention he was pretty miserable :(.  Took him to the drs and he was treated for bronchitis and he is finally feeling better.

Of course we didn't get off that easily, come on were are the Nault's... By wed. Avery spiked a 102 fever and started with a croupy cough.  Never a good sign.  However he looked great. I took him to the pediatrician and she gave him a strong antibiotic shot and ran lots of blood work.  Surprisingly, things looked good and she was comfortable sending us home with oral antibiotics.  Amazing!!!! Did you really think it was going to be that easy.... Of course not.  We were able to at least enjoy our Thanksgiving meal but then things quickly went downhill from there.  Avery became very congested and ended up spiking fevers overnight.  So by this point it was time to bring him in.  We drove him in and it was NOT pretty.  By the time we got to the ER he was rushed to the trauma room and things got pretty scary from there.  He was on high alert for intubation.  We were transferred to the ICU of course and the attending told me he was pretty worried about Avery and that his threshold for intubation was very low.  Needless to say, I didn't get much sleep that night.

At one point there was a Code Blue call on the unit and much of the staff assumed it was Avery and so did I for a minute.  Very scary!!!!  Thankfully, I have one tough guy and had a great nurse and respiratory therapy that did a ton to stabilize him overnight.  He has been able to avoid intubation which is amazing considering he was just diagnosed with RSV.  This is NOT a good diagnosis for a kid like Avery and certainly can be fatal.  But like I said, my boy is a trooper.  He is on continuous bipap on higher settings but doing well.  He labs were not looking so good by Friday but have really improved since then.  RSV is a long and nasty course and it peaks at day 5.  I am hoping that started on Wed. (the first day he got the fever) since he seems to be showing signs of improvement.  He is not out of the woods by a long shot but he is making some slow and steady progress.  Just praying that he continues on this path and that our life settles at some point b/c I feel like I am going to lose my mind.  Basketball season starts on Monday so what little help I was getting before is going to be even less.  I think that Superwoman cape is going to have to be sewn on permanently.

This life is Never Easy

Boy did I feel the rath of that this week…

Monday: Work, work work, and hunting down Methadone at four different pharmacies b/c the script was ordered way too late and it is very hard to find it in liquid form and in the insane amounts that Avery gets. Thankfully, by 7p I got it filled and headed home to the craziness of the house. As you can imagine I was exhausted.

Tuesday: Work and then my in-laws got new carpeting we desparately needed b/c of the poopy Nault boys over the past 5 yrs. My only condition with the installers is that it HAD to be done while the boys are in school b/c it would just be too crazy to displace them.  Well low and behold that did not happen and it was crazy than EVER here. However, it was worth it b/c the carpet looks fabulous!!!

Wednesday: Avery had an appt with the Spinal Orthopedic Dr.  NOT GOOD :( :(   I knew his curve was bad (60%) but it was far worse than I thought.  Basically, his scoliosis (curve in his spine) is 90% and kyphosis(bending forward of his back) is 80%.  I just wanted to breakdown but I still thought there were options.  Silly me.  After hrs of absorbing the appt. it was clear that there are no options but to love our boy and continue to make him comfortable.  Since he is only 5 yrs old he would need growing rods which would require surgery every 6 months as he grows.  Not to mention his bone strength.  He is having a bone scan in two weeks which would tell us how soft his bones are.  I am totally expecting them to not be viable for any type of rods.  I know the Dr. knows that but doesn't want to tell me upfront.  I don't blame the guy. How do you tell someone that your son's spine is so bad and yet surgery just is not a reasonable option.  Just sucks all around.  Eventually he will run out of lung space and have issues with his heart.  Talk about a knife through your heart.  Just awful.

Thursday: a loong workday, gym and come home to Isaiah with a 102 fever (which is highly unusual) and Avery who has some type of abrasion on his eye.  Good lord!!! We keep Isaiah's fever under control and hope it stays away, meanwhile my pedi wants Avery to be seen by Opthomology to rule out a corneal abrasion the next day.  Ugh. This really sucks b/c I HAVE to work and see 15 pts. in Boston only got rush back at (hopefully) noon to get Avery afterschool and bring him back to Boston and then sit in traffic forever.  Time for the Superwoman Cape!!!!

Friday: Head to work get ALL 15 pts seen and out by noon, Awesome.!! Get Avery and sit in the drs. office for two hrs only to be diagnosed with an irritation of his eye.  So now we sit in traffic for 1.5 hrs and I have had it by this point and so has Avery.  The only highlight of my week was so go over one of new my friend's house and have her kids love me.  Her baby was all over me and it was exactly what I need after the week I had. Not to mention the great company, I truly believe you meet people at certain points in your life for a reason.  Her family is exactly what I need right now :)

Sat-Sunday: Avery's eye looks much better but Isaiah has continued to have fevers and just not feel well.  It's heartbreaking b/c he has been waking up at night crying with fevers.  This is so unusual and heartbreaking b/c he never cries or gets this sick.  I know what to do with Avery when he gets a fever but with Isaiah I feel like I have no clue.  My poor boo.  Hoping he is feeling better by tomorrow b/c he is breaking my heart.

So there you have it.  A week in review.  Now you can see why I get overwhelmed at times.  Adding to the craziness with the kids, my job is getting very demanding and I HAVE to work (wish I had an option but I don't)' so I havet to get it done.  Not many mom's work with one special kiddo let alone two.  I'm just hoping my Superwoman Cape keeps working for me.

Since it's been over a week since I last posted and so much has happened that I don't even know where to begin??

Guess I will start chronologically.  Big storm came and again the Nault's were blessed with not losing any power at all.  Thank goodness.  As we all know,  I am total freak about that especially with my boys. However my heart goes out to all the families that did lose power and more.

 On 10/30 we would have left for Disney had I not cancelled the trip. Bittersweet really!!! Sad b/c of the realization of a family trip is just not possible and thankful b/c Mr. Avery validated why I cancelled b/c he ended up inpatient due to a 103.5 fever.  Thanks for the confirmation Av :)  At the same time, his alternative wish was perfect.  Avery skyped circle time for the first time this Monday and although the kids were not able to see him live but he still have a cute picture they were able to see, it was awesome.  Each kid said hi to him and Avery could see them and was cracking up laughing.  It was priceless :)

Thankfully, his inpatient stay was short lived.  He only spiked the one fever and actually looked and sounded the best he has in a very long time.  But b/c the fever was so high and he has a central line, it earned him a two day stay.  Which is not bad but I do have to say this being in the hospital every few weeks is killing me.  Honestly…

When I came home on Friday Janessa was sick with a sore throat and just looked dehydrated and coughing like crazy and Isaiah had been retching like crazy.  These kids sure know how to greet their momma.  She turned out the have something viral and Isaiah has been struggling more with his retching b/c he was lucky enough to get two weeks off :( poor boo boo.

During my stay at Children's I was asked by Palliative Care to do a focus group meeting with myself and 10 other Drs. from around the country about my experience with the Pact team.  As you all know that I am NOT shy and had NO problem talking their ears off.  It felt like 5 yrs worth of counseling for sure.  Just reliving everything our family has been with Avery was just a lot.  But it felt good :)  Of course they fell in love with me and Avery :) .. On a serious note, I think they got how difficult our life and all the decision that go along with it are and that we are just doing the best we can do.  I think I realized what a rock solid family we have.  Both Rick and I did not grew up with our share of difficulties but we have certainly made the best of what we have.  Our family is filled with love and I am so proud of that.

Speaking of being proud, Rick is being inducted into the Lawrence Boy's Club Hall of Fame tomorrow.  This is a very big deal.  The Lawrence BG is in the middle of a poor and struggling city that gives to so many kids and that includes my husband when he was growing up.  It is just an amazing place and was even featured on Oprah b/c of all the great work they do.  Rick is one of those guys that knows where he came from and is grateful and has given back to the community that has help him become the man he is today.  For that I am so grateful b/c he is the man that walks the journey with me no matter what.  Congrats babe!!!

Finding that Balance

That's is what we are trying to do....

We followed all the recommendations the Pulm dr. gave us and they were great, but I'm not sure Avery thought the same

Let's just say that small changes go a long way.  Avery does not do well at all with lots of changes.  This photos was taken at 3 in the afternoon which is highly unusual for him to be sleep.  Although he does look so so cute.  It was just too many changes at once. While the diuretics were a great choice, the dose was much too high for him as well as the sudafed 3 times a day. He became so dry that he was developing mucous plugs (thus the balance). However, he has never sounded clearer which is a godsend. By the end of the week he was able to thoroughly enjoy the kindergarten Halloween parade.

My lil gnome :)

We have a follow up appt. with her on frid. so we should be able to iron out most things then.
I don"t want to get my hopes up.  I am hoping and praying that this works for awhile. while he still has bad days b/c of pain and other issues, thankfully he doesn't sound like he is drowning on a regular basis.  He sounds so much better.  We have not even had to suction him in a week which is highly unusual.


Along the celebrating lines, I was just getting ready to celebrate that Mr. Isaiah has not retched in two week.  Well no sooner did I get the words out of my mouth he had the worst Sunday episode ever :(  My poor handsome man is just having a lot of issues with his stomach and he just does not seem happy at school.  I have no idea what is going on with him, sure wish I knew but it breaks my heart.  


Janessa is enjoying school dances, Halloween custom parties and just being a tween, UGH!!!! She is a good girl though.  


I have still been experiencing some intermittent pain in my right kidney. So much so that I had an ultrasound and a urine culture on Monday.  The culture was normal but the ultrasound showed a 1.5 cm fluid filled pocket in my right kidney.  It may very well be the remnants of the infection, but I am still paranoid of getting that sick again. We still have no idea of how I got and so therefore no idea how to prevent it.  Unless I develop fever or chills I am ok.  I'm just so afraid of feeling that way again.


Otherwise we are enjoying be normal at home and hoping it stays that way for a bit this time ;)

Quality of Life

The new buzz word of 2000??? So what does it really mean? I think it's all relative.  You can put ten families in one room and I think they would all define that differently b/c it is dependent on the situation.  This is always the statement drs. use when they are talking to parents of kids like mine.  At times it can be really hard to define b/c the boy's quality of life often get meshed with my feelings of "saving' them b/c I'm so afraid of losing them.  So it is a constant head check of whose quality of life you are thinking of.  I'm not sure if I'm even making any sense but bottom line, it's so so freaking hard.

That being said, I have had the whole spinal surgery on my mind constantly. Since our last hospitalization I felt like "how could I possibly let me kid die b/c he is running out of lung space, I gotta do it" Since my decision though I find myself waking up at night in fear of losing him b/c of my decision and having to live with that for the rest of my life.  Well on Monday, we had a big appt. with the Pulminogist that was supposed to seal the deal in my decision.  But it was total opposite, but for the better.  First of all, the back story is I previously changed drs. b/c I just did not like her views at all.  She ended up leaving the country for a yr for work and recently came back.  Since Avery is part of her home bound program I had to see her.  Needless to say I already had it in my head what she would be saying (no back surgery and to trach him). Turns out giving people a second chance worked out.  First off, she was upset with how much time he has been spending inpatient and that was her first goal of change.  Her belief is that when he goes inpatient the teams are too afraid to make changes and so what we have been doing for the past yr has not been working so time to make major changes.  She completely changed my view on her already!! Obviously she is not afraid to rustle any feathers.  My kind of girl...

Many of Avery's meds we eliminated. yahoo.. With kids like Avery once they acquire a medication they pretty much own them b/c people are afraid to change them.  Well not her.  She got rid of most of his nebulizer treatments which are so exhausting on him and may not even be effective.  She changed it to just dou nebulizers three times a day.  Much easier.  Something nobody else has done is look at him as a whole.  She combed through his 50, 000 chest X-rays and saw how wet he is on all his X-rays lately.  When he is inpatient they always say that they look fine.  Apparently not.  So she prescribed lasix by his tube twice a day and kept his PRN IV lasix dose for when he is very wet.  Whereas most drs. are busy trying to cover their own asses and don't want us using them.  So instead my kid is drowning, great.  Also she totally agrees with my NO trachea stance, YAY!!!! She also said that while she is not sure the surgery will be a big fix for him and it has ALOT of risks, it's still worth seeing the specialist and seeing what he recommends.  She also increased his bipap settings and recommended that when he starts getting real sick we should put him on for 24 hrs straight(hmm, this will be a bit of an issue with Mr. personality).  Basically she said we can do this ALL at home instead of living in the hospital where they are doing the same thing.  So now I love her :).  She is realistic and is not afraid to make the changes to accommodate us and our situation.

I know no dr. is going to save my kids. I'm very realistic and  I know this disease will win in the end. BUT I can choose how their quality of life will be in my terms.  Many people can look at my kids and say "what kind of quality of life do they have?" well spend 5 mins with them and you will know!!! While it is very difficult to make the best possible decisions for them and not my heart, I know in the end it will be about them.  Right now Avery has such a quality of life.  He is happy and loves school and knows when he is home or in the hospital and trust me he is not happy in the hospital.  So for now we are hoping this drs. plan will keep our lil guy at home and be able to continue his happy family life for as long as he wants to be here.

I forget to tell you that we decided on an alternative wish, a 27" IMAC computer.  We already got it in too :) what is even better is that his school is going to be Skyping circle time for the days he is not there.  They already have all the parents consents and we will be ready to go soon.  I think it is a perfect alternative.  Below are some pictures and fun he already has been having with it.  Now that's "quality of life"

Dragon Parents

If you read my last post and the article then you will get this post...

That article has hit me in so many ways. It was so well written and truly describes what our life is like. You can't help but read it and shed tears. Most parents won't be dragon parent bc that's just not the circle of life but until or unless you are you will not know how difficult it is. We are our own culture. That being said, I have begun to realize how small Rick and my circle is of "true friends" are. I had not realized it until I got a call today about how many people would be attending a ceremony of one of my husband's greatest accomplishments. It was sad bc we cannot even fill a table. In fact both him and I got a phone call regarding how many people would be attending and it was the same amount ( not enough to even fill one table). Very heartbreaking!!! I would like to think that we are both great people who do alot for other ps while dealing with our own lives with always a smile own our faces. This is what my Dr. told me the other day. Yet where are all these people? What it comes down to is people are afraid of us and our lives. Many say they get it but the fact of the matter is that they DON'T and it is likely that they will not have to bury 2 kids.

I guess I'm just sad for Rick and myself bc at the end of the day it's just us. But no matter what, we got this and will continue to inspire others.

We are home, lots and lots to think about but in the end I know what our decision will be.....in the best decision for Avery's quality of life. That being said a few parents shared this link on facebook.  This parent could not have said it better. So fitting to our life and so many other families I know, please take a moment to read it b/c you will truely understand what we feel and go through as Dragon Parents. Notes from a Dragon Parent

As predicted Avery was admitted on Sat. He was just not turning the corner as I'd hope he would, until he finally got a bed on the ICU unit. The hospital has been so busy since the start of school. The Dr. That I had been communicating with all week told me that there were no beds on the ICU & ICP units (which is where Avery has to go b/c of bipap). There was a chance he would get transferred to another hospital. Of course I was freaking out. The thought I going to a hospital that doesn't know Avery scares me to death. When we arrived at the ER Avery was really working to breath but within a half an hr he was as good as new. Of course that's when they told us they had an ICU bed for him. I did contemplate going home but then I realized the alternative was six 10 yrs girls at my house for Janessa's birthday sleepover so I figured I let Rick handle that one, lol.

Avery had a great night and we had our walking papers by 8am which is very rare. As usual he had other plans decided to hang on for a little longer. He was pretty respiratory all day and kept his nurse very busy. In rounds we talked about how frequently Avery has been inpatient lately as it has been alot. While I was thinking it was from school and getting used to the other kids, the attending brought up another great point. The one I keep putting off...Avery's curve in his back is so severe that is is really limiting his lung space and may be contributing more to his frequent respiratory issues. I think he is so right. He gave me the good, bad and the ugly of the surgery. I felt better about the possibility and benefits for Avery. It is not going to be easy and it may be a month or so of difficulties but the benefits after are so much for Avery. I have been teetering with this decision bc it's the biggest decision we will make for him yet. I gave the appt. With ortho in a couple of weeks and I'm anxious to hear his thoughts. I wish life wasn't so hard sometimes :(

It's been awhile

It felt like a week since I last updated but I guess it has been a little longer. Since I
last post, Rick and I went to NY for the weekend and had a nice non stressful weekend (aside from the rush hr. drive from the airport). It was just really nice to get away and be able to have some time together without our daily stresses. Meanwhile, Avery struggled at home respiratory wise. Basically he has continued to do so. I'm pretty sure the he will end up inpatient over the weekend if he is not able to turn things around.

This has been a tough couple of months for us medically. Since starting school, Avery just can't catch a break. Over the past month he has been on 3 courses of very strong IV antibiotics and within a week off them he gets sick again. It's so frustrating. Isaiah has not been much better. He has been retching nonstop. He cycles every single Sunday without fail and at least one weekday. Still don't get his patterns at all but I know it's severly affecting his quality of life. And for me, I'm still not back to myself. By midday I have body aches and just feel so rundown. Although it's getting better each day. I have a couple of follow up appts. for myself this wk that I hope I am able to keep.

I guess this silver lining in the past week is Janessa. We are so proud of her. She is such a smart cookie. She was competing for Student Council this week and her speech was amazing. So great that she won for her class. Don't know what we would do without her. Also I forgot to mention she turned 11 on Oct. 5th, where does the time go?

Another quick stay thankfully. Avery's body is still getting accustomed to the other kids at school. He keeps getting these respiratory infections that quickly resolve with IV antibiotics. I'm really hoping his body starts adjusting soon bc being in the hospital every two weeks is not quality of life for any of us. I don't want to pull him from school unless I really really have to. He ends his antibiotics on tues so hopefully he can get a good healthy stretch.

I too am done with my antibiotics on tues. I am nervous to remove this PICC line bc I worry that with having such a large abcess that it won't be fully cleared. The last thing I want is to get sick again and have another PICC line placed. I went to the drs. On Friday and the infection did not come from a UTI bc the culture was negative. So it came frim my kidney. The burning question is how the heck did it get there. Nobody knows!!!!!

Rick and I are supposed to be going to NYC for the weekend. We of course got the insurance but I really hope we don't need it. This family needs a break from hospitals for awhile.

Seriously!!!!!!

That's all I can say tonight as I sit in the ER waiting on a bed with Avery who is hot as an oven. Maybe I was too ambitious to ask for a month off bc at this point I can't even get a week off the hospital. I just feel defeated right now. This is when I say "how much more can I take????". Cuz I don't know anymore. I'm just tired of the medical crap already. Life feels so unfair right now.

I will keep you posted on lil man. I'm thinking it's his typical respiratory infection. Guess we will see.

Recovering......

Is gonna take a lot longer than I thought....

I ended up getting discharged late Friday.  It felt sooo good to get back in my own bed and not be woken up all night for vital signs and to the old lady constantly hitting the code button.  I had not realized how sore I was until I walked outside the hospital room.  My only movement in the previous days were to the bathroom and back to the bed.  Needless to say it was very slow going for me.  I took some powerful pain meds and slept great but felt like I had been hit by a mac truck in the morning.  It was not until the visiting nurse came in the morning to help with my IV meds that I realized how sick I was.  I guess kidney abcesses are pretty rare and especially to the severity that I had.  Also my lab work looked horrible so they were completely surprised to see that I was already home from the hospital.  In general I don't feel at all like myself, and I have no appetite to boot.  I tire easily and just feel really rundown.  Rick kept on looking at me today b/c he couldn't believe how sunk in my face looked :(  I know it's gonna take time to get better and my energy back.  This just seemed to come out of nowhere.  I am a very healthy person who just got real sick real quick.  I'm hoping by the end of the week I will start feeling and looking like my old self. 

check out my new accessory

Enough about myself.....

Thankfully, the boys totally behaved themselves while I was in the hospital and home sick.  Of course Isaiah has continued to retch every stinking Sunday (which we plan at this point).  Other than that he has been great.  Avery is so much better now that he has completed his IV antibiotics.  Having taken some now I can see why the poor kid was so miserable.  Today we went for a TPN appt. and we were able to reduce his time on the pump from 20 hrs to 18 hrs.  Not alot but it's better than nothing and it will make things much easier for us at home.  Drs. thought he looked great today so I am hoping and praying he stays that way for a good good stretch of time.  I am so done with hospitals at this point.  I knew we would pay dearly for having 4 months off.  We just go through so much in the house with very little breaks so it would be nice to have a normal life for just a bit.  People keep saying to me "you do too much". This frustrates me b/c  I wish I had another choice but I don't.  I have to work, take care of the house and the kids.  I do take time out for myself often, probably more than most people b/c I have nursing.  It's the medical stress of our life that I need a rest from but unfortunately we have no control over it.  At this point I will take a month of no hospitals!!!!!!

Total Role Reversal

I will start by saying I have a newfound appreciation for what my boys have endured over the years. They are such fighters, it's amazing. But we do know the apple doesn't fall far from the tree.

So I'm gonna highjack Avery's blog (bc we know it's usually all about him :))......

Backing up to my CT results from Wed morning which had all the drs. in a panic bc apparently they were very bad. The Urologist that came by was totally shocked to see me coherent bc he assumed based on the CT and labs that I would be "half dead". The abcess was 4cm which he said was extremely large and rare. He had no idea how the heck I got it (his guess is as good as mine) and was only symptomatic for 4 days. They started me on two very strong broad spectrum IV antibiotics which Avery has been on numerous. My stomach has been as mess so I can only imagine how he feels on these.

The plan for yesterday was to drain and culture the abcess. So I went down to CT to have this done. They did a repeat CT to check the size and placement and it was still 4cm. They put a local anesthetic on the area and gave me some versed. The dr was unable to aspirate anything so he said that the kidney was severely inflamed. Hmmmm.... Anyway you can imagine what it feels like after to have a needle stuck into a severely inflamed organ. I will tell you just minutes after the procedure I was in the worse pain of my life. I couldn't see straight, tears were streaming from my eyes and the swearing devil appeared. It took a good 45 minutes to get the pain under control with morphine. I continued to have a couple of episodes of this once the morphine wore off. Once the pain was finally relieved I spiked a temp of 102 yet again.

This morning I woke up feeling better than I have all week. The urologist stopped by bright and early and he is not convinced that there was not an abcess and never am I. The plan today is for me to get a PICC line placed (temporary central line) so I can go home with these strong abx. It's great they trust my medical knowledge to send me home with a line but one of the IVs runs for 4 hrs at 3x a day. ugh!!! I think that is the one they are sending me home with. I have no idea how I am going to do this with my normal life but I'm sure I will figure it out quickly bc there is no way I'm sitting in a hospital. I do have to say that adult care is so different than pediatric care. I have entered a different arena but Have quickly found a way to navigate it. Although this whole roomate thing is just plain weird, especially when you have to share a bathroom.....

Thankfully the kids have done well in my absence although there was a crazy moment that was resolved. Save that story for another time. I am sad that I was supposed to be in Canada today visiting my frien Debbie :(. I know I can reschedule though. I have to get myself better.

This weeks posting was supposed to be all about Mitochondrial Awareness Week but I guess it was in a way. For our family, this disease has meant nothing but hospitals, pain, tears, new equipment, procedures and constant adjustments and much more. One of the drs. Said to me today that her husband is a pedi. And they just had a discussion last week about Mito parents. He said they are so medical savvy and at times know more than him. I chuckled when she said it bc it's the truth. Its survival for us and our kids. The more people know about the disease the more help we can get for research to find a cure.

Never a Dull Moment

Gonna make this quick though. I've pretty very sick all week and treated for what was thought to be a kidney infection. Despite iv antibiotics and IV fluids as well as oral abx I'm still running a 102.5 temp and feel horrible with chills and severe pain. I knew something was not right. My dr. Sent me this morning for a CT scan and it showed a large abcess on my right kidney. Not good at all. So they sent me to the hospital for admission and drainage of the abcess. Doesn't sound like fun but I am anxious to feel better. They plan on doing it in the morning so I will try to keep you post. Thanks

The Downside of School

Germs......

Avery is so enjoying school and it is definitely the best thing for him except for the snotty nosed kids that are in his face. I think we are going to have to build a shield around his wheelchair, lol. Avery only has only gone to school for two days and he already ended up in the hospital by the third day :(. He didn't even make into the classroom on Tues bc he was struggling respiratory wise. He had to stay in the nurses office for chest pt, suctioning and O2. By the time he got home he was feverish and sounding really wet. We ended up giving him a dose of IV lasix so we could safely drive him to the hospital. When we got there he continued to struggle even dropping his blood pressures so low that they were about to give him dopamine. Thankfully he started to recover his pressures on his own. Of course these behaviors earned him a bed in the ICU. By the time we got up to the unit, he was resting comfortably on his bipap and doing great. He recovered really quick bc antibiotics were started immediately and also bc he got lasix at home. He was doing so well in fact that we went home today. This certainly seems like a record for ICU stays right. Not so fast......when I got home he started to spike fevers, grrr... For now I am just going to wait it out with motrin and Tylenol bc he is still on antibiotics. But I may have to head back in if he starts to work to breath. Hopefully this is just residual from whatever he had on tues. Bc I DO NOT want to go back!!!!!

I have to admit that I had reservations about sending Avery back to school but when I get reports of how much he loves it, there's no way I can't. I'm just hoping his body gets used to all the other kids bc he really loves school.

This is gonna be a long one

I will start off with the good stuff.  Both Janessa and Isaiah started school last week.  Janessa was so excited b/c she is now in the upper school where she has a locker and switches classes.  She is so so grown up it's unbelievable.  Isaiah was not so thrilled. I think he thought he was done with school having a few weeks off and all.  He pretty much had a horrible couple of days last week, although you would not know it by the photo :) (he never misses a chance to smile for the camera).  Avery started kindergarten yesterday and I have to say that is was the best decision to date that I have ever made for him.  I honestly never thought that I would have the courage to send him to school but I did.  And he loved every minute of it.  The kid could not stop smiling with that toothless grin of his and on the bus ride.  He was so happy to see the kids and they loved seeing him even though they had a ton of questions. 

So as you can see, school is a great thing for the kids :)

Onto the crazy stuff:

Let me start off my saying that my birthday weekend was tough, not only was I stuck in the house most of the weekend but see Isaiah basically be out of commission the entire weekend was difficult. His migraines are constantly changing.  He prefers to be really bad when I don't have a nurse. Over the past couple of months he has only be cycling on Sunday's but of course since I was there with he decided to cycle both Sat. and Sun.  Poor kid.  I am totally convinced at this point that there will never be a cure for him.  We have tried everything there is to try.  This is just going to be his cross to bear and it really stinks for him.  I guess my only solice is when he is better he is back to his crazy wild man self and pretty much a healthy kid.  It just makes me really sad that he has to go through this so often :(

Onto the trouble maker :)  So last time I posted he was have some bleeding issues and very low blood sugars. I don't have an answer about the blood sugars b/c they have been great lately.  But I do know why he was bleeding.  He had gotten a few vaccines the week before for school.  News to me and the pediatrician is that his weekly IVIG infusions cover him for his vaccines so he does not need them.  So he apparently had a very bad reaction to the vaxes he had gottent the week before.  On Friday he was due for two more (live viruses) and Thank God the nurse started asking a ton of questions beforehand.  I let her know he was immune deficient and she was immediately alarmed.  She paged Avery's immunologist who confirmed that Avery is NOT to get any vaxes.  EEKK...So So glad she caught this b/c it would NOT have been good if he would have gotten these shots.  I am so relieved.  He got a letter stating he was not a candidate for the rest of his vaxes b/c of his IVIG infusions.  I feel better at least that there was a reason for his bleeding.  It would have been difficult not knowing.

So that crisis was resolved and onto the next one......Today we had an appt with a physiatrist to look at Avery's curvature in his spine.  This guy is very difficult to get into and usually books out 8 months for new patients .  Isaiah had recently seen him so I was able to get a sooner appt. for Avery.  Well, it did not go how I wanted it.  I mean I knew his curve was really bad but not as bad as the dr. thought.  Basically it comes down to him either getting spinal surgery for quality of life (being able to sit comfortably in his chair or dying a very slow horrible death from all that comes with your back being so curved such as Congestive Heart failure and many more issues.  While it is a very very scary decision b/c I feel like I am pulling a trigger, I have to do what is best for Avery.  So I have made an appt with the top dr. who luckily happens to be at Children's Hospital for this procedure. 

This past 6-8 weeks have been really tough emotionally on me.  I feel like one crisis is resolved and then a worse one is to follow.  None of these decisions are easy at all.  I would have never thought I would have to make these decisions for my 5yr. old little boy.  It is so so tough.  Sometimes I just feel like I am going through the motions of how everyone expects me to be with a smile on my face and going to work and trying to live life when all the while I am dying inside with each decision I have to make.   But then just as I feel like I cannot do it anymore, I have tears of joy as I see that toothless grin on Avery's face riding that school bus or Isaiah cracking up laughing to Spongebob.  It makes it all worth it in the end.  Just wish I could take all their pain away. 

So there you have it.  I was hoping for a very upbeat post but it didn't happen so hopefully the pictures put a smile on your face.  How could they not....

I don't even know how to begin summing up this week.  Basically the emotional roller coaster has continued but more along the lines of stress, relief, sadness and frustration.  Coming off my last post regarding Make a Wish, we have decided to cancel the Disney trip.  I actually feel good about my decision but sad that we can never go on a family vacation (at least not with both boys).  I know it was the best decision for Avery though and that is what it's all about right now.  He is just too unstable and I cannot even imagine the medical crisis that would incur while we were in a foreign place with nurses and drs that don't know him.  We are just going to have to figure out something that would work for him. 

I am also relieved that we survived Hurricane Irene without losing any power at all.  I'm pretty sure I drove Rick completely nuts with all the supplies and tying down everything in the yard and powering everything we could possibly power in the house.  I have been through a pretty bad hurricane while I lived in North Carolina and that was pre having two special needs kids.  Although Rick's dad dropped off a generator, I was still freaking out b/c everything Avery uses requires power.  He would not have been able to use his bipap which is concerning. The last place I wanted to end up with inpatient.  Not to mention Isaiah would have had a complete nutty without his big screen tv (I am being very serious).   Rick assured me the whole week it would be fine (while rolling his eyes, lol) and  I am so glad it was.  There are still so many families across the state without power.  I sure hope it's restored soon.

On the morning of the hurricane, Avery decided he was going to started bleeding profusely from his central line for no apparent reason.  NOT GOOD.  Yet another reason for me to freak out.  He continued to do this for a couple of days.  Labs were drawn and his platelets were continuing to drop indicating that he was in the early stages of DIC (bleeding out).  Up until tonight his labs were looking like he was not clotting either.  Yet another reason for momma to freak b/c this is what he did last summer.  A redraw of labs by his veins showed significant improvement.  Which is so good.  Meanwhile ,this mornings labs as well as last weeks (which they chalked up to error) showed his blood sugars at 35 (so so not good) just minutes of getting off his TPN.  Since he had a low value last week, I decided to do a finger stick while he was on his TPN (IV nutrition) and it was 47 so I knew that the previous values were not errors and were true.  It seems the outpatient team is not too concerned about this which is frustrating the heck out of me.  Let's face it, my kid was bleeding out from his line this week with lowering platelets and has basically been in ketoacidosis and looking pale as all hell and they are not too concerned.  Really????? So can you see my frustration?

Avery is going to the pedi in the am to get his last round of shots so he can start school next week.  I will talk some things over with his pedi and have him take a look at him.  He has actually been in agreement with me about Avery's bleeding issues where as the outpatient team showed little to no concern.  I guess we just know Avery better and how he throws these little pebbles to let you know a boulder is coming.  Needless to say, I have no nursing all weekend and it's my birthday.  I have spent many of them at Children's so it would not be surprising if I ended up there.  I am just really concerned that something more is going on with my lil guy and it's not good.  I hate that mommy instinct sometimes.  Just hoping I am wrong this time. Guess only time will tell.....

Emotional Rollercoaster

Is the best way I can describe the past month....

I will start with the good emotions:

Happiness: I have no idea what we have done but Avery's fluid issues have seemed to resolved themselves for the time being.  His weight has been pretty good and his urine output has been better than it has in weeks.  We have done nothing different at all.  He hasn't even need a dose of IV lasix (Thank God), especially since everyone is so freaked out about it.  I mean I know it is a big deal but he did very well with it and is being watched very closely.  His labs that I drew yesterday were great.  I am just so happy he is good right now.  I can only hope and pray that it lasts.

Another thing that makes me smile is that the boys will be riding the school bus together.  Just makes my heart smile :) Although Avery will only be going 2 days a week, he will still ride with Isaiah.  They will also be going to two different schools.  It works out and I am so happy they get to be together.

We had a good weekend rafting.  I cannot say how therapeutic it is for Rick and myself to do normal things with other couples.  Plus, we really need our time away.  It was such a beautiful place and the rafting was CRAZY.  Our raft flipped over (all 8 of us) but we survived and honestly that made it so worth it.  I am just happy we are surrounded by such good people. 

Ok now onto the bad feeling.......

Sadness: My good friend Debbie from Canada lost her daughter Jaiden on Friday.  It is so heartbreaking and no parent should have to go through this especially twice.  They are such great people and wonderful advocates for their children.  They need your prayers to be able to get through this difficult time.  Rick suggested I go see her in the next couple of weeks as a birthday gift and I thought that was so good of him.  We share a bond like no other and I want to be there for her, if only for a weekend. 

Fear: that my boy is slipping away from us.  Sure he is doing great right now but the level of his care has changed so dramatically.  Anything could take his precious life at this point and it tears me and Rick apart.  Who would have ever thought that our dinner conversation would consist of us planning our baby boy's funeral.  But it does and THAT is our reality and it sucks big time. 

Confusion: the whole Make a Wish trip this Fall.  I am leaning towards not going.  Who am I doing it for us or the boys? Rick, me and Nessa can go on a trip anytime.  Let's face it, Isaiah hates being away from his tv for 5 minutes let alone getting on an airplane where he has NO idea where the heck he is going. He will be retching his guts out for a couple of days.  Then you have Avery who could very quickly go into a respiratory crisis either in the security line or on the airplane.  We would end up bagging him as we enter the plane.  Now does that sound like fun, I think not.!!!! He would be perfectly happy going to an Elmo show and so would Isaiah.  I don't want to make his situation worse b/c we felt like we needed to go on a family trip b/c the reality is that is not for our family. So while it sucks I feel that it is best for Avery and Isaiah. 

I think this about sums up my feelings over the past couple of weeks.  You know we are heading into Mitochondrial Awareness month and I find it so sad that in the past year we have lost about a dozen kids that I know just from online and in-person interactions.  We just really need to find a cure for this horrible disease b/c it just destroys lives including ours :(

Home

We have actually been home since yesterday but the transition is always crazy so blogging is the last thing on my mind. I have to say that this homecoming was much smoother than the last two. He is not as sick coming home, although getting his lasix orders finalized is crazy.

Over the weekend Avery did very well without lasix, actually much better than I expected. But he did require more fluids. So now his IV nutrition went from 16 hrs. a day to 20hrs to see if his body will tolerate it better. In the past few days he has shown that it is working. However we still have lasix (IV) in our back pockets. While this can be a scary med at hone and certainly not typical. Avery was monitored very closely inpatient on it and was very stable. So the team feels it's completely safe and effective. It will also keep us at home with close monitoring. Well once we were released seems the outpatient teams are freaking out which pisses me off. We nor I would do anything with Avery if it were unsafe. Everything is very thought out or we wouldn't go home. So orders have been changed bc people are trying to Cover their own asses and not what is in the best interest of my own family.

Anyway this has turned into a total rant but what r ya gonna do!!!

Just happy to have my lil man at home....

I hadn't even realized it's been a week with no update, Eek. Things have been a bit confusing from a medical standpoint so I'm going to try to speak in layman terms. Avery has been here for a week and I can't really say that we have accomplished a ton except his lungs are less wet. Which means he is better from a respiratory standpoint. It's all about the balance of his lungs vs. his kidneys. He has been requiring up to two to three doses of IV lasix (takes the excess fluids off his body) a day. Without the lasix he is not producing any urine at all, therefore his weight is increased in the am up to three pounds from the evening. This has to be watched closely because we know that any excess weight or fluid he has goes directly into his lungs. So we have been spending the week playing with his fluids as well as the lasix. Currently, his kidneys think he is dehydrated so any fluids he he takes in them store them but the rest he third spaces (leak) into his lungs. Surprisingly, he is very happy (even lost another tooth, poor kid is all gums) and he doesn't sound too wet which is very good. But we know the kind of kid Avery is, he can flip the script at any moment.

Last night we were home and the renal dr. that has been consulting on the case called me. What a nice guy. He explained a lot, and he felt that in fact Avery's kidneys were pretty "smart". That is why they are holding onto the fluid bc they think they need it. Some testing he did confirmed that but he said the lasix he has been getting also taint the test. Although he does not know why he is not urinating without lasix and is third spacing into his lungs. I told him it's likely mito related and also bc all the vessels around his head and neck are completely stenotic and he didn't disagree with that. His recommendation is to completely stop all lasix and wait and see. We know he will go over 24 hrs with little urine and be much heavier in the am but he feels if it's not affecting him respiratory wise then to just ride it out. He wants to be sure he actually needs lasix before testing it out without them. I do agree but it is going tough weekend for him and I'm so glad this is getting done inpatient. Again, there is a good possibility his body needs the lasix but he doesn't know why. If he needs it then he does. It's just another big intervention for home especially with a kid like Avery so I would like to avoid it if we can.

Hope you guys were able to understand at least some of it bc I'm not sure if I even get it myself. I'm hoping we get get things squared away by mid next week. Rick and I are going on our annual white water rafting trip it I would feel better if he was back to baseline while we are gone. I know wishful thinking!!! Only time will tell.

Please check out this video montage that we have started and let me know what you think?????

To say this has been a tough week for our family is a complete understatement!!!. Since Avery came home last Friday we (nurses included) having been running a critical care unit. He has been so very respiratory had due to fluid overload. His weight in the morning is b/t 3-5 lbs heavier than in the evening and as a result has been so wet in the lungs. I had been communicating all week with drs. at Children's but felt he was stable enough to be home. Looking back I was definitely out of my mind. Even Janessa knew as she said a number of times throughout the week that Avery needed to go back to the hospital. Sad huh, my 10 yr old knows better than me. Adding to the stress of trying to keep Avery stable is Rick was at a basketball camp that he has done for the past 30yrs. He was not home until 12am every evening so I was on my own and it was taking it's toll on me.  Also, my job had made a big announcement on Monday that they were laying off a ton of people so now more than ever I need to be going to work. On Tues Avery started out the morning rough but I had to go to work for a few hrs and felt my nurse could handle him and she did but nonetheless I felt anxious (what a horrible feeling). I basically said that if he gets bad just call 911.  Not good.

By the end of the week I promised to the nurse practioner I would take Avery to the pediatrian and decide from there if he should be admitted or not.  He had very stable times and once he was alseep on bipap he was fine so that is why I felt he was ok to still be home.Well that is when it all gets interesting!!!! I thankfully had my nurse with me. Avery was already sounding wet in the morning so I "think" I knew he was going in. By the time he arrived (25 min) drive, the decision was made bc here me and my nurse are bagging Avery in the parking lot with O2, sat monitor that is constantly going off. I mean we must have look like a bunch of crazy woman with this poor sick kid. So we get him stabilized and grab our to go crash cart and calming walk in where I tell the front desk staff that they will be needing to call an ambulance for us. They took one look at him and said " I guess". So we get back to a room and he starts up again and I now have to yell for the dr. Who sprints in the room and I'm sure is ready to have a heart attack. Poor guy. Now by this point my nurse and I have been doing this all week so we were very calm but I know he thought we were out of our minds which I'm sure we were. He calmly or least "acted" calmly as to not alarm his patients that were there for ear infections and what not. He then has a nurse quietly bring the code cart which he no longer needed bc I got a good suction and brought his sats to the high 90s. By this point you could hear the loud sirens ( so much for keeping this quiet) and we were on our way. Of course Avery now looks like a million bucks... Upon leaving the pedi wanted to try to get Avery directly admitted to the unit to which I said "good luck on that one" .

So now Avery and I are in the ambulance with sirens ( 1st time) bc of the Red Sox/ Yankees game, lol. Avery is happy and breathing on room air. We arrive at the ER and what do ya know, we are directly admitted....WTG Dr. R. The attending said Avery has the VIP pass, lol. Actually we got lucky there were open beds but I was happy either way. The dr. had a heads up of what was going on at home bc of all the logged in calls and they were shocked he lasted this long at home. Even though he looked great on arrival his chest xray was horrible. His lungs were full of fluid. I now feel so so bad that we let him struggle that long at home. We do so much at home that it is getting more and more difficult to find the line of when to take him in. At this point I feel relief that he is there. It was more than all of us should be handling at home especially my nurses who did an awesome job of keeping him stable. I
Really don't know how long he will be in and I'm not going to rush it. He needs to get better. I am just really getting sad, nervous, and scared that he is slipping further away from us and this is why I fight so hard to keep him home with us.

As I go through my own turmoil, I am completely heartbroken for my dear friend Debbie White and her family. They have taken their daughter Jaiden of all supports and are saying goodbye to their beautiful princess. It's awful. Her and I have had many conversations about the passing of our babies and I am just so sad that they have to go through this. Please send the White family who have a son Logan and another daughter Marlo also with progressive mito and her husband Scott prayers in the coming days, weeks and months. Nothing about what we deal with is right or fair, it just isn't....

The "Crazy" in our Normal life

We did make it home on Friday early afternoon but man did I need a shot of something after that caride home!!!! At this point, I can no longer drive Avery to and from the hospital alone. He is just too sick. Let's face it, what kid comes home straight from ICU in baseline condition. Umm, not my kid. He was a plain ole "hot mess". Janessa even commented an hr into our craziness of settling him in home " I don't think he was ready to come home mom". But at this point we know Avery's new baseline after each hospitalization is going to be "sicker". But still we choose to take him home because we know that is where he is the happiest. No matter how rough his day is at home, when you put him in his bed he has this smile that confirms where he wants to be.

That being said, the past few days have been an ICU treatment facility in the Nault household. Avery's oxygen levels have dropped so low that even at Children's ICU they would be nervous. For now we have been able to stabilize him by drawing labs, giving extra IV fluids and measuring inputs/outputs. These are all things that are completely foreign to most people but have become a normal way to keep our boy at home. Sure he makes my heart race while we are doing these crazy interventions, all the while Isaiah has gotten himself stuck in some obscure place and the dog is eating one of my towels while Janessa is asking me to do something for her. But it still beats sitting in a hospital waiting for someone to suction your child and eating the same food everyday and looking forward to rounds and young drs. Giving you permission to go home.

So hopefully we will be able to rough this storm at home with the help of drs and nurses from Children's bc in the end that is where a family should be!!!!

Homebound

Sorry for the lack of updates. There hasn't been too much going on except trying to clear up the nasty yeast rash Avery developed and improving his respiratory status. We think it was a reaction to the IV bactrim he is on. He has had this antibiotic numerous times but not in the IV form only because there has been a national shortage. So it seems to be a reasonable explanation that he developed the rash less than 24 hrs after starting the IV bacterium. Today is the first day it is starting to look a lot better. He is also requiring much less suctioning than he was at the beginning of the week. So as much as I hate being inpatient, he needed a good two weeks to get a few things worked out. He looks and sounds GREAT. He even got a picture with the New England Revolution Soccer team :)

I'm hoping to head home after rounds late morning. At least that is what I'm shooting for but we all know how long it takes to get discharged. I cannot wait to get back to our regular routine and I'm sure he is excited too. Rick just got back from his work trip and he will not be around next week because he has basketball camp. I honestly don't think I could make it another week with Avery still in the hospital and balancing work, hospital visits, the other kids and house stuff. This was a very tough week for me. Hopefully Avery has had his fill of the hospital for a long time, I know I have.....

Still here!!!!

We were actually supposed to be discharged on Sat (papers in hand and all) but we would have definetly had a repeat of the last time only we would have not even made it to the gas station.  Avery is still very junky and poops out so easily.  By 3p in the afternoon he is just a hot mess with high O2 requirements on his bipap and terrible blood gases and lactates (which all show he is tiring out).  He almost seems worse respiratory than when he came in. He was switch from IV antibiotics(abx) to tube form on thurs. and seemed to get sicker so they switched him back to IV.  They are questioning whether he is absorbing his meds through his gut is why they switched him back. 

He is still running on the dry side and his kidney numbers have showing the same (which are also worse than when he came in). There is question on whether the extra fluid in the IV abx (extra 200ccs) are helping him and so when they were converted to tube form he got more respiratory b/c he was losing another 200cc of fluids.  His secretions got thicker and he was producing a ton more and just working really hard to breath.  He seems to be in a vicious cycle of being respiratory, sweating profusely which then dries him out.  I really don't think he is sick with an infection as nothing has ever come back showing that.  Also he has a really horrible rash on his bum and back so the poor kid has been miserable today.  So as you can see we still have some issues to work out before getting discharged. 

Of course it could not have come at a worse time b/c Rick is out of town for a few days. It is the end of the work month for me and I have tons of stuff to do to close out the month.  Unfortunately, Avery will be hanging at the hospital a lot by himself this week b/c it is going to be too crazy (which makes me really sad ).  This is the really hard part about having sick kids and trying to balance it all.  I'm sure it will all work out in the end and I don't want to take him home prematurely only to head right back to the hospital, it's just going to be a tough week.

Update

Soon after I posted on sat. evening, Avery ended up going to the ICU bc his blood pressures were very low. This has only happened in the past when he has been septic. With having a central line that is always a concern. Thankfully overnight fluids corrected the problem. They started him on a couple of IV antibiotics and he seemed improved by Monday. So I was feeling like this was gonna be a really quick stay. Silly me, things are never that easy. Turns out we have a few things to work out before going home. Firstly, his weight has dropped quite a bit so the first thing they adjusted to his TPN but now his labs are showing that maybe he is using a lot of his energy to breath and is burning a lot of calories. Therfore,he dries out very quickly which makes his respiratory secretions really thick and difficult to manage. The curveball is that in most kids you give them extra fluids and problem is solved. But in Avery's case, the fluid does not always go where it should and sometimes goes into his lungs so there is a fine line and not something I'm comfortable doing at home. Also he is requiring more hours of bipap due to his fatigue but they are finding his blood pressures are dropping overnight and his blood gases are low. Not sure how the bipap affects his blood pressures but it is. All of his cultures have come back negative and yet he is presenting like he has a bacterial pneumonia. So they are treating him that way with antibiotics. So I want to make sure these things are resolved before taking him home otherwise we will be right back here. He is able to move to the step down unit but there are no beds. I'm sure he will be there as soon as one opens up. Hoping the boy doesn't have any dramatic tricks up his sleeve and we can get out by the end of the week. I will keep you posted.

Oh and some good news, Make a wish approved a full-time nurse to we are all going in the fall. I'm nervous as all heck but I hear it's so worth it.

Four Months

It's been four great months of being home. Our family has gone on a number of mini vacations and we have just enjoyed playing the role of a non medical family. Well unfortunately that has come to an end as we sit in the ER (Avery settled in on bipap) and about to head to the unit in a bit. I'm not at all surprised by this admission because he has been struggling for the past week. Avery scared the heck out of us on Sunday with desats into the 50's. He has also not been producing much urine at all the past couple of days. Basically the fluids he has been getting have gone straight to his lungs. He is very wet sounds with crackles throughout his lungs. Not really sure why he is third spacing bc his labs actually look really good. What sent us running in here was a fever. When Avery gets a fever pretty much all bets are off and I need hall my butt to the hospital. I'm glad I went with my gut that was telling me he was going downhill fast as he had not even spiked on the monitor but his body was hot and he was breathing really fast. By the time we got to the ER he was high and we were immediately sent to the trauma room (never a good feeling) but was able to be moved bc respiratory knows him well and got him on bipap quickly.

So knowing Avery, he has some type of respiratory infection. I will take that over a line infection any day. Hopefully he gets on his typical antibiotics and gets healed and back home soon. I will say that after being away for four months and seeing so many mito kids die lately, it was scary to come in here. You never know when it's gonna be your time at the stage Avery is at with his disease. I do feel a little encouraged that his labs are not too bad and we caught him early. Guess we will see what he has in store for us!!!!!!

Bittersweet

For those of you who have been following Eithene ( Avery's mito friend) who fought the hardest and longest battle I have ever known.  She earned her angel wings on Thursday morning.  I cannot begin to imagine the pain her family will go through in the coming weeks and months.  Please continue to pray for their healing as they face a new life without their princess Eithene. I hear many mito families who lost their babies in the last couple of years talk about them all greeting and playing with one another tube and pain free.  I can only hope that is what they are all doing up in heaven. I guess there is some comfort in knowing that.  I'm sure Eithene is in her prettiest princess outfit and waving her magic wand with her angel wings right now. 

On the same day Eithene's family had her service, we baptized my niece's baby Analeah.  So you can see why I used "bittersweet" as my title.  Rick and I are proud godparents to a beautiful baby girl.  Although she was not too happy about the whole ceremony.  But we are happy and honored to be her godparents

In other news, things are status quo with the boys.  Isaiah still continues to have cycles but I am hoping the med changes will make a difference, although I have little faith.  Avery seems to be doing well.  This week makes 4 hospital free months, Wooohooo!!!! I think he is starting to embrace life at home finally :)  He also got a new seat.  I am so excited to have found something comfy for him.  Although Rick is gonna kill me for buying yet another seating thingy but I promise to get alot of use out of this one :) 

I think I'm gonna like this one dad

Hello!!!!

I know it's been a long time since I have last blogged. I think we have been caught up living a normal life that there hasn't been a need to blog. Rick and I have been lucky enough to go on mini vacations (separately of course). I went to Martha's Vineyard for the weekend and to another part of the Cape last weekend. We have even gone to the Red Sox together and Ness and I have been enjoying time on a boat at a friend's house on the lake. So you see, all " normal things" that we were never able to do. It is truly amazing what life is like when Avery is not in the hospital. He is continuing to break records!!!! Next week will be four months hospital free, the longest ever in his life. I just feel so so blessed right now. Since I last posted, that little boy Zach lost his battle to mito after only being on hospice for less than 48 hrs. And Miss Eithene is still fighting and obviously God has a plan for her. Please continue to pray for her family, I cannot even begin to imagine how it is to see your baby at the edge of heaven's gates for this length of time.

While we have been living a normal life for the most part, it has not been without it's struggles for the boys. Isaiah's cycle are at an all time low. He has been having migraines every Thursday and Sunday which last a couple of days. So basically, he has 3 good days of the week. Not good at all!!!! We saw his mito dr. on Tuesday and she changed a couple of meds but she had little hope that we would ever cure him of this :( so so heartbreaking. On the plus side, she was thoroughly impressed with how strong he has gotten. She actually look up his labs from his last visit which shows how much mitochondria he lost from his depletion disease and he has 65% which she thought was great. This crazy boy has defied so many odds with this disease, it's such a blessing.

Avery has been dealing with increased apnea spells which are really scary. He has his days that have been horrible respiratory wise. I swore he would be inpatient but the next day he wakes up totally fine. We did have to increase his home bipap setting and switch his masks (due to skin breakdown). I am a little nervous that one of his nurses who is aggressive respiratory wise has left to Africa for 6 weeks. I'm just hoping we can all work together to keep him home for the rest of the summer and break more records. Other than that he is doing awesome. I did ask my mito dr. About the experimental medication for kids with Leigh's disease ( which is what Avery has progressed to) but she is not sold on it yet but will look into it more. But she is finalizing Avery's Disney wish trip. Hopefully we will have a date soon. We are nervous but excited :).

Guess that's it for now. No news is good news!!! Hoping our life continues to be drama free.

I can officially say it has been three months plus that Avery has been home from the hospital. Over the past week I have had such mixed feelings about this. Anywhere from joy, fear, to sadness. I am so thrilled that this is the longest he has made it home since he was a newborn. Fearful that the next stay could be a "major illness" type of stay. And sadness, while I am rejoicing my boy being home another mito family has laid their sweet boy to rest while another family is awaiting the day their princess leaves them.

We have certainly had a few hiccups in the past week but thanks to my nursing we are still home. Last week we spent the day at Children's because Avery's central line was pulled pretty bad. Thankfully it was still in place and he was able to go home with a functioning line. Also he has had some pretty bad respiratory days. One of his nurses that does a great job with stabilizing was on edge that he would surely end up inpatient. Somehow she was able to turn him around. I am nervous though because she is going home to her country for 6 weeks (in July). Just hoping we can all keep him healthy and out of the hospital. It is nerve wrecking though. Guess I am being a bit selfish!!!!

Isaiah, poor kid. He has been having the worse time with his cycles lately. He has been retching about every 4-5 days now and they last about 2 days. Add that up and it equals a pretty crappy summer. I honestly cannot figure him out after all the years but I do know it breaks my heart. We are seeing his mito dr. In two weeks and I am just hoping she has some new ideas. Although I know that over the past 6 yrs. We have run out of options. It just really sucks.

So while we have been enjoying life at home, it is far from boring. But I'm just happy to have my boys. Please continue to pray for these families.

Feeling Blessed and sad at the same time

Have I said how much I hate this disease? Well if it hasn't heard me then it better know how much I hate it. Way too many babies have been taken in the last year to this horrible disease. It is the new Cancer as I see it. It's brutal and leaves families devasted often taking many in the same family, much like a tornado.

Right now I feel blessed as all heck. It has almost been a year since we almost lost Avery. I would have never thought we would be sending him to school a year later. Instead we could still be mourning his loss. We have another year of his smiles and funny faces. For this I am so blessed. Isaiah just celebrating his 9 th birthday is another huge blessing. I will take what I can get at this point. I'm gonna cherish everyday I have with my boys bc at any point Mitochondrial disease can decide to take the upper hand.

Right now my heart breaks for three families that are losing the battle to this disease. One very special Princess is Eithene (please visit her blog and send prayers to her family on the side panel of my blog). I have known her since she was 6 months old and you could not help but fall in love with the girl with the little voice and tu tu. Many of nights her mom and I have spent at Childrens saddened by our kids ailments and struggles. Eithene has won the hearts of many all over the world. But tomorrow she will be placed on hospice. Please pray for her family as they face these difficult times. Also a beautiful three year old boy Zachary who in his short life has fought the beast called Mito will also be placed on hospice. Also one of my closest friends is fighting for her daughter, she is one of the best momma's I know. So please keep all these families in your prayers.

None of this is normal or fair. No parent should have to go through this especially with babies. Right now I am living a fantasy life (as I see it) but my time will come eventually. But for now I will continue to feel grateful and blessed and love my boys as much as I can.

Happy 9th Birthday Isaiah!!!!!

This is my most favorite Isaiah photo.  Look how handsome he is.  You would have never guessed by looking at this picture taken at 9 months of age that he was a sick boy afflicted with severe Mitochondrial disorder.  I call him my unsung hero.  I say that because Avery has taken the spotlight for the past 5 yrs with all his dramatic medical issues.  But way back when Mr. Isaiah was this beautiful baby that we never thought would live past a yr. b/c he was so sick with the disease.  In the earlier yrs, all the research said the sicker you are as an infant the worse the prognosis.  Hmmph, well this little boy proved them all wrong.  And I am so so grateful.  Honestly if it was not for his retching cycles which have such a significant affect on his life, he would be a physically disabled boy but nothing would stop him.  He is so smart and funny and a crazy little stinker.  He loves to bother the heck out of Janessa (pulling her hair), Milo (constantly steam rolling him or pulling bones out of his mouth), and Avery who is helpless to his older brother pulling all of his tubies.  He is just such a great kid and I am so proud that he proved all the drs. wrong.  I want to wish you the Happiest 9th Birthday ever.  We love you so much Zay Zay and keep proving everyone wrong, especially this disease.  And please no retching tomorrow for your birthday b.c we have lots of nice gifts for you :)

"Normalcy"

So that is the reason why I don't blog as much.  I feel like we are actually living a somewhat normal life.  Both Rick and I go to work and the kids go to school.  No crazy hospital runs and millions of drs. appts.  Although I'm always waiting for the other shoe to drop such as up & coming Holidays b/c Avery typically likes to spend them inpatient.  Or maybe it's b/c he is coming up on a 3 month stretch on June 15th.  It's kinda crazy that we make plans these days and are actually able to keep them.  I cannot tell you how many times in the last 10 yrs I have had to cancel b/c the kids were inpatient (and that includes Janessa).  All this can change on a dime but I have to say that I am getting quite used to the normalcy. 

So in the spirit of normalcy we just had Avery's IEP meeting.  For the past 2 yrs we have been discussing the possibility of Avery going to school physically. Currently he is homeschooled and it works well for him but he doesn't see his peers. And after everything that happened last summer I thought there was no way my boy would go to school with kids on the school bus.  Well the time has come!!!!.  My boy is the best he has ever been (sure he has some rough days but in the scheme of things he is great) so there is no time like the present, right!!!  It was decided that Mr. Avery will go to school 2 days a wk just for 2 hrs.  I know that is barely nothing but we have to start very slow with him.  Remember he does not have much of an immune system and what he has is a result of his weekly IVIG treatments.  He is not used to being around kids at all.  This is a scary thing for us all (including our pediatrician).  He even posed the question of "Are we doing this for Avery or for us" .  I thought this was a great question and this is why I think he is so good for us.  Truth be told, a year ago it was for "us" but now as I see Avery's personality flourishing as you saw in the Globe article.  How can I not send him to school to interact with the kids.  I'm sure the beginning will be rough as his immune system figures out things but in the end I think it will be great for him.  Who knows, if he does well then we will increase his school time.

 I really hope that it becomes the trend of our new life.  But then again, it is Mitochondrial disease.  Just when you think you are doing well it comes back to bite you.

But for now I am gonna continue on the positive path b/c it's working.  Avery has a couple appts. at Children's on Friday (he has not been there since his last inpatient stay) and while I would love to visit people on 11 south (our 2nd home), I probably won't b/c the last time I did after a 3mos hiatus we were inpatient the following wk. Hopefully it is a quick tube change and a quick hi to the Palliative Care team to tell them how awesome he is doing :)

Pictures and Update







Oh I thought it was MY birthday



Vacation Pictures





So as you can see we had a great vacation.  Although we all left with sunburns, especially Rick.  We did a day at Busch Gardens, then Adventure Island and Clearwater beach.  We had a few mishaps at the AI waterpark.  Yup, we lost our daughter in the park for awhile.  Nice huh!! For once our life is simple and we can't manage to keep track of one kid, lol.  luckily she was smart enough for find a life guard to search for us.  Never a dull moment with us.  Overall it is just really relaxing and fun to have a vacation that doesn't involve the medical world.  The boys did great had home.  No problems whatsoever.  I think Milo missed us more than they did, lol.

On the medical front, Isaiah has been struggling more with his cycles. I swear it is allergy related.  He always seems worse this time of the year.  I think he is getting allergy related headaches which triggers his cycles.  It just makes more sense to me.  Hopefully they will slow down during the summer.  In a few weeks Isaiah is going to be nine years old.  I have NO idea where the time went on that one. What a big boy he is.  This will be his last year in single digits, I cannot believe it :(

Avery has been more or less the same.  He has his days that he is really congested but we are always able to stablize him by the night.  He has been pretty good about the bipap which makes a huge difference for him.  For the most part he is doing great.  I don't have any complaints or concerns about him lately.  It's so great to see he is able to stay home for longer periods than the previous years.  I'm just so grateful.  There have been a lot of changes with my work and I very busy now so I try to get as much work done as I can when he is good b/c you never know when things change.  I have even manage to work out 6 days a week for the last 8 wks., wooohooo. 

Life has been pretty good around here and I am certainly counting my blessings :)