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 :)