Hope you like the new pictures. It is always tough to get new ones of the boys smiling. Although does Isaiah just crack you up or what. He is smiling in every picture and you can't even see his eyes. He just kills me.

Isaiah woke up this morning and Rick was bathing him and all you could he was him cracking up laughing. So this is our sign that he is feeling great and it is going to be a good day. Music to our ears.

Then there is Avery who typically wakes up screaming to get him out of bed. He is sooo congested these past few days. So we know what his day is going to be like. He needed alot of nebulizer treatments today. I am worried he might have another aspiration pneumonia b/c he has been also running a low grade few too. I sure hope not b/c it means more antibiotics and hello c-diff for the thousandth time.

Next week is going to be tough. The school is coming out to evaluate Avery for preschool services b/c he will be transitioning out of Early Intervention which is very sad and depressing. As I said before, I REALLY think we will keep him at home. He is just starting to be somewhat stable and I fear that he will go downhill once he is in school. Lets face it, he is NOT the social kid Isaiah is.

Also pallative care is coming out to add Isaiah on. It will be good b/c he will be followed closely in terms of his cycles and anything else that comes up. BUT.... it is weird having my Isaiah on Pallative Care. Two kids out of three on Pallative Care is not a good thing. But it is for the better.

Please pray for my mom as she is going through a tough time with losing her best friend this week. Thanks.

Picture Time

It has been almost a week that Isaiah has been retching and feeling really miserable. He has missed over 2 weeks of school. The previous week was b/c he nurse was out sick. It is so aweful and sad to see he like this. His cycles have really change to where they start off bad and drag on for about 6-7 days. Could you imagine has something where you were completely sick to your stomach for that long. He just looks so sad. Then by late afternoon we see our loving, happy and little stinker back. I am so happy he can finally go to school tomorrow.

Avery is hanging in there we are needing to shut off his feeds often b/c he belly really starts to get hyperactive and he becomes miserable. They just changed his TPN back to 16 hrs but more calories so we will see if he starts to gain some weight. I sure hope so.

Hoping for a quiet week but we will see :) Thanks for reading

I figured I would do something different tonight. Lately I have been getting alot of feedback from friends and family of the many questions asked about our family. So I will answer the top most asked questions.

1. Is it genetic?
Well many types of Mito disease are and it seems pretty obvious that ours is. Only nobody on either side of Rick and my family has the disease that we know of. We have no way of knowing what the gene or mutation is yet, so therefore they cannot even test Rick or me or Janessa. It is like finding a needle in a haystack but probably worse.

2. Why would you have another kid knowing your first boy was sick?
This question bothers me the most and you would not believe how common it is to ask people around us b/c they would not dare ask me or Rick. But getting to the point. We did go to Boston for genetic counseling after we had Isaiah and ran through all of the alternatives. The drs. really had no idea about the disease and the reoccurance. Besides that it was a VERY personal decision and we got what we were meant to have. Avery as I have always said is here for a specific reason and has changed our lives in so many ways for the better. I cannot imagine life without him :)

3. Are they going to get better?
No, it is progressive degenerative disease.

4. Janessa must really have a hard time?
She is very intelligent and could probably spat out medical terms better than most. Rick and I work really hard to make sure she gets all the love and attention she needs. She also sees a therapist 2x a month which is great. But she loves her brothers and is a great big sister.

5. What is their life span?
We have no idea it depends on how sick they are as babies. Frankly, Avery is much more limited and if he lives beyond 5 it would be a miracle and he just may. Isaiah, who knows I see him hanging out with us through his late teenage yrs.

6. What is there is quality of life?
This is very important to Rick and me. We will always put the boys quality of life FIRST. I guess it makes you enjoy the very simple things in life like laughs and smiles and Isaiah getting into things and pulling Janessa' hair. Avery cracking up when mommy talks b/c she is so animated. That is quality to us.

7. How do you and Rick keep things going?
We have been together for 18 yrs with MANY ups and downs and growing pains. I do have to say having our kids has brought us closer together and we are always united on our decisions. Sure some days are hard when I have been at the hospital or drs and Rick has work obligations but we always find a way to make it work.

8. What are the limitations of your life?
Well you can never plan on things for one, we can never go out as a family b/c it is so difficult for the boys and they really don't enjoy it. Nothing is predictable and you often have to dissapoint people b/c the boys can go downhill so quickly. Basically you just live in the here and now.

9. Do you ever imagine what your life would be without the boys?
My gosh, I feel like I would be this dull and insignificant person. You know before I had kids I had this spiritual journal that I kept up for some time. I was always questioning what my purpose in life was. I know this sounds all cynical but it is the truth. I really did not know my purpose until I had my kids and these boys. I have matured in so many ways and don't get caught in the material things and really try to enjoy the simple things and life and have become sooo much more grateful about life and knowing my blessings. So I CANNOT imagine what life would be like without them.

10. What are your support systems?
I have really found some great people in my life that have helped me along my journey. Some only last for brief periods and I believe each person is in our lives at the times we need them. We are lucky to have such great community around us and My MIL who is wonderful. Mostly for me I find alot of support in my online mom friends who really get it.

So hopefully I have answered alot of your questions. Hope this was helpful.

Very long appt. Update

Yesterday I was VERY frustrated at the lack of follow through with drs. Avery should have been seen by the TPN team weeks ago but noone ever followed through. It took me calling for 2 weeks then an appt. was finally booked only they did not inform me. Great huh. Also we were waiting to see the Ortho. dr. for sometime b/c Avery's scolosis has gotten real bad. We had one booked for today, then the office called to cancel it. Of course I went off and finally got seen. We were at the hospital for over 5 hrs. Very exhausting and frustrating.

Update

TPN/GI

1. Avery has actually lost weight since his hospital discharge. NOT GOOD. This is why they needed to see him much sooner. You have to be monitored very closely which is what they have failed to do with him. GRRRR.

2. He has to get to 1000mls of j tube feeds in gain steadily in order to get off of TPN. So as you can see we are VERY far from this goal. He is only taking in about 200cc and still vomits with that.

3. Goal is to keep line infections at bay and liver functioning normal which is accomplished by getting to 50% of j feeds and 50% TPN. Again we are no where near that. Hopefully we can.

4. A positive note, if his mito is causing his GI shutdown then it can go up and down and he MAY get it back but he MAYNOT.

5. We have to discuss further testing which could be really invasive for him and we have to weigh this out to see what benefits it will yeild in order for me to consent to doing it.

So for now they are going to increase his TPN calories and we try to put a little weight back on him.


Orthopedics

Avery does has significant scolosis and kyphosis (bending forward of the spine). Bracing may help or it may bring on more issues like respiratory or gi issues. It is really a trial and error. I sure hope we can see some positive results. Surgery would be the last option as the dr. said it would be a nightmare with a kid like Avery. So I am just going to think positive on this one.

Overall it was a VERY long and exhausting day. Avery did really well considering his bedtime is at 5pm and we got home at 7pm and no nap today.

Sorry I have not posted in a bit but it is actually b/c things have been quiet around here. My life has been boring the past 2 weeks, we are trying out something new, hehehe..... Avery has still been pretty happy during the day and even slept well the last couple of nights. Isaiah has been great but has missed alot of school b/c his nurse has been sick. He really needs to go to school this week though.

Although I might be jinxing myself b/ after his nap today he woke up pretty respiratory. Something we have not seen in at least 2 months. Despite back to back nebs he still sounds crappy and he has a low grade fever. So we wwill see what the next 24 hrs holds. Hopefully nothing big. Other than that we are fine.

Hey all, it is always good when I have little to report. Avery has been doing VERY well during the day. Notice I said day and not NIGHT.........

Yup, he is still up most of the night and have a nurse doing 2 night which I think will work out. So we will at least get 2 out of 7 nights sleep. Not bad, better then nothing. I gave him another haircut and he looks awfully cute and so much older, :( We had to cut his hair down for his cochlear implant which he has been wearing much more often (says the audiologist, eeek). He is giving us lots of smiles and giggles which is wonderful.

On a great note, we have started to give him some baby food again. The first bite he looks at you like "what the heck did you just put in my mouth", hehehe. Then by the third bite he is trying to figure out what to do with it. We are just trying to take advantage of him while he is feeling good.

Isaiah is doing well. We just changed alot of parts to his wheelchair as he has grown lengthwise tremdously over the last yr. He sits up like such a big boy now.

Janessa is getting to grown to quickly. She is wearing my clothes and as sassy as ever. We are in so much trouble.

Me, well I am embarrased to say that I am addicted to the Crash something game on the Wii. What a great stress reliever. Mind you I have never really played video games much before.

Rick is busy as ever with basketball and work. He is 8-0 and currently still number one in the state.

So overall we are doing very well. Always happy for all the smiles and blessings in our lives.

Thanks for reading :) As you can see I like doing the smiley faces these days :) :)