Update

Avery is one amazing little boy. He surprises you all the time. He is VERY sick on Friday night, I actually probably should have taken him in a bit soon. He had a great day during the day and by the time I relieved the nurse by 4pm he was going downhill quickly. His was so junky and I just could not get it up to suction it, it sounded very thick. Then with the high fever, it just threw him over the edge. We went to the local hospital and they did a pretty good job with him but then the will always have to transfer him b/c they don't have a pedi ICU and I don't trust them that much anyway. We got to Children's at 2am and stood in the ER all night and by the am he seemed better and thought we were taking him home but he quickly told us NO Way.

He was transfer to the ICP and was in rough shape. The attending dr. was thinking he might be intubated but obviously he doesn't know Avery. Like I said, he surprises us all the time with how much fight he has in him. They were deep suctioning him about every half hr and man was it the thickest, nastiest stuff the nurse had seen in a long time. It is amazing he wasn't struggling more than he was. He spiked another high temp yesterday and it did break in the afternoon. He was working really hard to breath even on o2. Typcially he has never been on o2 so this is a new concern for me.

He had a great night and looked so much better today. The diagnosis is bacterial pneuomonia with pseudomas again. He also has c-diff again b/c of the abx. So this is the 2nd time he has had pneumonia in 6 weeks. So as you can imagine and I am pretty scared for this winter for him. It is going to be really rough. But I did at least get my summer off.

We also discussed trying CPAP or Bipap at night at home for him to help expand his lungs b/c his muscles are so weak. He hated the mask when he tried it before but maybe being at home in a non-threatening environment might be different. Who knows, I feel like we are just grasping for straws at this point.

Well the plan is to come home tomorrow so we will see.

Summer is over !!!!!! (post by Dad)

Now you all know how Nicky was so excited to not have made a hospital trip for the entire summer. Well, I am sorry to be bearer of bad news but Avery was taken by by ambulance late last night. Of course he was then transferred from Lawrence GH to Children's Hospital. We were so sure that Avery would be coming home earlier this morning that I drove to Boston with the intention of bringing Nicky and Avery home. Just as we were about to leave the hospital Avery quickly took a turn for the worse and we had no choice but to admit him in ICP. Last report was that Avery was resting a bit more comfortable but was still extremely jumky. Both he and Nicky are very tired and still had gotten muc sleep in the last 24 hrs. Hopefully this is not the start of a very difficult fall and winter.

You all know how much of an amazing woman Nicky is, I just wanted to let everyone know that words cannot express how amazing she truly is. Our family is fortunate to be where we are and it is all because of everything that Nicky does.

Thank you all for your continued support.

I will post another update tomorrow.

Avery had a great week but of course that did not last too long. We have been trying to hold back from the suctioning and cough assist to give him an emotional break as he has been having a hard time emotionally. Well the downside is he holds tons of mucous and junk and today it hit. He was sooo junky and I was on my own at the worse. It is very scary b/c he sounds so bad and has extreme difficulty clearing despite suctioning, nebs, chest pt. (the whole nine yards) He is sleeping now on the max o2 concentrator and still satting in the low 90s. So backs are already packed in the car but I am really trying to wait him out. I will continue to give him nebs every 4 hrs. and hope that helps.

Rick and I are supposed to be going away for the night tomorrow. Well down the street, LOL. Let's see if we can make it happen....

It has been a really nice week with our visitors. We have done lots with the kids and they have had their typical kid bumps in the road but overall they did great. It was great for Debbie to meet the boys. She sure brought some good carma b/c Avery had a great week, the best in a long time. They could not get over how active Isaiah was and Logan really enjoyed playing with him. He is such a sweetie. I can't wait to meet her girls.

A little excitement though..... Rick and my car was broken into the first night they were here. Well, not broken into as we keep our doors unlocked. I know, I know. At 2 am we hear a car alarm go off and Rick gets up and looks outside and he sees his inside car light on. Very suspicious.... He went out side and his satelite radio and a few other items were taken, and same with mine. So crazy his gets in the car and thinks he is going to find the theif and do what??????? So then I am nervous and think he has gotten himself killed and call him. Luckily he is fine and came home. Of course we could not sleep all night. Poor Debbie was probably mortified by our crime filled state. Great first visit. LOL.

Now the crazy thing is Rick filled a police report in the morning. We were both ticked b/c we now have to replace alot of our chargers and stuff. Well..........
at 7:30 this morning a cop shows up with all of our stuff. Unbelievable huh and lucky us. We got a lesson and our stuff back. He said it was 2 teens who confessed everything. Apparently they hit a bunch of other neighborhoods too.

You know us, always some excitement.

This week marks the first time in 2 yrs. that I have taken time out of work for recreational and not for drs, or hospitals for the boys. Really sad, huh. I am very excited b/c I am picking up my friend Debbie and her son Logan. Janessa is so excited to hang out with someone with the same life as hers, especially b/c he is from Canada. We have quite a few things planned. It will be really nice to really hang out with someone for a bit that I don't have to explain things too b/c they get it. We can exchanged medical tips especially b/c our youngest are so similar. Sometimes it can be really hard to be with normal people with normal kids all the time to I have found that I seek out my friends that are in a similar situation especially with how sick Avery is these days. Not that I don't love my normal friends. But you all get what I am saying.

Kids start school next week. YAY. Isaiah will be pretty excited to get back to school.

We have hired a new nurse for Sundays, it is just getting to be too much. Avery is soooo much work and I get nothing done at home. It will be nice to have someone there so I can actually cook dinner and clean my house. On Sundays, I am typically stuck on the floor with the boys and with the winter coming up with how respiratory Avery has been, I am sure it will come in handy. We will see how it goes. Mr. Avery has the tendency to scare people off. LOL.

We have been quite busy with house projects these days. It is amazing of how motivating a guest coming can be. We painted Isaiah's house today it looks really good. I might escape myself there during the winter b/c it looks so cozy. Hehehe.

Avery has had a tough few days respiratory wise as usual. I thought the steroid was helping but I actually do not think it is, but it still may be early to tell. He is having much more anxiety episodes from all the suctioning and cough assist, poor thing just jumps if you approach him too quickly. So it is a really fine balance with clearing him which will never happen and his emotional well being. It is just really tough and sad at the same time.

Next week is my first 3 days in 2 yrs. taking time out of work for fun rather than for the drs. or hospital. Now that is Sad.. I am looking forward to some time off and visiting with my friend. Better enjoy it while I can.

I am awfully proud of Rick b/c Mr. Fix It. NOT.... Put up a ceiling fan w/out swearing or electricuting himself or blowing up the house. LOL.

The last 2 days have been crazy as I thought. First of all, sometimes impulsive decisions are the best. I decided the day before to see Boys to Men b/c I heard about it on the radio. I wore jeans and sneakers thinking it was an outside concert. Crazy me..... Turns out it was on the Gillette Stadium campus (home of the Patriots), they opened a new lounge called Showcase LIVE. It was soooooooo awesome. It was this suave, sexy upscale lounge. Boys to Men was soo good, I was so close to them I could see their pimples (LOL). And to boot there were 5 patriots players, including Teddy Bruschi just 10 ft. behind me. Yup, I felt famous for the night in my jeans and sneakers. Apparently Chaka Khan was there the previous night, so needless to say this will be my new hangout.

Earlier in the day I took my mom to see the Cardiologist (who is a sweet friend). Thank you so very much. She felt so much a ease and I was able to get the full picture of what was going on. Her blood pressure is ridiculous and the meds. she is on is not cutting it and so is her cholestorol. She does have some blockage in her right leg but if the BP and cholest. can be controlled, then I think she will be okay. Just alot of commitment to change on her part though. I am so glad I got her in there.

Today we saw metabolism/genetics/nutrition for the boys. Isaiah is way underweight, which we already knew but he is a crazy boy and doesn't stop for a minute so it will be really difficult for him to pack on the pounds. Avery is doing wonderful with his weight and height, he is actually in the 25th percentile for both which is a first ever.

Metabolism was a bit complicated and not sure if I even understand it myself. I like the dr. and he has followed Avery. Of course he agreed that Avery has progressed rather rapidly in his disease and alot of the symptoms were are seeing are Leigh's (which I already knew but sure hate hearing it). Isaiah, there were concerned about his procious puberty and hyptothermia and are testing his adernal glands and thyroid. We also need to seen an endocrine dr. for him.

He is also stumped about the type of mito they have and said that Isereal is doing certain testing on a few new forms that may fit the boys. He will see if they are willing to look at the boys bloodwork. Famous again. Also in only a few cases in the world are they testing all 20,0000 chromosomes for mito and he also is going to try eventually to get that on the boys. So they are eagerly trying to find the specific type and a mututation which is really encouraging. Who knows what will turn up. All this is for my Ness so she does not have to go through what we do. I would never want that for her.

Only 5 more days for my Canadian friend to come with her son. Can't wait...........