Post from Dad

You are correct if you are saying to yourself "this can't be good". If I am posting that means a negative report. So here it is -

Avery took a sudden change for the worst late this afternoon. After his afternnon Meds he fell into a comatose like state and stayed that way for the next 8+ hours. Now if you know Nicky at all, she wasn't settling for doctor's doing nothing. In order to clear his system he is being given a blood transfusion as I type this (10pm).

Hopefully he responds to this by tomorrow and can start working towards coming home. As always Nicky was right on the money with knowing what to do. As much as Children's hospital has been great for the boys it is very common that Nicky knows more then some of the doctors that work with them.

Please say a big prayer for my little guy and for Nicky who has to go through all this again.

Things are never simple with us

Things started off really easy. We went in and Avery was able to get the probes for the testing placed without anesthesia which was a blessing. A couple of hrs. later he had a high fever which would not have been good if he had gone under. When you have a central line and a fever, there is always a concern that there is an infection in the line. They run blood cultures to be sure. Luckily his were negative for line infection. So we were doing pretty good.

The day of the testing started off okay at 6am. They ran the test without any food in his gut and NO response. This was not good b/c there should still be a response. Then they fun the test with formual and should see a good response, still not a good response for Avery :( Then they give really high doses of meds and we see huge responses. So what does this all mean???

With the right medication we may be able to feed Avery in his gut. Problem is, there is only one med. possiblity and that is a big whatif. So basically back to square one.

Next problem: Avery has not even gained an ounce on TPN (IV food). Major PROBLEM.

Next Problem: because of his metabolic disorder it is REALLY difficult to change the concentration of his TPN without really causes major seizures and other big side effects.

So that brings us to another 4 day stay to fine tune his TPN and pray he has no major reaction to the change. We are supposed to be going home on Tues of next week. Man, I thought we would be home on Friday the latest of this week.

Next problem: Avery has a severe iron deficiency requiring iron IV infusion. This is not good b/c you can have a severe allergic reaction and from what I have been reading can be very dangerous for kids with mitochondrial disease.

Next problem: Avery spike another high temp. tonight so more blood cultures and pray he has no line infection.

So basically things are very technical. There is never an easy answer or solution. We just pray that we can get some weight on him quickly. Not the stay I had anticipated but then again this IS my crazy normal life, right.

Things are really going to be crazy in the next 2 weeks. Tomorrow I will find out what time we need to be at the hospital on Wed. Avery will go into the OR hopefully sometime Weds. morning and testing will begin very early Thurs. morning. Then he has 2 appts next week and I am off to Mexico next Friday. Yikes!!!!!

Time will surely fly by as we have so many things going on. I am just getting worried about the testing for Avery and leaving to kids to go away. I am getting a little OCD about losing my passport and getting stuck in Mexico or getting some horrible parasite. Yup, I know I am being completly crazy but I can't help it. I am so eager for a break and some fun in the sun but remember the last time I went away????? Thinking positive though, right.

Isaiah now has quite the cough and you can tell his throat hurts him b/c he winces after coughing. Tonight I put him to bed and he wakes up screaming and the poor kid face and bed is covered in blood. My heart sinks automatically. It was a bloody nose :( I am always afraid to walk in his room and see what he is up to. In the last week I have found his g-tube gauze stuck halfway down his throat, his legs stuck in the gate of his bed (Lord only knows how he managed that one), and the worst was he somehow reached the string to his blind and it was wrapped 3x around his arm. So he basically has to sleep in a stripped room. Kid is going to give me a heart attack I tell ya. He desparately needs a new safer bed.

Wish us luck this week. Thanks for reading.

I have been feeling VERY overwhelmed these days. As you can see I go through periods that I am fine and life is just going along and I am going with the flow of things and then there are times that I am thinking "How did I get here" and "Will it always be like this". There is just not enough time in the day. I feel like I have at least 3 fulltime jobs. My work, Janessa, the boys and all of their medical stuff. I am also a fulltime nurse case manager which is ridiculous sometimes. Then there is also running the household. Sometimes it is more than I can bare but what can I do, because I have no choice but to get up the next day and do the same thing.

Isaiah is better. I took him to the drs. on Friday in between work and they checked his blood and urine and everything else in between and nothing. We were there for almost 3 hrs. (unbelieveable) and of course it was "VIRAL". Man I hate that word. It basically means, we have no idea what it is and there is no medication for you. I also get so frustrated with our pedi. He has NO clue what to do with these boys. Everytime I ask him a question, he defers it to Children's Hospital. I feel like saying " Is there anything that you DO know?"

Avery is still vomitting almost daily with meds. which sucks. He gets so junky after. He is having intermittent desats at night and is up all night. We really do need to go through with this test this week. I am dreading it b/c it is NOT going to be pleasant. It is going to be awful in fact. I just hope that after making this decision that it yeilds us some important information. Always tough decisions with these guys. They changed the date to Weds. b/c pre-op flipped when they saw Avery's chart and wanted an ICU bed for him post-op. It is not post-op I am worried about but more the testing the next day. We will be in ICP which is the step down to ICU and they know Avery very well there so I feel much more comfortable.

Janessa is totally depressed b/c our friends moved into their new home today and of course took their dog with them. Luckily they only live 2 miles down the road. Janessa can still go for walks with Daisy. She was so great with the dog it was really cute. Maybe someday she will get one but I don't see one in the near future. She did say that she is going to put letters in the neighbors mailboxes asking to walk their dogs for money :).

Avery has been feeling pretty good over the past couple of days. We are hoping we stays this way b/c he is going in for pre-op for 7am tomorrow. Hopefully things stay quiet for the next 5 days so we can go through with the motility testing and get some much needed answers.

Crazy thing is I have to take Avery into pre-op and leave the house for 5:30am and then come back, drop off Avery at home and head into work which is another 45 min. drive and see too many pts. tomorrow. Ugh.

Now, Isaiah is not doing so good. He had a 104 fever tonight and did not look at all. So I have no idea what is going on with him. Poor kid has had it tough lately. I swear if it is not one kid then it is the other. But things could always be worse.

Some pictures of the weekend

None of the kids are thrilled at taking pictures as you can see. Except Avery and Demi.

It has been a busy end of the week. We had so much fun with my family, it is always sad to see them go.

A few things to report:

1, I got a call on Frid. morning from Children's saying that they had Avery on a list for manometry testing (probes are placed along the gi tract to test how he moves food). First of all we had no idea he was on the list so I was thrown for a loop. This requires Avery to go to the OR and have the probes placed through his feeding tube. Then next day they start hooking him up to testing in which they try formula at a high rate and different medicines to see how his system reacts to them. This will be REALLY tough on him b/c he cannot even tolerate meds. without vomitting let alone feeding at 100cc/hr. So this means that he will vomit like crazy. So the downside is that it will be a really tough test on him. Buuuttt, it may yeild some very useful information. Like will he have the ability to get off the TPN (IV nutrition) and is it even worth it to keep torturing with feeding his gut. He is scheduled Feb. 24th for inpatient. So I am not 100% sold on doing it but Rick and I discussed it at length and think it may be worth it. But Rick will have to be with me during the test b/c it will be horrible to see Avery suffer all day by myself.


Isaiah continues to have a real tough time with retching. He is now doing it almost daily and has very few days that he feels good all day. This is a MAJOR issue and we really need to get to the bottom of things. I am not sure what the cause of it is but he needs to be worked up.

Also labs were done on the boys in the summer and would you believe noone ever called back with the results. So why do they bother even doing testing if they never follow-up. I am so beyond frustrated with the system. I just feel that healthcare has gone so far down the tubes. You have to be a constant advocate for you kids and family and it is exhausting. Anyway, I ask the nurse from the pallative team to look up the labs. and one of Isaiah's was through the roof (norms being below 200 and Isaiah was 1700). OMG. I am fuming. This is an acid that your body cannot break down and Avery also had this show up high last yr. So in my research I found a specific type of mitochondrial disease that has this high methlymalonic acid level. So I emailed the neurologist and she now wants to test the boys for this.

Doesn't it sound like I should be getting paid as a dr. b/c that is what I feel like most of the time.

So needless to say, we alot going on as always. I am hoping that I get alot of things straightened out before my trip. Fingers crossed.

Hey all...

I am pretty excited as my trip to Mexico is all booked. Woooohoooo. It is just enough time to get away to rejuvinate. We are going to have a blast.


The pallative team met with us for Isaiah and also discussed some of Avery. I have to say Isaiah was feeling good and sooo sweet. He was just rolling all over them and touching their hair and face. I am sure they fell in love with him, I mean how could you not :). They thought he was much too skinny and they we are going to get him hooked up with nutrition and a number of other dr. Ugh. But it really is time that he got alot more of the attention as Mr. Avery has totally monopolized it. LOL.

Avery is going to be coming off the valium as that was only meant to be temporary and will start on baclofen which is an anti-spasm med. It works in the same way valium does but meant to be more long term. If he does well on it, he may have a pump inserted into his spine to give him constant doses of the med. As we know his stomach does not digest meds well. We also talked about his increase in respiratory issues and they said it was expected with the valium and may still happen on the new med. So there is never an easy solution to things.


More exciting news though!!!!!!! My sister is coming with her kids on Friday and staying with us :) :). So it should be a great weekend. Can't wait to hold my baby neice again.

We had a really rough night. Avery was so congested and having trouble keeping his
o2 levels up despite bipap and oxgyen. I had to do alot of deep nasal suctioning to get him to keep up his sats (which I hate to do). He was up most of the night.

Isaiah was in rough shape today. He now seems to get fevers with his cycles which I believe is his body's way of responding to the stress of it all. He sure is having alot more frequent cycles these days. It just really sucks. Pallative Care is coming on tues. to add him on so we will be discussing this and how we can better help him.

Avery actually had a better day today full with lots of smiles despite some congestion. NO vomiting today. Yay. The first time in days. Probably why he had a better day.

Janessa is histerical and so cute. She has been rallying for a dog for some time and I think she would be great with one, however I just don't see how our family can add that to our plates. Our friends that are staying with us has a dog and it was her birthday today. Well Miss Janessa and her friends planned this whole party for the DOG.....

She made up invitations and put them in all the dog owner boxes in the neighborhood. We actually got an RSVP from one of the owners. LOL... She made hats for all the dog. Hid some bones in the yard and even had everyone bring gifts. Daisy made out better than most kids for her birthday. So 2 other dogs showed up and played and did some of her planned activities. Can you believe it. I actually think she has a future as a party planner instead of a dog owner.

and

I FINALLY finished painting the kitchen and it looks really nice. I picked "coffee" it is going to look really nice with the countertops. Can't wait.



The boys are not doing to well. Isaiah had a great time at the play and laughed tons. But when he got home he started a cycle and has been retching since. Poor guy.

Avery is soooooo junky, just like old days. We are back to using the Bipap, suctioning and the cough assist. I just hate it. I am not sure if it is the valium or not. I sure hope that it is not here to stay b/c that would so suck. Poor kid is so congested and gets so tired and can barely cough. It is just a vicious cycle b/c he vomits daily with meds and get congested from vomitting. So not only do we have stomach issues which will keep up on TPN but also have respiratory issues the painful spasms.


All this can be so overwhelming, which is why I am happy to say that I am going to MEXICO. Woooohoooo. Me and a few other girlfriends are going next month to relax and enjoy a vacation. Can't wait and hope I can keep Avery stable.

Still busy working on the renovating the kitchen. If you know me, once I have an idea in mind I don't stop until it is completed. The stove came in and it is soooo nice. Never thought about a stove before but this one is something else. Countertops are ordered and will be in a couple of weeks. So we are getting there. I will post picture of the finished kitchen. I might have a before picture (I hope)

Back to medical stuff which I am sure you all are so eager to hear about. Just kidding.

Isaiah is doing great. We are going on a school trip to a play tomorrow. Can't wait to hang out with my boy. I will take pictures. I hope he does his usual cracking up laughing out loud. It just brings tears to your eyes. Yesterday my nurse brought her little dog that weights all of 2 lbs if that. Isaiah was just rolling all over cracking up at the dog in a skirt. Too cute.


Avery has been continuing to struggle with respiratory issues. I believe it has to do with the increase in his valium. We needed to increase for the spasms and pain but it decreases his whole system including his respiratory. So another catch 22. Ironically his neurologist called today and we are throwing some other med. options out there. It is really such a fine balance between comfort and other issues that meds can bring. He is still vomiting tons with meds. His gut is just horrible. Thank god for the TPN or we would be screwed.

Janessa is now obsessed with dogs. Poor kid swears we are getting her a bulldog so she can name her LuLu and dress her in clothes. LOL. You can all picture that in this already mad house. She says " It would be perfect for our family mama b/c they are fat and don't like to go out walking and we don't have the time to walk it" ....

Sounds like a perfect fit to the family.

Sorry I have not posted in a couple of days. Been doing some kitchen remodeling. I finally decided to start taking down the ugly wallpaper in my kitchen and what a nightmare. The previous owner just loved green and every painting fab (sponging, stenciling etc) Ewww is all I have to say. We are getting new countertops and needed a new stove. So hopefully in a couple of weeks it will have a new fresh look.

On the medical end of things...... Avery has had a rough few days. I blame this on his drs. as they wanted him off the neurotin b/c they felt like it was not helping and think he is on too many meds. I agree he is on too many meds but we had him comfortable and happy for about 2 weeks then they decided to pull the plug. This I did not agree with from the start but my hands were tied. So we are seeing alot of his old pain responses and not sleeping well and just looking very uncomfortable. Uggh. Poor kid.....

So I called the drs. over the weekend and let them know the situation. They are not putting him back on the neurotin but upped his valium dose and we are probably going to start him on baclofen soon for his muscle spasms. Hopefully the valium increase will buy us some time.

He also had his school evaluation and I must say, Avery performed perfectly. Haha. The nurse was there changing his port needle and taking blood and Avery also did his usual vomitting and spasms. So I think she was convinced that he will qualify for home based services. Yay. We still need to go through all the proper channels but I am happy that my baby gets to stay home where he is comfortable.

I am a little concerned b/c he vomitted coffe grinds today. Which is old blood basically. He has not done this in a very long time. Just odd and concerning at this point. Something we will have to keep an eye on.