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.