Friday, September 30, 2011

Quick update

We are home, Zach got discharged tonight around 8pm after his last dose of antibiotics. We are praying all goes well this weekend and we will not be making anymore trips back to hospital for a while. We are supposed to hear from the Dr at Pittsburgh on Monday for him to tell us when they want Zach there. Hoping it is very soon, we are just playing russian roulette with this central line....we are lucky with the last two infections, he hasn't lost a line.
Also tomorrow his new pediatric walker will be delivered. This will hopefully allow him to move around more and build his endurance and muscle strength back up. When PT came today, she tried to have him walk around some and after about 20 steps or so he was breathing really heavy..he just doesn't have the energy or endurance anymore to move around.
Please help us pray that baby Zach makes it to Pittsburgh this week and stays infection and fever free :)

Thursday, September 29, 2011


So plans have changed...again...instead of going to Cincinnati, since insurance wasn't approved for transplant or GI consult we have decided to go to Childrens Hospital of Pittsburgh instead. I have heard it is a great hospital and insurance approves...which means a lot. The dr's and hospital social worker are all working on getting him up there. He finished his antibiotics tomorrow, and will stay over the weekend to be observed then hopefully early next week we will drive up for the consult and admit to get him evaluated. The thing about Pittsburgh I am looking forward to is they have a Mitochondrial center as well, so at least we can get that testing done while we are there. His genetic dr here has done some broad testing but no specific Mito tests yet but since it is a big possibility, I think the sooner the better would be nice on the testing.

He has been doing pretty good today, only a few meltdowns....I am really beginning to wonder if he has this disorder called sensory processing disorder...he seems to really struggle with some sensory issues and gets a overloaded a lot causing big meltdowns. He also doesn't like stuff on his hands, has to have his sleeves pulled down on his shirts, over sensitive to loud noises, is easily distracted and much more but I will ask OT about it when they come by. His dr ordered PT and OT to come by and check out his issues with walking and muscle pains right now. He doesn't want to walk anymore and if he does he is very wobbly so hopefully they will have some insight.

And, yet again..I have said it thouand of times...I love his his Pediatrician and his entire office!! I can't tell you why but just believe me, they are the best and most caring people around. I will never know how to repay them for all they do for our family. They truly love Zach and his brother.

Tuesday, September 27, 2011

Genetics, Insurance and waiting

Two weeks ago, the genetic doctor ordered some broad DNA bloodwork to check for abnormalities. He was supposed to have his follow up appointment next week but hopefully we will not be here so his GI doctor went ahead and asked the genetic dr to come by.

We had not met him yet, he just ordered the bloodwork last time but didn't come by because he was out of the office so I was very happy to finally get to meet him and ask him questions. His nurses came by first and went through all of our family history and zachs medical records, my pregnancy with him and my other sons health. Then Dr C came in and did his physical exam and went over some of the bloodwork that he has had done and said we are still waiting on more bloodwork to come back. But what has come back has only told us that he has the correct number of chromosomes.

He told us that with Zach having so much going on and with it being progressive and our history of maternal issues being passed down, Zach has a high probability of having some sort of Mitochrondrial disorder. He wants him to have a muscle biopsy taken the next time he is under anesthesia for any reason. And he already has his DNA stored so they can do further testing without having to draw more blood. He wants to wait on the micro array to come back and whatever else he ordered first then try to narrow down from there.  Unfortunately this could put a damper on him being approved for an intestinal transplant if he ends up going down that road.

On another note, We found out today that our insurance company does not have Cincinnati in their network for intestinal transplant centers so if we went there (if he ends up having to have one) we would have to pay for the entire thing out of pocket. The only hospitals they approve are Pittsbourgh in PA, Georgetown in Washington D.C and one in Fl. So that was a shock today, to say the least!

His GI doctor said for now lets at least get him to Cincinnati to be seen by the top GI's first and if they decide he does need a transplant then we will transfer to another hospital, one of the approved ones. But we are still just waiting on the green light to just to get seen by the GI doctors. And since it has taken all week, we can't go till next week if we are given the green light. 

The dr from there called me yesterday to see what my expectations were, and let me know what they were going to do. He said they may or may not repeat tests and there is a good chance they won't find any answers since he has already had an extensive workup here and no answers have been found.

Today, Zach has been having more pain and is draining a lot of dried blood/coffee ground looking stuff from his G tube and we have had to start draining his J tube again due to the pain and leaking. He also threw up last night so that is unexplained. He has been very moody and irritable, it is heartbreaking. We tried to take him outside to the rooftop playground earlier and he took 2 wobbly steps and then crawled back up into Nana's arms and wouldn't get down saying he was hurting.

So we are stuck waiting again for answers....I have heard the Mito wait is long, it is not one that we want to be on but I know we are not alone, there are a lot of special kids on this road as well and we pray for them.

Sunday, September 25, 2011

Looking forward

We are looking forward to this coming week, he is definitely being transferred up to Cincinnati Childrens Hospital. We are just waiting on the day, and the transportation to get taken care of. I believe we will be taking the Angel Med Flight, since he is stable but he is still on 3 IV antibiotics, replacement fluids and TPN. He is also still under isolation for his rhinovirus...which is simply a cold but we can't risk him getting anyone else sick or him catching anything else while his body is under so much stress already.

Lately, Zach has been experiencing more autonomic dysfunction symptoms, he has been getting flushed in his face and ears really easily..every time he gets excited. He also has not gone to the bathroom in a week...7 days!! But it doesn't seem to be bothering him so we will jut wait and see what happens there. His liver enzymes are showing elevations again, so they are decreasing his lipids in the concentration...lipids are the makeup of fats so less fat but that is better for his liver. He has also been having more leg pains or muscle pains and cramps over the past week or so. And he won't walk, well maybe 2 or 3 steps and that is it. When he does, you can tell he is having difficulty....its like he doesn't bend his knees and he is really wobbly so tomorrow they will also check a CPK to see any muscle inflammation.

We are not going to be here for his immunology or genetic follow up appts so they are going to have them come by and see us before we transfer. I am really anxious to actually talk to the genetic dr. I haven't met him yet, he just ordered a bunch of labs a couple of weeks ago so hopefully they are back and we have some answers there.

Tomorrow is also Daddy's birthday, he gets to spend it at work and us in the hospital so not much fun there but we love him and wish him the best birthday!!!


Yesterday, daddy and Luke came and picked me up at the hospital to spend the day together. We went to Mt Airy and watched the Mayberry days parade with all the old Andy Griffith memorabilia. That was cool, then we made our way to Pilot Mountain...or as Luke calls it Moleberry Hill. That was really nice, it is so beautiful there and it was nice to get out and get some sunshine and see my babies. I am really going to miss them when we transfer.


Friday, September 23, 2011

Long over due update

So I know it has been a couple of weeks since I updated, and  a lot has happened and is still happening. We got discharged last thursday the 15th only to be readmitted on tuesday the 20th.
They sent him home with intentions to send him to Cincinnati Childrens hospital outpatient and let them direct admit him so that he can see their motility specialists and intestinal failure team. He was stable when we left, no fevers and feeling pretty good. We got to spend a good weekend at home, he wasn't himself but we know he has been through a lot in the past month so to be expected. His legs have gotten really weak and wobbly and he has begun to complain about leg pains.

Sunday morning he woke up and was very shaky so his pediatrician had us get some bloodwork at the local hospital to check his CBC and some blood cultures. We were thinking it could be blood sugar issues but by the time we checked it was fine. Monday, his nurse came and drew labs again and this time his blood sugar was low, 60 so we have ordered a glucometer to help keep track of them. This could be all related to the TPN but who knows.

Then on Tuesday afternoon when he woke up from his nap, he had a temp of 103.5 rectally....really not good so off the the pediatrician again :(  We got there and he couldn't find any easy answers so he called the GI on call at Brenners and they decided he could stay home and wait for test results. So we had to go back to the local hospital and get labs drawn again. This time they ordered a new test called procalcitonin which shows elevated when bacteria sepsis is a high probability

An hour later the pediatrician called and said it was really elevated so he has to be admitted. So off to Brenner's again. We got checked in and to our room around 8pm tuesday night. And sure enough the next day, his cultures came back positive and he also has tested positive for rhinovirus. So poor baby, he can't catch a break.

His nap that led to his fever

Not himself, but glad we had a 4 day home vacation

He is feeling better now, no more postive blood cultures but still on IV antibiotics for 10 days. And we finally made some progress with moving forward to Cincinnati. After a long email to his primary GI doctor about how nothing has been done and nobody seems to follow anyone elses plans, we made progress. Cincinnati has all of his records and are reviewing them and coming up with their plan of action.

He is going to be seeing their motility specialist, Dr Kaul and their intestinal rehab/transplant specialist, Dr Koccoshis. Hopefully they will be able to come up with some plan for Zach. The GI on call this morning informed that he should be transferring up there next week. The financial person here at Brenner's is working on his insurance information and we just have to wait for Cincy to say we are ready for him. I am not sure if we will be going on the helicopter or what, but that should be interesting. 

And we are still waiting on immunology labs and genetic labs to all come back, maybe they will give us some insight to why he is having these issues. 

Thursday, September 08, 2011

A lot to take in

It has been an interesting last couple of days to say the least. All of his doctors finally got together on Wed morning, his surgeon, his primary GI and 2 other GI doctors that have taken care of him a lot. They all discussed him and what was next, what could be causing the issues and where do we go from here. They finally came to the agreement that his previous roux en y surgery was not causing any issues and that he did not have any mechanical obstructions anywhere in his intestines.

Everyone is pretty much in agreement that it is his small intestines that are just not working right. No matter what the motility test showed. He had one abnormal AD manometry study and 1 normal study so it could be intermittent but right now it is acute. They also decided to do some colonic manometry to see if he was having issues with his colon and could be pushing some stuff backwards. The other opinions were doing an illeostomy to bypass some of his intestines to see if that would help and the idea of an intestinal transplant.
They had a cancellation yesterday so he got to go down to have the colonic manometry placed in surgery. That test ran yesterday afternoon and most of today. It was pretty disgusting, actually...poop everywhere so that was fun. Still waiting on the results of that, probably will get them tomorrow. I am pretty sure it will be normal...he seems to not have a problem with having runny diapers.

Today, one of the GI doctors was also telling me about a child she had in fellowship that had similar issues like Zach and he went on to have an intestinal transplant. So that is a big possiblity. She doesn't think the illeostomy would work, if his small intestine is the problem then it won't help to just attach it to his skin. She is also going to talk with his primary GI about a trial run of cisapride. She said the med used to work wonders for motility issues and they took it off the market because 2 children died while taking it (they had undiagnosed heart issues) but still, scary. He would have to have a full cardiac workup first. But since the medicine is just coming back on the market, they are only doing limited trials and might not be doing it here.
If he did have to have an intestinal transplant they reccomend Pittsburg as the number 1 choice. I have never been there so at least a change of scenery.

They pulled half of his immunolgy labs this morning and are going to pull the other half tomorrow morning. His blood count has taken a huge hit with all the blood they have been taking and so he might have to get another transfusion soon. And she said today that his labs are showing that the TPN is already starting to damage his liver...which is not good at all, he has only been on it since April and it is vital to his survival.
So as you can tell, we have a lot to take in...there is no signs of going home right now, which is fine because going home scares me.  He is still having lots of pain and they can treat it better here. Also they are going to send genetics by to do an assesment on him. This is very helpful, if he has some sort of genetic issue it could change the course of action for eveything. Well I think that is everything for now. Please keep praying for him. He is feeling better right now which is good but even through his good days he is very sick on the inside.

He tries so hard to always feel good and always smile and laugh. He is such a wonderful 2 1/2 year old and has stolen every nurse's heart up here. He has gotten so rotten, but we love him.

Sunday, September 04, 2011

Part 3

Zach had surgery on thursday to look for a possible obstruction in his small intestines. The surgeon couldn't find anything so he just placed a longer j tube...about 10 inches long into his intestines to see if that helps. The first day he was very sore and was on morphine every 2 hours as he recovered. Luckily the surgeon used the same incision he had used for his roux en y so still only 1 long scar. He was also very nauseus the first day but they had to take out all of his intestines and then put them back in so his body had to adjust. He lost lots of fluid during surgery and whole day he drained dried blood from his g tube. Which is all normal they tell me. Yesterday he was still draining bile from his g tube so they were holding off trying out the new tube just yet. Last night it also started leaking bile from around the stoma, which is the problem we were having to begin with so obviously the tube didn't solve that. We will have to wait and see what happens when we add feeds back in. We are still waiting on him to use the bathroom first before we can start feeds and it has almost been a week and nothing. This coming from a child that has 4-5 dirty diarrhea diapers a day so to me something isn't working in there.
Tomorrow we should get to see our regular doctors again so maybe they can answer some more questions or bring more insight.