We are settling into a good routine. Alex and I are doing school for about two hours each morning. He has therapy twice a week and I am back going to the gym. So it is nice to be on a schedule and feel somewhat normal.

I th​ought Monday was going to be a hard day for me. It would have been Buggy's first day of school. One of those milestones that every mother remembers. So I was shocked to wake up Monday morning and be FINE! I met "my girls" (playgroup) for breakfast after they had dropped the kids off and we had a great time. Buggy helped by telling me he was glad he was homeschooli​ng because otherwise "he would miss me so much he would cry all day". My sweet boy always knows the right things to say!

As for medically, we are trudging along. Things are pretty stable. We will keep doing what we are doing and see if Alex's body can adjust to less fluids and space out his need for them. he has been really pale all week but is acting okay. My only other concern is that he is starting to bruise again. We see hematology towards the end of September so we'll see what they say.

Thanks for being a constant blessing in our lives.
Ali

It has been a nice few days!

Yester​day we went to the mall to get Alex a few things and to let me spend some of my Birthday money. It was good family time. Last night a friend called and asked me to meet her for dinner. Bruce agreed to keep Alex so I had a girls night out which is always a special treat.

This morning was special to me. For the first time in a long time we headed back to church! Church has always been special to me and something that has been a part of my being since I was a little girl. Alex was in Sunday School the whole year he was three. Then last year because of being in the hospital all year we weren't able to attend. Today was the kickoff for this year. (We rarely go in the summer because the teachers jump around to much for my comfort level) Alex had a ball! he is calleing it "playschool" because its so much fun. I am so blessed to have a group of ladies that have really taken to looking after Alex. When i went to pick him up, his teacher let me know that Alex had spent a little time with another class while his class went outside. The Sunday school director knows Alex well enough to know that he would struggle in the heat without his cooling vest and chose to keep him inside. It is that kind of caring that gives me the peace of mind to leave him. So thank you Keller United Methodist Church!

On the medical front... While in theory our plan sounded great. In practice it is not working as we had hoped. I got a call on Friday giving me specific instruction​s. Basically if Alex's pee for a 24 hour period is less then 10 oz or 300 cc's, we are to replace. While the hope was that this would allow Alex to be deaccessed for several days at a time. We are basically having to replace every other day. So we have not been able to deaccess at all. One, I am not willing to stick him with a needle every other day and two it would increase not decrease his infection risk if I were to access him that many times a week. So I will touch base with everyone tomorrow and see if anyone has any other thoughts!

Al​i

I had a really heartwarmin​g moment this morning!!!

A​s a Mom of a child with major medical needs, I am so much more then Mom. I have to be Doctor, Nurse and Teacher along with a hundred other things. Because of this sometimes I have to be the one who inflicts pain on Alex instead of comforting him. For example I have to give Alex a shot every week and access his port (stick him with a needle in his chest) at least once a week. There is always the fear that Alex was start to resent me of be mad.

So anyway, Alex is very warm and loving. He is always giving me lots of hugs and kisses. Well this morning I walked into his room and he clung on my neck and said, "Mommy, I love you so much you can't even imagin​e it".

He is so sweet!
Ali

I feel much better about things after speaking to the GI nurse numerous times over the past few days!
Everyo​ne is in agreement that Alex can not go very long without replacement fluids. They do not want him to be dehydrated or feel bad. However, they would like him to be deaccessed some. After watching him, his urine output and his labs closely this week, we feel like he may be able to have fluids replaced once every three days and hold his own. For the next little while we will still watch his numbers closely and speak to GI daily to sort through our new plan. If this plays out as it has we would access Alex's port twice a week just for one night and he will be deaccessed the rest of the time.
This sounds like a good plan so now we will just watch and wait to see if it will work!

School has been wonderful so far! Alex and I are really enjoying all the books and work we have been doing. He even asks to do more at times. I love watching him learn and be excited about learning.

Th​anks for allowing me to have this place to vent and share our lives.
Ali

Feel free to ignore this post because it is nothing but venting my frustration!!!​

Ever since coming off of his TPN (IV nutrition) we have been giving Alex replacement IV fluids. He has felt great. As anyone who has seen us over the last few months can attest, Alex has more energy then he has ever had and just feels good. As I have posted several times over the last week these fluids came into question. So we have stopped them for the past few days per doctors orders. Alex is grouchy, not feeling good, not peeing, generally j​ust not himself! His ammonia level is high for the first time in 4 months. I am just annoyed! Why do we need to rock the boat when he is doing well. I understand that there is a risk of infection from leaving his port accessed, BUT we have been very careful with his care and have only had one infection in a year and a half. I just feel like his quality of life should count for something!

T​he picture at the top of this page is of Samuel and Landon, 2 of Alex's "mito friends". Dr. K calls these "her boys" because the three of them are hospitalize​d more then her other patients. This is the first picture we got of all three together.

Th​anks for letting me vent this morning!
Ali

I wanted to come on and update, but I don't have much news! Of course, it is always a good thing when I have no news. So let's see...
Thurs​day was my birthday and we had a nice day. We spent some time as a family and the boys took me out to dinner.

Friday was therapy day. After some thought and discussion. Liz and I both agree that Alex really needs two days a week of therapy. He has so many different areas that need attention and Liz just can't possibly accomplish everything in one hour a week. So starting September 1st we will go to 2x/week.

The weekend was also quiet. This morning we went to the duck pond to feed the ducks. The ducks weren't interested in our bread, but the turtles and the fish were fighting over it!!!

Thursd​ay, when Alex woke up he begged me to start school. I was happy to oblige. Alex is LOVING our new curriculum. he is so happy to be immersed in the world of animals and have that be his "work". He even surprised himself. One of the books we are reading is a chapter book with very limited pictures. He was complaining when I first pulled it out that he didn't like books without pictures. Well we started reading and lo and behold he loves it. We are actually 20 pages ahead of where we should be in that book because he never wants me to stop reading it! So we are off to a great start!

The only negative of the last few days is medical and self induced! As I mentioned in our last post there is some disagreemen​t between the doctors about Alex's replacement fluids. Everyone agreed to let Alex's body make the decision. It was decided we would hold fluids for 2 days and then run labs and urine and see what it shows. So Alex got no IV fluids last night and will not get any again tonight. We will run labs tomorrow morning. We know Alex is pretty dehydrated because he has hardly peed at all today. He is a little cranky which is to be expected. Hopefully, we will get answers tomorrow and put this issue to rest.

I will update tomorrow once labs come back. Hope everyone had a wonderful weekend!
Ali

We are safely home!

Yester​day was a good day with lots of information. First we saw Dr. Koenig. I cannot tell you how heartwarmin​g it was to watch Alex with Dr. Koenig and LaKeesha. He could not stop hugging and kissing them. He was just SO excited to see them and the feeling was mutual. They let him have free rein of the office and kept him busy while I checked in and talked to the medical students. I am glad with all that Alex has to endure in Houston, he truly loves these special ladies. They are such a big and important part of our lives. After spending so much of the last year in the hospital they are so much more like family.

So on to the appointment... We discussed the upcoming flu season. As most of you know, Alex has had the flu every year since he was born. Each year he has been progressive​ly sicker. We decided that from September to March we are going to have be EXTREMELY careful with Alex. We will be limiting his exposure to crowds and to children. We will continue to enjoy playgroup, but will not do birthday parties, sunday school, etc. He also will not be allowed to go to stores. Dr. koenig will also discuss with Dr. Pacheco whether we need to have Tamiflu on hand. We also discussed how we will handle Alex's high fevers should he get sick.

The next discussion caught me by surprise. As I posted last month Alex had a seizure in the OR during his liver biopsy. This is Alex's fourth or fifth seizure. All have been pretty major (tonic clonic for all you medical people). Dr. koenig is concerned about this. She does not want to add another medication to Alex's long list if we can help it, but we also cannot allow him to continue to have seizures. So we decided we would watch for now, but if/when he has another seizure we will have to put him on seizure medication. She also let me know that Alex's EEG was not normal last year which I did not know.

The biggest discussion was in regards to Alex's replacement fluids. Currently Alex is hooked up to two tubes. His Jtube goes directly into his intestines and is the tube we feed and give medications through. His Gtube is in his stomach and is hooked up to a bag that drains bile. Normally, you would not have bile in your stomach because it is made in your intestines. But Alex has reverse motility where it moves backwards into his stomach. We also have stomach contents. In a normal person these would leave the stomach and be reabsorbed in the intestines. In Alex's case his stomach doesn't empty at all so we have to drain this fluid out into a bag. He is currently losing almost a liter a day in fluids and electrolyte​s. We have to replace these fluids through Alex's port (permanent IV). So all that to explain what this discussion was about... Dr. koenig would love nothing more then to be able to "deaccess" Alex's port. (Take the needle out). A central line infection always has the potential to be deadly especially in a kid like Alex with an immune disorder. However, we also don't want Alex to be dehydrated. Dr. Koenig asked us to keep track of Alex's fluid balance (his in's and out's) over the next little while and try and decrease his replacement fluids.

Afte​r Dr. Koenig's appointment we headed to see our GI. I knew they would not be in agreement about decreasing replacement fluids and they were not! They are adamant that Alex needs those fluids. So after a lengthy discussion we came to an agreement that everyone is happy with. We are going to hold Alex's replacement fluids for one night and do labs and urine smaples the next morning. If they show Alex to be dehydrated we will continue as we have been. If they do not show Alex to be dehydrated, we will try and decrease the fluids. i am very comfortable with this plan.

We also got some good news. While the mitochondri​a in Alex's liver do not look good and do not function correctly, there is no active inflammatio​n at this time. So while we know his liver will detiorate over time, at this point it is still doing well. Dr. Koenig had already told us she thought liver failure was years away, but it was very nice to have that confirmed. We will repeat liver biopsies every 1-2 years or more frequently as indicated by the labs.

So all in all the appointment​s went very well. We also got to see some of our friends which always makes the trips to houston better. To those of you we didn't get to see this time hopefully, next time.

A cute Alex story to finish... Alex has picked up a few French phrases. And he loves to "speak" French. We went to a Mexican restaurant for lunch on Monday and he insisted on wearing his tophat in. As we were making our way to the table he was tipping his hat to all the employees and patrons saying "Bonjour". i don't think anyone knew what to do with him! That's my Alex. You never know what he will do or say!!!

Phew!!! that was a lot if you got this far, I'm impressed!

T​hanks for following our journey,
Ali

Alex and I are in Houston for our quickest visit ever! We came in yesterday afternoon. This morning we met with Alex's surgeon. Alex's Jtube (the one we feed through) is not very user friendly! It leaks all the time and the extension (part we put into the button to feed) pops out if we don't tape it. So his stomach is taped 24/7. There are very few tubes that can be used in the intestines. So we were meeting with the surgeon to decide if we could change it. We can, but it has to be done under anesthesia. So we will change it in November when he is already going under for a number of other procedures.​
After our appointment we spent a few hours with Alex's girls (aka the nurses and child life). He was thrilled to see everyone and I think the feeling was mutual!
Tomorrow we see Dr. Koenig and then GI. We will get on the road as soon as our appointment​s are over and head home.
I will update on thoser appointment​s from home.
Ali

Home sweet Home! Well for a few days at least...

We got home Wednesday evening. We enjoyed the rest of our visit and were sad to leave as always!

Yest​erday we did our weekly labs and they look really good. That is always a relief after being on vacation! We are enjoying quiet days today and tomorrow.

Su​nday Buggy and I head to Houston for three appts. Assuming all goes well we should be back Tuesday night. We will see Dr. Koenig, GI and surgery. The surgeon is going to try and change Alex's Jtube to a more user friendly tube. If he can't do it in the office it will hopefully be done in November when we already have a procedure scheduled with anesthesia.​

Please continue to pray for Bruce's job situation. I am trying to find peace in the knowledge that God has the perfect plan for us and will show it to us when we need to. But it is hard not to let the fears and frustration​s take over. If any of you hear of companies hiring or sales jobs please let us know.

Thank you for your continued love and support.
Ali