Three Appointments Down, but still stuck in Houston

Bare with me, it's been a crazy day!

We arrived in Houston on Sunday in time to celebrate Samuel's fourth birthday. Alex considers Samuel his brother so it was important for us to be here for it. Alex loved painting pottery and made an awesome frog box. Then it was early to bed to gear up for our appointments.

Yesterday we saw our surgeon, Dr. Tsao. I trust him completely and he has proven to always have Alex's best interests in mind. He makes all his decisions to help Alex have the most hope for the future. This week he proved this once again. The easy answer would have been to just replace Alex's port. However, a person only has a limited number of spots to put a central line. Once you use a spot it is gone. Knowing that Alex will most likely need a port his entire life, Dr. Tsao is doing everything in his power to preserve Alex's current port as long as possible. So Alex will be admitted on Thursday and they will once again use a "clot busting medication" to get Alex's port functioning again.
Dr. Tsao also looked at some tissue that had grown around Alex's Jtube and is pushing the tube up. He decided to use silver nitrate to "burn" the tissue. This is very unpleasant and Alex was a real trooper. On Thursday, he will look at the site again and determine if he needs to take Buggy into the OR to remove the tissue surgically. At this point, it looks likely that that will be necessary as the silver nitrate does not appear to be taking care of the problem.

Today we had appointments with both Dr. Koenig and Dr. Navarro (GI). As always Alex couldn't wait to get to Dr. K's and waited by the elevator for her to get there this morning. Then he ran into her waiting arms. What a blessing to have a doctor that loves Buggy and that he loves so much. The appointment was much more "serious" then I anticipated. Alex has a syrinx in his spine. A syrinx is a cavity of fluid in the spinal cord. We have been routinely watching this through frequent MRI's and other tests. Up until now he has been asymptomatic. Recently he has developed some symptoms that "may" be caused by the syrinx. We are going to run some simple bloodwork (once his port is working) to rule out some easy fixes for these symptoms. If the labs are ok, then we will quickly schedule an MRI and SSEP. We are praying this is not the syrinx. If the syrinx is the cause then we will have no choice but to put Buggy through a pretty serious surgery and place a shunt in his spine. This has some pretty scary possible side effects so we are hoping we can avoid this. We also discussed Alex's improvement since his Keppra (seizure med) increase. The last thing we talked about was his overall trouble with absorption. Other then that Dr. Koenig is pretty happy with things overall.
After leaving Dr. Koenig we headed to Dr. Navarro's. Alex is at his GI baseline. It is not the world's best GI situation, but it is Alex's normal and we are happy to be there as we have seen much worse. I was expecting to have Dr. Navarro come in say keep things status quo and see you in a couple months. Unfortunately, that was not the case. He came in and told me he was concerned with Alex's weight. Over the last year and a half, Alex has had no net weight gain. They have decided that it is the time to address this. He hasn't even gained as well as expected on TPN. His best growth has been when he is on both TPN and feeds together. We are trying to avoid TPN so the decision was made to put MCT (medium chain triglycerides) oil in Buggy's tube a few times a day. This is the only fat source that does cause the pancreas to release enzymes. Of course with Alex's history of pancreatitis we would not want to do anything to aggrevate his pancreas. I left there thinking we had a great plan assuming Buggy could tolerate it. BUT...after talking to Dr. K this afternoon she told me that MCT can sometimes cause metabolic issues in these kids. Alex has shown us in the past that he can become metabolically unstable pretty easily so we will have to keep a close eye on him. We are going to start this inpatient this week so we can monitor his labs and make sure he is doing okay.
We were supposed to have 2 appointments tomorrow (Hematology and cardiology), but both have been moved to next Wed.
I think that about sums up our crazy start to the week. It looks like we will be here through next Thursday unless something changes.
I will continue to update as the week progresses.
Ali

On the Road AGAIN!

Alex and I are packing up and getting ready to head out to Houston. This is my very least favorite kind of trip because I have no idea how long we will be there! Let me explain.... Ignore the smoke coming out of my ears, BUT Alex's cardiology appt was cancelled for what amounts to the 5th time this year. Alex has never seen a carsiologist and the heart is an organ that is often effected in mito. So we need to see the cardiologist. I left a very unhappy message the day I got the call. I got a call back and they can see Alex but not until Sept 8th. What?!!! So variable number one is whether I will extend my trip an entire week to see cardiology. Also adding to the fun is two surgery issues. Alex's port is once again not drawing back blood for the umpteenth time and he is having pretty significant pain around his jtube where there is quite a bit of scar tissue. So depending on how they decide to fix his port and if they need to do anything to his Jtube will determine when we come home. Not easy to pack when you have no length of stay, don't know if you will be inpatient for any of it, etc. Thank goodness the Knights are so good to us and not only let us stay for any undetermined amount of time, but also let me leave whatever supplies I want in an attempt to make life easier. Missy and Ben- I will forever be grateful for being so selfless and for loving Alex and I like family. You make every trip to Houston so much more like a vacation. Because of you, Alex and I look forward to our trips instead of dreading them. Mis- I'll even try to not yell at you if you keep me up all night!!!

In other exciting news....We started school this week. Alex seems to be really enjoying it so far. It is called Hideaways in History. His favorite parts are "Indiana Stickenstones, Sticky and Chirp" (three bugs that take us back into history and tell stories), making his "hideaway" for the week, and his readers which are pretty advanced and will have him reading chapter books by the middle of the year. I am impressed with how quickly he has jumped back into our routine. So far he doesn't seem to have lost any skills over the summer. Reading is by far his best subject. This makes me happy because I have always been a reader. I hope he continues to find joy in books.

I will have my laptop with me so I will be updating. Please pray for a good trip and as always for Bruce to find a good job.

Ali

What a Whirlwind!

Whew! What a crazy 6 weeks we have had. I can barely catch my breath!!! On Saturday, Bruce's mom flew in. On Sunday, Bruce's sister joined us. It has been way too long since we have seen them. Grandma hasn't seen Alex in a few years and Cassie hasn't seen him since he was a baby. Sunday we just enjoyed some quiet time. Monday we went to the zoo in the unbearable heat and showed Aunt Cassie around texas.I loved watching them enjoy getting to know him again. He fell in love instantly. He was glued to Cassie's hip. He can't wait until they can come visit again. Tuesday they got twatch him do therapy and then off to a tearful goodbye at the airport. We hope next time we can have a longer visit!

Julie and Cass- Thanks for coming to visit. We loved having you guys. We hate that we don't get to see you more often. My biggest wish is that we could see ALL our family more often.

For our medical update....Labs when we got home were pretty good. Buggy is having trouble keeping his Vitamin B12 at an acceptable level. We also realized that his keppra (seizure med) level in his blood is too low which explains the increase in seizure activity we have seen. The problem is that he is getting "enough" of both of these meds. He just doesn't absorb anything like he should. This also explains why he can never gain weight. Dr. Koenig and Dr. Navarro (GI) are brainstorming and trying to come up with the best solution.

This morning we saw a local metabolic geneticist that we see once a year in the rare case that we have to be hospitalized locally. We need to have someone who can advocate for Alex's metabolic needs. She thinks that Alex clinically "screams" a certain form of mito (mainly pancreatic, liver and hematology issues). However, we had done the bloodwork for this syndrome a few years back and it was negative. She says there is research that shows that sometimes it shows in other tissues and not the blood. She will be consulting with Dr. K to see about testing Alex's liver (we have a sample left from his biopsy) for this syndrome. Other then that I just filled her in on the last year's worth of history and we were on our way.

We have a few days to slow down and then we head back to Houston the last week of August for some appts.

Here are a few pics of Alex and cayman that I never got to include.

Ali