Appointments with LOTS of information

We have made it home! We had a great weekend. Alex and I both had fun catching up with our best friends and enjoying some down time. We braved some crazy rain on Saturday to head out to the annual UMDF Energy for Life Walkathon. It was amazing to see hundreds of our friends and fellow fighters standing in the pouring rain ready to do our part to raise money to find a cure. As the walk was about to start the rain stopped and the skies cleared and we were able to walk without getting soaked! What a testimony to the love and strength of so many fighting this disease and their families who were willing to support this cause regardless of the weather.

Yesterday was supposed to be an easy day with two quick appointments. It was a lot of information!

We saw a new cardiologist first. We really liked him. He came in and we talked about Alex's symptoms and history. He confirmed Alex's dysautonomia and scheduled him for a test to look at exactly what happens in his body and the best way to treat him. Your autonomic nervous system controls your heart rate, blood pressure, sweating, digestion, repiration rate, pupil dilation, etc. Alex struggles with many symptoms of dysautonomia and it will be great to have a treatment in place to help in this area. After we spoke for a bit they did an ECG and Echo. During the echo they found an ASD (Atrial Septal Defect) or a small hole in Alex's heart. I was not prepared for this finding as Alex has had numerous echo's in the past without this finding. Unfortunately when the Doctor came back in to tell me we would schedule a closure I didn't ask any questions. Oops! What I do know is that in April, Alex will go to the Heart Catheterization Lab and they will go in through his groin area and feed a catheter up through a vein to his heart and place a closure device. We are unsure how this will effect Alex, but we are hoping it reduces his fatigue, helps his chronic paleness and exercise intolerance. Certainly not the appt I was expecting!

After 4 hours in the Cardiology clinic, we headed to meet with our GI. Our GI had wanted to see us to check on how Alex was doing since the procedure in January. Overall, Alex has done pretty well. However, our two main problems have been bloating and pain. After discussing these symptoms with Dr. N. We decided to let Alex use the ferrell bag for drainage whenever he is very uncomfortable. It is great to be able to give Alex some relief when he is hurting. He looked at his stomach and saw the amount of distention we are dealing with. He decided to put him on 2 weeks of an antibiotic to see if small bowel bacterial overgrowth (very common in kids with poor motility in their GI systems) could be adding to the distention. We are hoping to see some improvement in this area. Since we have been able to stop using the ferrell bag 24 hours a day, Alex is no longer losing a liter of fluids a day. We are going to cut Alex's volume of TPN (IV nutrition) in half and see if Alex can tolerate it. We will closely watch for signs of dehydration and/or weight loss over the next few weeks. Assuming things go well, we will attempt to come off TPN in the next few weeks.

So as you can see it was quite a bit of info for just 2 appts! I am so excited about all the positive changes we have seen in Alex's care over the last year. My hope and belief in all that Alex's life can hold is higher now then it has been in years. I continue to pray for treatments that will improve Alex's life as well as all the other kids living with mito.

Thank you for always praying for us and surrounding us with your love.
Ali

Fun Weekend

We are in Houston for a weekend of fun! Tonight we are celebrating our sweet Lauren's 7th Birthday! Tomorrow we will walk in the Energy for Life Walkathon. Monday we have two appointments and then we plan to head home.

Alex continues to do pretty well. At this point he has stopped licking all foods as it really bothers his stomach and causes quite a bit of pain and nausea. However, he is pushing himself to continue to make it without a ferrell (drainage) bag. He is doing a great job with school and his behavior has been fabulous.

This week we had to get new tires for my van. I hate car touble, but we were told it was not safe to drive by two different places so it had to be done. Oh well, our safety is more important then money!

I will update after our appts!
Ali

A Longer Update

I am going to try and given a more detailed account of our trip to Houston last week.

  We arrived on Monday and had a relaxing afternoon hanging out with with our good friends Nate and Amber from Florida. The boys have not seen each other in a good year and they had so much fun just being boys. I am shocked we didn't get kicked out of the hotel. Alex also managed to teach his younger buddy some not so great habits so hopefully Amber will let the boys play again! Late that afternoon we headed to Joy's house where we met up with all Alex's favorite girls in Houston (Lauren, Riley, Reagan, and Rebecca)! The kids had a blast playing and the mom's had a blast chatting. It is always neat to hang out with a group of other mom's who are used to tubes, oxygen, dr. appts and all the other craziness that is our life!

Tuesday we saw Dr. Navarro first. A few minutes into the appt. he decided he wanted to go ahead and schedule the Pyloric dilation that had been cancelled twice already. We talked a little about when we might be able to try and wean off TPN, but Dr. N really wanted to wait and see what happened after the procedure. We also talked about Alex's continued deficiency of Trace elements and how we could bring those levels up.

We had some time to cut up in the waiting room with Nate and Amber again while we waited for Dr. Koenig's appt. If ever anyone could get kicked out of a Doctor's office for being too crazy it would have been our two boys, who were laughing, yelling, spinning, falling and amusing all the waiting patients. They certainly didn't appear to be two boys waiting for an appt with a life threatening disease, that's for sure! Nate, the consumate Gator, also taught Alex how to Tebow!

We really didn't have much to discuss with Dr. Koenig. We talked a little about the upcoming procedure and spent a short time discussing our genetic results. While we have genetic confirmation that Alex has epilepsy it does not tell us the course his seizures may go. We may never see them get worse or they could progress over time. We just have to wait and see. We also talked about a new medication that Alex will be starting to help his liver. It was recommended by Stanford. We hope to get it going in the next week or so. After this appointment we had lunch with Joy, Riley, Amber and Nate before making a quick trip upstairs to see all our friends on the 9th floor of the hospital! Then it was back to the hotel for an early night!

Wednesday was an early morning as we hurried to the hospital to our 8:15 cardiology appt. I was not happy when we got there and they told me we weren't on the schedule and they couldn't fit us in. Those of you who know me know I am super organized bordering on anal and would not confuse an appt time! Added to the fact that this is the same doctor who cancelled 5 times in a row last year and I was not a happy camper. I have decided to switch Alex's cardiology care to a more reliable Doctor! With no appt to attend we headed towards the hospital to meet up with anesthesia clinic. On the way we ran into our beloved surgeon who offered to be in the OR during Alex's dilation the following morning to help if necessary. We are so blessed with a medical team who work well together and love Alex. After our anesthesia appt we once again met up with Nate and Amber to get in some fun time before our last appt of the day. After lunch we had a follow-up with our ENT. He was really happy with the way Alex's ears look and his hearing was perfect. I love going to an appt and getting all good news! ENT still once to see us every 6 months, but I don't mind an easy appt now and then.

Wednesday was our much anticipated sleepover at the Knight's . Alex couldn't wait to get there and play with Lauren. The kids had fun. Us mom's were up way to late as usual. Nothing really changes at our home away from home.

Thursday was procedure day. I was so proud of Alex. He is normally absolutely panicked about anesthesia. We have known about this procedure and Alex and Dr. Kristy have spent some time talking about it. I also talked to him quite a bit. He handled himself better then i ever expected. While he was still nervous, he didn't panic and didn't cry. It helped that we had our very favorite anesthsiologist. I was nervous because I wasn't sure what was going to come of it. There were a few scenarios as he headed into the OR. Luckily, for us things went as well as we could have hoped for and they were able to dilate the pylorus without having to do "true surgery". Alex was back to me less then 2 hours after I left him. My sweet boy was zonked and took quite a while to wake up!

Friday morning we clamped his gtube and stopped draining his stomach for the first time in almost 4 years. We all held our breath and then were cautiously optomistic as several hours passed without incident. We started feeds very slowly around noon and gradually increased them. Alex ran several temperatures and we talked as a team and decided to just watch him. Saturday he continued to do okay, but his belly became visibly distended throughout the day. Sunday we were discharged and headed home.

So where does this leave us...to be honest I am not sure. Alex continues to not drain his stomach and is not nauseaus however his stomach is extremely distended. For now we continue to wait and watch. If things continue to go well we will talk about weaning his TPN in the next few weeks. In the meantime, I am praying my boy continues to tolerate not draining and is not scared to say if he has had enough.

I think that about sums it up! We have a busy few months coming up again. We will be in Houston the first week of February, March, and April! February 4th is our annual Energy for Life Walkathon. Many of you have supported us in the past. We are so thankful for that support. If you are interested in supporting us again or walking with us please go to the following link:

 http://www.energyforlifewalk.org/site/c.9hJMK4MEJeL2H/b.7655405/k.BDD6/Home.htm We have seen firsthand this year the amazing things that research can do! Thank you for all the love you show to our family.

Ali