A Good Day

Overall things went pretty well today! Alex woke up feeling pretty good. He had both an echo and an ekg this morning. I am assuming they were fine, but I haven't officially heard. Then we removed his pressure dressings from his groin. He was happy that he could get off bed rest and move around.

We saw hematology this morning. They let me know that we need to stop his aspirin therapy which was only started last night! They do not want him to have it until he is afebrile for 24 hours. They also let me know that he is anemic once again and may need blood. His coags are also elevated meaning he isn't clotting correctly so they have started Vitamin K once again. They wanted to review some of the tests regarding his DVT and occluded femoral vein, but felt that most likely we would just leave it alone unless Alex starts to become symptomatic.

Dr. Pacheco came by this afternoon. She made the decision to stop Vanc and is trying to find an oral antibiotic that is safe for Alex. She wants to watch him for a few days and make sure he isn't going to get sick now that we have stopped the IV antibiotic. His CBC looked great this morning(from an infection standpoint) so we will repeat it in the morning to make sure things still look good.

We started feeds slowly this afternoon at 10 cc/hr. We should be up to goal rate on Sunday. We are hoping to be discharged Sunday or Monday. I need to schedule a few followups for next month, but it will feel good to be home.

Ali

One Lucky Boy

Today was not anything like I had thought it would be. Going into this week, I really thought that Dr. N was going to come out of the Heart Cath Lab and tell me there was no need to close the hole as it wasn't causing any problems. That is not quite how it went down...

I was extremely nervous and emotional this morning. Much more then I usually am, even on surgery days. I was trying to stay strong for Alex, but I was really worried. We had to leave the hotel at 6:15 for the hospital. We were brought back into pre-op about 7:15 and waited and chatted while multiple people came in to discuss the procedure and have me sign consent. Alex did great. He was a little nervous, but played and held it together. Around 8:45, we were brought down to the Heart Cath lab. Alex had a few tears ( as did I), but he was asleep when I left him at 9:00. They still needed to get him prepped and finish administering anesthesia and getting him intubated. I have to brag on the heart cath lab here as the two nurses were INCREDIBLE about keeping me updated. I got my first call at 9:45 to let me know they were just gaining access to the groin to start. They had told me it would take an hour to an hour and a half to do all the testing and determine if the hole warranted closing. To my surprise the next call came at 10:05 to let us know that they had enough data to close. At 11:00 they told me they were still working and did not have the device placed yet. They finished at 11:45 and Dr. N called us into the conference room. I was shocked with the news. They do a test called the bubble test. They inject bubbles through an IV and if the bubbles cross the ASD and into the brain it is considered positive. Three or more bubbles warrants closing. Alex had about 80 bubbles cross into his brain. They feel we are extremely lucky that we found this and that Alex's previous DVT did not cause a stroke. The other news to surprise us is that the DVT is still present and occluding his femoral vein. When they injected the die it had to use "collateral" veins to get around that spot. We should see hematology tomorrow to discuss this and if any treatment is warranted.

Alex has done okay the rest of the day. Once again he had a seizure while going under despite a loading dose of the medication we use to stop his seizures. The anesthesiologist feels like we need to hook him up to an EEG the next time he has anesthesia to see exactly what is going on and if we can continue to safely use one med. Alex has had a minor allergic reaction to something causing a rash and itching on his back and legs and swelling in his face. Then he had red man syndrome from his antibiotic despite treatment so it is being run four times slower then the norm. He has also been running a temp all afternoon. We are hopeful it is just his "usual" post anesthesia fever. However, we are watching closely as Dr. N was concerned about infection because the end of his central line sits right where they were working.

We are very tired. Alex is already sleeping and I am hoping to join him soon. I will update tomorrow. Thank you to everyone who prayed for us today.
Ali

Interesting Day

Alex and I got on the road early this morning to head to Houston. We were scheduled for a test at 3:00 to look at the function of Alex's autonomic nervous system. The test is called a tilt table test. Alex was on a table with all kinds of leads hooked up. He was then strapped to the table so he couldn't move. For about 10 min. they recorded all sorts of information. Then they tilted the table to 70 degrees so he was almost standing. They continued to watch the same information. After about 30 min, they put the table back down and watch for another 5 minutes. Sounds easy enough, right? Unfortunately for someone with dysautonomia it is extremely uncomfortable to be in this position without the ability to move.  I was extremely proud of Alex (as were the dr. and nurse) as he tried to move and fought back tears, but hung tough for the entire test. His findings were significant and we will begin to treat his dysautonomia. I was interested to hear that a lot of the fidgeting that Alex does and what we consider to be hyperactive behavior is most likely his brain trying to signal his body that something is wrong and get more blood to his brain. His brain perfusion dropped almost 20 points when upright meaning his blood pools in his lower body and he doesn't have the ability to get the blood back to his brain. I am hopeful that the new medication will help!

At the end of the test, the cardiologist let me know that our hematologist wants Alex admitted tomorrow to check labs and see if their is any treatment needed before his Cath on Thursday. Unfortunately our hematologist is out of town and there is no record of this. So as of now I am unsure of if we are being admitted tomorrow or not.

We head back to the hospital tomorrow to meet with anesthesia and have a GI test done. I will update when we get back.
Ali

A Week of Fun

We had such an incredible week! Last Friday (Alex's Birthday) we took it easy and just had a quiet day at home. We knew we had a busy weekend coming up.

On Saturday, we had Alex's party for his friends. We held it at Urban Air Trampoline Park. The kids had such a blast on all the trampolines.
Alex loved having a day to spend with so many of his good friends!

After such much needed rest Saturday afternoon and night, we packed up Sunday to head to the Great Wolf Lodge. We have wanted to take Alex to Great Wolf for several years.  It is a hotel with an indoor water park and a host of other fun activities. We decided to save up and surprise Alex for his birthday. I have to say we had a blast! It was worth every penny. Bruce, Grandpa, Alex and I all loved the hotel and the water park. We were there all day Sunday and were allowed to use the facilities until 9 PM on Monday, but by lunchtime we were all exhausted and headed home. This weekend was definitely one of my favorite memories. I hope we get a chance to do it again!

I had a waterproof disposable camera with me, but most of the pictures were too dark to see. And somehow I only managed to get one picture with Grandpa! Dad was with us until this morning, but we really had a quiet week after Monday. We were all exhausted. Alex loves having my Dad around. they like to give each other a hard time and joke around a lot. We were sad to see him go, but were glad he got to be here for Bug's birthday.

Now we switch gears. Alex and I leave Tuesday morning for Houston. We are going down to complete a couple of tests that we had to reschedule as well as the Heart Cath and potential closure of the hole in his heart. If I am being honest, I am more worried then usual about this test. Our last admission was when Alex pulled out his tube and wound up inpatient for almost a month with pancreatitis and seizures following anesthesia. We have been forewarned that we will not have one of the anesthesiologists who know Alex well and they most likely will use a different protocol. That is very scary to me. Alex doesn't do well with anesthesia on a good day, much less when things are different. There is also the unknown about the outcome of the procedure and how long we will be in Houston. I like it much better when I know what to expect!!! I ask that you pray for us this week. Pray for peace of mind for me, for Buggy to be safe and not too worried, for the Dr's to make the best decisions for Bug. I will update throughout the week.

Ali

Happy Birthday, Bug!

I cannot belive my Buggyboo is 8! We certainly have not had the 8 years we expected, but I wouldn't change it for anything. I am so thankful for every minute with this precious boy who brings a smile to my face everyday. He is blessed with such a gift of joy! What a year we have had. We have made some strides this year in healthcare that give me hope for the future. We started EPI-743 and saw a 50% increase in function in his mitochondria in his brain. We started  IVIG  and have seen a major decrease in illnesses! We are so excited about these changes that have made such a difference! I also promised I would tell you about our surprise.... We decided to throw financial caution to the wind and spend 1 night at Great Wolf Lodge. We will spend a night and enjoy the water park and other attractions! As hard as it was to put out the money, I am so excited for my boy to get an awesome night of pure fun!

My sweet Buggy-

God blessed me 8 years ago when he decided to make me your Mommy. You make me so happy. I am so proud of you. You always find fun in everyday. No matter what you have to go through you never get mad or upset with me. You always smile and hug me and tell me you love me. Everyone you meet falls in love with you. You light up every room. You are so kind, smart and funny. I love you more then anything.


Love, Mommy

Good while it lasted

The last week has been CRAZY! Just when I think we are going to go through a quiet time some little issue rears it's ugly head! Last week after labs came back they ordered D5 with potassium and asked me to repeat labs the next day to recheck. I ran the two large boluses that were ordered. They weren't done until midnight so I unhooked Bug in the dark and headed to bed. When I woke up the next morning, Alex's eyes were really red and swollen. It looked like an allergic reaction. After checking in with Alex's doctors and checking vitals we wound up having to give benadryl and albuterol for low Sats. After the benadryl the swelling went down and Alex looked much better. Labs also looked a little better, but were still not back to normal. Dr. N decided we needed to go back to doing IV fluids every night. Oh well! Wednesday night we started the fluids earlier so I could watch him. Once again his eyes swelled and got red and he had a few hives. We stopped the infusion. Thursday, I left multiple messages, but couldn't get in touch with GI to get the fluids changed.  Theresa (Alex's nurse) and I decided it was not safe to run the fluids again, so Alex went without fluids Thursday night. Friday I called the company that manufactures the D5 to try and get some more info. I did find out that all of their Dextrose is derived from corn. Alex is anaphylactic to corn so the reaction is not as surprising. The only question is why he doesn't react to the dexrose in his TPN. The company has their scientists looking into it, but they think that the D70 is more highly processed then the D5 possibly removing more corn. As you can imagine this causes huge issues. My child has to be able to have Dextrose. In all mito children it would be important, but especially with Bug's severe hypoglycemia. Anyway, I finally got in tough with GI on friday, but our doctor and his dietician, who we work very closely with, were out. The covering doctor and nurse were insisting that it was not possible he was allergic to the dextrose and they didn't want to order different fluids. It was decided we would try one more time using a different filtered tubing. We started the infusion and within 25 min, Bug was swelling agin. I immediately stopped the infusion and they did order different fluids. Not sure where this leaves us in terms of dextrose, but we will talk to Dr. P, our allergist soon.

In the meantime, Alex's Dexcom (continuous glucose monitor) arrived on Friday. I had spoken to the rep and she felt like I would be fine inserting the sensor without training. We started using it Friday evening. It has been interesting to say the least. Alex's blood sugars are all over the place. His sugars are high much more often then we realized. We have also learned that he is dropping into the 40's even when on feeds. It has been enlightening to say the least. Alex has been such a trooper as always. Not only is it another needle weekly to place the sensor, but we have to calibrate it every 12 hours with finger sticks. I did not realize this before and had told him we wouldn't be doing finger sticks anymore. Now we are doing more then before! He just smiles through it all!

In fun news, Grandpa came a few days early. He flew in Sunday morning and Alex has been so excited to have him here. We are excited for a weekend of fun as Alex turns 8 on Friday and we have lots of fun surprises planned! My birthday update will go into all that!

Thanks for all the love and prayers.
Ali