Alex and Maddy

Alex and Maddy

Wednesday, May 30, 2012

A Bump in the Road

Really...already! Our fun was just getting started! Alex has been feeling really good and we have been taking advantage of our time without his port accessed. Alex has been asking for baths several times a day. We have been to the pool multiple times. Just trying to make the most of it. Yesterday we did our weekly labs. It was apparent right away that the doctors were not going to be thrilled. His potassium and chloride were both significantly low along with other signs of dehydration. Anyone who has followed our blog for a while knows this is always our rock and hard place. Alex simply cannot tolerate feeds at a rate high enough to get in enough fluids. So we wound up giving 2 large fluid boluses through his port last night. We are rechecking labs today and then a decision will be made regarding where we go from here. I am disappointed in that we have only had 10 days without using his line. My hope was for a full summer. But, I know that what matters is keeping Alex healthy. We will make the summer fun whether we need to use his port or not!

On the bright side, his continuous glucose monitor was approved. We should be able to get training next week and start using that. I am so grateful that insurance approved it. It will take a big worry off me, especially overnight to know that something will alert us if his glucose becomes dangerously low!


Sunday, May 20, 2012

A Monumental Milestone

This has been a hard week for so many of our friends. So let me start by asking that you continue to pray for the Voelkel family who sent their precious girl Lily to heaven this week after a long battle with CHARGE syndrome. Tyson and Christi were there for me so many times when I needed something or just a friend to chat with. We spent many days and nights together in the halls of CMHH chatting and helping wach other through the craziness of hospital life. My heart breaks for them as they struggle with the loss of their sweet littel, Lily!
Please also keep the McNair family in your prayers. Heather has been a dear friend of mine for several years now. She is one of the first people I met when Alex was diagnosed with Mito. She had been down this path for numberous years already and has always been a trusted friend who I turn to for advice or just to lend a hand, She is someone who makes sure I take some time for me despite all of Alex's needs. Tuesday morning, Heather's husband, Michael (an avid biker) was hit by a car less then 2 miles from their house. He remains in the ICU in critical condition. he is on a ventilator and is trying to recover from a broken neck, broken back, broken hip, broken pelvis, brain bleed, lacerated spleen and more.  As hard as this journey with Mito has been, I am so thankful for the incredible friends I have made along the way. I have truly been blessed in so many ways.

Onto my sweet Buggy! He has been doing great. I love when he is feeling well. When he feels well, he is happy and well behaved, which makes me happy and well behaved ;) and the house is so much fun. He is cruising through the last few weeks of school. We should be done the first week of June. But our big news is we have him back on full feeds and all Jtube meds!!!! Such an accomplishment based on where we were a few weeks ago. The decision was made to once again stop TPN for the first time in 9 months. TPN is hard on his little body, but also hard logistically and emotionally. It is a lot more "work" and supplies for me to make TPN and IV meds and adminster them. It also means we are using his port around the clock. This means that he is only deaccessed (needle removed) once a week. That means he can only get wet once a week. Before last summer this is how it has been for the last 4 1/2 years. Last summer, Alex got a taste of what it was like to be able to take baths everyday, go to the pool, go to the beach, the waterpark, etc. He has never been able to do any of those things in his memory. The doctors and I decided if we were going to trial Alex off TPN this year, we would do it during the summer so it would improve his quality of life vs. in the winter when it wouldn't have the same benefits emotionally for him. We have sure made the most of it this weekend! Yesterday, we headed to six flags where Alex got to ride the flume and came off Roaring Rapids drenched and grinning. We all came home exhausted, but happy. This morning we headed to the pool in our neighborhood where Alex picked up where he left off last year and loved that he could still "swim". This makes my day:

 We are not sure if this time off TPN will be for a few weeks, a few months or a few years, but we will make the most of each moment we have!

Friday, May 11, 2012

Last Day of Appts and Home

This was definitely one of the easiest trips we have ever had to Houston! Everything went as planned. There were no surprises, no complications. Definitely a nice treat!

Wednesday we had three appointments. We saw Opthamology first. It was pretty simple and routine. Alex's eyes showed a slight decline in vision. She did not feel like we need to correct it, but expects Bug will need glasses in the next year or two.

After Opthamology we grabbed lunch with the Knights and then headed to Endocrinology. I knew this would be an important appointment because of the severe hypoglycemia Alex struggles with. I'll start with the good. Since being on TPN last September, Bug's growth is awesome. For the first time in over 5 years, he is on the growth chart for both height and weight. He is still scraping the bottom of the charts, but just being on them is a huge accomplishment for us! Then our talk turned to the hypoglycemia. Blood sugars below 40 are extrememly dangerous, even potentially fatal. In the last month, Alex's blood sugars have been below forty at least 12 times that we know of. We can not allow this to continue to happen. More importantly it would be horrible if his blood sugars dropped overnight when he could not let us know. By the time I woke up in the morning it could be too late. The fact of the matter is we count on man made machinery to keep him alive. Machines that are supposed to alarm if there is a problem, but don't always. Machine's that are supposed to stay connected, but come unhooked. You get the idea. As I mentioned several weeks ago, we are working on a service dog,  but that is at least a year away. So we needed another safety net. Our plan is to get Alex on a Continuous Glucose Monitor. It is a small needle that will be inserted into his stomach once a week. A transmitter attaches to the top (about the size of a paper clip). He will then keep a "beeper" or receiver within 5 feet of him. The monitor will check his blood sugar every 5 min and if his blood sugars drop below 55 it will alarm and let us know he is too low. Hopefully the CGM will give us some piece of mind overnight.

Our last appt was hematology. Our normal hematologist was out so we saw her partner. We have seen her frequently in the hospital so she is very familiar with us. Our main conversation was about Alex's upcoming heart cath. There is a risk of bleeding as well as a need to anti-coagulate if they close the ASD. This becomes tricky because of Alex's history of clotting issues. We came up with a plan so hopefully all will go well.

Yesterday, we said our goodbyes to the Knight's who are family to us. We made plans for our next trip and got on the road. It always feels good to be home, but we miss all our friends when we leave.


Tuesday, May 8, 2012

Good Week

This has been one of the easiest weeks I can remember in Houston! We drove down last Thursday. We headed to the hospital at 5:30 Friday morning for Alex's MRI of his spine and SSEP. Alex was worried about having anesthesia again, but he did great. We tried a different routine for starting the anesthesia and he handled it much better both physically and emotionally. I left him at 8:30. He was asleep, but was having seizures. They wound up having to treat his seizures before starting the testing. It took right around 4 hours to finish both tests. We got to a room around 3:30. Bug finally woke up aroud 5:00. He played for a few hours and we both went to bed early. Alex woke up Saturday having made it through the night with no fever! This is only the second time in his life he hasn't had a fever after anesthesia. We were discharged about 11:00 on Saturday morning for a fun weekend with the Knights!

Yesterday, we saw Dr. J the pulmonologist. She was happy with how Alex looked. She had thought about starting him on oxygen overnight, but since he is growing and doing well right now we are going to hold off a little longer. She wasn't real happy with his PFT (Pulmonary Function Test) so she sent us for a chest xray to make sure that his lungs had healed from the pleural effusions and atelactisis he had during our last addmission. The plan is to keep things the same barring anything on the chest xray.

Today we quickly saw Dr. Koenig so she could go over the MRI results. The SSEP is not back yet. The Syrinx in Alex's spinal cord is slightly bigger, but still does not appear to be compressing the spinal cord. For now we will continue to watch it.  After leaving Dr. Koenig's we went to GI. They are happy with where we are following his pancreatitis. We have feeds up to 20cc/hr and about 60% of our meds back to Jtube meds. We are switching his TPN back to 12 hours which is so much better for Alex! he is excited not to have to lug around that heavy bag and pump everyday! Dr. N also talked to us about the issues we are likely to see arise due to Alex's recurrent pancreatitis. We are hopeful the damage does not occur for years, but we can expect to deal with diabetes and pancreatic insufficiency requring enzymes at some point. After our appt we spent a fun afternoon at the park withthe Roeh girls!

Tomorrow we see opthamology, endocrinology and hematology before heading home Thursday.
Thanks for all the love and prayers.