Alex and Maddy

Alex and Maddy

Tuesday, April 30, 2013

Results are in!

I have had Alex's results for about a week and have avoided updating because I hate to update when I don't have any good news. I really try to stay upbeat in life and follow suit on this blog. I hate to blog when I have nothing, but bad news. Unfortunately, I decided I couldn't wait any longer.

Dr. O called last week and while the results weren't surprising they weren't encouraging. Alex's stomach did not contract at all except with one of the two medications. However, that medication is a medication that is known to be troublesome to some mito patients and has been tried twice in the past with Alex with bad side effects both times. However, if we have any hope of Alex's stomach working at all to try and eliminate the stomach secretions and gas that build up we may have to give it another try. Dr. K and Dr. O are in conversations to try and weigh the risks vs benefits and come to a decision.  As for his small intestine, that didn't look so hot either. The contractions he had were completely erratic and not in the organized pattern they should be. He was officially diagnosed with Chronic Intestinal Pseudo-Obstruction. Basically his intestines act like there is an obstruction when there isn't. His is neuropathic in nature or caused by nerve issues and there isn't really anything you can do to "fix it". We are adding in a med Alex was on as a baby, but we didn't see huge benefits back then and aren't expecting huge changes now. Which leaves us between a rock and a hard place. Alex's continual GI issues are causing worsening Respiratory issues and at some point breathing takes precedence. The last resort is a surgery that I would have a hard time consenting to at this point.  We continue to pray that Alex's little body keeps fighting and beats the odds. He has come so far in his little life and we know he will continue to amaze us all!

On a fun note...yesterday we had the pleasure of volunteering at Wish Upon a Par. We always love out volunteer opportunity with Make a Wish and the family we have found through this incredible organization. They have given us so much more then a one week trip. They continue to bless us with the opportunities and friendships we cherish! Alex got to play with his good buddy Kendall and I got have some "girl time" with Ginger (Kendall's Mom).  It was an awesome day all around!

Thanks for checking in with us!

Sunday, April 21, 2013


I realize I never updated on Thursday about Alex's testing. It was a much harder test then we had anticipated. As I had mentioned on Wednesday while Alex was under anesthesia he had probes placed throughtout his stomach and small intestine. Early Thursday morning those probes were hooked up to a computer that monitors the pressure and thus contractions in the GI tract. Alex was hooked up at 7:30 to start the test. At that point we had to stop draining his stomach like we always do and have done for the past 5-6 years. The first 3 hours of the test was supposed to be watching his GI tract at rest. Alex was extrememly uncomnfortable. This should have been the "easy" part of the test, yet Alex lay there with tears running down his face complaining of pain. Dr. O had the GI nurse tell us to go ahead and drain his stomach again. They also discovered that Alex had displaced a tube the night before when he had gotten sick and it was in the opening of the esophagus. They had to pull that tube out about 10 cm. Alex was so upset and worried about this, but he was a trooper as we got the tube correctly positioned.  After that he was okay and the rest of teh 3 hours went without incident. At 10:30 they gave Alex the first of 2 medications (erythromyocin). This is a medication that some mito kids and Alex specifically don't do well with. We had tried it in the past with probelems, but Dr. K had okayed a one time dose to see if it would help his motility for the testing purposes. Several minutes into the infusion Alex became violently sick adn was puking bile over the end of the bed from laying flat. He was also having trouble with choking because he wasn't allowed to sit up. He was miserable. He was shaking uncontrollably for over an hour after this medication. I checked in with Dr. K who felt like it was probably from the medication, but seeing as it was just one dose it should be fine. I was shocked as Alex is just not a puker at all. Once that med was done they administered the second medication (octreotide) which also caused major wretching and vomiting. We were glad when the medication portion was done! He was given about 45 minutes to be sure that the medications were out of his system and then the plan was to give him 200 cc's of formula over 30 minutes into his stomach. Any of you who have followed us for a while know that we don't use Alex's stomach because it simply doesn't work. After the morning we had had neither the nurse or myself were optomistic about teh feeding portion. We started the feed and watched as Alex became more and more bloated and uncomfortable, but never vomited. They watched him for an hour after the feed and then we could stop the test. We just needed to put in a new tube and we could drain his stomach again. Unfortunately, it was not our day and his tube wouldn't fit as his stoma had closed around the smaller tubes used for the test. We had to stick a catheter in temporarily while the nurses went to get a smaller button to place. He was certainly happy when a tube was in place and we could drain his belly again. As soon as we hooked up the ferrell bag (drain) all the formula we had fed 2 hours earlier came out. Dr. O came by that evening and let me know that he would have full results for me early this week. The one thing that definitely jumped out at him is that Alex never had stomach contractions the whole test except when we gave the erythromyocin. Even the feed didn't generate any contractions which explains his extreme bloating and lack of vomit. I have to wait to figure out what else we learned.

Last night, I was lucky to get to spend the night with some girl friends. Alex also had his friends over. It was a fun filled evening. I had a Lia Sophia Jewelry party. I have decided to leave the party open until next Saturday. If any of you that aren't local or couldn't come would like to look at the catalog i will post the link here. There jewelry is really pretty adn affordable. If interested you can go to  Click on Browse Jewelry. Then click on find Hostess adn search by my name. Once I am listed as the Hostess you can click on the spring summer catalog. There special right now is by 2 full price items and get up to 4 at half preice. The cool thing is that you can use your least expensive items as the full price and your more expensive items as the half price!

I will update this week when I hear from Dr. O with the remainder of the results.

Wednesday, April 17, 2013

Finally done!

What a long day it has been! Let me back up though and start from yesterday....

Yesterday morning, Alex and I headed in to Fort Worth to Cook children's Hospital for an appointment with Dr. O, our GI. Dr. O direct admitted Alex following the appointment so we could complete some procedures and testing. Luckily, they had a room at the hospital ready for us as soon as our appointment was over, but it makes for a long day entertaining a healthy 8 year old boy in a hospital room. Alex was pretty good and we had anesthesia come by to discuss Alex's history and past problems with anesthesia. I was pleasantly suprised by how open and receptive the doctor was to listening to me and our past history and trying to come up with the best plan possible for Alex. His hope was that he himself would be assigned to Alex's case this morning, but he assured me that if not he would pass on all the info. Late yesterday afternoon I got a call from a 2nd anesthesiologist who was actually going to be doing Alex's case and he went over a bunch of info as well. He even went so far as to call our favorite anesthesiologist in Houston to discuss Bug with her. I was very happy with the overall time and care that was taken to assure that Alex was safe.

This morning we were scheduled for 9:30. They called up and said they were running a few minutes behind and would be up in a bit for him. They came to grab us about 10:15. When we got to pre-op, we spoke to a few people and then they let us know that there was a problem and it would be about an hour. Okay, no big deal....well 1 hour turned into 3 hours! They finally took Bug to the OR about 1:10. I was able to go with him and he did the best he has ever done from an anxiety standpoint. They gave him a steroid and something to control seizures and started the anesthesia. I left him shortly after. They were doing several things while under. They did an upper endoscopy (which looked good except for irritation by his Gtube which he has been complaining about), a pyloric dilation with botox (to open the opening between his stomach and intestines) and they placed probes throughout his GI tract for an advanced test that will be performed tomorrow. Dr. O came out to talk to me shortly before 3. Alex did well. His pylorus was once again very tightly closed (this is the third time we have done surgery on it) Dr. O is hoping that the dilation with the botox will work better this time.  At this point Alex is still sleeping and is doing well despite running a temperature. We are watching him closely, but overall things went as well as could be hoped for.

Tomorrow they will hook up the probes to a computer and see how his GI system is working both with and without feeds running. We will also try 2 medications to see if either one improves his GI motility. Our hope is that this test will give some good information that will help us with Alex's GI system and especially the bloating that is causing so many respiratory issues at this point.

I will try and update at some point tomorrow.


Sunday, April 14, 2013

Update on Appointments

We made it home from our trip to Houston late on Wednesday. It was kind of a mixed bag.

Monday we only had one appointment with our Pulmonologist. It was by far the "worst" appointment of the week. Alex has been complaining of shortness of breath for some time. I knew it needed to be addressed, but wasn't ready for some really bad news! We repeated Alex's PFT's (Pulmonary Function Test). Two and a half years ago, Alex's PFT's were perfect at 100%. We have been watching them slowly decline since then. Presently, he is sitting at 40%. More concerning, they are showing a "restrictive lung disease" pattern, not asthma. This confirms our worst fears that Alex's GI problems are negatively impacting his respiratory status in a big way. His GI bloating and distention has always been a problem. It is so extensive at this point that it is resttricting his lung space and not allowing his lungs to expand properly. This needs to be addressed immediately. Unfortunately, we have already been aggressively trying to control the bloating. It is a real possibility that we may lose feeds do to this problem. It would be devastating to go TPN only because of this after working so hard for so many years to keep some feeds going. We see GI Tuesday and this will be a big topic of conversation.

Tuesday we saw Dr. Koenig. She is overall very happy with where Alex is, but concerned about this new development. We also saw the opthamologist. Alex's eyes are nearsighted, but because he doesn't drive we can hold off on glasses a little longer.

Wednesday we saw Dr. Pacheco who was also thrilled with how Alex looks and is doing. Things are status quo from her stand point. We also saw cardiology. Alex's ASD closure looks great with the closure device in place and no leaking around the edges. YAY!

In other news, we have withdrawn Alex from school this week. At the beginning Alex loved going. He looked forward to it. I think a big credit goes to his first teacher who worked really hard to make Alex feel included. In February, she moved into an administration position and he had a new teacher. We have also noticed Alex's anxiety and OCD getting worse. We had a couple of thoughts on cause, but we knew the pressure of the upcoming end of grade test was one of the causes. That coupled with the chaos of our new schedule were a lot for him to handle. He wanted to go less and less and was acting out more and more. Weeks we didn't have school we noticed a totally different child. Regardless, we were sticking it out....until I got a call last week informing me that Alex's homebound teacher was also being removed and changed to someone new. Another change in teacher after working so hard to establish a relationship with our other teacher is not in Alex's best interest. It was the final straw. Alex and I have been very happy and successful homeschooling for many years and are ready to get back to "normal".

We have had a really nice visit with Mimi and PopPop and are really looking forward to our summer trip!

Friday, April 5, 2013

No news is good News

I know, I know, I have been totally slack about updating! As always, no news is good news! Alex is doing great. We are finally seeing some of the spark in him that we have always seen when he is on TPN. It took so much longer this time. I was speaking to a doctor we have seen for a while and she really felt that him taking so long to come around really speaks to how big of a hole we had dug nutritionally. She commented that this is the best she has ever seen him look!

Staying on the medical front...Alex and I head to Houston this week for a number of follow up appointments. It should be a pretty laid back trip as I don't have a whole lot of concerns. My one main concern is that Alex is having a lot of respiratory issues. He is feeling short of breath multiple times a day. All of the doctors feel like this is most likely related to his GI distension/bloating, but I still need someone to address how we can make him more comfortable.

Alex is bouncing off the walls with excitement because Mimi and PopPop are arriving tomorrow for a vacation! We can't wait for them to get here. They will be joining us on our trip to Houston and then staying for a few more days when we get back!

Alex and I trialled a Homeschool Co-op this morning that we will be joining in the fall. Alex had an absolute blast. We had only planned on staying for the first 2 hours, but wound up staying the last hour as well because Alex didn't want to leave. He was chattering about lots of new friends on the way home. He can't wait until the fall when we can join them on a regular basis. I am always amazed by Alex's confidence and grace as he handles new situations and questions. He just loves people and thrives around kids of all ages. It was nice to be in a group of nice, well behaved Christian kids with the same values we have. Alex felt right at home. On the way home, he told me,"I think homeschool kids have are more imaginative, just like me". So true!

Alex continues to love his Mia. She really is such a good match. She loves to play with him, but also is really sweet and cuddly. She is so smart. She is definitely a water dog. Twice now she has taken a flying leap into the bath tub to join me or Alex! I think swimming is in her future.

Bruce continues to love his job. Life around our house is so much better with him so happy at work. We are feeling very blessed right now.

I will update next week from Houston.