The End of Summer and Back to School!

Finally!! Finally, I have a free moment to sit and write about the last two months of our lives. We've had a pretty good summer and thank goodness, it has been mostly uneventful.
Hollie filled her summer days(and nights)with a summer drama camp. She participated in Kids Backporch Productions rendition of The Music Man. She had a really good time and it was a good experience for her.
She learned a lot and is super excited about choir and drama in her upcoming high school years.
Aidan learned a lot this summer too. He learned that daycare isnt quite as exciting as he thought it was. He had a good time at his little summer daycare but he realized pretty quickly that he's much rather be at home. ;) He got to go on a lot of field trips and enjoy several new activities. He has almost mastered the task of swimming without floaties! I thought that was a pretty big accomplishment for a 5 yr old but he added the icing on the cake. He also learned to ride his bike without training wheels. Boy, can he ride like the wind!
Both of them started school this week(this was an accurate statement when i started this post TWO weeks ago)and they made it through the first week of high school and Kindergarten. Can you believe it? It just doesnt seem right that they are both so big! Aidan had a really good week. He seems to have a really nice teacher who already appreciates his personality. Thank you Jesus! Hollie has had a little bit more of an issue adjusting to high school and not having the same group of friends in her classes but she's made it just fine. I remember my freshman year being one of the hardest years of school. We talked about how changes are a part of our lives from our very first breath we take up until our very last. She told me "Dont worry Mama, I"ll be fine!" Thats my Hollie girl. Straight forward and to the point.

Now for our sweet Lydon. He's handled this summer amazingly well. He has had some trouble with fevers that we've become pretty accustomed to and he has still had trouble with vomiting when he gets over heated or exerts himself too much. We discussed this with our GI doc and agreed that since we cant control the extreme temperatures we are experiencing or keep him from playing or make him grow new mitochondria, we should put him on another stomach emptying medication. We started that the end of July and the vomiting has seemed a little better over the last two weeks. We are so happy because most of the stomach emptying meds on the market are only proven to work about 50% of the time. He has adjusted amamzingly well to our nurses. And we absolutely love both of them! They have enriched our lives so much. Without them, it would not be possible for me to work outside of the home at all. Lydon has done so great with them and his therapists. I'm gonna ask permission and post of pic of him with each of them soon. They have truly become like part of our family. His communication with signing has been so rewarding absolutely adorable to watch and the words are coming. He has several new words and a couple of two word phrases. My favorite of which is "My Mama!!!" And he loves to "argue" with his brother and sister with this phrase. ;)He is doing so well in fact that we want to take advantage of him being well and try to make up for some of the past times. So, we are increasing his weekly visits with the speech and occupational therapists to three times a week with each one. At this rate, he'll be ready to audition for a monologe in the years Christmas play.
This past week I had a telephone conference with Lydons neurologist. He is a vital part of our team because so many things that Lydon deals with ultimately stem from neurologic issues. When Ly was so sick and hospitalized in June, we checked several different lab levels. One of them was his CoQ10 level. CoQ10 is a chemical or vitamin like enzyme that is vitally important in the energy making process. It is also directly linked to cardiac health because of the high energy requirements of cardiac cells. Because of this, it is very common to give this supplement to mitochondrial patients. However, it is not very common to see a CoQ10 deficiency but Ly's CoQ10 level was quite low. So, this deficiency is indicative of what is going on in Lydons body. Coincidentally, his Complex IV of the energy making process is functioning lower than the rest and it relies solely on CoQ10 for it's energy to complete its process. Additionally, his CPK level was high. CPK is an indicator of myopathy. Because muscles cells are so energy intensive, they are full of mitochondria. So if they are deficient or dysfunctional, you would expect that there would be some myopathy. We deal with that. We treat his poor GI function and dysmotility. We accomadate for his muscular weakness with therapy and work on increasing his strength and endurance. But on the other hand, we do not want those myopathy levels to increase too much too quickly because eventually that will end up effecting more than his tummy or his weak core muscles and start effecting his heart. Cardiac myopathy is something we do NOT want to have to deal with and I pray that we never do. So, we are starting him on very high doses of COQ10 or Ubiquinol in hopes of correcting his CoQ10 deficiency and preventing in further increase or progression of his myopathy. We will recheck his lab levels in a couple of months.
The next thing we discussed was Lydons liver. Dr. F explained that one of his liver enzymes was elevated along with his ammonia levels and his PT/PTT (bleeding time) levels. One of these things individually may not be a concern but with all of them coupled together it is indicative that his liver is not functioning as well as it could be. So for now, the plan is to play the waiting game. We will recheck these lab levels as well in a couple of months after he has been on the ubiquinol and another mineral/enzyme supplement. Of course, I didnt like discussing any of this but at least we know that it is something that we need to stay on top of. We finished the conversation by discussing how much improvement we've seen in Lydons speech and energy level since starting some of the medicine and supplements. We believe that we can improve Lydons current lab levels and continue to work on his physical endurance and developement.
We have an appointment with Immunology and Opthamology in another week. Hopefully we will hear back soon from the new mito testing we sent off for back in July I really wanted to post pictures but I havent posted anything for two weeks. Maybe I'll get the pics posted next time....

Say a little prayer

for my "wittle" man Aidan. He is a sick little boy right now. He came home with high fever on Tuesday and we fought with it through the night. Jono took him to the pediatrician yesterday. He has pink eye in both eyes, double ear infections and a sinus infection. We are still fighting the fever despite already having antibiotics on board. He is miserable. :( His eyes are burning, his throat is raw and his legs are aching. We are doing our dead level best to keep him isolated from Lydon by staying in our bedroom in the back of the house (which he hates loves by the way) but it is still difficult. Our fear is that Aidan was obviously sick and contagious for several days before we knew it and Lydon could have possibly already been exposed to whatever bugs he has. Our hope is that all of the IV antibiotics that Lydon received last week in the hospital will continue to cover and protect him and will hopefully prevent him from coming down with any of this. Aidan also has a really hard time with medicine. He has a very sensitive tummy so the combination of fever, fever meds, antibiotics and a very sore throat is not a good combination. So, say a prayer that the antibiotics kick in and help Aidan feel better soon and that Lydon will stay well and out of the hospital!!

I think I'm gonna, I think I should...

wrtite a book!Seriously, I could write a book right now only I have no idea what kind of book it would be...mystery, drama, comedy, medical journal? The list could go on and on. Oh, I almost forgot, here is your disclaimer. This blog will be long and parts of it will be less than uplifting. If you are looking for a warm fuzzy read, this post by most standards will not qualify. Sign here to continue reading. ;-)
Ok, so I have to give a little bit of background here and bring you all up to date before I can continue. A couple months ago, I was presented with an awesome job opportunity. It didnt really seem like the right timing to us but during the last year or so, Jonathan and I have tried hard to accept and live by the rule that God's timing is not always the same as ours. Despite many reservations, we decided if all of the "ifs" and "what-ifs" worked out and a job offer was made, I would accept. Like a swift moving river, everything did fall in to place, even the little seemingly insignificant things. Hence, I started working as a nurse paralegal the first week of May at a great law firm near the Galleria. So far, I've really enjoyed it and it has been a great blessing to us.
Several months prior to me starting my job, we starting planning a vacation. We discussed several different options. Originally, we talked about planning something for the two of us but the more we talked about it, the more we decided we needed to plan something that involved our older two kiddos. We decided that they have endured a rough year as well with all of Lydons illness and hospitalizations and that they most likely wanted and probably needed the individualized attention from the two of us. So, we planned a cruise and they were beyond excited. We talked, planned, and shopped for this past week for months!
We had gone over everything and had even the fine details worked out. Everything seemed just perfect. That is until a couple of days before we were to set sail Jonathan checked the weather forecast and found that there was a 40-60%of rain and possible tropical development at both of our ports of call. We decided that every other time, the weathermen are wrong so surely they would be wrong this time. Then, Jonathan started feeling some of the same bizarre symptoms that he'd experienced last year when he was septic. So, with little encouragement from me, he left work early last friday (the day before our ship left) and went to see his PCP. She was immediately concerned. His blood pressure was very high...according to her, it was above stroke level and it didnt move or change regardless of him laying down, resting, etc. She gave him a couple of prescriptions and told him to take it easy for several days. "Not a problem" he said. "We are leaving in the morning for vacation." He went straight to the pharmacy and picked up his medicine and headed home. Later, he told me the floor in the store was "wavy" and the people were blurry.
We were both a little unnerved by this but decided it was all okay. Surely the weather would be fine and as far as Jono goes, better safe than sorry and we were grateful that he'd not procrastinated and gotten it taken care of. So, off we went. We had a great couple of days at sea and arrived in Progresso, Mexico on Monday. It was dreary and overcast but wasnt raining when we arrived. We decided to shop a little before heading to the beach. We walked down the street, through a couple of stores near the beach. Hollie hated it and all that Aidan wanted was a sword but we were glad that they had gotten to experience the streets and lives of people so obviously different from home. Many young people sat on the corner selling fruit, vegetables, or other homemade goods. We took about 20 minutes which is all they could stand and headed over to the beach. Again, it was overcast and windy but it wasnt raining and the balminess of the beach's atmosphere was actually refreshing. Some of the locals had massage tables set up on the beach under a tent for a very nominal fee. I found heaven and for 30 minutes I even forgot about my throat that had almost overnight become totally raw! :) The kids ran and played on the beach. I finished my massage and we headed back towards the terminal. We walked inside and literally within seconds, a torrential downpour sat right on top of us. We kept hoping it would stop but no it did not. The wind blew the pouring rain so hard that it stung our arms and legs as we made our way back. We had to take our shoes off because they were causing us to slip so bad. We were so happy to walk back on to the ship and get the warm towel that was handed to us. Everybody was looking forward to getting a quick warm shower and dry clothes and heading to the dining room for a late lunch. I got the kids situated in their room and Jonathan grabbed my phone to call home and check on Lydon.
I was just about to walk back in to our room when Jonathan told me that I needed to come to the phone without the kids. My mom proceeded to inform us that Lydon had been admitted to the IMU (step down ICU) at Hermann Hospital. She said that around 9pm Sunday evening his fever started going up around 103. She bathed him and gave him motrin and put him to bed. By 1 am, he woke up screaming and vomiting and had a temp of 105.2. She again gave him motrin and jumped in the car and headed to the ER. They immediately took him back and started running blood work, x-rays, etc. They took his clothes off of him, covered him in wet rags and put a fan blowing on him. It took four hours for them to finally get it under 103. They of course admitted him despite most of his routine labs looking pretty good. Regardless, the doctors and nurses could see how sick he was and his fever continued to spike. They started him on high powered antibiotics and blew nearly every vein available in doing so but it had to be done. Between my mom and the nurses they made contact with our neurologist who takes care of his mito issues as well. Some of his mito labs (ammonia, CPK) were abnormally elevated but that isnt unusual. His bleeding time was elevated and that's a first. Overall, they never did find a source of the fever but because of his mito issue, small kidneys, etc they did what they could in treating him with the "big gun" antibiotics. There was a question as to why his routine labs looked so good and he was so sick. We will address this with the immunologist in the next few weeks. The neuro ordered some very important special labs as well. I will post about those in a couple of weeks when we get the results back.
This of course put a bit of a damper on the rest of our trip. I know I shouldnt have been but I really was shocked this time and just not expecting it. Lydon has seemed so stable and healthy lately. It had been almost 10 weeks since our last hospitalization and that's the longest time we've been out of the hospital since Sept. It hurt me to think that he just got sick with no obvious explanation to say nothing of the fact that his body wasnt responding well to anything including the medicine they were using to treat him. Nevertheless, I knew he was in good hands. My mom is the closest thing to me( probably better if you ask my kids) and she knew exactly what to tell all of the medical staff. I knew that I also needed to put on a good face and have fun for Hollie, Aidan and Jonathan. I was upset later that evening but we had a good time the next two days and thankfully, the weather calmed down after that too.
I dont understand why after working hard and planning for months for this one week out of the year, we were hit with Jonathans scary blood pressure issue, my sore throat, bad tropical weather, and Lydons hospitalization. But, I have to believe that it all worked together for good. It was good that it was near impossible for me to get back home to Lydon. Otherwise, I would have and our vacation would of ended prematurely for our other kids. It was good that I didnt have to miss work for this hospitalization. (My vacation was preplanned and they knew that when they hired me.) It was good that Jonathans blood pressure spiked the way it did before our ship left and not after. The weather, I have no explanation for other than that I wished all of that rain would've fallen here in South East Texas instead of Mexico. To top it all off, last night Aidan started vomiting and covered every layer of our bed and furniture all the way down to the mattress. I have washed and cleaned all day. Jonathan and I have felt it necessary to eat nothing more than toast and sprite today. The good news there is that Lydon has seemed otherwise fine and the rest of us have been too. I dont know if any of this will make any sense to those of you who are reading this but it is what has transpired in our life the last couple of weeks. Happy July 4th!! Till next time...

Even more insight

Ok I realize it has been way too long since Ive updated. A lot has transpired since my lost post and as what has now become habit, I have been "sleeping on this one". Not because I dont want to share what is going on with us but sometimes Lydon's case just seems so complicated to explain. I dont ever want to post something that isnt true and sometimes I find myself regretting pushing that "Publish Post" button because it seems like almost as soon as I post something, just that quickly, things change with Lydon. These ups and downs are what makes his whole diagnosis so difficult. This whole scenario has become quite frustrating to me and honestly very hard to deal with. Which brings me to this post. I will preface this at this point by saying (aka warning you) that this post will probably be very long. I am going to try to update from our last hospital stay till now.
From the last update you all know that we were in hospital because Lydon had started running very high fever and we found that his J tube was out of place. In replacing this, anesthesia is used. This of course made us very nervous since Lydon has obviously had problems with anesthesia and intubation in the past. So, we met with the anesthesiologist prior to the procedure. We (Jonathan and I) explained to him Ly's recent results and that there had been some question in regards to the missing mito gene but that the previous neurologist didnt feel like Lydon had mitochondrial "disease". We told him how nervous we were about this procedure and Lydons previous reaction with intubation and anesthesia and would he please follow the same anesthesia precautions as before. He proceeded to tell us that he was comfortable using anesthesia that is not normally used with mito patients. We tried to "plead our case" with him but it was to no aveil. And, as luck would have it, they had trouble in the OR and before they could give report in the recovery room Lydon was in distress. Aggrevating to say least...I'll come back to this issue later.
We spent several more days in the hospital recovering from all of this and went home. Within days of being home however, Lydon started having tremors that were becoming increasingly frequent and alarming. We were referred to a new neuro to have this issue evaluated. When I spoke to the nurse about this we were sent back in to the hospital and readmitted. This new neuro met with us and looked over all of Lys previous testing. He explained to us that Lydons spinal tap that was done in SEPTEMBER showed a low neurotransmitter. This transmitter is responsible for signals involving speech and energy. Insert hand over open mouthed gasp...."Why imagine that!!" Futhermore, when its low, it can cause tremors. Needless to say, this was news to us but we quickly got over being irritated about not being informed sooner because we were so grateful to finally know that there was something else that we could do to help Lydon. All of these issues with the lack of energy,significant speech delay, tremors, GI issues, anesthesia issues were brought up with the new neuro in the hospital who assured us he would thoroughly look over Lydons chart. We had an appointment to follow up with him in clinic a few weeks ago and he kept his word. He sat down with me and everything he could put his hands on with Lydons name on it. He went through and showed me some of the most common diagnostic tools/tables that are used to diagnose mitochondrial disease. He actually "showed" me and didnt just tell me(or not tell me) what was important about all of Lydon's results. He said that he agreed with what genetics had told us months ago, that the missing gene associated with his chromosome deficiency IS an issue. There is no way (at this time) for us to prove that your body can work normally with only one copy. Obviously, Lydon only has one copy and his body is not working normally! He finished by telling me that Lydon has a definite mitochondrial disease (with a complex iv deficiency for those who are interested).
I left that appointment with more insight and emotion than I had planned on experiencing that day. It took me a few weeks to go through the plethora of emotion I felt with all of it. I was mad for weeks! Why were we told that there was no mito involvement causing any of Lydons problems when according to several different physicians opinions, diagnostic tests and more than one reference tool there is? Why were the anesthesia precautions so prematurely removed from his chart when he has had problems with respiratory distress and acidosis everytime? Why did my baby have to suffer unnecessarily?
Our new neuro couldnt answer all of these questions for me but he answered a lot of questions about Lydon and what is going on with him. He told us we should continue to see improvements in his speech and energy level from treating the neurotransmitter deficiency. He also started him on a combination of vitamins and supplements that should help him have a more constant energy supply and not have to work so hard to make more. He has been on all of this for about six weeks and we have seen some definite improvements. He is babbling a lot more and really trying to talk. He has had some really good days of late. In fact, a couple of times during the last two weeks or so, he has eaten pretty normally and made it almost all day with just a very short nap. He still has his issues. Last night he accidentally touch the top of the toaster and burned just the edge of two of his fingers. Within just a few minutes, he had vomited and spiked fever over 101 but after getting to bed early and sleeping a little extra, he woke up happy and energetic today. All in all, a lot of good has come from all of this. We had to go through a couple of roller coaster rides to get to the bottom of things but ultimately, we got there. We have what we feel like is a definitive diagnosis that makes sense. We now have a neurologist that doesnt require Lydon to meet certain criteria in order to continue treating him but instead will treat him regardless and continue to do what is in Lydons best interest. We have once again proven to ourselves that fighting for what you believe is usually the right thing to do. Its not always the easiest but it is worth it. We've had several other changes over the last two months, I will blog about more of that later, but everyone including Ly is adjusting and doing very well. The best news of all....We havent been in the hospital in almost two months!!! More to come later..

Back to our results

I'm sitting here with time on my hands so what betteer should I do than blog. It seems I always have so much I could write about but just don't always have the time. I don't really think that anything I write about is profound but I do hope that in some way some of things I write about can be inspiring to my friends or a help to somebody else that may be going thru some of the same things as we are with Lydon. So, last week we met with Dr. Koenig who is the mitochondrial disease doctor. As I said before, we were very anxious about this appointment because when we met with genetics, they had told us that included in Lydons chromosome deletion was a nuclear encoded gene for mitochondrial disease. Dr K explained that based on Lydons results, she does NOT think that Lydon has mito. Ill admit, I was confused and thrown off by hearing this. I even felt a little let down. Not because I wanted Lydon to have mito in any way but I do want Lydons medical issues recognized and treated rather than dismissed. Especially when the so much of the decision as to how or when to treat some of Lydons issues has seemed to hinge on a mito diagnosis. I explained this to Dr K and she explained to us that she felt sure that Lydons body was compensating for the missing mito gene but was struggling with the chromosome deletion. She said that his deletion does explain everything from his growth and language delay to his small kidneys, energy deficit, and GI issues. So, that made more sense to us and we were relieved to have the mito monkey off our backs. For now, we will take things as they come without the worry of a progressive degenerative disease! Dr. K asked my permission to send an email to Lys physicians explaining what we talked about and that Lydons issues needed to be addressed and treated accordingly since we can't very well make that part of his chromosome reappear. That gave me some peace of mind and reassrance. Although, I am thankful that we seemingly no longer have the worry of everything that goes along with mitochondrial disease we still have some of the same issues and now, some that are very different. There isnt a "Chromosome 16 Parents support group". There isn't a doctor that specializes in Chromosome 16. We dont have to worry about this deletion progressing but we dont know what this deletion will mean for Lydon long term. Despite all of this, I do feel quite confident in all of Lys doctors and I am very optomistic that we will be able to move forward now in doing what is best for him. He is such a sweet innocent little angel boy and shouldn't have to endure anymore pain or stress on his little body than necessary. I chose nursing as my career because I love medicine. The science of most of it just clicks and makes sense to me. I chose pediatrics because I love children and babies. I feel like they are straight from Heaven and still posess a pure and innocent spirit without the corruptness that most of us carry by the time we hit puberty. Now, more than ever my goal is to take care of Lydon and his specific issues based on just that. I want for him to enjoy his childhood with as little pain, fear etc as possible. He has already taught me so much about life. How can I complain about something that is really big to me (like my thighs or my rear end or sleeping in a window seat in the hospital night after night) but yet be SO insignificant when he struggles just to communicate with us and is seemingly punished by his GI system for eating an extra cookie or drinking a little more apple juice than he usually does? He deserves so much more and I am so blessed to be his Mommy. My prayer is that I can somehow continue to help Lydon live life to the fullest through all of these complications without hindering him or holding him back in any way and maybe just possibly learn something that will help and encourage others and bring joy and a purpose to this unknown path that we are enduring.

I shouldve known

So, the plan was to go to surgery today to replace Lydons tube. Well, guess What? That did not happen. When we talked to Gi,surgery,and the Anesthesia team today everybody was pretty concerned about Lydons high fever that we came in with. They all felt that the fever coupled with his airway issues were too big of an anesthesia risk. I was hoping that we may get lucky enough to convince them to do it tomorrow and let us go home because Lydon didn't have fever all day. But, wouldn't you know it,about 7 o clock tonight Ly spiked back up close to 102 and even an hour ago it was still sitting at 101 after Tylenol and cool rags laying on him. So plan B it was. We took out his GJ at the bedside this afternoon and replaced it with a plain g button. We are running continuous feeds since Lydon didn't want to eat at all today and we will do so slowly all weekend. Monday, we will go to the OR do an endoscopy to peek at everything an place a new GJ. Hopefully we will not have any anesthesia or airway issues and will be able to come home by Tues. So, we will spend yet another weekend in the hospital but that is the safest thing for Lydon so I won't complain. We were really blessed the last two days. My mom was off with Aidan and so Jono took off to be with us. It was really nice to have him here and not be alone this time. And thank goodness we've had some awesome nurses which is pretty much the norm here but you never know for sure how things will go. God is so good!!

In the hospital...

I know a lot of people are curious as to what happened and why we are here. This was obviously not a planned visit. Wednesday evening Lydon spiked high fever(103.4). I was concerned but I've learned not to panic or worry as quickly or as much as I use to. This is where the phrase "new normal" is inserted. So, I gave him motrin along with his other med and put him to bed. He slept fine until about five when he woke us up hysterically screaming and back to 103.4! So, I went thru the whole routine again but could only get it dowm to 102. He had no other symtpoms so I called our pedi and we were told to bring him in. Despite all our effort, he was at 104 and climbing when we arrived. They immediately put us into a room but it took hours before we were seen. He was beside himself mmiserable. They finally got labs xray etc done about two o clock. About four they came into the room and said that everything "looked fine" and we should take him home and come back if anything changed. That didn't sit too well with me. So they went out to call our pedi and take a second look at things and wouldn't you know it suddenly his xray looked very different! His J tube (the tube that is in his intestine) had completely twisted and coiled into his stomach. So that really started spinning things into a different direction. They started makin calls to the surgery team and trying to get a bed for us. We finally got into a room about 8 last night. We still aren't sure what to think about the fever and whether or not it is related but we are concerned about the potential for the tube to perforate his stomach since it was held in place with a wire. The plan is to go to surgery later today and hopefully have no respiratory issues and go home tonight or tomorrow. We had a really good appointment with Dr K regarding all of Lys results. I will post more about that later but for now he is sick and we will deal with this first. Keep us in your prayers.