Twas the week before Christmas

and since we seem to have plenty of responsibilities, drama and deadlines to adhere to, we decided to take a break from it all and try to steal a moment to "smell the roses" and see the lights. I was sitting in the waiting room of a doctors office several weeks ago (imagine that!) and saw that the Hill Country area does a lot to decorate and celebrate the Christmas season. I knew that it would take a

lot of planning and effort to take such a road trip but I wanted desperately to be away from all of the hussle and bussle of our everyday life even if it was just two days. So we quickly planned and made the arrangements necessary to make it happen. Jono and I took off on Friday. We got up early that morning and headed out. It wasnt the easiest trip for sure but it was such a nice get-a-way. The kids had a blast! A lot of it was hard on Lydon but he loved every minute of us being together...after all that was the purpose. Here are some pictures. Hope you enjoy.

PRICELESS!!! Worth all the planning, packing, praying and paying from start to finish. ;))

Christmas Eve

Well, wouldnt you know it? It took me till Christmas to find the time to add an update but so much has transpired over the last two months that I want to get this added while I can. Where do I begin?
On the Monday after Thanksgiving, we had an appointment with the pulmonologist/sleep specialist. We discussed the results from the sleep study we did with Lydon earlier in the year. He had several apnea episodes and some desaturations where it took him several minutes to recover. We also talked about how he has been doing lately. Of late, Lydon has had some weird sleep/wake patterns, he gets very short of breath with little exertion and frequently ends up vomiting. This is a direct result of his energy deficit. So, we agreed that the best thing to do was to start him on some low dose oxygen at night and see if that helps at all. We worked and got him adjusted to the pulse ox after the first several days. The oxygen wasn't quiet so easy. He protested the mask and the nasal cannula and made it very clear that neither would be his choice! But, every once and a while, Mommy can be even more stubborn than the lil tough guy. So, I persisted (Sissy helped) and we won! He's been a trooper. He is now sleeping every night with his pulse ox on his toe (AKA His rudolph toe) and his super-hero super power AKA (His nasal cannula). We have struggled with this decision emotionally but we understand that it is something that helps him not have to work as hard physically to keep up with the metabolic demands of his body especially when he is sleeping and needs the extra rest.
Our next appointment was unexpected. The neurologist we met with last month called me in to discuss the results from the Mitochondrial labs we sent off back in JUNE!! Yes, it really took almost six months to get these results and this is considered normal for this test! He was not seeing pt.'s that day but he came in to the office just to sit and discuss everything with me. I told him that we were planning to follow up with him in January but he said that he didn't want for me to wait any longer! What a sweetheart and how blessed we are to have him on our team. He talked with me about a couple of dominant genes that came up. He explained we (Jonathan and I) needed to do some blood tests to be absolutely positive but regardless he didnt feel that either of these two genes were responible for any of Ly's issues. On the other hand, he had six other genes that were reported and all of them were mito/metabolic in nature. He told me that from a neurologist's point of view these were very significant because three of them go hand in hand with each other and all of them go along with Lydons clinical symptoms. He said that he had researched all of the genes that were listed on the report and reviewed their significance in the body's energy making process in addition to reviewing all of Lydon's history. He said that he thought we should draw another lab test to be positive but he felt confident in saying that this was all indicative of the mitochondrial disease MINGIE's. What??? Even though I knew I was there to discuss these results and I knew that he had called me in unexpectedly, I was shocked and taken by suprise when I heard him say this. I didn't know whether to laugh, cry, or throw up. I mean after all, we have been waiting for over two years for an answer or explanation for Lydon's issues. We have had to fight for Lydon's safety several times because we didn't have a definite answer or diagnosis. But still, hearing something so concrete about my boy kind of felt like my stomach got pushed against my backbone. We talked for a very long time. I was overwhelmed with the extent of his knowledge and patience and willingness to talk to me until all of my questions were answered. I left feeling overwhelmed with what all I had heard but also very relieved to have more of an answer at the same time. He wrote an order for some labs that we will have drawn when we go in for his EEG in January. We talked a lot about his dysautomia. Dysautonomia is a malfunction of the autnomic nervous system. The ANS is what regulates all of our unconscious systems of our body like our heart, stomach, endocrine and metabolic systems. We believe Lydon is having these issues because of the diseased mitochondria in his body that causes his brain to not receive the total amount of energy it needs. This is causing Lydon to have some fluctuations with his blood pressure, pulse, oxygen level and of course his GI system as well. There is a cardiologist at UT who is one of the only doctors in the area who specializes in dysautonomia. So, we are going to make the necessary arrangements to get with him to do a couple of specialized tests. I will post an update after we get all of this done.
So, for now that is the latest medical update. Let's hope this is the last one for this year! Lydon seems to be doing very well, all things considered. He is a happy stable growing little boy. Tonight, my heart is full. I am so very thankful that all three of my sweet babies are asleep in their own beds and we are not in the hospital. We spent the majority of last December in-patient. I promise to try to post some pictures in my next post if I can get the time to compose another one! ;) Merry Christmas!!

Happy Thanksgiving!

As is normal for us I realize this post is a couple of days late but I couldnt let the week finish out without this post. We have SO much to be thankful for this year. I've gone back over the last year several times this past week. Lydon has made so much progress in the last twelve months. He is doing amazingly well overall. He still has his chronic issues but so far this year we've been able to stay home. We've only been in-patient this year a few times which is a far cry from the months we spent in-patient last year. We have gone from this..

to this...

To this!
So, yes despite all of the challenges, we've obviously had a great year!







We had a wonderful Thanksgiving,

with each other,

and lots of family! On medical note, we have seen some improvement since starting the new supplements and increasing his Amitriptylline. He's seemed a little off with his stomach and coloring of late but overall very stable. We got a call that the results from the specialized labs we sent off in June have finally come in!! I havent been able to speak to the doctor myself yet but from my conversation with the nurse, it sounds like we may have some answers! Finally....some answers! Cant even begin to describe how just the idea of that thought seems!! We have an appointment on Monday with Pulmonology. We have a dental appointment which we may put off until Jan. Other than that, we are very much looking forward to the next month of more time off together. Here's to some cold snuggle weather and no colds, viruses or infections of any kind!!

November

I promised I would do my best to update from our last appointments so here goees. This is going to be very brief because I am once again working with only one arm/hand. As you may know, I had a compressed medial nerve and had it repaired several weeks ago. Well, as luck would have it, my radial nerve was compressed as well. So last week I had the surgery to correct and decompress it as well as clean out and repair the tendonitis in my elbow. It has been painful but hopefully it fixed my aching problem and will be worth it.
So, back to our appointments. We first saw GI. The worst part of that appointment was the wait. We arrived at 11:20 for an 11:30 appointment and were finally called back and seen at 1:00!! Not much to report really besides the fact that we doubled his nightly dose of Amitriptylline(again) in hopes that this will help with Lys vomiting. After the long wait,we were happy for this appt to be short and sweet. Next was nephrology. This appointment went very well too. The only issue we really spent time discussing was the fact that Lydon has started drinking A LOT! Many days, he will drink until he vomits if we let him and since vomiting is an issue for him, this really is a concern. This can be indicative of a kidney issue (Diabetes Insipidus). That can be associated with some of the findings on Lydons last U/S and is a common condition with some mito kids. So, we will do a water deprivation test the next time we are in patient. Otherwise, his urine and blood pressure looked good. Finally, we met with Neurology. This was the most important appointment of the day. Poor Lydon was so exhausted. He was pretty much done with anybody in a white coat at this point. Regardless, we tredged through and made it. I was anxious about this appointment. We desperately needed a Neuro that was here locally and on staff at Childrens Memorial Hermann but we also needed that person to recognize all of Lydons issues and diagnoses so that we can treat him appropriately. This person was exactly what we needed and hoped we'd find. He talked to us about all of Lydons history. He talked about his chromosome deletion and his mito issues. He agreed that Lydon has obvious mito issues that need to be treated as such. The newest thing we discussed was his dysautonomia. Basically, Lydons neurological system isnt doing what it should to regulate his temperatures,sleep, etc. We will start Melatonin to see if this helps any. We also discussed the fact that several of these things point to an issue within his hypothalamus which could also go hand in hand with the mito issues. We made some medication changes in order to protect his liver, kidneys, and stomach. So, three less meds for Lydon. YAY for that!! We are scheduling a 23 hr EEG. This will not be fun but it is necessary. We will follow up when this is done. Next week we will see our pedi and hematology. Until next time.....~Jana~

Update

We saw GI, Nephrology, and Neurology today all were good informative visits. I will update with more detaiils soon but we are very relieved to go to bed tonight with another great neurologist as part of our team! We are having to make a few changes with some meds etc and we are happy with the outcome of all of our visits but so glad today is over! More to come later....

PING-PONG!!

During the last several days, if there were an item that would describe me, I believe it would be one of those little out of control bouncy balls that kids love and adults hate. Often times, it seems that life throws us around from side to side but sometimes that may be what makes the course interesting and allows us to appreciate the straight and narrow. I’ve become accustomed to that very well over the course of the last year. But following the events of the last week, I’ve felt like I was not going along the straight and narrow or side to side but more up and down. This should give you some idea.
Sunday- Take care of breakfast, meds, changing and dressing so I can hurry up and get a little more work done before I go to work the next morning. Work as fast as I can to make up for all of the work I haven’t gotten done so far. –Guilt: Guilt for not being at church, Guilt for not getting all of the work done yet, Guilt for not being with my kids while are happy and playing. LOW
Get some work done. Eat lunch with the kids. Run a couple of errands. –Relief: Relief for getting some things accomplished. HIGH
Found out that my cousins baby is very very sick and has been rushed to TCH in serious condition. -Sorrow and Concern. LOW
Receive a call from my sitter for Monday that he is unable to work. –Stress- that I wont be able to go in to my office. HIGH
Getting everybody bathed, fed and in bed for the night. My stupid jacked up arm that is suppose to be fixed is aching horribly.- Pain-LOW
Wake up Monday morning and my husband is terribly sick again! -Stress and worried of what any kind of illness in our house could mean at this point. HIGH
Still can’t get any work done because my kids are crazy, I do not have nursing help and Jonathan is sick. – Frustrated- LOW
Lydon climbs out of his crib before and after nap!! –Anxiety with what this could possibly lead to. I cant handle that tube being pulled out again. HIGH
The afternoon is better but I still didn’t get as much work done as I wished. My arm is still really hurting. I’ve cleaned mess after mess from the boys. I’m exhausted and don’t know how I’m going to get up and go to work tomorrow. –Overwhelmed!- LOW
Finally get the day wrapped up and the boys in bed but not before realizing that Lydon has a 101 temperature. –Panic: Panic of what the fever is from if anything…who knows?!- HIGH
Get up at five to get ready but worried about Lydon, my arm is hurting. Decide to stay home a couple more hours and go in a little later- Stress and worry-LOW
Get a text that my mom’s household has a stomach bug! Sympathy and worry of what that could mean for her and thankful we haven’t been with them in several days so we should be safe from the puking bug! HIGH
Finally get to work but feel overwhelmed with responsibility and worry for my boy. Wish I were home with him to keep a closer watchful eye. LOW
A co-worker stops and asks me about how things are at home. HIGH,LOW,HIGH, LOW!!
She send me a sweet encouraging email. HIGH
I work hard and get several things done but leave later than I planned. Traffic is horrible! LOW
I stop and pick up dinner and get home and hear about the kids day. They are both happy. HIGH

I clean up all of the mess. Give Lydon meds and put up a crib tent to solve the climbing out monkey business. Relieved that he and his tube are safe and sound and contained in the bed. Sad that he has a tube and can’t graduate to a toddler bed. Glad that all of this done at 9:00. Bummed that I missed the show I wanted to watch. Happy that I get a few minutes on the couch with Jono and Hollie. Irritated that my arm is still hurting so much. HIGH, LOW, HIGH, LOW, HIGH, LOW!!!!
If you are still reading this, would somebody please catch me and place me gently on a smooth surface so that I can roll along! ;0)

Surgery and Hospital Update

Ok, so I'm going to try to make this one quick but get in all the important details. We are home! Thank you Jesus!! Lydon is napping at the moment which is a lot of what he's been doing since we got home. Aidan is playing with birthday toys (don't 'cha just love it when they get new toys and actually play with them?!). Hollie has gone running...my little health concious teenager! And Jono is watching the UT game with Dad. So, I have a quiet moment to update. I am suppose to be here in our home office working on real work that I have brought home from my job but I just cant focus. I've never really had trouble focusing. I've always been pretty self disciplined (except with food and excercise! ;0))but right now, I am having trouble getting it together. I do really good during all of the procedures and most of our entire in patient stay but when I get home and feel the exhaustion and I go over all of the events of the last several days, that is when my heart aches and nerves seem fried! So, as guilty as I feel about not working on "work work" right now, I'm gonna try to get this post posted and hope I feel better.
So, Wednesday morning, as planned we headed to the outpatient surgery dept. We got up at 4:30 (yuck!) and checked in at 6. Thank you Paw Paw for coming and help get Mr. Aidan to school so that Daddy could go with us. Let me back up just a little. On Tuesday, I called the anesthesia department. I was so worried and so tired of going through the same routine that we go through each time Lydon has anesthesia. I talked to one of the wonderful anesthesiologist there and explained all of Lydons history to her. She said she would pull his chart and look it over and for me not to worry. I felt some comfort in this but didnt really have a lot of expectations. Once we were pulled back into pre-op, the same sweet lady came in to talk to us. She asked us some more details about exactly what happens to Lydon. We told her how he comes in to PACU breathing ok for a little bit but then ends up in distress and becomes acidotic. She said that she saw that from the record she pulled from our last procedure in March but she felt that our biggest problem then was the fact that they gave him Propafol and didnt follow any Mito precautions. Finally we all agree on something! She assured us that wasn't going to happen this time and that she was going to do the safest thing possible. She asked us if we had ever had a non eventful experience and we said no. So she pulled all of his anesthesia records at that point and told us the more she read the more concerned she got.

So, she said we needed a safer more controlled plan than what has gone on in the past. She said she would take him back to the OR and see how he did and go from there but she prepared us to end up in ICU. So, consents were signed, everybody bunnied up and back to the OR we went.

After the procedure ( which ended up being almost an hour late because of all of the discussion of what we should do) she came out and talked to us again. She explained that she gassed Lydon down with one of the "mito safe" gases and got a couple of IV's started. She then switched him over to the IV meds and got a quick tube of blood to check his gases. He was already mildly acidotic at that point. So that sealed the deal and bought him an overnight stay in the PICU. She told us her opinion of what is happening and we all agree that Lydon does ok for a few minutes post op but after that, he just doesnt metabolically have the ATP reserves available to keep up with what he needs to recover from the anesthesia and breathe on his own. He is energy deficient, metabolically acidotic and has poor muscle tone with his narrowed airway as a result of all of that. So, as soon as Dr. R- GI doc was done, they went up to the PICU. By that time, he was even more acidotic so they turned up his IV fluids and remedicated him and kept him intubated for a couple more hours. He did pretty good until they allowed us to come in the room and when he heard our voices, he started waking up. They really werent ready for him to wake up and had planned to keep him intubated even longer and possibly wean him off to CPAP. CPAP is continuous positive airway pressure that basically blows air forcefully enough to keep your airway open. Apparently, that was not what Lydon had planned! In the midst of them trying to medicate again and keep the tube stabilized, he reached up and pulled it out! This sent the entire team rushing around frantic because as strong physically as he is and as strong willed as he is, he just wasnt ready respiratory wise and wasnt really where they wanted him metabolically. But, like we told them, Lydon plays a different ball game with his own set of rules! ;)

They were very quick and got things stabilized within about an hour. During that hour, both of the IV's they had started in surgery blew. They called the pedi transport team who is the best of best and frequently starts IV on teeny tiny infants and frequently are in stressful situations out in the field. It took seven sticks to get an IV in and even then, we couldn't pull blood from it. That was a stressful hour!! So, all in all it was a rough stay. We pushed lots of fluid through him and a full 24+ hours of steroids. They kept him calm and out of pain. When we got home he was SO very exhausted!! He has layed around and slept a lot! Yesterday, he did start eating some. Thank you Poppy for the wonderful chicken spaghetti dinner. It was awesome!! As much as we are drained from the whole experience, we feel relieved because finally, FINALLY
we feel like we have a team who is looking at Lydon and recognizing his issues and doing their best to keep him safe, protected and well taken care of. Hopefully, this whole event changed the course for us and we will not have to fight for him anesthesia wise again. God is so good!

The Good, The bad and The ugly...

Im going to start with the ugly becaue that is how i feel. Just ugly or really I guess mad but being mad is ugly and I dont like it at all. Maybe I'll feel better after I post this update. I am all kinds of mad right now. I am angry mad, sad mad, hurt mad, upset mad, the list could go on and on. Let me explain.... On Friday, I stayed home to allow my arm another day to recover from the surgery I had done last week for a compressed medial nerve. Thats a story for another day. My nurse came in about 7:30 that morning to get Lydon out of bed and she found his entire GJ tube completely pulled out and laying in the crib! This did not make me mad. So we started scrambling to make phone calls and try to make arrangements to get to the hospital to get a new tube placed before the weekend while Mommy was home and able to take care of it. That did not happen of course but this did not make me mad. We talked to the nurse and everyone else who needed to be informed and consulted and finally put him on the surgery schedule for Wed. In the midst of all this trying to come up with the quickest easiest plan to get Lydon taken care of is what upset me and led to me being mad. See, for the last year, every single time we have gone into the hospital for any type of procedure we have ended up admitted usually in the IMU unit because of respiratory distress or acidosis. So, the idea was brought up about replacing Lydons tube in Interventional Radiology instead of the OR with traditional anesthesia. This is where I got mad. I am mad because in a city as big as Houston and all of the WWW, I feel like I am doing all of this and making decisions like this alone. To clarify, I do not mean that in the truest sense of the word. I have my wonderful husband and my family and friends who often send us uplifting messages and words of encouragement and listen to countless conversations but it angers me that I feel alone with this diagnosis and everything that goes along with it. In the beginning, I felt like maybe having a close knit group of moms was the silver lining in having to deal with such complicated medical issues but now, I feel isolated from the mito community and I’m really not sure why? Maybe it was because I sought a second opinion? I dunno… I went to who I was referred to…we went through months of testing and we were then politely dismissed from that office. I sought a second opinion just like I was told to but somehow it seems like that is when things changed. Thats what so weird to me. I didn’t want for Lydon to have these problems. I’m not able to “wish” his stomach into working or not working. I’m not the one who makes him react to the anesthesia he receives. I am the one who holds his little body and feel the tremors during the night and sees the glassy look in his eye. I am the one who cleans up vomit everyday and sees when he is too exhausted to play. I am the one who gives the slew of meds several times a day. I didn’t look for somebody to label him anything in particular. I just wanted somebody to recognize his issues and connect the dots. That is what happened when we found/saw Dr. Frye. Now he has received an incredible opportunity at Arkansas Children’s Hospital. This makes me very sad for us and what we may have to go through all over again to establish ourselves with another neurologist here in Houston but I am sure he will be such a blessing to the people of Arkansas. So, that’s the ugly. I was mad because all I wanted to do Friday afternoon was call or text or email an experienced mom who lives with all of the daily issues that we do and get some advice about the Interventional Radiology route. I’ve never faced these issues as a mom before. So, here’s the bad. I’m sure you may have already figured this out but “the bad” is that Lydons tube is out and we will be having surgery on Wed. We have to be there at 6 am in the morning for the tube replacement. The good news is that Jono will be with me and our wonderful pediatrician is on service in the hospital if we do indeed end up admitted. The other good thing is that we have an incredible family, some incredible friends and a great group of doctors with one of the best hospitals in the country. And above all…we serve an awesome God who meets our every need. Say a prayer for us in the morning. I will update when it’s all over.

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.

The missing piece of the puzzle

We have found our missing link! Literally. On Thursday, we met with the Genetics team at UT. As some of you may remember, we did some genetic testing per Dr.K's request back in December. When Lydons results came back we then had our blood tested. So, in the last week or so all of the results have come in. The "team" of doctors and nurses came in armed with toys and a box of Kleenex. I was so impressed! Two and half hours later, I realized both of those items benefited them as well as us. Once again, thank goodness for my wonderful Mother. She was able to get off and go with me in Jonathans place. I couldnt have made it through this appointment without her. The appointment was obviously very involved and included a lot of detail to say the least. I wont relay everthing here that they relayed to us since blogspot doesnt allow for the Magna Carta but I will try and relay this all to you as simply as I can. The simple answer is that Lydons chromosome panel showed a missing portion of Chromosome 16. Neither Jonathan nor I share that same loss. So, this is a mutation/deletion that is specific to Lydon and is the underlying cause of all of his problems. This deletion is universally associated with growth delay, developemental delay, speech/language delay and seizure disorders. At this point in the visit, Im a little confused. Lydons growth, developmental and speech delays although they have been a great concern, they have certainly not been our biggest concerns. What about his on going significant GI issues or his extreme fatigue and energy deficit? The list could go on and on. Then, they explained to me that the chromosome is the place where the genes "live". The genes are what tell us certain detail specific information. So, the missing piece of Lydons 16th Chromosome is the "house" for 7 different genes. They dont know what all seven of the genes are responsible for however, one of the genes specifically is a nuclear encoded gene for mitochondrial disease. And that would explain all of his other medical issues and what we have been thinking for months now. That there is indeed a mitochondrial dysfunction. Now, for the curve ball because in case you forgot this is Lydon we are talking about. They have only been able to identify disorders/diseases with this area of Chromosome 16 for the last five years. So, the technology isnt available at this time to identify the specifics of this gene and exactly what mitochondrial disease or dysfunction it causes. So, we know now what is behind Lydons problems and where it all stems from (This was a genetic "glich" that just happened. It was not passed on from either of us and he didnt develop this after he was born.) but we still do not know exactly what this is or what this means for Lydons future. We know that for a while at least, he will continue to have a delay in growth, developement and speech but we do not know to what extent that will be. We are very anxious to meet with the mitochondrial disease doctor again next week and she what she has to say in regards to this gene discovery and the role it would play in Lydons medical/mitochondrial issues. So, all in all, it was a lot to take in but we are indeed narrowing things down and getting some answers and now we do at least have a primary diagnosis. Lydon has a Chromosome 16p deletion. Im sure we will have some more information to add to this after we see the mito doctor. The genetics team are looking in to research studies across the country which may already be in progress to examine to possibilities of getting all of the specifics of this gene broken down and identified. If they do not find one, they will work to get a research study started here at UT and Lydon will be the subject. The head genetics doc told Lydon very sweetly while she examined him, "You are a little mystery man and you're gonna help us write the book on this one." I felt like that was a bit of a confirmation to what I've been saying for months...Lydon is special and God has had a plan and specific purpose for him all along.

EEG and Sleep Study

I dont have any results yet but I thought Id post a couple of pics from Lydons EEG and sleep study. It was miserable for him. The sleep tech was very knowledgable and compassionate but that didnt change Lydon's mind about how irritated he was.

He hated all of the leads (more than 25). Like I said, I dont have any results but the machine kept going off while he slept( just blinking not buzzing) indicating his CO2 level was too high. Today, we saw the Immunologist, Dr. P. Love her btw, so tender and gentle. Lydon could sense her sweet spirit I guess because he let her hold him the whole time until she started to walk out of the room with him. ;) So, I mentioned the CO2 to her. It was her opinion that his CO2 level was a little on the high side so Im assuming that he is indeed having oxygen desaturations while he sleeps. Guess we will see what Dr. J (the pulmonologist and sleep specialist) has to say about it all on Monday.

Tomorrow, we meet with Genetics about some of our previous blood work that has come back. Not too sure what exactly we will hear but its all part of the process I guess. Say a prayer for us as we continue on this journey of finding out exactly what is behind all of Lydons problems and what we can do to help him feel the best and be the best that he can be.

Update

Ok so...its been a while since I updated and Im a little a loss as to even what exactly I should say. Lydon is being Lydon...complicated but then again, I guess that's just normal. We received a call from the hypertension clinic last week. The nurse told me that she had gotten all of Lydons results in and they would like to move my appointment up a week. So we went in on Thursday. Dr. S came into the room and told me that he was a little dumbfounded. He said he had a whole spill of things ready to discuss with me such as what blood pressure medications were appropriate and which ones had which side effects. His plan was to discuss this and send Lydon home on bp meds. But...Lydon being himself threw us a curveball and gave us a bp that was the lowest we have ever seen it. It was still a little on the high side but nothing like what we have been seeing. So, Dr. S said that he was a little uneasy started him on a new medicine if his blood pressure is going to fluctuate or start to even out. Guess we'll see... We are starting to lean a little more towards the fluctuating issue because we have started seeing him exhibit HUGE mood swings for no apparent reason whatsoever! The other thing Dr. S is worried about is that the renal scan showed that his kidneys were smaller than they should be. He is wondering if there is a narrowing within his renal vessels that is the underlying cause of the high bp. He said this could likely be caused by his small kidneys. We are struggling with our therapy appointments. Our Physical therapist doesnt feel like she can proceed with any PT plans until we are sure about what is going on with his bp. We are having lots of trouble with Speech. It just seems like we arent getting anywhere speech wise. He says a word one time but then doesnt repeat it or use it again. Today he did terrible during therapy. He did nothing but cry, scream and grab his blankie to go back to bed after only being awake for about 20 minutes. She (the speech therpist) talked to me about something called Apraxia and possibly developing a more detailed motor developement plan(whatever that means). He is still running his low grade fevers like he has been for weeks now. We have an appointment with Immunology on Wednesday. Genetics on Thursday. Our sleep study is scheduled tonight. I will update later in the week with more info.

On a more urgent note, please keep the family of Cooper Knight in your prayers. His lost his courageous battle with Mitochondrial disease and his family are saying their final goodbyes today and tomorrow. Even though I didnt know this family personally my heart breaks for them like family. Godspeed Little Man.

Echo and Renal scans

Tomorrow (Tuesday) we will be having renal scans,xrays and the echo of Lydons heart done. Please say a prayer that we can get this done without sedation. I know it will be hard on Ly but being sedated is even harder for him. I will post an update asap. Also, please pray that we can get his sleep study done this week as well. We had to cancel it twice last week due to all of us being sick. And most importantly please pray for two of our mito friends. The Agnew family and Cooper Knights family. Both of these sweethearts are in the very last stages of mito and aren't expected to live more than another day or two. I can't imagine the pain that these families are facing. They need strength and uplifting. Thanks for the love and prayers being said for all of us.

Enough Already!

The title pretty much says it. Ive had enough of this already! But, Gods timing and HIS plan obviously is different at this point than we wish it was so we will continue to trust him and move forward. We saw the Nephrologist/Hypertension doc on Thursday. We arrived there about 2:20 and didnt leave until after 5:00 p.m. By the time we walked out, Lydon was exhausted and I was frazzled! Of course we spent a lot of time on Ly's history and physical exam. I think I lost count of how many blood pressures we took. Dr. S said that he was very concerned about Lydons blood pressure. He said that looking at it over the last 6 months it has slowly trended higher and higher. He is also concerned about what the underlying cause is for the high bp. In children, it is most commonly caused by some type of kidney issue either a constriction within the renal vessels or possibly a kidney disease. Second most common is the heart. The wall of Lydon's heart could have become thickened from the stress that his little body has been under. He told me that in theory, either could be the cause for Lydons issue but he was a little bit more concerned about the kidney aspect of it all because Lydon has had a high aldosterone level in the past. Aldosterone is a hormone that is involved in the absorption of sodium and secretion of potassium. It increases blood volume and can thus increase blood pressure. So we now have to do an echocardiogram, bloodwork and scans and xrays of his kidneys. I tried to get all of that scheduled for next week sometime and was told that it could only be done in the afternoon and that he had to be NPO(without anything by mouth/tube) for 6 hours!! 6 HOURS!! Needless to say, that will never work. Lydon does pretty good maintaining his blood sugar but metabolically there is no way I would take that chance with him. So, I started making calls to be granted the "exception to the rule" card and was not very succesfull. I did talk to a couple of nurses including the mito clinic nurse who all agreed with me but nobody I talked to seemed to have the power to grant any exceptions. So, after our appointment with GI tomorrow and trip to the lab and our sleep study/EEG tuesday night I guess I will try again to get in touch with somebody who does have the power to make exceptions. Otherwise, I guess we will have to be admitted in order to get this done. Once all of this is done we will go back to Dr. S and come up with a plan of how we are going to best treat this. If Lydons pressure remains where it has been, there is no doubt we will have to put him on medicine. If it stabilizes a little bit we may be able to wait until after he is two and look at things again in the summer. GI issues...heart?....kidneys? You know, Ive expected for things to frequently come up with Lydon but heart, kidneys, stomach, intestines....REALLY?! Couldnt it just be an ingrown toenail? A cavity maybe? Two cavities even.. I'm not too proud to say that I have bargained some with God. Ive offered Him all of the above options as alternatives to the heart or kidney issue. I'm also not too proud to say that He hasnt given me any answers. But, He has given us some promises. He promised that He would never put more on us than we could handle and He promised He'd never leave or forsake us. "The LORD himself goes before you and will be with you; he will never leave you nor forsake you. Do not be afraid; do not be discouraged.”
Deuteronomy 31:7-9 I am hanging on to this scripture and frequently reminding myself that HE has it all under control. If these tests do show an issue with Lydons heart or kidneys then obviously it is something that we need to know and be aware of. Thank you to all of those who have continued to check on us, pray for us and send an encouraging email, text, phone call or visit. It really means a lot and keeps us going on days like last Thursday. I will update again after the appointments we have this week. Please keep praying for us and Lydon. Please pray specifically for our current nursing care situation. It is really being a difficult adjustment for me. I do need some help/relief with Lydon but it is being very hard for me to accept having somebody else here in my home and not doing everything for him myself. I need help with this. Much love to all!

Appointment Update

As promised, here's the update from our appointments this past week. On Monday, we saw the specialty pediatrician. I was looking forward to this appointment for quite some time. Up until now, we have not had a managing physician for Lydon. The pediatrician we were seeing has been wonderful to us but Lydon's continuing issues and need to maintain continuity of care has forced us to find someone who specializes in children with special needs. We were there for almost three hours. We discussed lots of things from the general to the detail-specific. First, we went over all of Lydons history and physical exam with one of the upper level interns. Then we met with the social worker and discussed some managment issues with his daily care like his therapies, equipment needs etc. She also asked me about what and if Jonathan and I had talked about what we would or wouldn't want done for Lydon in an emergency situation. Not your typical "well child" check up conversation! Then the doctor came in. He said that since he had gained a little length and weight(he's up to the 8th percentile) that he didnt feel it was necessary for us to see Endocrinology. Good news! One less specialist on the list. At least that's what I was thinking; but instead, we traded Endocrinology for Nephrology. The doc said that he had looked at Lydons vital signs past and present. He said he felt like it was safe to say that Ly is hypertensive and needs to be put on blood pressure meds. So, we have now been referred to a Nephrologist since kidney docs are the ones who take care of blood pressure issues in kiddos. Ive known for some time now that Lydons blood pressure was high. Ive mentioned it to several different docs before. Regardless, it has been overlooked or ignored. Im glad that it has finally caught the attention of the right person and is going to be treated. It is still upsetting to me that there is yet another problem and probably at least one new medicine to say nothing of the continued follow up exams and testing this diagnoses will bring us. He also talked to me about the issues with Lydons Igg(Immune system indicator). He had his nurse make some calls and got us in to see the Immunologist in about three weeks instead of three months.
We left there and walked over to the hospital to have some xrays done of Lydons tummy and see if it showed us what was going on there. The next morning we had a GI appt. I was very anxious about this appointment; because, in my opinion, Lydon has not been doing good GI wise. I'm not sure what happened to our GI doc since the last time we saw him. Brain aneurysm? Bi-polar event? I dont know? What I do know is that after our visit, it was clear that his recommendations were not what was right for Lydon. This type of issue/confrontation is always hard for me but the more it happens, the easier it becomes. Lydon is my priority...period! After all was said and done, we have an appointment to see a different physician who is familiar with Lydon and has a different approach. I am believing that this is going to be right person for us long term. Adding a new physician is one thing but I HATE having to change physicians! So, we will see the nephrologist about his blood pressure this coming Thursday, the new pedi next Monday for a follow up, then the new GI the next Monday. Our list of specialists keeps growing... it includes a Speciality Pedi,ENT, Gastroenterologist, Immunologist, Nephrologist, Neurologist, Pulmonologist/Sleep Specialist, a speech therapist, an occupational therapist and a physical therapist that all come at least once a week and we're a little late but we need to add a dentist and Opthamologist to that and probably some other "ologist" that Ive forgotten. Immunology, the Sleep specialist and Neurology/Mito Specialist appointments are all scheduled for March. Its a busy schedule thats for sure! I use to hope that we could find answers for things. Now, I dont know how much more I really want to know. I just hope that we can do whatever needs to be done in order to keep Lydon as strong, healthy and stable as possible. So, with that being said, we will continue doing what we are doing and trust that we are walking down the path that God has intended. As many of you know, we were hoping for a "snow storm" here in the Houston area today and that didnt happen but we made the best of it and did what we could to keep everybody happy and warm. Heres a pic of how the boys started off their day.

Who Knows??

I've had so many things on my mind the last several days that I havent been able to really put my thoughts together and put it all into words but Im gonna take a stab at it. Lately it just seems that we are swinging from one issue to the next. Everything in my life lately seems "out of sorts" and yet I still feel that I am so blessed. To shed just a little bit of light and explain things... Hollie is our 13 year old. 13 yr old female..enough said! She is beautiful and smart. She is sassy and opinionated. She is mature and responsible while being immature and irresponsible at the same time. Bipolar=my definition so far of the teenage years!! Aidan is our five year old. 5 yr old boy...again, enough said. He is sweet, loving and an incredibly funny precrocious little boy. Oh did I mention hes amazingly beautiful too? (Dont tell his Daddy I said that!) But...he is messy. Full of messes. Dramatic and full of energy. So much energy that even if I had the time to be working out again right now, I dont know where I would pull the energy from. He can literally climb my wallls! And then, theres Lydon...whose middle name is "complicated". I dont know what his middle name really means but they should add complicated to the list. He has so many issues and it seems that once we think we've solved one, another one creeps up. He is having severe GI issues. His tube is backing up. It really seems to hurt him. He is vomitting off and on and even occasionally has milk in his tummy after being fed through his intestines all night long. Your intestines should drain down, not up into your tummy! On the other hand, he seems to handle his fluids during the day and he certainly is enjoying his cheerios, crackers and occasionally even bites of spaghetti or oatmeal. So what do we do? Take away his daytime eating? Add more medicine? Drain his tummy when he gets too full? That is all up in the air. We will be doing xrays and seeing the GI doc on Tues. He is also struggling with "the crud" from all of this weather change. Again what to do? Keep him inside all the time or let him go out and experience the beauty of the sunshine and its warmth or the crispness of the cold winter wind? When he was seen in the ER a couple of weeks ago, they did a lot of bloodwork. One thing was to test his immune system. That test came back looking pretty low. So, we will be seeing an immunologist now to discuss this new finding. I spoke with the nurse from the mito disease doctors office the other day. Some of his bloodwork from their office came back. It showed an abnormal loss of a chromosome. What you might ask does that mean? Well, her response to me was it could mean nothing or it could mean everything. Who knows? Jonathan and I will have our blood drawn this next week to see if there is any correlation. If there is, then in theory it means nothing for Lydon. If neither of us have the loss then only Lydon has it and it could be the underlying cause of his problems. We now have Lydons medicaid and MDCP all approved which is great! But, that also means working out nursing so that it best fits our family and benefits Lydon. I hope this all doesnt sound negative. I do "see the silver lining" in things. I have a beautiful teenage girl who is more than capable of helping me out with things. She gets herself up every morning for school. Makes nothing but straight A's in all Honors classes and does her own laundry. Aidan, thank goodness, is all boy! He seems happy, healthy and very secure despite being tossed around from place to place on occasion. Lydon also has a lot of good days. I am thankful he is walking and able to enjoy a cheerio or a cracker. He isnt really talking to us but he certainly lets us know what he wants or doesnt like. All very normal behavior for a 21 month old. So, I dont really expect for any of this to make sense to anybody else. This is where "its my blog" logic comes in. I am really just putting my thoughts down in order to try and get them out of my head for a moment. That is until the next cup of chocolate milk is spilled! Thanks for all of those who continue to pray for us. I know that is what is getting us through. I will post an update after our appointments next week.