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.