Update

Quick update Lydons j tube has migrated back into his tummy. Weve been here since yesterday trying to figure out a plan. We at this point have not been able to get access. The plan is to put in a line and then replace his tube sometime this morning. Hopefully that will happen. We are of course exhausted but hoping and praying for the best.

Honest Emotions

There's really not a lot to say.... Nothing new to update with Lydon. He has done well since starting on the beta blocker. He had a good weekend spending some time with my mom and his "Paw-paws". As long as he is watching movies, playing with one of our phones, reading books, or playing calmly in the house all is well but as soon as he exerts any activity the symptoms reappear. Tonight after I got home, the weather was great. Not hot, not cold, not windy so I took him out in the garage hoping he could play for a little bit. He did play but after just a few minutes he started looking dusky around his mouth and the corners of his lips were blue. I let him keep playing on his car and horse until he got short of breath and then we came inside. I heard from the ENT scheduler today and it looks like Ly's procedure is a go for the first week of April. Unless something changes, our ENT's partner is going to look at Lydon's airway and then when he is done, they will start the cardiac procedure. I am trying really hard to stay upbeat and positive but it seems harder than ever. I hate the waiting and the unknown....I hate not knowing what to expect and whether or not this is going to fix Lydon's issues. I hate not knowing if these newly diagnosed issues are even what's causing his problems or is it disease process and no amount of medicine or highly skilled surgeon in the world could fix it! I guess time will tell. We are holding on tight to the prayers of others and what strength and faith we have. Somedays it feels like there isn't any left but then we get a silly grin or a sweet smile and our empty tanks are suddenly refilled.

Ly-Ly's Heart

I've written and re written this post several times over the last couple of weeks. It seems a little ridiculous to say that we've had a lot going on with our boy. Ever since his cold and ear infection a couple of weeks ago, he has become increasingly more symptomatic with activity. We started noticing these episodes right before our Cardiology appointment back in January. But since then, we've watched his heart rate be very irregular...up, down and all around. Additionally, he has seemed to have very little endurance and become increasingly short of breath, pale and cyanotic-(blue lips or duskyness around the mouth). Over the weekend, we took him to a near by bounce house for one of our good friends little boys birthday. We allowed him to bounce for just a minute or two and then Hollie took him down the slide. Apparently, that was more than he could handle even with oxygen. He was extremely short of breath, he was super pale, his lips were almost purple and he started gagging and vomitted. Events like this have started happening several times a day. I called the Cardiology nurse and we saw the doctor Wednesday morning. He told us his Holter Monitor results showed that he is chronically tachycardic or has a constant high heart rate. He gave us an order to start him on Propranolol which is a beta blocker. Our hope is that this new medicine will take some of the stress off of his heart so that he can enjoy a little bit more activity without having to pay for it! Finally we talked about his PFO or the hole in his heart. Because he has recently become so symptomatic, and has so many different things going on, we decided the best thing was to schedule the procedure to potentially close his PFO. We've tenatively scheduled it for the first week of April but that may change because we are trying to coordinate this with ENT since they want to look at and reassess his airway again. So far, he's done good with the new medicine and hasn't experienced any side effects. Our hope is that the procedure along with the new meds will make a big difference with his everyday excercise intolerance and endurance level. Otherwise, we are faced with the possibility of this just being disease process. Obviously, this is NOT where we want to be. Our Cardio's advice was to "not get your hopes up" or "expect too much". We should know more about a definite date for his surgery after next week. For now, we are doing our best to remain optimistic.

Update

This will be short and to the point. We ended up in the ER and admitted last week. Lydon had a horrible ear infection that ended up perforated before we could get it treated despite our very best efforts. He was exhausted before the admission and still is. This started out as "just a cold" but it was draining to him. It wasn't easy for Mommy or Daddy either; we are so tired of seeing him being poked and proded over and over again for something as simple as "a cold". We are discussing our options for possibly getting some more permanent access. This is a huge decision and not one we want to make but we hate seeing the pain and suffering Lydon goes through with every admission. For now,we are home, he is still feeling bad and full of cold symptoms but hopefully he will continue to get better this week without another admission. We have an appointment with Orthopedics tomorrow morning for evaluation of his walking and balance issues. We have follow-up appointments with all of his specialists in February and March. Stay tuned!

Two little boys and an ER run.

Lydon started feeling bad on Sunday. He seemed to just be a little "under the weather". I wasn't too worried and enjoyed cuddling with him in the recliner several times throughout the day. I guess big brother was jealous because he came home Monday from school and pulled his own stunt! He was outside playing with all of the neighborhood kids. They had pulled out the entire transportation fleet from our garage. Aidan decided to play fireman. So he drove our "firetruck" aka a little tykes cozy coupe car and his little girlfriend from down the street was his "engine." So she was pushing him as he was "driving" down the sidewalk. All was well until the front wheel caught some uneven concrete and out went the fireman onto the concrete, flat on his face! I got ice packs and gave lots of mommy sugar (all the while still cooking dinner and coddling Lydon) but I quickly realized that wasn't working out too well when he suddenly got very very sleepy, barely responsive and vomitted all over the floor! Can you say SCARE-REEEEE?? So, off to the ER we went!! Lots of prayers went up and by the time they started talking about IV's and CT scans, he woke up and started talking to us. We laughed at the coincidence but we had seen him be so sick and lifeless up until that point that we were grateful for the improvement. His CT looked ok and by that point I had talked to my mom who informed me that Lydon was miserable. So we got the discharge papers and they told us they'd save a bed for us if we needed to bring Ly in. Luckily we made it through what was left of the rest of the night. The bad part was that I couldn't get an appointment for him to be seen anywhere Tuesday and I was pretty concerned since he had been sick since Sunday. Coughing a yucky cough, mixed with a little stridor. Lots of mucus and a runny nose. He's been very gaggy and has complained of an ear ache. So far, we have held our breath own here at home. Hopefully we can stay home and he can manage this on his own but that's the problem with this disease. When he is sick and fighting something even this "simple", his body is pulling energy from another organ system that needs it just as much. We can see that be evident with his decreased energy and increased tummy drainage. If we do ok through the night, we will go into the sick clinic and hopefully not the ER tomorrow. For now, we cross our fingers and say our prayers!

Cardiology Appointment

So, like I've mentioned before in a couple of my previous posts, our appointment with the special cardiologist was today. We have known for some time that Lydon had some dysautonomia. He has always had problems maintaining his body temperature. It fluctuates as does his blood pressure, heart rate , appetite, energy level etc. But lately, we've noticed that with temperature changes or excercise, Lydon becomes short of breath and his lips turn blue. This obviously is not normal for a two year old. It was a very involved appointment. We arrived about 8:45 and left at 2:00!!! We were physically (and emotionally) exhausted! While we were there, Lydon had an EKG and an Echo done. He was such a trooper!! Poor guy was so worn out and happy to get a bottle he layed perfectly still for the echo without any sedative. So although it was exhuasting, we may have gotten some key clues for some of Lydon's issues. His EKG showed some variables that are common to mitochondrial disease. The ECHO showed a PFO. A PFO or Patent Foramen Ovale is a hole between the left and right atria. Everyone has this while in the womb but it is suppose to close shortly after birth. About 25% of the time, this hole doesnt close but it generally doesnt cause any problems or symptoms at all. However, in mito patients the stress that this puts on the body is much more profound and obvious. There is also a risk of oxygenated blood mixing with deoxygenated blood. This process called left to right shunting also increases the risk of a stroke. So because of all of Lydon's symptoms and the new findings on these tests run today, we brought Lydon home with a Holter monitor. This will monitor the activity of his heart for the next 48 hours. After this is complete, we will follow up with the doc and come up with a treatment plan. At that point, we will decide whether or not we will close the PFO. Needless to say, this appointment, like many of them lately, was a little overwhelming. Sometimes ignorance is bliss, sometimes knowledge is power.

For those of you who know us well and are intereted, Jonathan's MRI is scheduled for Friday morning. We are hoping this doesnt reveal anything worse than what we already know and that we will have results by Monday so that we can proceed with getting a biopsy done asap. Please pray that all of this will work out this way. Thanks so much!

A brief update

Last week, Lydon's tummy decided to act up. I should've known better because it had already been almost 90 days since our last admission...he's still sort of rocking along in trimesters. Somebody please send him the memo his 3rd birthday is in April!! Any way, he would act good during day, eating drinking and taking his formula like normal but every night after the first couple of hours on his feeding pump, he would wake up vomiting. So after the third night, we ended up in the ER. We were concerned that his tube was out of place again or he could possibly have an obstruction. We got there and they brought us right back and started the work up. They did all of his labs and an x ray of his belly. We were there for almost six hours before they finally gave us the all clear and let us go up for the EEG we had scheduled. Getting all 21 leads glued to his head was worse than sitting in the ER for six hours if you can imagine that! But finally, we got it done. He relaxed but still didnt want to eat anything besides a bite of cracker here and there. He was so exhuasted from all of the fighting that he slept pretty good that night. What a blessing that was!! I was exhausted! He didnt eat more than a few morsels of a cheese cracker all day Saturday and Sunday but he was doing a lot better. Today, he had a great day! He ate a tone of food. I was happpy that he ate and got to play with Mandy (one of his nurses)today. He was happy too!I had only been home for about 45 minutes when as I was heating up food for Holli and Aidan when he gagged and covered my kitchen floor with vomit. So, here we go again...who knows?! Tomorrow we have an appt with a special cardiologst. Wish us luck! ~Jana~