I keep forgetting the BIG NEWS.... Alex has NO pacis anymore!

Ali

The weekend days are LONG in the hospital! Things are trekking along. At this point all signs of infection are gone. His feeds are up to 15 cc/hr. (1/2 oz) He is having quite a bit of gas and bloating and some stomach pain, but the doctors are having us try to push through. His TPN is also still running. It is a really delicate balance between his metabolic labs and trying to get the right nutrition in him and keeping him comfortable. It is like walking a tightrope.

I will continue to update.

Ali

Sorry I was in the internet dead zone from hell! I moved rooms and have asignal. I am so happy to be able to be back online.

Alex had a much better day yesterday and that has continued into today. We are having severe temp drops overnight. We are having to warm him with hot blankets. Other then that we had one lab that was pretty bad this morning, but was better when we repeated it.

Still looking good for late next week. Fingers crossed!

Ali

As I mentioned yesterday, the signal stinks so bear with me. Yesterday was a rough day, Alex was extremely weak and lethargic. He never got out of the bed all day. He is much better today. We have already been to the playroom and are happy. Not much news just trying to get our tummy going.

I'll let you know when I hear something.T​hey are telling me best case scenario is coming home late next week.

Ali

My internet was acting crazy last night so I didn’t get to update. All in all yesterday was a pretty quiet day. Dr. Koenig and LaKeesha came by in the morning for a while. I don’t have nearly as many questions now. It is more like friends coming for a visit. She asked me about doing some media stuff for her. I told her I’ll talk to anyone as we all know!!!! Basically at this point we are just watching Buggy pretty closely and praying his belly will start working. Blood pressure was EXTREMELY low around 4:30 yesterday afternoon and then we took his blood sugar and that was 48 which is also really low. I always joke with the doctors and nurses that he doesn’t lime to give us a moment of peace. We bumped his dextrose until his TPN was ready and he was fine. He has done great so far on his TPN. It needs a lot of tweaking. They have been really cautious because of his history of reactions. Right now his TPN has No vitamins. We will add those today. WE can also add an IV form of his reflux med right into his TPN. The second part is his lipids or fats. They were extremely concerned because those are egg based and Alex is allergic to eggs. You “shouldn’t” react because it is purified and all that, but you never know, however Alex has done great. But in typical Alex fashion we couldn‘t go 24 hours without some excitement. Things were going great until 4 am. Alex’s blood pressure was 68/21 and then 65/24. His blood sugar was okay. His rectal temp is 95.3. We just gave him additional IV fluids and his blood pressure has come up some, but despite being wrapped in warmed blankets his body temp is not coming up. We are not sure what the problem is. They are running more blood cultures this morning. They have seen some mito kids have their temperature​s drop when they have an infection instead of rise. Their fear all along because Alex broke out in spots for so long is that the infection is in his body just sitting somewhere. Once I see Dr. Koenig I will update again.



We changed rooms late this evening. We really liked our room, but they had a room open 2 doors down from Jen and Landon so we moved over here so Jen and I could be closer for support but still keep a close eye on the kids.



I had a good day. I spent about thirty minutes this morning with two of the child life girls chatting and laughing while Alex played in the playroom. Then this afternoon I spent about an hour with three more girls chatting and having a good much needed cry. So Kelly, Laura, Stacey, Ginger and Leigh Ann THANK YOU!!!!!!!! You guys make this so much more bearable and enjoyable for both me and Alex I am so blessed to call you girls my friends. And as much as we dread coming down here we are happy to see you too. For those who don’t know what I’m talking about. Alex said 2 things when I told him we had to come back. One, W had to come to Houston “because Dr. Koenig missed him SO much” and two “ those child life girls are going to be so happy to see us”.



Anyway, Please keep us in yours prayers. The internet is really finicky so bear with me.

Ali

I SLEPT!!!!

The first thing I need to say is that we are so very fortunate to be surrounded by so much love and support. Our family and friends continue to rally around us and show their love in so many ways.

We are truly blessed to have found a Dr. like Dr. Koenig. She is one of a kind. She is a kind, compassiona​te woman who has complete trust in the parents of her patients and who also happens to be one hell of a doctor. She is able to explain so many things thatno one has been able to explain for four years. It is so reassuring to me when she can help me understand why a lab is off or why Alex's body responds in a certain way. when it makes sense it is just plain easier to take.

We are starting Alex's TPN tomorrow. It is much needed. Alex was down to 27.5 lbs tonight. I was appalled to see a 27 on the scale. We have worked so very hard for every hard earned pound and they fall off so easily. It will take us a long time to get those same pounds back on. We will be here at least into next week. Dr. Koenig does NOT want us to come home on TPN. TPN can be very damaging to the liver and kidneys. It is not meant to be used long term. They will be checking Alex's labs daily and making sure he is doing okay. Depending on how quickly Alex's belly responds to accepting feeds will determine how long we are here.

We also saw Infectious disease and dermatology. Both agree if we get any more spots we will biopsy them.

GI wants us to do one more test to sdetermine what we will do about the reflux surgery.

I think that is most of it for today. Alex was pretty quiet and lethargic today. He wanted to stay in bed other then the two walks in his stroller we took. Hopefully he will feel better once he starts getting some nutrition.

M​ore tomorrow...​
Ali

This is purely a vent and nothing more...

Dr. Koenig put Alex on the IMU to watch him more closely and so Jen and I could be more support to each other since we are both here by ourselves. I forgot how much I hate this unit! As bad as it is on the regulas floor with them coming in every few hours for vital signs and meds. On the IMU, in addition to that Alex is hooked up to continuous heartrate, oxygen saturation and resperation monitors. Well, Alex's "norms" are not normal so the monitors beep every ten minutes all night long. Really conducive to sleep huh? Can we go home yet?

Here's what I know so far...We will see GI, Infectious Disease, Immunology, Dermatology​, and of couse Dr. Koenig. Some of Alex's bloodwork has already come back off. We have 2 separate issues that we dealing with. The spots which are some sort of infection (we are not convinced it is folliculiti​s at this point). And the fact that Alex cannot tolerate his feeds. We have to get nutrition into Alex. The most likely plan is to start the TPN or IV nutrition tommorrow. If that happens we will be here at least through the weekend. I have a computer with me so I will keep you updated.

Buggy is in good spirits and feels very much at home here. Please pray that he does not have to endure any painful testing. I am doing okay. I am just really tires from all the travelling and lack of sleep the last 6 weeks. Please pray for my body to hold up and for my mind to quiet at night so I can rest and recharge. The hardest part for both Alex and I is the boredom that sets in after a day or two. The nurses and child life girls try to keep us entertained​, but we're a hard sell! The days are long!

Ali

We are safely in Houston! I will update tomorrow after talking with all the doctors.

Ali

We are off again! Dr. Koenig emailed today and in her words "that boy can't live on sugar water". We are heading back to Houston in the morning. They are going to put him on TPN or IV nutrition for a few days and get a few pounds back on him and run some tests to try and figure out what is going on. Alex still doesn't feel bad. His belly just won't work.

Please keep us in your prayers.

Ali

We are still kind of in the same place. Alex is not doing great, but we are hanging in there. Dr. Koenig is back in town this weekend so we will touch base and see where we go from here. Alex has been on IV fluids since Wednesday. We are going to attempt some VERY slow half strength feeds this morning. He has been such a trooper. It has been a hard week for both of us. He has been tethered to an IV pole. I am sleep deprived. Having to give meds around the clock. And the house feels like a hospital ward. We have been able to fool ourselves into believing Alex really isn't very sick, but it isn't very easy to do that this week. As always, my buggy has handled himself with more grace then anyone I know. Always a smile on his face, whether I am coming to stick him with a needle for the tenth time that day or wake him up for the fifth. I am so very proud of him.

Ali

We are happy to still be home! I am so thankful for Alex's port. We have switched Alex to Iv fluids and stopped is feeds once again. Immediately his temperature and blood pressure returned to normal. This just verifies what we were thinking...​Alex's stomach is not working right now. We think because he is still fighting the infection he picked up in NC. With mito it all comes down to energy. Alex's body doesn't have enough energy to fight the infection and digest any food so his body is having to work too hard thus increasing his blood pressure and temperature. We will wait a few days and try again.

Thank​s for all the prayers.

Ali

This child is going to be the death of me! He finally had a poop last night...wat​ery diarrhea again. And all of his vital signs have been off for 2 days. I am in contact with Dr. Koenig's nurse. Dr. K us out of the country until the weekend. I am just praying we don't have to go back down to Houston.

I'l​l keep you posted,

Ali

We are safely home. Alex is happy to be back and playing with his toys. I am exhausted. I barely moved off the couch all day yesterday. For those of you who have never been in the hospital let me explain what it is like. This is how our last night played out (pretty typical):

10 pm check blood sugar

12 am check vitals

2 am give a dose of benadryl

3 am check blood sugar

4 am check vital signs

5 am start antibiotic

6​:30 am first Dr. comes in.

So as you can see sleeping is nearly impossible. As soon as you start to dose off you are woken up again. I slept like a log the last 2 nights. Anyway, I will update more about our trip in the next few days, but wanted to let everyone know we are home.

Ali

We are waiting for the Doctors to come discharge us, but in typical Alex fashion we had to have a little excitement first! Alex had a pretty bad seizure last night. Luckily the nurse was in there with me. I had the oncall doctors call Dr. Koenig. She is okay with us going home. She is giving us medication in case it happens again. We will come back in September for further testing.

Can'​t wait to see and talk to everyone.

Al​i

Woohoo! We are coming home in the morning. Alex's belly is doing pretty well. There is still sme concern that he is getting new spots. We are going to come home and finish our course of antibiotics there with the knowledge that if we get more after we finish the meds we will have to come back.

Other good news is that his heart still looks good. They saw some kind of regurgitati​on in the heart, but they are not worried.

Ali

We are doing better! We are anticipatin​g being discharged tomorrow. Alex's belly is SLOWLY coming around. We are up to a little less then an ounce an hour of half strength formula.

As of yesterday, Dr. Pacheco did not want to do IVIG infusion this illness. Alex got a few new spots yesterday which is concerning after 13 days of a strong IV antibiotic so we will see if that holds true today. The only other news from innumology is that he lost all of his titers or antibodies to his pneumoccal vaccine for a second time. This is oncerning because it means we really do have immune problems.

We met with GI yesterday. As most of you know I have really struggled with the GI team here. I won't go into details yet b/c I am still figuring everything out, but the surgery is on hold for right now. In the meantime, we are doing some more testing to be sure that Alex's stomach and chest pain is definitely caused from GI issues and not something we are missing.

We had an EKG and an echocardiog​ram of his heart this morning. No results yet.

We are expecting to meet with Dr. Koenig, Dr. Pacheco (immunology​), and an Infectious disease doc today. Once that all happens and we get Alex's feeds going better we will be out of here.

Thanks for all the prayers and love. As always, Alex has been a real trooper.

Ali

So much for our "easy" trip to Houston this time! It looks like we could be setting up shop for a little while. Our plan yesterday was to start with sugar water through Alex's Gtube at a very slow rate and slowly bump up every 2-3 hours. We started at 11:15 yesterday and did well with our first 2 rates which were laughably slow (less then an ounce an hour). At 4:30 we went up to 50 cc/hr which is a about an ounce and a half an hour. At 7:30, Alex's cheeks were starting to look flushed and he felt warm, but his temp wasn't too high yet. At 8:30, he had spiked a fever and was complaining of his belly hurting. So we are back down to 20 cc/hour and waiting to hear what the next plan is.

We are waiting to see Alex;s immunologis​t. We saw her resident yesterday adn are pretty sure we are going to be doing an IVIG infusion. This is replacement immunoglobu​lin to try and help his immune system. Alex's immunoglobu​lins remain low and since he is having trouble fighting this infection it looks like this will be needed.

We are supposed to see both GI and Surgery today. They are trying to decide if we should just do the fundo (reflux surgery) or if he will also need another procedure at the same time called a pyloroplast​y. That helps things move faster from stomach to intestines. We are hoping to schedule the surgery sometime in September.

I​n good news...Alex feels pretty good. he is charming all the nurses and doctors. He loves going to the playroom and seeing his girls from child life. Child life (responsibl​e for making hospital visits as easy as possible) fell in love with Alex our first visit here in February and have been wonderful to us ever since. They are so sweet to Alex and bring him everything he wants. We are also happy to have Jen and Landon in the hospital with us. Landon had his muscle biopsy the same day as Alex and Jen and I have been friendly ever since. Landon has the same rare complex III mito disorder. Right now the boys are not allowed to see each other because Dr. K doesn't want them to get each other sick. There are a LOT of similaritie​s between the boys although Landon is sicker then Alex. Jen and I have been sneaking downstairs to get coffee, lunch and dinner together every day.

Jen and another friend Melody brought me take out and cupcakes last night for my birthday. It was fun to sit "at the park" in the hospital and have real food and chat with friends. Aunt JennJenn, Uncle Jimmy and Skye Skye sent me and Alex balloons. Child life brought me a balloon, card and flowers. So I got to at least have a little "celebration​" on my birthday. THANK YOU GIRLS FOR MAKING MY BIRTHDAY SPECIAL!

We had a good appointment with Dr. Koenig. Alex loves all his doctors, but asks all day for Dr. Koenig. She "tried" to explain some of the genetics to me. Genetics is the most confusing stuff ever so I won't even try and explain what I got of that. She is a little concerned about Alex's amount of sleep increasing so much over the last few months. When we come back in October we are going to do a sleep study. But we think it is just progression of the disease. We will also see pulmonology and Cardiology in October for routine yearly checkups. (We are NOT concerned about heart or lungs). The "blinking episodes" we were seeing she thinks are seizures, but they have subsided for now and were not the type to cause any damage so we will keep an eye on it, but are not planning on treating. That was pretty much all of the highlights.​
Once I see our ped team and Dr. Koenig this morning I will try and update and let you know where we stand.

To all of those that called....I am so sorry i didn't get to call you all back. All of the calls and emails are appreciated.​
Love,

Ali

The fun continues... We tried to start Alex's feeds this morning at half strength and a very slow rate. He got a fever not long after so we had to stop feeds. We are going to stay on IV fluids tonight and try again tomorrow.

The pediatricia​n's are pretty sure that his spots are folliculiti​s. We are starting 5 more days of IV antibiotics to try and kick it.

We are off isolation and allowed out of our room so that was great news. Also, Dr. koenig said that Alex's EEG was normal so his brain still looks good. YAY!!!!

Dr. Koenig will be by tomorrow morning to sit with us for a n hour or so and go over all my questions/t​est results etc. We are definitely doing the fundo at this point. Alex is in a lot of pain and is pointing to the center of his chest every time we ask where it hurts. We are hoping to see surgery tomorrow and do the surgery some time next month.

More tomorrow...​
Ali

Alex was admitted to the hospital in houston last night. We think he has a virus. He has diarhea, stomach pain and a sore throat. He was not tolerating his feeds so his doctors were concerned. He is on IV fluids and we will monitor him today. In the meantime, Alex is breaking out in spots again. So we are also trying to decide what to do about that.

We did do Alex's EEG yesterday. I have not discussed it with Dr. Koenig yet, but the tech told me they saw no seizure activity, but some abnormal brain spikes. I am not sure what it means at this point. We are expecting to see Dr. koenig and an infectious disease doctor today.

The hardest part right now is that Alex doesn't feel that bad so it is hard to be cooped up in the hospital. And we aren't even allowed to hang out with Landon or go to the playroom because we could be contagious. Jen and I did have lunch and dinner together yesterday so that was a nice treat.

I will update later with more news.

Ali

We are in and out...Our flight last night was delayed. We finally walked in the house at 1:00 AM. After a long day of bill paying, laundry and packing we are ready to head off again. We leave at 6:30 tomorrow morning for Houston. We have a full week of appts. I will attempt to update from the hotel, but it is hard without a computer.

Ali

Just wanted to let everyone know that we are doing well. Alex has done great on the antibiotic ever since adding IV benadryl. As some of you know, Alex is allergic to dextrose. When I looked at the bags of medication they were mixed with benadryl so we are pretreating with the benadryl and he is fine. It is nice to know we finally have an antibiotic we can use. We are all feeling better and our spots are FINALLY starting to fade. We are just trying to enjoy the last few days of vacation before we are back to real life.

Ali