Just a quick update to let you know of a change!

We have stopped the erythomycin. That was the new motility medication which we knew could effect the mitochondri​a. Alex's labs were HORRIBLE this morning and one of the things that jumped out at us was his lactic acid. (Kids with mito are prone to lactic acidosis, so this is a lab that lets us know if metabolical​ly he is doing well). His Lactic acid was 2.9 up from .9 yesterday. We all assumed it was a lab error. We repeated it and it was 2.6. The high end of normal is 1.8. Dr. Koenig immediately made the call to stop the erythromyci​n. Other then that things are fine. We are scheduled for early Monday morning.

Ali

Today was a little bit better! Last night once again Alex was up until after 11:00. I was really at my wits end. After talking to Dr. Koenig and her nurse we made the decision to drop his feeds back to 10cc's an hour with the hope of keeping him more comfortable until surgery. We will continue on 10 cc's an hour until Sunday. On Sunday we will stop all feeds to allow his stomach to empty for surgery. Surgery will be Monday. Then Tuesday morning they will do a contrast study to be sure the tube is in place. Once we know the tube is in place we will start over at 5 cc's an hour and try to increase by 5 cc's a day until we reach our goal. We are unsure if we will be able to increase this quickly. In the past we have had a lot of trouble controlling Alex's ammonia levels while increasing feeds. If it keeps getting higher and higher we will have to stop and give him a break every other day. We are hoping not to have to do this as it will prolong our stay. Dr. Koenig will also be following some other labs pretty closely during this time.

Alex is getting a blood transfusion as I update. This also should help him to feel better. So far it is going well.

Other then that things should stay pretty much status quo until surgery.

Tha​nks for checking in with us!

Ali

Thanks for all the kind words and thoughts. My poor baby has really struggled through this last week and it breaks my heart. Luckily, there is an end in sight. We on scheduled to place a G/J tube on Monday. This is our last shot at a G/J tube. A surgeon will place it and stitch it in place. If it comes out we will have no choice, but to place the separate J tube.

In the meantime, Alex continues to need pain meds for his stomach pain. We did find a regimen last night that allowed him to sleep so that was a blessing.

I will continue to update as we know more.

Ali

We continue to trek on...We have been at 50% formula at 10 cc's an hour for 2 days now. Alex continues to struggle with stomach pain. Dr. Koenig and I have talked extensively the last few days and have agreed to keep trying until Monday. She has talked to all of the Gi specialists as well as the surgeon. If we are still having trouble tolerating feeds and/or need lots of pain medications come Monday we will surgically place the separate J tube.

Other then that we need a transfusion tomorrow. Our hemoglobin dropped faster then usual this time, but it is not unexpected with surgery and his central line infection.

W​e continue to pray for Alex to sleep as he has been struggling.​
Ali

Alex has remained at 25% formula at 10 cc's an hour through his G tube. He is struggling with stomach pain and cannot sleep.

I have had a few people ask so I am going to clarify....​For the past 8 months Alex has had a G-J tube that we fed through the J portion directly into his intestines. he was doing wonderfully until he got pancreatiti​s. The only problem we experienced was keeping the tube in place. Every time we placed one the J portion would coil back up into his stomach within 2 weeks. The decision was made at that point that once Alex's pancreatic enzymes were down we would sugically place a "separate" J tube so Alex would have 2 buttons. When we came in for this admission it was to place that tube. When the surgeons, GI doctors and Dr. Koenig met the surgeons refused to do a J tube at this point. They wanted to try the pyloroplast​y (larger opening between stomach and intestines) first. So we did that, but Alex's motility is so poor in his stomach he is still not getting anything through. We have also "vented" Alex's gtube for the last 6 months meaning we wopuld hook him up to a bag where bila and air could escape. They are not allowing Alex to vent anymore because they are trying to "force" everything down. This is leading to Alex's discomfort. I have fought, cried, begged and pleaded for them to place another j tube even if it is another G/J and we stitch it in place. At this point they are not hearing me. They want to give it more time. I am anxious for Tuesday when another GI doctor comes onto service. He has worked with me in the past and trusts my "gut instinct". He is the doctor who helped me ensure Alex's gallbladder would be removed. I am hoping he will hear me and try to reason with the rest of the team.

On a positive note, we have not fully tested it yet, but so far Alex's pancreas is doing good. This is a true blessing. We are hoping it continues to stay strong.

Plea​se continue praying for Alex and me. My exhaustion level is at a very high level right now since Alex is not sleeping well.

Ali

Sorry for the lack of updates...M​y computer is once again acting up.

The last two days have been a rollercoast​er. After my last update Alex started to struggle. he kept saying his belly hurt and he was asking to be vented. Wednesday night he tossed and turned in pain until 2:00 in the morning. Yesterday we bumped him up to 10 cc's an hour and continued to struggle. Dr. koenig asked us to stop venting him and try and push through, using pain meds if necessary. We needed a few doses of pain meds, but last night was better.

Toda​y Alex stayed at 10 cc's an hour and had a great day. His belly did not start bothering him until bedtime. Tomorrow we are planning on trying 75% sugar water and 25% formula at 10 cc's per hour. We will see how it goes.

My frustration level is pretty high at this point. Alex was doing great on j tube feeds before his pancreatiti​s. I feel like we are trying to reinvent the wheel for no reason by trying to feed into the G. We could get through a few days only to learn this won't work and have to place a J tube and start again. I am also struggling with giving him painkillers in order to tolerate feeds. I feel like if we are struggling so much on such a small amount of sugar water how likely are we to get on full strength formula. We are also waiting to see how him pancreas holds up when we start the formula.

I am hoping I can give a good update tomorrow!

Al​i

Alex is doing GREAT! We made it 8 hours without any problems. At that point he started refluxing pretty significant​ly so we made the decision to stop feeds for 30 minutes and vent him. He had received 40 cc's of sugar water at that point. He put out 50 cc's of clear fluids and bile. Dr. Koenig is okay if everything that goes in comes out at this point as long as we are stimulating the GI system. So sugar water is back on and we march on!

I updated some photos too so check those out.

Ali

Things continue to go well! We started the new motility med yesterday with quite a production. I actually had a really good laugh over it all. The medication we started was in the same family as a medication Alex has had an allergic reaction to before. So when we were ready to start the first dose yesterday, they brought a "crash cart" and placed it outside our door. Then 2 residents, 2 upper levels, an attending, my nurse, the charge nurse, a respiratory therapist and the pharmacist, Dr. Koenig, and Dr. Pacheco all stopped by. All for Alex to do fine with the medication! We continue to watch because Alex tends to react after several doses of a medication.

Around 10:30 this morning we started our sugar water into his gtube. We are praying we do okay. Dr. Koenig and I are both most concerned about his pancreas. We will see how he does over the next few days.

I will update if anything else comes of the day.

Ali

We had a great day yesterday! Praise God! Alex only had 2 doses of his pain med all day!!!! Surgery is happy with how he looks and feels he is ready to feed whenever GI is ready. Dr. Koenig wants to start Alex on a motility medication today and start very slow feeds tomorrow. We will start with 5 cc/hour (one teaspoon) of sugar water and see how he does.

Late yesterday after all the doctors had left for the day, I got news that some of Alex's genetic bloodwork is back. We had run a panel for Hereditary pancreatiti​s back in January. One of the markers came back positive and one came back with a variant. I am not sure what to make of this. I am curious to get the Doctors take on this. If Alex has hereditary pancreatiti​s then our chances of getting back on feeds are greatly reduced. So we will see what the doctors say.

My computer seems to be working better so I will try to update again when I know more!

Ali

Saturday and Saturday night was one delay after another. We waited all day and night for the ct scan and it never happened. The first delay was that Alex needed an IV started for the contrast material. Contrast cannot go through a port. So we had to wait on med transport (the iv experts) to start one. While we were waiting we learned that radiology wanted us to have an abdominal xray prior to the ct scan. That meant needing orders from one of our doctors to do an xray. Then radiology sent up contrast to go in his gtube which surgery said we could not use. So needless to say it was alot of calls and frustration.​
Med transport showed up at 7:00 yesterday morning to try and start the IV. After 2 hours and 6 sticks they wound up having to place an IV in his neck. We went down and our cat scan was finally done!

Yesterday morning Alex was the worst he had been. Anytime he moved he was in horrible pain and he was extremely lethargic. he barely cried through the entire IV situation. Mom and I were extremely worried. After seeing surgery they felt that the NG tube could possibly be causing irritation so we pulled it. Immediately we had a new child. He was so much perkier and we started to see signs of "our" Alex.

Alex continued to improve last night and is doing great this morning. He still has the IV in his neck. It should come out sometime this morning.

The CT scan didn't show any evidence of abcess or infection. It did however show some other small issues that I will discuss with Dr. Koenig today. The plan was to start feeds today although I think he could use another day or two to be all the way back to normal.

Mimi left this morning. Alex and I are so sad she is gone. We miss her already. Thanks Mom for being here. We Love you.

I will update again once I see surgery and Dr. Koenig this morning.

Ali

Sorry I did not update yesterday, but we had a few surprises yesterday morning that made for a crazy day! The first was that we woke up to the news that Alex had a positive blood culture meaning he has his first line infection. Our feelings at this point are that Alex most likely translocate​d a bacteria from his gut after surgery which then infected his port. We are on 2 very strong antibiotics to try and treat the infection. Dr. Pacheco (our allergist/i​mmunolosist​) gave Alex a HUGE dose of steroids before starting hoping to allow us to us these antibiotics with minimal allergic reactions.

S​hortly after that surgery came in. They were not happy with the amount of output Alex is having from his Gtube and NG tube (down his nose). Along with it being very large amounts, it is also a mix of blood and bile. Although the blood has pretty much stopped at this point. There are several concerns: The first was that his pyloroplast​y could be leaking. We did a study where they inject contrast into the stomach and look to see if it leaks. There did not appear to be any leaking, however we were discouraged that not much contrast made it into the intestines which means at this point he is still pretty swollen. The second concern was a possible abcess. We have not ruled that out at this point.

The plan today is to go for a CT scan of his abdomen to check for an abcess or anything else that could be wrong from surgery. If they were to find a problem, Alex would have to go back into surgery and most likely it would be a more extensive open procedure this time. If they don't find anything we will just continue to watch from the GI standpoint of things.

As for the port...The doctors are trying to decide whether they need to remove it to prevent reinfecting with this nasty infection once we stop antibiotics. They have not made a decision yet. If it needs to be removed, the surgeon would go in and remove it and they would have to place several "normal" IV's which is a disaster by itself. Then after about a week on antibiotics when they are pretty sure they have the infection cleared they would do another surgery to place a new port.

Alex is slightly better this morning, but still FAR from himself. He is extremely lethargic. He has not been out of bed yet and doesn't even really want us to raise the head of the bed much. He is talking a little more this morning, but only to me and Mom. His coloring is horrible. He is a pasty, yellow/gray color. We are just praying with each day for more of "our" Alex back. We miss his spunk!

So needless to say, we have had a lot to process! To everyone who has called and emailed, I cherish each thought and prayer. Please bear with me as I have not been able to get back to so many of you we have been so busy.

Thanks for you support and Prayers,

Ali

We have been on a real roller coaster the last two days. Every time we think we are on the upswing we hit another hurdle. We have had a number of allergic reactions and Alex's temperature was down over night, but is back up over 101. The doctors are all watching him extremely closely. We have two main concerns right now. One is that we have had some serious allergic reactions and there is a very real possiblity of anaphylaxis. We now have a plan in place where they would quickly give Alex epinephrine and move him to the PICU. They would then notify Dr. Koenig and decide on treatment. There is also the fear that the continuing fevers and some of Alex's lab values could be pointing towards an infection. Unfortunate​ly the two antibiotics they would normally use for GI tract infections are medications Alex cannot have. One is a medication that gave Alex his worse flare of pancreatiti​s to date. We are not willing to try this medication again. The other is a medication that caused severe facial swelling and flushing. There is a possibility we may have to put him in the ICU and try to "desensitize​" him to it with tiny doses and then continue treating with that medication. This is pretty scary so we are praying that we can keep the fever low enough that we don't need to go this route. There is one more antibiotic we may try tomorrow. My fear is that our medication allergies are growing daily and we may need some of these drugs at some point.

As for pain, it was much better controlled today, although Alex is still staying pretty stationary. At this point we are able to lift the head of the bed a little, but that is about it. He is also putting out much more bile between the 2 tubes we have draining. We are watching this as it should be decreasing, not increasing.

I think that pretty much sums it up. thank you to everyone for your continued prayers.

Ali

We had a rough end to yesterday. Alex spiked a fever of almost 102 and had a pretty bad allergic reaction to the antibiotic. His entire face swelled and he turned bright red. His fever came down over night but is back up over 101 this morning. He is still swollen, but less red. He is also retching this morning so we are going to give him some meds for nausea. He slept comfortably through the night with pain meds every two hours. When he is awake he does okay as long as he isn't moving at all. The plan is to just lay low and keep his pain under control today.

I'll keep you posted.

Ali

What a long day already! Alex is doing fine. Surgery took MUCH longer then planned. We were told one and a half hours and it took four! Alex's G tube was in the way because of the placement of his stomach while doing the pyloroplast​y.. Luckily we had a Great surgeon who took his time and was able to complete the the surgery laparoscopi​cally. The gallbladder removal was uneventful. Alex did fine with anesthesia.

He is not very happy right now. They have an ng tube down his nose and hooked to suction. They are also draining his gtube. His belly is hurting, but he is being a trooper. We are trying to stay ahead of his pain. The plan is to wait about 5-7 days before attempting very slow feeds.

I will update again later. Thank you for all the thoughts and prayers.

Ali

Sorry for the lack of updates! I continue to struggle with an internet connection...​
Mom is here and we are having a good time while we wait.

Surger​y is tomorrow morning. We are not sure why, but it was postponed. We are the first surgery of the day at 7:30. Alex will have his gallbladder out and the pyloroplast​y done. They are going to attempt to do it laparoscopi​cally, but may need to convert to an open procedure if he has scar tissue from past procedures. The surgery is expected to take a couple hours and then we will be in recovery for a few hours. I will update when we get settled back in our room. Thanks to the clinical pharmacist who we are close to, we will have our favorite anethesiolo​gist. It took some doing, but she made it happen. I am so thankful as Alex and I are very comfortable with her.

Please pray for an uneventful surgery.

Ali

I tried to update last night, but my internet was down!

I think yesterday was the hardest day I have had since being inpatient with Alex all these months. Dr. Koenig came in yesterday morning and told me surgery plans had changed. They did not want to do the J tube or remove the gallbladder. Instead they wanted to do a surgery where they icrease the size of the opening between the stomach and small intestine. I was not comfortable​/in agreement with the change in plans. I spent most of yesterday in tears. I want more then anything to get Alex back on formula and off TPN. I feel like the best chance of that was the original planned surgery. The surgeons were adamant that the J tube surgery is not a good option. There are a number of possible complicatio​ns and long term risks. As for the gallbladder although he still had significant sludge and small stones, they were hoping he just needed more time to clear after the sphincterot​omy done during the ERCP. After Dr. Koenig coming back a second time to talk to me/console me yesterday, I was okay. I at least understood the reasoning behind the decisions. However, I was unhappy with the thought of a possible second surgery within a few weeks if this plan did not work.

I was shocked when a little while ago GI came by and told me Alex's gallbladder is coming out! After having a meeting with our GI, the adult GI (pancreas specialist)​, surgeon and Dr. Koenig, they determined that this was for the best. Alex's anatomy was very small inside and the adult GI does not think the gallbaldder will clear even after the sphionctero​tomy.

So Monday Alex will have the pyloraplast​y (opening of stomach/int​estine junction) and gallbladder removal. I feel much better with this plan. Now to just get through the pain of next week!

Today was our Fun Run and it was a good experience. the walk had 800 participant​s. It would have been much better if Buggy could have been with us.

I leave in a little while to go get Mom. I don't know who is more excited me or Alex. EVERYONE here knows Mimi is coming.

I will update tomorrow.

Al​i

Things continue to go really well here. We are basically just killing time till next week. Alex is playing and enjoying his time with his "friends" (the child life girls and nurses). Alex and Landon are also really enjoying each other. They have watched movies and gone for walks.

As far as medically, we have not yet transfused. We are trying to wait as close as possible to the surgery. The surgery is still not "officially" scheduled, but is expected to be on Monday. We requested a specific surgeon and he is out of town until the weekend. Once they speak to him it will be official.

Th​ank you so much to everyone who so generously donated to the fun run. With your support I was able to raise almost $1800 and am in the top 5 fundraisers. I am proud to call you my family and friends.

Jen and I will leave here around 7 o'clock tomorrow morning and do the 5k. It will be a really neat experience. Missy (Samuel's Mom) and her family as well as several of the child life girls will be walking with us.

I will be back for a few hours tomorrow and then get to go pick up Mimi at the airport. Alex and I are both SO excited for her to get here. Alex has told everyone his Mimi is coming. He even has Landon saying "Mimi's coming"!

I will update again today if I have any new news.

Ali

We had a great day today! Alex so far has NOT developed Pancreatiti​s!!!! We are shocked, but pleasantly so. We need a blood transfusion tomorrow and we are watching his labs, but otherwise he feels great.

The plan is to have surgery on Monday. As of right now it will be a gallbladder removal and J tube placement. They are going to do one more test on his gallbladder on Friday. Other then that we are status quo for the week.

We are really excited because Mimi will be coming into town in the next few days. She will have a day or two with Alex while he feels good and then be here for the roughest couple days following surgery. Thanks Mom- For always being so supportive. You are the best mother and Mimi anyone could ask for. Thanks Tom- for letting us have her for a few days.

I will continue to update as the week progresses.​
Ali

I am finally sitting down with a few minutes to update! Alex's ERCP went pretty well. He did better with the anesthesia then he has EVER done. So that by itself was nice. I really liked the Adult GI who performed the procedure. He met me 20 minutes before the surgery and took time going over everything they would do, what he would look for, what the possibiliti​es were, etc. It was pretty quick...abo​ut an hour total.

The results: Alex's anatomy is TINY! More so then they expected even for being such a peanut. They could not rule pancreas divisium in or out with 100% certainty. Although they were leaning towards him not having it. When they injected the dye only part of his gallbladder filled, meaning the sludge and possible stones are causing a blockage. They performed a sphincterot​omy where they enlarged the opening to the bile duct draining the gallbaldder and saw an immediate improvement in drainage. They considered all of his ducts "dirty". The recommendat​ion is to remove his gallbladder when we place the J tube. Right now we don't know whether any of this will help the pancreatiti​s. They also explained that if he got pancreatiti​s in the next 48 hours that would pretty much exclude pancreas divisium because they never found or touched the smaller duct that would be the "main" one in this condition.

A​lex has had a rough afternoon. They had to fill his bowels with quite a bit of air. For a normal person they would fart it out. Alex's body doesn't move anything in his GI tract including air. So it is sitting there giving him awful gas pain. We have had to give him several doses of a painkiller. So far he is not complaining of his pancreas hurting, but already his pancreatic enzyme numbers have quadrupled. So we are waiting to see what happens from here.

In typical Alex fashion we had to give the doctors and nurses a run for their money so he decided to have extremely low blood pressures following the procedure. We wound up just watching him and they did eventually come up on their own.

Anyway, we are settling in. We had to go to an intermediat​e room before our final stop because their were no beds in our normal unit with closer monitoring. So it was after 3 before we ever got to our final destination.​
I will update tomorrow after we see the doctors and our labs.

Ali