Jacobs birthday was AMAZING!!  He first woke up in a very good mood, which is rare.  But he knew it was his birthday so I was happy to see him happy.  We surprised him saying that daddy AND Tyler were both taking the day off for this special day.  So he was happy about that too.  We let him rest and watch tv/youtube for a while until we had to get ready for the fun.

Around 1130 some of his cousins came over to say happy birthday.  We invited them over, knowing what was going to happen.  But Jacob still had no idea.  He thought they just came to spend some time with him.  A little after noon there was a knock at the door.  Tom got the door and Tyler ran to the door and started yelling and laughing.  Jacob looked at the door and saw Red Bird from the cardinals there!!  He was so excited.  He brought along some friends.  Lots of people from the Cardinals organization and CHEERLEADERS!!  Jacob was so embarrassed haha.  It was really cute.  They stayed for a little over an hour.  They brought cupcakes from sprinkles and lots of awesome gifts.  They even brought gifts for tyler and their cousins.  Jacob got all kinds of cardinals gear.  A jersey, hat, shirt, water bottle, footballs, a bracelet, ear phones, gloves, and more!!  It was so nice of them to do this for our boy.

After they left we went out and did a few fun things and then it was time for more!!  At around 5 some more friends and cousins started showing up for a little birthday party.  We had a balloon artist and then SPIDERMAN!!  Spiderman was AWESOME.  He played with these kids until they all wore out haha.  He was playing hide n go seek, tag, and other fun games with them.  All the kids thought he was the best!!

Jacob had a pretty good birthday, I would say.  The best part was seeing him open up all the cards.  He was so happy.  We are still opening some of them.  We didnt get them all open.  So we are taking our time with it.  It has been such a great experience.  I am overwhelmed by the support we have gotten.  So many people praying for our boy and wishing him well wishes.

Jacob's birthday

After our big scare at the hospital, we thought we wanted to do something really great for Jacob for his birthday.  He was feeling really down and knew that his birthday would be so horrible this year.  So I wanted to make him have the BEST birthday, yet!  So I decided that I would set up a PO box and see if I could get a few people to send him some cards.  I got the smallest PO box they have and thought I would just pick it up the day before his bday.  I posted it on his facebook page and it blew up from there!!  People were sharing his facebook page left and right.  I was so shocked!  I didnt think it would take off like it did.

A friend of Toms gave us dbacks tickets from his work.  They donated them to us and it was amazing.  And then Tom works with a guy who knows 2 of the dback players.  So he talked to one of them and told him we were coming to the game.  And what they did was amazing.  They got a jersey for Jacob that had the name Priestley on the back!  And he also gave him a signed ball.  And then took him and Tyler to the clubhouse where they got to meet a few players.  And the best day of Tylers life came, when he got to meet his idol, Aaron Hill!!  So the game starts and we are all flying high!  It has been a great game and then someone comes and sits behind us and says to tom "are you tom?"  He says yeah, and the guy says "Is this your son?" And Tom says "yep, thats Jake!" And then the guy talks to Jake a little bit about his jersey and then says "here I got this hat for you to match your jersey" and gives Jake a hat!!  It was so awesome.  Then the next inning they put us on the jumbotron and says we would like to welcome Jacob Priestley and family to the game!  It was awesome.  We had such a great time and Jacob was so happy and smiling the whole game.

The next day Tom sends a message to all of the news channels about a letter he wrote to the dbacks saying thank you.  Someone from Channel 10 emailed him back and said they wanted to do a follow up story on him and Jake.  So they came over later that day and did a news story on him.  It aired the next morning and it was great.  Here is the link to that.  http://www.fox10phoenix.com/story/26372078/2014/08/26/d-backs-create-special-day-for-boy-battling-life-threatening-disease

There is still tons more to come when his birthday actually gets here tomorrow.  I cant wait!!  It has been such a great week so far and I love seeing him so happy.

The worst hospital stay, yet!

So after our last hospital stay, things were going good!  Jacob had finished his antibiotic and was feeling pretty good.  We decided to take him out to see fireworks.  That is something we NEVER do, but we really felt like we should this year.  So we took him out and had a great time.  He fell asleep in the middle of it...but who cares.  He loved it and thats all that matters.

A few days later he started throwing up.  Its weird because he has NOTHING go in his stomach except meds.  And so he shouldnt be throwing up.  We knew something had to be up but the doctors didnt seem concerned so we werent either.  Doc gave us meds to help the nausea but that didnt seem to help much.  He was throwing up 8-10 times a day.  And it was all just bile.  And when he threw all of that up, he would just dry heave.  After a week of throwing up, we took him to the ER because things just were not getting better.  They did blood work and a bunch of tests and said everything looked good.  There is nothing indicating a problem.  Gave us different meds that seem to be working.  It would help for about 4 hours and as soon as the 4 hours would come, he would be throwing up again!

He started sleeping A LOT.  We thought it was the meds because it is a mild sedative and we also have to give him benadryl before because he had a little allergic reaction to it.  So that would make anyone sleep.  But he was sleeping 18-20 hours a day.  And I would only give him the meds like once or twice a day.  He would wake up to throw up and then go back to sleep.  We realized that he was getting worse and not better.  His heart rate was also a little elevated.  So we took him to the pediatrician.  He didnt say much either.  It was all the same advice.  Just make sure he isnt getting dehydrated and watch him closely.  The next day he started getting a fever.  With the broviac, if he has a temp over 100.4 we need to take him to the ER.  His temp kept getting up to that point and then would go back into the 99 range.  So we just watched it all day.  Around afternoon it was staying around 100-101 and his heart rate was a little higher.  So his nurse told me that it was time to take him back to the ER.  This time we went with more symptoms and they really took us serious.  They were thinking of admitting him straight to the ICU because of his heart rate.  The ICU doctor came to look at him and said he seemed ok enough to go to the regular floor.  So they admitted us to our favorite 4th floor.  We got there and his favorite nurse was on staff and she was so excited to see him.  We got all settled in and just waited while he slept...and slept...and slept.  No one saw any smiles from him or any jokes.  This is NOT typical Jacob.  Even when he is sick he is still smiling and talking.  Especially about minecraft.  But no one could get him to talk about minecraft either!

A few days passed and things were still more of the same.  He was getting anti nausea meds around the clock so his throwing up got less and less which was good.  We were in the process of moving.  So I had left and we went to our new house to look at some things.  On the way there I got a call from the oncall ped saying that due to his heart rate going even higher we needed to get him to the ICU right now.  I called my mom because we were not even close to going back to the hospital and someone needed to be there with him.  So she hurried up down to the hospital to pack up our room and stay with him until they got to the ICU.  When we got back, he was still in his regular room, thank goodness.  They transferred him about 20 min after we got back there.  It was crazy when we got down there.  Lots of nurses and doctors in the room trying to do all kinds of tests.  The doctor did a spinal tap and Jacob was a rockstar!!  He did so good.  They took more blood to test for different things.  When they were getting the spinal tap, someone called from the lab with critical lab results.  They said his lactic acid was at a 6...normal is 1.  But no one seemed really concerned.  I was, because I know a little bit about high lactic acid.  Its VERY common with mitochondrial disease.

Things in the ICU were a whole lot different.  We had 1 nurse and she was just for us.  She would sit right outside his window and watch his monitor.  We had to keep the door open and people were in and out all day and all night.  The floor has their own doctor thats oncall at all times.  And they are a whole lot more efficient on that floor.  It was different, but we really liked it.  I liked the attention that he got.  We would have to wait 30 min sometimes for a nurse to come turn off his beeping pump upstairs on other floors because they have so many other patients.  So this time, when his pump went off, she came right in to turn it off.  We also loved the nurses.  We had some great ladies who seemed to really care about him.  And they were a great listening ear for me, as well.

Jacob started to have a lot of confusion and hallucinations.  It was kinda scary.  He would wake up and start saying weird things.  And then go right back to sleep.  They were taking his blood daily because it was changing so much.  His levels were all over the place!!  They had no idea why he was so sick.  But something was going on.  They did a spinal tap, blood cultures, lots of blood work, and ultrasounds and MRI...it was always something!  But NOTHING was showing what was wrong.  There was just lots of things that pointed to what was wrong.  But they were all symptoms of something big.  But no one could figure out that big thing. They knew he was VERY sick and most likely was not going to come out of this sickness.  They didnt have much hope for him.  I have never given up hope...but this time I did.  We knew for sure this was the end for Jacob.

Then the scariest day of my life came.  This day was odd because he just got really sick.  His respiration's were in the 50's and 60's, his heart rate was 160-200, and his blood pressure was really low!!  The doctor came in and said that they needed to get an art line put in him!  That is an IV type thing that goes into the artery.  And it is able to give us real time blood pressure readings.  So they were able to watch it closer.  She made us go into a different room while they did it.  Tom had just gotten off work so I was going to take him back home while they did all of this.  My mom and my sister were there with Jacob so I felt ok going home.  I went home and I just felt sick to my stomach.  I just couldnt stay here.  So I went back up tot he hospital and I went to go in his room and they were still doing the procedure.  It should of only taken them 5 min but it was going on 30 min.  I found my mom and my sister and I was talking to them for a while and about 15 min later the nurse came in and said they are almost done.  His blood pressure went even lower and they had to give him a lot of emergency blood pressure meds to make sure he didnt crash.  It was so scary!  But they got everything in, and let us go and see him.  He was still very sick and sleeping but we were so happy he came through it all ok.  We thought that was when we were going to lose him.  I just kept praying over and over again to please dont take him yet.  I am not ready!  I wanted him to see our new house...his new room.  I wanted him to turn 12!  Have his birthday party this year.  I wanted to do so much more with him.  I was so scared.  My dad and brother in law came over and gave him a blessing and prayed over him.  My dad basically told him not to be scared.  And to be comforted.  We all felt a STRONG presence of angels in the room.  I knew we were headed down from there.  I went home that night and my sister stayed with him.   And I guess during the nigh he made a HUGE turn around.  He woke up and was up and talking for a while.  His blood pressure was stabilizing.  His fever went away and they were able to take him off one of the emergency meds.  It was a miracle!! He should NOT of pulled through this.  The doctors were all shocked.  They kept telling me that he shouldn't of gotten better.  All I k now is that there is a reason Jacob was not taken from us.  There is a reason that he got better!  We stayed about a week and a half longer, but he just kept getting better and better!!

There are A LOT of things that got taken from him that we wont ever get back.  But at least I have my son! The doctors keep telling me that he is still very sick.  ANYTHING can trigger the lactic acidosis and make his body shut down again.  We have to keep him very calm and away from any germs.  But I will do anything just to have him longer.  We have had to modify our lives and Jacobs live a bit but thats all little things now.   We will celebrate every single day we have with him.  Because we dont know how much longer we will have with him.  And we do not want to take anything for granted.  

Darn TPN!!!

So around the 2nd week of June, I started to see some changes in Jake.  He is normally very pale looking.  Well he looked like he had some color.  He didnt look so pale.  So I kinda thought something was wrong.  I didnt think much into it because he was acting pretty normal.  His nurse came a few days later and I asked her what she thought and she agreed that something just didnt look right.  So I called his pediatrician to get an appt in to see what he thought.  I had talked to my mom about it and said he looks kinda yellow but it was hard to see.  Well we went to her house and Jacob came with me.  And when we got there, he was VERY yellow.  I knew in fact that something was not right.  Thankfully our appt with the doc was that afternoon.  So we got in and he agreed that he looked yellow and wanted us to get blood test done right away.  We had scheduled one for the beg of the next week but he did not want us to wait.  It was a friday afternoon and I knew it would be hard to get a blood test done.  We have to get our home health nurse to come and draw his blood out of his broviac.  If we dont have a scheduled appt with her, its pretty hard to get her to come.  The doc wrote a STAT order and sent it over to them.  I called her and she said she was actually right around my house and she would come right then and take the blood.  She came and got it to the lab just in time!  It was supposed to close about 10 min later.

So we knew we would get the results back that following week so we didnt stress out much about it.  Saturday came, and Tyler had a basketball game.  So Jacobs nurse came that morning and we went to his game and had a great time.  We decided to go get some lunch after his game.  On our way to lunch I get a phone call.  It is from a GI doctor ive never heard of.  So I was a little confused why she was calling me.  She said that Jacobs liver enzymes were VERY high and we had to get him to the hospital IMMEDIATELY!!!  She said that something could be seriously wrong and he could be in danger.  So the faster we get him there, the better things could be.  We rushed home and packed up some bags and drove to the ER.  We sat in the ER for 5 hours while they did test after test trying to figure out what was going on.  I was just so worried because I had no idea what was wrong and they were not telling me much.  They admitted us so that they could do more tests and watch him closer.  When they did the ultrasound to see if his liver was enlarged, they found that his gallbladder was big and it was full of sludge.  That was not the cause of the high liver enzymes but it was a problem we had to take care of.  Thankfully we caught that because that could cause a lot of pain.

After all the testing it was proving that the TPN was the problem for all of this.  Being on TPN is very hard on your liver.  And so it was really effecting Jacob and his liver...and also his gallbladder.  TPN is hard on almost every organ it touches!  And being on it 24 hours a day is not good.  The more you are on TPN the harder it is on your organs.  So they took him off TPN for a few days and his numbers started to normalize.  They changed around his formula and then said we could no longer be on 24 hours.  Jacob gets dehydrated VERY fast and VERY easy, so it was difficult to take him off of 24 hours.  So they upped his fluid in the bags and gave him supplemental fluid that would run at the same time as his TPN but once the TPN stopped, the fluids would still run.  That is different on your organs because its just normal saline and has no long lasting effects.

The doctors were finally satisfied with his numbers and how everything was...and we were all set to go home!!  And then they came in to change Jacobs dressing on his broviac.  And it was draining some nasty stuff!!!  So the surgeon was called in to look at it.  And what do you know?  He has a staph infection!!  They took cultures from the drainage and sure enough, it was a staph infection!!  So that kept us in a week LONGER!!!  We were so mad.  The ended up having to pull his line because it was not getting better despite him being on some really heavy duty antibiotics.  So they pulled it and placed a PICC line in his arm.  Its the same thing, but its just used for short term access rather than long term like the broviac.  The day they placed pulled his line and placed the PICC line, jacob started having tummy troubles.  It was then determined that he had contracted CDIFF.  And then the doc told us just to go home haha.  We had to wait another week to get his broviac placed again.  But they said that since he was just getting sicker staying at the hospital, we should just go home!!  So they gave us the meds he needed IV form and sent us home.  It was pretty funny.  But we were so glad to be home.  

updates

We just saw the nutritionist.  This is the first time since getting his TPN that we have seen her.  Jacob has not been tolerating much formula anymore so I knew that she wouldn't be too happy with the way things are going.  And I was right.  He has actually lost 3 lbs since coming home from the hospital, which is pretty discouraging.  I knew that they were starting his TPN on the low side because we were hopeful, leaving the hospital, that he would tolerate more formula through his GJ tube.  Well that hasnt been the case.  So she wants to up his TPN.  And I figured that would be the case.  But since we are not getting much of anything in him, formula wise, its hard to figure out what to give him in the way of TPN.  She said if it was up to her, she would take him off formula all together and just focus on getting him to gain weight and getting his nutritional needs met.  But if we take him off formula all together, then we are making it so that his intestines die because they are not being used.  She then thought it would be a good idea to just give him a very slow drip at about 5 ML/hr just to make sure the gut is getting fed something.  That way it keeps it alive and working.  But then again she doesnt want to do that because any time he has anything in his stomach or his intestines, he has pain.  So that would just cause him constant pain.  So she was going to go talk to the doctor and see what she wanted to do.  They are going to up his TPN for sure.  Other than that, I really have no idea.  She told me that we will probably never get around his stomach hurting all the time.  Its just something we are going to have to deal with.  I am so tired of hearing that!!  I have heard that 3 times in the past 2 weeks and I just want to scream!  Every time we have a doc appt I leave in tears.  I am just so stressed out with the way Jacob is doing.  It scares me to death because its been a long time where I have head the words "he is doing great!  See you next year" haha. 

Jacob has been having granulation tissue around his broviac site.  For those who are not familiar with granulation tissue, its tissue that grows on the outside of an opening.  It usually grows when the body is trying to heal itself.  Normally you get it around the gtube site.  And its pretty common.  Its not common around the central line or broviac sites.  For Jacob, its very painful.  It bleeds and it has nasty discharge.  And so we went to the doctor to make sure it wasnt infected.  And he said it was just granulation tissue like we thought.  And we heard those famous words...and there is nothing we can do about it.  His body seems to be rejecting the broviac.  So not only is it trying to heal and its not working, its pushing his line out of his body!  We go back next week to see if it has come out any further.  If not, then we will just deal with the granulation tissue like we are.  If it is, then the doc will have to pull the line and we will have to place it somewhere else.  I REALLY hope its not the 2nd option!!  But Jacob loves to be rare and he always gets those rare side effects.  So why not!

I will update more when I know the updates from the doc appts.

Another appt, another diagnosis

Yesterday, we had an appt with Dr P who is the pulmonary doctor.  We started going to him because Jacob was having some shortness of breath and he was having some major dips in his oxygen sats.  So our pediatrician sent us to see Dr P.  When we first went to him, he said that he was shocked at how well Jacob was doing when it comes to his breathing.  He said that he has Jacob on his watch list and any time we get admitted for anything breathing related, I need to call him ASAP!  Well after listening to what has been going on, he wanted a sleep study done.  We got it done and went back to his office for the results.  He said there wasn't much on there and he was surprised that it wasn't worse.  Well that is...until our last appt.  He looked at the sleep study again and totally missed something the last time.  They put this little part in his nose and it measures how much he is breathing and how much air is getting through.  Apparently, not much is getting through his nose.  So she said that it could very well be something called upper airway resistance syndrome.  He read that and said...ah ha!  The missing piece of the puzzle.  So he said he wanted a cat scan done of his sinuses.  I remembered that about a year ago Jacob got a MRI of his brain and I remember that it said something about his sinuses in the report.  And so I told him that I am pretty sure he can look at the MRI and see what he needed.  So off he went to look at the MRI and he said...yep, exactly what I was looking for!  He called me over and showed me what was going on.  In a normal persons sinus cavity, your sinuses are straight.  In Jacobs, the left one is straight but the right one curves.  So its blocking most of the air coming through.  Upper Airway Resistance Syndrome  is.. During sleep the muscles of the airway become relaxed. The relaxation of these muscles in turn reduces the diameter of the airway. Typically, the airway of a person with UARS is already restricted or reduced in size, and this natural relaxation reduces the airway further. Therefore, breathing becomes labored. It can be likened to breathing through a straw.  And to top it all off, Jacob always has lots of sinus drainage.  So that's even more blocking his airway.  

So what does that mean for Jacob?  Well, he is now going to be on oxygen at night to help him breathe better.  He is also going to be on hydroxyzine and hopefully that will help his allergies a little bit.  We are also going to start a nasal spray and hopefully that helps as well.

Hospital visit and surgeries!

Since last update, we were able to schedule and get the port placement. We were admitted the day before so that Jacob could get the fluids he needed. And then the next afternoon we were supposed to be wheeled down to surgery and get it placed. Well we woke up around 8 am to our nurse saying...umm so apparently the doctor wants you to get it done now. So he is taking you guys next!! AHH!! I was really super nervous about it, but it didnt leave me any time to be nervous. I had to call my mom and Tom and let them know that we would be moving the surgery WAY up! We head down to the pre op and get everything ready and around 930 Dr G comes in to go over the surgery. I still dont know what type of port they will be putting in him. So Jacob asks the doctor which one they are doing. He tells me that he had talked to his other doctors and they all agree that the port o cath would be the best one to go with. But he asked me how I felt about it. I told him that I just felt more comfortable with the broviac port instead. Because even though the doctor may only want him to have fluids a few times a week, I have a feeling that we will need to use it more often down the road. He told me that he agreed with me, and broviac is the one we should pick. Surgery went great! Jacob came out of it great. And they taught me how to use the broviac and off we go to get released.

Everything was going good with the new broviac. They actually ended up ordering fluids to be done EVERY DAY. So I am so glad that I had the instinct to go with the broviac. That one is more for being accessed every day. We were supposed to be getting his new GJ tube BEFORE the port surgery. But scheduling kept having issues and then they couldnt talk to the radiology people and it was just going around and around in a big circle and nothing was ever getting done. We had to measure his tube again to see what size they had to order (and it ended up being the same size he has now...sheesh) So finally they got the piece ordered and we were able to get it placed. So we went in like always, the day before. He got it placed early the next morning. And then we went to start his feeds. He really had a hard time tolerating them. He would get sick and horrible pain and everything else associated with it. So after a day of realizing he is not going to tolerate it, they finally started him on TPN. TPN is nutrition that they put through IV or through the veins. So he may not be getting a lot of food through his tube but he is getting some nutrition through the port. Again...SO GLAD we decided to go with the broviac! So we were there for 6 days and left on TPN and probably will be on it for a long while. We are trying to up his feeds but every time we do, he gets lots of pain. So I know its going to be a sloooow process. We are supposed to up his feeds on his feeding pump 1 ml/hr every 3-4 days. And it needs to go up at least 40 ml/hr. We already tried to up it and it was fine for a few hours...and then he was back to having pain and having a very big full feeling. So pray that we can get it up soon and we can start tapering him off of his TPN.