Yes, like the title says...we FINALLY have answers. The answers we want? No...but its something!! Earlier this week we finally found the genetic link to Jacobs mito.
I cant believe I am saying (or typing) those words. We FINALLY have his puzzle figured out!! Yes, he has had a mito diagnosis for 2 years. But its been based on the results from his muscle biopsy. It could of been many other things from those findings. But since his clinical presentation matched up with mito, that was the only other explanation. But now we have a GENETIC link to it! I cant believe it.
I am having a really hard time with it. I have no idea why. I guess I always had hope that maybe it wasnt mito. And that maybe it was something else that was treatable. Or maybe I made things out to be worse than they really are and he would get better and we wouldnt be dealing with this anymore. But those dreams and that hope was shattered. It was thrown away in one day. Those dreams and hopes I had went into the garbage can. Now I have been focused on the what ifs. Researching his mutation has been tiring. Its been sad and depressing. I dont want any of these things to happen to Jacob. I dont want ANYTHING else to happen to Jacob. I am done. I am waving my white flag. I dont want to do this anymore. I want my boy back. My happy boy who loves food, who loves to sit at the computer for hours and play video games, the boy who loved playing with legos, and loved playing tag outside with his brother. One thing this disease will never take away from Jacob is his happiness. No matter what is going on he is so happy and loves to smile. When people are around Jacob acts like he isnt sick at all. But its once those doors are closed and we are no longer around other people, he gets sick again. He crashes and lets it all go. He does not like letting people see him be sick. He feels like he has to be "normal" for other people.
I am constantly worried about Jacob. Any time his temp starts to rise, I worry. Or if he starts to get a stuffy nose, I worry. I cant relax because when I do, I feel like something is going to happen. I have nightmares every night of things that will go wrong. Or jacob getting really sick again and us being back in the ICU. Knowing that the mito is here to stay isnt helping at all. It has really made things worse.
Right now Jacob is doing ok. He is really struggling with keeping his blood pressure stable. His blood pressure has been REALLY low. Like when we are in the hospital and his BP goes down this low, they are transfering him to the ICU and giving him BP meds to get it up. He hasnt been feeling good all day. He has had a headache for 2 days and has will get little bouts of energy but quickly gets tired and lays back down. His refulx is so bad right now. When we give him his meds, they come right back up. He will reflux on nothing all the time. We need to up his meds. He is on 2 different meds to help reflux. Doesnt seem like its doing its job. We are out of the hospital so I guess thats good. His temps have been fine since we have been home from the hospital, the past week. But today they started spiking back to 99's. We dont know why and if something is brewing again. It seems like once his temp goes to 99 that usually means something is about to pop up. We are praying so hard that he stays out of the hospital during thanksgiving.
I just want to thank everyone again for their love and support. It means a lot to us even if we dont show it.
This is a blog about Jacob, who is 10, and his fight with Mitochondrial Disease (Kearns Sayre Syndrome) and Autism.
Saturday, November 15, 2014
Friday, October 3, 2014
Friends
Tonight was a rough one for Jacob. He was supposed to be sleeping and I hear him crying. So I go to his room to see what is wrong. Naturally I thought he was in pain somewhere. But then he says hes fine and he doesnt want to tell me whats wrong. Finally I coax him into telling me whats wrong. He tells me that is he sad because he doesnt have a friend. I tell him that he has tons of friends!! He says no, he wants a best friend. I tell him that he has his brother who is his best friend and I kinda leave it at that. I leave and he calls me right back in. So I go in and he says, you want to know why I was really crying? He said he wishes he had a best friend. A kid who has no medical problems. So that he could cheer him on when he is in the hospital and someone to cheer him up when he is sick. This just about broke my heart. I feel so bad for him. I wish he had friends. I really do. I just wish I could do more for him. I cant make a kid be his friend. We have tried to have boys come over and play minecraft. But its not fun when they can only stay for a short time before Jacob is tired and needs to sleep. Or his TPN needs to be changed, or he needs his meds, or something that scares the kid off and he never wants to come back and play.
It got me thinking about my own life. I told him, I dont have friends either. I know what its like to not have any friends. Sure, I have lots of people I call a friend. But I dont have many who I can call at a minutes notice and go hang out. Or someone who knows when im having a bad day and we get to go out and forget about things for a while. I used to...but not anymore. I miss having my best friend here. I get lonely and wish I had friends to hang out with during the day. But kinda like Jacob, once they get to know my life...they leave. Its not a desirable life. I am stressed out beyond stressed, I rarely get time for myself, I have to put Jacob first. And in many times I have had to cancel last minute because of something with Jacob.
Moving into a new subdivision has been hard. Lots of awesome people I would love to be friends with. But the reality is that no one really wants to be my friend. I dont have little kids like everyone does. I dont go to playgroups or park days. I dont sit outside while my kids run around and play with the other kids on the street. The kids are all much younger than Tyler and Tyler has no desire to really play with kids who are a lot younger than him. I take Jake out for walks in his wheel chair. I see everyone starring. It doesnt bother me. It doesnt bother Jacob either. Thats our normal. I am sure its weird to some people. They see us and im sure they think that we would be much better off not being bothered. That we have enough to handle and we dont need people bothering us. But its the exact opposite. We would LOVE to meet more people and have them as friends. We NEED time away! We need to destress sometimes.
I guess the moral of this post is that we have each other and thats really all that matters. Jacob and Tyler are each others best friends. I know to Jacob, thats not enough. He watches all these shows on Disney and nickelodeon and they all have best friends. I hope that Jacob realizes he has a ton of people who are always cheering him on when he is in the hospital. I told him next time, I will show him all the posts from people. He has the biggest cheering section. He has tons of people who love him. He will understand that one day, I hope.
It got me thinking about my own life. I told him, I dont have friends either. I know what its like to not have any friends. Sure, I have lots of people I call a friend. But I dont have many who I can call at a minutes notice and go hang out. Or someone who knows when im having a bad day and we get to go out and forget about things for a while. I used to...but not anymore. I miss having my best friend here. I get lonely and wish I had friends to hang out with during the day. But kinda like Jacob, once they get to know my life...they leave. Its not a desirable life. I am stressed out beyond stressed, I rarely get time for myself, I have to put Jacob first. And in many times I have had to cancel last minute because of something with Jacob.
Moving into a new subdivision has been hard. Lots of awesome people I would love to be friends with. But the reality is that no one really wants to be my friend. I dont have little kids like everyone does. I dont go to playgroups or park days. I dont sit outside while my kids run around and play with the other kids on the street. The kids are all much younger than Tyler and Tyler has no desire to really play with kids who are a lot younger than him. I take Jake out for walks in his wheel chair. I see everyone starring. It doesnt bother me. It doesnt bother Jacob either. Thats our normal. I am sure its weird to some people. They see us and im sure they think that we would be much better off not being bothered. That we have enough to handle and we dont need people bothering us. But its the exact opposite. We would LOVE to meet more people and have them as friends. We NEED time away! We need to destress sometimes.
I guess the moral of this post is that we have each other and thats really all that matters. Jacob and Tyler are each others best friends. I know to Jacob, thats not enough. He watches all these shows on Disney and nickelodeon and they all have best friends. I hope that Jacob realizes he has a ton of people who are always cheering him on when he is in the hospital. I told him next time, I will show him all the posts from people. He has the biggest cheering section. He has tons of people who love him. He will understand that one day, I hope.
Friday, September 26, 2014
Some thoughts
My heart has been very heavy the past few months. Just seeing Jacob suffer in the hospital is just horrible. I sit there and cry a lot because I just feel helpless. When he was so sick and had to be put on a bunch of meds to help him come back to us, I got in my car and I just cried and cried. I have done a pretty good job of holding things together until I get alone and start to think and then the walls just break. I cant be strong anymore. I cant be the rock. I am so beyond scared that the worst is coming. We have had doctors tell us that. That it seems like this is all the calm before the storm. We are very good friends with the ICU floor. Every nurse and doctor know us. Thats not something I want to be friends with. I mean they are all great people...but that just means Jacob has been there too much!!
This last hospital stay I realized that these people really do think of us as family. We were having nurses come from other floors to say hi and to check on Jacob. We have people from the respiratory team fighting over Jacob and who gets to see him. We have a girl from the line team who left to another job and she came to say good bye to Jacob. It was really sad and he ended up crying a little...which if you know Jacob, you know that he does NOT cry over things like that. We have doctors who work harder for us because they know Jacob and they know that he needs a little bit more attention. We have doctors that dont even have to look at past notes because they know Jacobs history by heart. We have nurses who bring Jacob his favorite things before we even get to the room because they know he loves seeing his gifts on his bed.
This is not a life that I wanted to live. I thought for sure that my 2 boys would move out and get married and give me lots of grandkids. And that Jacob would play basketball because he was so tall. But instead we get to worry about Jacob being sick all the time, and going to the hospital every few weeks, and what we are going to do with Tyler because he is left without a mom for a while, we deal with behavior problems with Tyler because he is tired of Jacob getting all of the attention. I wish that Tyler would understand that Jacobs attention is not fun! But of course he doesnt. He is only 9 and doesnt really understand a whole lot.
We know that Jacobs time is coming soon. When...well we dont know that part. But I just want Jacob to live a good life. I want him to experience things he should be able to experience. I want him to rest easy knowing that he had a good life. I am trying so hard to give him that life. To take mito away for just a little bit. To make sure that he gets experiences. To make sure that he knows that he is loved and that there are people all over who love him and pray for him daily. I try hard to make his life as normal as possible. I want him to get in trouble. I want him to do things he isnt supposed to do. He is supposed to be a tween! He is 12 years old and I want him to be able to enjoy things a normal 12 year old gets to enjoy.
Sorry this post is all over the place. I am struggling with emotions right now. I just wanted to write some of my thoughts down so I dont forget them...haha.
This last hospital stay I realized that these people really do think of us as family. We were having nurses come from other floors to say hi and to check on Jacob. We have people from the respiratory team fighting over Jacob and who gets to see him. We have a girl from the line team who left to another job and she came to say good bye to Jacob. It was really sad and he ended up crying a little...which if you know Jacob, you know that he does NOT cry over things like that. We have doctors who work harder for us because they know Jacob and they know that he needs a little bit more attention. We have doctors that dont even have to look at past notes because they know Jacobs history by heart. We have nurses who bring Jacob his favorite things before we even get to the room because they know he loves seeing his gifts on his bed.
This is not a life that I wanted to live. I thought for sure that my 2 boys would move out and get married and give me lots of grandkids. And that Jacob would play basketball because he was so tall. But instead we get to worry about Jacob being sick all the time, and going to the hospital every few weeks, and what we are going to do with Tyler because he is left without a mom for a while, we deal with behavior problems with Tyler because he is tired of Jacob getting all of the attention. I wish that Tyler would understand that Jacobs attention is not fun! But of course he doesnt. He is only 9 and doesnt really understand a whole lot.
We know that Jacobs time is coming soon. When...well we dont know that part. But I just want Jacob to live a good life. I want him to experience things he should be able to experience. I want him to rest easy knowing that he had a good life. I am trying so hard to give him that life. To take mito away for just a little bit. To make sure that he gets experiences. To make sure that he knows that he is loved and that there are people all over who love him and pray for him daily. I try hard to make his life as normal as possible. I want him to get in trouble. I want him to do things he isnt supposed to do. He is supposed to be a tween! He is 12 years old and I want him to be able to enjoy things a normal 12 year old gets to enjoy.
Sorry this post is all over the place. I am struggling with emotions right now. I just wanted to write some of my thoughts down so I dont forget them...haha.
Hospital again?!?!
The title says it all. We were in the hospital AGAIN! UGH!!!
Last tuesday night I decided to randomly take Jakes temp. He wasnt acting very sick but I just thought I would take it. I am so glad I did because he had a fever!! Jacob never gets fevers so I knew he was sick. I waited for a little while to see if it would go down but it never did. It just got higher. So I took him to the ER. Jacob had been sick for a few weeks off and on. He has had a bunch of viral sicknesses. So the ER doc said that his blood work looked great and it didnt show any signs of infection. So we could go home because it was just a virus. I was happy because we had a very important doc appt to go to the next day. We have been waiting to see this doc and I did not want to have to cancel the appt.
I went and bought tylenol and motrin to give to Jake hoping that his fever would go away. When I gave him the meds the fever thankfully went away. So I went to sleep and woke up a few hours later to take Tom to work. I went in and took his temp and it was almost 104!! I gave him more meds and prayed that his fever would go away for this doc appt we had later that morning. Thankfully it did, but Jacob was very sick and you could tell. He had chills so bad and was in and out of sleeping. He would just sit there and whine because he did not feel good. We went to the appt and everything went good...until Jacob threw up!! It was so sad. I felt so bad for taking him out when he is obviously sick. The doc knew he was sick and wanted us to take him to PCH rather than cardons. He wanted us to see a doc at PCH and knew that we would get to see him sooner if we ended up inpatient there. I told him I would think about it and if we had to take him back to the ER, I would take him to the PCH ER.
After the appt we took Jake back home and I went to get gas because if we were going to go to PCH I needed gas and I knew that Jacob wouldnt be too happy sitting in the car while i gassed up. I got home and Karla (his nurse) said that his temp was back up to 102 and that we should probably take him back to the ER. Right as we were about to leave I got a call from Cardons ER. They said that the cultures they took of Jakes line last night came back POSITIVE for infection! And that we needed to get back there ASAP so they could admit us and start him on antibiotics. I hung up with her and the door bell rang. It was the district nurse here for our yearly appt! I was so stressed out haha. She came and I told her what was going on and to ask Karla all the questions because I was trying to get things ready. Her appt lasted about 10 min and I was ready to take Jake to the hospital.
We get there and get taken right back because they had been expecting us. So they take all kinds of blood work and cultures of his line again and we wait a few hours and finally they put us up on our floor. When we got there Jacob was getting really sick. His temp went up to 105! It was really scary. I have never seen a temp that high before. His body started to shut down. His blood pressure was starting to go low again. They made the decision to send us back to the PICU. So back down to the icu we went. We were only on the floor for 2 hours haha. Once we were there the doc was able to start him on a rescue med for his blood pressure to bring it up. Except it wasnt coming up! So he had to stay on this med for a whole day. Finally it started coming up and stayed up the rest of the time. He was on 2 different antibiotics to fight off whatever was giving him problems. They finally found what the bacteria was that was causing the infection and got us on the right antibiotic. And that did the trick. We were able to save the line and we only stayed in the hospital for 8 days! Compared to the last 2 stays, this was a breeze haha.
Last tuesday night I decided to randomly take Jakes temp. He wasnt acting very sick but I just thought I would take it. I am so glad I did because he had a fever!! Jacob never gets fevers so I knew he was sick. I waited for a little while to see if it would go down but it never did. It just got higher. So I took him to the ER. Jacob had been sick for a few weeks off and on. He has had a bunch of viral sicknesses. So the ER doc said that his blood work looked great and it didnt show any signs of infection. So we could go home because it was just a virus. I was happy because we had a very important doc appt to go to the next day. We have been waiting to see this doc and I did not want to have to cancel the appt.
I went and bought tylenol and motrin to give to Jake hoping that his fever would go away. When I gave him the meds the fever thankfully went away. So I went to sleep and woke up a few hours later to take Tom to work. I went in and took his temp and it was almost 104!! I gave him more meds and prayed that his fever would go away for this doc appt we had later that morning. Thankfully it did, but Jacob was very sick and you could tell. He had chills so bad and was in and out of sleeping. He would just sit there and whine because he did not feel good. We went to the appt and everything went good...until Jacob threw up!! It was so sad. I felt so bad for taking him out when he is obviously sick. The doc knew he was sick and wanted us to take him to PCH rather than cardons. He wanted us to see a doc at PCH and knew that we would get to see him sooner if we ended up inpatient there. I told him I would think about it and if we had to take him back to the ER, I would take him to the PCH ER.
After the appt we took Jake back home and I went to get gas because if we were going to go to PCH I needed gas and I knew that Jacob wouldnt be too happy sitting in the car while i gassed up. I got home and Karla (his nurse) said that his temp was back up to 102 and that we should probably take him back to the ER. Right as we were about to leave I got a call from Cardons ER. They said that the cultures they took of Jakes line last night came back POSITIVE for infection! And that we needed to get back there ASAP so they could admit us and start him on antibiotics. I hung up with her and the door bell rang. It was the district nurse here for our yearly appt! I was so stressed out haha. She came and I told her what was going on and to ask Karla all the questions because I was trying to get things ready. Her appt lasted about 10 min and I was ready to take Jake to the hospital.
We get there and get taken right back because they had been expecting us. So they take all kinds of blood work and cultures of his line again and we wait a few hours and finally they put us up on our floor. When we got there Jacob was getting really sick. His temp went up to 105! It was really scary. I have never seen a temp that high before. His body started to shut down. His blood pressure was starting to go low again. They made the decision to send us back to the PICU. So back down to the icu we went. We were only on the floor for 2 hours haha. Once we were there the doc was able to start him on a rescue med for his blood pressure to bring it up. Except it wasnt coming up! So he had to stay on this med for a whole day. Finally it started coming up and stayed up the rest of the time. He was on 2 different antibiotics to fight off whatever was giving him problems. They finally found what the bacteria was that was causing the infection and got us on the right antibiotic. And that did the trick. We were able to save the line and we only stayed in the hospital for 8 days! Compared to the last 2 stays, this was a breeze haha.
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.
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.
Wednesday, August 27, 2014
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.
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.
Wednesday, August 13, 2014
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.
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.
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.
Tuesday, April 22, 2014
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.
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.
Thursday, March 27, 2014
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.
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.
Wednesday, March 26, 2014
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.
Thursday, February 6, 2014
Appt update...take 2!
So we had an appt with the dietitian today. I knew that she was going to be worried with his weight loss and so I prepared myself for that. I didnt prepare myself for everything else that took place today.
When we saw Dr T on Monday, he had mentioned it was probably time for a port ( A port is a small medical appliance that is installed beneath the skin. A catheter connects the port to a vein. Under the skin, the port has a septum through which drugs can be injected and blood samples can be drawn many times, usually with less discomfort for the patient than a more typical "needle stick"). for IV fluids. He told me that I should talk to GI about it when I saw her on the 18th. I said ok and marked it down in my book to remember. Well when we were at the dietitian she also brought it up. She asked if we had looked in to getting IV fluids done at home. I said yes, and that we had just talked about it. She said that she thinks it would be good to start Jacob on TPN (Total Parenteral Nutrition. It provides your body with nutrition such as protein, sugar, vitamins, minerals, and sometimes fat (lipids). TPN is used when you are unable to eat or cannot get enough nutrition from the foods you eat. ) Because he is not being able to tolerate his formula and he is just loosing weight. So she wants him to be on it until we can gain some weight. But she said that she isnt a doctor and would have to check with his GI doc first before we did anything further. So she went out to go find her and came back a few min later and said that she wanted to see us ASAP so she wanted us to come back a few hours later when she had an opening. So off we went back home for an hour and a half. When we arrived back at the GI office, she came in and sat down and had us tell her about what was going on and what the dietitian said. I gave her a 30 second update and she said...oh I have to talk to Dr T about all of this.and then gets up. I was a little confused so I asked her what? She said that she had called him and he just called her back. She wanted to talk to him to come up with a good plan moving forward and wanted to see what would work best for Jacob. I was so shocked. I have never had a doctor that was willing to talk to other doctors to find a good plan for Jacob. So I was excited to see what they came up with. But I was not expecting everything haha.
She came back about 15 min later and said that they are going to go ahead with the port. And that as soon as we were done with her, we needed to go downstairs to surgery and ask them to get us seen ASAP. She wanted us seen TODAY. However, after we went down there, I was informed that there were no doctors at the clinic until Monday. They were all on call at different hospitals. So we have an appt for Monday at 11 to talk about the port placement. And then she said that she wanted to take out jakes G tube and put in a GJ tube (A gastrojejunostomy or GJ feeding tube is a combination device that includes access to both the stomach and the jejunum, or middle part of the small intestine. Typical tubes are placed in a G-tube site or stoma, with a narrower long tube continuing through the stomach and into the small intestine. The GJ-tube is used widely in individuals with severe gastric motility, high risk of aspiration, or an inability to feed into the stomach. It allows the stomach to be continually vented or drained while simultaneously feeding into the small intestine) That way it will bypass the stomach and go right into the intestines. It should make it that he does not have reflux or throw up anymore and hopefully not have anymore pain. Which is a good thing. I asked her if it would solve the problem he has about him not having an appetite and getting super full. She said no...that those feelings are more of a nerve problem with the neuropathy. She said that some kids she has with this problem have no problems tolerating feeds. Then she has kids who have issues and have to be on TPN long term because they cant take in feeds.
I wont lie, I am pretty scared. I have really wanted to avoid the port as long as possible because I know there is a chance of getting bad infections. And that can lead to a bunch of different problems. I know staying on TPN is not good either. It can harm some of his other organs. I know that none of this is necessarily great...but this is our only option right now. We have tried everything and it seems like nothing will work. Now that we know we are dealing with neuropathy we will try to figure out how best to manage it and the best way go around it. Seeing Jacobs health get worse is just so scary to us. We hate seeing him in pain all the time and feeling so sick. I really hope this does more good than bad. I am just so worried that it may harm more than it will do good for him. But I have faith. I know that the doctors would not do this if they didnt think it would help. Its just scary sometimes.
When we saw Dr T on Monday, he had mentioned it was probably time for a port ( A port is a small medical appliance that is installed beneath the skin. A catheter connects the port to a vein. Under the skin, the port has a septum through which drugs can be injected and blood samples can be drawn many times, usually with less discomfort for the patient than a more typical "needle stick"). for IV fluids. He told me that I should talk to GI about it when I saw her on the 18th. I said ok and marked it down in my book to remember. Well when we were at the dietitian she also brought it up. She asked if we had looked in to getting IV fluids done at home. I said yes, and that we had just talked about it. She said that she thinks it would be good to start Jacob on TPN (Total Parenteral Nutrition. It provides your body with nutrition such as protein, sugar, vitamins, minerals, and sometimes fat (lipids). TPN is used when you are unable to eat or cannot get enough nutrition from the foods you eat. ) Because he is not being able to tolerate his formula and he is just loosing weight. So she wants him to be on it until we can gain some weight. But she said that she isnt a doctor and would have to check with his GI doc first before we did anything further. So she went out to go find her and came back a few min later and said that she wanted to see us ASAP so she wanted us to come back a few hours later when she had an opening. So off we went back home for an hour and a half. When we arrived back at the GI office, she came in and sat down and had us tell her about what was going on and what the dietitian said. I gave her a 30 second update and she said...oh I have to talk to Dr T about all of this.and then gets up. I was a little confused so I asked her what? She said that she had called him and he just called her back. She wanted to talk to him to come up with a good plan moving forward and wanted to see what would work best for Jacob. I was so shocked. I have never had a doctor that was willing to talk to other doctors to find a good plan for Jacob. So I was excited to see what they came up with. But I was not expecting everything haha.
She came back about 15 min later and said that they are going to go ahead with the port. And that as soon as we were done with her, we needed to go downstairs to surgery and ask them to get us seen ASAP. She wanted us seen TODAY. However, after we went down there, I was informed that there were no doctors at the clinic until Monday. They were all on call at different hospitals. So we have an appt for Monday at 11 to talk about the port placement. And then she said that she wanted to take out jakes G tube and put in a GJ tube (A gastrojejunostomy or GJ feeding tube is a combination device that includes access to both the stomach and the jejunum, or middle part of the small intestine. Typical tubes are placed in a G-tube site or stoma, with a narrower long tube continuing through the stomach and into the small intestine. The GJ-tube is used widely in individuals with severe gastric motility, high risk of aspiration, or an inability to feed into the stomach. It allows the stomach to be continually vented or drained while simultaneously feeding into the small intestine) That way it will bypass the stomach and go right into the intestines. It should make it that he does not have reflux or throw up anymore and hopefully not have anymore pain. Which is a good thing. I asked her if it would solve the problem he has about him not having an appetite and getting super full. She said no...that those feelings are more of a nerve problem with the neuropathy. She said that some kids she has with this problem have no problems tolerating feeds. Then she has kids who have issues and have to be on TPN long term because they cant take in feeds.
I wont lie, I am pretty scared. I have really wanted to avoid the port as long as possible because I know there is a chance of getting bad infections. And that can lead to a bunch of different problems. I know staying on TPN is not good either. It can harm some of his other organs. I know that none of this is necessarily great...but this is our only option right now. We have tried everything and it seems like nothing will work. Now that we know we are dealing with neuropathy we will try to figure out how best to manage it and the best way go around it. Seeing Jacobs health get worse is just so scary to us. We hate seeing him in pain all the time and feeling so sick. I really hope this does more good than bad. I am just so worried that it may harm more than it will do good for him. But I have faith. I know that the doctors would not do this if they didnt think it would help. Its just scary sometimes.
Monday, February 3, 2014
So I guess I will post a little update on Jake. He has been going back to his old ways lately. Not tolerating feeds, getting full way too fast, getting super dizzy and passing out...all those fun things. It is a constant battle every single day to make sure that he eats and goes pee. There have been quite a few days lately that he isnt even peeing! But the doctor has reassured me that its only because he is not taking in any fluids. We cant get the required amount of nutrition in to him because he isnt tolerating much of anything. So he has lost sooo much weight! Even after leaving the hospital last month. He is down to 87 lbs. It seems fine...and there are kids who weigh more than him...but he is also 5'4 and 3 months ago he weighed 113 lbs. So seeing all of that weight loss has been really concering on us all.
So we had 2 appts today. Neurology and Pulminology. Neuro was first. I cant even begin to say how much I love his office! I am good friends with his nurse and we talk many times a week on the phone. However, I have never met her. But I saw her talking to someone today and I was so excited to get up and meet her. She asked how Jacob has been and the floodworks came out! She knows that I am very worried about him, and she says the same thing. Its so hard to talk about your child and how they are not doing good. But sometimes holding it in is never a good thing either. So she tells me that I really need to talk to Dr T about the concerns im having and she would help me figure out a good plan. So in to the appt I went. I was a little mad because he was really behind and we still had Pulm to get to. So I only had an hour to talk to him. I know it seems like a lot but our appts are usually 2-3 hours. I guess thats why he was so behind haha. He takes so much time with everyone. I just love how attentive he is. So I start talking about my concerns...all which he agrees with and then he goes to do his assessment on Jacob. And I tell him (like I have in the past) that Jacobs feet are still really tingly and that its starting to get painful now too. So he puts the thing he uses to check reflexes with on his foot. Jacob says he cant feel it. So he does it to the other foot. Again, Jacob says he cant feel it. He goes up his leg and Jacob immediately screams and says its cold! So then he checks his hands...same thing. Jacob could not feel it on either hand but once he put it up his arm...there it was. So Jacob has now been diagnosed with Nuropathy. I had no idea what that was so I told Tom to google it. He did and said that its loss of feeling in hands and feet. Well duh...thats what he has. We were so worried because he has been tripping SO MUCH more, and we have had to use his wheel chair almost everywhere we go because he has a really hard time walking. So its nice to know why. He had some nerve testing done a few months back and it showed there was nerve damage but we never put a diagnosis on it. Now with his new symptoms...he for sure has it. So what is neuropathy? Well the Peripheral nerves carry information to and from the brain. They also carry signals to and from the spinal cord to the rest of the body.
Peripheral neuropathy means these nerves don't work properly. Peripheral neuropathy may be damage to a single nerve. It may be damage to a nerve group. It may also affect nerves in the whole body. Which is what Jacobs does. It effects many nerves in his body. Not just 1.
So on to our pulm appt. I got there and wasnt late...phew! So he read the test results of the sleep study that we did a week ago. It came back "ok" he wasnt thrilled with the results but did say it was better than he expected. So thats good. I guess his body moves 2 times every hour. And he told me that is a neuropathy thing for sure. It doesnt disturb his sleep, so thats good. Then we did a lung function test. His was in the low end of normal, so we are going to keep an eye on that for sure. Then he looked at his throat and nose. And he was fine until he got to his nose. I guess he has major congestion way up and some big fancy word was big and it shouldnt be haha. So he is putting Jacob on flonase and see if that helps. If it doesnt he is going to have to further investigate and see why its too big. He also said that if this med doesnt work we are going to have to look at his vocal cords to see if they are rubbing together. If they are...Jacob can say "I have nodes. I am living with nodes. But I am a survivor, but I have to pull back because I am limited. Because I have nodes." hahahaha...its a pitch perfect quote.
We see the dietitian on Thursday so I will update more when I go to that appt.
Around Christmas, Jacob just stopped tolerating his feeds. Every time he would get his feeds he would be in a ton of pain. So he wasn't getting anything to eat and no fluids. That caused Jacob to get very dehydrated. I have seen his dehydration symptoms so much that I can usually tell when its coming right before it starts to get bad. And I have noticed it a few days after Christmas. On the 29th, Jacob was very sick. He was lethargic, he was dizzy, his heart rate was up, and just not feeling good. So he stayed in bed most of the day. When it was time for bed, I was laying in there with him and he had a seizure. I had left to go tell Tom and started watching tv. Jacob came out again and said that he had another seizure and was slurring his speech and could not walk very straight. I got him back to bed and came back and was watching tv. About an hour later I hear a big bang. So I went to Jakes room and tried to open the door and I couldn't because he was in front of his door. I pushed it open and I tried to talk to him and to see what happened. I could not understand him at all. He was not talking in normal words. But what I did get out of him is that he got dizzy and passed out. I asked him why he was trying to get out of bed and he said he wasn't. He was confused and had no idea where he was. I got him back in to bed and went to bed myself. The next morning I expected him to feel better, but he wasn't. He was pretty much the same. So I called all of his doctors and they all told me to take him to the ER. I was about to take him and I got a knock at the door. I answered it, and it was the district nurse coming to do her 90 day check up. She saw him and said to take him to the ER ASAP. So that's what we did. When we got there, they took his sugar and it showed it was very low. So that's what they are saying caused the seizures and passing out. So they went to start an IV on him and they could not find a vein that would be good enough to give him fluids. They tried for an hour and a half and every time they would find a vein it would blow or just disappear. Finally they found one and they were able to get the IV started. They gave him a bolus of D50 which is sugar water, to get his sugars up. That worked, and then they gave him D10 fluids. We were in the ER for a LOOOONG time, when the doc came in and said that we needed to admit him for fluids because he was severely dehydrated.
We got admitted and talked to all the doctors. The main issue we had was Jacob stopped tolerating his feeds. So we wanted to start him on a new formula that would help him and start slow. We started at 10 Ml/hr and that gave him stomach pain right away. So they gave him his pain meds to make sure his stomach didn't hurt. It would take away the pain for a bit but not for the whole time it was supposed to. So they upped his formula rate by 3 every 4 hours until they got to 60. He had pain the whole time and they wouldn't really do anything other than give him pain meds. The Gi doc told me that we would just have to deal with the pain and there wasn't anything they could do. I talked to the dietitian and she thought we should change his formula. So we changed it to another kind and that did not give him any pain at all! It was amazing! So we decided to go with that formula and hopefully it helps him.
We are not quite sure why this all happened. His GI system just completely shut down and usually it goes back after some rest. But his has not and will not ever go back to normal. The disease that he has causes organ failure and we think this is what is happening to him.
Hopefully this will be the last of the hospital visits for a while. Since October we have been in the ER or hospital 8 times for a total of 32 days all together! I am so tired of the hospital, to say the least. We celebrated the New Year in the hospital. So it was a pretty crappy way to ring in the new year, for sure. My goal for the new year is to keep Jacob out of the hospital for at least 1 month in a row. So far January is out of there...I have high hopes for February.
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