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.

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.