We discussed why we went to Utah, and what was recently going on with Hailey. Since we obviously knew Hailey has neurological issues and was unresponsive to us they wanted to do an EEG of the brain. She has never had one, but I did mention that I was curious as to why they never ordered one in Utah when we were there. I asked one of the neuro doctors on her team there and they said that in Hailey's MRI it's all white matter (or lack there of) that they were seeing issues with. Seizures occur in grey matter and there didn't seem to be an issue with that. Not knowing what to expect with an EEG we were all curious how Hailey would react to it. Like usual she was a champ. A little fussy with the probes being stuck all over, but nothing too major. When I watched the screen of the EEG and all the crazy lines all over the place I was sure there was going to be some thing new we would learn...luckily there wasn't. We were told that her EEG was consistent with a child that has leukodystrophy.
So they gave her the enema and then did the test. I remember the nurse and her CNA doing the test and saying, "that looks like a negative to me, all done." So no blood in her stool. Where did the blood go? We would have noticed if she was leaking liters of blood and I haven't seen any. When I talk to the doctor to ask, "now what?" They tell me they are waiting on the Guaiac test. Um...you already got the results it was a negative. Well, they couldn't find the results of the test anywhere. I guess the nurse didn't log it where they were looking for it and it took HOURS to confirm that the results were indeed negative. Oy! My head!
So I can ask again, "now what?" Well now we still have to figure out where the blood went. We talked to a hematologist, who was really great with Hailey and getting her to do what was asked of her. In the end we discover that Hailey had Hemolytic anemia associated with the IVIG treatment she had. It was a side effect that she had never had before, but was having now. She was going to need a blood transfusion. In order for that to happen she will need a PICC line placed. Yes...a PICC line. Like the one we JUST removed!! She will need to be put under and have it placed and that was going to happen this afternoon.
In my head I am not freaking out because I knew that she did well just a few days ago with it being put in and the placement takes about an hour and then she'll have the blood transfused. Well like usual things don't go as planned. We are taken up to the ICU and we wait for about an hour and a half before anyone even comes in to tell us that they are running late. She was supposed to have the PICC line placed at 2pm and they didn't get started until 4pm. Well when 5:30 rolled around and we had heard NOTHING, we start panicking. We ask the nurse to check and give us info. We are waiting in Hailey's room and at 6:00 the room phone rings and unfortunately I answer it. It was the Placement Team. The head doctor was on the phone and says, "we are unable to place the PICC line. Hailey is too dehydrated and we can't get a vein. We have tried repeatedly. It's urgent that she get the blood transfusion and the only other option is a central line." I have no idea what that means? I asked what that entailed and he said that it's like a PICC line but it's placed in the neck and they needed permission to do it...NOW. Well, crap. So I quickly explain to Josh what the heck is going on and we both know we weren't going to say no, stop, don't do it. When I ask how long more the procedure will be they tell me, it'll be about 15 more minutes, but Hailey is fine, breathing normal, oxygen is good. So I give consent and wait...again.
They tell us that we can go up and wait for her or that they will bring her down when she's done, but we go up and walk her to the room. They get the cold blood from the blood bank and there were a lot of signatures needed in order for it to start to flow. It was going to take nearly 4 hours for the whole transfusion. I just sat and watched in wonder. Who was so kind and generous to give their blood to my daughter?
Tuesday morning we get the approval from insurance which I would have bet money on that I didn't think we would get. We did have to sign a ton of papers and say that we would pay if the insurance didn't.
Then we were told that the Vegas team got called out to Reno and don't know when they would be able to get us. When Primary Children's heard that we couldn't give them an ETA they arranged to come get us instead. Primary Children's to the rescue...again!
I am not good with motion. I get motion sick so easy. I always have. I was worried about going with Hailey. How would I take care of her and her needs if I am puking and can't take care of myself? So I took precautions. Josh bought me dramamine and these motion sickness wrist band pressure point things.
When I could see ground I was relieved but still beyond motion sick. Hailey looked scared, I was scared. I was also so mad at Josh for saying that he would have wanted to be the one to fly with Hailey while I drove. After we land they transport Hailey to another ambulance and the flight crew had to clean out that plane and clean the puke off of everything before we could leave. I asked for a trash bucket and told the flight nurse to find the suction and get it ready but making sure that it worked this time.
The flight crew said that they had NEVER experienced such a rough flight. I told them that they will always remember my little Hailey then. I said it was our gift to them...LOL!
Once we arrived at the hospital there was a whirlwind of doctors and nurses ready to take care of Hailey. Poor thing was covered in vomit and was in pain since she threw up her Baclofen. The doctor gave an order for valium and we started to get Hailey all cleaned up. They took off her dressing that covered the central line and re-did all the tubing and put clean dressings on. They had orders for Hailey not to eat but start an IV and if she wanted to drink I had to add thickener to the liquid first. Well, Hailey was pretty out of it and I wished so bad that I had some of what she was having because I was going to have some serious PTSD after that ordeal. Around 11 pm I sneak out of the room and my stomach was wanting food even though my brain questioned it. I walked down to the cafeteria and thought out loud, "What are we doing back here? Why does Hailey have to go through all of this? There has to be an easy way out of all this. I got my food which just ended up being fries and a Diet Dr. Pepper and went back to the room. Josh got there about an hour later and we tried to figure out sleeping arragments. We didn't have a room at the Ronald House yet. I gave him the couch and I slept in a chair. Morning came way to fast.
Now in my head I am thinking, the team will come in already knowing that we are here for a G-Tube placement and we'll get the ball rolling with that and we'll be out of here is 3 days tops! The team came in and started talking about how they have a schedule set for an EEG, a swallow test, meeting with infectious disease, and an exome sequence test....Um? Where is place G-Tube in that list? Why all these other tests? You tell me last time that our daughter has pretty much the worst of the worst of diseases and that we need to enjoy the time we have left with her even though they can't tell us exactly how much time that is and now you want to do MORE tests?! They tell me that they just want to make sure that they are crossing everything off the list. If there is by some chance that Hailey has something else that presents like Leukodystrophy but that has a treatment they want to do that. Then on the other hand if that falls through you have to relive the leukodystrophy diagnosis...So then I bring up that Hailey literally just had an EEG like 4 days ago. Since it was done in Vegas they would feel better if they did the test themselves. They also do a more extensive test. The swallow test has to be done before you put a kid through surgery. To make sure that something that drastic is needed. So then why this Exome Sequencing test now. We were told last time we were here that the team decided against it. Well Hailey's case of have leukodystrophy isn't a common case. Usually when kids get diagnosed with it they just go downhill immediatly. There is no "getting better" with IVIG or Steroids. All those times she was in the hospital and went home and walked again and talked again, and ran and was better then she ever was should NOT have happened, does NOT happen. How did she go from completely unable to walk, talk, and bare weight to, running, jumping and speaking in complete sentences and then back down again. Right now she hasn't walked in over a month, hasn't spoken, hasn't been able to sit up, or eat or drink. Now it's happening too fast. With Exome sequencing they will need to test over 2500 of Hailey's genes. Then they also take my blood and Josh's too. They should be able to see where the "faulty" gene is from. We learn more about the Exome test and it ends up sounding like a bad fortune teller that only tells you bad things that will happen to you. If I have the gene for breast cancer it will tell us. If we have the gene for Huntington's Disease it will tell us. It will tell Josh and I that we have the gene for any specific diseases that there is most likely no cure for. So why did I sign the papers to want to know that?? I couldn't tell you. I guess first and foremost if there is something in that test that will help Hailey then I want to know. Second, if I do have a gene for a really crappy disease, well...I already have it. I can't get rid of it. Third, if we can find out if this is a gene that will show up again in one of our siblings or siblings children it would be helpful for them to know. They most likely won't do anything with that information but if it were me and I had heard there was a chance I would have a child born with a genetically fatal disease I would have NEVER had wanted to have children. So we signed the consent papers and with that you are agreeing to finding out the results...ALL the results, not just the ones that would be helpful for Hailey. The test comes back in 15 weeks and the anticipation of those results are going to give me an ulcer.
In the mean while they take Hailey's blood and the kids crew comes by with a project for Hailey and I to do together. Here is a pumpkin we painted together.
Hailey's dad thinks it's no big deal to get your blood drawn. He just sits there all calm and lets the lab tech do what she needs to do:
swallow study. We go down to the x-ray room and suit up in our lead aprons. They have different consistencies of food that they will be giving to Hailey while they record live x-rays of how it goes down. With pudding first Hailey swallows but the pudding only goes half way down which causes her to swallow repeatedly. It keeps going back up and she swallows and finally it goes down. Then with a nectar consistency, her brain doesn't tell her trachea to close so now the doctor on standby is ready for Hailey to aspirate. After the second try he calls off the test and says it's too dangerous to continue. In other words...She fails with flying colors.
They decide that until they can figure out if it is safe for Hailey to under go surgery they will place an NG or Nose Tube that will be giving Hailey food. It's something that the nurse does and It was not easy. First the have to measure Hailey to see how long the tube will have to be. Then they lube the tube so they can stick it up her nose and down her throat. In order for this to work, Hailey has to swallow at the exact right time. They try 3 times and finally it goes down with a swallow. It's bothersome and Hailey doesn't like it, but after 1/2 a day she is done trying to mess with it and they can begin feeds.