22 days

Today is day 22 in the hospital. After much harassing, crying and hoping everything was set for her to be discharged on Monday. We waited patiently (kind of) for the TPN supplies to be delivered and teaching to be done. The expected time moved from 2 to 4 to 8pm. Finally at 6 my husband went home with all of the supplies we already had so our son wouldn't be getting to bed at 11pm. Through out the day Audrey's heart rate had been going up and up. The doctors were too concerned, "she's a little anemic" "mito kids have higher heart rates" "your PMD can follow up outpatient." Not wanting to press the issue too much, we REALLY wanted to go home, I sat and watched her heart rate get higher till it's 160-170 at rest. That can't be right. Finally at 930 pm the supplies come! Yay! But wait, that's not a TPN nurse dropping them off, that's just the delivery guy. So now it's nearly 10 o'clock and people are calling to figure out what's going on. Discharge is not looking likely. Then her nurse turns and looks at me and says, "I've taken her temperature three times, her temp is 39.8." That's 103.6 for you non-metric minded folks. Never mind about discharge, I know enough, children with central lines and high fevers don't get to go home. I knew something wasn't right with her heart rate! Though she didn't have a temp all day this is how Audrey rolls. One minute she is 99 and 10 minutes later she can be 105. She always keeps us on our toes. So our girl earned blood cultures and 48 hours of IV antibiotics until a line infection was ruled out. We also had to go down on her feeding as we watched her belly get bigger and bigger. I don't know why a fever in Audrey equals not gut motility but that's what happens.

Now it's Thursday. Last night she had her last dose of antibiotics, blood culture still negative. Her feeding went back up to her go home rate. The TPN nurse is scheduled for 10 AM this time. We are cautiously optimistic she will make it out the door! Her supplies are delivered, everything at home is ready, now it's just us learning how to do her TPN and then we are good to go. We are terrified but we are ready to go...

2 weeks and counting

2 weeks in the hospital. I know many people stay longer but this is our longest stay yet and I am more than ready to go home. Yesterday was the much anticipated family conference. It happened a day later then planned and was more conference and less family, but finally we got her major doctors in one room at the same time to talk about Audrey's plan. Here is what they came up with:
1. Audrey has a "J-shaped" stomach. Shouldn't impair feeding or be affected by her g-tube. Not the reason why her stomach doesn't work.
2. Her stomach and small intestine definitely have dysmotility. Her stomach doesn't seem to work at all and everything else takes forever to get through the small intestine.
3. On top of her dysmotility she has this altered pain sensitivity. She seems to be lacking (or they aren't working) the nerves which turn off the pain signals. We had suspected this as she is still in extreme discomfort from her g-tube at the insertion site.This is why she has so much pain at a higher rate of feeds.

So given all this Audrey is at 12ml/hr of feeding. She is up to full strength but has too much pain when we go above 12. Her goal rate is 35ml/hr. Our plan now is a new medication to help with the pain. It is odd to give an antidepressant to a 2 year old for pain, but I understand the chemical process and why it may help. The medicine is not without side effects and reading them made me have double and triple thoughts, but ultimately we have to do something for her! We are also going home on TPN with her central line. This was exactly what I wanted to avoid. We have enough trouble keeping her well, now we have a line to worry about infections too. The thought of trying to care for her and still encourage her normal play and growth while hooked up to a feeding pump and IV pump and sometimes oxygen is just too much for me to contemplate. Before she carried everything in her backpack but that was as much weight as she can handle. She definitely can't carry a new backpack with an IV pump and TPN bag. Not sure how we will figure this out. It was presented as a choice but our alternative is to stay in the hospital until she gets to full feeds which they warn me may not happen. So there really is no choice. Before we go home the GI doctors want her on half feeds. So in the next few days we have to get her up another 5ml/hr which may prove to be impossible for her. I guess we will find out.

At least she is feeling better. Her weight is going up and she seems happier. Below are some pictures of her having fun. My fingers, legs and toes are crossed that she tolerates the increased rate and is able to go home soon. Wouldn't it be great if she made it home by mother's day??

She loves her tubie friend Cat! Cat has been very helpful for Audrey when she is scared of all these medical procedures.

Silly girl! Who knew a balloon covered in glitter would be such entertainment.  Needless to say she will have glitter everywhere for days!

Do not leave her unsupervised with a pen! She drew all over her PICC dressing too if you can see it.

The roller coaster that only goes down??

I am frequently reminded that patients with mitochondrial disease are on a roller coaster with several ups and downs. Audrey's roller coaster has only been going down. For a solid year now we have been getting bad news after bad news and she's had more and more interventions with little success. If this is indeed her roller coaster, we should be going up at any moment now, right??

So following her procedures Monday, they started pedialytle through her new j tube. It was started at 15mls per hour, which I thought was ambitious since her normal rate is 35, but what do I know. By Tuesday morning she had 400 mls of bile out her g-tube. Maybe mom does know something. The pedialyte was stopped and she was a happy camper. Tuesday went by well and Tuesday evening we restarted her pedialyte at 10ml/hr. In the morning she had 70mls out. Not too terrible! The resident came and said lets go up to 20ml per hour. Then GI came around and said lets elevate her g-tube. Within 2 hours my girl was screaming in pain and retching. The g-tube was put back to gravity, the pedialyte was turned off, x-ray came around. There was nothing remarkable on her x-ray, her g-tube put out 100mls in the first 3 hours it was down and she was restarted at 10 of pedialyte. It went well overnight.

So now everyone is stumped. Why is she putting out so much from her g-tube? Why isn't she tolerating anything through her j-tube? We were hoping this would be a temporary fix all but once again Audrey has to be unique. Nothing seems to be moving through her and we are at a loss about how to give her meds. If we put them in her g-tube she screams for the hour it's clamped and then the meds come pouring out when we open it. If we put them in her j-tube there is less absorption and she cries from the pain of it about 10 minutes after her meds are given. So this is where we are. 8 days now in the hospital and still losing weight, not tolerating feeds. Her roller coaster needs to go up any second now!

Not feeling good. Sleeping after screaming for an hour.

Poor baby girl!

On the right track

Finally I can rest. Sunday brought the scariest experience I have ever had with Audrey. Just as I was getting to go downstairs for the CureMito event, she woke up crying that her arm hurt. Her IV was out again. The doctors had decided that if she lost her IV they would place the PICC at the bedside with conscious sedation. Everyone was assembled and Audrey thought the hats and masks were kind of funny. The first versed dose makes a drowsy, goofy girl. They give fentanyl and she a little less drowsy and trying to sit up. Three doses of each are given and she is maxed for the amount she can receive on the floor. The doctor says sorry she will have to get her line in IR and everyone leaves. In the meantime Audrey is increasingly restless. She wants me to hold her but when I try to pick her up she starts choking me and pulling my hair. I try to put her down and she grabs on so tight I have bruises. She is shrieking and kicking, something is wrong! The nurse calls the doctor who says she was just disoriented, no way, this is a drug reaction! I hold her in my arms while she screams "Help me!" at the top of her lungs, kicking and thrashing around. We are both crying. I have never been so scared for my girl! Finally after an hour she falls asleep. She ended up sleeping for 12 hours! While that is normal at home, it's impossible in the hospital since they bother you so much. I thought about in January when she had fentanyl in the recovery room after her g-tube was placed. She acted in a similar manner (though not as severe). At the time we thought it was pain but maybe it was the medication! I call a friend who's husband is an anesthesiologist. He says some kids have disinhibition with fentanyl. I quickly google "disinhibiton and fentanyl" extreme irritability, agitation, outbursts, can have hallucinations. That was definitely her! She is scheduled for the morning for her PICC placement and G-J tube and I'm really scared we will go through this again.

Monday morning comes. I am sure to have my husband come because I am not strong enough to go through that again. A mom can only take so much helplessness and I knew I would have to leave the room if that scene played out again. We went down stairs and I made sure she was documented to have a reaction to fentanyl. I spoke with the radiology nurse practitioner, the physician placing the line, his nurse and finally the anesthesiologist. Making sure all of them understood what happened and that she was not to get fentanyl again. Off they took her. I was able to go into to floroscopy with her for the induction. They decided to give her only gas and they would try morphine if she need any pain control. She was such a good girl and so brave with the mask. Very quickly she fell asleep and I left. It's really hard to see your baby go limp and unconscious, even knowing that it is intentional. Though she was scheduled for an hour ant even 40 minutes goes by and our pager goes off. Shes done! We sit with her in recovery and after some initial confusion (they put her pulse ox on her thumb she sucks on!!) she lays quietly in my arms. Though she continues to sleep, at one point she asks for her blanket and then her hair before falling back asleep. This is the best recovery ever!! Her dad and I breath a sigh of relief. I think we have found the reason she has such a hard time with anesthesia and hopefully it is a simple solution.

So now her procedures are done. There is nothing else we need to do to her. She has her new G-J tube and she has a PICC so we don't have to keep poking her for IV's. Now it's time to start feeding her to get her to gain weight! She is on full TPN now that she has her line. Fingers are crossed that she is able to transition to feeds soon and start gaining weight.We are hoping we won't be here too much longer! Here are some pictures of her stay thus far...

this was at admission, not a happy camper!

so skinny!

this is how she spends her days

back from her procedure, doing well

"Cat" her Tubie Friend!

Finally feeling like playing! That is her PICC in her left upper arm and her new G-J is in the same spot as her old g-tube.

So that's where we are. Hoping she is able to get home soon!