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!

Now it's the weekend

Audrey is still in the hospital with no upcoming plans about going home. Yesterday she went down for an upper GI. Many people have these tests, I've had one myself and it is fairly non-invasive. However, my 2 year old is terrified of x-ray. I know this so I asked ahead of time for Child Life to go with us, hoping for better tolerance. She showed up with a basket of distraction tools which made her gurney ride through the hospital lots of fun. However as soon as we got to x-ray the crying and clinging started. The radiologist was very nice and understanding but Audrey doesn't seem to understand "lay still" "keep you legs straight" "no kicking" especially once she gets so worked up. So that took about an hour and then we had a few hours of x-rays in her room which barely went any better.

So, after much miscommunication the fellow finally came in to say she does have delayed gastric emptying, her stomach is shaped funny which may be part of the problem, and she has mild SMA syndrome. Huh? I don't know that one. SMA syndrome or superior mesenteric artery syndrome was described to me as something that happens when you quickly lose weight. the fat pad between the aorta and superior mesenteric artery, which gives blood to your entire gut, is decreased causing compression on the duodenum lead to a bowel obstruction. So the plan was to consult surgery and notify GI to find their recommendations but ultimately she really needs to gain weight. So of course, once the doctor left I hoped onto google. I know it's bad and I always advise my patients to be cautious of what they read but I can't seem to help myself. The first article I read said "this is a very rare but serious and life threatening event with a mortality rate of 1 in 3." What?!?! This seriousness was definitely not relayed to me by the doctor. So I continued my google search and found lots of interesting information, not all of it reassuring, but learned there are varying degrees of seriousness and hers is still mild. That being said it is so important to get her to gain weight to prevent progression.

So here we are on Saturday, and as some people know, nothing happens on the weekend in the hospital. I know my girl has this rare but potentially serious condition on top of her already-difficult-to-treat mitochondrial disease. We can't feed her through the g-tube and they weren't able to do the PICC line so she has an IV in her hand which does not last long. She had 4 IV sticks already and they will continue to add up this weekend. I know eventually they will go to her right hand. They have avoided it so far because she sucks her thumb, but it's bound to happen and she will not be a happy camper. I am obviously still very worried about though maybe I am relieved to find an answer?? At least this new problem has a potential solution where as everything else she has is just hoping and treatment of symptoms. So here's so to at least getting a plan for Monday since nothing will happen on the weekend!

Admission #3

For the third time in 9 months Audrey has been admitted into the hospital. I hope it is just a strange coincidence that she is here every three months and not a sad prediction of our future. This time we are here because she continues to not tolerate her feedings. It has been over 2 weeks since she was on formula. She is losing more weight each day and today she is so weak she cannot even sit up. I am glad that we came in yesterday, I would be much more panicked today at home if I had found her so weak.

I find myself struggling with the inpatient speed. We have been here for almost 24 hours and we've spoken to many different doctors who are going to contact the other doctors we've spoken to to determine a plan. In the meantime the only thing that has happened is she has an IV with fluids running. I know things run differently here and I know nobody has the sense of urgency that I have. It is my little girl laying here too weak to play. I will remind myself to have a little patience, but not too much as someone needs to advocate for her!

Unfairness

I know one of these times I will be able to post how well everything is going. It is probably just that when Audrey is feeling well, I am busy enjoying what she is able to do. When she is not well I need to turn to my blog to spew everything that she is going through.

Audrey continues to get sick every other week or so. While we are waiting to see an immune specialist, we are doing our best to keep her healthy. Since her last illness she has not yet made it back to her regular feeds. As a mom, there is nothing so distressing as not being able to feed your child. If I can't do anything else for her I want to at least be able to feed her. But apparently this is a big, long-standing problem for mito kids. Whenever their little bodies are stressed their GI system just stops. I know it's hard to imagine. She has a feeding tube to her stomach, how hard can it be to feed her? Well I can put the formula in but it will just sit in her stomach until she throws up or I vent her and let it all come back out. It's been almost 2 weeks since she was on full formula feeds. This mommy is getting worried! I'm watching her get more tired and lose more weight, knowing we aren't giving her what she needs to get healthy and beat this vicious cycle she seems to be caught in.

For one afternoon Audrey had fun. It was hot on Saturday and though she wasn't tolerating her feeding she woke up from her nap feeling OK. I let her outside with her brother to play in the sprinklers. Normal, 2 year old fun! She played for an hour and though I knew she was quickly burning through her energy stores, I didn't have the heart to stop her. Finally after an hour I made her come in. She quickly collapsed on the couch and there she stayed until bath time. She was wiped out Sunday and Monday also. How sad is a disease where one hour of play causes 2 days of exhaustion?!? It is so unfair that these children endure so much medical stuff all for one hour of play here and there.

In our quest to keep her healthy, we are looking into alternative care for her. She can't go to daycare any longer. Not only does she become ill if someone even looks at her, she just doesn't have the energy for it. When I picked her up today she was a mess. All of her feeding had backed into her bag, she was crying begging to go home and so exhausted she slumped forward in her carseat to suck her thumb since she couldn't lift it up to her mouth.. I can't watch her go through this. I know I've written about our struggles for services for her but it is so unfair! Every program or waiver I look into I get the same answer..."If she has medi-cal she will qualify." Her pediatrician wrote for home nursing care. The insurance company authorized 400hours per calendar year of nursing care. That works out to one 8 hour shift per week. The nursing company said not to worry, we can just put her on medi-cal and they will cover the rest. The problem is she doesn't qualify for medi-cal! We are doing everything in our power to take care of her and remain working, contributing members to society but the system is working against us. Instead of funding programs that help working families keep working, I'm encouraged to quit my job so my child can get what she needs. Unfair!

And that's where I am right now. It's unfair! It's unfair that my daughter can't eat any food. It's unfair that I am unable to provide her with the services she needs. It's unfair that she has to work so hard for just a little fun. It's unfair that she has to be affected by mitochondrial disease at all! No child should have to go through what she, and thousands of other children like her, go through every day. I know that there are many other children who are sicker than her, who are fighting for their lives or have already lost the battle. But right now, for my daughter, all I can say is it's not fair.