We made the decision ...

Emily was discharged from the hospital last Thursday late at night, after a 16 day stay and antibiotic treatment for a line infection. She gets line infections because her motility is horrible, and causes a leaky gut. So we went home Thursday night, and I got her TPN prepared. When I lifted her shirt to connect the TPN to her Broviac, I realized that the nurse who discharged us must have pulled the line somehow. It was coming out of her skin a bit and had been bleeding. I took pictures, redressed it, and sent the pictures to the GI and TPN nurse. They called the next day, Friday, and said to bring her in for xrays. We had to be sure that her Broviac line was still in far enough to use for TPN. As I prepped us for a quick ER trip for an xray, Emily spiked a fever. Of course. So we headed to the ER, only 14 hours after being discharged. They did labs and blood cultures, but her fever had dropped and she was acting fine. They sent us home. Saturday night they called to say her cultures were positive and we had to come back inpatient. :( We went in late Saturday night, and got a room of the pediatric floor early Sunday morning. This coming Monday, Emily will go to the OR to have her Broviac pulled. The will place an IV so that she can continue some nutrition, although it won't be her regular TPN. She is scheduled for surgery Wednesday, to place a new Broviac. She is also going to be getting a tracheostomy. This was a hard decision. We have gone back and forth over whether she should have a trach or not. Her breathing is great, her lungs are clear. But her vocal chord spasms are out of control and there is nothing else we can do about them. When she has these spasms, she has horrible coughing episodes and gagging/choking spells. She desats, turns blue, but worst of all she really PANICS. It scares the crud out of her. We can't easily suction her, because of her oral aversions. I manage to suction her orally but it's not easy. Trying to deep suction her, through her nose, takes four adults to hold her down. It's not feasible. So in order to prevent aspiration and allow her to breathe without coughing and panicking, we are going ahead with the trach. It is an impossibly hard decision to make. I am trying to remain optimistic. I am hoping that she will be able to make noises and laugh audibly, even with the trach. This is big surgery she is having. She will be taken to the PICU after surgery. I will try and update here as soon as I can!

Where we go from here

We had TPN clinic yesterday, and I think I left equally frustrated as I arrived. It went beyond the fact that we weren't seen until 3:45, and our appointment time was 2:40. I make appointments for as early in the day as possible when we go to UCLA, because the traffic coming home gets horrid. It took me over 2 hours to get home, and it's only 42 miles.

The good news is that Emily weighed 15.9 kg, which is officially 35 pounds! So her nutritional status is good on TPN, at least it's doing one good important thing.

Her retching and vomiting however are uncontrolled. We discussed a few things, and for now we are going to try Baclofen. The thought is that it will relax the muscles in her stomach, and hopefully ease the retching. As a bonus, it should relax the spasicity and clonus in her feet/ankles. The downside is that it will further relax the rest of her body, which is already low tone. He said that if her drooling gets worse and she's choking more on secretions, we will increase her robinol. So we add a med, which will make us increase another med ... and then he has the audacity to say "She's sure on a lot of meds, we should see what we can discontinue soon". Seriously? Ugh.

If the baclofen doesn't work, we will consider a seperate j-tube. She doesn't tolerate gj-tubes, she vomits bile and screams with j-tube feeds. The thought behind doing it is that at least we could get her meds in her, without them being retched up. We leave her g-tube open to gravity 24 hours a day, except for right after giving meds. We're supposed to clamp it for 30 minutes to let them absorb, but I wait 60 minutes. Even at 60 minutes, a large portion of them drains out.

We go back in 2 weeks. I am crossing my fingers that the Baclofen works, without having too much detrimental effect.

Home again!

I'm sorry I haven't updated since the last post, which was quite a crisis time. She stabilized that night, and hasn't had another episode like that. Thank goodness! The metabolic geneticist came in the next day and talked to us in depth. He feels that the episodes were not true metabolic crisis but rather a reaction to pain. It makes a lot of sense, when we review it looking back. Her lactic acid was high, but that is expected when you are in pain or stress. This was the first time we had met the geneticist at this hospital, and he had a lot of good information for us.

He truly believes that Emily has some sort of maternally inherited mitochondrial disorder. I have reflux, migraines, fibromyalgia. Jacob has migraines and reflux. Emily has everything under the sun. We are waiting on authorization to do a mitochondrial gene array, and a whole exome study. He is also referring us to a different metabolic Dr, who is the leading mitochondrial specialist on the West Coast. His waiting list is about 12 months long, but hopefully he will get us in sooner when he sees how fast Emily has been going downhill.

GI wise, things are the same. She is on TPN for 22 hours a day. She retches a lot in the morning, but it's not too bad during the day. She manages to keep most meds down now. They started her on Rifaximin for bacterial overgrowth. It's a nasty, thick, orange medication. As long as we give it at a different time than her other meds, she usually keeps most of it down. As a side effect, her poop is bright orange colored which is interesting.

We go to GI clinic on Thursday, and then we are going out of town for four days. We are going to visit some friends we haven't seen in a while. It will be SO nice to get away for a bit! I can't wait. We have told Emily that she is NOT allowed to relapse, as we have vacation plans! :)

It's been a rough road but it seems to have calmed down!

I haven't kept this blog updated, and I feel bad for that. I hope that most of you have found me on facebook, because I am better at updating there. I have lost almost 50 followers over the last 4 months. :( Hopefuly I can earn them back!

So here's the recap. Emily had been retching and vomiting every morning for months. It started in January, and progressively got worse. She had been getting three bolus feeds of blenderized diet during the day, and 12 hours of g-tube feeds overnight through the pump. She started throwing up in the morning to the point that she was losing weight and becoming dehydrated. The GI said he thought it was seizure related.

In March it was so bad one day that I took her to the local ER, because I assumed it was out of control seizures. They loaded her with ativan and such and sent us home. The next day it was just as bad, so I drove to UCLA where we see neuro. We were admitted through the ER for an overnight VEEG. The vomiting and retching were unrelated to seizures. We were discharged after two days. We made it a week at home, and then had a GI appointment. I explained that it was NOT seizure related, and it was getting worse. The GI said (in his exact words) "She looks like shit. You need to be admitted right now". We were admitted to CHOC and began testing. An Upper GI showed a loose but intact fundoplication and no hernia. An endoscopy showed redness consistent with persistant vomiting but no abnormalities. Biopsies were normal. Her g-tube was changed to a gj-tube, and we tried feeding through the j port instead. A gj-tube goes in the stomach and then threads into the jejunum (intestine) to allow you to feed the patient and bypass the stomach. This made things much worse, she would scream in pain and vomit bile non-stop. She had a PICC line placed, and was sent home on TPN.

We were home for 3 days and then we had a pediatrician's follow-up appointment. She sent us straight to the ER. Emily was still retching non-stop, even on TPN, and had a UTI. They discharged us with meds.

Two days later Emily had her VNS (electric implant for epilepsy control) surgically placed at UCLA, she went home the same day. The next day we went to CHOC's ER for continued vomiting. This time she had an Upper GI done with small bowel follow-through, although it was done incorrectly so it didn't give them any information. They started worrying about pancreatitis or gallbladder problems. We did ultrasounds four times, a CT scan, and many xrays. She has sludge in her gallbladder but nothing more. During this stay she pulled her PICC line out and had to get a new one. We were inpatient for 8 days and discharged still on TPN.

Two days later we took her to the ER at UCLA because CHOC had said they didn't know what else to do with her, and she was still retching. She was diagnosed with another UTI and had another Upper GI done. She was discharged after 3 days.

Two days later (notice the 2 day at home pattern) we had to go back to UCLA to the Urgent Care, because she was throwing up the antibiotic they had prescribed for the UTI. She was then prescribed IV antibiotics which I did at home with the TPN.

The day after this ER trip, we went to the ER at CHOC because she was draining blood from her g-tube. They said it was to be expected when you retch as much as Emily has been. Her blood counts looked okay, so we were sent home.

Two days later (see!) we were back in the ER for screaming and retching. We were admitted to CHOC for almost 3 weeks. They ran more ultrasounds, xrays, etc. Durning this stay she finally had a Broviac placed. It came out two days later and was replaced. She was sent home again on TPN, and CHOC said they had no clue what to do with her. They kept saying she should go to UCLA and be considered for a small bowel transplant. But they couldn't tell me what was wrong with her, so how could they say this? It was a nightmare. At one point during this three week hospital stay we were told we should take her home on hospice. Ugh. The pain team was consulted and she got some relief. Thankfully we decided not to do hospice since, again, they had NO idea what was wrong with her.

We were home for almost a week and then took her to the ER with a fever. Anytime you get a fever over 101 with a broviac (central line) in place, it's an immediate ER trip for blood work. Luckily her labs were good, so they said it was a virus and safe to take her home. Two days later (damn this two day pattern!) they called back and said to return her immediately to the ER. Scared the crud out of me. Turns out she had a UTI and they needed to prescribe antibiotics. They couldn't just call them in to the pharmacy?

We made it almost three weeks at home, and then the retching got worse again. We went to a GI appointment and were told "Emily is a mystery. Somtimes we don't even try to figure her out anymore". That is not very reassuring! So we took her to the ER at UCLA because their GI and motility departments are award winning. She was admitted for 10 days. She was diagnosed yet again with a UTI. She had a gastric emptying study, electrogastrogram, and some test where they determine your caloric needs based on your O2/CO2.

During this last stay at UCLA, they managed to get her off of 6 meds CHOC had put her on. They started her on a couple of new meds. The biggest thing we did her was to compress her TPN to only 18 hours a day, instead of 24. In between, she now tolerates some blenderized diet again! Before we feed her, we give her something called "magic mouthwash" through her g-tube. It's a mixture of viscous lidocaine, benadryl, and mylanta. It numbs up her stomach enough that she tolerates some feeds.

She has finally gained some weight, she is 33 pounds! They would like her at 35. Remember she's almost 8 years old. She's teeny. :) She is finally into 5T clothing for once. We still don't know for sure what is going on, but we have narrowed it down a bit. We know her stomach works decently, it's all the small bowel and intestine that don't. What we will do about it depends on how she does over the next couple of months. The hope is that her GI tract will wake up a bit and she will be able to get off of TPN.

That is the long version. :) In the meantime, Jacob graduated from 5th grade and is now officially a middle schooler. Emily is a 3rd grader now. We are getting ready for Jacob's birthday party this Saturday, I cannot believe I have an 11 year old! I will post again soon, and hopefully be a regular updater again. :) Thanks for not giving up on us!

In and out, in and out

That's how we have been lately. In the hospital, out of the hospital. In the hospital, out of the hospital. Three weeks ago it was two trips in a row for seizures. Then on the 24th it was in for feeding intolerance, retching, pain. That stay lasted 12 days and we were finally discharged, but TPN dependant. Her g-tube was switched to a gj-tube, and she doesn't tolerate j-feeds any better than she does g-tube feeds. An endoscopy showed significant irritation in the esophagus, but nothing serious. An Upper GI showed that her fundo is intact, although a little loose. She had a PICC line placed (twice, since she pulled the first one out). We came home Friday on TPN 24/7. By Monday morning she was retching, gagging, crying again. Luckily we had a pediatrician's appointment already scheduled, and she sent us straight to the ER. They ran labs, bolused extra fluids in, and diagnosed a UTI. The GI said we should go home with zofran and antibiotics, and we were on our way. This morning she retched just as badly. I called the GI first thing this morning, because he told the ER he would see us today. Now it's 11:30 and they still haven't called back to tell me when we'll be seen today.

She is going in tomorrow at the other hospital for her VNS placement. I have explained all that is going on, and everyone is okay with her proceeding with the surgery.

Please pray she does well with the VNS placement, and that we really get to go home after like they say we should. Please also pray that someone can get to the bottom of her GI problems. Her gut is shut down for whatever reason. It's a mess.