Waiting for more surgery

Emily is inpatient again. She had the UTI last week, and the antibiotic they had called in was not the right one. By the time the sensitivities came back, she had landed herself in the ER again. She had a fever of 101.2 and was trembling and lethargic. Her urine is still positive, so the antibiotic was changed. This time her blood cultures were positive as well.

Here's the thing. We go to "First Class Big Huge" University Medical Center. Last admit, they transferred Emily to the sister hospital of this one, in the next city over. Same University affiliation but NOT the same level of care. It was awful. Her central line had been placed on Feb. 28th, and by March 3rd it was out of place. I had been telling them that the line was sliding in and out of her skin again, this is what caused the line to come out on Feb. 27th. So I wanted a different type of line, or a different type of securement. This is the fourth time a Broviac has slipped out of her chest without being pulled. Obviously, her body doesn't like this type of line and the internal cuff does not develop scar tissue to hold it in place. The surgeon at the sister hospital said he would be placing a Hickman when he took her to the OR. He specifically reminded me that her current (4 or 5 day old!) line couldn't be fixed because it had slid out of the skin some. They never, ever, ever re-instert a central line that has come out of the body. It is almost guaranteed to become infected. Imagine my surprise when she came out of surgery with not only NOT a Hickman, but the surgeon told me that he had re-inserted the line she had in. My jaw dropped.

Prior to her going to the OR, I had asked the surgeon about a port in place of a broviac. I was told no. He said that "First Class Big Huge" University Medical Center does not place ports on kids. Never. Not ever. I said I didn't believe this was true, that I knew kids at our regular hospital (not this little sister hospital) that had ports. He said they must have been placed elsewhere. Oh. *shaking my head in disbelief*

This leaves us where we are now. Inpatient at our regular hospital, because I refused transfer back to the sister hospital. We will never go there again. Her central line is infected, and has to be pulled. They are now saying "It may have been caused by the surgeon's actions, but we have no way of knowing that". Keep in mind that even after the surgeon re-inserted her line, it still continued to slip in and out with each dressing change.

I am so irritated. Had the surgeon done the right thing by either putting a new Broviac/Hickman in, or placing a port, we would probably not be inpatient right now. Yes, she has a UTI, but we could have continued to treat that at home. The bug growing in her blood is not the same as the bug growing in her urine, so it did not translocate internally. This is a different infection. So frustrating!

In better news, she is not too miserable. She is sleeping a lot, almost all day. She is not running a fever anymore, and the trembling has stopped. She is getting two different IV antibiotics for the next 14 days, so we are inpatient for a while. After that time, we will most likely be placing a port. Please continue to keep Emily in your thoughts and prayers. No surgery is easy on her, and her other issues continue to get worse. Her sleep study showed a lot of central apnea. Her neurological status is worsening. Her general instability is huge. This (undiagnosed specifically) mitochondrial disorder is kicking her butt. She still continues to smile at us, through it all!

Bad blogger .... bad blogger!

I just typed an entire long update and then my computer ate it. :( The short version is that Emily is home right now on IV antibiotics for a urinary infection. We should know later today whether we can continue to keep her home and treat the infection or if we will end up inpatient. It depends on some final labwork. Emily had a sleep study last week, to figure out the cause of her desaturating during sleep. I know she did it during the study because they had to keep calling the pulmonologist to ask what to do. We started out on room air, and then had to keep going up and up on the oxygen until she hit 2.5 liters. So hopefully the results will be in soon and we will know what to do next with our girl during sleep. Emily had a (second) muscle biopsy done a few months back, looking for a mitochondrial diagnosis. Unfortunately there was a lab error and the sample never got to Baylor University. We have come preliminary results which are pointing towards a COX10 Deficiency. This diagnosis fits Emily's symptoms, unfortunately there is no treatment or cure. Because the sample cannot be formally tested any further, we are drawing labs on Thursday for a whole exome study. They will draw blood from Emily as well as her Dad and I. Hopefully this will (FINALLY!) lead to a diagnosis for Emily. That's about all that's going on right now! We take this all day by day, and today's goal is to get good news from the lab and be able to stay home with our girl. We don't want another admit, again! We just had a week long admit for a central line replacement, because her line came out. We would love a long, long stay at home now. :)

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!

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!