Another week, another ER trip

Emily earned herself a trip to the ER yesterday, after a long 4 days of trying to avoid it. On Friday, she had a bronchospasm that lasted three hours. It was horrible, but she was managing to maintain her oxygen saturations, so I waited it out. It did worry me and I packed a bag for the hospital, but then of course the spasms stopped. It was definitely the scariest breathing episode she's ever had. The GOOD thing is that it proved that the trach was the best thing we did for her. She was able to breath and get nebulizer treatments and suctioning without a fight. What a relief the trach is! Saturday she had another bronchospasm that lasted an hour. During the spasm I gave her a dose of Ativan, thinking it might calm her down. Thirty minutes later she was still spasming so we gave her breathing treatments even though they weren't due for an hour. Then she got some other medications that she was due for, and finally it calmed down. This time when she was spasming, she was not maintaining her saturations. Again, thank heavens for the trach! I was able to hook her up to oxygen and manage her at home. I did page the pulmonologist when this episode finally ended though, because I needed help with managing these. She called in a prescription for Atrovent nebulizer treatments, and told me to try that along with the Ativan in the j-tube next time it happened. So of course, Sunday, it didn't happen at all. We thought that whatever it was, was over. Until Monday morning, when she woke up doing it at 8 AM. Her morning nurse banged on my door to wake me up (Don't judge! LOL Jacob had the day off from school so we were sleeping in). She said "She's not breathing good! Come see please". Ugh, nothing like that to get a Mama out of bed quickly. I went in, and she was spasming again. We gave Atrovent via the nebulizer, Ativan via the j-tube, and waited a little. It stopped after about 20 minutes. We thought we were good, and then it started again. It went on, off, on, off. I paged the pulmonologist again and said we needed to be seen. She told us to come to the ER. We went in and did a chest xray, trach sputum culture, and some swabs. Her xray was normal and her swabs for RSV and Influenza A & B were negative. They tested her CO2 end tidal volume, and it was good. The decision was that we could go home, and raise her oxygen a bit while we wait for a sleep study. We know she has sleep apnea, and it's most likely central apnea. The pulmonologist feels that she will end up on a vent when she's sleeping, and wants to do part of the sleep study with her awake. Seems like an oxymoron to me. ;) She wants to get a better idea of what the spasms are. So, here's to another week of another journey. Hopefully it was the last issue we will have for a while! We have been home from the hospital for 3.5 weeks, which is a pretty good run for us lately!

Doing well at home!

Emily had trach surgery on January 19th. She was discharged directly from the PICU on January 27th, the day before my birthday! What a great birthday present. She did great during the trach surgery, and was smiling within 6 hours of surgery. She is amazing! She came off the vent in recovery and was off oxygen and on room air within 24 hours. :) She's a rock star!

Being home was a bit rocky at first, but we are settled in now. There is quite a bit more care involved now, and leaving home is quite a feat. The number of pumps/machines/bags that must go with us is a bit nuts. In the end though, she's doing fantastic so no complaints.

Tomorrow is a big day for us. We are meeting with the most involved in Emily's team of doctors - GI, TPN nurse, Neurologist, Palliative/Pain Dr, and social worker. We have been asked to sit down and detail what we want in the future for Emily. What our choices are about her medical care. For example - if she was to become septic and need to be put on a ventilator, I say do it. Give her a chance to fight the infection and recover. But if she is no longer alert and aware and isn't going to recover and come off the vent, I don't want her on it. I am not willing to keep her alive in a shell of herself. Her personality, her facial features, her love - those are Emily. If we lose those, we've lost her. This meeting is not going to be fun. I am happy that my husband has taken tomorrow off of work and will be going with us. Emily has some new nurses finally, and a nurse will be with us as well to keep Emily occupied and entertained. If you have a moment, please pray that we get through this meeting and can come to some decisions. Look at this girl - this is what we fight for!

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!

Seizures on the bus

Emily has had a rough week so far seizure wise. She hasn't had any big seizures thank goodness, but it seems like non-stop small ones. Yesterday after she left school in the afternoon she had a seizure on the bus. Her head dropped down and didn't come back up. The bus driver pulled over and checked on her. She was breathing fine, but was completely unresponsive. She called dispatch and they said since Emily was breathing okay to take her back to school. When they got there her health tech came out and checked on her. She said her breathing was normal and her pulse was fine, although she was still nonresponsive. They decided it was safe to take her home on the bus, so she came home.

The bus driver was freaked out, and I guess she had a seizure on the morning bus ride as well yesterday. So we have two freaked out bus drivers. I totally understand, they can't be worrying about Emily's seizures while driving. It's not safe for them to constantly be looking in their rear view mirrors at her to make sure she's okay.

Emily has never had an aide on the bus, and she's ridden since the day she turned 3 years old. I called the school district this morning and spoke with the head of special education. Getting an aide on the bus is a long process but she is going to try and rush it through as urgent. Hopefully it won't take too long.

In light of the increase in seizures, I called to check on our UCLA follow-up appointment. When we were discharged on Nov. 10th, we were told to see our neuro in 2 months. Our appointment is set for Feb. 28th! That is NOT 2 months, that's almost 4 months! I was pissed when the lady was talking to me on the phone today. I told her that Emily is having more seizures than normal, as we wean the Trileptal. She is on Keppra and a small dose of Trileptal, and that's it. The Mysoline was weaned off when we were inpatient. We are supposed to start Felbatol or something, but I have no orders. We were supposed to get them at the 2 month appt ... which is now a four month appointment.

Her response was "Well, I recommend you email the doctor directly then and see what he says. I can't get you in any sooner".

Ugh. Her last comment was that perhaps I need to just take Emily to the UCLA emergency room and that way she will be admitted and see immediately. That is NOT a fun idea!

She seems to have a lot more seizures in her sleep now too. Almost every night she wakes up after 3 hours or so having a seizure. She continues to twitch for another hour or two before she goes back to sleep. This is not a great pattern! We are all exhausted, and it makes me wonder how much she seizes all night long.

I wish we could get in to the neuro quicker! Wish me luck that my email to him gets us somewhere.

Home from the hospital with some answers

We were inpatient for 4 days of continuous VEEG testing, and a PET scan. They end result is that Emily is having myoclonic and atonic seizures all day, every day. She has them so often that the neuro said "There's one. There's one. There's one" in between sentences while talking to us. Ugh.

There is no focal point for these seizures, they come from all over her brain. We didn't manage to capture any tonic/clonic seizures, so we don't know if the big ones have a focal point or not, but it doesn't really matter. We wouldn't put her through a resection or any other epilepsy brain surgery to try and control some of them, when she has so many others. The thought is that with so many small seizures, she is bound to have break-through big ones. So ... that's it. I am relieved she doesn't need brain surgery, but not so happy with the thought of her having seizures forever.

The biggest thing we got accomplished during this hospital stay was the actual diagnosis of Lennox-Gastaut Syndrome. It was mentioned to us in her last hospital stay, but wasn't verified. It was thought to be a casual thing thrown at her with no proof. Now it is official. Her EEG shows the classic slow spike-wave pattern of LGS.

We are weaning her off Trileptal, because it can INCREASE myoclonic and atonic seizures in LGS patients. Hello - we thought we were dealing with LGS a while ago, why did we get put on this med? We never had atonic seizures until we started it. So there is a possibility that some of her small seizures may actually decrease, as we wean this med.

If she continues to have seizures or has more when we wean the Trileptal, then we are going to try her on Felbatol. If that doesn't help, there was another med mentioned but I can't remember the name of it. The problem is that it is not FDA approved. You have to get it from a pharmacy in New York that imports it to the US, and pay cash for it. Insurance won't cover it. I don't even remember what it's called, but financially this is not an option for us. So if we need a new med, I pray that the Felbatol helps.

As I am typing this, she had four atonic seizures. She literally just fell over while sitting up and watching TV. It's heart breaking. I hate seizures.

At least we have an answer to this portion of the Emily Puzzle. We are still waiting on lab work that was sent out testing for Tuberous Sclerosis, Cornelia deLange Syndrome, and the SCN1 gene.

Nothing new to report .. just seizures and ick

Emily has had a pretty horrible no good bad kinda week. She has been retching and gagging like crazy, non-stop. I can't imagine how horrible it must feel. :( Her lips literally turn blue when she retches so much because she can't catch her breath. Her seizures are kicked up as well. Yesterday she seized on the walk to the bus, as I was putting her on the bus, and three times on the (hour long) drive to school. By the time she got to school she was post-ictal and lethargic and grumpy. They tried to help her relax and rest, but she continued to seize off and on. She only lasted until 9:00 before they called me to come get her. :(

This morning she had one pretty big seizure, and then only a few small ones at school.

I am so tired of seizures. I hate them. I hate even more that I don't even know what is or isn't a seizure anymore. She is such a moving retching sad thing, it's hard to tell.

Our testing hospitalization on Nov. 7th can not come soon enough. Yesterday was so bad, I almost drove her to UCLA to try and get in earlier through the ER.

Did I mention that I *HATE* seizures? Boo!

Home again, home again, jiggity jig!

First, I have to say thank you to everyone who reads here, posts comments, follows us on Facebook, etc. You guys are all awesome and you definitely keep my spirits up when I am stressed. THANK YOU! :)

We saw Dr. Lerner at UCLA. He was incredible. His personality is fabulous, he flirted with Emily, he was so good at explaining things, and I felt like he really wants to help. He asked me about a few tests that no one at CHOC would do, even when I asked about them. The first was that she has some strange patches of skin that are lighter than the rest of her skin. He asked me if anyone had ever mentioned them to me and I told him that CHOC told me it was a birthmark. He laughed and said "of course it is. But did they mention it could mean anything?" No. Never. So he went and got this fancy light (looked like a black light) and checked her whole body. She has quite a few places of abnormal skin tone. He wants to have her tested for Tuberous Sclerosis. He also wants to have her tested for an epilepsy gene, something I had never heard of.

The plan is that we are going to go inpatient at UCLA for a lot of testing. We are going to start with a 24 VEEG. If we catch enough seizures during the 24 hours we will go home. If not, we will be staying until we do. We are also doing a new brain MRI, and a PET scan. I have been asking about a PET scan for over a year and no one would listen to me. This was one of the first things Dr. Lerner asked if she has had done. I am SO glad he is being proactive!

His hope is that we can identify for sure that all of her seizures are coming from the same place. Or that at least MOST of her seizures are coming from the same place. The most recent EEG's showed spikes all originating in the left temporal lobe. If we can isolate the seizure location, then we can hopefully do a resection and remove that piece of brain. If they are not localized, or the area of the brain is not okay to remove, we will do a vagus nerve stimulator.

Hopefully the authorizations for these things will be done within a week or two and we will get in to UCLA for these tests in November. In the meantime, he gave me a new schedule on how to increase her Mysoline better. He said to contact him with any questions or concerns. He mostly does email contact with parents, which is AWESOME. I love that. I wish all doctors would let you email them!

It was a great appointment. I really liked the doctor, I felt like he really listened to me and my concerns, and is going to be a huge help in Emily's care. :)

Please help us raise money for our Children's Hospital! Donate to Princess Emily's team for the CHOC Walk! click hereAny amount helps, even a few dollars. :) Thank you!

Trying another new med

We met with the neurologist yesterday and he wasn't thrilled to hear of her having so many generalized tonic/clonic seizures lately. We don't normally have this many (grand mal) so it was definately out of the norm for her. He always says he thinks "it's a progression of her epilepsy" which is concerning.

We are weaning off the Topamax, since it didn't help her seizures and in fact seemed to make them worse. Plus all she does is sleep and whine while on it. We are starting Trileptal in it's place, to go with the Keppra we are still on. He explained that Trileptal works for focal seizures, which is what her EEG showed last time. Then they spread to secondary generalized ... so if we could stop the focal seizures, we should be in turn stopping the grand mals.

Cross your fingers. He says we are getting to the end of his expertise, and the end of the meds available. We will be moving on to UCLA Epilepsy Center soon. Our neuro is private practice, so there's no one else there to consult with or get a second opinion from. He said he thinks we will need to start discussing vagus nerve stimulators or brain surgery. Ick.

Does anyone have experience with VNS? He thought that the patient has to be 60 pounds to get one. We are barely 30 pounds, and not getting bigger any time soon. Does anyone know if that is true? I can't find information online since it is only "approved" for kids 12 and up.

Thanks in advance. And thanks for always reading our seizure blahgs. ;)