MRI today!

Last month, KayTar had a really strange day. It was a Saturday and we were just hanging out at home, having a relaxing kind of day, and KayTar kept saying, "Am I dreaming? Is this a dream??" in a very serious way. She was almost to the point of being a little panicky about it. The first few times, I didn't really pay much attention, but as it continued, it definitely caught my full attention. She was having real difficulty knowing if the world was real or if she was suddenly in a dream, in fact, she even went upstairs to check and see if her room was still there! It happened maybe a dozen or so times through the day.

We talked about the feeling she was experiencing. I asked if she was feeling floaty or light-headed (thinking it might be migraine/aura activity) and she said no. I described deja vu and she said it was not that either. She tried to draw me a picture, it showed her with a thought bubble that said, "Am I dreaming?" and at the top in capital letters, she wrote "NOT DEJA VU!" It wasn't elucidating, but it showed how much she was trying to communicate this feeling to me.

I called her neurologist/headache specialist on the following Monday and the nurse called me back and said not to to worry about it if is was an isolated day, but if it happened again, to call them back. It could be a migraine phenomenon or a simple partial seizure. Simple partial seizures are not the "normal" kind of seizure and the main difference is that the person experiencing them stays conscious for the seizure. If KayTar is having these, it is probably either a sensory seizure or psychic seizure, based on her description (click for more information)  A couple of weeks later, it happened to her at lunch at school. Again, it was short-lived and she came out of it on her own, but I still called the neurologist to let her know. She didn't call me back, but the following day I got a call from central scheduling about a new MRI. So that is what we are doing today and why we are doing it, and they will also be getting images of her brain stem for the pulmonologist. If the MRI comes back normal or KayTar-normal, then the pediatrician will be ordering a new EEG...and if it isn't normal, I'm sure there is a set of testing awaiting us behind that door, too.

The pediatrician wrote an order for half-normal saline and D5 to be run during KayTar's MRI, so hopefully we can avoid those labile glucose levels she has experienced after the past two sedations...and I'll be sure to tell everyone NO LR! Fingers crossed for a smooth day today!

***

I intended to post this before we left this morning, but my browser locked up on me before I could! We are now home from the MRI and it went swimmingly! Everyone listened to my requests/the pediatrician's orders, she was giving D5 1/2NS  as requested, put to sleep with propofol in her IV instead of through the inhalant mask (which she HATES), and it went really well. She had the same nurse anesthetist as her last surgery (when recovery didn't go so well) and she remembered her (and the rainbow IV arm she requested) and it made me feel much better to have her in familiar hands! She woke up wonderfully and her glucose has been GREAT and STABLE! I'm so happy!

She wore her sock monkey hat to the hospital (which she got loads of compliments on from doctors, nurses, the valet guy, and random people in elevators!) and on the drive in she told me, "If they forget to take off my hat, they will get pictures of my monkey's brain and the report will just say 'Stuffing.'" It cracked me up!

She brought Mrs. Chicks with her, a pink gorilla who shares an affectionate nickname with KayTar's school nurse (which is who gave her the gorilla), because she wanted to feel like she was close to her while she was in the hospital. Mrs. Chicks got an MRI, an IV, and she got to go back to the scan with KayTar...I didn't even get to do that! Thank goodness for loving gorillas who take good care of her when I can't be there. ;)

Gorilla MRI

Pokes for everyone!

Recovering nicely. :)

And my favorite picture of the day...

My very favorite monkey! ♥

All in all, it was an extremely smooth procedure and I'm so pleased that the new protocol worked well for her!

Following Up.

Today was KayTar's follow up with her mitochondrial specialist. It wouldn't be a visit to that particular clinic without a little insurance drama, so at check in they told me AGAIN that they don't take our insurance and I explained AGAIN that the doctor is in network for our insurance and we have seen her successfully before and our insurance has paid for it. She called billing and said that we would have to pay for the visit out of pocket and then file for reimbursement...it is never awesome to hear you are going to have to pay 300-400 dollars our of pocket as a surprise, but we didn't want to back out of the visit at that point, so I agreed. I have no doubt my insurance will pay as I've called them about 15 times about this situation and they always, always insist the doctor is IN NETWORK and needs no further authorization. It is a very frustrating situation, but hey...a few years ago we didn't even have insurance to get frustrated about, so I know it could be much worse than this. And the truth is that insurance is more than willing to pay, the clinic just doesn't seem to want to do the legwork.

On to the actual follow-up part of the appointment...R came with us again, in case KayTar needed a little attention while I was talking to the doctor, but it was unnecessary (but fun to have her company, as always) as KayTar was completely entertained with the two brain models that were in our room, along with a clear plexiglass head to put them in. She pretended to do brain transplants for most of the visit. At one point she changed from a brain surgeon into a zombie and cut the brain into "brain pie" and served it up to us from the head/bowl. So many fun things to do with brains! She really, really wants her own model brains (and tuning fork) now. As for the medical part of the visit, KayTar's labs came back within normal limits and her MRI was read as normal, too. I'm somewhat confused about the MRI as she has had 2 previous MRIs that were abnormal. I'm not complaining about a normal brain, but I'm not really sure how it went from abnormal to normal. She hadn't looked over her previous films (she didn't realize she had them), so she couldn't really explain it to me. She did say that certain metabolic diseases can present with brain abnormalities that later normalize. Also, two vials of KayTar's blood from last time was lost, so we had to re-draw labs for the acyl-carnitine panel and glycosylated tranferrin panels. KayTar was not a fan of the lab work, BUT the tech got it in one stick, so I was pretty pleased. The doctor said she is not going to diagnose her with mitochondrial disease because in looking through KayTar's medical files, she thinks that KayTar may have a treatable metabolic disease and she isn't ready to stop looking at this point. After mitochondrial disease is diagnosed, the search is off and we're not there yet. I nodded my head and said, "Yeah, okay." and she said, "Wow, you took that better than I thought." I guess some people come in there really looking for a diagnosis and take it hard when it isn't there right away....or maybe she is secretly reading my blog (yeah, right) and knows how much I want an answer for all of this. Yes, I'm looking for a diagnosis, I practically dream about it...BUT I want the right diagnosis when all is said and done. We started seeing this doctor because we were told she is the kind of doctor who cares about finding the right answer, whether it is mito or something else, and thinks a diagnosis is important. In our experience, many doctors don't feel this way, so I'm glad that she is committed to helping us figure it out, regardless of what the ultimate outcome is. She is referring us to a metabolic geneticist that she works with regularly and after we see her, the two of them will formulate a plan together...it will likely include another fasting study which is my absolute least favorite test of all time. I wonder if *I* can be sedated for this one. ;)

After the appointment, M (who is my savior in this clinic, she has been so patient and helpful through all of this which allowed me to keep my sanity!) came in and said we didn't have to pay up front, because she told billing they had to bill my insurance, because we already have proof they will pay. She told them that they either need to get their doctors pulled out of our plan so we can apply for out-of-network care or just agree to bill the insurance. Yay! But then I came home to a message about KayTar's pulmonology visit next week at the same facility...they said if we don't self-pay up front for the visit, they won't see us. The same goes for further appointments with the doctor we saw today. Since the metabolic geneticist we are being referred to also works out of this office, I'm sure the same goes for her. AGH! It took over an hour to get KayTar's pulmonology visit scheduled because the schedulers were insistent that they do not take our insurance...so I know the same will happen when I call to get her in with the geneticist. All of these doctors are in our provider directory, but that office just doesn't want to deal with our insurance because the clinic is not contracted with our plan. So now we are looking at possibly switching plans to see if we can get both our hospitals and all of KayTar's medical team covered, which span the two different hospitals and affiliations with two different medical schools...but I'm a little scared to switch based on a provider directory, as I've learned just how WRONG they can be! All of KayTar's doctors are listed in our current directory, but we are still having major issues with it. It doesn't really seem right that if insurance is willing to pay, the clinic can still keep us from being seen, but it seems there is little that I can do about it. I guess I should look at it as a learning experience, because by the time I am on the provider side of the medical billing process, I'm going to know more than I ever wanted to know about things like this!

Doing Better!

Yesterday was rough, at least at the start. In spite of me getting up at 2am to re-dose her with Zofran and again at 3am to give her fluids...she woke up with a glucose of 55. That is NOT what we want to see! Her glucose was very sluggish to respond, but she was tolerating clears by g-tube, so we kept at it and it started to come up. She needed Zofran through the day, but in the afternoon we were able to get her back on formula rather than just clears. In the evening, we trialed her off of Zofran. It started well, but about an hour and a half after her dose would have been due, she started complaining of a "hurricane in [her] belly", so we gave her a dose and went back to clears for a bit. She only urinated once yesterday, but we got in 30 ounces of fluid...so by bedtime, things were looking up. This morning she woke up with a glucose of 84, MUCH better! She's off Zofran and back to normal (well, her new level of normal) feeds. Yay! Oh, and I was pleasantly surprised to get an email last night from someone in her mito doc's office to check on how things went with the MRI. I'm sure she wasn't expecting anything to be amiss and got more of an answer than she was expecting, but it was nice for her to think of KayTar, especially when she wasn't even in the office! Note to self: If I can't be the kind of doctor who has gobs of time to communicate with all my patients personally, at least be the kind of doctor who has a super awesome staff. It makes a HUGE difference in how cared for a patient/family feels.

I'm still not sure what happened. She's been under anesthesia many times prior to this test and she has never reacted this way. Our pediatrician is going to request the list of medications and fluids that she was given, so we can figure it out. The only thing I know she was given was lactated ringer, which is contraindicated in mito/metabolic disease patients due to the risk of metabolic/lactic acidosis. She isn't diagnosed with anything specific, soooo I didn't speak up when I saw them hook her up...maybe I should have. Hindsight and all of that. It may not be related to that at all, though. Hopefully we can determine what the cause was an avoid this in the future. The other issue is that she has been in a bit of a decline right now anyway, so perhaps that fasting was just too much for her at this time. Not knowing what exactly happened makes me a wee bit nervous about the fasting for her gastric emptying scan on the 29th!

I'm also a little nervous about school starting this week (has summer flown by or what?!). Classes start for Josh and I on Monday (Calculus, agh!) and the kids start on Tuesday. First and fourth grades! My BubTar is in his last year of elementary. How does this happen?! I know KayTar will be in great hands at school; I met with the nurse, new librarian, her teacher and intern (a male intern, and KayTar's first question about him was "Is he HOT?" He is young and handsome, so I told her yes, but followed it with "No flirting with teachers!" and she said, "Well, I can flirt in my mind." Oh Lordy.) , on Wednesday to go over the plan...which is less of a plan and more of playing it by ear. She will be trying to wear her pump backpack all day now, because of her frequent feeds and I have no idea how it will go. I hope she transitions back to school well, but if we do hit any bumps that need to be ironed out, I know that she has a very capable team to help figure it all out.

Can you believe this tiny little thing has grown up into a big first grader?!
She was about 3.5 here.

She saw me looking through these old photos and said,
"Oh my gosh! That's my first jumper! And my first hearing aid! You took pictures?"
LOL. Of course I did, kiddo!

One of those days!

It is 3:30 in the afternoon and I've been awake for 12 hours already. Yeah, it has been quite a day!

Yesterday, the hospital called me and told me that KayTar's MRI slot was 5:30am. With the hospital about an hour away, and KayTar having to be totally NPO at 4am, I had to be up by 3:30am. I *almost* overslept as my alarm didn't go off, but KayTar slept with me last night and started talking in her sleep at exactly the right time to get us out the door on time. When we arrived, there was no one in the surgery area except for another mom/daughter duo. Someone came in around 5:45 and told us that they open LATE on Thursdays, so nobody would be there for a while. Uhh? Why did we both get a 5:30 time slot if nobody was going to be there that early?! They took us back to the PACU pretty quickly, but it was between 9:30 and 10:00 before she ever went to imaging. She was pretty anxious and teared up a few times during the wait, she was scared they would use the mask to sedate her and even though everyone promised her they wouldn't, she couldn't quite get over it. The saddest one was when she said, "But why do I need this test? What is wrong with me?!" I think she is getting to the age where she wants a reason for these things and we can't always provide them, being in the dark as we are. Luckily there is wifi in the hospital and we were able to stream some Phineas and Ferb from Netflix via the iPad to keep her distracted for most of it.

A girl and her (MY) iPad.

The light fixture in her PACU room.
She said they chose butterflies just for her.

We got to be bracelet buddies.

Lidocaine patches are AMAZING!
Our normal hospital doesn't use them, but wow, did they work!

She was in the scanner for about an hour, during which I obtained coffee and a pastry and entertained myself by watching The Office on my iPad. 3 episodes later and I was called back to recovery! She was already awake when I got there, but she was feeling super loopy. At one point she started slapping herself in the face and when I asked her what she was doing, she said, "I'm feeling too woozy!" I convinced her that resting a little would be more effective than slapping herself to attention. She drank a little water and fell back to sleep for a while. The entire time she was in recovery, her monitors were alarming. It was almost continual bradycardia/low HR. She came around and said she felt like she was going to puke, so they ordered some IV zofran. We waited a while and they had me tube her 2 ounces of Pedialyte. Everything seemed fairly okay, so we were discharged around noon.

Shortly after getting home, she vomited up 150cc's of fluid. 60cc's of Pedialyte and the rest was the water she drank for me. UH OH. Puking with zofran on board after fasting for so many hours is bad news for our girl! Her glucose was okay then, 77, but I emailed the pediatrician to be safe. She told us to give her 15ml of Gatorade every 30 minutes. I did that successfully 3 times, but after the last time, she got gaggy and said she was going to throw up. She didn't, but we're walking a fine line here. We're going to wait an hour between fluids this time to try to avoid more vomiting. Her glucose is down to 71, which is still fine...but trending the wrong direction. Hopefully, she will turn a corner soon and we'll be able to give her the fluids she needs! After waking up at 3:30, a trip back to the hospital sounds like even less fun than usual!

PS: Things were touch and go for a while today, her glucose was dropping (61) and she started feeling queasy ever with the tiny amount of Gatorade we were giving. She hadn't peed since 6am or so. However, she ate an entire Popsicle--first time ever--and her glucose started responding (114) and she peed (high ketones). She had another dose of Zofran shortly after, and I ran in 2 ounces just a few minutes ago. I really hope she's on the other side of this now and we make it through the night and morning just fine. It looks like I'll be short on sleep again tonight, making sure she gets her fluids!

It is true, some doctors are irritating.

I spent the latter half of Friday taking KayTar to her endocrine appointment and acquiring medical records (that the hospital failed to send) for the new doctor she is seeing. The endocrine appointment was frustrating and unnecessary. Endocrine appointments always seem like a bit of a waste now, because KayTar carries a ketotic hypoglycemia diagnosis and they are done searching for an underlying cause. It is basically a check in, which I don't mind, but the attending who came in at the tail end of the appointment was really aggravating. She had never seen KayTar before and clearly had not read her medical records. She came in, observed a normal looking kid with a g-button, questioned me about why she has a g-button (because she has a feeding disorder and her other physicians and I felt it was the best route to ensure she receives adequate nutrition, perhaps?), told me that she probably has ketotic hypoglycemia because of the g-button (she blamed it on dumping, but I don't buy that for a second with KayTar's history, she was becoming ketotic and hypoglycemic before she ever had the tube, we just didn't realize it until we took nutritional deficiencies out the equation with the g-button), and implied that we needed to try to get rid of the button so KayTar's issues would go away. Never mind that the g-button is the lynch pin of this whole "healthy kid" thing KayTar has going on these days. After trying to briefly explain KayTar's history (hearing loss, brain lesions, history of global developmental delays, feeding disorder, ect.) I mentioned that she was seeing a neurometabolic specialist who thinks that KayTar has an underlying metabolic disorder, possibly mitochondrial disease, and she stared at me for a prolonged amount of time...you know, an AWKWARD amount of staring time...then said, "Well, she looks fine to me." Thank you, Dr. I'veNeverMetYouBefore! Clearly, you are more in touch with my daughter's complex case than the other 8 doctors she is followed by! As an endocrinologist, you certainly understand my daughter's GI functioning better than her GI doctor. As a doctor who has been in a room with her for 3 minutes, you definitely understand the complexities of her case more than her pediatrician of 5 years does! Oy. I know I want to be a doctor, but this is the kind of doctor I DON'T want to be...the kind that makes uninformed judgments based on superficial interactions and doesn't listen to the patient/family. Truthfully, it doesn't matter. We all agreed that the plan is to keep doing what we've been doing, since KayTar is doing very well with it, but it was just irritating that she felt that she needed to give commentary on issues that she has a limited amount of information about and are not in her realm of expertise.

I also went by diagnostic imaging to pick up a CD of KayTar's MRIs for her new doctor and they had accidentally burned a second copy...so now I'm the happy owner of my own copy of KayTar's MRIs! Doesn't she have the cutest little brain ever?

Here she is, as an alien in a scrub mask...

And this is what it looks like when KayTar's brain feels sad...

(see its cute little frowny face?)

Yes, I had way too much fun looking at her images on Friday night. I don't know whether to blame it on the premed thing or the parent of a medically-complex child thing...but yeah, I spent my Friday night scrutinizing MRI images. Fun times. ;)