Update Number 6: Wizards!

April 4, 2013

Ellie Update: I just got off the phone with the woman who schedules appt's at Milwaukee's CVS clinic. She received our (huge!) packet and all Ellie's medical records (over 20 pages, geesh). Now we wait as Dr. Li reviews it all and then passes it back to her to do the scheduling piece. Dr. Li is currently out of the country. I sent the packet in 2 weeks ago and hadn't heard anything. I must admit I started second guessing myself. Like maybe Ellie isn't "bad enough" to go see the Wizard of CVS. But, that's just my insecurity and/or anxiety creeping up. It will be so so wonderful to talk to professionals who know about CVS.

This week we went down to Children's Hospital here and met with another wizard, but he is the Wizard of Sleep. Our regular pediatrician suggested waiting the 4-5 months for an appointment with this guy because he is a BIG wig as far as pediatric sleep specialists go. She figured he would know more about CVS and it's association to poor sleep. Nope. He knew of CVS but not much else, at all. That part was disappointing but he did have some really interesting thoughts about Ellie's sleep issues (she still wakes at least once a night, often more and often with stomach pain). So, we will do another sleep study at his clinic at the end of May. We also are going to go see another orthodontist who works with the Wizard of Sleep. Ellie has an "abnormal" cranial structure (in our house we just call that the Granstrom head) and he said a lot can be helped with airway issues through orthodontic work. There are more details but I think it's boring...

Something that Ellie and I are both pretty excited about is a CVS 5k run/walk the first weekend of June. Ellie keeps asking when it is. I think she is curious to meet another kid with CVS. Here is a link to the event: CVS Run/Walk This is the blessing of living so close to Milwaukee! It's a fundraiser of course, but I think it should also allow us to meet other kids/families with CVS. So far I only know of two other families. I have not met her but have talked to one other mom on the phone a few times and she is WONDERFUL! She has a teenage daughter who has grown out of CVS (and into migraines, but only occasional). I cried and cried when we talked because it was like hearing Ellie's own story--and there is just something powerful in knowing others have been through exactly this. I also cried and cried because this woman is so kind and supportive. The other is an old college acquaintance, but she lives far away and her daughter has numerous health issues that are much more serious than only the CVS. My point is, I'm kind of hoping we make a friend or two at this walk (Dear God: a 6-year-old girl friend would be perfect, Thanks). But if we don't that's ok too.

Ellie is currently on a fairly strong medicine that does seem to be helping. But neither B or I would like her to be on this medicine for a very long period of time if possible, hence heading to the Wizard of Sleep at Children's. He also would not like Ellie to be on this strong medicine. Ultimately the medicine knocks her out, makes her woozy in order to help her sleep more, in order to help her have less episodes. It is working, but the Wizard of Sleep and Brian and I would all like to figure out if there is a more foundational issue that needs addressing with Ellie's sleep. Right now Dr. S (sleep genius) thinks that there is still a breathing issue for E when she lays down. Anyway...for now I'm glad for the respite. We are having mini episodes (no vomit, just nausea and pain) about once a week and it's easily solved with zofran, snuggling and distraction. Our recent long road trip caused numerous mini episodes but that's just to be expected--excitement, poor sleep, lots of driving.

I hope this wasn't too long.
Thanks again for all the love and support!

Update Number 5: Our first CVS check-up

Thursday, January 31, 2013
So, I must admit, I just noticed that it's only really been about 2 months since Ellie was officially diagnosed with CVS and it feels like it has been months and months and months. I don't know why. Maybe it's because I've learned so much in such a short amount of time (regarding the illness and the best ways to help Ellie) or maybe it's because it's exhausting and a little overwhelming. Regardless, a little over 2 months and we had our first CVS check-up appointment this week with Ellie's GI doctor. Thought I'd share the update:

What I was able to report to the doctor was: we know Ellie definitely has episodes when her body encounters a virus. B and I agree that has been made crystal clear. She has an episode for what seems like no particular reason (meaning her sleep patterns were fine and no highs or lows in life that we could see) and then a day or two later she is sick with a bad cold. We know, as we have, that her sleep patterns also strongly affect her episodes--she has clearly had a bad or "off" night or two of sleep and then a night or two later--episode. And, Christmas made it also very clear that extreme highs set her off.

What the doctor and I talked about: Lately Ellie has had more episodes but they have been at least 50% less intense than they were for a while there. Not really sure what this means...one possibility is that she is recognizing her symptoms sooner. She yelled out the other night, "Mom! I need my zofran!" which was a surprise for many reasons, the greatest being that she knew the name of the medicine, but this might be an example of her beginning to understand and read her own signs and symptoms. We were all back to sleep within an hour that night.


Her doctor said again that the best maintenance for her CVS and the best cure when she is in the depth of an episode is sleep. He was surprised to know that Ellie's zofran alone does not "knock her out." And, it's been problematic trying to add the benadryl because who wants to take a shot of spicy berry flavor when they are on the verge of puking? He was also concerned with the amount of episodes she continues to have, even if they are less intense. So, we are trying a new medicine that is supposed to be preventative and has had some good results in CVS people. It's called Amitriptyline. Way, way back in the day they used it to treat depression but soon realized it was calming people and making them super tired but not doing anything about the depression...perfect for CVS people! Unfortunately she is so small that it's a pretty big dose because there would typically be no reason to give this to a kid. I don't love that, but he says lets try it for a month or two and see what happens. The biggest side effect is sleepiness, which in Ellie's case is the point. The other big side effect is that if you had a heart murmur this medicine could exasperate it. So, she had an EKG done. Oh, and we have to watch her weight. She is a fine and healthy weight right now but he says we don't want her to loose weight at this point. She lost about 3 lbs since November, so we gotta watch that.

Other interesting tidbits I found out this appointment:
I asked if he thought people have always had CVS and it's just now being diagnosed. He said yes, that's the assumption. He said that kids with CVS were said to have "weak stomachs" or depression or high anxiety, etc, etc. Now we know it's neurological but there is still a ton to figure out (like migraines, still so much unknown). He also said that for a long time it was assumed that this was mainly a childhood disease (that's what I've read too) but that now there is this whole generation of people who were diagnosed as kids or teens in the last 10-20 years and have grown up and the disease hasn't changed for them so they are moving away from labeling it a childhood disease but still about half the people diagnosed have much, much less symptoms as adults.  I asked how rare CVS was (I was wondering if I could meet other parents in our area whose children had it--HA!) He said that Ellie is his only patient right now who has CVS. That was shocking for me. I didn't realize it was quite *that* rare. He said that there are probably about 75/100 or so kids/adults in our area who have diagnosed CVS who just go to Milwaukee to see Dr. Li (the CVS guru)...and that's where we are headed. I asked if we could go see him, Dr. Li, to be connected, ask questions and help with research. He was clearly excited I asked that and said he will call up there himself in the next few days to see about getting us in sooner than later (meaning in the next 2-3 months).


And that's the update! A parenting lesson through all of this--as her Mom I dwell on her episodes, worrying, wanting to "fix" it, constantly checking my phone during the day in case school calls, etc, etc. It has been draining for me personally. BUT, as a happy, amazing, brave, sweet, bossy, confident, intelligent, stable 6-year-old--she generally has an episode in the middle of the night and the next day it is over and that is that. No dwelling, no worrying, just moving forward. It's been a beautiful thing to witness and learn from. There are exceptions of course, on Christmas day she was in the middle of a fairly strong episode. Lots of nausea, lots of pain and she sort of had a massive meltdown. I swear if she knew the word "fuck" she would have used it! She was yelling and crying and just generally pissed off. But, that's ok, it was a really long episode and it was Christmas and she felt awful. A little meltdown seemed right on track! Generally though, she has been amazing and I've made sure to tell her ALL the time how amazing she has been.

Ok, bed...
xoxoxooxo

Update Number 4: The CVS Informative Update

Saturday, November 24:
I'm hoping this is the last news about Ellie's health for a long, long time.

While we do have one more semi-serious test to do, our GI doc doesn't think we will find anything abnormal. It's a test that has a name that I can't remember. She will abstain from food for so many hours, then drink some "sweet" stuff that will make her insides glow, then they will take a whole bunch of pictures of her digestive system from top to bottom to make sure her anatomy looks normal. It's pain-free and precautionary, but really the doc says we can now officially diagnose Ellie with Cyclic Vomiting Syndrome (CVS). That's the one we were hoping for (well, I mean, instead of EE) and it's the one we felt like really matched what has been going on with Ellie.


Our doc had some different things to say about CVS than the other articles we've read. Did I mention that his previous boss/mentor is the leader in international study of CVS (which by the way is located at the children's hospital in Milwaukee)?!? I think this probably helped him recognize it quickly, although he told me last week that our previous GI doc was also trained under Dr. Li in Milwaukee so why she didn't catch it??...grrrrrrr. Anyway, he told us that it's a lifelong condition, she will have it forever but it could change as she grows up. About half of the people who have CVS will stop having the vomiting and instead have migraines as they become teenagers or young adults. He also talked again about how this syndrome is so strongly connected to sleep issues (which as many of you know has been such a HUGE problem for Ellie for all her six years). He said the greatest precautionary thing we can do is practice diligent sleep hygiene.

He said CVS is a neuro-gut disease. Something in her brain is wired wrong and when she experiences any sort of "off" or "unusual" in her life, her ability to sleep well and deeply is off which triggers her brain to tell her stomach and intestines to start freaking out. How is that for a scientific explanation? So, her brain tells her stomach to freak out and the only way to push the reset button and stop the pain, nausea, vomiting is to "snow" her. That means make her fall asleep hard and fast. I worry I am boring you, but fyi, I have to say I feel like I could write a really interesting parenting resource about this stuff--meaning, it totally makes sense for Ellie. Over the summer she would puke, puke and then sleep (whether for 20 minutes or 2 hours) and wake up wanting to play and eat breakfast. I would always think, how are you playing with dolls and asking about the beach when you were puking 2 hours ago?? But last week the GI doc said that it is fine for her to be "snowed" at school (meaning drugged), sleep in the nurses office and then return back to her classroom (really??!!) because the light switch is turned back off and a lot of times kids with CVS do feel totally fine. Of course, getting to this place will take a while and take practice and maturity on her part. Her last bad episode when we ended up in the ER she was pretty wiped out for 2 days, but that's partially because we didn't know what it was, she puked about every 2-4 minutes for well over an hour and so her little body was exhausted. But then her last episode that I'm pretty sure we escaped, I quickly gave her the zofran, she slept and woke up fine. And, she hasn't vomited since then!! In fact, this is the longest stretch of no vomiting since all this started in July. One of our dear friends said, "she is starting to look like herself again, her coloring and her energy." AND, she has put on a little more than 2 pounds which is great because she'd lost at least 5 (which is a lot when you are only 45 lbs).

Her "off" or "unusual" feelings that could turn on that button in her brain are just normal things for you and I. Remember how so many of you wondered in the summer if she was feeling nervous or anxious about kindergarten?

Well, when we had the GI appt last week I was saying that I wish I knew why it happened once/twice a week over the summer. After talking/teaching me, it kind of all made sense. First of all, Ellie's birthday (exciting/puking) was at the beginning of August. Then she got to be spoiled by Gram and Grandpa for a week (exciting/puking), then she came home and we were counting down the days to kindergarten (super exciting, super puking). None of it was bad, in fact quite the opposite for her. She practically skipped into that kindergarten room, hugged us goodbye, told us we could go now, helped the other kids who were crying...there was NO FEAR whatsoever. But the GI doc says that is exactly right. It is any sort of up or down, not necessarily bad or scary (although that can trigger it too). He also said that it doesn't have to be any big events like those I just listed. He said--thunderstorms, a test at school, your team winning the game, Christmas, travel of any sort--pretty much anything that disrupts her daily "normal" could set off that button. He said while a counselor could help her learn to train herself to take care of herself during these big or little ups and downs, that it's not a psychological issue she has, it's a physical screw up in her brain. And in that sense we should know that as parents (and family and people who love us) there is only so much we can do. She still gets to be scared of thunderstorms and ridiculously excited about kindergarten, we just have to know that the best thing we can do is keep her life rhythm, ie sleep!, as healthy as possible while she goes through life. And, keep the meds on hand at all times. I don't know how they know all this, which leads me to my last thing and then I promise I'll stop.

He also asked me twice during our meeting last week if we'd be interested in participating in the research for CVS. I think we are pretty good candidates because we live close to Milwaukee (other families make appts to see this doc and fly in from all over the place) and because Ellie is still so young and seems sort of text book. Brian and I agree that yes, we'd love to help but only paperwork, maybe a visit or two to Milwaukee and definitely no more hospital tests unless she really needs it. But I have to say, it feels pretty good to say not only do we have a diagnosis (in only 6 months when the average is 2.5 years for a kid to be diagnosed with CVS) but also we could help with research for it. Probably I also feel so positive b/c she hasn't puked for a month...but whatever, we'll take it!!

This is from the website from Dr. Li:

CVS is one of the more unrecognized and misdiagnosed childhood diseases. It is marked by severe, recurrent attacks of vomiting in a child who is otherwise completely healthy for weeks or even months at a time before and after the attacks. Over the course of 24 to 48 hours, a child may have more than 30 vomiting episodes, occurring every five to 10 minutes at its peak.
Gastroenteritis is a common misdiagnosis of CVS, as are food poisoning, gastroesophagealreflux and even bulimia.
"With stomach flu, once the stomach is empty, the vomiting stops," Li said. "With CVS, the physical act of dry heaving will continue long after the stomach is empty. The drive to vomit is relentless."
Such episodes often begin in the early morning around 2 a.m. to 6 a.m. The child is non-responsive and pale, often curled in a fetal position.
The peak age for the first onset of CVS is 5 years. Sixty percent of those afflicted are female.

It's so Ellie! Ok, I'm done now. Lots of love and appreciation from me. Hopefully my next physical update will be about her breaking her leg during an amazing soccer save--ok, probably not Ellie, but something along those lines.

Third Update: Probably CVS

Friday, November 16, 2012
Generally, Hopefully Good News!

I finally couldn't stand the waiting and called the nurse on Wednesday afternoon. She said, "Well the labs came back and they all look great, but the doctor is so surprised by this that he wants to look at it all himself before calling you back. What he saw in the endoscopy was quite different than what the lab reports are saying." So, I felt cautiously optimistic. Then I finally got a quick call from the nurse again this morning saying the doctor looked over all the slides and he agrees, there was no sign of EE! But he is not sure what is causing the inflammation of her esophagus. She could be having esophogital spasms, not sure if it would be linked to Cyclic Vomiting Syndrome or something else...anyway, we are going in to talk with him on Wednesday. The first visit with him he told me that before we could officially diagnose it at CVS we'd have to do a few other tests to rule out kidney issues, immune disorders, etc. He said she would need an ultra sound of her stomach. My guess is that based on the way her endoscopy looked to him, he will still want to do some of those tests. Unfortunately (or fortunately) I feel like as her parent who is listening to the specialized GI doctor be confused about the discrepancy, I too feel like we should do some of those "just in case" tests. Before her endoscopy I was thinking maybe I would just call it quits (well, Brian and I) and just call it Cyclic Vomiting Syndrome because in my opinion that diagnosis REALLY matches up with our Ellie. But, since he feels unsure now I too feel like maybe it's worth it to do a few more tests. I guess Brian and I will have to just go one step at a time and decide. I'd rather she didn't have to do another invasive test, like the endoscopy, because, well, it sucked. But then again I'd like to not miss something big.

Generally though, I feel like this is GREAT news. So far Ellie's CVS seems on the milder side (compared to what a lot of other families apparently go through) and so far she has responded really well to zofran. Also, now that we (almost) have a diagnosis we will better know how to treat it quickly so hopefuly no more long bouts of puking, or if there are we will know to go to the ER for IV and meds. I have a lot of questions, like if it will get better or worse and if there is a risk of her not responding to the medicines down the road, but that's just my worry-mommy stuff coming out, right now should be about celebration, I think...right?

I sort of wish I could send you all a personalized thank you card for listening and caring!

Love,

JJ

Second Update: Endoscopy and EoE

Monday, November 12, 2012:
Nope, I haven't heard from the doctor yet but I am nervously awaiting that phone call and until then, it helps to write to you all. My brothers and sisters have heard all this stuff, you can skip this "update" if you want. Once we hear from the doctor I'll write again with results.

Ellie had an upper endoscopy on Thursday. I must say, for someone who is not easily impressed by doctor stuff, Hospital Pediatrics did AWESOME. With kids they sort of treat endoscopy's as a "real" surgery. You have to check in to the hospital for the day (get a room) and they put the kids all the way under (verses "twilight" which is what adults get for upper or lower endoscopes). In Ellie's case they also had to put a breathing tube in because her respiration wasn't stellar. So.. we get there and the doctor is running behind and so the very first thing my nervous girl gets to do---go to the "fun" room and *paint* a jewelry box! They also gave her a homemade blanket (sewn, a quilt) and a stuffed animal. Then later when we are in her room and the nurse is prepping her, she tells us to go back to the fun room and ask for the doctor's kit--a whole box full of real doctor things--masks, a stethoscope that really works!, bandages, tape, an empty IV bag--the whole bit. Ellie was thrilled.

Her anesthesiologist was a total character, making her laugh so hard that she had to stand up and go potty in pre-op. There were two nurses with her the whole time, and I noticed that both of them touched her a lot--one was playing gently with her hair and the other was rubbing her arm. That made me feel soooooooo good. I had given Ellie a doll that resembles the character in the movie "Brave" that morning, they kept the stupid doll on her surgery cart the whole time!! AND, after surgery everyone was joking with us about "Merideth's" vital signs etc, and when I finally told one of the post-op nurses that the doll's name is actually Merida, she said, "Oh, they have her in the charts as Merideth." THEY HAD HER DOLL IN THE CHARTS!  

Anyway, waking up from the procedure was the hardest part of the day for our Ellie. She cried a lot, was scared and in a lot of pain (her throat and tummy). And in classic Eleanor form, one of the first things she whispered to me was, "Mom, you told me a lot of stuff, but you never told me my throat was going to hurt!" 

She was quiet and worn out the rest of the day but did fine. Popsicles, movies, and soaking in all the special attention. Then that night she was up for a good portion of the night with a horrible sore throat. At about 3am she starting showing signs of typical Ellie episode. White as a ghost, dripping sweat and saying her tummy hurts really, really bad. BUT this time I had zofran at home and I do believe we missed a puking episode because of that medicine, which I now think of as gold. 

Snuggling it off.

The doctor said that Ellie's anatomy has the classic signs of EoE (also called EE). He even took pictures and showed me afterward. EE is the prognosis we were hoping would NOT happen. Even her superstar allergist said a few weeks ago, "EE is something we don't want her to have. It's horrible." It's a condition where there are a lot of a certain kind of white blood cells in her esophagus, stomach and small intestines. No one should have them in their esophagus but people with EE do. Generally, it's caused by allergies, and generally (although not always) it's because of food allergies, aaaaannnddd, generally it's not just one food that kids are allergic to. We would start by doing all sorts of patch testing to try to determine the foods that she is reacting to (so far blood tests and skin pricks have all come back negative). Then the hardest part about this is that it is a disease that would cause us to have to read every single ingredient in food packages like learning the different names for "Milk" and "gluten" that you would never have known was in that food package, that sort of thing, in order to clear out whatever she is reacting to and heal her esophagus, which is super inflamed right now.

The new GI doc (who we really like) took a bunch of biopsies to test for EE and a few other things (like celiac disease). He said it's a black and white diagnosis from the tissue he took. She either has it or she doesn't. But then the real work comes with determining what is causing it. So those are the results we are waiting for. I really hope he calls today or tomorrow, he said he thought he'd get them back by then.

The only strange thing is that, right before the endoscopy, the doctor told us about a syndrome called Cyclic Vomiting Syndrome. Everything he said about it and everything I read online about it describes Ellie's symptoms to a T! So, there is still hope that she doesn't have EE. Cyclic vomiting syndrome is no fun either, and has a much more grey diagnosis (meaning we just have to take note of her triggers and be ready with meds at all times) but it seems easier to manage.

Alright, I feel better having written all that out. I am so sorry I am so long-winded. I honestly think it just makes me feel more calm and more in control to write it out and to know that I have this great support of people in my life listening as we figure all this out. I will never be offended if you have to skim or even skip an "update." I just appreciate being able to press send!  :0)

Lots of love.

First Update, After the Storm

October 23, 2012: 

Hey, for those of you who didn't know, here's a recap:
Ellie was vomiting about once, sometimes twice a week during the last month and a half of summer. That's when we started seeing a GI doctor. We have been on meds since then. I tried taking her off dairy and 50% off gluten for a few weeks and got great results but the GI doctor said not only did she think it was unnecessary, it would actually screw up an intestinal scope that could check for things like celiac disease if we ever got to that point. So, I let down my guard completely the last few weeks, let her have the normal amount of dairy, gluten etc (but felt nervous the whole time). Kept up with the meds, tracked her pooping (constipation was a concern for the vomiting...)....then....

Almost two weeks ago E had to get picked up from school for vomiting and it was INTENSE, puking every 10-15 minutes, I thought for sure this must be a stomach flu not her stomach issues. But, she did have cheese pizza the night before and regular milk in her cereal that morning. But no one in class was sick, no one in class caught it, none of us caught it and a few hours after the hardcore horrible puking episode (which lasted about 2 hours) she was better and asking for food.

Then yesterday we went for a lactose breath test. It was to test to help us determine if she is lactose intolerant. She did fine during the test and it came back negative (in some other e-mail remind me to tell ya'll how amazing my kid is...relating to this test in particular I mean). I knew not to dump greasy food on her right after the test but I did let her have kraft mac n cheese for dinner last night which she hasn't had in ages.

About 5am Ellie was crying that her tummy hurt "so, so bad" then a little while later she vomited. Typical "Ellie stomach issue" type puke, hardly any food, all watery and bile but vomit nonetheless. Then again about 15 minutes later, then 10 and pretty quickly it was like her stomach was convulsing and couldn't stop. She was literally (no exaggeration, ask Brian) vomiting, or trying to, every 2 minutes. It was AWFUL to watch. She quickly ran out of steam, couldn't talk, couldn't open her eyes, we had to hold her up to puke...scary stuff for this mommy. So after we endured about an hour of that I finally said, "that's it, I'm taking her to ER!" Laid her in the backseat and went.

It didn't stop till we got meds in her at the ER. ER people were great, I mean, it would be hard not to because she looked so awful, couldn't open her eyes or talk and every couple minutes started "puking" but just spit and mucus came out. They did so great taking us right back, everyone was telling her it was going to be better soon, calling her "peanut", getting blankets/towels, and I think we saw the doc within about 20 minutes of being there and the meds within 10 minutes of that--uber fast for ERs in my opinion.

As always, there is no way to know for sure if it was a food reaction or a flu, and only the next few days will help us know...things like how fast she recovers and if anyone around her gets it. But, I had some choice words for the GI doctor this morning. Choice. Words. Of course there is always a little part of me that worries it is just the flu, every time this happens I question myself, my gut.

She is scheduled for an endoscope on Friday. Should help us determine if she does have celiac disease or if she is actually having an allergic reaction to dairy (the breath test was just for intolerance, as it turns out--choice words--and this sort of reaction would be more allergy-like).

I am super grateful for such a great experience at the ER this morning and super grateful that we've made some great friends in the immediate area who can help out. Very grateful neither of my kids have any sort of horrific disease like cancer, I couldn't handle this on a regular basis! Also grateful to have some family nearby (last week during Brian's ER visit Uncle Terry was "on-call" for middle of the night duty and Marylisa took Colin for like 6 hours while that all went down!). All of our friends have been amazing these last two weeks, amazing. Lots to be glad about.

Anyway, I wanted this to be fast and of course it's not... sorry. I'll keep you updated.

Love, love.