Crossing into adulthood and starting again

 Just as he is about to turn 18 in couple of months we will start to transfer to adult providers. Thankfully its only Traditional head migraines now.

Luck would have it. His youngest sister just got dx cvs today, but she is milder than he is but still have landed in the hospital 4 times in the last 2 years. Happy Dx Day Cara.

10 Year Anniversary in June

 Hard to believe it's been 10 years since finally giving a name to the crazy, intense, vomiting! I'm happy to report Andrew did out grow CVS around age 12 and switch to migraines now which are managed at home. We've not been in the hospital since port removal in Fall of 2019. It was an insane, constant state of affairs for 7 years, and now it's a thing of the past that the memories remain, some scaring on the veins probably, and a skewed sense of what constitutes a lot of vomiting.

It's not an exaggeration to say these episodes included vomiting every 3-5 min for hours. Easily 10 rounds of vomiting an hour was the norm. Light episodes it might be 6x hour. Total shut down withdrawal also standard as he'd lay eye closed and hid out in quiet dark room and try not to move. I will forever be thankful for the friends we've made along the way who simple said "You too? You mean I'm not the only on?". It is for all of us I chose to write and share our unique story of Cyclic Vomiting Syndrome.

I'll still be supporting fellow parents on the Facebook group. 

This will hopefully be my last blog post on the topic. 

Rice Family

Chronic illness in the ER

When home med plans fail, often those with chronic conditions need treatments that require prompt medical treatments not handled in the office. Specialists realize this and often write protocol letters listing when to implement treatment and how. Patients present to the ER where Dr are treating more unknown emergencies and dx problems. Issue is .. we are not looking for their dx skills or even their thoughts on our treatment plan. Both ER provider and patients get put at risk when plans are not in place. 

Recently we presented to the ER for our protocol which is one file and flagged and signed off on at our local hospital. This day however this dr either never pulled up said record or was going to a different way. This I understand, it’s on him. However he could have been upfront and honest I don’t feel comfortable with this, what do you think is best next step? If I had thought lesser meds were only option I would said ok but if that’s the case send us now to pedi floor where those who know him can manage. 

Nope they gave standard protocol and then wanted to discharge. I said he needs to pass the can he move without throwing up test. ( I did not say have your staff come in turn on all lights take blankets and yell migraine kid with autism he had to walk around and go home) I said before you take IV let’s see if he’s actually any better than when he when he got here (which he clearly was not). So he was asked talk which he did barely, and then forced to sit up and stand which of course he threw up. 

( side note after 70 ER and admissions both him and I know when it’s manageable at home home based on is he swallowing, can he open eyes, is he asking for food, is his heart rate below 100, what is his skin cool, and he motion with his fingers pain scale) he communicated all those things without being made to move.

Upon throwing up they handed him off to pedi team finally. Nurses asked why don’t we go to Boston hospital since they treat him. Well this local hospital has successfully treated him for 7 years here. All the pedi staff know him ( knowing he’s good to go when asks for ceremonious cheeseburger and chocolate milk indicating episode is over) why would I want to travel an hour for a staff that has no working knowledge of him?

In the end we were advised again when arrive to request pedi hospital staff to order manage his care. Updated files sent and hopefully next time will be better. I’m thankful we have a team that after 7 years will still go to bat for us and say to others we’ve tried that for years first hand we know it doesn’t work and requires this. We know if you do xyz mom is correct just send him to be admitted. Please help us help them and save us all time and frustration. 

There are those who do get it and those who will advocate for chronic patient care. It involves ER dr who really are needed elsewhere helping those who don’t know what’s going on..or those still searching for treatment plan. I do appreciate ER teams are treating high numbers of patients many psych and addiction cases who are dumped in ER as place to find help but that’s a whole other topic in itself. Let’s streamline chronic kids needs enabling them to stay close to home, and be treated by a team who become like family which removes a level of unknowns for both patient and providers.  

O

Growing research .... 2019 Shaping CVS dx

GROWING RESEARCH TREND 

ABOUT CYCLIC VOMITING SYNDROME

        According to Up to Date Cyclic Vomiting Syndrome (B UK Li) Updated March 2018  there are 7 emerging parthenogenesis that are emerging as a result of the last 10 years of study in over 70 published studies. ( Document by subscription or ask your child's PCP for a copy of it. below is just  highlights)

1. CVS and Migraine-  many have strong family history of migraine and progress to migraine as                                 they get older. about 80% of children respond positively to anti migraine therapy.
2. Mitochondrial disorders-  disorders of fatty acid oxidation, MELAS and mitochondrial deletions                             can cause metabolic crisis and vomiting when fasting.
3. Autonomic dysfunction- many also show signs of low tone and meet criteria for postural                                         orthostatic tachycardia syndrome (POTS)
4. Hypothalamus-pituitary adrenal axis hyperactivity- known as Sato variant with hypertension and                            prolonged episodes
5. Endocrine (catamenial CVS)- similar to menstrual headaches during onset of period and its                                     treated with low dose estrogen or progesterone.
6. Food allergy- Sensitivity to cows milk, soy and egg white protein may trigger episodes in                                          children. This relationship is still uncertain.
7. Chronic Cannabis use- Cessation of prolonged high -dose cannabis has been associated with                                    episodic vomiting suggesting a casual link ( THIS IS NOT TOPIC FOR                                        THIS PARTICULAR POST AT THIS TIME)

  These differences can make a difference and knowledge can help choose appropriate treatments.

             Meeting basic criteria is the foundation piece of leading to better treatments and a cure. Patients need to understand the criteria and discuss with their providers what might be their underlying parthenogenesis. If you do not meet the criteria you might another condition or similar one not mentioned that MIGHT respond to similar treatments.

              Adults see this more than kids currently... patient says they have CVS and do not fit the dx criteria.... and Dr know this ... and tell patient CVS is not real.... Well CVS is real .... the question is ...... IS IT THE CAUSE OF YOUR SYMPTOMS you are presenting with...given your presentation, and other related conditions..... Some Dr use CVS as a distinct condition... other use it for any intense vomiting or pain that they cannot explain.  Hopefully new studies that are currently underway will clarify this ... In the FALL 2018 or early 2019  this is to be clarified with new adult guidelines being published with the help of Cyclic Vomiting Syndrome Association.

Hopefully for adults these clarifications and proper use of them will lead to better quality of life.. But again not all intense vomiting or intense pain is CVS.

Whats the Weather Forecast?

What's the Weather Forecast? 

Reflection on how to track and describe episodes

Like most people, you probably check the weather forecast to see what it'll be.
Maybe you have a beach day planned..will it get rained out? Or maybe its winter will there be a snowstorm with enough to cancel school or just  a little not even plow-able.

Reasonable questions to ask right?

However when it come to describing our child's cyclic vomiting syndrome sometimes many people speak only in generalizations which are not helpful. One parent might say "oh my child has been in an episode for over a month" while another might say this is the 3rd episode this month
 So which is more accurate... 

Well it all comes down to the hourly forecasts :) and breaking down the length of peak level vomiting nausea and pain which is considered severe, functional / impact and recovery times.

Back to our weather analogy. The forecast says 90% chance of snow on a given day. So that means odds are you are going to see snow. WHAT IT DOESN'T SAY IS HOW MUCH OR HOW LONG. Wouldn't you really need to know more?

YES you'd need to know if it a snow squall that going to come and go all day and be annoying but not majorly effect your day (similar to needing maybe light recuse meds but still able to participate in some day to day activities, walk talk and have breaks of where they are able to intake fluids and bites of food).

Or if say its a blizzard and you will be home bound and limited to house and be prepared for power outages (Intense vomiting and nausea that has no breaks no fluids get in no food goes in and often requires hospitalization, there is no regular functioning at all during these storms)

You might start off a day with the squalls and then gear up to a full winter storm... have a lull and it will start again. It's IMPORTANT TO NOTE the AMOUNT OF TIME IN EACH PHASE  (Peak) / Recovery (mild  to moderate) and not just say... oh it snowed all winter.... because unless it literally did......and you have 30ft of snow outside your door... YOU ARE MISSING KEY INFORMATION to share with providers. Its not about comparison of one child is worse than the other. In medical field doctors help us better with the more accurate detailed descriptions we give them. We are the witnesses to the events and our observations matter.

There can be as mentioned before in other posts yo-yo phases or cycle within a cycle.... when you think the episodes is over and then withing 1-3 hours it starts all over again.  The actual breaks here are not long enough to maintain hydration or nutrition. Dehydration is still a big risk.

Oh this episode lasted 24 hours ? 

Imagine an episodes that only last 3 hours (meeting the peak rate of 4x hour vomiting and then gradual decrease over time) and then maybe 2 hour recovery and the rest of the 19 hours of the day being able to function take in fluids etc.

Another person might say oh the episode lasted 24 hours and mean the active vomiting phase (meeting dx criteria or more per hour ) lasted a full 19 hours with a 3 hour recovery.

DID BOTH EPISODES LAST 24 HOURS?

• the first was actually only 5 hours... if the person starts getting sick again the next day... 

                                                    IT IS A DIFFERENT EPISODE

• The second example the actual episode lasted 19 hours, if the person starts getting sick again it's possible that its the same episode the length of the active phase was so long.

When speaking about Cyclic Vomiting Syndrome it is very important in the early days to track the rate per hour of active vomiting and pain levels. This information is a clue about the intensity and nature of the episodes. This helps dr to take us as a community seriously when we use a common vocabulary and have a common understanding of the condition ourselves. 

I might say oh my son has been throwing up all morning meaning 30x in the past 5 hours and you might say the same thing and mean 5x in the last 5 hours.  
                                             THIS DOES MAKE A DIFFERENCE.

Being that CVS is a diagnosis by exclusion it might mean that its a misdiagnosis.  Watching these subtle things can help plan intervention and get to the cause of the episodes. It is common that later children are dx with other conditions that look like CVS.

IN REALITY WHAT DOES THIS LOOK LIKE?

                     On our trip there were episodes avoided most of the week. I would not say he was in a week long episode. Did he get sick on the trip yes.. he showed signs everyday and took meds daily to keep it at bay..... we enjoyed 90% of the trip.... the last day he did end up unable to function at the end and landed in 3 hospitals in one day. The start of the active retractable vomiting the mark of the start of that episode and lasted til he was able to eat or drink which was 24 hours...

We were able to fly home the following day... and 24 hours later he started all over again and yet again ended up in the hospital... It was still the same trigger... excitement from the trip....but it was 2 different episodes in a short time. Given the situation.... We really LUCKED OUT as it could have been a non-stop one that never responded to meds. We could have ended up with an additional week long stay at Nemours....
                                        Instead I say we  had a VICTORY Over CVS!!

What if my child is diagnosed CVS and NEVER has had severe nausea 

and vomiting to the level mentioned?

If your child mild vomiting that always stops once the stomach is empty, is able to go to school through the pain and nausea, plays and stops to throw up and keeps play?

Couple of things:

Could be the early ages of CVS that might progress over time.

Could be more what international headache society describes as  recurrent GI disturbance 1.6.1 which does not have to have the severity but has common patterns and triggers etc. 

Most of the treatments are similar for both. With the Recurrent GI disturbance (RGID) hospitalization, IV fluids, and sedation are rarely needed.

(in process of being updated 1/16/2020)

What makes Cyclic Vomiting Syndrome Different?

• My child throws up once a week in the early morning... He only throws up 4x between 5am-8m Why?
• Every time my child gets overtired they throw up overnight a couple of times..why?
• My child throws up every couple of months in the early morning and then recovers in hours and no one else ever is sick...what is this?
• My child throws up so intense and pukes 5 or more time an hour  for several hours cannot keep fluids in and keeps ending up in the ER to stop the vomiting... Why?

There are hundreds of reasons why children throw up in childhood. Some have sensitive gag reflex, food allergies other, stress, eating too much, viruses, enlarged tonsils or even a developing digestion system....

So what makes CYCLIC VOMITING SYNDROME different??? 

They key diagnostic criteria that sets it apart from all of these is THE INTENSITY. 

According to Rome IV criteria 

• Occurrence of 2 or more periods of INTENSE, UNREMITTING nausea and paroxysmal vomiting LASTING HOURS to DAYS within a 6 month period.
• Episodes are stereotypical in each patient
• Episodes are separated by WEEKS to MONTHS with return to baseline health in between.
• AFTER appropriate medical evaluations, the symptoms CANNOT be attributed to ANOTHER CONDITION.

International Headache Society (ICHD-III beta criteria) describes it as :

1. At least 5 attacks of INTENSE nausea and vomiting  and fulfilling  2 and 3.
2. Stereotypical in the individual patient and recurring with predictable  periodicity 
3. ALL OF THE FOLLOWING
1. At least 4 attacks per hour of nausea and vomiting
2. Attacks last more than 1 hour and up to 10 days
3. Attacks occur MORE than 1 weeks apart
4. Complete freedom from symptoms between attacks
5. NOT ATTRIBUTED TO ANOTHER DISORDER

North American Society for Pediatric Gastroenterology, Herpetology and Nutrition (NASPGHAN)

1. At least 5 attacks in any interval, minimum 3 in 6 months 
2. Episodes of INTENSE nausea and vomiting lasting 1 hour-10 days and occurring at least 1 week apart  
3. Stereotypical pattern of symptoms in the individual patient
4. Vomiting during attacks occurs at least 4 times per hour for at least one hour.
5. Return to baseline health between episodes
6. Not attributed to any other disorder.

What other features that distinguish CVS from other reasons for vomiting....

Or another chart that compares chronic vomiting vs cyclic 

Is it still Cyclic Vomiting Syndrome

 if it meets some of criteria but not all?

LINKS TO MEDICAL INFO REFERENCED ABOVE

1. fleishers-empiric-guidelines.pdf (cvsaonline.org) 2008
2. Sept07VenkatasubramaniArticle.pdf (ficomputing.net)2007
3. Managing cyclic vomiting syndrome in children: beyond the guidelines | European Journal of Pediatrics (springer.com) 2018

Childhood periodic syndromes. Click link to full medical journal article. 

Cuvellier JC1, Lépine A.

Abstract

This review focuses on so-called "periodic syndromes of childhood that are precursors to migraine," as included in the second edition of the International Classification of Headache Disorders. Presentation is characterized by an episodic pattern and intervals of complete health. Benign paroxysmal torticollis is characterized by recurrent episodes of head tilt, secondary to cervical dystonia, with onset between ages 2-8 months. Benign paroxysmal vertigo presents as sudden attacks of vertigo lasting seconds to minutes, accompanied by an inability to stand without support, between ages 2-4 years. Cyclic vomiting syndrome is distinguished by its unique intensity of vomiting, affecting quality of life, whereas abdominal migraine presents as episodic abdominal pain occurring in the absence of headache. Their mean ages of onset are 5 and 7 years, respectively. Diagnostic criteria and appropriate evaluation represent the key issues. Therapeutic recommendations include reassurance, lifestyle changes, and prophylactic as well as acute anti migraine therapy.

WHY DOES IT MATTER??

It matters to the families who children do have this intense vomiting that does not respond to basic medications (over the counter or zofran) , taking sips of fluids and end up being sick for days unless seeking treatment. 

It matters when we tell ER staff he or she has been throwing up all day meaning more like at least 4 more more in the first hours and then usually it increases to more like 6-10x hour there after ... so easily an episode is 50x a day if medications don't work. Sound extreme? Like exaggeration? Its not ... its the truth of Cyclic Vomiting Syndrome and fits the criteria currently established last updated in 2008 to distinguish it from other vomiting/ belly pain conditions.

It is important to be clear when talking with medical providers what "all day" looks like... 2x in the morning and 4x at night is a very different than CVS episode. This type of vomiting or rate of 1x an hour is more consistent with abdominal migraine presentation or chronic vomiting.... 

Do you see the difference? Its not to say that vomiting 4+ times an hour for multiple hours is any worse than the unrelenting pain. (Honestly I think abdominal migraines are harder to deal with  because dr never know what to do with that... vomiting they are quick to act to prevent severe dehydration.... but pain not so much).

If we want to be the best advocates for our children knowing the difference helps us to communicate the situation better.  If the medical team is not familiar with Cyclic Vomiting Syndrome they might look up basics and the criteria to review....

 What happens when you come in and claim it CVS and don't fit the criteria....

1.. The ER staff might assume its just a variation or mild form....

                                                   or

2. The ER staff might just think you have a doctor who just dumped all unexplained vomiting into this rare diagnosis to give you a condition or billing code for your  records to document .

                                                    or

3. They might just say again you were internet diagnosing yourself.. and thus odds are its just a virus... or a kid who ate too much chocolate cake. 

            If we are going to HOPE for a cure... we have to have an idea of WHAT it is we are trying to cure one thing at a time.... We might get lucky that one type of treatment will help both conditions but odds are these distinctions matter. The more specific we are about what a condition is the easier it is to study it and try specific treatments based on similar presentations. 

EVERY KID IS DIFFERENT .....AND A MILLION TYPES OF  VOMIT ONLY A SMALL PORTION OF KIDS WITH INTENSE VOMITING HAVE CYCLIC VOMITING SYNDROME.

CVS is no longer considered to be rare anymore!! 

Still only 3-5 % of children suffer with it. 

In our family we have all kinds of kids who puke... One who when younger could throw up early morning couple times a week, it was challenging and annoying as well heck... yup... for that one it was enlarged tonsils triggering the gag reflex... It lacked intensity despite its's stereotypical onset and duration...Getting his tonsils out and age help that child stop the puking. This child still puked in foreseen ways after that.... seeing gross things in the trash continued to get him sent home from school at least once a week for next 2 years....

My CVS kiddo on the other hand was always an intense unremitting vomiting type.. Once he started I knew he would not stop and it resembled something you'd see on an episode of House. No stop retching to the point of brown/ black slimy goo coming out even after they put in IV and gave zofran.  At age 3 was his first hospital stay for dehydration...He had been in the ER had fluids went home and it still continued so we ended up returning hours later because the vomiting still had not stopped. He didn't get dx til age 5 when even getting zofran around the clock for 7 days it was still ongoing...

I have another child who I swear at age 13 has thrown up maybe 4 times in her whole life...... 4 times period if they didn't look alike I'd wonder if she was really related to them :)

Even on our first Disney trip in April ... out of 6 kids 3 of the ended up throwing up on the trip.... One had motion sickness and might have thrown up average 4-8x a day but was other wise totally fine.... Another only threw up the first night there... maybe about 6x overnight...his history is the excitement factor..(usually he just spikes 102 fever for few hours when traveling this time he decided to throw up a lot) Neither one of them have CVS.... those vomiting episodes though stereotypical for them under certain conditions etc... and a lot of vomiting still lack the dx criteria to be considered CVS and even with family history.... IT'S not all CYCLIC VOMITING SYNDROME 

So what does this mean for our Cyclic Vomiting Syndrome Community?

To use the term Cyclic Vomiting Syndrome..... I hope that all of your can say that your child has met the intensity of vomiting piece of 4x+ an hour for at least an hour. I know with medications use we can get it to less than that...sometimes.... But the HALLMARK to CVS is the intensity of the vomiting and unrelenting nausea.. which often leaves children lying on the bathroom floor unable to move or swallow their own saliva..... If your child is walking and talking eating and drinking in what you consider the peak part... its not CVS.

Mild CVS  would refer to those who's intense episodes are 3-4x a year... and respond well to medication, or short in duration (couple of hours not days).

Kids with ongoing dull or moderate belly pain that never seems to leave is more an abdominal migraine and looks different... And yes a child can alternate between both types of episodes... but knowing the difference... helps communicate better.... and medical teams to trust us and know we understand the conditions we are helping our children live through. 

From age 4-9 my son had vast majority Cyclic Vomiting Syndrome type of episodes and a handful of abdominal migraines thrown in there.....Around age 10 he began transitioning to traditional migraines with a couple of Cyclic Vomiting Episodes thrown in there as well.....I tend to keep track of the differences and how many of each type he has so that we can work with his Neurologist to design a plan that knows how to respond to each type.... and we do treat them differently with 2 different med plans of how to respond to them. Knowing his factors are key to leading to a cure or better management to his vomiting episodes given his presentations and underlying related medical conditions.

Vomit is NEVER FUN for parent or child. 

But I'd invite you to look hard at the criteria and talk with your medical team how much your child's vomiting fits the criteria. Do they have abdominal migraine type or cyclic vomiting type?

Not every diagnosis of Cyclic Vomiting Syndrome given is a final diagnosis... often in children its a working diagnosis until other reasons can be sorted out through either time or further testing....What tests should be done will be topic for next post in the coming weeks.

THE END GOAL OF ALL THIS

 IS TO HELP AIM TREATMENTS 

TO AVOID THIS THE BEST WE CAN

Disney 2018! (So what if CVS tagged along I don't care!)

Yikes I can't believe its been almost a year since I last blogged.... We started homeschooling 2 children and are now expecting number 7 in October. So its been a very busy year.  I'm still very active in offering daily support on Rare But Not Alone Facebook group.  You can almost always reach out to me there.

We had an amazing fall. We went not hospital from June 2017 until Jan 2018!  Imatrex has been amazing in our avoiding needing IV's!

In April we had a rough month 2 hospital stays.. one triggered by an amazing thing. WE WENT TO DISNEY WORLD! We have planned only to stay at the hotel a day before we got on the Disney Dream Cruise ship (figuring low key Disney no lines or crowds and easy access to the room).  We decided to go into Magic Kingdom for the day!! It honestly was one of the best days ever. I could not stop smiling the whole time.

When we first got there we headed straight to the ride Andrew had been waiting to go on for YEARS (thank you You Tube) Haunted Mansion.  The wait was only 40 min! There was plenty to see and do in line and people did not crowd you in like I had feared... He LOVED IT!!! After that we were free to do whatever but I was not able to go there and have him miss out on that.

CVS eventually hit the LAST day of the trip when we were suppose to fly home. It didn't just hit him by myself as well. We ended up in the ship infirmary, local hospital and eventually transferred to Nemours in Orlando (HIGHLY RECOMMEND THEM TAKE UBER TO GET THERE THOUGH) We made it home a day late.  Yeah we ended up right back in our local home hospital once home... but we were HOME.

In all this mess we finally decided a medi port is a MUST.  2 of the stays it took about 10-16 tries to get an IV in delaying medication getting in for up to 4 hours... So next month the Port is going in!!

I've been reviewing a
lot of new research on CVS! There's lots of new things to share. I am hoping to have another blog entry about that over the summer. The kids are in school til..... June 22 here.. It's a New England thing.