Cyclic vomiting syndrome (CVS) is a debilitating disease characterized by episodes of severe nausea and persistent vomiting interspersed with periods of wellness. CVS affects about 2 percent of school-aged children, and also affects adults, although in adults it is often not recognized. Getting a diagnosis can be challenging, and sometimes takes a long time. Episodes of CVS can be extremely debilitating, and are sometimes difficult to treat and require hospitalization.
My daughter has suffered from this disease for 10 years, since she was 2 years old (see her story here). Her episodes were somewhat predictable when she was younger, but have changed and become less predictable, and more difficult to manage with medication, as she gets older. Although we try to avoid triggers such as stress and fatigue, being a pre-teen girl, she likes to have sleepovers with her friends and stay up chatting all night. Unlike other pre-teen girls, however, she suffers the aftereffects of the sleepovers sometimes by vomiting for 24 hours or more.
What Causes Cyclic Vomiting Syndrome?
Although it has long been thought to be related to migraines, many sources state that the cause of cyclic vomiting syndrome is not known. Mechanisms that may be involved include episodic dysautonomia (malfunction of the autonomic nervous system that can result in a variety of symptoms), mitochondrial DNA mutations that cause deficits in cellular energy production, and heightened stress response that causes vomiting. However, there is mounting evidence for the role of mitochondrial dysfunction in the pathogenesis of this disease, a fact that is not often understood by the average practicing gastroenterologist. The connection to mitochondrial dysfunction has important implications for effective treatment of cyclic vomiting syndrome.
Mitochondrial Dysfunction, Cyclic Vomiting and Other Conditions
Mitochondria are small organelles within the cell responsible for energy production and other critical functions. Because of these crucial functions, Dr. Richard Boles, Director of the Metabolic and Mitochondrial Disorders Clinic at Children’s Hospital Los Angeles, explains that “30 years or so ago, many scientists couldn’t believe that mitochondrial disease could exist, because how does the organism survive?” However, mitochondrial dysfunction plays a role in many diseases, including CVS, and according to Dr. Boles:
“these are partial defects. Mitochondrial dysfunction doesn’t really cause anything, what it does is predisposes towards seemingly everything. It’s one of many risk factors in multifactorial disease. It can predispose towards epilepsy, chronic fatigue, and even autism, but it doesn’t do it alone. It does it in combination with other factors, which is why in a family with a single mutation going through the family, everyone in the family is affected in a different way. Because it predisposes for disease throughout the entire system.”
DNA mutations that affect mitochondrial function can occur in the DNA that is found in the nucleus of the cell (genomic DNA), or they can occur in the DNA that is found within the mitochondria themselves. Mitochondrial DNA is inherited differently than nuclear DNA. Most people are familiar with the inheritance of nuclear DNA, in which we have two copies of every gene, and we inherit one copy from each of our parents. However, mitochondrial DNA is inherited exclusively through the mother; therefore, mutations that affect the mitochondrial DNA can be traced through the maternal lineage of a family.
A possible relationship between cyclic vomiting syndrome and mitochondrial dysfunction was suggested by the finding that in some families, CVS was maternally inherited. Mitochondrial DNA mutations and deletions have been reported in patients with CVS, and disease manifestations of mitochondrial dysfunction have been found in the maternal relatives of patients with CVS. In other words, conditions such as migraines, irritable bowel syndrome, depression, and hypothyroidism, are often found in the maternal relatives of patients with CVS.
Mitochondrial DNA mutations don’t cause CVS directly, in the way that a DNA mutation causes cystic fibrosis, for example. In some patients, mitochondrial dysfunction plays a greater role in the causation of their disease, and in other patients, it may be less of a factor. Dr. Boles explains: “In some cases it’s a clear mitochondrial disorder, they have multiple other manifestations and it drives the disease. However, in most patients, it is one of many factors in disease pathogenesis.” Patients with classical mitochondrial disorders have disease manifestations such as muscle weakness, neurological problems, autism, developmental delays, gastrointestinal disorders, and autonomic dysfunction. Some patients with CVS have these other disease manifestations, and some have only CVS symptoms.
Treatment for Cyclic Vomiting Syndrome and Mitochondrial Dysfunction
As with many diseases, understanding as least some of the cause of CVS has allowed for the development of treatments tailored towards fixing the root cause. Co-enzyme Q10 and L-carnitine are two dietary supplements that have been used to treat a wide variety of conditions. Both supplements may be able to assist the mitochondria with energy production and thus, help compensate for mitochondrial dysfunction. A retrospective chart review study found that using these two supplements, along with a dietary protocol of fasting avoidance (having three meals and three snacks per day), was able to decrease the occurrence of, or completely resolve, the CVS episodes in some patients. In those patients who didn’t respond to treatment with supplements alone, the addition of amitriptyline or cyproheptadine, two medications that have been used for prevention of CVS episodes, helped to resolve or decrease the episodes. Treatment with the cofactors alone was well tolerated with no side effects, and treatment with cofactors plus amitriptyline or cyproheptadine was tolerated by most patients. Therefore effective treatment for prevention of CVS episodes does exist, although it may not be widely employed by most gastroenterologists.
My daughter is currently trying to treat her CVS with the combination of co-enzyme Q10 and L-carnitine. So far she hasn’t experienced any side-effects, and over the next few months we will see if she experiences a decrease or even a complete cessation of her episodes. My hope for her is that she won’t have to choose between missing out on a fun night with her friends, and being able to be functional for the rest of the weekend. Maybe she can be like every other teenager and go to a sleepover, and just be grumpy the next day, instead of spending the next day vomiting and lying on the bathroom floor in pain.
Dr. Richard Boles, MD: Dr. Boles completed medical school at UCLA, a pediatric residency at Harbor-UCLA, and a genetics fellowship at Yale. He is board certified in Pediatrics, Clinical Genetics and Clinical Biochemical Genetics. His current positions include Associate Professor of Pediatrics at the Keck School of Medicine at USC, an attending physician in Medical Genetics at Children’s Hospital Los Angeles, and Medical Director of Courtagen Life Sciences. Dr. Boles practices the “bedside to bench to bedside” model of a physician-scientist, combining an active clinical practice in metabolic and mitochondrial disorders with clinical diagnostics (DNA testing) and research. Dr. Boles’ clinical and research focus is on polymorphisms (common genetic changes) in the DNA of genes involved in energy metabolism, and their effects on the development of common functional disorders. Examples include migraine, depression, cyclic vomiting syndrome, complex regional pain syndrome, autism and SIDS. He has 50 published papers on mitochondrial disease.
Postscript: Using this advice, we were able to manage my daughter’s vomiting. Here is the follow-up story.
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This article was first published on January 28, 2014.
My neighbor’s daughter had this. Severe post nasal drip from a deviated septum caused it. It took them 2 years and a smart NP at an ENT to figure it out.
So what you are saying is that your neighbors daughter had a problem with a deviated septum, not cyclic vomiting syndrome. My daughter has been diagnosed with CVS for 31 years. I wish it was as simple as fixing a deviated septum. Thank you for sharing.
I’m not sure if you will read this but I am in the exact same position, after two years I’ve finally figured out its CVS being triggered by my nasal issues. Do you know what course of action the ENT took??
My whole life has been taken over, I had to quit my job and I never leave the house
Not sure if you’re still reading the comments on this but regardless, I have to thank you SO much for this article. I found this article last August, at a point when I was having episodes/going to the ER every 3 or 4 weeks. I started the CoQ10 & L-Carnitine immediately and have not had an episode in almost 9 months. I’ve also gained about 15 pounds, which is INCREDIBLE for me as I’m very thin and have never been able to gain weight. I honestly feel like you saved my life and my sanity with this information and I cannot thank you enough.
<3 Cassandra
I’m happy you have had so much success with it!
Our sincere thanks for this article and research. Our daughter is 13 and has had bouts of CVS since she was a toddler and we are now very optimistic that she has a regimen to be pro-active with her condition.
Her last episode was about 4 weeks ago, triggered on a School Band trip by all the usual suspects – fatigue, over excitement, too much sugar, and so on. Once that episode passed she started on the CoQ10, L-Carnitine supplements and they look to be working. She’s had a couple of situations since then that would normally put her over the edge, but she’s hanging tough and (touch wood), we’ve finally cracked this nut.
Providing our daughter with the tools to manage her situation is a life changer. Can’t thank you enough for this total change in direction in how we have managed this condition in the past and empowering our daughter to take some control.
Thank you.
I am so happy that it helped your daughter also!
Thank you so much for your informative articles. On the subject of mitochondrial disease and genetic predisposition, I am wondering if you know of research regarding mitochondrial disease/genetic predispositions towards preservative/chemical sensitivities? I have a combined personal and family history of strong side effects to MSG,red dye,tyrosine, cow protein allergy, and certain vaccines (DTAP). If you have any inforation that could help enlighten me I would appreciate being pointed in the right direction. Your bio on Dr.Boles had me wishing I could ask him as well! Thanks so much and I truly appreciate your knowledge sharing.
Hi Briana,
I would suggest talking to a functional medicine doctor–I think they have an understanding of mitochondrial issues and chemical sensitivities as you describe.
Philippa
My dad who is 83 has had cyclical hiccups for 2 years. The cycle is 10 days on then 10 days off, and repeat. This is continuous and non-stop, like clockwork. On the on-cycle, the hiccups are quite debilitating, he has trouble swallowing because of acid reflux, eats less, and loses weight. And on the off-cycle, everything is normal, he feels good and eats more. His GP has no idea as to the cause. We have tried many, many meds including baclofen, gabapentin, pro-kinetics, PPI’s, and others. None of these have made a difference. He is seeing a gastroenterologist, who also does not have an answer. And my Dad has just begun to see a neurologist, who has ruled out a brain tumour, and at this point does not know what is going on either. What I find so unique and puzzling is the cyclical nature of the condition. This article reveals a similarity between CVS and his cyclical hiccups. We have not yet tried CoEQ10 and l-Carnitine. I would really appreciate your opinion. Sincerely, Dan.
I think I have it figured out. Your article was a big help! Thankyou so much. Dan
I am glad it was helpful! I must say that I’ve never heard of cyclical hiccups as you describe. The supplements are very safe though so they may be worth a try. I hope you can figure out something that helps because it sounds quite debilitating. Please keep me posted–I would love to know how things go for him.
Hi Dan.
I have cyclical vomiting every two weeks. My episodes last 6-9 days. Always start early in the morning and finish by 2pm each day. I also get hiccups. Interestingly my hiccups have decreased since starting L’Carnitine and CoQ10. It’s worth a try for your Dad. It’s not expensive,
Thanks for the write up! I need to get my daughter on these to help prevent episodes as much as possible. I believe in giving the body what it needs nutritionally to help support it with what it’s lacking to function properly. Breaks my heart that she has had to deal with these episodes most of her life. Thankful to have found something natural that stops an episode when it starts but need to work on preventing them too.
I hope it works for her! These supplements have been a godsend for my daughter.
Hi Phillipa, my son 14 years old, got diagnosed recently with CVS. Though the symptoms were there and we visited numerous doctors he was being misdiagnosed until now.
But now his cycles are every alternate week for last few months and its breaking my heart. He is on Ami 50mg and I just now ordered CoQ10 and L Carnitine. He also has severe constipation with it so it makes it worse. Do these enzymes cause constipation? Hoping for the best.
How is your daughter doing now? How old is she now?
Thank you for creating this blog and helping caregivers like us!
Hi,
Sounds like you are having a rough time! My daughter did not have any constipation with the supplements. You could talk to your doctor about taking a magnesium supplement for the constipation. It can help with that and is very gentle.
My daughter is doing well. She is almost 15. SHe’s been on the supplements for 2.5 years. She hardly ever has episodes any more. I hope you experience improvement as well!
Could you please let me know what dosages you give your daughter of CoQ10 and l-carnitine? My son is 8 and we just heard about CVS. We think that is what we have been dealing with. I am thinking of starting him on 200 mg CoQ10 and 2000mg l-carnitine.
This paper describes the dosages, but you should check them with a doctor to make sure that they will be ok for your son: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3163531/. The doses described in the paper are:
Co-enzyme Q10: Participants were treated with co-enzyme Q10 (ubiquinone) in liquid or gel capsule form (from a variety of brands) at a starting dose of 10 mg/kg/day, or 200 mg, divided twice a day, whichever is smaller.
• L-carnitine: Participants were treated with Carnitor brand or generics at a starting dose of 100 mg/kg/day divided BID, or 2 grams twice a day, whichever is smaller.
My daughter takes 500 mg L-carnitine twice a day, and 200 mg of co Q 10, twice a day.
Thank you so much for your response. The CoQ10 and L-Carnitine really helped for awhile. We started him on half the dose you daughter is on. He is starting to have episodes again, so we will up his dose and see if he responds. I really appreciate you articles
!
I’m glad that it helped!
Thank you SO much for your blog posts here, as well as your posting on CVSAonline. I have found them very helpful. My 9 year old has CVS and I am eager to try these supplements instead of the Amitryptiline. I appreciate your link to the dosing chart. I am wondering if you give your daughter liquid form or capsule or if you have any preferred brands? Thank you!
Hi, Philippa! Thanks so much for posting L-carnitine and CoQ10 dosing information here. I am looking at a supplement line that I like, Pure Encapsulations, and it has a combination formula for the two:
https://www.vitacost.com/pure-encapsulations-coq10-l-carnitine-fumarate-120-capsules
However, the dose in that product is not anywhere near as high as what you list above. Do you think this is a worthwhile option, or would you recommend finding else?
Thanks! I’m looking at these options as a method of hyperemesis gravidarum prevention, per the mitochondrial connection mentioned in another article on this site.
Hi Diana,
Pure Encapsulations is a great quality supplement line, but I think that the dosing for CVS is quite different than what is used for other indications. These two supplements are often used for other purposes so I suspect the Pure Encapsulations dosing is based on that. You could try the lower dose, but I suspect it won’t be as effective for this purpose as the higher dose.
Thanks a bunch! I will look into something that has a higher dose – I’m currently thinking NOW brand, which I also like. Thanks so much for getting back with me, and I will definitely report in later to let you all know how this goes for HG prevention!
Diana
Hi, Philippa! A quick dosing question:
You stated, “L-carnitine: Participants were treated with Carnitor brand or generics at a starting dose of 100 mg/kg/day divided BID, or 2 grams twice a day, whichever is smaller.”
Did you mean that the person should take 2 grams per day, divided into two doses of 1 gram each (for a total of 2 grams per day), OR that the person should take 2 grams twice a day (for a total of 4 grams per day)?
With the CoQ10, you stated 200 mg divided twice a day, so I’m assuming that means two doses of 100 mg each.
Thank you so much for the clarification!!
I was stating the dosing the way it was written in the paper, but it is a bit confusing, isn’t it?
So for the L-carnitine, imagine you weight 60 kg. Your dose by weight would be 100×60=6000mg per day (6 grams), divided into two doses, in other words 3 grams twice a day. But because that is more than 2 grams twice a day, you would take the 2 grams twice a day.
CoQ10, yes it means two doses of 100 mg each.
Thank you so much! That’s perfect! I will use that dosage, and will report back (on the original article, regarding HG and mitochondria) on how the CoQ10 and carnitine affect attempts at HG prevention. Thanks again!
Great article, Thank you! I need to get my daughter on these to help prevent episodes as much as possible. I believe in giving the body what it needs nutritionally to help support it with what it’s lacking to function properly. Breaks my heart that she has had to deal with these episodes most of her life. Thankful to have found something natural that stops an episode when it starts but need to work on preventing them too. This encourages me!
Hi Angelita,
I am sorry you are suffering so badly with this disease. It is an awful disease, but there are treatments that can help. Do you have a good doctor? If you are feeling suicidal please call your doctor, go to the hospital and tell them you are feeling this way, or call a suicide helpline (1-800-273-8255).
My daughter has been helped a lot by two dietary supplements…I wrote an article about her success with this treatment. http://www.hormonesmatter.com/hope-cyclic-vomiting-syndrome/. There are other options if this doesn’t work for you.
You can also contact the Cyclic Vomiting Awareness Association (http://cvsaonline.org/). If you don’t already have a good doctor who understands CVS, they may be able to help you find one in your area.
Please comment again and let us know how you are doing.
best wishes,
Philippa
Please help me get help for myself b 4 it kills me. I am a 49 year old student. I just found the name for this because my teacher had it also. Please I have no help & I’ve had this 30 years. I go to the hospital everyday please I need to talk with someone that can help me b 4 this or I kill myself.
Angelita, please do not give up, there is hope. Nutrient therapy works for some as indicated by Philippa’s posts. Her daughter has done remarkably well with two supplements coq10 and l-carnitine. Look into those others who have used these. There may be others too.
Jazmine, I believe it would depend on your weight and your blood levels. Maybe a doctor or a naturopath could advise you?
How much of the medicine is taken?
Greg, that is wonderful that the supplements have helped you so much! I think they are very safe, so definitely worth a try.
T. Garcia, perhaps try printing this article out and taking it to your son’s doctors? Also, there may be a list of doctors who are knowledgeable about CVS on the Cyclic Vomiting Association website: CVSAonline.org. If not I would contact them and ask if there is a doctor near you that might be able to help you.
My son has had over 18 surgeries. He has a shunt and suffers migraines, for years, and including this morning, he can’t keep anything down, no food, no water. He aches in pain for 2 to 3 days, throwing up anything and everything he can. Most of it ends up being snot like. He’s had dna testing, he is develop mental delay. But he’s in college. I JUST read about this today! How can so many doctors get it wrong? Once or twice a month he has episodes of severe vomiting with pain. He’ll feel better after about 2 days. I’m lost. He does have migraines, but what am I suppose to do?
I just want to let both the writer of the article as well as anyone here seeking answers know that the combination of L-Carnitine and CoQ10 completely resolved my problems. For nearly 10 years CVS ruled my life. I had been given countless prescriptions over those years with little to no success. After a GI doctor had done some research he advised me to give these two supplements a try. It’s been over a year since my last episode (and I was averaging over 10 ER visits/year prior). I can’t guarantee the same results, but I would recommend that people at least give it a try.
I don’t know iF this will be read since it was posted in 2014, but I hope so. Greg, when you say the two supplements have stopped the CVS, are you saying that is all you take for it or did you just add this? I have recently added those two also, but I’d like to give my doc examples of people who have cut down on the amount of different pills they take solely for CVS.
Thank you for the information Derrick Lonsdale. It sounds very interesting.
Kathy, have you tried the supplements discussed in the article (co Q10 and L-carnitine)? They are readily available in the US, can be used with other meds, and generally have no side effects. My daughter seems to be doing very well on them. I know how much it affects her life and every aspect of your family’s life :(. I hope you can find a treatment that works.
My 13 year old has CVS… it’s awful… it effects all aspects of her life and the family’s. Is TTFD available anywhere in the US? Is it dangerous/side effects with other meds.?
Thanks,
K
Many years ago I became interested in the vital importance of vitamin B1 (thiamine). In about the middle of the last century Japanese investigators had discovered that there was a disulfide derivative of thiamine found in garlic and called it Allithiamine. Experiments over many years in both animal and human studies found its therapeutic potential in energy metabolism, particularly in brain cells. It led finally to the most modern derivative whose chemical name is thiamine tetrahydrofurfuryl disulfide (TTFD). I have held an independent investigator license from the FDA since 1973 and have treated literally hundreds of patients with varying degrees of success. TTFD is made by Takeda Chemical Industries in Osaka, Japan and is sold under the trade name of Alinamin. Unfortunately, it is treated as a new drug by the FDA and would therefore have to go through all the myriad studies required for import to the USA. It is available in many countries throughout the world, including Mexico. Because this post concentrates on cyclic vomiting, I am reporting below an experience with a particular case. To show that it is not specific for cyclic vomiting as a disease process, I have included reports on two other cases, both of which are related to energy metabolism in the brain.
Case 1. One of my patients was a 12 year old girl who had repeated episodes of cyclic vomiting. A typical story for her went like this: she would vomit repeatedly until she’d lost about 14 pounds in weight and would be admitted to a hospital. With spontaneous remission she would develop hyperphagia, eating everything put before her and she would quickly regain the 14 pounds. During this period of rehabilitation, she would develop repeated nosebleeds. Thiamine deficiency produces capillary bleeding from deficiency of energy generated by the capillary cell mitochondria, leading to the breakdown of the capillaries in the nose. When, during rehabilitation, she began taking an excess of calories, energy metabolism was compromised because of insufficient thiamine. When I started her on TTFD these episodes were completely prevented permanently.
Case 2. A boy aged 12 years with epilepsy had received every drug known to the pharmaceutical companies, without success. He was currently in the hospital for a trial on a new drug. For several technical reasons he went into “status epilepticus”, a situation where the seizures are perpetuated and require emergency treatment. I gave him intravenous TTFD that successfully treated the status.
Case 3. An 18 months old girl was admitted to hospital with Reye’s Syndrome. Although this often lethal disease was ultimately found to be due to the administration of aspirin, standard treatment at that time was usually unsuccessful. The disease eventually led to a comatose state, requiring a respirator. I gave her TTFD, both intravenously and by oral administration. She eventually recovered consciousness, and subsequently walked out of the hospital. This case can be reviewed in more detail in my blog “Oxygen the spark of life” under the title of “An Unusual Recovery”.