PPM1D

Ongoing Research

CVS in JdVS.

Research by 
B U.K. Li MD
Emeritus Professor of Pediatrics
Medical College of Wisconsin,
Milwaukee, Wisconsin, USA


What is cyclic vomiting syndrome (CVS)?

CVS was first described in 1882 and affects 2% of children and adults.  It resembles severe stomach flu or food poisoning that keeps recurring while no one else is ill.  The child returns to their regular state of health between attacks.  Approximately 6 out of 10 JdVS patients appear to have CVS.  Although the cause is unknown, there is a strong relationship to migraines, a dysfunctional autonomic nervous system and exaggerated stress response.  There is no diagnostic test and the main diagnostic criteria include the pattern of repetitive vomiting episodes (4-10 over 12 months) lasting 2 hours to 7 days at least a week apart that are stereotypical, with each episode resembling previous ones.  Unfortunately, CVS is frequently misdiagnosed as reflux, stomach flu, food poisoning or a psychological issue.

What are the main symptoms of CVS?


During attacks, the child is pale and listless, sometimes unresponsive, with severe nausea, abdominal pain and vomiting (multiple times an hour) causing dehydration and Emergency Room (ER) intervention.   Upon recovery, 39% may have lingering abdominal pain, nausea, headache.  Many have daily comorbid symptoms including anxiety/depression, limited stamina, sleep disruption, postural orthostatic tachycardia syndrome (POTS), and constipation/irritable bowel syndrome.  Approximately 75% of parents can identify triggers including excitement (birthdays, holidays), infections, lack of sleep, dietary (aged cheese, chocolate, MSG) and menses in teenage girls.  During adolescence, the CVS may resolve but many evolve into migraines or POTS and a few continue CVS into adulthood.

How is CVS treated?

The treatment is similar to that of migraines.  Lifestyle interventions can prevent episodes such as adequate fluids and calories (protein over carbohydrates), regular exercise, sleep hygiene and avoiding triggers.  If episodes are mild (< 24 hours long, no ER visits), lifestyle changes, mitochondrial supplements, behavioral approaches may suffice.  If episodes are moderate (24-48 hours, occasional ER visits) or severe (> 48 hours, repeated ER visits) daily propranolol, cyproheptadine, aprepitant, possibly amitriptyline or topiramate, may prevent episodes.  NSAIDS and nasal triptans, or aprepitant, if given during the prodrome (warning phase) may abort the episode.  A written plan of management for the ER can facilitate early IV intervention using ondansetron or fosaprepitant, NSAIDs for pain and mild sedative (diphenhydramine), which may forestall hospitalization.

Which doctors can help my child?

Most primary care physicians know little about CVS.  In the US, pediatric gastroenterologists and neurologists know more about diagnosis and treatment.  In other countries, pediatric consultants may be the best resource.  The Cyclic Vomiting Syndrome Association has the most up to date literature, treatment guidelines and educational videos (CVSAonline.org).