What are the other Names for this Condition? (Also known as/Synonyms)

• CVS (Cyclic Vomiting Syndrome)
• Familial Cyclic Vomiting Syndrome (subtype)

What is Cyclic Vomiting Syndrome? (Definition/Background Information)

• Cyclic Vomiting Syndrome (CVS) is a condition characterized by recurrent, prolonged episodes of severe nausea and vomiting. Episodes of vomiting may last hours or days
• Other signs and symptoms during episodes may include intense sweating, paleness, weakness and fatigue, abdominal pain, diarrhea, fever, dizziness, and headache. Most people with CVS are symptom-free in between episodes, but some people have milder symptoms
• The condition can begin at any age, but it most often begins in childhood. A subset of people with CVS also have neurologic or neuromuscular disorders; the condition in these cases is referred to as "CVS plus"
• The underlying cause of CVS is unknown. Data suggest there is a strong genetic component for CVS in children, involving changes (mutations) in mitochondrial DNA
• Researchers believe that the condition primarily affects the brain, causing abnormalities in how the brain and gut interact. Many people with CVS report "triggers" for episodes, such as excitement, stress, infections, eating certain foods, and menstruation. People with a family history of migraines may be more likely to develop CVS
• Treatment strategies for controlling symptoms may include avoiding triggers, medications to prevent or relieve nausea, tricyclic antidepressants, anti-migraine medications, and supplements called coenzyme Q-10 and L-carnitine.
• While some people with CVS outgrow the condition within a few years, others continue to have episodes through adulthood. Children who outgrow CVS may go on to develop migraines

(Source: Cyclic Vomiting Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Cyclic Vomiting Syndrome? (Age and Sex Distribution)

• Cyclic Vomiting Syndrome can affect both children and adults; although, onset of the condition is more common during childhood
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Cyclic Vomiting Syndrome? (Predisposing Factors)

• Many people with Cyclic Vomiting Syndrome report "triggers" for episodes, such as excitement, stress, infections, eating certain foods, and menstruation
• People with a family history of migraines may be more likely to develop CVS

(Source: Cyclic Vomiting Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cyclic Vomiting Syndrome? (Etiology)

The underlying cause of Cyclic Vomiting Syndrome is unknown.

• Data suggest there is a strong genetic component for CVS in children, involving changes (mutations) in mitochondrial DNA
• Researchers believe that the condition primarily affects the brain, causing abnormalities in how the brain and gut interact

(Source: Cyclic Vomiting Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Cyclic Vomiting Syndrome?

The signs and symptoms of Cyclic Vomiting Syndrome may include:

• Anorexia
• Ataxia
• Attention deficit hyperactivity disorder
• Autism
• Cardiomyopathy
• Cognitive impairment
• Exercise intolerance
• Gastrointestinal dysmotility
• Generalized hypotonia
• Growth delay
• Hearing impairment
• Intellectual disability
• Lethargy
• Microcephaly
• Migraine
• Mitochondrial inheritance
• Motor delay
• Multifactorial inheritance
• Nausea
• Pallor
• Seizures
• Strabismus
• Vomiting

Most people with CVS are symptom-free in between episodes, but some people have milder symptoms.

(Source: Cyclic Vomiting Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Cyclic Vomiting Syndrome Diagnosed?

Cyclic Vomiting Syndrome is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cyclic Vomiting Syndrome?

The complications of Cyclic Vomiting Syndrome may include:

• Severe malnourishment
• Heart abnormalities
• Disruption of a balanced physical and mental growth in children

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Cyclic Vomiting Syndrome Treated?

• Treatment of Cyclic Vomiting Syndrome (CVS) aims to prevent episodes (prophylactic therapy), stop episodes (abortive therapy), and provide supportive care while symptoms are present (supportive therapy)
• There is no specific treatment that has been proven effective in controlled trials, but several therapies based on observation and experience (empiric therapies) have been effective in case series
• Treatment options for each person may depend on the person's age, whether there is a family history of migraines, the severity of episodes, and how often episodes occur
• People with CVS should consult with their doctor about a personalized treatment plan

Prophylactic therapy options include:

• Avoiding triggers of episodes (e.g. certain foods, physical exhaustion, sleep deprivation, or psychological stress)
• Various medications (usually for patients with more than one episode per month). Examples include cyproheptadine, amitriptyline (a tricyclic antidepressant), various anti-convulsants, propranolol, and erythromycin. There is consensus among experts that amitriptyline should be used as the first treatment for children over 5 years old, and cyproheptadine for children under 5 years old
• Approximately 80% of those with a family history of migraines respond well to anti-migraine medications
• Supplements called coenzyme Q-10 and L-carnitine. Retrospective studies have shown these to be very effective

Abortive therapy options include various anti-migraine and anti-nausea medications such as ondansetron, promethazine, prochlorperazine, and a group of drugs used for migraines called triptans (e.g. sumatriptan).

Supportive therapy is needed when both prophylactic and abortive therapies are unsuccessful. Supportive care is an extremely important aspect of treatment while a person is having symptoms. This may involve:

• Oral fluids if possible
• IV fluids if needed
• Sedatives or pain medications to allow for sleep and a break from severe nausea.

(Source: Cyclic Vomiting Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How can Cyclic Vomiting Syndrome be Prevented?

Currently, it is typically not possible to prevent Cyclic Vomiting Syndrome. However, controlling some of the known triggers (such as certain foods, stress, and treating infections early) may help avoid the vomiting episodes.

What is the Prognosis of Cyclic Vomiting Syndrome? (Outcomes/Resolutions)  

The long-term outlook for people with Cyclic Vomiting Syndrome varies from person to person.

• Many children with CVS "outgrow" the condition by late childhood or early adolescence, but some continue to have symptoms through adulthood
• It has been reported that many children with CVS go on to develop migraine headaches by late childhood
• Some of these children experience a phase of abdominal migraines in between
• In adults with CVS, the course of the condition and how long it lasts have not been well-studied
• There is substantial morbidity associated with CVS in adults, possibly due to lack of awareness and long delays in diagnosis
• Although people with CVS are symptom-free about 90% of the time, the condition can be quite disabling
• Children with CVS may miss many days of school and may need home tutoring or home schooling
• Because of increased likelihood of episodes during times of stress or excitement, CVS may interfere with birthdays, holidays, and vacations

(Source: Cyclic Vomiting Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Additional and Relevant Useful Information for Cyclic Vomiting Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/