When should prophylactic therapies be used, Do diet and supplements play a role? Questions for my doctor: and what medications are tried? We don’t know about the role of diet in CVS. However, As mentioned previously, prophylactic treatments some patients do benefit from special diets which in- are medications given on a daily basis to try to pre- volve the restriction of certain foods (cheeses, choco- ______vent episodes from coming on. Studies suggest late, legumes, wine and other foods). Because energy ______that in patients with frequent episodes (every 1-2 depletion can be a trigger for episodes, most doctors months), prophylactic treatment can lessen the are recommending even pacing of meals and frequent ______frequency and severity of episodes. Therefore, in healthy snacking. The avoidance of fasting is highly ______Cyclic patients having frequent episodes (i.e. every 1-2 recommended. months) prophylactic treatment should be consid- ______ered. However, if episodes are infrequent (i.e. Will I ever get better? ______once a year), prophylactic therapy is probably un- Fortunately, once properly diagnosed and treated, most pa- ______Vomiting necessary. The most common prophylactic medi- tients improve. In addition, some small studies of children cations include , cyproheptadine, and suggest that many children may “outgrow” their illness, most ______propanolol. In some patients with resistant disease, commonly during their pre-teen or teenage years. ______anticonvulsants (i.e. medicines usually used to treat seizures) may be used. These medications include ______Syndrome , levetiracetam. While all these prophy- How can I learn more? ______lactic medicines are generally safe, each has a Talk to your physician, to other patients, and also ex- ______different side effect profile, and so the benefits and plore the website www.cvsaonline.org. If you are inter- ______101: risks of prophylactic therapy need to be reviewed ested, join the Cyclic Syndrome Association, with your physician. ______which offers educational materials, conferences, and A primer for Can dietary changes be helpful? multiple opportunities for support coping with this illness ______While there is no firm medical evidence to prove ______Patients and parents that they are helpful, some physicians have used ______the dietary supplements including L-carnitine, co- enzyme Q10, and Vitamin B-100 as additional ther- ______apies to treat CVS. Commonly-used dosages for ______the first two supplements can be found at ______www.cvsaonline.org. ______What about ? ______In some patients, CVS may be triggered by either ______physical or psychological stress, most often happy excitement such as holidays or vacations. Physical ______stresses that can trigger episodes include ______such as colds and viruses. A subset of women may ______develop CVS or around their periods. Psychological factors also play a role. Some pa- ______tients will have episodes triggered by negative CVSAUSA/Canada ______(unhappy) stressors, such as tests or term papers. P.O. Box 270341 Others will have episodes triggered by positive ______Milwaukee, WI 53227 stressors (such as holidays and visits with rela- ______Athos Bousvarous, MD, MPH tives). However, many cannot identify a specific (414) 342-7880 stressful event as a trigger for CVS. While the ill- ______Children’s Hospital Boston Email: [email protected] ness is not caused by stress, stress can make ______things worse, and CVS is a stressful illness. There- www.cvsaonline.org fore, in many patients treatments to promote relaxa- ______tion (counseling, yoga, acupuncture) may help.

What is cyclic vomiting syndrome? between episodes is between 1 and 3 months for malrotation. In children with malrotation, the intes- What are the abortive therapies (therapies that most patients. However, some patients will have tines are abnormally positioned in the body from If you’ve made your way to the CVSA website, you might help DURING an episode)? more frequent episodes (every 1-3 weeks), and birth, and can twist on themselves. A second condi- may suspect you/your child has cyclic vomiting others will have episodes that occur rarely (every 6- tion to be considered is ureteropelvic junction ob- Once CVS episodes start, they can be very hard to syndrome (CVS). Cyclic vomiting syndrome is 12 months). struction, in which urinary flow out of the kidneys is stop. For many patients, the best treatment is sup- not a single disease, but rather a specific pattern of blocked, leading to backup of urine into the kidney, portive, and can include intravenous fluids with a high vomiting that can be seen in a number of disease Patients can have many other symptoms during an which in turn leads to vomiting. At the present, re- percentage of dextrose and a quiet room in a hospital. states. There is no proven test to diagnose cyclic attack, including listlessness, paleness, weight loss, search is taking place to study the effects of using Anti- medicines, including vomiting syndrome. Therefore, the correct diagno- , high blood sugar, reduced urine output, for CVS Very rarely, brain tumors or other (Zofran), promethazine (Phenergan), and chlorproma- sis is made by having a doctor take a careful histo- and abnormal blood chemistries. lesions in the head can present with recurrent vomit- zine (Thorazine) are sometimes used to reduce the ry, performing a careful physical examination, and ing. Lastly, metabolic disease (hereditary enzyme feelings of nausea. Because patients may be anxious conducting tests to exclude other diseases. How does a doctor distinguish CVS from other causes of vomiting? deficiencies) can cause recurrent vomiting, particu- and just feel lousy during an attack, they may benefit What features in the medical history suggest larly in infants and young children, because there is from an antianxiety medication such as Diagnosing cyclic vomiting is difficult for most doc- CVS? a missing enzyme in the patient leading to buildup of (Ativan). Other patients may benefit from antimigraine tors, because CVS is an uncommon condition that toxins in the blood and urine. treatments like sumaptriptan (Imitrex). The combina- Many different conditions cause recurrent vomiting. has no simple test to make a diagnosis. Often, tion of Thorazine and Benadryl is often used to sedate In most cases, however, CVS can be differentiated young children presenting with CVS are said to What testing should be performed if cyclic vom- the person deeply enough to all rest and possibly from other conditions causing vomiting by three have “a stomach virus” (viral ). How- iting is suspected? break the cycle After enough time passes (usually main features: paroxysmal (sudden onset), stereo- ever, when patients develop recurrent bouts of vom- hours to days), most patients come out of the episode. In general, the history will strongly suggest CVS. typical (similar episodes) with accompanying pale- iting lasting hours or days, without diarrhea, and are However, in many cases, a physician may need to ness and listlessness, and periods of wellness in completely well in between, the doctor (or parent) perform further tests to exclude other conditions. between. should consider CVS. These tests may include: an upper GI series (x-ray First, the vomiting in cyclic vomiting is paroxys- What other conditions cause vomiting? of the stomach to exclude malrotation), an ab- mal (sudden onset). Most patients with CVS feel dominal ultrasound (ultrasound of kidneys and There is a long list of diseases and problems that fairly well, until they get a sudden attack of nausea, gallbladder to rule out pathology), and a CT scan or cause vomiting. These include: gastroesophageal which usually progresses to vomiting a little later. MRI of the head. In addition, during a CVS epi- reflux (acid reflux), stomach infections or inflamma- The nausea and vomiting often start in the early sode, blood tests and urine tests may need to be tion, food allergies, pancreas inflammation and uri- morning and can even wake the patient from sleep. obtained to evaluate for other causes of the episode. nary infections. However, most conditions that including , inflammation of the pancreas, Second, the vomiting episodes are stereotypical. cause vomiting tend to either be self-limited (i.e. and metabolic enzyme problems). In some patients, “Stereotypical episodes” means each vomiting they have a beginning and an end), or chronic (i.e.: endoscopy (examination of the and attack resembles similar episodes they have had people vomit a little bit every day or two). Having stomach with a scope) may be needed. previously. Most often, the attacks last between 8 recurrent bouts of severe vomiting separated by well and 24 hours. However, for some patients, attacks periods is very unusual, and should make a physi- If I think my child or I has cyclic vomiting, what can be as brief as 1-2 hours, and for others they cian or family suspect the diagnosis of CVS. should I do? can last up to days. Episodes often begin with nausea, and progress to vomiting, with some peo- What causes vomiting in a CVS pattern? The most important thing to do is to find a physician who can take a careful history and determine if the ple vomiting several times an hour. During the A number of medical studies suggest that in most history is consistent with CVS. While some pediatri- vomiting episodes, patients often like to be left patients, CVS is related to . The sudden cians are comfortable and have experience in mak- alone in a dark quiet place. Other symptoms can onset of attacks with spontaneous resolution is also ing the diagnosis, very often a definitive diagnosis also occur during the episode. Many patients be- seen in patients with migraine . Most will require referral to a pediatric specialist, either a come dehydrated, and benefit from being hospital- (but not all) children with CVS have a family history neurologist or gastroenterologist). After the history, ized and given intravenous fluids. The episodes of migraine. As affected children get older, many examination, and testing, if your physicians feel that usually resolve by themselves without any obvious will go on to develop migraine headaches. In addi- CVS is likely, they will discuss therapy with you. intervention or explanation. tion, many of the treatments used to treat migraine Athos Bousvaros, MD, MPH Third, most patients feel completely well in be- headaches are also effective in treating CVS. What therapies are available? Children’s Hospital Boston tween episodes (intervening wellness). After the Do other conditions need to be excluded? Treatment for CVS is divided into two major types: episode resolves, the affected patient often returns abortive therapy and prophylactic therapy. Abor- Because there is no definitive test to diagnose CVS, to their “normal self” within a few hours, and starts tive therapy means giving treatments to stop the a number of other conditions may need to be ex- drinking and eating. The period of wellness in episode once it starts, and only giving that treatment cluded as causes of the disorder. These include during the sick, in order to prevent episodes from anatomic abnormalities of the bowel such as coming on.