CVS in Adults

Introduction

Cyclic vomiting syndrome (CVS) is a chronic disease marked by severe, recurrent episodes of nausea and vomiting in a person who is otherwise healthy. 

CVS episodes often flip-flop with states of wellness. Vomiting may occur every 5-10 minutes for several hours during an episode. On average, CVS episodes last 3-7 days but vary with each person. An episode may be accompanied by stomach pain, tiredness, sensitivity to light, and/or headaches. Episodes may begin at any time of the day, but often start in the middle of the night or early morning hours. They may be triggered by positive ‘stress’ (vacations) or negative stress (job loss).

CVS can be quite debilitating and has a significant negative impact on patients, families, and the healthcare system. Short-term disability and absenteeism from work and school are common. 

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The Condition

Many patients have a long history of symptoms and frequent emergency department (ED) visits and hospitalizations. A clear diagnosis may have been difficult to obtain which can be frustrating! 

The symptom episodes of CVS tend to follow the same pattern for each individual patient over time. The symptom episodes are characterized by four phases:

Research

Studies of adults with cyclic vomiting syndrome (CVS) show that the most consistent symptoms are repeated episodes of nausea, vomiting, and abdominal pain, separated by symptom-free periods. Adults tend to experience more severe abdominal pain than children. Patients ranged in age from 18 to 62, showing that CVS symptoms are similar across different ages.

The specific cause of CVS is unknown, but both genetics and environmental factors likely play a role. While some genetic mutations have been associated with CVS in children, whether or not these actually cause symptoms is unclear. A system called the endocannabinoid system has also been shown to affect CVS. Our bodies produce substances that are similar to those in cannabis which are helpful in combating stress and preventing nausea and vomiting. Problems in this system may be one reason patients develop CVS. Also,  studies of the brain using MRI (a type of scan that uses magnetic radiation) show that patients with CVS respond differently to stress. 

Treatment

The management for CVS involves lifestyle changes, daily medications (often referred to as prophylactic or preventative medications), medications to take when you start getting symptoms (often referred to as abortive medications), and supportive treatment during an episode. Know your lingo! This will help when you meet with your physician.

Lifestyle changes are very important. Staying well hydrated, avoiding excessive alcohol and taking your daily prescription medications are all important. Also, there are several triggers for CVS. Learn how to recognize and manage them.

Medications:

Preventive Medications

• Mitochondrial supplements: L-carnitine, Co-enzyme Q10, and Riboflavin (Vitamin B2)
  These supplements have been found to be effective in reducing the frequency and severity of CVS episodes in small studies. They may help your body produce energy, which is important when you have an episode.
• Amitriptyline (Elavil®) and Nortriptyline (Pamelor®)
  These medications belong to a class of medications known as tricyclic antidepressants or TCAs. These medications have been shown to decrease the number and severity of CVS episodes as well as decrease pain (They also may help with migraine headaches). You will need EKGs (a safe and painless test that records the electrical impulses of your heart) before you start taking these medications and throughout your treatment because this medication has the potential to change your heart waves. Other side effects include weight gain, dryness of mouth, and constipation. Nortriptyline (Pamelor) may have fewer side effects, but amitriptyline (Elavil®) is generally tried first, as this has been shown to work in studies, and there are more data to support its use. Amitriptyline is generally used at a dose of 75-100 mg based on your response to the medication and how you tolerate it. It has been shown to be effective in 65-85% of patients. Nortriptyline is generally used at a dose of 75-100 mg, like amitriptyline.
• Topiramate (Topamax®)
  This medication is mostly used in patients with moderate to severe CVS. This is traditionally used as an anti-seizure medication; however, it has also been found to reduce the frequency and severity of CVS episodes and is also used in migraine headaches. Side effects may include cognitive dysfunction, tingling or numbing sensation on the skin, headache, fatigue, dizziness, or mood problems. This medication may interfere with the efficacy of oral contraceptives.
• Zonisamide or levetiracetam
  These medications can be used in patients who do not respond or are unable to tolerate amitriptyline. Zonisamide is an anti‐epileptic (AED), but studies show that it also works in CVS. The starting dose of zonisamide is 100 mg daily is typical, but your doctor may increase the dose up to 400 mg/day if needed.
  Levetiracetam is typically started at 500 mg twice daily and may be increased by your doctor until the target dose of ~1000–2000 mg is achieved. Generic formulations are not particularly expensive. Side effects commonly reported include fatigue, confusion, impaired concentration, or headache. These can be managed by switching to the alternative AED. Patients can switch from zonisamide to levetiracetam and vice versa.

Abortive medications (Medications commonly used when an episode is coming on)

• Sumatriptan (Imitrex®) nasal spray or Zolmitriptan (Zomig®)
  CVS and migraine headaches are thought to be linked, because of this the use of anti-migraine medications may be useful in stopping a CVS episode. It is most effective when used at the first sign of symptoms. A nasal preparation may be more effective than a tablet as you may just vomit the pill. Side effects may include tingling, numbness, dizziness, nausea, and drowsiness. These medications should not be given to those with underlying coronary artery disease, peripheral vascular disease, hypertension, or stroke.
• Ondansetron (Zofran®)
  This is an anti-vomiting medication used at the beginning of your symptoms. It can help control your nausea and vomiting. Generally, it is prescribed as a tablet that dissolves under your tongue. You will need an EKG before beginning this medication as this medication has the potential to cause changes in your heart rhythm. Side effects, while uncommon, may include headache, dizziness, drowsiness, diarrhea, constipation.
• Diphenhydramine (Benadryl®)
  This medication makes you sleepy and this can help stop the cycle of vomiting. Alcohol, sedatives, and tranquilizers may increase drowsiness and should be avoided when taking this medication. This medication may impair the mental and/or physical abilities required for the performance of potentially hazardous tasks, such as driving a vehicle or operating machinery.
• Promethazine (Phenergan®)
  This is an anti-vomiting medication that may help stop your nausea and vomiting. This medication may impair the mental and/or physical abilities required for the performance of potentially hazardous tasks, such as driving a vehicle or operating machinery. The impairment may be amplified if used with other central-nervous-system depressants such as alcohol, sedatives/hypnotics (including barbiturates), narcotics, narcotic analgesics, general anesthetics, tricyclic antidepressants, and tranquilizers. Side effects may include drowsiness, blurred vision, dizziness, confusion, disorientation.
• Prochlorperazine (Compazine®)
  This medication helps decrease severe nausea and vomiting by altering chemicals in your brain that may be causing your symptoms. This medication can be prescribed as a tablet, time released capsule, as an injection, suppository or through an IV. Your physician will work with you to find the correct dose for you but should start with the lowest recommended dosage. You should communicate with them about how it is working to ensure you receive the correct dosage. Side effects may include drowsiness, dizziness, amenorrhea, blurred vision, skin reactions and low blood pressure.
• Aprepitant (Emend®)
  This is a newer anti-nausea and vomiting medication that has been shown to be helpful in patients with nausea/vomiting due to chemotherapy. (It is not a cancer medication.) This medication is used when all others have failed and has been useful in patients with moderate to severe CVS. It is generally very well tolerated; however, it may reduce the efficacy of oral contraceptives in some patients. This is an expensive medication, and your doctor will need to do a prior authorization. This medication will come in a kit containing three tablets to be taken one a day for three consecutive days. Your healthcare provider may have you repeat this course of treatment once weekly as needed. Side effects may include hiccups, fatigue, increased appetite, mild headache, constipation. This medication is also used as a preventive medication.
• Lorazepam (Ativan®)
  This medication helps relax you, which may make you sleepy and help stop your nausea and vomiting. This medication is not meant to be used daily as it is habit forming. Side effects may include respiratory depression (slow and ineffective breathing), sedation, dizziness, weakness, and unsteadiness.

For other information and support, contact:

Cyclic Vomiting Syndrome Association
PO Box 270341
Milwaukee, Wisconsin 53227
Email: cvsa@cvsaonline.org
Website: www.cvsaonline.org
Phone: (414) 342-7880

Adapted from article 818- What is Cyclic Vomiing Syndrome by Thangam Venkatesan, MD, professor of Medicine, Division of Gastroenterology and Hepatology, Medical College of Wisconsin, Milwaukee, WI; Katja Kovacic, Assistant Professor of Pediatrics Division of Pediatric Gastroenterology, Medical College of Wisconsin, Milwaukee, WI; Melanie Kruefer BSN, RN, NPD-BC, System Education Coordinator-RN, Organizational Learning