Part of the problem is the lack of continuity of care for these patients, especially those who present repeatedly to hospital emergency departments. Dr. Fleisher suggests that more CVS centers should be created and staffed by two to three physicians available 24/7, as well as by nurses and mental health professionals. A patient can be managed routinely by his primary care physician, and then referred when necessary to a CVS center cognizant of his history, he added.
The Cyclic Vomiting Syndrome Association (www.cvsaonline.org
The average delay in making the diagnosis of CVS is 8 years from the time symptoms first appear. DR. LI
Knowledge, Patience Key to Managing the Four Phases of Cyclic Vomiting Syndrome
A physician must be knowledgeable, accessible, patient, nonjudgmental, and quick to respond when treating adults with cyclic vomiting syndrome, Dr. Fleisher said, and follow a rational treatment plan, tailored to the phase of the disease, that includes sedation when symptoms rage uncontrollably.
The CVS cycle has four phases, said Dr. Fleisher, who has treated more than 350 pediatric and adult CVS patients at the University of Missouri Hospital and Clinics, Columbia. The time between episodes, when the patient is feeling well, can be considered the first phase. The goal in this phase is to prevent a CVS episode by recognizing and controlling triggers, such as menstruation, noxious stress, pleasant excitement, fatigue, or infection. Some patients have interepisodic dyspeptic nausea and abdominal discomfort, especially in the mornings, and they may respond to proton-pump inhibitors.
The physician should help the patient regain a sense of being in control, rather than being at the mercy of CVS symptoms; this can be critical in determining overall treatment success, he said.
Dr. Fleisher considers CVS to be a manifestation of migraine diathesis in some patients. In a group of 41 adults with CVS, 70% had migraines during or between episodes, and 57% had first- and/or second-degree relatives with migraines. For these patients, migraine prophylaxis is essential to prevent CVS symptoms.
For two-thirds of Dr. Fleisher's group, severe anxiety and panic attacks triggered CVS episodes, and the panic symptoms persisted for hours or days. He likens the symptoms to those of an “adrenergic storm” seen in patients with pheochromocytoma. Patients begin to fall into a vicious cycle of anticipatory anxiety, whereby the worry about having a CVS episode increases the likelihood of another attack.
Some CVS patients have a propensity to both migraines and anxiety/panic attacks. A careful medical history can help physicians recognize the pathogenic factors specific for each patient, leading to an appropriate preventive strategy.
In the prodromal phase, the physician and patient have the chance to abort the emetic phase. In Dr. Fleisher's group, 93% had recognizable prodromes; common symptoms included nausea, sweating, epigastric pain or pressure, fatigue or weakness, feeling hot or cold, cramping urge to defecate, abdominal pain, shivering or shakiness, insomnia, food aversion, palpitations, irritability, and panic.
Depending on the symptom, appropriate medications during the prodrome include lorazepam, alprazolam, and/or ondansetron orally or sublingually for nausea, analgesics for abdominal pain, antianxiety medications for anxiety, and a triptan for headaches. Sleep may also be beneficial.
During the emetic phase, the goal is to rapidly terminate the episode, preferably within 1 hour of onset. In a sample of 39 adults with CVS, more than half had vomiting episodes lasting 3 days or more. Steps to take include prevention or correction of dehydration with IV fluids, and IV administration of antiemetics, antianxiolytic agents, and H2-receptor blockers or proton-pump inhibitors. In some cases, IV opiates are necessary for pain control. Patients should be checked for electrolyte depletion, tetany, hematemesis, and secretion of inappropriate antidiuretic hormone.
If the CVS episode cannot be terminated, Dr. Fleisher recommends sedating the patient in a dimly lit and quiet room until the episode passes. “A CVS patient needs to know there is an escape hatch that gets them out of their misery. Without that, the more they will suffer and the more they will coalesce,” Dr. Fleisher said, referring to the process in which CVS episodes become more and more frequent. He recommends chlorpromazine (0.5–1.0 mg/kg) plus diphenhydramine (0.5–1.0 mg/kg) in normal saline over 15 minutes, which can be repeated as often as every 3–4 hours if needed.
The duration of the recovery period reflects the adequacy of management of the emetic phase. Patients with severe fluid or electrolyte deficits will have a more difficult and prolonged recovery. Some patients can tolerate a normal diet soon after the emetic phase passes, while others will tolerate only clear liquids.