A 28-year-old postdoctoral researcher experienced multiple episodes of vomiting from the age of 3 years. The episodes occurred with upper respiratory tract infections and asthma, and generally lasted 24-48 hours. The patient's mother had nearly identical episodes during childhood. The maternal grandmother experienced hyperemesis gravidarum. The patient's sister and paternal grandfather had classical migraine headaches.
The patient first presented to the hospital at age 10 1/2 years. A typical vomiting attack had been triggered by a cold, but had not resolved after four days. Despite treatment with intravenous fluids, the vomiting continued for a further two days before resolving. Cyclical vomiting was diagnosed. She was subsequently hospitalised repeatedly with episodes of about four days duration, with vomiting occurring every 5 to 10 minutes at peak. She continued to have similar attacks during every infection, but never without prior infection. The symptoms were refractory to anti-emetics available at the time (eg. metoclopramide, prochlorperazine).
Shortly before Christmas of that year (1986), a 6 1/2-day vomiting episode was triggered by pneumonia, and the patient spent the Christmas period in hospital. The following year (1987), a cold triggered a CVS attack two days before Christmas. Having spent two successive Christmases in hospital, the patient hoped to be well the next year. However, she was woken by nausea in the early hours of Christmas Day 1988 and began vomiting. Uniquely, this episode had not been triggered by an infection, and the patient (then aged thirteen) recognised this. Subsequently, episodes rapidly increased in frequency: compared with an average of 7.3 episodes per year in the three preceding years, the patient averaged 22.3 episodes per year over the three years following Christmas 1988. Many further episodes now occurred without infection, but coincided with visits to certain places (e.g. friend's house), or commenced on specific days of the week (e.g. Saturday). Most episodes resulted in 3-8 day hospitalisations. At one point, a repetitive cycle commenced, where each episode occurred exactly two days after discharge from hospital following the previous attack. The patient was aware of this pattern and consciously afraid of becoming ill at the expected time. Her attacks always commenced nocturnally and she became reluctant to go to sleep, fearful of another episode beginning.
The patient experienced exhaustion and severe, intractable nausea for the entire length of her emetic episodes (up to 6 1/2 days), and the nausea caused marked behavioural changes. Sleep was the only state that allowed her to be insensible to nausea, but she was often unable to lie down, as this intensified her nausea, causing retching and heartburn. Instead, she spent hours sitting motionless in bed with her hips and knees flexed, her arms and head resting on her knees and her eyes closed. She was unable to lie supine for abdominal palpation. She would repeatedly expectorate, as she experienced hypersalivation and was too nauseated to swallow. Accumulated oral secretions, and the profound fatigue and confusion that accompany nausea, contributed to her verbal unresponsiveness. Attempting to reduce intolerable nausea, she frequently induced vomiting by drinking large volumes of water. This also diluted bile and gastric acid, thus lessening oesophageal and oropharyngeal discomfort during vomiting.
Consistent with the natural history of the condition, the attacks gradually became less frequent as the patient grew older. Availability of newer anti-emetic agents (5-HT3 antagonists) also contributed to the improvement: prompt treatment of episodes with intravenous ondansetron, ranitidine and analgesics reduced the duration and severity of attacks, and the same medications were partially successful as prophylactics. Thus, having achieved partial symptom control, and an understanding of how her anticipation of episodes was exacerbating her condition (and therefore when to use prophylactic medication), the patient felt more in control of her illness, feared it less, and conditioned responses to situations previously associated with CVS attacks gradually diminished. To date, the patient has been hospitalised nearly 100 times, however now experiences vomiting attacks only rarely (fifteen episodes over the past decade, including only one episode over the past five years).