January is National Migraine awareness month. And while most people have some level of familiarity with migraines, relatively few have ever even heard of cyclic vomiting syndrome, or CVS even though it shares many similarities with migraines.
At our home, we know CVS so well that we’re on a first-name basis. It’s a relatively rare condition that our daughter has on top of all of her other medical rarities. A diagnosis that we hunted for two dreadful, exhausting years.
So even though this isn’t probably the most popular topic in the world, I’m going to take a few minutes to describe what I see when my child falls victim to a CVS episode — because if there’s the slightest chance that I can help a family with a child like mine finally find some answers, then it is time well spent.
Basically, in the blink of an eye, CVS transforms a child full of life, energy and sparkle into huddled mass of pain, incomprehensible nausea and uncontrollable vomiting.
In my daughter’s case, the transformation lasts 7-12 days. Before she was diagnosed, she only had short one-week breaks between episodes. Now, after tight management of her condition, we are coming up on one year since her last last episode. Here’s what it looks like, feels like through my eyes…
October 3, 2007…
I see that look. I know it well. That instant her penny-brown eyes glaze over with a veil of altered consciousness. It haunts me. Taunts me. Lures me into a web of self-blame and guilt over a false sense that something could be done to stop it. That once again I have failed to recognize what that something was. That I let it slip by on my watch like a worthless security guard with a fondness for sleeping on the job. “Oh!” I tell myself, “Look! Look what you’ve done! Just look what you have allowed to happen to your child! You’ve let it happen… again.”
Logically, I know it’s not my fault. That there was nothing that could be done. That nothing could have stopped it. But it’s so very torturous for her. Such a horrific, soul-rending thing to watch, that I often can’t resist the pull to blame someone. I can’t blame her. I won’t blame God. So when someone is to blame, I blame me.
This time, it happens shortly after breakfast.
She runs down the hall, a bundle of silliness, and wiggles. Ebullience incarnate. Then she abruptly stops, lurching in her tracks. And that look takes over, draining the animation from her frame, of all that I know to be my child. That look has suddenly become all.
Her jaw goes slack and a trickle of drool traces slowly down one side of her chin. Her eyes go completely dead before rolling back as she crumples to the floor in a fit of violent retching and gagging. She won’t throw up though, because she can’t. At the age of 18 months she had a surgery called a Nissen Fundoplication. It was supposed to fix everything.
A simplified description of her Nissen: The upper portion of her stomach was stretched and sutured around the base of her esophagus so that as her stomach fills, the base of her esophagus is squeezed closed, shutting off the possible exit route from stomach to esophagus. The modification essentially makes it impossible for her to throw up.
Because she can’t throw up she experiences intense pain as the pressure in her stomach builds as she retches. I run for her decompression tube, grabbing it from its permanent post on the kitchen counter before running back to the spot in the hall where she has collapsed. Her decompression tube is essentially a two ounce syringe with the plunger removed and a length of tubing fitted to the tip. It plugs in and with a twist, locks into her G-Tube. Her G-tube is a small button-looking valve in the wall of her abdomen, slightly above and to the left of her belly button. Until she reached age four, it was through this valve that she was fed. All of her meals. Every one of them liquid. She eats on her own now, but the G-Tube stays because of this awful thing called CVS.
I roll her over onto her back as she continues to retch violently, her tiny frame quivering and tensing with each wave. I plug in the decompression tube — it must be done quickly so that she doesn’t blow the alterations made during her Nissen surgery completely apart with the building pressure. Something that could cause rupturing, internal bleeding — requiring emergency surgery. The tube is in. A violent rush of air and bilious-looking stomach contents surge into the tube. I hold it slightly higher as it threatens to overflow.
And I watch.
It’s all I can do.
I hold her hand and watch.
My tears splash down on her brow mingling with the tiny beads of cold, clammy sweat that coat her face in a death-pallor-like sheen. I wait as the retching finally begins to abate until finally, her whole body relaxes into limp lifelessness. I stand and leave her in her momentary peace as I go to prepare a comfy nest of blankets and pillows for her on the couch. I return, making certain to have the decompression tube in hand, scoop her up and begin making my way down the stairs. The movement trips another fit of retching and I position her carefully onto the couch and begin the process of the decompression tube all over again while longing to rip out my own heart and fling it somewhere where it won’t ache so dreadfully.
Over and over and over.
Sometimes as often as every 3-5 minutes. Sometimes as infrequently as once every couple of hours. This violent, horrific cycle of retching interrupted by silence and the absence of all that is my child. She is totally and completely incapacitated. Incoherent. She has to go back on tube feedings, back into pull-ups. The longest she has gone in-between episodes is 10 months, the shortest, 2 days.
We never know when one will hit.
With our daughter, it is most often triggered by gluten, or casein in her diet or too large of a meal. Sometimes it is triggered by stress, illness, or excitement. As best we can tell, this most recent episode is the result of a combination of the latter three. She has been completely unresponsive to medication. Strict management of her diet has helped immensely in reducing the frequency of the attacks, but there are always those final three factors that we have so very little control over.
A CVS episode literally stops life in its tracks. There will be no play groups this week. No running outside. She will miss her very first ballet class. She will alternately sleep and retch and nothing more until sometime late next week or early the week after. This is what I see for the entire 7-10 day duration of a CVS episode. And I can do nothing. I hold her, sing to her, stroke her hair, fill hot water bottles, tell her stories while she sleeps.
For two weeks, she is gone.
The world stops spinning and I live for the glimpse, that moment when the spark re-ignites with a flash of life in her beautiful browns. This is what it is like. This is what I see. This is what no one knows or understands.
So how is she doing now?
Through tight management of her triggers, we have now been free of episodes for the past 10 months. Her longest stretch ever. We have also found that while traditional medication has been completely ineffective in preventing and managing these episodes, she has received remarkable relief from a rather unlikely source. We have found that on days when she exhibits any warning sign of an impending episode, if we give her one ounce of 100% mangosteen juice twice a day for 2-3 days, the episode never happens.
Why mangosteen juice works for her and neither Periactin or Topamax do, I have no idea. And I have no idea of whether it would work for anyone else or just for our daughter. All I know is that over the past ten months I have seen this eye-poppingly expensive fruit juice stop an eminent CVS episode in its tracks six times when nothing, and I mean nothing else could. And I’ll ask no more questions and hand over my thirty dollars for a 32 ounce bottle with a smile. (And incidentally, it’s not all that expensive when you consider that I freeze the juice into one ounce cubes and only use them as needed. We still haven’t used up our second bottle since we starting using mangosteen out of sheer desperation last spring.)
So far, we’ve had the best year ever.
And I hope with all my heart that this helps another family who is looking for answers.
Below you will find a more technical medical definition for those of you who are seeking information or are interested in increasing your understanding of CVS. You can also out more about CVS at the Cyclic Vomiting Syndrome Association website, at the National Digestive Diseases Clearinghouse webpage, and at In The Life of a Child by clicking on Cyclic Vomiting Syndrome under Categories in the sidebar.
What is CVS?
(A comprehensive explanation first posted by Jeremy’s Mom)
Definition:
CVS is an uncommon, unexplained disorder of children and some adults that was first described by Dr. S. Gee in 1882. The condition is characterized by recurrent, prolonged attacks of severe nausea, vomiting and prostration with no apparent cause. Vomiting occurs at frequent intervals (5-10 times an hour at the peak) for hours to 10 days (1-4 most commonly). [1.] The episodes tend to be similar to each other in symptoms and duration and they are self-limited. The child is generally well between episodes. [2.]
Onset:
The onset of CVS occurs in infancy through adulthood but most commonly between age 3-7. [1.] It can persist for months to decades. The episode may recur several times a year or several times a month. [2.] Females are affected slightly more than males.
Symptoms:
The child may be prone to motion sickness, and there is often a family history of migraine. [3.] Episodes may begin at any time, but typically start during the night or early morning. [1.] There is relentless nausea with repeated bouts of vomiting or retching. The child is very pale and resists talking. They often drool or spit and have an intense thirst. [2.] There is often intense abdominal pain and less often headache, low-grade fever and diarrhea. Prolonged vomiting may cause mild bleeding from irritation of the esophagus. [3.] Patients often describe being ‘possessed’, out of control and stuporous. [4.] The symptoms are frightening to the child and family and can be life-threatening due to dehydration and electrolyte imbalance. [5.]
Diagnosis:
CVS is difficult to diagnose because it is infrequently seen in clinical practice and because vomiting may be caused by a large number of common disorders other than CVS. There are as yet no blood tests, x-rays or other specific procedures used to diagnose the disorder. The diagnosis is made by careful review of the patient’s history, physical examination and studies to rule out other diseases that may cause vomiting similar to that seen in children with CVS. [1.]
Triggers:
Although some patients know of nothing that triggers attacks, many can identify specific circumstances that seem to bring on their episodes. Emotional stress, intense excitement (birthdays, holidays, vacations), [1.] and colds or flus [2.] are the most frequently reported triggers. Specific foods or anesthetics may also play a role. [3.]
Treatment:
Treatment is generally supportive with early intervention in a dark quiet environment for sleep and I.V. fluids when needed. [1.] Medication trials sometimes succeed in finding something to prevent, shorten or abort the episodes. [2.] An essential component of treatment is the doctor-patient-family relationship. It involves a physician who does his/her best to understand CVS, is supportive and willing to coordinate the care in collaboration with all involved. [3.] A family/professional network, such as CVSA, can help heal a family that has been in doubt and despair for years. [4.]
- 1. Fleisher, D. and Matar, M. (1993), ‘The cyclic vomiting syndrome: A report of 71 cases and literature review” J Pediatr Gastroent Nutr; 17(4), 361-369.
- 2. Fleisher, D. R. (1994). Cyclic Vomiting. In P.E. Hyman & C. DiLorenzo (Eds.), Pediatric gastrointestinal motility disorders (pp. 89-103). New York: Academy Professional Information Services.
- 3. Forbes, D. (1995). Cyclical Vomiting Syndrome. Journal of Paediatric Child Health, 31, 67-69.
- 4. Gee, S. (1882), ‘On fitful or recurrent vomiting’ Saint Bartholomew’s Hospital Reports, 18, 1-6.
- 5. Hoyt, C. and Stickler, G. (1960). ‘A Study of 44 children with the syndrome recurrent CVS’ Pediatrics, 25, 775-780.
- 6. Li, B U.K. (Ed.) (1995). Proceedings of the international scientific symposium on CVS held July 1994 in London, England, J Pediatr Gastroent Nutr; 21, (Suppl. 1).
- 7. Pfau, B.T., Li, B U.K., Murray, R.D., Heitlinger, L.A., McClung, H.J. & Hayes, J.R. (1996). Differentiating cyclic from chronic vomiting patterns in children. Pediatrics, 97, 364-368
Find out more about Michelle
Read Michelle’s weekly column
Meet Michelle here each Monday for Magic Marker Monday
Questions? Please feel free to email Michelle at childlif[at]gmail[dot]com or come and visit her at In The Life of a Child