chronic intestinal pseudo obstruction

  Hi! I am 27, and I have myasthenia gravis, multiple sclerosis and chronic intetsinal pseudo obstruction.  I am TPN dependent, and also have gtube for venting and jtube for very slow feeds.  I also have a shrinking spinal cord and cerebellum.  Can there just be one disease that is causing all this.  My doctors and I have searched everywhere and we are at a loss.  If you could give us a hint, I would appreciate it.
  Thanks,
  Jennie
Dear Jennie,

Chronic intestinal pseudo-obstruction results from ineffective intestinal propulsion secondary to defects in the either the enteric nervous system (specialized nerves which innervate the gastrointestinal system) or in the smooth muscle found in the intestinal wall. In other words, because the smooth muscle lining the small and large intestinal wall is damaged, food travels slowly down the intestinal tract and causes symptoms. These symptoms mimic an obstruction or blockage even though a mechanical obstruction is not present. Reflux, difficulty swallowing, abdominal fullness and bloating, nausea, vomiting, constipation, diarrhea, abdominal pain and weight loss may occur. In all cases a mechanical obstruction must be excluded with x-rays of the gastrointestinal tract. Sometimes a full thickness biopsy of the intestine must be performed to confirm the diagnosis. This may require surgical exploration. Some centers perform small intestinal manometry (pressure studies) to document poor intestinal propulsion. A family history is present in up to 30 percent of patients with chronic intestinal pseudo-obstruction.
Chronic intestinal pseudo-obstruction may be primary (idiopathic or without secondary cause) or secondary. In secondary pseudo-obstruction an underlying cause may be found. A variety of neurologic diseases, smooth muscle diseases, endocrine and metabolic disorders, anatomic abnormalities, drugs, disorders of the enteric nervous system and other diseases may be present. You mentioned several neurologic diseases that you have been diagnosed with. Parkinson's disease, viral illnesses, brain stem-strokes and tumors, spinal cord trauma, orthostatic hypotension syndromes (diseases where blood pressure decreases with postural changes) and pandysautonomia are the usual neurologic diseases associated with chronic intestinal pseudo-obstruction. If you have not done so already, I would recommend that you confirm your diagnosis with a neurologist.
Unfortunately, as you pointed out, treatment for chronic intestinal pseudo-obstruction is generally supportive. In addition to the nutritional therapies you are currently receiving you may want to try a class of medications called prokinetic agents. Examples of prokinetic agents include: cisapride (Propulsid), metaclopramide (Reglan), erythromycin and soon to become available in the U.S. domperidone (Motilium). These help to more rapidly empty out the stomach. Another consideration is to treat small bowel bacterial overgrowth with antibiotics as needed. Since intestinal contents move slowly down the gastrointestinal tract, the growth and proliferation of bacteria occurs. Small bowel bacterial overgrowth usually presents with diarrhea, poor absorption and weight loss.  I hope you find this information helpful.
This response is being provided for general informational purposes only and should not be considered medical advice or consultation. Always check with your personal physician when you have a question pertaining to your health.
If you would like to be seen at our institution please call 1-800-653-6568, our Referring Physicians' Office and make an appointment to see Dr. Muszkat, one of our experts in Gastroenterology.
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*Keywords: chronic intestinal pseudo-obstruction, causes, symptoms, treatment

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