Hey everyone! Happy September! This can be a hectic month...back to school, doctor’s appointments, and all of the other things we have to do on a day-to-day basis. We know it can be HARD, but we are here for you! With that said, let’s get into the featured illness for September: CIPO.
Chronic Intestinal Pseudo-Obstruction (CIPO), is a rare disorder that mainly affects the gastrointestinal tract. CIPO usually results from abnormalities that affect the muscles and/or nerves that are involved in the process of moving food through the digestive tract, which is called Peristalsis. These abnormalities cause Peristalsis to become inefficient, which results in symptoms that are extremely similar to those of an actual physical blockage of the intestines.
A physical obstruction of the digestive tract could be stool, food, a tumor, and anything else that stops food from passing through. A pseudo-obstruction often gives the patient the same symptoms, except the cause stems from muscles and nerves, not a physical blockage.
The symptoms of CIPO can include but are not limited to: nausea, vomiting, abdominal pain, distention (abdominal swelling), and constipation. On top of this, the patient can experience unintentional weight loss and anemia from malnutrition. A person may experience “acute attacks” in which the symptoms worsen for a period of time and go back to “normal” soon after. There can also be heartburn or chest pain present, as well as problems urinating (dysuria). Symptoms can vary in severity, but if it is not treated, it can result in severe complications.
The causes of CIPO include damage to the muscles or nerves in the gastrointestinal tract, certain medications like tricyclic antidepressants or narcotics, or toxins that affect a developing fetus (such as fetal alcohol syndrome), and other autoimmune diseases/disorders. There are some cases that can be genetic. Certain infections can also induce CIPO.
The Treatment (From NORD)
There is no specific treatment for individuals with CIPO. Treatment is directed toward the specific symptoms that are apparent in each individual, and to support adequate nutritional needs. Treatment may require the coordinated efforts of a team of specialists. Pediatricians, pediatric gastroenterologists, surgeons, pain management specialists, psychologists, dietitians, and other healthcare professionals may need to systematically and comprehensively plan an affect individual's treatment.The specific therapeutic procedures and interventions for individuals with CIPO will vary, depending upon numerous factors including the specific symptoms present, the site and extent of the affected portion of the GI tract, an individual's age and overall health, tolerance of certain medications or procedures, personal preference and other factors. Decisions concerning the use of particular therapeutic interventions should be made by physicians and other members of the healthcare team in careful consultation with the patient and/or parents based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors. In individuals with secondary CIPO, treatment of the underlying disorder is mandatory and can provide relief of CIPO symptoms. Treatment options for individuals with primary or idiopathic CIPO are complex and varied and often prove difficult. The specific treatment plan may be highly individualized and can include can dietary adjustments, total parenteral nutrition (TPN), enteral feeding, certain medications and surgery.
Individuals with CIP are encouraged to eat five to six small meals a day rather than three large ones and to follow a low-fat, low-fiber diet. Such a diet may emphasize liquids and soft foods. Fatty foods are believed to delay gastric emptying. High fiber foods are thought to be associated with abdominal bloating and discomfort. Some physicians recommend avoiding lactose because of the high incidence of lactose intolerance in the general population. Affected individuals may also be encouraged to follow a low-residue diet, which limits foods that leave undigested parts in the GI tract (these undigested parts normally become part of stool). A low-residue diet is similar, but generally more restrictive than a low fiber diet. Affected individuals may also need to take daily multivitamins and nutritional supplements, which are beneficial for individuals who experience nutritional deficiencies and/or malnourishment.
A variety of different medications may be used to treat individuals with CIP. Medications can help to control symptoms and to avoid complications. Antibiotics may be prescribed to treat bacterial infections and can help relieve diarrhea and bloating. Drugs that are effective against nausea and vomiting (antiemetics) may also be used. Prokinetics are a class of drugs that are often prescribed to individuals with CIP with the intent of improving gastrointestinal motility. Prokinetics improve gastrointestinal motility by increasing the frequency of contractions in the small intestines without disrupting their rhythms. They have proven beneficial in some cases, but overall their effectiveness has been limited. Examples of prokinetic drugs are erythromycin, metoclopramide, domeperidone, otreotide, tegaserod and lubiprostone. A drug called cisapride is a prokinetic agent that has been used to treat CIP in the past, but is no longer widely available after being voluntarily pulled from the market in 2000 because of an association with serious cardiac arrhythmias. Low-dose tricyclic antidepressants and gabapentin have been used to treat chronic pain. Pain medications classified as narcotics are avoided because they can further deteriorate digestive function.
Some individuals and children may be unable or unwilling to eat because of the severity of their disorder or they may be unable to maintain basic nutritional requirements even with supplementation and diet adjustments. In such cases, individuals are put on enteral or total parenteral nutrition (TPN). Enteral feeding is the use of a tube to deliver food directly into the stomach or small bowel. Eventually, affected individuals are given small amounts of food orally. It is important that infants receive small amounts of food orally in order for the infant to learn how to suck and to eat.TPN supplies all daily nutritional requirements such as protein, sugars, vitamins, minerals, carbohydrates and sometimes fats. TPN is a way to bypass how the body normally digests food. With TPN, a special intravenous (IV) line is inserted into a vein and nutrients are delivered directly into the bloodstream. The first dose of TPN is given at a hospital. Eventually, TPN is given at home. The amount of time a person requires TPN varies. TPN is more costly and dangerous than enteral feeding and every attempt should be made when possible to use the enteral path first.
Some individuals with CIPO may be treated by intestinal decompression, a procedure that reduces the pressure within the GI tract. Intestinal decompression is most commonly performed by inserting a tube within the intestines allowing physicians to decompress abnormally swollen (distended) segments of the intestines. Intestinal decompression can improve transport capacities and can result in a reduced rate of hospitalization for affected individuals. Bowel decompression may also be used to treat abdominal pain. In some cases, venting enterstomy can assist in relieving abdominal swelling and bloating. A venting enterstomy is a procedure in which the small intestines are diverted to an artificial opening in the abdominal wall.Rarely, and in very specific and severe cases of CIPO, surgery to remove a section of the intestines may be necessary. Because surgery can potentially worsen CIPO, it only has a limited role in the treatment of specific cases. When CIPO is limited to an isolated portion of the bowel, surgical bypass of the affected segment may be beneficial. Certain surgeries such as the surgical removal of a segment of the intestines (enterectomy) or the surgical creation of a passage between the duodenum and jejunum can potentially decrease the frequency certain symptoms such retching, vomiting and abdominal distention.
In extremely severe cases, such as for individuals who do not respond to other treatments (refractory disease), who were unable to maintain proper nutrition with other therapies, who are dependent on TPN, or who experience complications from TPN such as liver failure, small bowel transplantation may be an option. During a small bowel transplant, the affected portion of the small bowel is removed and replaced with one from a healthy donor. A variety of complications can occur with small bowel transplantation including organ rejection, infections, and lymphoproliferative disease. In addition, the procedure is expensive and requires the continued use of immunosuppressive drugs to lessen the chance of rejection. However, as surgical techniques and immunosuppressive agents improve in efficacy, survival has improved as well.
If you or anyone you know is battling CIPO, you are NOT ALONE! Hope you all have a happy and healthy school year, and that you keep fighting HARD!