Living with A Rare Disease
Chronic Intestinal Pseudo Obstruction
My Story
Isolating
Scary
Lonely
That is life with a rare disease for so many people.
Can you imagine life without food – not being able to eat for months or years at a time, or never again? How about life with frequent hospital stays, unnecessary surgeries, and invasive and uncomfortable procedures? On top of this imagine feeling like you are no longer a person who has a good sense of self and body because now your body doesn’t feel like it belongs to you. You have tubes running up your nose and down your throat, tubes coming out of your abdomen from organs, a piece of your intestine is sticking out of your belly, and there is a needle in your chest that runs directly to your bloodstream.
This is a small part of what life is like for the majority of people who have the rare disease I am here writing about today, a disease that I live with, Chronic Intestinal Pseudo-obstruction (CIPO). Because of CIPO I have intestinal failure and rely on intravenous nutrition that is pumped into my bloodstream through a port in my chest.
Chronic Intestinal Pseudo Obstruction is extremely rare, severe, usually progressive, and debilitating for many of it’s sufferers with only about 150 cases being diagnosed each year worldwide. In the United States a disease is considered rare if it affects fewer than 200,000 people. Almost all rare diseases do
not have a cure and there is scarce interest, research, or treatment available for them yet most of the people who live with them suffer greatly. Much like other rare diseases most doctors have never heard of CIPO and will never see a patient with it.
The lack of knowledge of this rare disease is one of the main things that contributes to the poor quality of life for people living with it and the high morbidity and mortality rates. For example: It’s common for patients to go through unnecessary surgeries because CIPO looks the same as a mechanical bowel obstruction on imaging tests due to the dilated loops of bowel with air fluid levels. For this reason a surgeon will operate under the impression that there is a mechanical obstruction they need to fix urgently when no real blockage is actually there. Surgery usually worsens GI motility so going through surgeries that are not necessary is a huge blow to the patient’s already poor health. In fact if I let a surgeon operate on me every time they’ve told me they needed to I’d have gone through over a dozen surgeries already.
Chronic Intestinal Pseudo Obstruction can be so severe that it is listed on the governments Compassionate Allowances List. To be included on this list a disease must be terminal or considered to be very serious so it meets disability standards because it is life limiting and disabling.
If I let a surgeon operate on me every time they’ve told me they needed to I’d have gone through over a dozen surgeries already
What Is CIPO?
Important information about Chronic Intestinal Pseudo Obstruction taken from medical journals. I keep these papers with me while hospitalized to help me communicate my condition to clinicians who see me and may not know of CIPO. Click on the images to read full text.
In healthy people food, fluid, and air are propelled forward through the digestive system by involuntary wave-like muscle contractions. This process (the wave-like contractions that push contents through the digestive system) is called peristalsis. In people who have CIPO peristalsis does not work; caused by either a problem with the muscles in the digestive tract (myopathy) or a problem with the nerves in the digestive tract (neuropathy). Since peristalsis is weak, absent, or inefficient the contents in the digestive system are not being pushed through and the body then looks and behaves as if it has an obstruction even though no mechanical obstruction is present. It is very painful for the person living with it and can become very severe.
Visualize It!
Imagine a clog in a pipe and what happens when you try to run water through it. Nothing gets through, or only a tiny bit can trickle through, and so the pipe swells like it wants to burst and contents back up and next thing you know you have water coming up from your drain. This is sort of what it’s like inside of my digestive tract. Since my peristalsis does not work to push contents through it in the way that it should my stomach and intestine swells (dilates) and contents pool and back up resulting in nausea, pain, and vomiting. Having a very dilated gastrointestinal tract is extremely painful. With CIPO the “clog in the pipe” isn’t created by any physical blockage like adhesions, a tumor, hernia, impacted feces, food, etc. Instead my digestive system just does not work – my peristalsis, that important process that pushes contents through the digestive tract, is ineffective.
Life Without Food?
Since I have intestinal failure as a result of CIPO I need to infuse my nutrition into my bloodstream. The solution used to nourish a person who is on this kind of artificial nutrition is called Total Parenteral Nutrition (TPN). I have had to use TPN many times over the years but in the past I would only have to do so for months at a time during particularly bad episodes and then I was able to come off of it. I’d eventually end up back on it once I had lost too much weight again. During the times that I wasn’t on TPN and had to maintain my nutrition by oral intake (or even tube
feeding) it was incredibly difficult to have a goodquality of life because it caused severe pain, nausea, vomiting, distension, and weight loss.
This last time I went back on TPN which was earlier this year I had a BMI of 15.6 and severe protein malnutrition. This life is very difficult because you have to eat to live but in many people who have CIPO you can’t live (as in have a life with much quality) if you eat. I became bed ridden and disabled and could not even tolerate tube feeding because my digestive system was not moving contents through me much at all. The tube feed formula would back up inside of me and cause vomiting, nausea, and pain.
My Life with CIPO
I can’t even begin to tell you how challenging my life has been because of this illness.
CIPO is usually progressive (gets worse over time) and that has been my experience so far. Every year my situation has become worse and in the past 3 years I have declined rapidly. I have been bed ridden and home bound the majority of time within the last couple years as things have progressed. My weight last month was 94.8 pounds (I’m 5’5″) putting my BMI very low.
My small intestine and stomach are extremely dilated. A normal small intestine is 2.5cm in diameter; my small intestine (I don’t have a large intestine) has diffuse dilation and ranges from 6 to 8.5 centimeters in most places. I am always at risk of a bowel perforation which is very scary. In the past decade there hasn’t been a time when my digestive system hasn’t been dilated. This is the radiographic picture of a bowel obstruction but when a person has CIPO there is actually no physical obstruction present. Instead the body behaves as it has an obstruction because the digestive system doesn’t propel food, fluid, and air through it like it should.
Some of my Story with CIPO
- Seven of the past 10 years I have been placed on TPN for various lengths of time. I would always want to come off it because of the risks but once I did my weight would drop again and my quality of life would be very poor.
- I have tried tube feeding twice during two different years but it failed because once formula goes into my digestive tract it just sits there and pools because of my extremely slow motility and how dilated my digestive tract is. I end up with very bad pain, nausea, distention, and vomiting. I have never been able to run my feeds at a rate fast enough to get enough calories.
- I have become so dilated that doctors rushed into my hospital room one night while I was sleeping after my heart rate monitor alerted them. They said an X-ray I had that day showed that my intestine was so swollen that it was pushing up my diaphragm and collapsing part of my lungs which is what was causing my high heart rate. They had to place an NG tube immediately to decompress my digestive tract and resolve the situation.
- I have done barium follow through tests where we looked at my digestive tract the next day under x-ray and there was still barium left in my stomach because it hadn’t moved further. During the most recent barium follow through test I had the radiologist took an X-ray before I drank the barium and freaked out at how dilated my intestine was. She did not want to continue without calling my GI to make sure it was even safe to proceed. Once the test was going we ended it after 7 hours when the barium still hadn’t reached my ileum.
- On several occasions I have vomited up undigested food that was consumed 24-48 hours prior because it hadn’t even left my stomach.
- It took a few years to get diagnosed properly and many years to find a specialist. The first person to mention CIPO was my first surgeon a decade ago. After that my other doctors were just calling my condition small intestinal dysmotility. CIPO was confirmed for sure once I found a motility specialist who treats it.
- I have had a midgut volvulus which can be common in CIPO, especially in pediatric patients. The volvulus resulted in an emergency surgery and my motility got even worse. I did my first trial of tube feeding which didn't work and so I was put back on TPN.
- I am constantly at risk of bowel perforation because of how dilated I am.
- Last year I was going to have a GJ tube placed (a tube that goes into the stomach and small intestine) which is normally a simple outpatient procedure done in interventional radiology. When I went to have it done the radiologist took an X-ray before he began and had to immediately stop the procedure because my stomach was so dilated he was worried about doing the placement. I have to have a surgeon do it but I have been putting it off because I have had so many complications this year I just want a break. The tube is going to be used to drain stomach acid and juices that back up in me and also vent out trapped air. This will hopefully reduce pain, nausea, and vomiting for me.
- I have gone 2 weeks without using the bathroom and I don’t have a colon. Two weeks is pretty extreme for anyone but especially for someone without a colon or rectum and has a J-pouch.
- Nausea, the terrible kind where the room is spinning and you just can’t even lift your head up from the pillow, is almost a nightly occurrence that wakes me in my sleep. It is present during the day too but not as severe.
- I have had to have tubes inserted rectally to drain my intestine and inserted through my nose and swallowed down to decompress my dilated digestive system. Having the rectal tube was very hard on me because I was young and very embarrassed by it.
My belly gets extremely hard and distended because of CIPO. This is much different from your average bloating; everyone gets bloated now and then but this kind of distention is caused by dilated bowel which is abnormal and indicates a problem. My stomach and intestine are diffusely dilated resulting in my small intestine being larger in diameter than normal large intestines are.
Diagnosing and Assessing CIPO
Chronic Intestinal Pseudo-Obstruction affects the small intestine (for some people it is only a segment of the intestine and for others it affects the entire thing) but can be severe enough to also affect the colon, stomach, esophagus, and even other organs like the bladder. CIPO is not to be confused with a paralytic ileus which is much more common, especially after abdominal surgery, but resolves over time.
Diagnosing Chronic Intestinal Pseudo Obstruction involves ruling out mechanical obstructions such as tumors, adhesions, hernias, impacted feces, and so forth. Dilated loops of bowel with air-fluid levels need to be present on imaging tests (CT, MRE, X-ray) without mechanical obstruction in order to diagnose CIPO and problems must persist more than 6 months. Manometry testing can be used to confirm or deny CIPO.
Normal manometry results rule out the possibility of CIPO. Mechanical obstructions look different than pseudo obstructions on manometry so this test can help differentiate between the two. Manometry can also determine whether CIPO is caused by a myopathy or neuropathy as well as which patients may need nutritional support. Transit studies that track the speed at which contents move through the digestive system also help in diagnosing CIPO.
For the past decade (I had never had testing to look prior) every X-ray, CT scan, MRE, barium follow-through test, etc., that I have had done has shown diffuse dilated loops of bowel with air fluid levels which is what a bowel obstruction appears as. Mechanical obstruction has been ruled out over and over and OVER again. Because I have IBD it is very important to rule out anything else that could be creating a problem.
Tests and Procedures I've had to Diagnose and Assess Chronic Intestinal Pseudo Obstruction
X-rays use electromagnetic waves to take pictures of the inside of your body. My images always show dilated loops of bowel with air fluid levels which is what a blockage looks like on imaging. If you have had dilated loops of bowel with air fluid levels during x-rays don’t panic and assume CIPO; there are much, much, more common reasons for that to occur.
Also called gastroduodenal manometry. This tests uses a catheter that has pressure sensors in it that get placed at different spots in the stomach and small intestine and measures contraction amplitude during fasting, after a meal, and after receiving IV erythromycin and an inection of octreotide. This is one of the most helpful tests in diagnosing and assessing the severity of CIPO but it is only performed in few centers in the US.
The results of my manometry were: Abnormal manometry. The findings of this recording are consistent with a generalized visceral neuropathy pattern as evidenced by her antral hypomotility throughout the study, her dysmorphic fasting duodenal activity, her lack of response to meal stimulation, and her blunted responses to pharmaceutical stimulation with both erythromycin and octreotide. There were several isolated bursts seen in the duodenum during fasting as was a dysmorphic retrograde phase III complex. Meal ingestion failed to elicit a fed motor response in either the stomach or small bowel. Erythromycin evoked an abnormally weak and brief response in the antrum and duodenum. Likewise, octreotide produced only a single brief phase III-like response in the distal duodenum although the response in the proximal duodenum was more intense.
CT scans create two and three dimensional images and help get a better look at the digestive tract compared to regular x-rays. This test can aid in ruling out any mechanical reason for a blockage.
Uses a magnetic field to get detailed images of the intestine. MRE’s do not involve any radiation so it is safer than a CT scan.
This test requires you to drink barium and have x-rays taken as it moves through the digestive tract. This test can track the time the barium takes to move to various parts of the digestive tract and it can also help determine if there are other things present that could be causing a blockage.
This test involves consuming a radioactive meal and drink and then having images taken to measure the speed at which the stomach empties it’s contents. My stomach is affected by Chronic Intestinal Pseudo Obstruction and is currently very dilated.
A small flexible tube with a camera on the end enters the body through the mouth (upper endoscopy) or anus (lower endoscopy) to look for abnormalities. Endoscopy can be used to rule out mechanical obstruction and can also be used to decompress the bowel during the exam which I have had done.
A hydrogen breath test does just what you think it would do – measure the amount of hydrogen in the breath. It is used to diagnose bacterial overgrowth which is a common occurrence in people who have CIPO.
I will be having a full thickness biopsy done of my digestive system during surgery. This will give us more clues into the nerve and muscle of my digestive tract and can also point out other possible illnesses causing me problems. I have currently postponed this surgery because I’ve had so many complications this year and I just need a break.
This test is performed in interventional radiology and involves barium entering the body through the anus via enema. The patient lays on an x-ray table and images are taken of the lower portions of the digestive tract to look for abnormalities.
Treating Chronic Intestinal Pseudo Obstruction
Currently there are no treatments that are aimed specifically at CIPO. For the most part treatment revolves around symptom management.
To correct malnutrition and restore weight tube feeding or TPN may be required though only when necessary; especially with TPN which comes with life threatening risks. Tube feeding and TPN can also help reduce pain, distention, and vomiting and provide a better quality of life. A venting G, J, or GJ tube may be used to decompress the bowel and reduce symptoms. Medications for nausea and pain may be required. If CIPO is caused by an underlying condition it is important for the patient to be treating that.
A type of medication known as a prokinetic is normally tried but
usually it’s not very effective in this patient population because they target the stomach or large intestine but don’t have much effect on the small intestine. A transplant may be required for patients who are TPN dependent and have liver failure or limited central venous access sites. Current success rates of transplantation are not very high and the motility problem tends to come back.
Moving Forward
My CIPO has gotten so bad that it has severely impacted my quality of life, especially in the past two and a half years. My BMI is only 15.8 and I am in intestinal failure and rely on TPN for nutrition. Two doctors at two different hospitals have now spoken to me about the possibility of needing a transplant in the future. I am currently trying to gain weight and manage my symptoms to improve my quality of life but managing pain and nausea continues to be a challenge.
I am extremely lucky to be under the care of a great specialist at the University of Michigan. He has literally saved my life and above all is a kind, compassionate human being who doesn’t give up. I am also under the care of a great nutrition team at the University of Chicago who oversees all of my TPN needs now that I am living in Illinois. This is just a overview of what CIPO is and how it has affected my life.
*Last updated in spring of 2017.
