My journey EDS and CIP by Guest Blogger Lucy Watts

My journey with Chronic Intestinal Pseudo Obstruction (CIP) began around my 15th birthday. We initially thought it was only oral thrush and food intolerances, then IBS, and then things spiralled out of control. I also have Ehlers Danlos Syndrome (EDS), a connective tissue disorder, and secondary Postural Orthostatic Tachycardia Syndrome (POTS).

My gut problems started in the Autumn of 2008. I was overweight (very) but was eating healthily and had lost a pound or 2 each week for a few weeks. We thought things were going well but my tongue had this horrible white coating on it, which would break off in patches leaving red, raw areas of my tongue underneath. It was unsightly and painful too, as were the mouth ulcers I kept getting. No amount of nystatin would clear the "thrush" up, nor could we stop the mouth ulcers from developing. It got so bad that I began cutting foods out of my diet which seemed to trigger or exacerbate them, namely milk, bread, sugary foods and limited the acidic fruits I ate as much as I could - hard for someone who loved fruit. This continued through the early part of 2009, adding abdominal pain, early fullness, nausea and trouble swallowing. 

Although we did not know it at the time, I ate my last meal in March 2009. It was potato with baked beans, and I struggled to eat it, not even managing half. Between March and July I only managed to eat 1 small bowl of carrots per day, which were cooked until they were so soft that I could wash them down with fluid. My GP organised for me to have an abdominal ultrasound, which inevitably came back clear. When my symptoms did not improve, an endoscopy was arranged which was also clear. Biopsies taken at the time were, you guessed it, clear. We had no answers as to what was going on or why I was having such difficulty.

In August things took a turn for the worse. I was now eating nothing, drinking very little and in bed all of the time, exhausted. My mum used to come in to check on me fearing I wouldn't be breathing. I was admitted to hospital after a trip to A&E (ER) malnourished, severely dehydrated and with my kidneys on the brink of failing. I had lost 6 stones (84 pounds) in as many months, which took me down to a healthy weight for my height, and I think my appearance counted against me, as I looked healthy. After proving that I did not have an eating disorder, my paediatrician signed off on an NG tube to begin enteral feeding. I was started on medication to speed up my gut to help with the constipation, Movicol, Lactulose and Senokot, and the nurses spent a good few days flushing my body with fluid to get my kidneys working again. I came home a week later, NG still in place. 3 months later my PEG was inserted for long term enteral nutrition.

My problems didn't end there, however. The weight loss continued, albeit slowed right down, and I still struggled with pain, especially related to the feeds. I was needing ever increasing doses of Movicol to keep my bowel moving, and I wasn't tolerating my feeds very well. At the advice of my rheumatologist, who deals with my EDS, I was referred to a top Neurogastroenterologist called Professor Qasim Aziz. He specialises in unusual and difficult to treat gut motility problems, especially those seen in patients with EDS. I saw him for the first time in April 2010, and have been seeing him ever since. Tests he ordered, including a small bowel manometry, oseophageal manometry and barium swallow, showed generalised gut dysmotility. He gave me a course of erythromycin, followed by starting a daily probiotic called VSL #3 as he was sure I had Small Intestine Bacterial Overgrowth (SIBO). I was also started on Domperidone to help with gut motility.

My gut problems slowly continued to deteriorate, and I was losing weight more and more rapidly. We tried many different high calorie powders and fat emulsions to no avail. We also tried putting an extension through my PEG so I could be fed into my Jejunum (small bowel) but this was not successful either. 

By May 2011 my weight had dropped to 7 stone (98 pounds) - extremely low for my 6ft tall body - taking my total weight loss to 11 and a half stones (165 pounds) in less than 3 years. My red blood cells, white blood cells and platelets were low, as was my potassium, and I was very dehydrated. I was admitted to the hospital where my GI team are based (The Royal London Hospital in Whitechapel, London, UK) and was started on TPN on the 13th May 2011, with my Hickman line being placed a week later. After 5 weeks in the hospital I was discharged and we were trained in how to administer TPN and care for my line at home. My weight had steadily increased ever since; in fact, I've gone the opposite way now and need to lose a bit of weight. It's hard to believe I've been on TPN for over a year.

My gut was still causing me a lot of trouble and I kept ending up in the hospital with bowel obstructions. The decision was made to have an Ileostomy formed, and so in February this year I had the operation to form the stoma. It was definitely a step in the right direction; my surgeon said that it could only be one more obstruction until my colon perforated. It was so severely impacted because nothing was moving. I was taking 36-40 sachets of movicol every week to try and flush it out but it was going around the impaction instead of clearing it. Even the dreaded bowel prep did not work.

Unfortunately my small bowel keeps "obstructing" (pseudo-obstructing, there is no actual blockage) which keeps landing me in the hospital. They usually have to put a catheter through my stoma into my small bowel to drain it off, give me IV fluids to replace what I've lost and monitor me before sending me home. I am currently waiting to see my team, possibly as an inpatient, to decide how we move forward.

My bladder is also affected by both EDS and CIP, it holds up to 2 litres and I have a constant UTI which we cannot seem to get rid of. I have just been started on a 3 month antibiotic course, which will alternate every 2 weeks between different antibiotics, and I might be having Botox injected into my pelvic floor to try and help me to go. I currently self catheterise when I am unable to urinate, but I don't do it as frequently as I should.

So this is my story so far with CIP. I am at the severe end of the spectrum, in intestinal failure, but I hope my story will help others. I want you all to know that you are not alone. I have met some wonderful people through my illnesses, and despite the many downsides to this illness I can say, with my hand on my heart, that I have had some wonderful experiences. I regret that I am too ill to pursue a career in medicine, as I dreamed of doing, and that I am too ill to get back into education or have a career at the moment, but I am hopeful for the future.

May today be a good day for you.

For those who do not know about my other conditions, below is a description of what they are.

Ehlers Danlos Syndrome is a connective tissue disorder caused by a defect in the protein collagen. Collagen provides the structural framework for the body, connects body tissues (e.g. tendons, which connect muscle to bone), protects the organs and stores energy. It is often described as the "glue" of the body and is present in bone, organs, blood vessels, cartilage, muscles, tendons, ligaments and corneas. When there is a defect in collagen, connective tissues are weak and stretchy. Think over chewed chewing gum: when you stretch it, it does not spring back; it tears easily; and the more you stretch it the weaker it gets. It causes joint hypermobility, joints that dislocate easily and frequently, low muscle tone, early osteoarthritis, flat feet, joint deformities, weak blood vessels, bowel and bladder problems and problems with vision, and many more problems which are widespread and can be debilitating.

Secondary to Ehlers Danlos Syndrome I have a form of Dysautonomia, which is dysfunction of the autonomic nervous system. The autonomic nervous system controls all the unconscious functions in the body: heart rate, blood pressure, digestion, temperature regulation/perspiration, respiratory rate, salivation, urination and sexual arousal. The form of Dysautonomia I have is called Postural Orthostatic Tachycardia Syndrome (POTS), which is an increase in heart rate of at least 30 beats per minute upon standing or changing posture, often accompanied by a drop in blood pressure. It means I spend a lot of my time laying down, as my body doesn't cope well with sitting up and I can only stand to transfer with help. I am on medication to control this, but they have not had the desired improvement.