- Posted February 3, 2014 by
This iReport is part of an assignment:
Living with a rare disease?
Superior Mesenteric Artery Syndrome- My Fight
"Superior mesenteric artery (SMA) syndrome is a very rare, life-threatening gastro-vascular disorder characterized by a compression of the third portion of the duodenum by the abdominal aorta (AA)"
I'd like to share some information about a very rare and disabling condition that I've had the misfortune to acquire. It is my hope that through my words and effort by sharing my story, others will be spared pain, and ideally, the medical community will attempt to find better treatment. I'm Janis Mason List.
Superior Mesenteric Artery Syndrome.
Before I share the medical description that you'd find online, let me try to briefly report in my words, through my experiences, and feelings.
I'm a 57 year old female, who had been physically fit, active, educated, hard working, and lived a busy, active life. In my late 40's, I was not your average middle aged woman. I prided myself on healthy eating, weight lifting, and weekly yoga. I won Body Building, Strength & Endurance and Figure competitions. I worked a full time job, and was completing more college classes in the medical field adding to my BA degree.
Three years ago this month, February 2011, my life began to change drastically. What began as abdominal pain, quickly escalated to difficulty eating, constant nausea, and weakness. The weight loss was fast, and shocking. Since I'd been exercising regularly, and had minimal body fat, the weight loss was noticeable. I ignored the first few months, thinking that whatever was wrong would change. It didn't, and I watched my weight drop from 122 to 91 pounds. At 5'5, with only 15% body fat, I didn't have anything to lose.
I lived on Greek yogurt and nibbles of food. My intake was so difficult that drinking an Ensure could take hours. Many doctors, tests, repeated procedures, different physicians, finally leading me to the largest teaching hospital in my state, the University of Michigan. Within weeks, I finally was put on medical leave, diagnosed with not only Nutcracker Syndrome, but, Superior Mesenteric Artery Syndrome. A Nasal Jejunum was tube placed, and home nursing care visited several times a week.
Surgery was planned, a duodenojejostomy, which is the standard for SMAS. I had it performed through a laparoscope. Luckily, I managed to stay alive after hemorrhaging post surgery, with low blood pressure, low oxygenation, and several units of blood. I thought it was over...
My belief was that once I recovered from surgery, my life would return to normal. When it didn't, I was told repeatedly that it would take time. I was still unable to eat, gain weight, I'd lost my ability to walk unaided most of the time. I passed out and fell, injuring myself several times. Eventually, after many other physician visits, tests, feeding tubes, hospitalization for malnutrition, medications for all the symptoms, I remain very much the same. My weight fluctuates between 88-94 pounds at this time. All muscles have atrophied. Luckily, my hair that was lost from earlier malnutrition is returning. Although I'm able to eat more than I could a few months ago, nothing else has truly improved. #lifealteringillness
Thanks to Superior Mesenteric Artery Syndrome being rare, misunderstood, somewhat unknown, complications are not addressed very well. The information available is most likely not accurate, nor does anyone follow patients once we leave the care of our surgeons. Complications are abundant, painful, constant, and possibly misdiagnosed. What I'm saying is not to place blame, merely a sad fact of a rare disorder that had no plan or protocol to follow when the only treatment known has been completed. The DJ surgery. If symptoms continue, or return (which they do) they are addressed as separate or new problems.
My goal and dream is to educate everyone who will listen or learn more about this illness. It is frequently misdiagnosed and treated, or left untreated. What I have not mentioned, is that thanks to technology, there are others like me I've met online. We are fighting for our lives, and the lives of others. At this time, I know of a couple SMAS patients who weigh less than 60 pounds, and without TPN, jejunal tubes, and multiple medications, they wouldn't be alive. Unless something improved soon, they may not survive. The statistics say one in three of us die from this illness. I'm afraid that the number is probably inaccurate since no one follows us. It is malnutrition, heart issues, kidney failure, intestinal failure or even suicide that may be the final diagnosis.
Christopher Reeves suffered from this syndrome after his horrific accident causing paralysis.
Thank you for reading about this illness, and becoming aware. Maybe one person will be able to offer a better treatment option.
The following is the rest of the except from the Wikipedia ( last update January 2013) article on the illness:
Superior Mesenteric Artery (SMA) Syndrome is a very rare, life-threatening gastro-vascular disorder characterized by a compression of the third portion of the duodenum by the abdominal aorta (AA) and the overlying superior mesenteric artery. The syndrome is typically caused by an angle of 6°-25° between the AA and the SMA, in comparison to the normal range of 38°-56°, due to a lack of retroperitoneal and visceral fat. In addition, the aortomesenteric distance is 2-8 millimeters, as opposed to the typical 10-20.
SMA syndrome was first described in 1861 by Carl Freiherr von Rokitansky in victims at autopsy, but remained pathologically undefined until 1927 when Wilkie published the first comprehensive series of 75 patients. Only 0.013 - 0.3% of upper-gastrointestinal-tract barium studies support a diagnosis, making it one of the rarest gastrointestinal disorders known to medical science. With only about 500 cases reported in the history of English-language medical literature, recognition of SMA syndrome as a distinct clinical entity is controversial, though it is now widely acknowledged. SMA syndrome is estimated to have a mortality rate of 1 in 3.
SMA syndrome is also known as Wilkie's syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arterio-mesenteric occlusion. It is distinct from Nutcracker syndrome, which is the entrapment of the left renal vein between the AA and the SMA.
<source-report: "superior mesenteric artery syndrome"; Wikipedia last update January 2014