With more than three decades of experience as a nurse and nurse manager, Carol Bolton of Exeter, Calif., was acquainted with most medical conditions. But in 2004 when she began experiencing abdominal pain along with episodes of diarrhea or constipation, she was baffled by what it could be. Most likely, she thought, it was related to grief over the abrupt death of her husband of 35 years.
But when the pain persisted and grew worse, she saw a gastroenterologist, who ordered a computed tomography (CT) scan. Carol was shocked to learn that a mass (about the size of a quarter) had been found in her mesentery. The mesentery, a membrane that anchors the small intestine to the back of the abdominal wall, is comprised of delicate folds or leaves filled with blood vessels and nerves.
Although she had not had a biopsy, she was told there was a possibility that the mass could be cancer of the small bowel. "I left the doctor's office devastated," says Carol. "I went to the car and cried. I looked healthy, and I wasn't convinced I had cancer."
She immediately contacted a surgeon in Fresno, and had a biopsy. The biopsy showed no cancer. Still, to alleviate her symptoms, the surgeon recommended the mass be removed.
Even with surgery on the calendar, Carol was not convinced it was the right course of action.
First choice for second opinion
"After more research and discussion, my surgeon and I agreed that I needed to get another opinion," says Carol, whose first choice for a second opinion was Mayo Clinic in Rochester, Minn. In 1977, Carol had brought her young daughter to Mayo for surgery to remedy a rare birth defect. This prior experience was the deciding factor in promptly scheduling a Mayo Clinic appointment.
At Mayo Clinic, part of diagnosing Carol's condition was ruling out what it wasn't. "It takes a team of skilled radiologists, pathologists and gastroenterologists to confirm a diagnosis," says Darrell Pardi, M.D., a Mayo Clinic gastroenterologist. Following CT imaging and analysis of a biopsy sample, Dr. Pardi gave Carol a diagnosis: sclerosing mesenteritis (SM).
This rare disease affects the small bowel mesentery with chronic, fibrosing inflammation. Masses that form are known to masquerade as cancers, and have also been associated with pancreatitis and intestinal fibroids. Growths may appear as single or multiple masses, or as a general thickening of the mesentery.
Erratic symptoms also make sclerosing mesenteritis a challenge to diagnose. While many people experience abdominal pain, bloating, distention, diarrhea and weight loss, others with the disease live with no symptoms.
Although the first known case of sclerosing mesenteritis was reported in 1924, research has made few strides toward understanding its underlying cause.
"SM has been difficult to study because it's so rare," explains Dr. Pardi, who conducted a research study of identified cases of SM diagnosed at Mayo from 1982 to 2005. The study — the largest to date anywhere — examined 92 cases.
Although the research did not uncloak the cause of sclerosing mesenteritis, several trails for further research were identified. For one, a prior history of abdominal surgeries was reported in 35 percent of the patients studied. Carol, for instance, had an appendectomy at age 21, and three C-section deliveries. These and other findings drive research.
Most importantly, the research yielded a treatment protocol that has shown to be effective in treating SM.
A treatment that works
Experience has shown that understanding each patient's unique manifestation of sclerosing mesenteritis is essential to staging medical treatment.
"My treatment began with an initial burst of prednisone for several months, which I was tapered off of," says Carol. "Also, I have been on raloxifene (a drug used for post-cancer care and osteoporosis) since my diagnosis in 2005."
The most widely prescribed medical therapy for sclerosing mesenteritis is tamoxifen (known to help prevent the recurrence of breast cancer), typically used in combination with prednisone, which has the effect of easing pain by settling inflammation or causing scars to regress.
Surgery to remove obstructions or growths is another option. The growth of masses is a common feature of SM. Masses frequently tether themselves to nearby bowel tissue, or retract and scar the delicate folds of the mesentery, making patients prone to bowel obstructions, which require surgery.
One of the more serious complications of SM is the growth of masses that encase the mesenteric artery. When this occurs special stents may need to be inserted to keep the artery open.
Finding her own way
Thus far, Carol's SM has been managed with medications. Following her diagnosis and initial treatment in 2005, she resumed her work as a nurse administrator despite pain that she describes as sometimes "excruciating."
"Learning to manage your pain is crucial," says Carol. "My pain is mostly associated with digestion and fast food transit into my small intestine, usually within an hour after eating," she says.
To stave off pain, Carol has learned a new way of eating. "I eat two meals a day, in smaller portions, limiting myself to simple carbohydrates and avoiding fibrous foods. I take supplements to compensate for the nutrients I miss in vegetables and fruits.
"There's no one answer to how to change diet," says Dr. Pardi. "Patients learn to listen well to their own bodies and identify those foods that trigger discomfort."
"I live in spite of the disease"
Carol admits that her new way of eating has changed the experience of dining out, a mainstay of her social life. "You make adjustments that can only be made with the help of a support system," she insists. "You can't do it alone. I have a friend who I can call even in the middle of the night. I've learned I can live within certain limits and enjoy a good quality of life."
As she focuses on managing the symptoms of SM, Carol continues playing harp and piano and quilting. She is actively involved in an international SM Web site to encourage greater awareness of the disease and especially to offer support to others who live with it.
Hope on the frontier
"I can't ask more from Dr. Pardi," says Carol. "He looks me in the eyes, is warm and interested in how I'm doing. I frequently send him emails detailing my status and I think this helps him in research being done at Mayo. If there's a frontier for SM research, the frontier is here at Mayo. If there's a new treatment that develops, I know I'll have access to it here. My confidence is here, and I am hopeful."
I have been suffering from SM for over a year. I couldn't handle taking the prednisone, it made me feel terrible. I have gone to the mayo clinic in Jacksonville. This disease is horrible. My stomach is constantly bloated and I'm in a lot of pain. How does one manage he disease? It only seems to be getting worse.
Hi Missy - We now have 100 in our group and i'd love to add you! Sorry your husband has been diagnosed with this. This disease can wreak havoc for a while as you first enter it sometimes which looks like has happened with you guys. My kiddo (she was diagnosed at age 14 and now 22) has been back inpatient the 7 of the last 11 weeks (2 more hospitalizations) (we have more days inpatient the last 2 years than we do at home again as things have escalated and she's on a new drug trial) - i understand the long stays and bowel obstructions with SM/MP. We have a pretty great group of people from all over the US and other countries. i wasn't able to read your full post - but saw he isn't tolerating tube feeds. My kiddo has been off/on TPN since 2008 - and has been back on full-time the last 2 years. Would love to add you to our group. My email is lisas444 @ yahoo if you want to shoot me an email and i can get your info and add you! Thanks! Look forward to hearing from you!
I have been diagnosed with Sclerosing Mesenteries back in June 2014. I have down 3 separate rounds of prednisone to keep the symptoms under control. Every time I have come off the medications the symptoms only come back and if not are worse. I am currently experiencing abdominal pain, nausea, loss of appetite, as well as bouts of constipation and diarrhea. I do not feel like I am getting better and this has been a daily struggle for the past 2 years ( symptoms since October 2013, diagnosed 2014). I am currently on a 5mg daily of prednisone but my GI specialist wants me to wean of the medications. I have attempted to do so but only got sick in the end and had to go right back to 5mg. Are there currently any specialist in the state of OH that can help with the treatment for this disease? Also has anyone found a cure or is there only symptoms management at this point? Thank you for your help!